A prospective audit of hypospadias correction in a regional paediatric surgery centre.

AIM: To prospectively review the management and treatment of hypospadias in a single regional centre, and in particular, to assess the spectrum of cases treated, techniques used and to determine the nature of the complications. METHODS: One hundred and fifty-three consecutive boys undergoing hypospadias repair during a 36-month period were included in the study. Information was collected prospectively and included the site of the urethral meatus, presence of chordee, surgical technique employed, use of urinary diversion, and the prescription of postoperative antibiotics and analgesics. Patients were assessed in the clinic following surgery at which time information on outcome and complications was obtained. RESULTS: One hundred and fifty-seven procedures for hypospadias were performed. Single-stage reconstruction was performed in 145 boys. GRAP (glanular reconstruction and preputioplasty) repair was the most common operation employed (n=112). The overall fistula rate was 11.7 % with the majority of patients having a satisfactory functional and cosmetic outcome following surgery. CONCLUSION: A variety of techniques can be employed to provide satisfactory correction of hypospadias with an increasing emphasis on single-stage day case procedures. GRAP repair is the favoured option for distal hypospadias and incorporates preservation of the prepuce.

Surgical management of ambiguous genitalia.

The surgical management of children born with ambiguous genitalia has always been difficult, subject to evolving attitudes and techniques, and at times controversial. Standard protocols have stressed the need for early diagnosis, gender assignment, and appropriate surgery in infancy.(1) In recent years some authors, backed by patient support groups, have claimed that such surgery is damaging or mutilating and, as it is essentially cosmetic, should not be performed until the fully informed consent of the patient could be obtained-that is, when the child becomes "Gillick competent".(2-)(4) There are, however, so many specific issues related to the different diagnostic groups that such a policy would seem to be too prescriptive.

The Pippi Salle procedure for neurogenic urinary incontinence in childhood: a three-year experience.

AIM: A three-year retrospective study was performed to assess the place of the Pippi Salle bladder neck repair in the treatment of neurogenic bladder incontinence in both sexes in childhood. METHOD: All children who underwent the procedure between November 1995 and November 1998 were studied retrospectively. There were 18 children: 12 with spina bifida, 5 with sacral agenesis and 1 with an idiopathic neurogenic bladder. There were 11 boys and 7 girls. Their age was 3-14 years (median 7 years) and the length of follow-up was 7 months to 3 years (median 2 years). A midline bladder wall flap was fashioned in all patients. The procedure was performed alone (n = 5) or in combination with a sigmoid colocystoplasty (n = 9) or a Mitrofanoff catheterisable stoma (n = 2) or both (n = 2). RESULTS: Diurnal continence (3 hours or more) was achieved in 11 of the 18 children (61%); 2 are dry for 2-2.5 hours and 5 are incontinent. Eight of the 18 children (44%) are completely dry during the night, 2 are damp and 8 are incontinent. Twelve children (67%) needed oxybutynin to maintain a good result. Complications arose in 5 children (28%): difficulty with catheterisation (n = 4) and pelvic abscess (n = 1). Seven children (39%) subsequently required further operations: exploration of bladder for urine leak (n = 2), reinsertion of dislodged catheter (n = 1), drainage of pelvic abscess (n = 1), bladder neck injection with macroplastique (n = 1) and bladder neck closure (n = 2). CONCLUSIONS: The Pippi Salle bladder neck repair has an undoubted place in the management of neurogenic urinary incontinence in childhood. However, in our experience, it has a relatively high complication rate and failure rate.

Hyperammonaemia due to Klebsiella infection in a neuropathic bladder.

A boy with a neuropathic bladder and a single hydronephrotic kidney developed hyperammonaemic encephalopathy during a urinary tract infection with Klebsiella oxytoca. Although particularly associated with Proteus infections and prune belly syndrome, hyperammonaemia can complicate infection with any urease-producing bacteria if there is urinary stasis.

The use of the detrusorrhaphy for vesico-ureteric reflux: the way forward?

