Congenital urethral fistula with normal anus: a report of two cases.

Congenital rectourethral or anourethral fistulae without imperforate anus in males are rare, representing less than 1% of anorectal malformations. We report our experience with two males with "N type" urethral fistulae. One, a 5-year-old boy, presented with recurrent urinary tract infections (UTIs) and passage of urine per anus. Investigations included a voiding cystourethrogram (VCUG), which demonstrated a fistula from the urethra to the anus. On physical examination, a small perianal opening was noted just outside the anus, which drained a small amount of urine after voiding. The fistula was excised via a perineal approach. The second patient is a 5-year-old boy with a long history of recurrent UTI requiring multiple hospitalizations since the newborn period. Chronic renal failure developed as a complication of repeated urinary tract infections. Investigations showed a single hydronephrotic pelvic kidney and a small bladder. He underwent numerous diagnostic and reconstructive procedures including cystoscopy and augmentation cystoplasty. Recurrent infections continued and an N type anourethral fistula was eventually diagnosed. The fistula was located between the anal canal and the membranous urethra. An anterior perineal approach was also used. Both fistulae were easily located, and reconstructive surgery of the urethra was not required. Postoperative VCUGs in both patients were normal. They have been free of infection with normal urinary continence since resection of the fistula. Congenital N type anourethral fistulae are rare, but should be considered in cases of recurrent urinary tract infections. The diagnosis may be missed by endoscopic procedures, but VCUG should demonstrate the fistulous tract.

Use of a urethral splent in one-stage hypospadias repair.

Over a 9-month period, 28 patients with distal penile or more proximal hypospadias underwent one-stage surgical repair. Bladder drainage was achieved in the traditional fashion with either an indwelling Foley catheter or suprapubic catheter or by using a modified urethral silicone stent ("splent"). Twenty-two patients had repair with a perimeatal skin flap, and the remaining six patients had major urethral reconstruction with a vascularized preputial island flap. Use of the urethral splent was associated with shorter postoperative hospitalization and minimal short-term complications. The authors' experience has shown that use of a urethral splent for urinary drainage is efficient and effective in postoperative management after hypospadias repair.

The evolution of artificial sphincter cuff in growing animals.

An animal model was developed to simulate the effect of implantation of artificial sphincter cuff on the urethra in children. The study was conducted on 28 pigs, 15 castrated males, four uncastrated males and nine females, divided into four groups: control unoperated, and three operated groups. Group I contained young piglets (castrated males, uncastrated males and females), group II contained adult animals and group III contained sham operated animals. An AS 800 belt occlusion cuff was implanted in the deflated state distal to the bladder neck around the urethra in the young and adult groups, while no sphincter was implanted in the sham group. Neither primary nor secondary activation was done. The pigs were followed for a period of six to eight weeks and then sacrificed. The morphological and histological observations on the effects of the artificial sphincter cuff on the underlying urethral tissue showed significant transmural atrophy of the urethral and prostatic segment underlying the cuff in the young growing castrated and uncastrated male group with mild changes in the young female group. Bladder rupture occurred in three uncastrated males. There were no changes encountered in the adult or sham operated groups. Variable degrees of upper tract changes and renal deterioration were seen in the young group. These changes occurred mainly in the male piglets while a lesser degree occurred in the female piglets. No changes were demonstrated in the adult and sham groups. This study suggests the possibility of similar changes occurring in young male children who have undergone artificial sphincter implantation.

Acute renal failure in an infant with congenital neuroblastoma - successful treatment by hemodialysis.

Acute renal failure developed in an infant aged 30 days during chemotherapy and irradiation for hepatic neuroblastoma (IV-S). Acute hemodialysis via a profunda femoris - long saphenous vein shunt was accomplished on 9 occasions in this 4 kg infant. The complications of septicemia and acute respiratory embarrassment caused by hepatomegaly were treated by appropriate antibiotic treatment, creation of a temporary ventral hernia and assisted ventilation. Careful attention to correct pediatric hemodialysis technique, as described, can permit long-term survival if the underlying disorder is also amenable to treatment.