Cases reported "Urogenital Abnormalities"

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1/16. A case report of caudal regression syndrome associated with an intraspinal arachnoid cyst.

    We report here a rare case of caudal regression syndrome associated with an intraspinal arachnoid cyst. The patient was a 6-month-old baby girl with multicomplex congenital abnormalities: sacrococcygeal dysgenesis and ventral curvature, large terminal cyst (myelocystocele), spinal arachnoid cyst, cerebellar hypertrophy (suspected), high imperforate anus, partial dysgenesis of the large intestine, omphalocele, atresia of the vagina, bilateral incomplete ureter duplication, incomplete pseudoduplicated bladder and bilateral talipes equinovarus. We performed plastic repair of the myelocystocele and perineal lesion for caudal regression syndrome and partial removal of the cyst wall for the intraspinal arachnoid cyst. She has been well for 3 years postoperatively, and her mental development is normal.
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2/16. Long-term results of treatment of single-system ectopic ureters.

    Single-system ureteral ectopia (UE) encompasses a spectrum of malformations involving the bladder trigone, ureter, and kidney. The clinical presentation is variable, and both diagnostic and therapeutic problems are common. Reduced renal function in these patients may result from primary dysplasia, obstruction, vesicoureteral reflux, or recurrent infection. Based on our experience of seven patients, suggestions for diagnostic procedures and criteria for renal saving versus nephrectomy are offered. The relationship between ostium localization, renal function, and long-term results was investigated. From 1972 to 1990, five female and two male patients were studied. During the same period, 31 patients with UE and duplex kidneys were seen. Ages ranged from 1 day to 7 years. A ureteric opening into the bladder neck was associated with dilatation of the ureter and renal pelvis. Two patients had vaginal ectopia and severe renal dysplasia. In one, a cyst of the vaginal wall (Gardner's cyst) was detected at birth. A male newborn had multicystic renal dysplasia on the left and ureteric ectopia to the ductus deferens on the right side. To our knowledge, he is the first patient reported with renal function totally dependent on a kidney with severe UE. Follow-up ranged from 4 to 9 years. One patient died in the postoperative period because of renal failure and sepsis. All the others are well and have normal creatinine values. Improvement of renal function was noted after ureteral reimplantation (URI) in patients with bladder-neck ectopia. The numbers of infections were also drastically reduced. Our observations suggest that the combination of ultrasound, cyst urethrography, and cystoscopy will be diagnostic in most patients. A suspicion of UE should be raised in symptomatic patients with apparently solitary kidneys, enuresis ureterica, or atypical obstructive uropathy. Reduced renal function in some patients with ectopia to the bladder neck will improve after URI. This may be of importance in patients with bilateral anomalies and marginal renal function.
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3/16. Treatment of ureterocele on duplex ureter: upper pole nephrectomy by retroperitoneoscopy in children based on a series of 24 cases.

    OBJECTIVE: The objective of this study is to present the results of a preliminary series of 24 upper pole nephrectomies performed by retroperitoneoscopy in children between 1995 and 2000. MATERIAL AND methods: The patient was placed in the lateral supine position and 3-4 trocars were inserted. Parenchymal section was performed by ultrasound or unipolar scalpel. This series of 24 children consisted of 15 girls and 9 boys with a mean age of 22 months. RESULT: Three cases (12.5%) required open conversion. Nine intraoperative complications (37%) were observed and repaired intraoperatively. Five postoperative complications (20%) consisted of residual perirenal collections, requiring drainage under anaesthesia in only one case. The mean operating time was 2 hours 40 minutes. The mean hospital stay was 3.4 days. The mean follow-up was 32 months. No cases of secondary atrophy of the lower pole were observed. CONCLUSION: overall, these preliminary results are comparable to those of conventional open surgery. The advantage of this method is a reduction of skin and musculo-aponeurotic scars.
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4/16. Male genital malformations associated with right renal agenesis.

