[Show abstract][Hide abstract] ABSTRACT: Because prepubertal testis tumors are rare, their management has historically been based on experience with the more common adult testis tumors. However, recent studies highlighting the natural history of these tumors and their response to therapy have resulted in a modern management algorithm that optimizes testicular preservation and minimizes the morbidity of adjuvant therapies. Many prepubertal testis tumors are benign and can be managed with testis-sparing tumor excision. Localized malignant tumors (yolk sac tumors) may be managed with excision alone. Recurrent tumors and metastatic disease can almost always be treated successfully with platinum-based chemotherapy.
[Show abstract][Hide abstract] ABSTRACT: The Prepubertal Testis Tumor Registry was established by the Urologic Section of the American Academy of Pediatrics in 1980 to record data on a large number of prepubertal testis tumors regarding presentation, treatment and outcome to define appropriate management better. We reviewed the registry data in the context of other modern studies to elucidate the appropriate management of these rare tumors.
Relevant data in the prepubertal testis tumor registry were tabulated and analyzed.
There were 395 prepubertal patients who had a primary testis tumor. Generally benign tumors accounted for 38% of cases. A significant proportion of tumors were benign regardless of patient age. alpha-Fetoprotein levels for patients with benign and malignant tumors overlapped in children younger than 6 months. Of the patients with yolk sac tumor 80% presented with stage 1 disease and overall survival was excellent. There were no metastases or deaths among the patients with teratoma. Of all patients with stromal tumors metastases developed in only 1.
We recommend initial excisional biopsy for all amenable prepubertal testis tumors, except those with an alpha-fetoprotein level that is clearly increased for patient age. Patients with benign tumors may be released from oncological followup. Patients with stage I yolk sac tumor should be monitored closely, and those with recurrent or metastatic yolk sac tumor should be treated with chemotherapy. Retroperitoneal lymph node dissection is reserved for patients with recurrent retroperitoneal masses following chemotherapy. Aggressive treatment of metastatic Sertoli cell or undifferentiated stromal tumors is warranted.
The Journal of Urology 11/2002; 168(4 Pt 2):1675-8; discussion 1678-9. DOI:10.1097/01.ju.0000030749.27823.f5 · 4.47 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Little data are available regarding sports participation and appropriate long-term followup of children with a solitary kidney. We determine the current practice patterns and recommendations among pediatric urologists regarding sports participation and followup of these patients.
A survey was mailed to the 231 active members of the American Academy of Pediatrics, Section on Urology. The survey included questions regarding counseling of patients with a solitary kidney and physician estimates of long-term risk to overall renal function.
Of the 231 surveys 182 were returned for an overall response rate of 79%. Of the respondents 68% recommend that patients with a solitary kidney avoid contact sports. Recommendations in regard to participation in contact sports were further stratified as strongly against participation (27%), against participation with rare exceptions (30%), no recommendation either way (14%), allow participation (25%) and no restrictions be made (4%). Of the respondents 88% agreed that the estimated risk of renal loss from a child participating regularly in contact sports is less than 1% and 60% recommended special medical followup.
Despite the consensus that the risk of renal injury in contact sports is low, a significant number of pediatric urologists advise avoidance. There appears to be a lack of consensus regarding long-term medical surveillance of these patients. Studies designed to obtain accurate clinical data regarding these issues are warranted to establish evidence based guidelines for the long-term treatment of children with a solitary kidney.
The Journal of Urology 11/2002; 168(4 Pt 2):1811-4; discussion 1815. DOI:10.1097/01.ju.0000028011.26729.68 · 4.47 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Stromal testis tumors are rare and generally exhibit a benign behavior in prepubertal patients. We reviewed the Prepubertal Testis Tumor Registry to elucidate further the behavior of these tumors.
Epidemiological and clinical information on stromal testis tumors was compiled and reviewed from the Prepubertal Testis Tumor Registry. In addition, original pathology reports were requested for all patients registered as having undifferentiated stromal tumors.
There were 43 patients registered with stromal tumors. Of the 21 patients with unspecified stromal tumors pathology reports were obtained on 11. Eight patients had truly mixed or undifferentiated stromal tumors. Mean patient age at presentation was 38 months (Leydig cell 70, Sertoli cell 52.5, juvenile granulosa cell 1.5 and mixed/undifferentiated 41.2). No patient with a Leydig cell, Sertoli cell or juvenile granulosa cell tumor had metastases at presentation or metastatic disease during an average 24.6 months of followup. One undifferentiated tumor demonstrated malignant behavior by presenting with metastatic disease. Pathological examination revealed a poorly differentiated tumor with extension into the adjacent tunica and frequent mitotic figures. While other stromal tumors displayed mitotic figures, none showed local invasion.
