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European Association of Urology
H. Riedmiller, P. Androulakakis, D. Beurton, R. Kocvara, U. Köhl
1. Phimosis 5
1.1 Background 5
1.2 Diagnosis 5
1.3 Treatment 5
1.4 References 5
2. Cryptorchidism 7
2.1 Background 7
2.2 Diagnosis 7
2.3 Treatment 7
2.4 References 8
3. Hypospadias 11
3.1 Background 11
3.2 Diagnosis 11
3.3 Treatment 11
3.3.1 Complications 12
3.4 References 13
4. Incontinence 15
4.1 Background 15
4.2 Classification 15
4.2.1 Enuresis 15
4.2.2 Urinary incontinence 15
4.3 Diagnosis 16
4.4 Treatment 16
4.4.1 Nocturnal enuresis 16
4.4.2 Diurnal enuresis 16
4.4.3 Urinary incontinence 16
4.4 References 17
5. Dilatation of the upper urinary tract 19
5.1 Background 19
5.2 Diagnosis 19
5.2.1 Ultrasound examination 19
5.2.2 Voiding cystourethrography 19
5.2.3 Diuresis renography 19
5.2.4 Static renal scintigraphy 19
5.2.5 Intravenous urogram 19
5.2.6 Whitaker’s test 19
5.3 Treatment 20
5.3.1 UPJ-stenosis 20
5.3.2 Megaureter 20
5.3.3 Ureterocele 20
5.3.4 Retrocaval ureter 21
5.3.5 Bilateral hydronephrosis 21
5.4 References 22
6. Urinary tract infection (UTI) 24
6.1 Classification 24
6.2 Diagnosis 24
6.3 Treatment 24
6.3.1 Asymptomatic bacteriuria 24
6.3.2 Acute UTI without pyelonephritis 25
6.3.3 Pyelonephritis 25
6.3.4 Complicated UTI 25
6.3.5 Antibiotic prophylaxis 25
6.4 References 25
7. Reflux 27
7.1 Background 27
7.1.1 Secondary reflux 27
7.2 Classification 27
7.3 Diagnosis 27
7.3.1 Secondary reflux 28
7.4 Treatment 29
7.4.1 Conservative therapy 29
7.4.2 Surgical therapy 29
7.4.3 Endoscopic therapy 29
7.4.4 Open surgery 29
7.4.5 Follow-up 30
7.5 References 30
8. Paediatric nephrolithiasis 33
8.1 Background 33
8.2 Diagnosis 33
8.3 Treatment 33
8.3.1 Conservative treatment 34
8.3.2 Metaphylaxis of paediatric nephrolithiasis 34
8.4 References 34
9. Obstructive pathology of renal duplications:
ureterocele and ectopic ureter 37
9.1 Background 37
9.1.1 Ureterocele 37
9.1.2 Ectopic ureter 37
9.2 Classification 37
9.2.1 Ectopic ureterocele 37
9.2.2 Orthotopic ureterocele 37
9.2.3 Caecoureterocele 37
9.3 Diagnosis 38
9.3.1 Ureterocele 38
9.3.2 Ectopic ureter 38
9.4 Treatment 38
9.4.1 Ureterocele 38
9.4.2 Ectopic ureter 39
9.5 References 39
10. Intersex (abnormal sexual differentiation) 41
10.1 Background 41
10.2 Classification 42
10.2.1 Female pseudohermaphrodite 43
10.2.2 Male pseudohermaphrodite 43
10.2.3 True hermaphrodite 43
10.2.4 Mixed gonadal dysgenesis 43
10.2.5 Pure gonadal dysgenesis 43
10.3 Diagnosis 43
10.3.1 The neonatal emergency 43
10.3.2 Late diagnosis and management 44
10.4 Treatment 45
10.4.1 Genitoplasty 45
10.4.2 Indications for the removal of gonads 45
10.5 References 45
11. Urodynamics 47
11.1 Standardization of investigations 47
11.1.1 Residual urine 47
11.1.2 Uroflowmetry 47
11.1.3 Cystometry 47
11.1.4 Video-urodynamic evaluation 47
11.1.5 Urethral pressure profile (sphincterometry) 48
11.1.6 Electromyography of the external sphincter 48
11.2 References 48
12. Abbreviations used in the text 50
At the end of the first year of life, retraction of the foreskin behind the glandular sulcus is possible in only about
50% of boys; this rises to approximately 89% by the age of three. The incidence of phimosis is 8% in 6- to 7-
year-olds and just 1% in males aged 16 to 18 years.
Paraphimosis must be regarded as an emergency situation: retraction of a too narrow prepuce behind
the glans penis into the glandular sulcus may interfere with perfusion of the preputial surface distally from the
constrictive ring and possibly of the glans penis with the risk of consecutive necrosis.
If the prepuce is not or only partly retractable, or shows a constrictive ring on drawing back over the glans
penis, a disproportion between the width of the foreskin and the diameter of the glans penis has to be
assumed. In addition to the constricted foreskin, there may be adhesions between the inner surface of the pre-
puce and the glandular epithelium and/or a frenulum breve. A frenulum breve leads to a ventral deviation of the
glans once the foreskin is retracted.
The diagnosis of paraphimosis is made by physical examination.
Treatment of phimosis in children is dependent on parents’ preferences and can be plastic or radical
circumcision after completion of the second year of life. In case of complicating findings, such as recurrent
urinary tract infections (UTIs) or ballooning of the foreskin during micturition, circumcision should be performed
without delay regardless of the patient’s age. Plastic circumcision has the objective of achieving a widening of
the foreskin circumference with full retractability, while the foreskin is preserved cosmetically. In the same
session, adhesions are released and an associated frenulum breve is corrected by frenulotomy with ligation of
the frenular artery. Routine neonatal circumcision to prevent penile carcinoma is not indicated. Contra-
indications of the operation are acute local infection and congenital anomalies of the penis, particularly
hypospadias, as the foreskin may be required for plastic correction.
As a conservative treatment option, a corticoid ointment (0.05–0.1%) can be administered twice a day
over a period of 20–30 days. This treatment is not indicated in babies and children still wearing diapers, but
may be considered around the third year of life.
Treatment of paraphimosis consists of manual compression of the oedomatous tissue with the sub-
sequent attempt to retract the tightened foreskin over the glans penis. If this manoeuvre fails, a dorsal incision
of the constrictive ring is required. Depending on the local findings, a circumcision is carried out immediately or
can be performed in a second session.
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Advantages and disadvantages of neonatal circumcision [letter]. JAMA 1997; 278: 201.
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The prepuce: specialized mucosa of the penis and its loss to circumcision. Br J Urol 1996; 77: 291–295.
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Cohort study on circumcision of newborn boys and subsequent risk of urinary tract infection. Lancet
1998; 352: 1813–1816.
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Post-circumcision meatal stenosis. N Z Med J 1998; 111: 57–58.
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20. Wiswell TE, Geschke DW.
Risks from circumcision during the first month of life compared with those for uncircumcised boys.
Pediatrics 1989; 83: 1011–1015.
21. Wiswell TE, Tencer HL, Welch CA, Chamberlain JL.
Circumcision in children beyond the neonatal period. Pediatrics 1993; 92: 791–793.
The incidence of maldescensus testis after the first year of life is 1.8–2%. A distinction is made between
abdominal, inguinal or prescrotal testis retention and epifascial, femoral or penodorsal testis ectopy. Sliding
and pendulous (retractile) testes are particular variations of cryptorchidism. Sliding testis with a too short
spermatic cord relocates into its non-physiological position when pulled into the scrotum and then released.
Pendulous (retractile) testis with hypertrophic cremaster muscle fibres is associated with an intermittent
retraction of the usually orthotopic testis.
Maldescensus testis is diagnosed by clinical examination and sonography. Sonography and magnetic
resonance imaging (MRI) may help in localizing the impalpable testis; the accuracy of the latter is 90% for intra-
abdominal testis. Once abdominal retention is suspected, laparoscopy has been established as a diagnostic
and therapeutic procedure. In this procedure, the testis can be localized in its abdominal position and placed
scrotally using the technique appropriate to the anatomical conditions. A human chorionic gonadotrophin
(HCG) stimulation test, as evidence of testosterone-producing testis tissue, should precede operative
exploration for bilaterally impalpable testes.
The objective of treatment is to achieve an orthotopic scrotal position of the testis, before the child’s second
birthday, in order to prevent irreversible damage of spermatogenesis in the affected testis. Hormone therapy
(optional) is applied for testis retention only. It is ineffective for ectopy, but can be helpful for preparation of
local tissue: HCG as an intramuscular injection (9000–30,000 IU in different protocols) or luteinizing hormone
releasing hormone (LHRH) as a nasal spray (400 µg, three times daily). Both methods are effective in about
20–30% of cases. Follow-up is important because the benefits may fail after a period of time.
Surgical orchidofuniculolysis and orchidopexy are first-line treatment options. Pendulous (retractile)
testes are not indicated for surgical repair. Absolute indications for a primary surgical approach are testis
retention after failed hormone therapy or after previous inguinal surgery, testis ectopy and all maldescended
testes with associated pathology (hernia and/or open processus vaginalis). Inguinal access of the spermatic
cord is gained after opening the inguinal canal. Associated pathological conditions (open processus vaginalis,
inguinal hernia) are dealt with in the same session. After the spermatic chord and testis have been freed of con-
nective tissue and cremaster fibres have been resected, the testis is relocated tension free by pexis in the
scrotum. If no testis or spermatic funicle tissue can be found during exploration of the inguinal canal, opening
of the peritoneum and intraperitoneal orchidofuniculolysis is performed. If the spermatic funicle is too short, the
Fowler-Stephens technique (ligation and dissection of the spermatic vessels) can be applied. Pre-conditions
are intact deferent duct and epididymis vessels; these are tested by a temporary clamping of the testicular
artery. In rare cases, auto-transplantation by microsurgical anastomosis of the testis vessels with the epigastric
vasculature can be considered.
Table 1:Management of cryptorchidism
Physical examination
Detectable Unilateral undetectable Bilateral undetectable
MRI (optional) + HCG stimulation
Therapy Laparoscopy Intersex ?
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4. Bourguignon JP, Vanderschueren Lodeweyckx M.
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11. Davenport M.
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12. Davenport M, Brain C, Vandenberg C, Zappala S, Duffy P, Ransley PG, Grant D.
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13. De Boe V, De Backer A, Braeckman J.
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14. Docimo SG.
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J Urol 1995; 154: 1148–1152.
15. Duckett JW.
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16. Esposito C, Garipoli V.
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comments]. J Urol 1997; 158: 1952–1954.
17. Fahlenkamp D, Winfield HN, Schonberger B, Mueller W, Loening SA.
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18. Fedder J, Boesen M.
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testicular localization within the first week after birth. Arch Androl 1998; 40: 181–186.
19. Ferro F, Lais A, Gonzalez Serva L.
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20. Gill B, Kogan S.
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21. Gordon N.
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22. Hadziselimovic F.
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23. Hadziselimovic F, Herzog B.
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improves the chance of fertility later in life. J Urol 1997; 158: 1193–1195.
24. Hamm B.
Sonography of the testis and epididymis. Andrologia 1994; 26: 193–210.
25. Kiely EA.
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Br J Clin Pract 1994; 48: 37–41.
26. King LR.
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J Urol 1998; 160: 2457–2460.
27. Lam WW, Tam PK, Ai VH, Chan KL, Cheng W, Chan FL, Leong L.
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preoperative localization of impalpable undescended testes. J Pediatr Surg 1998; 33: 123–126.
28. Lee PA.
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Curr Probl Pediatr 1995; 25: 232–236.
29. Maddern GJ, Sutherland PD.
Laparoscopic exploration for a presumed intra-abdominal testicle. Endosc Surg Allied Technol 1994; 2: 293.
30. McAleer IM, Packer MG, Kaplan GW, Scherz HC, Krous HF, Billman GF.
Fertility index analysis in cryptorchidism. J Urol 1995; 153: 1255–1258.
31. Nane I, Ziylan O, Esen T, Kocak T, Ander H, Tellaloglu S.
Primary gonadotropin releasing hormone and adjunctive human chorionic gonadotropin treatment in
cryptorchidism: a clinical trial. Urology 1997; 49: 108–111.
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33. O’Hali W, Anderson P, Giacomantonio M.
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J Pediatr Surg 1997; 32: 918–920.
34. Palmer LS, Gill B, Kogan SJ.
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Depending on the localization of the external urethral orifice, hypospadias can be subdivided into distal (75%;
glandular, coronary, subcoronary), intermediate (13%) and proximal (12%; penoscrotal, scrotal, perineal) forms.
Differentiation between functionally necessary and aesthetically feasible operative procedures is important for
therapeutic decision-making. As all surgical procedures carry the risk of complications, thorough pre-operative
counselling of the parents is crucial.
Apart from the description of the local findings (position, shape and width of the orifice, size of the penis,
information on the curvature of the penis on erection and inflammation), the diagnostic evaluation includes
assessment of associated anomalies:
Open processus vaginalis (in 9% of cases)
Testis in a high position (in 5% of mild forms of hypospadias; in 31% of posterior hypospadias)
Anomalies of the upper urinary tract (3%)
Severe hypospadias with unilaterally or bilaterally impalpable testis and scrotal transposition require a
complete genetic work up. In case of ambiguous genitalia, retrograde genitography should be performed soon
after birth to exclude an adrenogenital syndrome (AGS).
A thorough physical examination, urinalysis and usually sonography are performed routinely in all forms
of hypospadias. Excretory urogram or voiding cystourethrography (VCUG) are required only when the findings
in the investigations mentioned above are inconclusive. Urine trickling and ballooning of the urethra requires
exclusion of meatal stenosis by careful inspection.
Surgical intervention is recommended for intermediate and more severe forms of hypospadias, and for distal
forms with associated pathology (penile curvature, meatal stenosis and phimosis). In simple distal
hypospadias, cosmetic correction should be performed only after a thorough discussion of the psychological
aspects and clarification of the lack of a functional indication.
The therapeutic objective is to correct the penile curvature, to form a neo-urethra and to bring the neo-
meatus to the tip of the glans penis, if possible. The use of magnifying spectacles and special suture materials,
knowledge of a variety of plastic surgical techniques (use of rotational skin flaps, as well as free tissue transfer),
the handling of dermatomes, wound care and post-operative treatment are essential for a satisfactory
Pre-operative treatment with the local application of testosterone propionate over a period of 4 weeks
can be helpful. For distal forms of hypospadias a range of techniques are available (e.g. Mathieu, MAGPI, King,
Duplay, Snodgrass, Onlay). Along with the ‘skin’ chorda, the connective tissue of the genuine chorda and the
distal corpus spongiosum, running longitudinally under the glans on both sides of the urethral channel, are
usually responsible for the curvature. If there is a residual curvature after chordectomy, and if the remaining
skin channel of the open urethra is thin and of poor circulation, incision or excision of the urethral plate may be
required. In corporeal dysproportion, orthoplasty (modification of Nesbit dorsal corporeal plication) must be
added. Orthoplasty (Nesbit, modified Nesbit, Schröder-Essed) and closure may be considered in a two-stage
The Onlay technique with preservation of the urethral plate and avoidance of circumferential
anastomosis is the method of choice, with low complication rates for moderate and severe hypospadias.
Prerequisite is an intact and well-vascularized urethral plate, or a satisfactory result after the first session with a
straight penis and a well-covered shaft. If the urethral plate is not completely preserved (after excision or
division), a tube-onlay flap or an inlay-onlay flap are used. The two-stage procedure may be an option in severe
hypospadias. If preputial or penile skin is not available, buccal mucosa, bladder mucosa and free skin grafts
can be used.
