Article

Congenital HydrocolposA Review with Emphasis on Urologic Aspects and a Report of Four Additional Cases

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Abstract

Hydrocolpos, found chiefly in the new-born, characteristically presents as a mass in the lower abdomen associated with a cystic bulge at the introitus or an imperforate hymen. There is a membranous obstruction at the vaginal outlet in conjunction with excessive secretion of the glands of the infantile uterine cervix. A vaginogram made by injecting the cyst with radiopaque material helps to confirm the diagnosis. The best treatment is simple incision of the occluding vaginal diaphragm. In 6 of the 40 cases described in the literature since 1940, hysterocolpectomy was done, and the diagnosis was made only after examination of the removed surgical specimen. In 3 of the 4 cases reported by the author the condition occurred in newborn babies; in the fourth it became manifest at the age of 2 months.

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... 2A.-With haematocolpos (in girls, hydrocolpos) [122][123][124][125][126]. It is generally made manifest by progressive intra-and post-menstrual dysmenorrhoea present from menarche. ...
... The vaginal complete transverse septum, or imperforated, can present in the girl as an hydrometrocolpos that produces several complications derived from the compression [122,123,186,187]. In other cases it may not present symptoms until puberty, when the hematocolpos forms itself, with episodes of pelvic pain and primary amenorrhea similar to vaginal atresia. ...
Article
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Female genital tract malformations are known to be a frequent cause of reproductive problems. Besides, complex malformations also frequently generate serious gynaecological problems, often ones with inappropriate surgical solutions, as the gynecologist does not think of the malformation as either the cause of the symptoms or of its embryonic origin. Apart from analyzing the embryological bases in the development of the female genital and urinary tract, it is presented the update of the clinical and embryological classification for malformations. Knowing the embryological origin of the female genitourinary malformations, the schematic drawing of the malformations and their related symptoms, will allow the gynaecologist to choose the best therapeutical approach for the patient. Therefore, a discussion on ethiopathogenical deductions and therapeutical management of malformations is also done with clinical recommendations for the general gynaecologist.
... . Since then, about 100 cases have been reported in the English literature [5]. In the last 25 years some large collective reviews [1,4,18,19] and series [9][10][11][12] on h y d r o m et r o c o l p o s have helped to clarify its pathogenesis and management. In the neonate, it usually presents as a surgical emergency: a lower midline ab-Offprint requests to: M. Rohatgi dominal mass with or without protrusion of the h y m e n , often associated with respiratory distress, vomiting, a n d / o r constipation, obstructive uropathy, and genitourinary anomalies. ...
... In the last 15 years, we have seen only 3 cases of neonatal hydrometrocolpos [10,17], including the present case. The rarity of this anomaly in this age group is probably due to difficulty in diagnosis and the high mortality observed in earlier series [1,4,6,11,14,16,18]. However, its awareness is important in the differential diagnosis of abdominal masses in the newborn [9,17]. ...
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An unusual case of neonatal hydrometrocolpos is reported where uterovaginal distension with fluid had produced not only a midline abdominal mass, but also bilateral cystic gluteal swelling. Perineal extension of a hydrometrocolpos causing bilateral gluteal swelling has not been reported previously in the literature. Our patient also had multiple associated anomalies suggestive of a defect in mesodermal embryogenesis similar to that in the mllerian dysgenesis syndrome.
... Hydrocolpos has been associated with urinary obstruction causing bilateral hydroureter and hydronephrosis. [3][4][5][6][7][8][9] When this is severe, there may be in utero oligo/anuria resulting in oligohydramnios. Some cases may be less severe and may present later in infancy as an abdominal mass with urinary retention and frequency. ...
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This is a case report of an unusual cause of Potter sequence. Autopsy showed lethal pulmonary hypoplasia in association with a transverse vaginal septum, hydrocolpos, and a secondary obstructive uropathy.
... the vena cava, and respiratory distress caused by elevation of the diaphragm [5,9,12]. Rarely, ¯uid from the hydrometrocolpos enters the abdominal cavity through the tubes [3]; this may cause ®brinous peritonitis, resembling meconium peritonitis [15,21]. Early diagnosis in the neonatal period is important to decompress the vagina and the urinary system, and avoid early complications. ...
