Neil McMullin

Congenital Prepubic Sinus: Possible Variant of Dorsal Urethral Duplication (Stephens Type 2)

We report on 3 patients with a midline sinus extending from the skin overlying the pubis to the anterior wall of the bladder. Two sinuses were lined by transitional epithelium. The sinuses were surrounded by bundles of smooth muscle and lacked any inflammatory reaction, which suggests a developmental origin. Possible etiological causes for this unusual congenital anomaly are discussed. We conclude that these sinuses may represent a variant of epispadiac duplication of the urethra.

The Kelly Technique of Bladder Exstrophy Repair: Continence, Cosmesis and Pelvic Organ Prolapse Outcomes

PURPOSE The Kelly technique of radical soft tissue mobilization, an alternative to osteotomy and modern staged repair, has been used extensively at our tertiary referral center for bladder exstrophy in the last 2 decades. We present what is to our knowledge the first long-term followup of the Kelly technique in 31 patients treated at our institution. MATERIALS AND METHODS Patients admitted for bladder exstrophy at our institution since 1980 were identified and the medical charts were reviewed. Continence questionnaires were completed during followup appointments or by mail. Continence was defined as complete-dry greater than 3 hours during the day and night with 2 or fewer night wets per month and partial-dry 2 hours or more during the day and 3 or greater night wets per month, and/or stress incontinence. The degree of pelvic organ prolapse was assessed in females older than 12 years. RESULTS Data were available on 31 Kelly patients, including 14 females, 4 to 25 years old and 13 patients, including 4 females, 2 to 29 years old treated with another staged technique. Of 30 Kelly patients without urinary diversion 21 (70%) were completely or partially continent. Of the 30 patients 17 voided spontaneously without clean intermittent catheterization or augmentation, of whom 12 (71%) were continent. Lower abdominal appearance was graded as abnormal in 11 of 12 male Kelly patients vs in 2 of 7 nonKelly males with pubic approximation (p = 0.01). Of the 12 females assessed none of 9 Kelly patients had prolapse, whereas 2 of 3 nonKelly patients had prolapse (p <0.05). CONCLUSIONS The continence rate after the Kelly operation compares favorably with that in recent series. The abnormal appearance of the lower abdomen and bony pelvis in Kelly males may result from a lack of pubic approximation. Importantly pelvic organ prolapse may be decreased in women after the Kelly technique.

Female pseudohermaphroditism in children with cloacal anomalies

Three patients are described with 46XX genotype, pseudohermaphroditism, a cloaca, and preserved renal function. Endocrinological causes of the phallic enlargement and labial fusion were absent. The increasing number of reports of this constellation of abnormalities is suggestive of a syndrome where embryogenesis is independent of hormonal status. Masculinisation of the external genitalia appears to be secondary to a fundamental derangement of urogenital development. An early anomaly well before sexual differentiation might prevent the genitalia from acquiring their normal, androgen-dependent growth controls, allowing the genital tubercle and genital folds to develop autonomously.

Percutaneous Transluminal Angioplasty for Transplant Renal Artery Stenosis in Children

Severe hypertension developed secondary to renal artery stenosis in 11 of 229 children who received a renal allograft. Renal artery stenosis was suspected because of de novo development of hypertension or exacerbation of pre-existing hypertension, which was detected 1 to 24 months after transplantation. Selective renal angiography was performed 2 to 74 months after transplantation (mean 13 months). Follow-up was 1 to 8 years (mean 2.5 years). The stenosis involved the anastomosis in 5 patients and was distal to the anastomosis in 6. One graft had an arteriovenous malformation. Seven grafts were suitable for vessel dilation; percutaneous transluminal angioplasty was partially successful in 4 cases in which the stenosis occurred at the anastomosis. The remaining patients were treated with medical therapy alone and the grafts were not lost. Our findings suggest that strictures distal to the anastomosis rarely are amenable to percutaneous transluminal angioplasty and should be treated medically whenever possible. Strictures at the anastomosis respond to vessel dilation but antihypertensive medication also often is required. An operation should be reserved for patients who do not respond to these measures.