Only fine absorbable suture materials should be used (6/0–7/0). For blood coagulation, bipolar
instruments are required along with swabs soaked in 1:10,000 epinephrine solution. Glans preparation may be
facilitated by infiltration with a 1:100,000 epinephrine solution. Tourniquets should not be used for longer than
20 minutes.
After preparation of the dorsal neurovascular bundle, modified Nesbit sutures (monofilic non-resorbable
suture material 4/0–5/0, e.g. Goretex, Prolene) are placed with the knots folded in. Urine is drained via a
transurethral or suprapubic catheter. In case of a suprapubic catheter, the neo-urethra should be stented. For
urethral stenting and drainage, an 810 F catheter with multiple side holes is used ending in the bulbar urethra
(not into the bladder). Circular dressings with slight compression, as well as an antibiotic administration have
become established procedures.
3.3.1 Complications
Whereas meatal narrowing after splint removal can be corrected by careful stretching and the Dittel device,
operative revision is needed in cases of a scarred meatus as slitting and stretching are ineffective in the long
term. In urethral stricture, open surgery should be performed after one attempt at internal urethrotomy. With
fistulae, revision should not be planned before a lapse of 6 months. Urethral stricture should be excluded intra-
operatively as a cause of the fistula. To prevent the recurrent fistula formation, a dartos flap or a free tunica
vaginalis patch may be used. Attention should be paid to adequate subsequent cover with mobilized Scarpa’s
Correction of a minor residual curvature, sometimes reported by the parents, should be discouraged, as
it has no functional relevance. It can be easily corrected after puberty, if significant.
Table 2: Algorithm for the management of hypospadia
Diagnosis Intersex
at birth
Paediatric No
urologist reconstruction
(foreskin, hormone therapy)
Distal Proximal
Chordee No Chordee
Urethral plate Urethral plate
cut preserved
MAGPI, Mathieu, King, Tube-onlay Onlay
Duplay, Snodgrass etc. inlay-onlay, local skin, buccal mucosa
two-stage procedure
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6. Baskin LS, Duckett JW, Ueoka K, Seibold J, Snyder HM.
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10. Burbige KA.
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11. Buson H, Smiley D, Reinberg Y, Gonzalez R.
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12. Caldamone AA, Edstrom LE, Koyle MA, Rabinowitz R, Hulbert WC.
Buccal mucosal grafts for urethral reconstruction. Urology 1998; 51: 15–19.
13. De Grazia E, Cigna RM, Cimador M.
Modified-Mathieu’s technique: a variation of the classic procedure for hypospadias surgical repair.
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14. Dubois R, Pelizzo G, Nasser H, Valmalle AF, Dodat H.
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Prog Urol 1998; 8: 1029–1034.
15. Duckett JW.
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16. Fichtner J, Filipas D, Mottrie AM, Voges GE, Hohenfellner R.
Analysis of meatal location in 500 men: wide variation questions need for meatal advancement in all
pediatric anterior hypospadias cases. J Urol 1995; 154: 833–834.
17. Fichtner J, Thüroff J.
Leitlinie zur Hypospadie und therapeutische Empfehlungen. Urologe A 1998; 37: 665–666.
18. Fichtner J, Fisch M, Filipas D, Thüroff JW, Hohenfellner R.
Refinements in buccal mucosal grafts urethroplasty for hypospadias repair. World J Urol 1998; 16: 192–194.
19. Ghali AMA, El-Malik EMA, Al-Malki T, Ibrahim AH.
One-stage hypospadias repair. Experience with 544 cases. Eur Urol 1999; 36: 436–442.
20. Hayashi Y, Mogami M, Kojima Y, Mogami T, Sasaki S, Azemoto M, Maruyama T, Tatsura H,
Tsugaya M, Kohri K.
Results of closure of urethrocutaneous fistulas after hypospadias repair. Int J Urol 1998; 5: 167–169.
21. Kinkead TM, Borzi PA, Duffy PG, Ransley PG.
Long-term follow-up of bladder mucosa graft for male urethral reconstruction. J Urol 1994; 151: 1056–1058.
22. Kocvara R, Dvoracek J.
Chirurgicka uprava hypospadie zivenym lalokem onlay. Rozhl Chir 1995; 74: 322–326.
23. Kocvara R, Dvoracek J.
Inlay-onlay flap urethroplasty for hypospadias and urethral stricture repair. J Urol 1997; 158: 2142–2145.
24. Meyer Junghanel L, Petersen C, Mildenberger H.
Experience with repair of 120 hypospadias using Mathieu’s procedure. Eur J Pediatr Surg 1995; 5: 355–357.
25. Mollard P, Basset T, Mure PY.
Traitement moderne de l’hypospade. J Urol Paris 1996; 102: 9–17.
26. Mollard P, Mure PY.
Hipospadias proximal. Arch Esp Urol 1998; 51: 551–559.
27. Mureau MA, Slijper FM, Slob AK, Verhulst FC.
Psychosocial functioning of children, adolescents, and adults following hypospadias surgery: a
comparative study. J Pediatr Psychol 1997; 22: 371–387.
28. Ohsumi N.
Post-operative compressive penile dressing using fibrin seal (Tisseel) and tulle gauze for hypospadias
repair [letter]. Plast Reconstr Surg 1998; 101: 1737–1738.
29. Palmer LS, Gill B, Kogan SJ.
Endocrine analysis of childhood monorchism. J Urol 1997; 158: 594–596.
30. Perovic S, Vukadinovic V.
Onlay Island Flap Urethroplasty for severe hypospadias: a variant of the technique. J Urol 1994; 151: 711–714.
31. Retik AB, Bauer SB, Mandell J, Peters CA, Colodny A, Atala A.
Management of severe hypospadias with a 2-stage repair. J. Urol 1994; 152: 749–751.
32. Riedmiller H.
Hypospadie 2000. Akt Urol 2000; 31:147–148.
33. Rosenbloom AL.
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34. Smith EP, Wacksman J.
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35. Snodgrass W, Koyle M, Manzoni G, Hurwitz R, Caldamone A, Ehrlich R.
Tubularized incised plate hypospadias repair: results of a multicenter experience. J Urol 1996; 156: 839–841.
36. Snodgrass W.
Tubularized incised plate hypospadias repair: indications, technique, and complications.
Urology 1999; 54: 6–11.
37. Snow BW, Cartwright PC, Unger K.
Tunica vaginalis blanket wrap to prevent urethrocutaneous fistula: an 8-year experience.
J Urol 1995; 153: 472–473.
38. Stock JA, Hanna MK.
Distal urethroplasty and glanuloplasty procedure: results of 512 repairs. Urology 1997; 49: 449–451.
39. Ulman I, Erikci V, Avanoglu A, Gokdemir A.
The effect of suturing technique and material on complication rate following hypospadias repair.
Eur J Pediatr Surg 1997; 7: 156–157.
40. Uygur MC, Erol D, Germiyanoglu G.
Lessons from 197 Mathieu hypospadias repairs performed at a single institution.
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As a result of maturation, children usually become continent during the day-time by the second year and at
night by the fourth year. In cases of persistent wetting, a distinction has to be made between enuresis and
urinary incontinence. Particular attention should be paid to:
Anatomic defects of sphincteric muscle and bladder (e.g. complete epispadias, bladder exstrophy,
urogenital sinus, ectopic ureter)
Impaired innervation of bladder and sphincteric muscle (e.g. myelomeningocele)
Functional disturbances of the bladder and sphincteric muscle.
4.2.1 Enuresis
This is defined as a normal void occurring at an inappropriate or socially unacceptable time or place. Children
with nocturnal enuresis void in bed while asleep and are generally not aroused by the wetting. The condition is
mono-symptomatic and has a clear familial tendency.
Primary nocturnal enuresis
Involuntary bed wetting from the time of birth without an uninterrupted period of at least 6 months.
Secondary (onset) nocturnal enuresis
Involuntary bed wetting after a dry period of at least 6 months.
Nocturnal polyuria enuresis
This describes nocturnal enuresis in children who have urine production in excess of their functional bladder
capacity, as shown by voiding charts.
Diurnal enuresis
Wetting in attention-deficit disorders; voiding is complete; bladder and urethral function are normal.
4.2.2 Urinary incontinence
This is defined as the involuntary loss of urine, objectively demonstrable and constituting a social or hygienic
problem. It denotes a symptom (patient’s recognition of involuntary urine loss), a sign (the objective
demonstration of urine loss) and a condition (the urodynamic demonstration of urine loss). Day-time, night-time
or both day- and night-time incontinence may be encountered. Urinary incontinence is classified according to
Incontinence associated with anatomic abnormalities of the urinary tract
Ectopic ureter, ureterocele, prune-belly syndrome, bladder exstrophy, epispadias, posterior urethral valves and
cloacal abnormalities.
Incontinence associated with neurogenic disorder
Spinal dysraphism, caudal regression condition and other central nervous system (CNS) disorders.
Functional incontinence in non-neuropathic bladder sphincter dysfunction (urge syndrome and dysfunctional
Often associated with recurrent UTIs and occur predominantly in girls.
The basic diagnostic evaluation includes:
History (including family and social history)
Physical, urological and gross neurological examination (urinalysis and culture, specific gravity)
Sonography (residual urine, bladder wall thickness, upper tracts)
Frequency-volume chart (after treatment of infection)
If the basic evaluation does not reveal any pathology, an enuresis (uncomplicated, mono-symptomatic)
can be assumed and no further examinations are required.
Further evaluation is required if any pathology is revealed in the basic evaluation and includes:
Uroflow (if pathological, repeated uroflow and uroflow-electromyography (EMG) study)
VCUG in case of thickened bladder wall and/or residual urine, in case of pathological, but co-ordinated
(Video) urodynamics on suspicion of functional voiding disturbances
Intravenous urogram (optional if sonography is normal)
Examination under anaesthesia (urethrocystoscopy, urethral calibration, mainly for UTIs)
Extended neurological, radiological and psychiatric examinations (including MRI of the spinal cord)
Radionuclide renal study to assess renal function
4.4.1 Nocturnal enuresis (mono-symptomatic)
Therapy is started when the condition becomes distressing and the child becomes motivated to be dry, usually
after the age of 5–6 years. Behavioural therapy including motivation, counselling about regular voiding and
drinking habits, classical conditioning with an alarm-clock, and appropriate handling of constipation, is the first
option. Complete dryness in 70% and considerable improvement in 12% of cases can be achieved by
treatment with 10–40 mg DDAVP (Desmopressin) nasal spray over a maximum period of 6 months. However,
relapse occurs in almost all cases after discontinuation of the medication. Oxybutynin can be helpful in some
cases with wetting at the beginning of the night (uninhibited contractions of the bladder).
4.4.2 Diurnal enuresis (in children with attention disorders)
Support and education of parents, appropriate school placement and pharmacotherapy (tricyclic
antidepressants), usually handled by child psychiatrists, are the treatment options.
4.4.3 Urinary incontinence
If recurrent UTIs are present, long-term antibiotic therapy should be started, initially for 6 months. Urinary
incontinence is treated according to the aetiology and to urodynamic findings.
Incontinence associated with anatomical abnormalities of the urinary tract
Treatment is within the framework of the operative repair of the abnormality.
Incontinence associated with neurogenic disorder
The cornerstone of treatment in young children is oriented towards the protection of renal function and efficient
evacuation of the bladder. When the child becomes motivated to be dry (usually after the age of 5 years), the
persistent urinary incontinence is addressed. Clean intermittent catheterization to ensure an efficient bladder
emptying is mostly applied in patients with detrusor-sphincter dyssynergia. Medical therapy is applied
according to the urodynamic findings:
Detrusor hyperreflexia: oxybutinin, propiverin, tolterodine
Detrusor sphincter dyssynergia: alpha-blockers, polysynaptic inhibitor (baclofenum)
Surgical therapy can be conducted in the case of renal function deterioration, in persistent high filling
detrusor pressure and/or urinary incontinence. Options include bladder augmentation, treatment of sphincter
weakness and formation of a catheterizable channel.
Functional incontinence in non-neuropathic bladder-sphincter dysfunction
Urge syndrome: Bladder rehabilitation (counselling about regular voiding and drinking habits, about the
technique of voiding); pharmacotherapy (oxybutynin, propiverin, tolterodine); intravesical stimulation and
transcutaneous neuromodulation (optional).
Dysfunctional voiding (detrusor sphincter dysco-ordination): Bladder rehabilitation (counselling about regular
voiding and drinking habits, about the technique of voiding, biofeedback, clean intermittent catheterization if
residual urine is significant) and pharmacotherapy (alpha blockers, polysynaptic inhibitor-baclofenum).
Lazy bladder syndrome: Counselling about regular voiding; clean intermittent catheterization; treatment of
constipation and intravesical stimulation.
Hinman syndrome: According to urodynamic examination; counselling about regular voiding, usually clean
intermittent catheterization if emptying not complete.
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3. Elder JS.
Bladder rehabilitation: the effect of a cognitive training programme on urge incontinence.
J Urol 1997; 158: 1642.
4. Hjalmas K.
What’s new in nocturnal enuresis? Old concepts and new knowledge.
European Union of General Practitioners Reference Book - Clinical Care. Kennedy (ed), 1998/99.
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Assessment of lower urinary tract dysfunction in children with non- neuropathic bladder sphincter
dysfunction. Eur Urol 1999; 35: 57–69.
6. Hoebeke P, Raes A, Vande Walle J, Van Laecke E.
Urodynamics in children: what and how to do it? Acta Urol Belg 1998; 66: 23–30.
7. Hoekx L, Wyndaele JJ, Vermandel A.
The role of bladder biofeedback in the treatment of children with refractory nocturnal enuresis
associated with idiopathic detrusor instability and small bladder capacity. J Urol 1998; 160: 858–860.
8. Holland AJ, King PA, Chauvel PJ, O’Neill MK, McKnight DL, Barker AP.
Intravesical therapy for the treatment of neurogenic bladder in children. Aust N Z J Surg 1997; 67: 731–733.
9. Holmdahl G, Sillen U, Bachelard M, Hansson E, Hermansson G, Hjalmas K.
The changing urodynamic pattern in valve bladders during infancy. J Urol 1995; 153: 463–467.
10. Imada N, Kawauchi A, Tanaka Y, Watanabe H.
The objective assessment of urinary incontinence in children. Br J Urol 1998; 81 Suppl 3: 107–108.
11. Kaplinsky R, Greenfield S, Wan J, Fera M.
Expanded followup of intravesical oxybutynin chloride use in children with neurogenic bladder.
J Urol 1996; 156: 753–756.
12. Kjolseth D, Madsen B, Knudsen LM, Norgaard JP, Djurhuus JC.
Biofeedback treatment of children and adults with idiopathic detrusor instability.
Scand J Urol Nephrol 1994; 28: 243–247.
13. Loening Baucke V.
Urinary incontinence and urinary tract infection and their resolution with treatment of chronic
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14. Madersbacher H, Schultz-Lampel D.
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15. Nijman RJ.
Pitfalls in urodynamic investigations in children. Acta Urol Belg 1995; 63: 99–103.
16. Norgaard JP.
Technical aspects of assessing bladder function in children.
Scand J Urol Nephrol Suppl 1995; 173: 43–6; discussion 46–47.
17. Norgaard JP, Van Gool JD, Hjalmas K, Djurhuus JC, Hellstrom AL.
Standardization and definitions in lower urinary tract dysfunction in children. International Children’s
Continence Society. Br J Urol, 1998. 81 Suppl 3: 1–16.