    A 30-year-old man visited our department complaining of discomfort on micturition. Hypoplasia of the bilateral epididymis, impalpable vas deferens, and prostatic tenderness were noted. diagnostic imaging revealed agenesis of the right kidney, cystic dilation of the right seminal vesicle, and a prostatic utricle cyst. Examination of the genital tract confirmed aplasia of the left seminal vesicle and vas deferens and hypoplasia of the right seminal vesicle. On the basis of postoperative extravasation of contrast medium to the right epididymis, cystic dilation of the right seminal vesicle was diagnosed.
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5/16. retrocaval ureter.

    Two cases of retrocaval ureter are reported. A 22 years old male patient presented to us with the complaints of right flank pain and hematuria and a child of 2 years referred to us for evaluation of right side moderate hydronephrosis detected on ultrasound. The diagnosis was confirmed on intravenous urography and retrograde pyelography. Both the ends were transected and anastomosed above a JJ stent in front of the inferior vena cava. Postoperatively the patients remained asymptomatic and the hydronephrosis improved.
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6/16. Renal transplantation in prune belly syndrome.

    The first reported case of renal transplantation in a patient with prune belly syndrome is presented. Transplanatation should be considered for patients with this syndrome in whom renal failure develops despite appropriate urologic reconstruction. A thorough preoperative evaluation ascertaining the status of the lower urinary tract is especially important in these patients.
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7/16. Pyeloileovesical diversion in a case of prune belly syndrome.

    A case of prune belly syndrome that was managed at birth with cutaneous ureterostomies and later reconstructed with a pyeloileovesical diversion (reversed 7) is presented. This constitutes the first report of such treatment in this anomaly. The indications, operative procedure and results after long-term followup are discussed.
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8/16. Torsion of the spleen and associated 'prune belly syndrome'. A case report and review of the literature.

    Splenic torsion as a complication of wandering spleen is rare. We report the clinical findings, diagnostic problems and treatment of a 1-year-old Coloured child (with classic 'prune belly syndrome') in whom the spleen had undergone torsion, thus simulating an intra-abdominal abscess. The postoperative course was uneventful.
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9/16. Surgical management of vaginal vault prolapse in a woman with a neovagina and pelvic kidneys.

    BACKGROUND: women with Mayer-Rokitansky-Kuster-Hauser syndrome have congenital absence of the uterus and upper two-thirds of the vagina, which is frequently accompanied by skeletal and renal anomalies. Mechanical dilation or surgical creation of a vagina allows for function but does not provide endopelvic fascial support of the vagina. Vaginal prolapse may occur. CASE: A 32-year-old woman presented with pelvic kidneys and a 5-year history of prolapse of her mechanically created neovagina. She underwent a sacrospinous ligament suspension with a cadaveric fascia lata bridge. The apex of the neovagina was 5 cm above the hymen 30 months postoperatively. CONCLUSION: An allograft colpopexy to the sacrospinous ligament is an effective method of surgical treatment of women with a prolapsed shortened vagina and an inaccessible presacral space.
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10/16. Congenital scaphoid megalourethra: report of two cases.

    Congenital megalourethra is a rare congenital malformation of the penile urethra. It is defined as the diffuse dilatation of the anterior urethra due to the absence of development of the erectile tissue of the penis. Since the initial description, nearly 80 cases with megalourethra have been reported in English literature. Congenital megalourethra has been classified into scaphoid and fusiform types and is usually associated with additional urinary tract and other system anomalies, irrespective of its type and severity. We report two rare cases of scaphoid megalourethra without any associated anomaly. A 13-year-old boy and an 8-month-old boy were admitted to the Department of Pediatric Surgery with complaints of penile swelling and dysuria since birth. physical examination and retrograde cystourethrogram confirmed the diagnosis of congenital scaphoid megalourethra. Both patients underwent a reduction urethroplasty. They did well postoperatively and voided with a normal stream without any abnormality of the penile shaft.
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