Surgical management of persistent müllerian duct syndrome.

OBJECTIVES: To describe the optimal surgical management of the testes and müllerian duct structures in patients with persistent müllerian duct syndrome. METHODS: We performed a comprehensive Medline literature search regarding the surgical management of persistent müllerian duct syndrome and extracted information regarding the etiology, pathogenesis, and treatment of this disorder. We specifically assessed the risks of retained müllerian structures versus surgical excision of the infantile uterus and fallopian tubes. Using this information, we formulated a comprehensive strategy for the management of patients with persistent müllerian duct syndrome. An illustrative case is described. RESULTS: No malignant degeneration of persistent müllerian structures has been reported. The risk of testicular neoplasia in persistent müllerian duct syndrome approximates the risk of neoplasia in other intra-abdominal gonads. Fertility has rarely been reported although virilization is unaffected. Surgical excision of the infantile uterus and fallopian tubes risks damage to vasa deferentia and the deferential blood supply to the testis. CONCLUSIONS: Surgical excision of persistent müllerian duct structure may result in ischemic and/or traumatic damage to the vasa deferentia and testes. Optimal surgical management is orchiopexy leaving the uterus and fallopian tubes in situ. Meticulous proximal salpingectomy and hysterectomy is indicated only in patients whose müllerian structures limit intrascrotal placement of the tests. Orchiectomy is indicated for testes that cannot be mobilized to a palpable location.

Testis sparing surgery for steroid unresponsive testicular tumors of the adrenogenital syndrome.

PURPOSE: Surgical management of steroid unresponsive testicular tumors of the adrenogenital syndrome has been orchiectomy. Magnetic resonance imaging (MRI) of these tumors accurately delineates the extent of disease. Testis sparing surgery is an important consideration, since male individuals with congenital adrenal hyperplasia are potentially fertile. We present our results of surgical management of this tumor based on MRI findings. MATERIALS AND METHODS: Four boys with steroid unresponsive testicular tumors of the adrenogenital syndrome were evaluated with MRI, testicular ultrasound and color flow Doppler examinations preoperatively and postoperatively. Three patients had 21-hydroxylase deficiency and 1 had 3-beta-hydroxysteroid dehydrogenase deficiency. Contralateral testicular abnormalities included a vanished testis, testicular atrophy due to trauma and bilateral tumors in 1 boy each. Bilateral orchiectomy and surgical enucleation were performed in 1 and 3 patients, respectively. Followup ranged from 8 to 18 months. RESULTS: Postoperative MRI of the testis in 2 of 3 patients showed no evidence of recurrent tumor. Postoperative testicular sonography revealed no tumor and vascular flow in 2 of 3 patients. All 3 patients who underwent testis sparing surgery have a viable testis in the scrotum without evidence of recurrent disease. CONCLUSIONS: MRI of the testis in patients with testicular tumors of the adrenogenital syndrome accurately defines the extent of disease. Surgical enucleation of this tumor has been performed successfully without recurrent disease. This surgical approach should be considered for any patient with a steroid unresponsive tumor and contralateral abnormalities. We believe that surgical enucleation is the procedure of choice for all patients with this tumor, since it maximizes future fertility potential.

Minimizing surgical problems of peritoneal dialysis in children.

We review our experience with peritoneal dialysis catheter placement in children. The most common surgical problems after peritoneal dialysis catheter placement are inguinal hernias, catheter obstruction and dialysate leakage. We describe specific techniques to minimize the development of these problems.

Perineal lipoma and the accessory labioscrotal fold: an etiological relationship.

Congenital scrotal disorders are unusual, including penoscrotal transposition, bifid scrotum, ectopic scrotum and accessory scrotum. The latter 2 entities are extremely rare. Because accessory scrota usually arise in the absence of associated anomalies, an etiology has been enigmatic. We present 2 cases of accessory labioscrotal folds, including a female patient. An exhaustive literature review allowed comparison with all reported cases (23) and showed a frequent association with perineal lipoma (83%). On that basis, we were able to classify accessory labioscrotal folds into 2 types and recommend a different course of management for each. Accessory labioscrotal folds usually develop when intervening mesenchymal tissue disrupts the continuity of the caudally developing labioscrotal swelling.