18. Opsomer RJ, Clapuyt P, De Groote P, Van Cangh PJ, Wese FX.
Urodynamic and electrophysiological testing in pediatric neurourology. Acta Urol Belg 1998; 66: 31–34.
19. Schultz-Lampel D, Thüroff JW.
Enuresis. Urologe A 1997; 36: W265–274.
20. Shoukry MS, El Salmy S, Aly GA, Mokhless I.
Urodynamic predictors of upper tract deterioration in children with myelodysplasia.
Scand J Urol Nephrol 1998; 32: 94–97.
21. Starr NT.
Pediatric gynecology urologic problems. Clin Obstet Gynecol 1997; 40: 181–199.
22. Wan J, Greenfield S.
Enuresis and common voiding abnormalities. Pediatr Clin North Am 1997; 44: 1117–1131.
23. Wennergren H, Oberg B.
Pelvic floor exercises for children: a method of treating dysfunctional voiding. Br J Urol 1995; 76: 9–15.
24. Williams MA, Noe HN, Smith RA.
The importance of urinary tract infection in the evaluation of the incontinent child.
J Urol 1994; 151: 188–190.
25. Wojcik LJ, Kaplan GW.
The wet child. Urol Clin North Am 1998; 25: 735–744.
26. Van Gool JD et al.
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27. Yannakoyorgos K, Ioannides E, Zahariou A, Anagnostopoulos D, Kasselas V, Kalinderis A.
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Hydronephrosis is detectable within the uterus by ultrasound from the 16th week of pregnancy. The
commonest causes are ureteropelvic junction (UPJ)-stenosis, megaureters, urethral valve syndrome,
vesicorenal reflux and multicystic renal dysplasia.
5.2.1 Ultrasound examination
Ectasia (anterior-posterior diameter of the renal pelvis, caliceal ectasia), kidney size, thickness of the
parenchyma, cortical echo-pattern, width of the ureter, bladder wall thickness and residual urine are assessed
during ultrasound examination. With a diameter of the renal pelvis greater than 15 mm, obstruction of the upper
urinary tract is likely and correction may be considered. The first ultrasound examination of prenatally
diagnosed ectasia of the renal pelvis should be carried out within the first 2 days of life, after 3–5 days and after
3 weeks. A normal ultrasound during the first days of life can be secondary to the oliguria of the newborn.
5.2.2 Voiding cystourethrography (VCUG)
Of patients with UPJ-stenosis or megaureter, 14% show a vesicorenal reflux (VRR) at the same time. Reflux
should be verified or ruled out by conventional VCUG pre-operatively. Isotope VCUG (lower exposure to
radiation) is used for follow-up.
5.2.3 Diuresis renography
Because of its low radiation exposure, Tc99m-MAG3 is the radionuclide of choice in diuresis renography. The
examination is carried out after standardized hydration with a transurethral catheter in place. Renal arterial
perfusion, intrarenal cortical transit and excretion of the tracer into the collecting system are measured. If
excretion is impaired, it takes longer for half the maximum activity of the radio-isotope to reach the renal pelvis
(T1/2) after application of furosemide. With rapid absorption of the tracer and prompt washing out effect on
diuresis (T1/2 < 10 min), obstruction is unlikely. Impaired or deteriorating split renal function in newborns or
young infants with upper tract dilatation may be the best indicator of significant obstruction.
5.2.4 Static renal scintigraphy
Renal scintigraphy with di-mercaptosuccinic acid (DMSA) is an ideal method for assessment of renal mor-
phology, acute infectious changes, renal scars and functional impairment, for example in multicystic renal
dysplasia and reflux nephropathy. This investigation should not usually be used within the first 2 months of life.
5.2.5 Intravenous urogram (IVU)
The IVU is an optional examination method and may be performed pre-operatively and in case of inconclusive
findings on sonography. The indication for an IVU in the first year of life is problematical.
5.2.6 Whitaker’s test
Whitaker’s test is carried out as an optional antegrade pressure flow study if diagnosis of obstruction is
obscure. The measurement involves continuous perfusion of the renal pelvis via a percutaneous puncture or, if
necessary, nephrostomy. Shortcomings of Whitaker’s test are the unphysiologically high perfusion rate, lack of
normal ranges in children, dependence on the examiner and the invasiveness of the procedure.
Table 3:Follow-up of prenatally diagnosed dilatation of the upper urinary tract
1. -2.Day of life Ultrasound No dilatation
Dilatation 3. -5. Day of life
repeated ultrasound
Bilateral Unilateral No dilatation
ultrasound control
3. -5. Day of life 2. -3. Week
(urethral valves?) (reflux)
4. –6. Week
IVP (optional)
5.3.1 UPJ-stenosis
Symptomatic UPJ-stenosis requires surgical correction, whereas asymptomatic unilateral UPJ-stenosis can be
subjected to surveillance depending on the grade of obstruction and split renal function. If function does not
improve within 3–6 months in patients with a renal function of less than 10% on the affected side, nephrectomy
should be considered, especially when symptoms occur (e.g. UTI, hypertension, flank pain). Patients with a
function of less than 40% should undergo pyeloplasty. If the partial function is greater than 40%, operative
intervention is indicated only if new symptoms occur or function drops more than 10% with time. Sonography
should be performed every 4 weeks. Renal function scintigraphy must be repeated within 1 year. Children with
a pre-operative partial function below 45% show enhanced growth and an improved overall renal function
5.3.2 Megaureter
According to the official international classification, megaureters are subdivided into primary and secondary,
obstructive and/or refluxive and non-refluxive, non-obstructive. Treatment of the refluxive megaureter is dealt
with in the section on vesicoureteral reflux (VUR). The diagnostic evaluation is the same as in unilateral
hydronephrosis. The degree of obstruction and the split renal function is determined by renal scintigraphy and
IVU. In view of equivalent results of surveillance, surgical intervention based only on the excretory urogram is
now rare. With spontaneous remission rates of up to 85% in primary obstructive megaureters, high drainage by
means of Sober’s or ring-ureterocutaneostomy is no longer justified.
Operative ureterocystoneostomy according to Cohen, Politano-Leadbetter or the Psoas-Hitch-
technique can be considered as an operative measure. Indications for surgical treatment of megaureter are
recurrent infections under prophylactic antibiotic medication, deterioration of split renal function, reflux
persisting for more than 1 year under prophylaxis and significant obstruction.
5.3.3 Ureterocele
Orthotopic ureterocele is a rare finding in children, exclusively observed in females and mostly associated with
a single kidney system. It seldom requires surgical intervention, mostly due to complications (e.g. lithiasis).
Diagnostic evaluation follows the scheme ‘Unilateral hydronephrosis’. VCUG is mandatory prior to a planned
operation. An excretory urogram and cystourethroscopy provide additional information on specific questions.
Ectopic ureterocele is dealt with in the section ’Obstructive pathology of renal duplication’ (see chapter 9).
5.3.4 Retrocaval ureter
A retrocaval ureter is not an anomaly of the ureter but of the vena cava. Diagnostic evaluation follows the
scheme ‘Unilateral hydronephrosis’ (including excretory urogram). In this rare condition, the ureter is divided, its
course corrected and end-to-end anastomosis is performed.
5.3.5 Bilateral hydronephrosis
Bilateral hydronephrosis and megaureter are very rare in girls and require an individual approach, which cannot
be dealt with by an algorithm. In boys, the most frequent cause is infravesical obstruction by urethral valves.
The obstruction may result in the formation of a trabecular bladder with secondary megaureters and
hydronephrosis. After sonography, a VCUG should be carried out without delay. If no pathological findings are
discovered, further evaluation follows the ‘Unilateral hydronephrosis’ scheme. In cases of reflux, diagnostic
evaluation follows the ‘Reflux’ scheme (see chapter 7). In cases of infravesical obstruction with urinary
retention, a suprapubic catheter should be inserted immediately and an antegrade VCUG carried out later.
Sonography and serum creatine controls should be monitored daily. If sonographic findings improve and serum
creatinine falls below 0.6 mg/dL, endoscopic valve resection should be planned between the first and sixth
month of life (depending on the baby’s size and weight). Endoscopy should take place when the traumatization
of the urethra can be minimized. In case of early endoscopic treatment, a stent can be placed in the urethra
(6–8 ch) pre-operatively. If there is no improvement of sonographic findings and the serum creatinine does not
fall below 0.6 mg/dL, supravesical diversion is necessary and delayed reconstructuion 6 to 9 months later
should be planned.
Immediate supravesical diversion is indicated (rarely) in a septic patient with a gross bilateral
dolichomegaureter and renal impairment, or in a gross bilateral dolichomegaureter and renal insufficiency that
does not respond or continues to deteriorate. The endoscopic cold valve ablation (transurethral or suprapubic)
is then performed according to the development of patient conditions and the possibility of spontaneous
voiding. A VCUG and a radiological imaging of the diverted upper urinary tract should be carried out pre-
Table 4: Management of prenatally diagnosed hydronephrosis I
Split renal function < 15% Percutaneous nephrostomy
Recovery No recovery
Reconstruction Nephrectomy
Table 5: Management of prenatally diagnosed hydronephrosis II
Split renal function 15 – 40% Observation by ultrasound
Repeated scintigraphy 3
month of life
Function < 40% Function > 40%
Reconstruction Observation
Table 6: Management of prenatally diagnosed hydronephrosis III
Split renal function: a.p. diameter < 15 mm a.p. diameter > 15 mm
Ultrasound every 3 months Ultrasound every month
Scintigraphy every 6 months Scintigraphy every 3 months
Function decreases < 40%
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21. Rickwood AM, Jee LD, Williams MP, Anderson PA.
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23. Roarke MC, Sandler CM.
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Asymptomatic bacteriuria
Significant bacteriuria can be documented in consecutive urine samples without any symptoms.
The infection is limited to the bladder; irritative symptoms are present, but no systemic symptoms or fever.
Acute pyelonephritis
Febrile infection of the renal parenchyma.
Complicated UTI
Due to a urine transport disturbance, malformation or a relevant bladder voiding disturbance.
Indications for chemical and microbiological urine examination include fever of unknown origin, unclear growth
impairment in infants, unclear abdominal complaints or flank pain, frequency, dysuria, smelly urine and gross
haematuria. In infants and small children, urine is usually collected in a bag attached to the external genitalia. A
positive urine culture requires confirmation by suprapubic puncture (withdrawal of urine by transurethral
catheterization is optional). Once the child can void on demand, mid-stream urine is used in boys and
transurethral catheterization in girls.
The urine examination consists of paper-strip tests, microscopy and microbiology. Only the examination
of bladder puncture urine achieves a sensitivity of > 95%. In cases of a positive urine culture, complete blood
count, differential blood count, erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) are
determined together with clinical examination.
Sonography is performed to assess kidney volume, parenchymal echogenicity, thickness and scarring,
anomalies (e.g. duplex system), hydronephrosis, dilatation of the ureter and calculi. Bladder wall thickness,
bladder configuration, dilatation of the ureter (VCUG, to rule out reflux) and residual urine are assessed with a
full bladder. In case of pyelocaliectasis, an IVU should be considered. A VCUG should be carried out after
successful antibiotic therapy.
Table 7: Differential diagnosis of pyelonephritis
Pyelonephritis likely Pyelonephritis unlikely
ESR > 25 mm n. W < 25 mm n. W
CRP > 1 mg/mL < 1 mg/mL
Body temp. > 38.5 °C > 38.5 °C
Leucocytosis/left shift Present Not present
Leucocyte casts in urine Evidence No evidence
Kidney volume Enlarged Not enlarged
ESR = erythrocyte sedimentation rate; CRP = C-reactive protein
6.3.1 Asymptomatic bacteriuria
No treatment is required in children with a normal urinary tract; functional voiding disturbances should be
6.3.2 Acute UTI without pyelonephritis
Trimethoprim/sulphamethoxazole, trimethoprim (mono-), oral cephalosporins or amoxycillin (third choice) is
given for 3–5 days.
6.3.3 Pyelonephritis
Intravenous antibiotic therapy with a broad-spectrum penicillin or cephalosporin is started immediately. In case
of non-sensitive bacteria, a reserve antibiotic agent is chosen. In newborns, treatment should last for 14–21
days. The therapy is then continued orally for 7–14 days. In impaired renal function, the dosage is adjusted
according to the serum creatinine level. Therapy can be stopped when urine culture is sterile and all signs of
inflammation have disappeared. DMSA scintigraphy may be performed 3 months after the end of treatment. In
obstructive pyelonephritis, urinary drainage should be considered as an emergency.
6.3.4 Complicated UTI
Efficient management of complicated UTIs requires appropriate treatment of the underlying condition (e.g.
impaired urinary drainage).
6.3.5 Antibiotic prophylaxis
Nitrofurantoin and trimethoprim are currently considered as first-line treatment; alternatives are amoxycillin or
cephalosporins. Long-term antibiotic prophylaxis is required particularly in children with VRR. Other indications
for antibiotic prophylaxis are recurrent cystitis with or without voiding disturbances, medullary sponge kidneys,
kidney duplication with reflux to the lower pole, ectopic ureters, dilatation of the upper urinary tract, ureterocele
and neurogenic bladder dysfunction (e.g. MMC or tethered chord syndrome).
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VUR or VRR are defined as non-physiological back-flow of urine from the urinary bladder into the ureter or the
renal pelvis. The worst consequence of both primary and secondary VRR is the development of progressive
renal failure secondary to recurrent pyelonephritic episodes and subsequent loss of renal parenchyma.
Ten to 15% of patients with reflux suffer from a renin-dependent hypertension as a sequela of focal
ischaemia due to parenchymal scarring. The probability of hypertension is highly correlated with the extent and
number of parenchymal scars. Reflux is found in 0.5–1% of normal children without UTIs. While in newborns
the incidence of reflux is the same in both sexes, in later life, girls are approximately four times more frequently
affected than boys.
In children with recurrent UTI, the incidence of VUR is significantly higher (about 14–29% of
kindergarten and school girls and about 30% of boys of the corresponding age).
The main symptoms of VUR are recurrent and occasionally febrile UTIs. Radiological investigations
verify uni- or bilateral reflux in 30–50% of cases. A VRR may also make itself apparent in later stages by its
consequences, such as hypertension, renal insufficiency or delayed renal growth. Undetected reflux may lead
to reflux nephropathy.
7.7.1 Secondary reflux
Predecessor of a secondary reflux is not an inborn primary disturbance of the terminal ureter, but rather an
anatomically or functionally obstructive or inflammatory illness of the bladder, or direct damage to a previously
intact orifice. It seems that many refluxes, especially of lower grade I–III, are secondary refluxes and may
disappear with the maturation of the bladder function.
The International Reflux Study Committee introduced a uniform system for the description of reflux, based on
the earlier classification by Heikel and Parkkulainen, in 1985. Furthermore, a standardized performance of a
voiding cystourethrogram was described to allow comparative studies.
Table 8: Grading system for reflux
Grade I Reflux does not reach the renal pelvis; varying degrees of ureteral dilatation
Grade II Reflux reaches the renal pelvis; no dilatation of the collecting system; normal fornices
Grade III Mild or moderate dilatation of the ureter, with or without kinking; moderate dilatation of the
collecting system; normal or minimally deformed fornices
Grade IV Moderate dilatation of the ureter with or without kinking; moderate dilatation of the collecting
system; blunt fornices, but impressions of the papillae still visible
Grade V Gross dilatation and kinking of the ureter, marked dilatation of the collecting system; papillary
impressions no longer visible; intraparenchymal reflux
Apart from reflux grading, a description of the position and morphology of the ureteral orifices (normal, stadium,
horseshoe or golf-hole orifice) are helpful for planning treatment. The position of an orifice should be
categorised as A (trigonal), B, C or D (markedly lateral).