OBJECTIVE: To assess the outcome of all detrusorrhaphies carried out for simple single-system vesico-ureteric reflux (VUR) in children at one institution over a 4-year period. PATIENTS AND METHODS: Twenty-nine patients (11 boys, 18 girls, 43 renal units; mean age at presentation 23 months; range, antenatal to 72 months) were analysed in two groups. Group 1 comprised those undergoing asynchronous bilateral procedures (two patients, four units) and unilateral procedures (15 patients, 15 units), and group 2, those undergoing synchronous bilateral procedures (12 patients, 24 units). The mean (range) follow-up was 17 months (6-39) and 15 months (7-24), respectively. RESULTS: Three patients had antenatal hydronephrosis related to VUR post-natally and 26 had urinary tract infections (13 recurrent, one with haematuria, seven 'breakthrough', one with calculi and four with enuresis). The mean age at operation was 54 months (range 14-167). The mean (range) duration of anaesthesia was 69 min (40-120) in group 1 and 80 min (65-120) in group 2. All patients were catheterized urethrally for a mean (range) duration of 3 days (2-4) in group 1 and 5 days (2-15) in group 2 and the mean hospital stay was 3 days (2-6) and 6 days (4-16), respectively. Post-operative complications occurred in 14 patients including one bladder spasm, five urinary tract infections, two with urinary retention, three with haematuria and one each of pneumonia, epididymo-orchitis, anuria, failure to stent and conversion to Cohen reimplantation. Operative success and clinical success were similar within groups but differed between groups, although not significantly (15 of 17 in group 1 and eight of 12 in group 2). CONCLUSION: Unilateral but not synchronous bilateral detrusorrhaphy seems an appropriate surgical treatment for VUR.

Is delayed surgery really better for congenital diaphragmatic hernia?: a prospective randomized clinical trial.

Delayed surgery has become widely accepted in the management of congenital diaphragmatic hernia after comparing outcomes only with historical retrospective controls. It was the aim of this study to compare early and delayed hernia repair in a randomized prospective clinical trial. Fifty-four infants were randomized to receive either early repair (within 4 hours of admission) or delayed repair (more than 24 hours after birth). The survival rate was higher for the delayed group (57% v 46%), but the difference was not significant (difference: -11; 95% confidence limits: -37.5, 15.5). There were no significant differences between the two groups with respect to length of hospital stay, ventilator dependency, or survival time. Recorded preoperative risk factors were similar for the two groups. Eight infants in the delayed repair group died without having undergone surgery. The optimum time for surgery still needs clarification.

Dorsal relieving incision for the non-retractile foreskin.

The operation of dorsal relieving incision (DRI) for non-retractibility of the foreskin is described and the results of a retrospective review of 67 boys so treated are presented. We conclude that DRI is a simple, safe and effective procedure for the non-retractile foreskin not affected by fibrotic scarring.

Surgical aspects of chronic peritoneal dialysis in the neonate and infant under 1 year of age.

Since 1982 eight patients under 1 year of age with end-stage renal failure have been treated by chronic peritoneal dialysis (CPD) following insertion of an abdominal Tenckhoff catheter. We routinely perform a partial omentectomy now, and in males undertake bilateral exploration of the groins at the time of catheter insertion, with herniotomy or ligation of the patent processus vaginalis as required. Up to January 1990, 19 straight double-cuff catheters had been inserted with a total follow-up of 244.5 patient months. The median age at the initial catheter insertion was 14.6 weeks (range, 2 days to 11 months) and the median weight was 3.89 kg (range, 2.2 to 5.5). Peritonitis was the most common complication, with 46 episodes, representing one episode of peritonitis per 5.3 patient months on dialysis. The frequency of peritonitis has decreased in the last 6 months since all patients have been dialysed by two caregivers. The present rate of peritonitis is 1 episode per 10 patient months on dialysis. One patient has died of septicemia secondary to associated congenital abnormalities, one patient has regained renal function, and two patients have been transplanted, one successfully. Five patients are currently dialysing via their abdominal Tenckhoff catheters and awaiting transplantation. We conclude that neonates and infants under 1 year of age can be treated satisfactorily by CPD to enable successful preparation for transplantation later in childhood.

Oesophageal and severe gut involvement in the haemolytic uraemic syndrome.

Between 1982 and 1989, 78 children with diarrhoea-associated haemolytic uraemic syndrome (HUS) were referred to this hospital. Most presented with abdominal pain, bloody diarrhoea and vomiting. Seven had severe gastrointestinal involvement, four of whom required resection for bowel perforation or necrosis. One also developed an oesophageal stricture, a previously unreported complication of HUS. These seven children had a high incidence of other complications including hypertension, and cerebral and pancreatic involvement. One died from severe cerebral involvement, one has a residual neurological deficit and one has residual renal impairment. Severe gastrointestinal involvement did not significantly affect the long-term outcome. Simple haematological indices helped predict severe gut involvement. Four of the 78 children had undergone appendicectomy before the diagnosis of HUS was made. The operative findings were in no case typical of primary acute appendicitis, although histological examination did confirm inflammation of the appendix in two patients. Diagnosis is difficult in early disease, but increased awareness may help prevent unnecessary appendicectomy.

Comparison of postpyloromyotomy feeding regimens in infantile hypertrophic pyloric stenosis.