Stromal testis tumors are rare. Data from the Prepubertal Testis Tumor Registry confirms the benign behavior of most of these tumors. However, undifferentiated stromal tumors may exhibit metastatic behavior. A high index of suspicion is appropriate when there are a large number of mitotic figures, the tumor is poorly differentiated or when local invasion is present in the primary tumor. Metastatic evaluation and close followup are warranted for this select group of patients.
The Journal of Urology 01/2002; 166(6):2338-40. DOI:10.1016/S0022-5347(05)65583-8 · 4.47 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: We describe a novel technique of laparoscopic transvesical cross-trigonal Cohen anti-reflux ureteroneocystostomy.
A 10, an 11 and a 32-year-old patient with symptomatic unilateral vesicoureteral reflux underwent laparoscopic cross-trigonal ureteral reimplantation. Two 5 mm. balloon tip ports were suprapubically inserted into the bladder. Using a transurethral resectoscope with a Collins knife a 4 to 5 cm. cross-trigonal submucosal trough was created from the refluxing ureteral orifice to the contralateral side of the bladder. The refluxing ureteral orifice and intramural ureter were completely mobilized intravesically, advanced transtrigonally and secured to the detrusor muscle at the apex of the trough with 3 deep interrupted sutures. The elevated mucosal flaps of the trough were suture approximated over the ureter to create a submucosal tunnel. All suturing was performed by freehand laparoscopic technique.
Operative time was between 2.5 and 4.5 hours and blood loss was 10 to 50 cc. Adequate submucosal trough creation, ureteral extravesical mobilization and intravesical advancement, and bladder mucosal flap reapproximation were done to create a submucosal tunnel in all cases. Satisfactory transtrigonal anchoring of the neoureteral orifice to the detrusor muscle and mucosa was achieved with 3 stitches. Hospital stay was 2, 2 and 1 days in the 3 cases, and the Foley catheter remained in place for 3, 1 and 1 week, respectively. At 6 months reflux had resolved in 2 patients, while in 1 grade II reflux persisted, which was improved from grade IV preoperatively. All patients have remained infection-free without antibiotics.
Laparoscopic transvesical cross-trigonal antireflux ureteral reimplantation is technically feasible. Intravesical laparoscopic suturing is possible. Potential advantages include a decreased hospital stay, decreased narcotic requirement and better cosmesis. Further experience is necessary to refine the technical nuances and evaluate outcomes compared to the open technique.
The Journal of Urology 12/2001; 166(5):1811-4. DOI:10.1016/S0022-5347(05)65680-7 · 4.47 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Survivors of childhood Wilms tumor have been followed by large collaborative studies for approximately 31 years. In this time a number of second malignant neoplasms have been documented in these Wilms tumor survivors and they are at higher risk for such development compared with the general population. To our knowledge no renal neoplasms have been previously reported in patients successfully treated for Wilms tumor in childhood.
We reviewed the cases of 4 adults in whom Wilms tumor was treated in childhood by radical nephrectomy and adjuvant therapy and who presented to our institution with complex cystic or solid renal masses in the contralateral kidney. Parameters, including patient age at Wilms tumor diagnosis, Wilms tumor treatment modalities, age at second malignant neoplasm presentation and resected renal lesion pathology were outlined. A thorough literature review was performed to identify the development of renal malignancies as second malignant neoplasms in survivors of Wilms tumor in childhood.
The International Society of Pediatric Oncology and National Wilms Tumor Study have followed patients treated for Wilms tumor for no greater than 31 years. Renal neoplasms, including 2 renal cell carcinomas, 1 oncocytoma and 1 atypical cyst, in the solitary remaining kidney of relatively young adults 34 to 50 years old who were treated for Wilms tumor greater than 31 years ago were successfully treated with partial nephrectomy at our institution. Neither the International Society of Pediatric Oncology nor National Wilms Tumor Study has identified renal cell carcinoma as a second malignant neoplasm in patients successfully treated for Wilms tumor.
Our experience suggests that relatively young adults with a history of childhood treatment for Wilms tumor may be at increased risk for renal neoplasms at ages not yet achieved by those enrolled in large multicenter trials. This possibility should be considered when planning the long-term followup of these patients. The potential to develop this type of second malignant neoplasm again raises the clinical issue of performing a primary nephron sparing procedure in children presenting with Wilms tumor.
The Journal of Urology 07/2001; 165(6 Pt 1):2013-6; discussion 2016-7. DOI:10.1016/S0022-5347(05)66283-0 · 4.47 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: The aim of this study is to report the initial experience with needlescopic surgery (2-mm optics and instrumentation exclusively) for the cryptorchid testicle.