All patients awaiting clarification of a reflux should undergo a basic diagnostic work up, comprising laboratory
tests (kidney function), urine status and urine cultures, together with a detailed medical history and physical
examination. Direct evidence of a reflux or its exclusion, obtained by VCUG or sonography supplements the
initial diagnosis. An optional IVU is performed before surgery and in any case with an inconclusive sonography.
During the examination, a catheter should be in place.
Without exposure to radiation, sonography can provide detailed information on the size of the kidney,
any possible duplicate formations, the shape and size of the collecting system, and also permits assessment of
the renal parenchyma. Detection of reflux itself is only possible by using special contrast media and in case of
gross reflux.
If VCUG or sonography is negative, but clinical evidence of a suspected reflux persists, the examination
should be repeated after an interval; the grade of the reflux is not constant under different conditions of
If residual urine is apparent after micturition, without presence of an infravesical obstruction, a
urodynamic examination should be performed in order to exclude bladder/sphincter dysfunction and thus
secondary reflux.
Evidence of or exclusion of a reflux can also be provided by nuclear medicinal methods. Due to the
much lower exposure to radiation, a radionuclide cystography to verify or to exclude reflux can also be used,
especially for follow-up examinations.
Endoscopic examination is helpful in case of planned operative treatment and can provide evidence of
a pathological configuration of the ureteral orifices and position, as well as the exclusion of infravesical
obstruction. The examination is carried out under anaesthesia and preparation made for subsequent reflux
correction. For any reflux therapy to be performed endoscopically, the configuration of the ureteral orifices is of
greater importance than the reflux grading; in case of a golf-hole orifice, the prospects of success are markedly
Table 9: Algorithm for the stepwise diagnosis of a primary reflux
Basis diagnostic history
physical examination
blood pressure
laboratory tests
urinalysis and culture
Sonography dilatation
renal parenchyma
duplication of the kidney optional IVU dilatation/obstruction
bladder configuration
voiding optional MAG-3 dilatation/obstruction
DMSA split renal function
Uroflow in children over 3 years optional Cystometry
VCUG grade of reflux
reflux in a single or optional Endoscopy infravesical obstruction
duplicated kidney orifices
bladder configuration
ureteral configuration
surgical treatment
Decision making on treatment strategy
medical treatment
IVU = intravenous urogram; DMSA = di-mercaptosuccinic acid; VCUG = voiding cystourethrography.
7.3.1 Secondary reflux
Diagnosis and treatment of the underlying disease are, of course, primary aspects in secondary acquired reflux.
If reflux persists after successful treatment of the underlying condition, therapy of reflux is oriented with respect
to clinical conditions. In order to diagnose a secondary reflux, it has to be ensured that VCUG was carried out
in a non-inflammatory interval and, if necessary, repeated after clearing up the infection. Further diagnosis then
proceeds according to the same rules as primary reflux.
The objective of treatment is the avoidance of late complications, such as reflux nephropathy. Therapeutic
options consist of conservative medical treatment and endoscopic or open surgical procedures. The choice is
influenced by the age of the patient, the grade of reflux, the position or configuration of the ureteral orifices and
the clinical course.
Table 10: Treatment of reflux I
1 year Conservative
1 – 5 year(s) Grade I - III Conservative
Grade IV – V Surgery
> 5 years Boys Indication for surgery is rare
Girls Surgery (due to higher rate of infections,
esp. during pregnancy)
Table 11: Treatment of reflux II
Recurrent febrile infections despite
antibiotic prophylaxis surgery
(not before the age of 6 months)
Additional malformation (double kidney,
Hutch diverticulum, ectopic ureter)
7.4.1 Conservative therapy
The objective of conservative therapy is prevention of febrile UTIs. Along with an ample supply of liquid and
regular complete voidance of the bladder (if necessary with double micturition), a good hygiene and a low
dosage of prophylactic long-term antibiotics are central aspects of conservative therapy.
The assumption that in some patients VRR disappears without surgical intervention justifies a
conservative approach. The chance of a spontaneous improvement only exists, however, for young patients
with a low-grade reflux and without serious pathology of the ureteral orifices. If reflux persists up to an age in
which spontaneous disappearance can no longer be expected, then girls should be submitted to operative
reconstruction. In boys > 5 years, antibiotic prophylaxis may be stopped as the indication for reflux correction
is rare. If febrile UTIs occur under antibiotic prophylaxis, the conservative strategy should be omitted and
surgical intervention should be planned.
7.4.2 Surgical therapy
Surgery should not be performed before the age of 6 months.
7.4.3 Endoscopic therapy
Currently, experience with endoscopic treatment as an operative option is limited.
7.4.4 Open surgery
Various techniques for reflux correction have been described (e.g. Lich-Gregoir, Politano-Leadbetter, Cohen,
Psoas-Hitch), the principle being to lengthen the intramural part of the ureter by submucosal embedding of the
ureter. A high success rate of over 95%, with only a small rate of complications, is shared by all surgical
As a rule, prior to extravesical procedures an endoscopy should be performed, whereas the ureteral
orifices can be directly visualized with intravesical operations. Other important technical details are an
absolutely tension free ureteral anastomosis, as well as meticulous preservation of the blood supply of the
distal ureter. In addition, a sufficient length and width of the tunnel is mandatory.
In case of a bilateral reflux the Lich-Gregoir as well as the Psoas-Hitch procedure should be performed
in two stages to prevent bladder dysfunction.
7.4.5 Follow-up
After surgical reflux correction, patients require peri-operative antibiotic therapy, this being continued as
prophylaxis for 6 weeks post-operatively. Optionally, a VCUG is carried out 3 months post-operatively in order
to prove successful reflux correction. Obstruction of the upper urinary tract is ruled out by sonography on
patients’ discharge and this should be repeated after 4–6 weeks if any sign of obstruction is present. In all other
cases, a routine ultrasound is carried out 3 months post-operatively.
All patients with parenchymal damage at the time of reflux correction should receive a control
scintigraphy 12 months post-surgery. The follow-up examination should include blood pressure controls for
early detection of renal hypertension.
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Stone disease in children is uncommon and stone formation is often associated with metabolic disorders, chro-
nic infections and/or morphological anomalies of the urinary tract. Recurrence rate is only high in cases of
metabolic disorders and may be the initial manifestation of a congenital and subsequently chronic disease. It is
important to realize that stone formation is not a disease in itself, but a consequence of an underlying disorder.
Treatment of paediatric nephrolithiasis must therefore comprise a strategy to clear the actual stone burden
reliably, diagnose and, ideally, eliminate the underlying pathology. Effective measures should be taken to
prevent recurrent disease.
Children are considered to be high-risk patients and need a full metabolic work up. The patient’s history is
particularly important as many metabolic disorders are hereditary [e.g. autosomal recessive disorders
(cystinuria, primary hyperoxaluria, 2,8-dihydroxyadenine and xanthine calculi); autosomal dominant disorders
(renal tubular acidosis, idiopathic hypercalciuria)]. In cases of a positive family history, an unfavourable clinical
course has to be reckoned with.
In children, hypercalciuria with hypercalcaemia is frequently caused by immobilization (e.g. during
fracture treatment). Another rare condition is hyperparathyroidism. As stone formation only occurs in a subset
of immobilized children, a complete metabolic work up should be performed, as other metabolic disorders may
facilitate the manifestation.
It must also be emphasized that the normal values of the excretion rate of lithogenous and inhibitory
agents in adults cannot be applied to children, but requires correction for body weight (mmol/kg/d) as below.
Variation between laboratories must also be considered.
Table 12: Critical values for renal excretion in children: when to start therapy
Calcium > 0.1
Oxalate > 0.0063
Uric acid > 0.063
Citrate* < 0.01
Cystine heterocygotous 0.0058–0.0117
homocygotous > 0.024
Magnesium < 0.045
Phosphate > 0.483
*Please note the presence of citrate splitting bacteria in the urine sample
The first-line treatment of paediatric nephrolithiasis in the absence of obstructive uropathy is extracorporeal
shock wave lithotripsy (ESWL). When planning interventional stone therapy in children the necessity of general
anaesthesia must be taken into account for percutaneous litholapaxy (PNL), ureterorenoscopy (URS) and,
depending on the child’s age, for ESWL. For this reason, the treatment option with the highest probability of
success in a single or at most two sessions should be chosen. In a few cases, this can only be achieved by
open surgery, which, generally, is indicated if obstruction of the urinary tract requires correction in the same
Several factors affect interventional stone treatment in children: size, localization and composition of the
calculus, and urinary tract configuration. With larger stones, the probability of a single ESWL session for
complete clearance decreases. Disintegration is particularly difficult in cystine calculi. The size of the stone
must be viewed in relation to the size of the kidney and the draining system. A stone or a fragment may lead to
serious complications in children because of the narrow ureteral lumen. On the other hand, a relatively large
stone may pass more easily than in adults.
Indications and complications of interventional stone therapy in children are similar to those in adults.
Even problems with Steinstrasse are much less frequent in children. The ESWL treatment of children less than
100 cm in height was initially thought to be impossible. That has changed with special positioning techniques
(e.g. protecting the lungs with polystyrene) and lithotriptors with a more confined shock wave focus. Detrimen-
tal long-term effects on renal function and growth have been ruled out in recent studies.
In small children, general anaesthesia is mandatory. Percutaneous endoscopic procedures can be
performed safely in children beyond their fourth year of life. Ureteroscopy is also an option in the treatment of
nephrolithiasis in children.
8.3.1 Conservative treatment
Conservative stone treatment of paediatric nephrolithiasis follows the same rules as in adult patients (e.g.
medical dissolution of uric acid stones, antibiotic agents for infection stones).
8.3.2 Metaphylaxis of paediatric nephrolithiasis
Metaphylaxis of paediatric nephrolithiasis follows the principles detailed in the AWMF guidelines. (2) Dosage of
metaphylactic drugs, however, requires appropriate adaptation. Examples of adapted dosages are given below.
Table 13: Metaphylaxis in paediatric nephrolithiasis: corrected dosages
Urinary excretion 30–40 mL/kg*d
Sodium/potassium citrate 0.9–2.0 mEq/kg*d
Allopurinol 4 mg/kg*d
Magnesium 5.7 mg/kg*d
Ammonium chloride test 100 mg/kg*d
Metaphylaxis of paediatric nephrolithiasis also includes a high fluid intake, nutritional and dietetic aspects. In
patients with metabolic disease (e.g. cystinuria, renal tubular acidosis, hypercalciuria, rare cases of enzymatic
defects [uric acid]), only a medical metaphylaxis should be considered.
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8. Esen T, Krautschick A, Alken P.
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10. Grasso M, Chalik Y.
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15. Husmann DA, Milliner DS, Segura JW.
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term followup. J Urol 1996; 156: 741–743.
16. Kamoun A, Zghal A, Daudon M, Ben Ammar S, Zerelli L, Abdelmoula J, Chauachi B, Houissa T,
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physique des calculs au diagnostic etiologique. Arch Pediatr 1997; 4: 629–638.
17. Knipper A, Thomas S, Bohle A, Jocham D.
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Ureterocele and ectopic ureter are the two main anomalies associated with a complete renal duplication. At
present, antenatal ultrasonography detects both conditions in the majority of cases and diagnosis is confirmed
at birth by physical examination, radiography and, occasionally, cystoscopy. In other cases, for example in
adults, these anomalies are revealed by clinical symptoms: UTI, disturbances of micturition and urinary in-
9.1.1 Ureterocele
Ureterocele is a cystic dilatation developed on the intravesical part of the ureter corresponding to the upper
pole of a renal duplication. It is more frequent in females than in males, the overall prevalence being 1 in 4,000
9.1.2 Ectopic ureter
Ectopic ureter is less frequent than ureterocele (1 in 40,000 births), but is again more common in females (male
to female ratio, 1:5). Eighty percent of ectopic ureters are associated with a complete renal duplication.
In females, the ureteral orifice may be located:
In the urethra, from bladder neck to meatus (35%)
In the vaginal vestibule (30%)
In the vagina (25%)
In the uterus and Fallopian tube (rarely)
In males, the ureteral orifice may be located:
In the posterior urethra above the verumontanum and never below the external sphincter (60%)
In the seminal tract (vas deferens, ejaculatory ducts, seminal vesicles) (40%)
Ureteroceles are usually obstructive for the upper pole moiety, but the degree of obstruction and functional
impairment is variable according to the type of ureterocele and upper pole dysplasia. In the orthotopic form,
there is often no or only mild obstruction and frequently the function of the moiety is normal or slightly
impaired; the corresponding ureter may be dilated. In the ectopic form, the upper pole is altered, frequently
dysplastic and hypofunctional or non-functional. The corresponding ureter is a dolichomegaureter. In the
caecoureterocele, the upper pole of the renal duplication is always dysplastic and non-functional.
9.2.1 Ectopic ureterocele
Ectopic ureterocele is the most common form of ureterocele (> 80%) and occurs bilaterally in about 40% of
cases. It is voluminous, dissociating the trigone and slipping into the urethra, and can rarely prolapse through
the urethral meatus. The ureterocele orifice is tight, rarely large, located near the bladder neck either in the
bladder itself or in the urethra below the bladder neck. The ureter corresponding to the lower pole moiety is
raised by the ureterocele and frequently refluxing or compressed by the ureterocele, leading to an obstructive
megaureter. A contralateral renal duplication is associated in 50% of cases. Occasionally, very large
ureteroceles are responsible for reflux or obstruction of the contralateral upper tract.
9.2.2 Orthotopic ureterocele
Orthotopic ureterocele accounts for 15% of cases. It is exclusively observed in females, and is small and
located strictly intravesically. Orthotopic ureteroceles are mostly combined with a single kidney system.
9.2.3 Caecoureterocele
Caecoureterocele is very rare, occurring in less than 5% of cases. It is small, associated with an ectopic ureter
and located in the urethra below the bladder neck.
9.3.1 Ureterocele
Prenatal ultrasound reveals easily voluminous obstructive ureteroceles. In cases of a very small upper pole or a
slightly obstructive ureterocele, prenatal diagnosis will be difficult. If prenatal diagnosis has been impossible, the
following clinical symptoms – beside incidental findings – can reveal the congenital anomaly at birth or later:
1. At birth, a prolapsed and sometimes strangulated ureterocele may be observed in front of the urethral
orifice. In a neonate boy, it might cause acute urinary retention, simulating urethral valves.
2. The early symptom of pyelonephritis in either sex may lead to the diagnosis.
3. Later symptoms can include dysuria, recurrent cystitis and urgency.
In cases of prenatal diagnosis at birth, ultrasonography confirms the ureteral dilatation ending at the
upper pole of a renal duplication. It also demonstrates the presence of an ureterocele in the bladder, with a
dilated ureter behind the bladder.
At this point it is important to assess the function of the upper pole using excretory urography and/or
nuclear renography in the region of interest. Urography might visualize the morphological status of the upper
pole and lower moieties and of the contralateral kidney. A VCUG is mandatory in identifying an ipsilateral or
contralateral reflux and to assess the degree of intraurethral prolapse of the ureterocele.
When the differential diagnosis between ureterocele and ectopic megaureter is difficult,
urethrocystoscopy might reveal the pathology.