Pancreatic pseudocysts in children

Pancreatic pseudocyst (PPC) is an uncommon condition in childhood and is almost always associated with blunt abdominal trauma. Additional disease within the pancreas is rare, unlike adult experience. Prior to the advent of ultrasonography (US) assessment of the cyst was difficult. Subsequently, it has become apparent that PPC may develop but still undergo spontaneous regression. We have reviewed eight children with PPC seen at the Royal Childrens Hospital between 1967 and 1987: six males and 2 females ranging in age from 2 to 12 years. The aetiology was blunt abdominal trauma in seven cases and idiopathic pancreatitis in one. Four children presented acutely and the diagnosis of PPC was made by US within 10 days of admission. One of these children had a diagnostic laparotomy for traumatic pancreatitis and his PPC was identified subsequently on serial US. The PPC in this child and one other resolved spontaneously after a period of total parenteral nutrition and intestinal rest. In two cases, the PPC failed to resolve and drainage was necessary. Late presentation (5–16 weeks) after recognised injury occured in four children; all required surgery within 1 week of admission. No surgical complications occured. Conservative management of PPC has been condemned because of the risk of complications occurring during the waiting period. This applies to adults who have developed a PPC in a pancreas diseased due to alcohol and/or cholelithiasis; the natural history of PPC is quite different in children. We thus recommend a trial of conservative treatment for all children who develop a PPC early, but consider that surgery is the best choice for those who present later.

Minimal surgery in the prune belly syndrome

In the past 20 years 14 patients with the prune belly syndrome have been seen. A treatment regimen consisting of minimal operative intervention, prophylactic antibiotics, and regular surveillance has been followed. Four patients died in the first 4 weeks of life from grossly inadequate renal function and other anomalies. Another patient died in early adult life after a late presentation. The remaining 9 patients have recently been plan using minimal surgical intervention is a satisfactory alternative.

Reimplantation of obstructive megaureters with and without tailoring.

OBJECTIVES Reimplantation of megaureters may be complicated by excessive bulk of tissue when plicated, or stenosis at the anastomosis site when excisional tapering is performed. Some urologists in our institution reimplant megaureters without tailoring, while others routinely tailor megaureters. Aiming to compare success rates, we report our experience in reimplanting obstructive megaureters with and without tailoring. MATERIALS The medical records of 30 boys and four girls with primary obstructive megaureters (a total of 38 ureters) were reviewed. Median age at operation was 18 months (range 1 month to 8 years). Of the ureters, 16/38 were tailored and 22/38 were reimplanted without tailoring. RESULTS Preoperative mean diameter (per ultrasound) of the 16 tailored ureters was 16.1mm (range 10-26 mm). Postoperative ultrasound showed resolution of hydronephrosis in 3/16 (19%), an improvement in 11/16 (69%), and no change in the degree of hydronephrosis in 2/16 (13%). Preoperative mean diameter of the non-tailored ureters was 14 mm (range 10-27 mm), and postoperative ultrasound showed resolution of hydronephrosis in 11/22 (50%), an improvement in 10/22 (45%), and no change in the degree of hydronephrosis in 1/22 (5%). Of the tailored ureters, 1/16 needed re-operation because of anastomotic stricture, and one girl had recurrent urinary tract infections. Of the non-tailored ureters, four had recurrent infection, of whom one boy underwent redo reimplantation because of grade 2 vesicoureteric reflux. CONCLUSION In this series non-tailoring reimplantation was an equivalent option to tailoring in obstructive megaureters.

Prenatal intussusception of a vitello-intestinal duct in association with ileal atresia

A complication of patent vitello-intestinal ducts is intussusception producing a double-horned prolapse of small bowel through the duct. We report a previously undescribed variant where prolapse occured prenatally and resulted in complete atresia of the distal horn as far as the transverse colon.

The spectrum of bronchopulmonary foregut malformations

Bronchogenic cysts and pulmonary sequestrations have in the past been defined as separate congenital pathological entities. The association of both in one mediastinal lesion is very rare. Such a case is reported, and lends support to the concept that bronchogenic cysts and pulmonary sequestrations belong in a spectrum of bronchopulmonary malformations due to abnormal ventral budding of the primitive foregut. Their exact position in the spectrum is probably dependent upon several independent key embryological events.