Article
To define the neonatal management and ultimate surgical correction via the anterior sagittal transanorectal approach (ASTRA) in cases of neonatal urinary hydrometrocolpos associated with a persistent urogenital sinus (UGS). We report three patients with UGS (with no ambiguous genitalia) and urinary hydrometrocolpos in whom prenatal ultrasonography showed cystic dilatation of the pelvis. Two patients were temporarily treated with intermittent vaginal catheterization and antibiotic prophylaxis, and in one a cystostomy was necessary to temporarily drain the urine. At the age of 6-8 months the patients underwent reconstructive surgery of the UGS via the ASTRA, under a previous protective colostomy. The mean (range) age of the patients at the last follow-up was 18.7 (8-32) months. A good cosmetic result was obtained in all patients. The vagina was dilated briefly with no anaesthesia in each patient. Patients had normal faecal control after the colostomy was closed and none had urinary incontinence. When possible, intermittent vaginal catheterization permits sufficient temporary drainage of the urinary hydrometrocolpos associated with a persistent UGS. Subsequent ASTRA allows good exposure of the UGS, assuring the preservation of the anorectal innervation and of the sphincteric mechanism.
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In a consideration of hydrometrocolpos, the most important embryological concept is that of the formation of the primitive vaginal plate. Without the formation of this plate, the lower vagina does not develop and enlarge and there remains a persistent urogenital sinus and a malpositioned urethral entrance. A new surgical procedure for hydrometrocolpos has been described which allows for restoration of near-normal anatomy. This procedure is a “vaginal pull-through” operation which separates the genital from the urinary tracts obviating sepsis, retrograde urination, and recurrent urinary infection.
Article
The aim of this study was to demonstrate the clinical results of postoperative evaluation for a consecutive series of cases of imperforate hymen presenting at a tertiary medical center during an over 14-year period. We retrospectively reviewed the clinical records of 15 patients with imperforate hymen that had undergone hymenectomy between 1987 and 1998. After completing a questionnaire via a telephone interview regarding sexuality, fertility, menstrual problems, micturition and defecation after surgical correction, those patients were persuaded to come back to the hospital for further gynecological surveys during 2002. The mean postoperative follow-up was 8.5 years (range, 4-14 years). The mean age at diagnosis was 13.2 years (range, 11-16). The most common clinical symptom was cryptomenorrhea (15), followed by pelvic pain (11), palpable abdominal mass (9), urinary retention and other voiding problems (8) and problems of defecation (4). None admitted intercourse attempts before. Two patients also had uterine anomalies, but none had urinary tract or bowel anomalies. During the follow-up period, we found that the majority of patients had irregular menstrual cycles and were worried about their future fertility. Six patients suffered from dysmenorrhea; of 11 patients who began having intercourse two later delivered babies, and none complained of sexual dysfunction. After hymenectomy, the women with imperforate hymen were markedly relieved of cryptomenorrhea, and problems of micturition and defecation also greatly improved. Although complaints of irregular menstruation and dysmenorrhea gradually evolved as the center of attention during follow-up, most patients fared well in terms of fertility and sexual function.
Article
Congenital imperforate hymen is an external urogenital anomaly that has a small differential diagnosis. With careful antenatal sonographic evaluation of the female perineum, identification of a thin protruding membrane consistent with imperforate hymen can be made. We report a case in which an imperforate hymen was diagnosed in a fetus at 25 weeks' gestation. It appeared as a thin membrane that distended the vagina and spread the labia majora because of retained secretions. An associated renal abnormality was present, representing either a right multicystic dysplastic kidney or cystic dysplasia. Imperforate hymen and hydrocolpos can be diagnosed as early as the second trimester. Because of the additional renal anomaly in this case, it is suggested that the presence of prenatally diagnosed imperforate hymen warrants a careful survey of the remaining fetal anatomy to rule out associated abnormalities. The possibility of a familial occurrence should be considered, and the appropriate history should be obtained.
Article
Two cases of hydrocolpos are described presenting soon after birth. One infant who died on the first day had an intrauterine peritonitis caused by compression of the caecum on the pelvic brim. The other surviving infant developed a caecal perforation secondary to Hirschsprung's disease. Neither infant had rectal atresia, which is the commonest cause of intestinal obstruction in these infants. The association of hydrocolpos with polydactyly in one of these cases may represent an autosomal recessive trait.