9.3.2 Ectopic ureter
Most of the ectopic megaureters are diagnosed primarily by ultrasound. In some cases, clinical symptoms can
lead to diagnosis:
1. In neonates: pyuria and acute pyelonephritis.
2. In older girls: dribbling incontinence with concurrent normal micturition is characteristic. In such cases,
the ectopic orifice may be found in the meatal region. Significant fluor vaginalis may be the equivalent of
incontinence in little girls.
3. In pre-adolescent males: epididymitis is the usual clinical presentation and a palpable seminal vesicle
may be found on digital rectal examination.
Ultrasonography, excretory urography, nuclear studies, VCUG and cystoscopy are the diagnostic tools
to assess the function, to detect reflux and to rule out ipsilateral compression of the lower pole and urethral
obstruction. In the clarification of incontinence in girls, a methylene blue filling of the bladder can be helpful: in
case of loss of uncoloured urine, an ectopic ureter must be present. It is rarely necessary to perform a CT scan
to find a small upper pole moiety invisible on IVU or by sonography.
9.4.1 Ureterocele
The management is controversial between endoscopic decompression, partial nephro-ureterectomy or
complete primary reconstruction. The choice of a therapeutic modality depends on the following criteria:
clinical status of the patient (e.g. urosepsis), age of the patient, renal function of the upper pole, presence or
absence of reflux, obstruction of the ipsilateral ureter and pathology of the contralateral ureter.
Early diagnosis
1. In a clinically asymptomatic child with an ureterocele and non- or hypofunctional upper pole, without
significant obstruction of the lower pole and without bladder outlet obstruction, a prophylactic antibiotic
treatment is given for 3 months until surgery is performed.
2. In the presence of obstruction of the lower pole ureter or of the contralateral ureter, or urethral
obstruction, an immediate endoscopic incision or punction is recommended in combination with
prophylactic antibiotic treatment. After 3 months a re-evaluation is performed.
Re-evaluation: If decompression is effective and there is no reflux (approximately 25%), medical
treatment is stopped and follow-up is based on urine cultures and ultrasound. If decompression is not effective
or significant reflux is present or obstruction of the ipsi- or contralateral ureters and/or bladder neck
obstruction, secondary surgery is necessary varying from partial nephrectomy to complete unilateral
reconstruction (depending on upper pole function).
Late diagnosis
1. Non-functional upper pole, no further pathology: heminephro-ureterectomy is the method of choice.
2. Presence of significant reflux or obstruction: excision of the ureterocele and re-implantation of the
ureter/ureters, heminephro-ureterectomy according to the function of the upper pole.
Bladder outlet obstruction caused by ureterocele: endoscopic incision might be an optional therapy,
with awareness of secondary surgery in the majority of the patients.
9.4.2 Ectopic ureter
In the majority of cases, the upper pole is dysplatic and requires heminephro-ureterectomy. For the exceptional
case in which the upper pole has function worth preserving, ureteral re-implantation into the bladder or
uereteropyelostomy in combination with partial ureterectomy are therapeutic options.
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2. Borer JG, Cisek LJ, Atala A, Diamond DA, Retik AB, Peters CA.
Pediatric retroperitoneoscopic nephrectomy using 2 mm instrumentation.
J Urol 1999; 162: 1725–1729; discussion 1730.
3. Cain MP, Pope JC, Casale AJ, Adams MC, Keating MA, Rink RC.
Natural history of refluxing distal ureteral stumps after nephrectomy and partial ureterectomy for
vesicoureteral reflux. J Urol 1998; 160: 1026–1027.
4. Cendron J, Melin Y, Valayer J.
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5. Decter RM, Roth DR, Gonzales ET.
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6. Di Benedetto V, Montfort G.
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8. Husmann D, Strand B, Ewalt D, Clement M, Kramer S, Allen T.
Management of ectopic ureterocele associated with renal duplication: a comparison of partial
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9. Janetschek G, Seibold J, Radmayr C, Bartsch G.
Laparoscopic heminephroureterectomy in pediatric patients. J Urol 1997; 158: 1928–1930.
10. Jayanthi VR, Koff SA.
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J Urol 1999; 162: 1077–1080.
11. Moscovici J, Galinier P, Berrogain N, Juricic M.
Traitement des urétèrocèles avec duplicité pyélo-urétérale chez l’enfant. A propos de 64 observations.
Ann Urol (Paris) 1999; 33: 369–376.
12. Montfort G, Ruys JM, Coquet M, Roth K, Louis C, Bocciardi A.
Surgical management of duplex ureteroceles. J Ped Surg 1992; 27: 634–638.
13. Pfister C, Ravasse P, Barret E, Petit T, Mitrofanoff P.
The value of endoscopic treatment for ureteroceles during the neonatal period. J Urol 1998; 159: 1006–1009.
14. Rickwood AMK, Reiner I, Jones M, Pournaras C.
Current management of duplex system ureteroceles: experience with 41 patients.
Br J Urol 1992; 70: 196–200.
15. Roy GT, Desai S, Cohen RC.
Ureteroceles in children: an ongoing challenge. Pediatr Surg Int 1997; 12: 44–48.
16. Sherz HC, Kaplan GW, Packer MG, Brock WA.
Ectopic ureteroceles surgical management with preservation of continence. Review of 60 cases.
J Urol 1989; 142: 538–541.
17. Stephens D.
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Ectopic ureter
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Incontinence due to an infrasphincteric ectopic ureter: why the delay in diagnosis and what the
radiologist can do about it. Pediatr Radiol 1998; 28: 942–949.
2. Cendron J, Schulman CC.
Ectopie urétérale. In: Urologie pédiatrique. Flammarion Médecine Sciences: Paris, 1985, 147–153.
3. El Ghoneimi A, Miranda J, Truong T, Montfort G.
Ectopic ureter with complete ureteric duplication: conservative surgical management.
J Pédiatr Surg 1996; 31: 467–472.
4. Komatsu K, Niikura S, Maeda Y, Ishiura Y, Yokoyama O, Namiki M.
Single ectopic vaginal ureter diagnosed by computed tomography. Urol Int 1999; 63: 147–150.
5. Plaire JC, Pope JC, Kropp BP, Adams MC, Keating MA, Rink RC, Casale AJ.
Management of ectopic ureters: experience with the upper tract approach.
J Urol 1997; 158: 1245–1247.
6. Robert M, Ennouchi JM, Chevallier P, Guiter J, Averous M.
Diagnostic des abouchements ectopiques de l’uretère dans le tractus séminal.
Progr Urol 1993; 3: 1028–1033.
7. Smith FL, Ritchie EL, Maizels M, Zaontz MR, Hsueh W, Kaplan WE, Firlit CF.
Surgery for duplex kidneys with ectopic ureters: ipsilateral ureteroureterectomy versus polar
nephrectomy. J Urol 1989; 142: 532–534.
8. Sumfest JM, Burns MW, Mitchell ME.
Pseudoureterocele: potential for misdiagnosis of an ectopic ureter as a ureterocele.
Br J Urol 1995; 75: 401–405.
Disorders of sexual differentiation most often manifest themselves by the abnormal appearance of the external
genitalia as the baby is born, casting doubt on the baby’s gender and a lot of anxiety on his/her family. The
anatomy and physiology of differentiation of the internal and external sexual organs in the foetus is regulated
by factors at three different levels.
Table 14: Factors involved in normal sexual differentiation
Y = Testis determining gene
X = 5α-reductase gene,
Chromosomal factors androgen receptor gene
19 = AMH – receptor gene
Gonadal factors Testis = TST, AMH
TST Wolffian duct
Biochemical DHTST External genitalia
(endocrine) factors Andrenal androgens
TST = testosterone; AMH = anti-Mullerian hormone; DHTST = dihydrotestosterone
With very few exceptions, a normal 46XX or 46XY karyotype leads to normal sex determination and
gonadal differentiation. The presence of the so-called SRY gene (sex-determining region Y) on the short arm of
the Y chromosome leads to differentiation of the foetal gonad into testis, while the absence of one X
chromosome (i.e. 45XO) will lead to early loss of gonadal germ cells and ovarian fibrous degeneration.
The normally functioning foetal testis induces further somatic male differentiation by means of: a) anti-
Mullerian hormone (AMH), secreted by the Sertoli cells, which is responsible for Mullerian duct regression; and
b) testosterone (TST) secreted by the Leydig cells, which is responsible for the maintenance and male
differentiation of the Wolffian ducts and virilization of the urogenital sinus and external genitalia (genital
tubercle), after its intracellular conversion to dihydrotestosterone (DHTST) by 5αreductase.
Table 15: Somatic male differentiation via testicular hormones
Leydig cells
Genital tubercle Male external genitalia
Both AMH and DHTST need the presence of their respective cellular receptors in order to exercise their
function. Disorders of AMH or TST (DHTST) biosynthesis or action may lead to intersex states. The fact that the
androgen-receptor gene lies on the X chromosome explains the virilization of the female foetus in the AGS.
Table 16: Effects of inadequate biosynthesis or action of AMH, TST or DHTST
AMH Retention of Mullerian structures
TST Inadequate differentiation of Wolffian duct structures (i.e.
epididymis, vas deferens, seminal vesicles)
DHTST Inadequate differentiation of genital tubercle inadequate
masculinization of external genitalia (severe hypospadias,
AMH = anti-Mullerian hormone, TST = testosterone, DHTST = dihydrotestosterone
The classification proposed by Allen in 1976 (based on gonadal histology with subclassifications made
primarily by aetiology), has the advantage that gonadal histology is less uncertain to interpret than is
dependence on karyotype or morphology of the external genitalia.
Table 17: Classification of intersex states (modified from Allen 1976). Major categories
grouped by gonadal histology, subclassification by aetiology
I. Ovary only = female pseudohermaphrodite karyotype 46XX
A. Secondary to endogenous androgens (CAH)
21-hydroxylase deficiency
11β-hydroxylase deficiency
3β-ol-dehydrogenase deficiency
B. Secondary to maternal androgens (exogenous ingestion – endogenous production)
II. Testis only = male pseudohermaphrodite karyotype 46XY
A. Secondary to inadequate androgen (TST) production
20α-hydroxylase deficiency
3β-ol-dehydrogenase deficiency
17α-hydroxylase deficiency
17,20-desmolase deficiency
17-ketosteroid reductase deficiency
B. Secondary to inadequate conversion of TST to DHTST 5αreductase deficiency
C. Secondary to inadequate androgen (TST/DHTST) utilization (androgen receptor deficiency)
Complete (testicular feminization)
D. Secondary to deficient AMH hernia uteri inguinalis
III. Testis plus ovary = true hermaphrodite Karyotype 46XY, 46XX, mosaic
IV. Testis plus streak = mixed gonadal dysgenesis karyotype most often 45XO/46XY
V. Streak plus streak = pure gonadal dysgenesis karyotype 45XO (Turner’s syndrome), 46XX, 46XY
VI. Miscellaneous (? Dysgenetic testes ? teratogenic factors)
CAH = congenital adrenal hyperplasia; TST = testosterone; AMH = anti-Mullerian hormone; DHTST =
10.2.1 Ovary only (female pseudohermaphrodite)
46XX patients with normal ovaries and uterus, with virilized external genitalia due to the endogenous
overproduction of androgens by the foetal adrenal glands (CAH, AGS). These patients account for
approximately two-thirds of intersex states in clinical practice.
10.2.2 Testis only (male pseudohermaphrodite)
46XY patients with inadequate virilization of the external genitalia due to deficient biosynthesis of TST,
inadequate conversion of TST to DHTST (lack of 5α-reductase) or inadequate TST/DHTST utilization (lack of
androgen receptors). Also patients with AMH deficiency with adequate male external genitalia and retained
Mullerian structures, i.e. tubes and uterus (hernia uteri inguinalis).
10.2.3 Testis plus ovary (true hermaphrodite)
Patients possess both ovarian and testicular tissue in various combinations. Their karyotype varies, i.e. 46XX,
46XY or mosaic 46XX/46XY. True hermaphrodites make up approximately 10% of intersex cases.
10.2.4 Testis plus streak gonad (mixed gonadal dysgenesis)
The second most common category of intersexuality. Most common karyotype 45XO/46XY mosaicism. The
existing testis is infertile and Mullerian structures may be present on both sides. There is a high risk of
gonadoblastoma of the existing testis after puberty.
10.2.5 Streak plus streak (pure gonadal dysgenesis)
Phenotypic females with bilateral gonadal streaks with three subgroups of karyotypes: 45XO (Turner’s syndrome),
46XX and 46XY. The latter subgroup is particularly prone to malignant degeneration of the streak gonads.
10.3.1 The neonatal emergency
The first step is to explain the situation to the parents fully and kindly, and delay registering and naming the
newborn as long as this is necessary. A careful family history must be taken and the baby carefully examined.
Table 18: Diagnostic work up of neonates with ambiguous genitalia
Good history (family, maternal, neonatal)
Parental consanguinity
Previous intersex disorders or genital anomalies
Previous neonatal deaths
Primary amenorrhoea or infertility in other family members
Maternal exposure to androgens
Failure to thrive, vomiting, diarrhoea of the neonate
Physical examination
Pigmentation of genital and areolar area
Hypospadias or sinus urogenitalis
Size of phallus
Palpable and/or symmetrical gonads
Blood pressure
Buccal smear
Blood: 17-hydroxyprogesterone, electrolytes, LH, FSH, TST, Cortisol, ACTH
Urine: adrenal steroids
HCG stimulation test
Androgen binding studies
LH = luteinizing hormone; FSH = follicle stimulating hormone; TST = testosterone; HCG = human chorionic gonadotrophin
ACTH = adrenocortico tropic hormone
It must be remembered that if one can feel a gonad it is almost certainly a testis, therefore this clinical
finding virtually excludes female pseudohermaphrodites (i.e. CAH). The following laboratory investigations are
Buccal smear (if available with accuracy)
Plasma 17-OH-progesteron assay
Plasma electrolytes
These investigations will give evidence of CAH, which is the most frequent intersex disorder, and if this
is the case no further investigation is needed. Otherwise the laboratory work up proceeds accordingly. The
HCG stimulation test is particularly helpful in differentiating the main syndromes of male pseudohermaphrodites
and in evaluating Leydig cell potential and phallic growth potential.
HCG stimulation test in male pseudohermaphrodites:
Normal increase in both TST and DHTST = androgen insensitivity syndrome
Subnormal increase in both TST and DHTST with increasing androgen precursors = TST biosynthetic
Normal increase in TST but subnormal increase in DHTST = 5αreductase deficiency.
The following rules of thumb can be applied regarding a precise diagnosis:
Positive buccal smear test and no palpable gonads is CAH or female pseudohermaphrodite due to
maternal exposure to androgens. In the case of CAH, immediate medical therapy must be instituted
(corticosteroid substitution, electrolyte and blood pressure monitoring).
Negative buccal smear test and one or two gonads palpable (more often inguinal) – if there are Mullerian
duct structures, then the anomaly concerns gonadal dysgenesis or true hermaphroditism; if there are no
Mullerian duct structures, the anomaly concerns a male hermaphrodite due either to abnormal TST
biosynthesis, inadequate conversion of TST to DHTST (5α-reductase deficiency) or receptor anomaly
(androgen insensitivity syndrome)
The decision for appropriate sex assignment is taken on account of a precise aetiological diagnosis and
the functional potential of the genitalia. Schematically this practical outline can be applied:
Female pseudohermaphrodites (i.e. CAH) should be reared as female since genitoplasty can correct
virilization, and spontaneous puberty, sexual intercourse and fertility are to be expected.