Ten patients (age 8 months to 37 years) underwent 12 needlescopic procedures: orchiopexy (n = 8), orchiectomy (n = 2), and diagnostic exploration with attempted excision of testicular remnant (n = 2). Two patients underwent bilateral needlescopic orchiopexy. Needlescopic (2 mm) optics and instrumentation were used exclusively in the pediatric patients.
All procedures were completed successfully by needlescopic techniques. Mean surgical time was 110 minutes (range, 60 to 180 minutes), and blood loss was 6 mL (range, 0 to 20 mL). There were no intraoperative complications. All procedures were performed on an outpatient basis. In all 8 orchidopexies, the testis was successfully brought to a scrotal position.
Needlescopic techniques allow safe performance of various procedures for a cryptorchid testicle. The cosmetic result is excellent.
Journal of Pediatric Surgery 11/2000; 35(10):1426-30. DOI:10.1053/jpsu.2000.16405 · 1.39 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: The appropriate management of penile adhesions in circumcised boys is unclear. An important consideration is whether adhesions resolve spontaneously. We studied the incidence of penile adhesions as a function of patient age to assess the natural history.
We evaluated all circumcised boys presenting to our pediatric urology clinic. A standard form was used to classify adhesions as grade 0-no adhesions, 1-fine adhesions to the corona, 2-adhesions covering less than 50% of the glans and 3-adhesions covering greater than 50% of the glans. All boys were evaluated by 1 of 2 pediatric urologists. Previous treatment of adhesions was assessed and skin bridges were also noted.
We enrolled in our study 254 boys 1 month to 19 years 8 months old. Only 7 patients had a history of treatment of adhesion, of whom 3 had recurrent adhesions at evaluation. Patients were divided into groups based on age, including younger than 12 months (61), 13 to 60 (78), 61 to 108 (51) and 109 months old or older (64). In these groups we noted an adhesion rate of 71%, 28%, 8% and 2%, respectively. The rate of adhesions more severe than grade 1 was 30%, 10% and 0% in boys 12 months old or younger, 13 to 60 and 61 months old or older, respectively. The oldest patient with grade 3 adhesions was 31 months old. Skin bridges in 6 cases involved the circumcision line in 4.
Penile adhesions develop after circumcision and the incidence decreases with patient age. Although there is debate on whether to lyse these adhesions manually, our findings suggest that adhesions resolve without treatment. Based on our results we do not recommend lysing penile adhesions, except perhaps those involving the circumcision line.
The Journal of Urology 09/2000; 164(2):495-6. DOI:10.1016/S0022-5347(05)66572-X · 4.47 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Recent laparoscopic studies indicate a high incidence of a contralateral open internal ring in children undergoing unilateral hydrocele or hernia repair, raising the question of whether routine contralateral exploration should be done. Data on the long-term risk of clinical contralateral hernia or hydrocele after unilateral hydrocele repair are limited. To address this question we performed long-term followup in patients who underwent unilateral hydrocele repair.
We followed patients who previously underwent unilateral hydrocele repair performed by one of us before 1997. Patients were interviewed by telephone and encouraged to return to one of us or their pediatrician for evaluation.
Of the 101 patients who fulfilled study inclusion criteria 85 who were 5 to 107 months old (median age 37) at the original surgery were successfully contacted, including 45 examined by one of us or a pediatrician and 40 followed by telephone interview only. Contralateral hydrocele or hernia developed in 6 of the 89 patients (7%) 6 to 15 months (median 12) postoperatively. The remaining 79 patients have been recurrence-free for 6 to 153 months (mean 44, median 37). Of the patients 5 of 32 are (15%) and 1 of 53 (2%) who underwent left and right hydrocele repair, respectively, had contralateral recurrence.
The risk of a clinically evident contralateral hydrocele or hernia after unilateral hydrocele repair is approximately 7%. We do not recommend routine contralateral exploration in children undergoing unilateral hydrocele repair.
The Journal of Urology 10/1999; 162(3 Pt 2):1169-70; discussion 1171. DOI:10.1016/S0022-5347(01)68116-3 · 4.47 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Congenital lymphedema is a rare disorder that may result in disfiguring edema of the male genitalia. We reviewed our experience with 5 cases to advance our understanding of this challenging problem.
Four boys with significant lymphedema underwent excision of the involved subcutaneous genital tissue and coverage with local skin flaps. Two boys in whom this approach failed later underwent complete excision of the involved subcutaneous tissue and skin, and coverage with split thickness skin grafts. The boy with minimal edema was observed.