One hundred infants undergoing pyloromyotomy were studied prospectively to ascertain the optimum time at which to reintroduce feeds after operation. The babies were randomized to be fed after 4 or 18 h and were then assessed by monitoring the frequency of postoperative vomiting and the number of days taken to achieve full oral feeding. There was significantly more postoperative vomiting in the early group, while both groups required the same number of days to achieve full oral feeding. The conclusion reached was that a delay in the reintroduction of feeds until 18 h after pyloromyotomy is to be recommended.

Ureteral calculi in children: review of 50 consecutive cases.

Fifty children presenting with ureteral calculi over a twenty-year period were studied retrospectively. There were 35 boys and 15 girls, with a mean age of five and a half years. Nineteen patients had a urinary tract anomaly: 11 had an obstructed megaureter, and 8 had previously undergone urinary tract surgery. Forty-four children had infection stones and the remaining 6 oxalate calculi. Infected urine was found in 35; the infecting organism was a Proteus species in 23. Metabolic abnormalities were detected in 2 children. Operative removal of the stones was done in 39 patients, and in the remaining 11 patients the stones passed spontaneously. There were 6 recurrences, all within four years of initial presentation.

Thyroglossal-duct lesions in childhood.

A retrospective review of 300 cases of thyroglossal duct lesions is presented, with emphasis on reasons for misdiagnosis and recurrence. Of 28 cases misdiagnosed, 20 presented in atypical sites. The recurrence rate after Sistrunk's operation was 5% but no special features could be identified to explain these recurrences. While previous surgery does reduce the chance of success, inflammation does not seem to affect the recurrence rate.

Functional intestinal obstruction associated with malrotation and short small-bowel.

Two siblings are presented in whom a recognized syndrome of functional small-bowel obstruction with malrotation and short small-bowel was diagnosed. The previously described association with pyloric stenosis was not present in our cases. They do, however, support the view that this syndrome has an autosomal recessive model of inheritance. A deficiency of argyrophil neurones in the myenteric plexus has been noted in this syndrome but more precise neurohistochemical studies are required to define the exact deficit involved.

Endoscopic diathermy obliteration of recurrent tracheoesophageal fistulae.

Recurrence of tracheoesophageal fistula after surgical repair for esophageal atresia occurs in approximately 5% to 15% of cases. Further surgery may be hazardous and mortality rates of up to 50% have been reported. We report two patients in whom successful obliteration of a recurrent fistula has been achieved by diathermy at bronchoscopy. This experience is compared to previous reports of fistula obliteration using tissue adhesives.

Congenital auricular and preauricular sinuses in childhood.

Congenital auricular and preauricular sinuses, while seemingly trivial and often unnoticed or ignored, can give rise to troublesome symptoms if infection supervenes. Nine of 30 patients (30%) operated upon between 1969 and 1981 had postoperative recurrence, two of whom required two excisions. We would recommend aggressive treatment of infection followed by excision in 2-3 weeks before further infection can occur.

Perforated appendicitis in children: use of metronidazole for the reduction of septic complications.

Fifty-four consecutive children with performed appendicitis were treated with intravenous and rectal metronidazole combined with another antimicrobial agent. Positive cultures were obtained from peritoneal swabs from all children except from four patients who produced no growth on culture. Mixed growth of aerobic and anaerobic organisms were isolated from 44 patients. Four patients had pure growth of aerobes and two had pure growth of anaerobes. Results were compared with 49 cases of performed appendicitis treated with a combination of Gentamicin and Cephradine and who did not receive metronidazole. The overall incidence of complications was reduced from 44.9% to 14.8%. A significant reduction in the incidence of wound infection (p less 0.01) and pelvic abscess (p less than 0.025) was observed in the metronidazole treated group. The period of hospitalization averaged 13.8 days in patients treated with metronidazole as compare to 18.2 days in those treated without metronidazole.

Neonatal small left colon syndrome.

Five patients with neonatal small left colon syndrome are described together with some early investigative studies on rectal biopsy material. Current reports on this condition are reviewed. The precise aetiology remains unknown, but vigilance and early referral are recommended in view of the several reports of colonic perforation.

Diagnostic value of rectal mucosal acetylcholinesterase levels in Hirschsprung's disease.

Acetylcholinesterase (AChE) activity was measured in rectal biopsy specimens obtained from 68 children aged between 2 days and 14 1/2 years in whom Hirschsprung's disease was suspected. The diagnosis was subsequently established in 12; in these, the mean AChE activity was found to be 30.5 X 10(-7) units/g tissue (range 16.9 to 63.0). The 56 non-Hirschsprung cases had a mean of 5.0 X 10(-7) units/g tissue (S.D. 2.2), the highest value in this group being 10.9. The results were unaffected by age, sex, nature of biopsy procedure, or the presence of blood. It is suggested that the assay of AChE activity in rectal biopsy material is a simple and quick procedure that is useful in the diagnosis of Hirschsprung's disease.