Male pseudohermaphrodites with an inadequate phallus should be given androgenotherapy, i.e. TST,
and those with a poor clinical response should be reared as girls. The only exception is 5α-reductase
deficiency patients, if recognized, in whom a masculine puberty is expected and may be reared as
True hermaphrodites are preferably reared as girls as they have adequate Mullerian structures, i.e.
Mixed gonadal dysgenesis patients with inadequate phallus and intra-abdominal testis are preferably
reared as girls. Male sex may, however, be chosen when the phallus has adequate size and cavernosum
and the testis is palpable, inguinal or scrotal,
Pure gonadal dysgenesis patients are reared as girls.
10.3.2 Late diagnosis and management
Problems arise when cases with disordered sexual differentiation are brought for urological consultation during
late childhood or adolescence. These individuals fail to conform to their sex of rearing, cannot perform sexually
or still have gonads with neoplastic potential.
Female patients with CAH may complain of difficulties during intercourse because of a narrow introitus
and inadequate vaginal opening. The patient should be carefully examined, if needed under general
anaesthesia, and a gynaecologist should be called in to assess the need for revision vaginoplasty.
Male patients with CAH may present with signs of excess androgen production such as rapid growth,
hirsutism and precocious puberty. Steroid treatment will stall their precocious puberty and prevent premature
closure of the epiphyses.
Male pseudohermaphrodites due to 5α-reductase deficiency reared as girls develop at puberty the
secondary characteristics dependent on TST. They become aggressively male in behaviour, their ‘clitoris’
grows, the voice breaks, the body habitus becomes obviously male, whereas there is no acne or facial
hirsutism. Careful urological examination will reveal high lying testes. The question of gender re-assignment at
this stage may become the centre of prolonged discussion, and re-evaluation by a team of specialists,
including a psychiatrist, is mandatory.
Problems of ambiguous genitalia should, in principle, be managed by a team of specialists in order to reach a
prompt and correct diagnosis. Amongst this group, urologists should have an important role and work in close
co-operation with neonatologists, endocrinologists and geneticists, in order to achieve the best possible
management of these patients.
10.4.1 Genitoplasty
Masculinizing genitoplasty incorporates the following stages:
a) Androgenotherapy i.e. administration of TST. Its goal is to restore, if possible, a normal penile size,
hence it should be used in the first months of life. Systemic or local TST can be administered (the latter
mode involves the mother in the treatment).
b) Excision of Mullerian duct structures: firstly because subsequent urethroplasty may cause urine
retention and infection within an existing pseudocolpos, and secondly because casual discovery of a
retained Mullerian structure later in life may question the patient’s gender.
c) Urethroplasty with release of chordee and correction of scrotal deformities.
Orchidopexy of testes that are to be retained.
Feminizing genitoplasty in CAH should be performed once the patient’s general status, blood pressure
and electrolyte balance have been stabilized by systemic steroid substitution. This is usually achieved around
the second or third month of life. The family should be cautioned, however, that re-evaluation of the vaginal
opening and possibly revision vaginoplasty may be needed at puberty.
10.4.2 Indications for the removal of gonads
The gonads of intersex patients should be removed in the following cases:
a) Inappropriate gonadal type for the sex of rearing, as in male pseudohermaphrodites who are to be
reared as girls or true hermaphrodites where discordant gonadal tissue is not needed. This type of
surgery should be performed as early as possible in the neonate.
b) High risk of malignancy (gonadoblastoma/dysgerminoma). Particularly for patients with mixed gonadal
dysgenesis and those with true gonadal dysgenesis and 46XY karyotype. To a lesser extent, for male
pseudohermaphrodites with androgen receptor insensitivity and true hermaphrodites with 46XY
karyotype. The risk for gonadal tumour is real after puberty, hence this type of surgery can be
postponed until that age.
1. Allen TD.
Disorders of sexual differentiation. Urology 1976, 7 (Suppl): 1-32.
2. Androulakakis PA.
Pediatric Urology. Beta Publishing Co: Athens, 1993.
3. Diamond M, Sigmudson HK.
Management of intersexuality. Guidelines for dealing with persons with ambiguous genitalia.
Arch Pediatr Adolesc Med 1997; 151:1046-150.
4. Fékété CN, Lortat-Jacob S.
Management of the intersex child at birth.
Proceedings of Pediatric Uroendocrinology, ESPU Annual Course, 1996, Paris.
5. Imperato-Mc Ginley J, Peterson RE, Gautier T, Sturla E.
Male pseudohermaphroditism secondary to 5α-reductase deficiency - a model for the role of androgens
in both the development of the male phenotype and the evolution of a male gender identity. J Steroid
Biochem 1979; 11: 637-645.
6. Manuel M, Katayama KP, Jones HW.
The age of occurrence of gonadal tumors in intersex patients with a Y chromosome.
Am J Obstet Gynecol 1976; 124: 293-300.
7. Reiner WG.
Sex assignment in the neonate with intersex or inadequate genitalia.
Arch Pediatr Adolesc Med 1997; 151: 1051-1052.
8. Rubin RT, Reinisch JM, Haskett RF.
Postnatal gonadal steroid effects on human behavior.
Science 1981; 211: 1318-1324.
9. Savage MO, Lowe DG.
Gonadal neoplasia and abnormal sexual differentiation. Clin Endocrinol 1990; 32: 519-533.
10. Sohval AR.
“Mixed” gonadal dysgenesis: a variety of hermaphroditism. Am J Hum Genet 1963; 15: 155-158.
11. Whitaker RH, Williams DM.
Diagnostic assessment of children with ambiguous genitalia. Eur Urol Update Series 1993; 2: 2-7.
11.1.1 Residual urine
Except in infants, the normal bladder will empty completely. Unrepresentative results may be obtained after
voiding in unfamiliar surroundings, after voiding on command with a partially filled or overfilled bladder, or in
patients with a high-grade VUR. An isolated finding requires confirmation. Residual volumes can be considered
clinically significant when they represent on repeated occasions volumes of more than 20 mL, or volumes of
>10% of cystometric bladder capacity.
11.1.2 Uroflowmetry
This involves measurement, either sitting or standing, of voided volume, micturition time, average and
maximum urine flow rate. The dependence of the urine flow rate on bladder capacity, which in itself is age-
dependent, has to be taken into account. Assessment of the maximum urine flow rate is only valid if the
bladder is filled to more than half of its total capacity.
11.1.3 Cystometry
Cystometry is the measurement of intravesical and intra-abdominal pressures during storage and voiding
phases. The advantage of transurethral access in school children is based on minimal invasiveness and
recording of valid measurements. In younger children, the undisturbed assessment of the voiding phase
requires a suprapubic measurement catheter equipment (7 Charr, double lumen filling/measurement catheter or
a micro-tip catheter).
With a measurement catheter, rectal pressure sensor and two perineal electrodes to obtain muscular
action potential from the pelvic floor, the investigation is performed in the supine position. The neutral electrode
is applied to the thigh. Before the investigation the measurement catheter, electronic instruments, measurement
tubing, pressure transducer and bubble-free filled input are connected; the external pressure transducer is
adjusted to the symphyseal level and set to zero against atmospheric pressure. The correct position and
function of the measurement equipment are checked by adequate pressure responses on coughing at regular
intervals. The examination should be performed at the earliest 4-6 hours after sedation under infusion of
isotonic NaCl solution or contrast medicine or dye. Bladder sensations (first desire to void; normal desire to
void; strong desire to void; urgency; pain) are recorded; provocation tests (coughing, abdominal pressure rises
and voluntary contraction of the external sphincters) are carried out intermittently. If a neuropathic bladder is
suspected, provocation with 20 mL of ice water with about 50 mL bladder filling may be carried out. Non-
suppressible detrusor contractions are signs of neurogenic disinhibition.
The carbachol test (subcutaneous injection of 0.035 mg of carbachol per kg of body-weight for children)
allows differentiation between neurogenic and myogenic detrusor contractility. In neurogenic bladder
dysfunction, an intravesical pressure rise of more than 25 cmH
O as over-reaction of the bladder denervation to
the cholinergic stimulation after 20-30 minutes is recorded. Intact central inhibition prevents the increase in
tonus of the detrusor.
The following parameters are determined with filling cystometry: residual urine (mL), maximum bladder
capacity (mL), effective bladder capacity (mL), compliance (mL/cmH
O), intravesical pressure (cmH
abdominal pressure (cmH
O), detrusor pressure (cmH
O) and first desire to void. With voiding cystometry, the
following parameters are determined: opening time (sec), pre-micturition pressure (cmH
O), opening pressure
O), maximum voiding pressure (cmH
O), intravesical pressure at maximum urine flow (cmH
O) urine flow
(mL/sec), duration of micturition (sec), flow time (sec), maximum urine flow rate (mL/sec), time of flow increase
(sec), micturition volume (mL), coefficient of resistance (cmH
O /mL/sec), contraction pressure at maximum
urine flow (cmH
O), after contraction.
11.1.4 Video-urodynamic evaluation
Using a contrast media, video-urodynamic evaluation combines fluoroscopic recording of the bladder filling
and emptying with simultaneous urodynamic examination. The necessity of short transillumination periods, par-
ticularly during the filling phase of the urine bladder (e.g. to exclude a VRR during involuntary detrusor
contraction), depends on the demands of the attending physician. Digital X-ray equipment allows a 75%
reduction in radiation exposure compared with conventional techniques. The most important indication for
video-urodynamics evaluation is abnormal voiding (i.e. differential diagnosis of bladder outlet obstruction
versus neuropathic vesico-urethral dysfunction).
11.1.5 Urethral pressure profile (sphincterometry)
Resting or stress profile of the urethra is used to determine and quantify sphincter insufficiency; however, the
method cannot verify subvesical obstruction. Urethral pressure profile measurement is indicated in the
assessment of stress incontinence (e.g. post-traumatic, sinus urogenitalis, myelomeningocele). In children, only
thin micro-tip catheters with urethral and vesical pressure transducer should be used. A continuous withdrawal
is necessary to determine the functional length of the urethra (withdrawal speed of 2-10 cm/min; faster
withdrawal produces higher pressure values). At rest, the urethral profile is determined by continuous
withdrawal of the measuring sensor. In the urethral stress profile, the pressure in the urethra and the pressure
transmission are determined during intermittent intra-abdominal pressure rises (e.g. coughing, Valsalva’s
manoeuvre). After voiding to completion, the bladder should be filled up to 50% of its maximum capacity with a
maximum of 100 mL.
11.1.6 Electromyography (EMG) of the external sphincter
EMG is the study of electrical potentials generated by the depolarization of the external sphincter. For
recording, skin surface electrodes are used in children, positioned left and right from the external anal
sphincter. EMG should be interpreted in the light of the patient’s symptoms, physical findings and urological
and urodynamic investigations.
In normal subjects, there is a gradual increase in EMG activity from the pelvic floor during bladder filling;
at the onset of micturition, there is complete absence of activity.
The finding of increased EMG activity during voiding, accompanied by simultaneous detrusor pressure
and flow changes, is described by the term detrusor-sphincter dyssynergia (in neuropathic bladder/sphincter
dysfunction) or detrusor-sphincter dysco-ordination (in neurologically normal children). Because of the many
artefacts noted and the inability to distinguish electronic ‘noise‘ from true EMG potentials on the recording,
there is no consensus about using the EMG registration.
Table 19: Urodynamics: normal values in children
Storage phase
Maximum cystometric bladder capacity: Age dependent (approx. age x 30)
Residual urine: < 10 % of the bladder capacity
First desire to void: Bladder filling > 60 % of the maximum bladder capacity
Intravesical pressure: 14 cmH
O at the beginning, up to 24 cmH
O at the end of
the filling
Involuntary detrusor contraction: None
Compliance: > 25 mL/cmH
EMG: Stable
Micturition phase (mictiometry)
Maximum urine flow: Age dependent
Medium urine flow: Age dependent
Micturition pressure: < 75 cmH
O (age dependent)
EMG: Fluctuations
EMG = Electromyography
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A new technique for assessing detrusor leak point pressure in patients with spina bifida.
J Urol 1996; 156: 757-760.
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7. Ewalt DH, Bauer SB.
Pediatric neurourology. Urol Clin North Am 1996; 23: 501-509.
8. Hoebeke P, Raes A, Vande Walle J, Van Laecke E.
Urodynamics in children: what and how to do it? Acta Urol Belg 1998; 66: 23-30.
9. Hoebeke P, Vande Walle J, Everaert K, Van Laecke E, Van Gool JD.
Assessment of lower urinary tract dysfunction in children with non- neuropathic bladder sphincter
dysfunction. Eur Urol 1999; 35: 57-69.
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The role of bladder biofeedback in the treatment of children with refractory nocturnal enuresis
associated with idiopathic detrusor instability and small bladder capacity. J Urol 1998; 160: 858-860.
11. Holmdahl G, Sillen U, Bachelard M, Hansson E, Hermansson G, Hjalmas K.
The changing urodynamic pattern in valve bladders during infancy. J Urol 1995; 153: 463-467.
12. Jorgensen JB, Jensen KM.
Uroflowmetry. Urol Clin North Am 1996; 23: 237-242.
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Pressure at residual volume: a useful adjunct to standard fill cystometry. J Urol 1997; 158: 1268-1271.
14. Kaplinsky R, Greenfield S, Wan J, Fera M.
Expanded followup of intravesical oxybutynin chloride use in children with neurogenic bladder.
J Urol 1996; 156: 753-756.
15. Kjolseth D, Madsen B, Knudsen LM, Norgaard JP, Djurhuus JC.
Biofeedback treatment of children and adults with idiopathic detrusor instability.
Scand J Urol Nephrol 1994; 28: 243-247.
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17. Norgaard JP.
Technical aspects of assessing bladder function in children.
Scand J Urol Nephrol Suppl 1995; 173: 43-46; discussion 46-47.
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Standardization and definitions in lower urinary tract dysfunction in children. International Children’s
Continence Society. Br J Urol 1998; 81 Suppl 3: 1-16.
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20. Schultz Lampel D, Thüroff JW.
Enuresis. Urologe A 1997; 36: W265-274.
21. Shoukry MS, El Salmy S, Aly GA, Mokhless I.
Urodynamic predictors of upper tract deterioration in children with myelodysplasia.
Scand J Urol Nephrol 1998; 32: 94-97.
22. Starr NT.
Pediatric gynecology urologic problems. Clin Obstet Gynecol 1997; 40: 181-199.
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Enuresis and common voiding abnormalities. Pediatr Clin North Am 1997; 44: 1117-1131.
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Management of nocturnal enuresis in children with desmopressin and bladder physiotherapy.
Pediatr Surg Int 1998; 13: 281-284.
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Natural filling cystometry in infants and children. Br J Urol 1995; 75: 531-537.
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In view of the diversity and complexity of the addressed issues on the one hand and the necessity to limit the
number of cited articles on the other hand an incorporation of the references into the text seemed
AGS: adrenogenital syndrome
AMH: anti-Mullerian hormone
CAH: congenital adrenal hyperplasia
CNS: central nervous system
CRP: C-reactive protein
CT: computed tomography
DHTST: dihydrotestosterone
DMSA: di-mercaptosuccinic acid
EMG: electromyography
ESR: erythrocyte sedimentation rate
ESWL: extracorporeal shock wave lithotripsy
FSH: follicle stimulating hormone
HCG: human chorionic gonadotrophin
IVU: intravenous urogram
LH: luteinizing hormone
LHRH: luteinizing hormone releasing hormone
MRI: magnetic resonance imaging
PNL: percutaneous litholapaxy
TST: testosterone
UPJ: ureteropelvic junction
URS: ureterorenoscopy
UTIs: urinary tract infections
VCUG: voiding cystourethrography
VRR: vesicorenal reflux
VUR: vesicoureteral reflux
... 28 In the European Urology Association (EUA) Guidelines, the acquired undescended testis is defined as a pinched or spontaneously elevated testis after herniography, and there is no specific follow-up protocol on this subject. 33 The age at which testicular displacement from the scrotum to the proximal was most common was investigated in a retrospective study of 340 children who had undergone orchiopexy. In 151 (44.4%) children with acquired undescended testes, testicular displacement was most common between the ages of 5.8 and 11.1 years. ...