Yolk sac tumors and alpha-fetoprotein in first year of life.

Serum alpha-fetoprotein (AFP) levels are normally elevated in the first eight months of life. This is important information when using AFP as a tumor marker for patients in this age group. We report a case of a one-month-old boy with a yolk sac tumor of the testis. After radical orchiectomy and a negative workup for metastatic disease, his AFP level dropped, but remained mildly elevated over the normal range for six months. Using a half-life of five days it was predicted that it would fall into this range in eighty days. Although the elevated levels suggested residual tumor, they did continue to decline each month which led us to pursue some other explanation for this pattern. A literature search revealed the AFP levels are normally elevated in this age group. In retrospect, our patient's AFP was within this range less than sixty days after surgery. Dissemination of knowledge that the normal AFP ranges in young infants are higher than those in older patients will improve the clinical usefulness of AFP as a tumor marker in this age group.

Pelvic osteotomy for bladder exstrophy.

Twenty-five patients with bladder exstrophy underwent pelvic osteotomy at or before initial bladder closure and anterior abdominal repair. Ten patients underwent bilateral iliac osteotomies before 1977. An alternative procedure consisting of bilateral superior pubic ramotomies has been used in 15 patients since 1977. In all patients, successful tension-free closure of the abdomen was achieved. Long-term follow-up of these two groups of patients shows no difference in the degree of pubic diastasis, which was asymptomatic. Posterior iliac osteotomy requires two additional incisions, longer operative time, and postoperative immobilization. We therefore recommend bilateral superior pubic ramotomy as an alternative procedure in initial reconstruction of the anterior abdomen in patients with bladder exstrophy.

Primary congenital bladder diverticula in boys.

Three male patients ranging in age at diagnosis from eight to seventeen years were found to have presumed primary congenital bladder diverticula. All 3 patients had a solitary documented urinary tract infection (Staphylococcus 2, Escherichia coli 1), 1 patient presented with gross hematuria, and in 1 patient the diverticulum was an incidental finding. In all cases, there was no radiologic evidence on voiding cystourethrography of physiologic or anatomic ureteral or bladder outlet obstruction. In a nine-year-old boy there was ipsilateral renal agenesis, an intra-abdominal undescended testis, and an atretic ureter arising from the diverticulum. The older boy had Ehlers-Danlos syndrome. If the diverticulum is enlarging, compromises the ureterovesical valve mechanism, or by virtue of incomplete bladder emptying is believed to promote urinary tract infection, it should be removed. A surgically conservative course is followed in the Ehlers-Danlos syndrome because of the bleeding tendency and tissue abnormalities.

Congenital prepubic sinus.

We report the fourth case of a congenital prepubic sinus extending from the anterior bladder wall through rectus fascia to overlying skin. Intermittent erythema and discharge prompted exploration and excision. This lesion may represent a mild forme fruste of the midline closure defects.

The association of Wilms' tumor with nephrologic disease.

Three cases of nephrologic disease characterized clinically by nephrotic syndrome and histologically with mesangial sclerosis associated with Wilms' tumor are documented. Two children were born with ambiguous genitalia and an XY chromosome constitution. A third patient had a 46,XY karyotype and developed a Wilms' tumor but had a normal female phonotype. The evaluation and current management of this syndrome are discussed.

Postoperative intussusception in children.

Intussusception in the postoperative period is an unusual yet potentially fatal surgical complication. We present 3 illustrative cases and contrast postoperative intussusception with the more common classic childhood intussusception. Physician awareness of this condition is critical for early recognition and treatment.

Prepubertal yolk sac testicular tumors--report of the testicular tumor registry.

In 1980 the Section on Urology of the American Academy of Pediatrics established a registry of prepubertal testicular tumors. A total of 181 yolk sac tumors has been reported to the registry. Complete followup is available for 154 of these 181 patients. Most of the patients presented with low stage disease. Radical orchiectomy without adjunctive retroperitoneal lymphadenectomy, chemotherapy or radiotherapy was sufficient therapy for most stage 1 cancer patients. alpha-Fetoprotein is a reliable marker for this tumor. Chest x-rays and abdominal computerized tomography are reliable for staging. When metastases appeared they did so within 14 months of presentation so that 2 years of followup after any evidence of disease seems to be adequate. Approximately two-thirds of the patients with metastases were salvaged by chemotherapy, radiotherapy and/or an operation.