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We report the third case of hydrocolpos with vaginal ureteral ectopia. Diagnostic evaluation and surgical management are discussed.
Article
Nine cases with the hydrometrocolpospolydactyly syndrome (4 males, 5 females) from four unrelated families are presented. Leading symptoms of this rare disorder were hydrocolpos and postaxial polydactyly. Three affected girls had urinary hydrocolpos without vaginal septum or imperforate hymen, one had partial vaginal atresia, and one had no hydrometrocolpos. Glandular hypospadias and prominent scrotal raphe are added to the spectrum of malformations in this disorder in males. The literature is reviewed and problems in genetic counseling in this autosomal recessive disorder are discussed.
Article
Two cases of congenital hydrometrocolpos had a previously unemphasized complication: The genital secretions had spilled through the tubes into the peritoneal cavity prenatally producing a plastic peritonitis. This produced a completely different clinical and radiologic picture than usually seen, making diagnosis difficult. One appeared to have duodenal obstruction; the other case mimicked retroperitoneal neoplasia with ascites and inferior vena cava obstruction. Plastic peritonitis should be incorporated into the list of complications of congenital hydrometrocolpos with the corresponding widening of the radiologic, pathologic, clinical and surgical differential diagnoses.
Article
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Hematotrachelos, the engorgement of the cervix uteri with retained blood, is discussed with reference to clinical presentation, ultrasonographic findings, and etiologies. Other disease processes related to hematotrachelos such as hematometra, hematocolpos, and hydrocolpos are also reviewed.
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• Hydrometrocolpos was diagnosed in a neonate shortly after birth. A large collection of fluid was immediately removed from the distended vagina; hymenotomy was performed. The infant has remained well for more than a year following treatment. A simple technique for rapid diagnosis permits safe and appropriate management of this potentially serious disorder. (Am J Dis Child 129:1190-1191, 1975)
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The first case is reported of acute urinary retention caused by hematocolpos owing to uterus didelphys with a unilateral imperforate vagina. A brief description of this rare anomaly and its usual presenting signs and symptoms is presented. Finally, a synopsis of the embryologic defects responsible for the development of the anomaly and the recommended management of the disorder are outlined.
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To study clinical features of patient presented with severe hydramnios, associated with hydronephrosis, that was antenatally diagnosed and has been successfully treated immediately after birth. At a molecular level, we investigated the gene expression of key steroidogenic enzymes from the maternal ovary. Ultrasound scan, MRI, semi-quantitative RT-PCR SETTING: The patient was admitted to the University Hospital, University of Crete, Medical School, Greece, where all clinical data has been obtained. Gene expression studies took place at Biosciences, Brunel University, UK. Semi-quantitative RT-PCR analyses revealed that there is upregulation of key steroidogenic genes in the maternal ovary, including steroidogenic acute regulatory protein, and the cytochrome P450 heme-containing proteins CYP11A, CYP17 and CYP19. From a clinical perspective, the prenatal ultrasound scan and MRI findings showed a multicystic pelvic mass, bilateral hydronephrosis and prior to delivery severe polyhydramnios. This clinical case is the only one that we have found in the current literature where congenital imperforate hymen accompanied with hematocolpos is associated with renal obstruction in combination with polyhydramnios and increase in maternal steroidogenic enzymes.
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A newborn female infant presented with a lower abdominal mass and sepsis. Radiologic and ultrasonic studies established the diagnosis of pyocolpos. Surgical drainage was successfully carried out. Hydrocolpos is an uncommon anomaly resulting from imperforate hymen or atresia of the vagina. It usually presents in the first few weeks of life as an asymptomatic abdominal mass or with symptoms due to compression of adjacent structures by the enlarged vagina. Rarely, pyocolpos results from an infected hydrocolpos, usually associated with vaginal atresia rather than imperforate hymen. It may cause life-threatening sepsis; early diagnosis and prompt surgical drainage are therefore essential. The infant described demonstrates the diagnostic approach and the management of pyocolpos.