... The use of diagnostic laparoscopy in cases of nonpalpable testes is strongly recommended in both the AUA Guidelines and the EAU Guidelines. 28,33 Diagnostic laparoscopy is frequently used to diagnose intra-abdominal testes. It is also the most accurate method of diagnosing intra-abdominal testis. ...
... Moreover, it is recommended that children with undescended testicles should undergo orchiopexy before 12 months and up to 18 months of age at the latest, regardless of primary/acquired distinction. 33 There is no special protocol in the EAU and AUA Guidelines regarding acquired undescended testis cases diagnosed in the postpubertal period. The AUA Guidelines recommend that "In boys with a normal contralateral testis, surgical specialists may perform an orchiectomy (removal of the undescended testis) if a size has a normal contralateral testis." ...
Full-text available
Undescended testes are a common urological pathology in boys. This condition is categorized according to the examination findings, palpable or non-palpable, and according to the time of diagnosis, congenital or acquired. Orchiopexy is recommended for congenital undescended testes as early as 6 months and up to 18 months at the latest. It is not certain when cases of acquired undescended testes first occur, and most will descend spontaneously into the scrotum. For this reason, when an acquired undescended testis is noticed, the question of whether orchiopexy should be performed immediately or if follow-up is an option becomes important. In this study, we aimed to examine studies on acquired undescended testes in light of the current literature in order to find an answer to this question.
... The 10 patients underwent preoperative uroflowmetry and showed a pattern uroflussimetric with an average Qmax <10 mL (range = 8-12 mL). 6 patients underwent a single calibration to a month after surgery urethroplasty, two were required with multiple calibrations and 2 of meatus plastic [2][3][4]. 3 of these have had a final resolution, 1 continues to present a stenosis, for which we set from about 2 months a program of home care expansion thanks to the compliance of the parents and the small with Nelaton catheters Ch [6][7][8] three times a day. All patients treated for urethral stricture finally performed a calibration uroflowmetry post about three months and then six months with normalization of urinary stream. ...
... The study population included patients with both proximal and distal hypospadias, is subject multi operated from another hospital. According to the literature patients undergoing surgery showed a mean age of about 15 months, which is the best age for 'surgery [4]. Tekgül S et al in the Guidelines of Pediatric Urology indicates the best age for surgical treatment from 6 to 18 months. ...
... Functional assessment in children not yet continents is difficult, they may be asked questions to patients and parents to get information about urination and urinary stream, but more accurate data are obtained through the uroflowmetry, according to the parameters of the International Children's Continence Society (ICCS) [4][5][6][7][8]. Some studies recommend performing a uroflowmetry after the child has reached the continence and the follow-up of patients showing parameters of obstruction to urine flow or borderline values [8][9][10][11]. ...
Full-text available
em>Introduction : Hypospadias is one of the most common birth defects that affect the male urogenital tract. It can present as isolated anomaly, but sometimes can arise in the context of complex disorders of sex development (DSD). These malformations are characterized by a great variety of clinical manifestations and compromise the aesthetic appearance, but also functional and psychological impact that the malformation can determine the patient. Materials and Methods : We conducted a retrospective study of patients undergoing surgery for hypospadias from March 2000 to January 2015. The data was extrapolated from a prospective database. It was considered for each patient: demographics; type of hypospadias; surgical technique; average age for surgery; intraoperative and postoperative complications (early and late). Duckett’s classification was used. Results : 343 urethroplasties were performed. 320 (93%) were primary urethroplasties and 23 (7%) reoperations in patients who had performed many other surgical procedures. 7 patients with megameatus were excluded. The hypospadias have been ranked according to Duckett’s classification, 35 patients had associated diseases. In total were performed: 186 (55%) Snodgrass, 71(21%) Duckett,10 (3%) augmented Duckett, 42 (13%) Magpi, 16 (5%) Duplay, 1 (0,3%) Bracka, 1 (0,3%) was a Bianchi’technique and 5 (1,4%) were Standoli. In 4 patients (1%) were used mixed technique. There were no intraoperative complications. The mean age at surgery was 15 months (range 12-22 months). Postoperative complications were 12%. Long term follow up was done with uroflussimetrie at 3 and 6 months in those who had reached the continence and possible urethral calibrations in those who had submitted a stenosis in post-op. Conclusions : The improvement of surgical techniques, the use of optical amplification tools, the use of suture material (PDS) and the experience gained in recent years have enabled us to optimized the results. Though aware of the potential and actual complications that this type of microsurgical correction can lead to the results we have obtained are comparable to those of major international series and can be considered satisfactory, both from an aesthetic and functional.
... Flexible ureteroscopy (fURS), percutaneous nephrolithotomy (PCNL), and extracorporeal shock wave therapy (ESWL) are commonly used to treat renal stones in children. ESWL is usualy preferred for renal stones <2 cm in diameter (5). However, in children this modality requires general anesthesia (GA) and multiple sessions are needed for complete stone clearance (6). ...
... PCNL is the benchmark for managing renal stones >2 cm in diameter. Although invasive, it provides a high stone free rate (SFR) as a monotherapy (5). However PCNL was not very much favoured in children due to the risks associated with treating smaller kidneys with larger instruments that may result in parenchymal damage and the associated impact on renal function (9). ...
... The mean hospital stay was 2.5 ± 0.8 (2)(3)(4)(5) days. In 7 (13.7%) ...
Full-text available
Objective To assess the efficacy and safety of mini-percutaneous nephrolithotomy (PCNL) for small renal stones 1–2 cm in size in infants less than one year. Material and Methods This descriptive case series was conducted in the department of pediatric urology Institute of Kidney Diseases Peshawar, Pakistan, from March 2019 to March 2022. All the patients underwent mini-PCNL in prone position under GA with 14 Fr access sheath and 10 Fr nephroscope. Stone clearance was assessed by non-contrast CT KUB at 30th postoperative day. Patients with no residual fragments on the non-contrast CT KUB were defined as stone-free. Patients with residual fragments of any size were defined as procedure failure. Safety was determined in terms of intra and postoperative complications. Results A total of 51 infants were included in the study. The mean age of patients was 9.6 + 1.8 (5–12 month). The mean stone size was 15.8 + 2.7 (10–21) mm in length and 12.3 + 2.2 (8–17) mm in width. PCNL mean operative time was 51.6 ± 7.1 (40–70) minutes. Complete stone clearance at one month was observed in 46 (90.2%) patients. Residual fragments were seen in 5(9.8%) patients with a mean size of 1.6 + 0.4 (0.9–2.0) mm. None of the patients required any additional procedure for clearance of stones. In 7 (13.7%) patients, some post-operative complications were observe, all were grade I complications, including fever in 5(9.8%) and transient hematuria in 2(3.9%) patients. Conclusion Mini-PCNL is a safe and effective treatment for renal stones in infants measuring 1–2 cm with high SFR and an acceptable complication rate.
... 3 El tratamiento de primera línea de los pacientes pediátricos con detrusor hiperactivo neurogénico consiste en inicio temprano de cateterismos limpios intermitentes asociado a manejo con antimuscarínicos. 3 En pacientes refractarios al tratamiento con antimuscarínicos, ya sea por intolerancia al medicamento, por efectos adversos o por no respuesta, la aplicación intravesical de toxina botulínica es la siguiente opción de manejo, con diversos estudios que han demostrado su efectividad, 4,5 aunque su uso en población pediátrica por guías de práctica clínica sigue siendo off label. 3 Hasta el momento, hay pocos estudios realizados en Latinoamérica 6,7 que evalúen la efectividad de la toxina botulínica intravesical en esta población, y no encontramos literatura sobre la experiencia con su uso en Colombia. En el presente estudio, describimos nuestra experiencia con la administración de toxina botulínica tipo A en pacientes pediátricos con detrusor hiperactivo neurogénico refractarios al tratamiento con antimuscarínicos y cateterismos limpios intermitentes. ...
... 3 En pacientes refractarios al tratamiento con antimuscarínicos, ya sea por intolerancia al medicamento, por efectos adversos o por no respuesta, la aplicación intravesical de toxina botulínica es la siguiente opción de manejo, con diversos estudios que han demostrado su efectividad, 4,5 aunque su uso en población pediátrica por guías de práctica clínica sigue siendo off label. 3 Hasta el momento, hay pocos estudios realizados en Latinoamérica 6,7 que evalúen la efectividad de la toxina botulínica intravesical en esta población, y no encontramos literatura sobre la experiencia con su uso en Colombia. En el presente estudio, describimos nuestra experiencia con la administración de toxina botulínica tipo A en pacientes pediátricos con detrusor hiperactivo neurogénico refractarios al tratamiento con antimuscarínicos y cateterismos limpios intermitentes. ...
... 8 Actualmente, el tratamiento de primera línea para pacientes con vejiga hiperactiva neurogénica es la realización de cateterismos limpios intermitentes y la administración de antimuscarínicos. 3 Por otra parte, se ha descrito en la literatura que la eficacia de los antimuscarínicos orales se ve afectada principalmente por la falta de adherencia al tratamiento (adherencia del 75% reportada al año en la población pediátrica), 9 siendo el principal factor asociado los efectos adversos, que incluyen resequedad de mucosas, estreñimiento, así como falta de respuesta al tratamiento y/o falla terapéutica hasta en el 17,85% de los casos. 10 Respecto a las opciones de tratamiento de pacientes con vejiga hiperactiva refractarios al manejo con anticolinérgicos, las guías europeas 3 actualmente recomiendan el uso de la toxina botulínica intravesical como una indicación off label, así como la neuroestimulación (únicamente aprobada dentro de ensayos clínicos) y, en casos en los cuales el tratamiento con toxina botulinica falla, está indicada la cistoplastia de aumento. ...
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Resumen Objetivo Describir la experiencia con la administración de inyección de toxina botulínica en niños con vejiga hiperactiva neurogénica refractaria a manejo de primera línea. Materiales y Métodos Estudio observacional descriptivo (serie de casos) que incluyó a 14 niños con diagnóstico de vejiga hiperactiva neurogénica refractarios a tratamiento de primera línea sometidos a administración intravesical de toxina botulínica entre 2015 y 2021; se realizó el seguimiento teniendo en cuenta las variables clínicas, con evaluación de la respuesta y de los eventos adversos. Se reportaron frecuencias absolutas y porcentajes para las variables cualitativas; para las variables cuantitativas, se reportaron medidas de tendencia central y dispersión. Resultados Se incluyeron 6 niños y 8 niñas, con una media de edad 10,1 (desviación estándar [DE]: ± 4,4) años. Todos los pacientes fueron tratados previamente con anticolinérgico y cateterismos limpios intermitentes, con una media de uso de 2,8 (DE: ± 1.0) pañales al día; 11 (78,5%) pacientes tenían antecedente de infección urinaria, 13 (92,8%), estreñimiento, y 2 (15,3%), incontinencia fecal. En la ecografía, 7 (50,0%) pacientes presentaban engrosamiento de las paredes vesicales, y 6 (42,8%), hidronefrosis. Tras el procedimiento, 1 paciente presentó infección urinaria como complicación, 6 presentaron una respuesta completa, 7, respuesta parcial, y 1 paciente no obtuvo respuesta con la primera inyección, con un tiempo promedio efectivo de la terapia 8 (DE: ± 6,3) meses. Una segunda inyección fue necesaria en 6 (42.8%) pacientes, y, de estos, 3 (50%) requirieron una tercera inyección. Conclusión La inyección de toxina botulinica intravesical como terapia de segunda línea de manejo para vejiga hiperactiva neurogénica tiene buenos resultados, con bajas tasas de complicaciones.
... Even those with no neuro-orthopaedic change should have a preliminary urologic evaluation if they have spinal cord anomalies. The initial assessment should outline the type of dysfunction and offer information on the detrusorsphincter condition [29]. With careful examination, followup, and proactive management, the damage to the renal parenchyma acquired postnatally can be avoided [30]. ...
... VCUG provides anatomical information on the lower urinary tract, specifically the presence or absence of VUR. VCUG and UDS should be performed at least 6 weeks after closure of the defect, when it is expected that the spinal shock be overcome [29,30]. Reduced bladder capacity and compliance, as well as high detrusor leak point pressure (DLPP), are urodynamic parameters that predict renal deterioration. ...
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Purpose of Review The term neurogenic bladder (NGB) generally used for neurogenic lower urinary tract dysfunction (NLUTD) refers to storage and/or voiding dysfunction of the lower urinary tract, in patients after a neurological illness. The aim of management of NGB is to preserve renal function, achieving effective bladder emptying, urinary continence, and improving overall QOL. This is possible with detailed history taking, physical examination, relevant investigations, patient education, and an agreed treatment plan and a regular follow-up. Currently in India, there are no guidelines available for management of NGB. Considering India as a developing country and having a diversified health care system where the private sector in addition to the public health system plays an equally important role in delivering health care across the population, and with population distributed asymmetrically on the socioeconomic ladder, it is difficult to develop a single management protocol for management. This review is a step to understand current practices in India and how it differs in management of NGB, globally. Recent Findings The European Association of Urology (EAU) and American Urology Association (AUA) guidelines have been recently updated in 2022 and 2021 respectively. Summary Most clinicians in India follow a set protocol in line with available international guidelines suitable to local available services and expertise keeping in mind the best interest of patients. However, differences in management between concerned clinicians still exist; hence, there is a need for more research and formulating uniform consensus to approach management of neurogenic bladder in India.
Introduction: Unintentional injury is a leading cause of mortality and morbidity in children. There is no consensus on the ideal, discrete management of pediatric renal trauma (PRT). Therefore, management protocols tend to be institution-specific. Objective: This study aimed to characterize PRT at a rural level-1 trauma center and subsequently develop a standardized protocol. Study design: A retrospective review of a prospectively maintained database of PRT at a rural level 1 trauma center between 2009 and 2019 was conducted. Injuries were characterized regarding renal trauma grade, associated multi-organ involvement and the need for intervention. The benefit of patient transfer from regional hospitals and length and cost of stay were evaluated. Results: Of 250 patients admitted with renal trauma diagnosis 50 patients <18 years were analyzed. Of those, the majority (32/50, 64%) had low-grade (grade I-III) injuries. Conservative management was successful in all low-grade injuries. Of 18 high-grade PRT, 10 (55.6%) required intervention, one prior to transfer. Among patients with low-grade trauma, 23/32 (72%) were transferred from an outside facility. A total of 13 (26%) patients with isolated low-grade renal trauma were transferred from regional hospitals. All isolated, transferred low-grade renal trauma had diagnostic imaging before transfer and none required invasive intervention. Interventional management of renal injury was associated with a longer median LOS [7 (IQR = 4-16.5) vs 4 (IQR = 2-6) days for conservative management, p = 0.019)] and an increased median total cost of $57,986 vs. $18,042 for conservative management (p = 0.002). Discussion: The majority of PRT, particularly low-grade, can be managed conservatively. A significant proportion of children with low-grade trauma are unnecessarily transferred to higher level centers. Review of pediatric renal trauma at our institution over a decade has allowed us to develop an institutional protocol which we believe allows for safe and effective patient monitoring. Conclusion: Isolated, low-grade PRT can be managed conservatively at regional hospitals without needing transfer to a level 1 trauma center. Children with high-grade injuries should be closely monitored and are more likely to need invasive intervention. Development of a PRT protocol will help to safely triage this population and identify those who may benefit from transfer to a tertiary care center.