Splenic gonadal fusion.

Splenic gonadal fusion is a rare anomaly that is frequently associated with skeletal abnormalities. Although rare, this entity should be considered in the differential diagnosis of scrotal masses in children. Routine frozen section prevents unnecessary orchiectomy. We describe a child with limb anomalies in whom splenic gonadal fusion presented as an incarcerated inguinal hernia.

Anterior urethral valves resulting from congenital urethral diverticula.

Five cases of anterior urethral diverticula with resulting anterior urethral valves are reported. All children presented with a poor stream. Diagnosis was accomplished by voiding cystourethrography, and treatment was by open resection of the diverticulum in four neonates and endoscopic excision of the valve in a four-year-old boy.

Preservation of 32 human kidneys by simple cold storage for more than 48 hours.

Transplant centers are reluctant to use kidneys stored cold for more than 48 hours. During a 6-year interval we transplanted 32 kidneys preserved by intracellular electrolyte flushing that were stored cold for 48.2 to 61.4 hours. Of the recipients 91 per cent required dialysis within 1 week after transplantation. The mean serum creatinine nadir within 1 month was 3.0 mg. per dl. and graft survival at 1 month was 81 per cent. Short-term kidney graft function was not influenced significantly by the addition of magnesium sulfate to the flush solutions or by cyclosporin immunosuppression. The 1 and 2-year actuarial kidney graft survival rates were 72 and 58 per cent, respectively. The 1 and 2-year mean serum creatinine levels were 1.9 and 1.6 mg. per dl., respectively. Kidneys can be transplanted successfully after 48 hours of simple cold storage following flushing with an ice-cold intracellular electrolyte solution.

Experience with endoscopic incision and open unroofing of ureteroceles.

Endoscopic incision or open unroofing of a ureterocele was performed in 40 children between 1 day and 15 years old. Of these patients 20 showed such improvement in function of the primarily obstructed segment that subsequent nephroureterectomy was not deemed necessary. Only 2 of these 20 patients required later reconstructive, nonextirpative surgery because of recurrent urinary tract infection. The other 20 patients underwent elective heminephroureterectomy with ureterocelectomy and lower ureteral reimplantation sometime after they were 18 months old, except for 4 children who required earlier intervention because of recurrent urinary tract infection.

Gonadal stromal tumors: a report of the Prepubertal Testicular Tumor Registry.

A total of 15 gonadal stromal tumors in prepubertal boys has been reported to the Prepubertal Testicular Tumor Registry of the Section on Urology of the American Academy of Pediatrics. Leydig cell tumors invariably are benign and patients usually present with precocious puberty when they are 5 to 9 years old. Other gonadal stromal tumors either present in infancy as a scrotal mass and exhibit a benign behavior or occur later in childhood and may be malignant.

Hernias complicating chronic ambulatory peritoneal dialysis in children.

The sustained hydraulic pressure developed within the peritoneal cavity during chronic ambulatory peritoneal dialysis (CAPD) may develop any actual or potential defect in the inguinal or umbilical area into a clinical hernia. Seven of 19 children have developed inguinal hernias after initiation of CAPD. Demonstration of the presence of a processus vaginalis by peritoneography during catheter placement permits their repair at that time. Late development of clinical indirect inguinal hernias early in our experience after high ligation of the processus vaginalis emphasizes the fact that tight closure of the internal ring is necessary.

Müllerian remnant causing bladder outlet obstruction.

Less than one-third of patients with persistent müllerian duct remnants develop bladder outlet obstructive symptoms. We report on two cases of bladder outlet obstruction caused by massive dilatation of persistent müllerian duct remnants. Such structures should be suspected when outlet obstructive symptoms occur in patients with hypospadias and/or undescended gonads. Care should be taken to identify possible urethral obstruction distal to the prostatic urethra as a cause of gross enlargement of persistent müllerian duct remnants.