Two of the 4 boys who underwent subcutaneous genital tissue resection and coverage with local skin flaps are markedly improved, although 1 requires further revision. In the other 2 boys treatment failed, necessitating repeat genital tissue excision and grafting. While there have been no recurrences in the grafted areas, each patient has required additional operations to manage recurrent edema in adjacent tissues of the perineum and inguinal region, and in 1 significant contraction of the grafted skin developed. Mild genital lymphedema in the remaining patient has remained stable during 10 years of followup.
Congenital lymphedema of the genitalia is a challenging problem. Recurrences requiring multiple operations are common. We recommend expectant management of mild cases. In more severe cases excision without grafting should be attempted. While skin grafting may be the most definitive solution, it does not prevent recurrence in adjacent regions, and it carries the risk of skin contraction. Skin grafts should only be used when other techniques have failed.
The Journal of Urology 11/1998; 160(4):1485-9. DOI:10.1097/00005392-199810000-00100 · 4.47 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: The widespread use of prenatal ultrasonography has led to a marked increase in the incidence of ureteropelvic junction obstruction presenting in infancy. Which of these prenatally detected lesions requires repair has yet to be fully determined. Attempts to characterize prenatally detected ureteropelvic junction obstruction in regard to symptomatic obstruction detected later in life may provide useful insights into the management of these incidentally discovered lesions. We evaluated the presence of crossing vessels in ureteropelvic junction obstruction identified by prenatal ultrasonography.
We reviewed the records of patients who underwent dismembered pyeloplasty for primary ureteropelvic junction obstruction with specific attention to the mode of presentation and presence of crossing vessels associated with obstruction.
In 30 patients with a mean age of 6 months ureteropelvic junction obstruction detected prenatally was not associated with a crossing vessel. Ureteropelvic junction obstruction was identified postnatally in 125 patients who underwent open pyeloplasty. It was detected incidentally in 8, of whom crossing vessels were present in 1 (12%). There were signs or symptoms related to ureteropelvic junction obstruction in 117 patients. In contrast to the group with prenatally detected ureteropelvic junction obstruction in which crossing vessels were never noted, crossing vessels were associated with 36 (30%) of the 120 kidneys operated on in this postnatally discovered group.
No crossing vessels were present in patients with prenatally detected ureteropelvic junction obstruction compared with 30% in symptomatic patients. This finding may reflect the natural history of the disorder, or it may suggest that prenatally identified ureteropelvic junction obstruction is an entity largely different from obstruction presenting later in life.
The Journal of Urology 10/1998; 160(3 Pt 2):973-5; discussion 994. DOI:10.1016/S0022-5347(01)62673-9 · 4.47 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: UPJ obstructions occurring in anomalous kidneys require special consideration. Many anomalous kidneys are dysmorphic with extrarenal pelves, making the distinction between obstructed and nonobstructed hydronephrosis crucial. The radiographic evaluation must be thorough because there are often other associated anomalies of the ipsilateral or contralateral kidney. Operative intervention must be tailored to the specifics of the case. Although most UPJ obstructions in anomalous kidneys can be reconstructed successfully with dismembered pyeloplasty or treated with newer minimally invasive techniques, issues regarding access, operative detail, and adjunct procedures make these cases challenging for the urologic surgeon.
Urologic Clinics of North America 06/1998; 25(2):219-25. DOI:10.1016/S0094-0143(05)70010-0 · 1.20 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Surgical treatment of the children of Jehovah's Witnesses is a challenging problem both ethically and technically. The authors recently operated on such a child who had bilateral Wilms' tumors. Techniques used to minimize blood loss included erythropoietin, hemodilution, and the argon beam coagulator. A full understanding of the religious, legal, and ethical issues is essential when treating the children of Jehovah's Witnesses.
Journal of Pediatric Surgery 01/1998; 32(12):1759-60. DOI:10.1016/S0022-3468(97)90528-5 · 1.39 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Prepubertal teratomas of the testis are rare benign tumors. Knowledge of the epidemiology of testicular teratomas and associated alpha-fetoprotein (AFP) levels could assist in planning treatment for prepubertal patients suspected of having teratoma of the testis.
We evaluated data on testicular teratoma from the Prepubertal Testis Tumor Registry of the Section on Urology of the American Academy of Pediatrics.
Median patient age at presentation was 13 months. Teratomas occurred equally in the right and left testes. Most patients presented with a scrotal mass. Preoperative AFP levels were elevated in 20 of the 34 patients and postoperative levels were elevated in 9 of 10. Most patients were treated with radical orchiectomy and no metastatic disease was reported.
Data in the registry confirm the benign nature of this tumor. Normal AFP levels should not be expected in infants with teratoma.
The Journal of Urology 10/1997; 158(3 Pt 2):1191-2. DOI:10.1016/S0022-5347(01)64421-5 · 4.47 Impact Factor