Common Urogenital Sinus Research Articles

Vaginal reconstruction utilizing sigmoid colon: Complications and long-term results

Background/Purpose: The use of sigmoid colon segments to repair congenital deformities of the vagina is well established. There are little data, however, on complications or functional results in these patients. The purpose of this study was to evaluate complication rates and long-term patient outcomes in the use of sigmoid segments in vaginal reconstruction for congenital anomalies. Methods: The authors identified 28 patients who underwent vaginal reconstruction with sigmoid colon segments between 1985 and 2000 at their institution. Patient charts were reviewed for surgical technique, complication rates, cosmetic results, functional results, and psychosocial development. Patients were recalled for physical examinations and personal interviews to assess current status. Results: Of the 28 patients, 13 had male pseudohermaphroditism, 6 had Mayer-Rokitansky-Kuster-Hauser syndrome, 2 had true hermaphroditism, 2 had mixed gonadal dysgenesis, 2 had common urogenital sinus syndrome, 2 had adrenogenital syndrome, and 1 had penile agenesis. Mean patient age was 16 years (range, 6 to 21 years). Mean follow-up was 6.2 years (range, 2 months to 15 years). Postoperative complications included introital stenosis (4 patients), mucosal prolapse (4), partial small bowel obstruction (2), perineal wound hematoma (2), superficial wound infection (2), and vaginal prolapse (1). None of the complications have affected long-term patency or cosmesis of the neovagina, nor has mucous production significantly affected quality of life. Fourteen of 16 (88%) adult patients are heterosexually active, 1 is homosexually active, and 1 is asexual. Of the 14 heterosexually active patients, 11 (79%) are “very satisfied” with their psychosexual development and 3 are “comfortable.” Four patients are married, and 1 has carried a child to term. All adult patients felt that the appropriate time to undergo surgery was in adolescence. Conclusions: Reconstruction with sigmoid segments is an effective approach for many congenital conditions requiring vaginal reconstruction. Although surgical outcomes are not perfect, appropriately timed reconstructive vaginal surgery can provide most patients with an improved quality of life. For the best long-term results, a multidisciplinary team must be available from infancy to supply comprehensive support. J Pediatr Surg 37:629-633. Copyright 2002, Elsevier Science (USA). All rights reserved.

Demonstration of uterus didelphys on renal transplant scan in a patient with multiple cloacal anomalies.

25-year-old woman born with multiple cloacal anomalies, including an imperforate anus, common urogenital sinus with bladder hypoplasia, and a dysplastic left kidney, underwent a left nephrectomy and creation of an ileal conduit, to which the right ureter was anastomosed. The function of the right kidney diminished secondary to reflux nephropathy, and a right lower quadrant renal transplant was performed. The transplanted ureter was anastomosed to the native right ureter, draining into the ileal conduit. An area of increased radiotracer uptake on the Tc-99m DTPA renal scan represented one horn of the patient's uterus didelphys.

MANAGEMENT OF AN INFECTED HEMATOMETROCOLPOS IN A PATIENT WITH CONGENITAL ADRENAL HYPERPLASIA AND VAGINAL STENOSIS

A 12-year-old girl presented with abdominal pain associated with menses. She had a history of congenital adrenal hyperplasia, and had undergone clitoral recession and posterior flap vaginoplasty at age 18 months. Evaluation revealed abdominal tenderness and pyuria. She was treated for urinary tract infection. The pain worsened and was associated with fever, and the patient was seen by a urologist. Ultrasound demonstrated a pelvic mass consistent with a dilated, fluid filled vagina. The patient underwent endoscopy through the common urogenital sinus but the vaginal introitus could not be identified. Symptoms improved significantly on intravenous antibiotics, and she was referred for further evaluation. Physical examination demonstrated suprapubic tenderness, an enlarged clitoris and a single perineal opening. Rectal examination revealed a large bulging mass anteriorly. Urinalysis showed no infection. The perineal opening was cannulated and a voiding cystourethrogram showed mass effect with anterior displacement of the bladder. Despite several bladder fillings and voidings, no contrast material entered the vagina. MRI was performed to characterize the suspected hematometrocolpos. The sagittal fast spin echo series showed a large midline pear-shaped, fluid filled mass displacing and compressing the bladder and rectum (see figure). Superiorly the mass was contiguous with a small fluid filled uterus and inferiorly it appeared obstructed just above the urogenital diaphragm. The signal intensity of the vaginal and uterine contents was suggestive of proteinaceous fluid and did not follow the signal characteristics of simple blood products. Based on MRI and history, infected hematometrocolpos due to vaginal stenosis was suspected. At cystoscopy the bladder neck was 2 cm. from the perineal opening of the urogenital sinus. It was elevated due to compression from the posterior mass. The bladder contained abundant whitish debris but was otherwise unremarkable. The vaginal introitus was not visible along the urogenital sinus. Based on the MRI and using real-time transabdominal ultrasound guidance, the stifF end of a 0.038-inch polytetrafluorwthylene coated guide wire was passed through the wall of the urogenital sinus and into the vagina. The tract

Urinoma of the Fallopian Tube in Virilizing Congenital Adrenal Hyperplasia

Female patients with congenital adrenal hyperplasia typically are born with a common urogenital sinus, labial fusion, and clitoromegaly. ~ The common urogenital sinus allows for the reflux or pooling of urine into the vagina, and has been reported to predispose to urinary tract infection.: We describe an infant with congenital adrenal hyperplasia who developed urinary reflux into the vagina and the miillerian ducts, leading to massive dilation of one of the fallopian tubes. CASE REPORT

True hermaphroditism unassociated with a common urogenital sinus : David C. Hefelfinger, Kenneth R. T. Tyson, Dieter Assor and George T. Bryan. Pediatrics 43:896–900, (May) 1969

True hermaphroditism unassociated with a common urogenital sinus : David C. Hefelfinger, Kenneth R. T. Tyson, Dieter Assor and George T. Bryan. Pediatrics 43:896–900, (May) 1969

True Hermaphroditism Unassociated with a Common Urogenital Sinus

A child with true hermaphroditism, Group II variety (ovotestis:ovotestis) with separate vaginal and urethral openings has been reported. The cause of this unusual anatomical presetitation remains unknown.