TÓM TẮT Mục tiêu: Thời gian gần đây, điều trị bệnh lý khúc nối bể thận - niệu quản (KNBTNQ) ở trẻ em tại bệnh viện Trung ương Huế có nhiều tiến bộ cũng như có nhiều cách tiếp cận bể thận trong phẫu thuật mở, phẫu thuật nội soi hoàn toàn trong phúc mạc. Bên cạnh đó, chưa có nhiều nghiên cứu có tính hệ thống đánh giá kết quả phẫu thuật tạo hình KNBTNQ tại bệnh viện Trung ương Huế. Vì vậy, chúng tôi thực hiện nghiên cứu (NC) này để đánh giá kết quả phẫu thuật (PT) bệnh lý KNBTNQ ở trẻ em trong vòng ba năm gần đây. Đối tượng và phương pháp: Chúng tôi đã tiến hành nghiên cứu (NC) mô tả tiến cứu các bệnh nhi (≤ 16 tuổi) bị thận ứ nước do bệnh lý KNBTNQ được phẫu thuật tạo hình bể thận - niệu quản theo phương pháp Anderson - Hynes từ tháng 1 năm 2020 đến tháng 10 năm 2022. Kết quả: Chúng tôi đã thực hiện PT tạo hình cho 34 bệnh nhi, trong đó có 31 trường hợp PT mở và 3 trường hợp phẫu thuật nội soi (PTNS) xuyên phúc mạc. 24 bệnh nhi nam (70,6%) và 10 bệnh nhi nữ (29,4%), độ tuổi trung bình là 50,53 ± 49,26 tháng tuổi, nhỏ nhất là 3 tháng tuổi, lớn nhất là 182 tháng tuổi. Triệu chứng lâm sàng chủ yếu là đau vùng hông lưng (70,6%). Thận ứ nước độ 3 chiếm tỷ lệ cao nhất trên siêu âm (61,8%) và CLVT (70,6%). Thời gian trung bình phẫu thuật mở là 81,45 ± 23,74 phút, của phẫu thuật nội soi là 136,67 ± 15,27 phút. Thời gian nằm viện sau mổ trung bình ở BN phẫu thuật mở là 5,20 ± 1,17 ngày, đối với PTNS là 4,15 ± 2,04 ngày. Có 33/34 BN PT thành công, đạt tỷ lệ 97,05%, 1/34 BN có tình trạng tái hẹp, đã được PT tạo hình lại lần 2. Kết luận: Kết quả nghiên cứu cho thấy phẫu thuật bệnh lý KNBTNQ ở trẻ em đúng chỉ định là phương pháp điều trị an toàn và hiệu quả. Phẫu thuật đường ngang sau lưng ở trẻ em có nhiều ưu điểm và an toàn. Phẫu thuật nội soi xuyên phúc mạc cho thấy là phương pháp an toàn, tuy nhiên cần nghiên cứu trên số lượng bệnh nhân lớn để đánh giá được chính xác hơn. ABSTRACT EVALUATE THE OUTCOME OF PYELOPLASTY FOR URETEROPELVIC JUNCTION OBSTRUCTION IN CHILDREN Objectives: Recently, the treatment of ureteropelvic junction obstruction at Hue Central Hospital has made many advances such as there are many approaches for pyeloplastyin open surgery, totally laparoscopic surgery. Besides, there has not been a systematic prospective study evaluating the results of pyeloplastysurgery at Hue central hospital. Therefore, we performed this study to evaluate the surgical outcome of pyeloplasty surgery in children within the last three years. Methods: We conducted a prospective descriptivestudythatincluded pediatric patients (≤16 years old) with ureteropelvic junction obstruction operated by the Anderson - Hynes open and transperitoneal laparoscopic pyeloplasty method from January 2020 to October 2022. Results: We performed open and laparoscopic surgery for 34 pediatric patients, including 31 open and 3 transperitoneal laparoscopic surgery. There were 24 (70,6%) male and 10 (29,4%) female patients, mean age of50,53 ± 49,26 months, the youngest was 3 months, and the oldest was 182 months. Low back pain was the main reason for hospitalization (70,6%). Grade 3 hydronephrosis accounting for the highest percentage of Ultrasound (61,8%) and Uroscan (70,6%). The average time of open surgery was 81,45 ± 23,74 minutes, and laparoscopic surgery was 136,67 ± 15,27 minutes. The average postoperative hospital stay for open pyeloplasty was 5,20 ± 1,17 days, and for laparoscopic pyeloplasty was 4,15 ± 2,04 days. 33/34 patients were evaluated as having successful surgery, reaching the rate of 97.05%; 1/34 patients had restenosis, and had the second reconstructive surgery. Conclusion: Pyeloplasty is a safe and effective treatment for ureteropelvic junction obstruction in children. The dorsallumbotomy transverse incision in children has many advantages and safety. Transperitoneal laparoscopic pyeloplasty in children is a safe method; however, it needs to be studied in a large number of patients for a more accurate assessment.
Background We sought to determine which demographic, clinical and ultrasonography characteristics are predictive of testicular torsion (TT) and to determine factors associated with time to treatment. Methods We retrospectively reviewed all medical records of patients (0–17 years) with acute scrotal syndrome (ASS) who were treated in our hospital in Lithuania between 2011 and 2020. We extracted patients’ demographic data, in-hospital time intervals, clinical, US and surgical findings. TT was determined at surgery or clinically after manual detorsion. Test characteristics of demographic, clinical and US findings for the diagnosis of TT versus other causes of ASS were determined. We performed a multivariate analysis to identify independent clinical predictors of torsion, and factors associated with surgical delay. Results A search of medical records yielded 555 cases: 196 (35%) patients with TT and 359 (65%) patients with other ASS causes. Multivariate logistic regression analysis showed that age between 13 and 17 years (OR 8.39; 95% CI 5.12 to 13.76), duration of symptoms <7 hours (OR 3.41; 95% CI 2.03 to 5.72), palpated hard testis (OR 4.65; 95% CI 2.02 to 10.67), scrotal swelling (OR 2.37; 95% CI 1.31 to 4.30), nausea/vomiting (OR 4.37; 95% CI 2.03 to 9.43), abdominal pain (OR 2.38; 95% CI 1.27 to 4.45) were independent clinical predictors of TT. No testicular blood flow in Doppler US had a specificity of 98.2% and a positive predictive value of 94.6%. However, 75 (41.7%) patients with TT had normal testicular blood flow, yielding low sensitivity (58.3%) and negative predictive value of 81.3% for this US finding. In-hospital waiting time for surgery was longer in patients with TT with normal testicular blood flow by USS (195 min) compared with no blood flow (123 min), p<0.01. Higher orchiectomy rates were associated with longer duration of symptoms (p<0.001) and longer waiting time for USS (p=0.029) but not with false-negative US. Conclusions Pubertal age, symptoms duration of <7 hours, nausea/vomiting, palpated hard testis, abdominal pain and scrotal swelling are predictive factors for TT. Time lost between symptom onset and seeking medical care, and between arrival and US are associated with the need for orchiectomy. Preserved blood flow in USS does not rule out TT and may contribute to delays to surgery.
Ureteropelvic junction obstruction (UPJO) is the most frequent cause of dilatation of the upper urinary tract in neonates. The most important aim of the diagnostics and treatment is the prevention of progressive irreversible renal damage and the subsequent sequelae. The widespread and easy use of ultrasound has led to a significantly increased detection rate of dilatations of the urinary tract, especially prenatal. The current guidelines recommend as postnatal examinations ultrasound followed when necessary by dynamic radioisotope renography, depending on the extent of the dilatation. In special cases, voiding cystourethrography (VCUG), contrast-enhanced voiding urosonography (ceVUS) or functional magnetic resonance urography is also indicated; however, the challenge is not only to decide which children can be observed conservatively and which need surgery, but also which children need which of these imaging modalities and how to monitor them by morphological imaging during follow-up.
Numerous studies indicate that outcomes for pediatric patients are improved when the anesthesia caregiver has advanced training and knowledge of pediatric anesthesiology. Essentials of Pediatric Anesthesiology is a unique new handbook, providing a clinically relevant and easy-to-read review of all key topics in this important field. Written and edited by leading pediatric anesthesia physicians, each chapter takes a consistent approach, guaranteeing this book is user-friendly and authoritative throughout. Topics include physiology, anatomy, equipment, a comprehensive overview of relevant disease states, and special topics such as regional anesthesia, complications, and anesthesia for remote locations. Numerous diagrams, tables and figures help to organize the information for easy reference. Whether you choose to dip into a particular chapter or read the book cover to cover, Essentials of Pediatric Anesthesiology is a valuable review book for all residents, fellows and clinical practitioners needing to improve or refresh their understanding of pediatric anesthesia management.
A total of 320 intersex patients with a Y chromosome were classified into four groups; (1) gonadal dysgenesis, (2) asymmetrical gonadal differentiation, (3) virilizing male hermaphroditism, and (4) feminizing male hermaphroditism (testicular feminization syndrome). Of these 320 cases, 98 were from the files of The Johns Hopkins Hospital and the remainder from the literature. The incidence of tumors in relation to age and clinical classification was analyzed by computer. The results were plotted for each group. It was found that the percentage of tumors rose appreciably soon after the age of puberty in the first three groups, and it was concluded that the gonads were best removed before the age of puberty. In the case of testicular feminization patients, procrastination until the age of 25 could be considered, if one were willing to assume the risk of neoplasia of about 3.6 per cent until then.
The highest figure for first-time UTI is found in infants below one year of age. These early infections are often pyelonephritic in character, but they are easily overlooked because symptoms are unspecific, high fever and failure to thrive being the most important. It has been shown that delay in start of treatment increases the risk of the child developing pyelonephritic scarring. There is reason to believe that undetected and therefore untreated attacks of pyelonephritis may be associated with renal scarring revealed later in life. This type of renal damage is associated with development of hypertension in about 10 per cent of children and it accounts for around 20 per cent of the children entered into dialysis and transplant programs. Prevention of such long-term problems would be of great value and pyelonephritic scarring is a potentially preventable disease. The majority of infants and young children with UTI are probably managed at the primary care level. It is therefore essential that general practitioners are well informed about the epidemiology of UTI in infancy and childhood and that adequate diagnostic facilities are provided. For example, suprapubic aspiration to obtain uncontaminated urine is a technique that may well be used in an outpatient setting, and dipslide cultures are accurate and inexpensive. In addition to young age, vesicoureteric reflux and repeated attacks of pyelonephritis are risk factors associated with development of renal scarring. Therefore, diagnostic imaging to detect children with anomalies within the urinary tract are especially important in the very young. Furthermore, long-term supervision should be provided and the parents advised to consult the doctor when there is suspicion of a new infection to avoid delay in treatment. There is no reason to perform general screening for bacteriuria in healthy infants. Although bacteriuria may be found in 1 to 2 percent, asymptomatic children have a very high rate of spontaneous clearing of the bacteriuria and they seem to constitute a low-risk group. Instead, frequent culturing of urine from febrile infants would be much more important.
Es wird über 20 Kinder mit Harnsteinen berichtet, die mit ESWL behandelt wurden. Eine gute Steindesintegration konnte bei alien Patienten erreicht werden, eine postoperative Instrumentierung war lediglich bei 2 Kindern notwendig. Bei Entlassung waren 14 von 20 Kindern steinfrei, zum Zeitpunkt der Nachkontrolle sind 90% der Kinder steinfrei und ohne zwischenzeitliches Rezidiv. Es konnte gezeigt werden, daß unter Berücksichtigung der nötigen Vorsichtsmaßnahmen die ESWL auch bei Kindern die Methode der Wahl zur Behandlung des Harnsteinleidens des oberen Harntraktes darstellt. Anatomische Abflußbehinderungen müssen ausgeschlossen sein, die Frage von Langzeitschäden auf das kindliche Nierenparenchym bleibt bisher offen.
Vesicoureteral reflux, from the perspective of the primary care physician, should be thought of primarily as a risk factor for pyelonephritis. Reflux is not a cause of urinary tract infections, nor does it cause renal damage. In its most benign state, one could simply conceptualize it as an interesting radiographic finding. Unfortunately, that is often not the situation.
Background. - It is always of importance to define the cause of urinary calculi disease in children to prevent recurrence and possible impairing of renal function. Nevertheless, etiology is not always easy to prove and must be deduced from both clinical and biological arguments.Patients and methods. - The aim of this prospective study including 39 Tunisian children with urinary stones was to identify etiology and stone risk factors and detail the part of clinical and biological data and results of physical analysis of stones in determining the cause of the stone.Results. - In 31 cases among 39, clinical and biological data were not sufficient to identify clearly the stone etiology. When considering the structure and stone composition, the cause of the stone could be determined in 97.4% of the cases. An inherited disease was found responsible for the stone in 11 children, urinary tract infection in 13 cases, idiopathic hypercalciuria in nine cases and a nutritional deficiency disease in seven cases. In one case, polycystic kidney disease with metabolic risk factors could explain the stone process. No precise etiology was found in one case. Among infection stones, struvite stones could be related to urea-splitting bacteriae while other calculi, containing whitlockite and protein matrix could be related to other micro-organisms. Earlier severe chronic diarrhoea episodes were noted in six among seven children presenting stones with a nucleus mainly composed of ammonium urate.Conclusion. - Clinical data, biological data from both urine and blood of the patients and also the structure and composition of the stones are needed to identify the cause of urinary calculi. Such a procedure could provide the stone etiology in most cases.
. To ascertain the outcome of childhood vesico-ureteric reflux (VUR), 226 adults (37 males), mean age 27 years, were studied after 10–35 years, extended to 41 years by postal questionnaire in 161. At presentation (mean age 5 years) all had VUR (grade III–V in 68) and urinary tract infection (UTI); there was renal scarring in 85 (acquired before referral in 11 and during follow-up in 1), hypertension in 6 and impaired renal function in 5. They were managed and followed prospectively by one paediatrician; 63% of these children remained free from UTI; VUR persisted in 63 and had resolved in 69% of 193 children managed medically. At follow-up, 61% of adults had remained free from infection; 17 adults had hypertension and/or raised plasma creatinine, 16 with scarred kidneys. Their deterioration was predictable because of scar type, blood pressure or plasma creatinine levels in childhood. No new scars developed after puberty. Renal growth rates were unaffected by initial severity or persistence of VUR. On the later questionnaire, 9 further adults, mean age 38 years, had moderate hypertension. The adults with complications were those with extensive renal scarring and/or at least borderline hypertension in childhood. Those with VUR, but no scarring, and managed carefully in childhood, did not suffer serious consequences as adults. There is a need for early recognition and treatment of children with VUR and UTI to limit scar development.
The sonographic markers of female and male external genitalia have been documented in early and late gestation. The aim of the present study was to report our experience of possible sonographic markers of fetal genital anomalies. Sonography was performed with a vaginal probe in early gestation and an abdominal sector scanner in advanced gestation. The following genital anomalies were observed: hypospadias, epispadias, ambiguous genitalia, and testicular feminization or Smith-Lemli-Opitz syndrome. It is therefore concluded that prenatal diagnosis of some genital anomalies is now possible.