Article
Full-text available
Female genital tract malformations are frequently a cause of reproductive problems. Besides, complex malformations also frequently generate serious gynecological problems, often ones with inappropriate surgical solutions, as the gynecologist does not think of the malformation as either the cause of the symptoms or of its embryonary origin. Apart from analyzing the embryological bases in the development of the female genital tract and insisting on the well known müllerian origin of the uterus, the ratification of the embryological hypothesis about the origin of the human vagina from the Wolff ducts and the Müller tubercle allows us to not only advance in knowledge but to use that knowledge for better clinical management of the problems generated by the complex malformations of the female genital tract. In this review we study the methods and means for the diagnosis of female genital-urinary malformations, their classification and clinical findings, and especially those cases involving unilateral renal agenesis, vaginal ectopic ureter and other complex malformations. We also analyze the related obstetric and gynecologic pathology, and especially that cases of complex genital malformations. We review the related literature and some interesting case reports, as well as our experimental studies in rats. After re-asserting our hypothesis about the embryology of the human vagina as derived from the Wolff ducts and the Müller tubercle, we suggest different recommendations for a better approach and management of the malformations of the female genital tract according to the findings observed in different diagnostic procedures.
Article
Three cases of hydrometrocolpos in infants are presented, which demonstrate that despite thorough documentation of the clinical findings, the misdiagnosis and the ensuing mismanagement of this condition remain a problem. The presence of other genitourinary abnormalities and the association with anomalies of the anorectal area make it imperative that these infants be evaluated, treated and followed by clinicians who are skilled in these areas. Introital revision may proceed at an early age to avoid the necessity of repeated drainage procedures and allow normal voiding but close followup is necessary.
Article
Hydrocolpos is characterized by a vaginal accumulation of cervical and vaginal secretions. It often occurs in newborn child and infant. Imperforate hymen is frequently associated; in rare instances, complete higher vaginal diaphragm or partial vaginal atresia is diagnosed. Hydrocolpos can be treated by hymenectomy using imperforate hymen. Because of additional malformation, the treatment can be more complex for vaginal obstruction. The authors report a series of four patients with hydrocolpos: three neonates and one 2-month old infant. Ultrasonography confirmed the physical findings. Three patients with imperforate hymen underwent hymenectomy in X. The fourth which had a virginal atresia and pyocolpos drainage was treated using celioscopy. The three first patients outcome was favorable except for the patient with pyocolpos who died with septicemia.
Chapter
Dossot wies darauf hin, daß die Beschreibung der Harnstauungsniere und ihrer Ursachen nicht nur die Aufzählung so gut wie aller die harnleitenden Wege betreffenden Erkrankungen erfordert, sondern darüber hinaus auch die Beschreibung einer Anzahl krankhafter Zustände anderer Systeme notwendig macht. Eine im Rahmen eines begrenzten Handbuchbeitrages gegebene Darstellung der Harnstauungsniere, ihrer Ätiologie, Pathophysiologic, Histologie, Diagnostik und Klinik kann naturgemäß nur einen skizzenhaften und unvollständigen Überblick über dieses große Gebiet geben. Bewußt mußte manchmal auf die Vollständigkeit der Darstellung verzichtet werden, wobei allerdings versucht wurde, die den Internisten besonders interessierenden Themen hinreichend zu berücksichtigen.
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Hydrometrocolpos usually occurs in the neonatal period. The majority of cases are caused by vaginal occlusion by a transverse septum combined with cervical secretion. Although not well appreciated genital distension by urine may occur in some cases with a persistent urogenital sinus or cloaca.
Article
Full-text available
A 32-year-old primigravida was referred to our hospital at 36 weeks of gestation with a fetal pelvic mass. Ultrasonography showed the fluid-filled area to be a 9 x 4 x 5-cm pear-shaped retrovesical mass with a funnel-shaped blind pouch at the distal end of the fetal vagina. Marked left hydronephrosis resulting from mass compression was also detected. Fetal magnetic resonance imaging further defined a pelvic lesion extending cephalically into the abdomen and caudally into the vagina. Membranal protrusion of the introitus was clearly identified. Therefore, the diagnosis of congenital imperforate hymen with hydrocolpos was established. At 38 weeks of gestation, a 2,966-g female infant was delivered vaginally with good Apgar scores. Physical examination of the neonate revealed a bulging membrane covering the vaginal opening. The presence of syndromic disorders (McKusick-Kaufman, Ellis-van Creveld or Bardet-Biedl syndromes), genitourinary and anorectal anomalies were excluded. The karyotype was 46,XX. A hymenotomy was performed on the second day of life. The infant recovered fully after hymenotomy.
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