Urinary incontinence - an unusual indication for early vaginoplasty in late presenting congenital adrenal hyperplasia.

BACKGROUND: Congenital adrenal hyperplasia (CAH) is an autosomal recessive condition leading to deficient cortisol with an incidence of 1/16,000. Patients with CAH typically present early with ambiguous genitalia or as an emergency with adrenal crisis. CASE: We report an atypical late presentation of a 4-year-old girl with pubertal-like symptoms and urinary incontinence, due to a persistent urogenital sinus (UGS). An early vaginoplasty was successfully performed allowing the patient to achieve continence. CONCLUSION: Literature describing the symptoms of CAH with UGS is scarce. The case is unusual in demonstrating pubertal-like symptoms and urinary incontinence due to the late presentation of a persistent UGS, highlighting the need for an open mind in assessment of children with urinary incontinence. Timing of surgery is controversial, and cases need to be considered on an individual basis.

Peritoneovenous shunting is an effective treatment for intractable ascites.

AIM AND METHODS: A retrospective review was carried out of children undergoing peritoneovenous shunting for intractable ascites. RESULTS: 11 children, aged 3 months to 12 years (median 31 months) underwent peritoneovenous shunting over the past 17 years. The duration of ascites ranged from one month to 2.5 years (median two months). The primary pathology consisted of previous surgery in eight (three neuroblastoma, one renal carcinoma, one hepatoblastoma, one adrenal teratoma, one renal artery stenosis, and one diaphragmatic hernia), and cytomegalovirus hepatitis, lymphatic hypoplasia, and lymphohistiocytosis in one patient each. All patients had failed to respond to previous treatment including peritoneal drainage in six patients, diuretics in five, and parenteral nutrition in five. There were no intraoperative problems. Postoperative complications included pulmonary oedema in three patients, shunt occlusion in three, infection in two, and wound leakage in one. Ascites resolved after shunting in 10 patients. Five shunts were removed one to three years after insertion without recurrence of ascites. Three others are free of ascites with shunts in place for less than one year postoperatively. Three children died from their underlying disease: two after resolution of ascites (neuroblastoma) and one in whom the ascites failed to resolve (lymphohisticytosis). CONCLUSIONS: Peritoneovenous shunting is an effective treatment for symptomatic intractable ascites in children (10 of 11 successful cases in this series). Elective removal of the shunt after one year is recommended.

The use of ureteral stents and suprapubic catheter in vesicoureteric reflux surgery.

The use of ureteric stents in reimplantation surgery is important. The younger the patient, the more important the stenting of ureters post reimplantation becomes, because even minimal oedema following surgery will produce ureteric obstruction unless stents are in place. JJ stents are now the preferred method of choice in ureteric reimplantation surgery, but in the past the patient required another admission to hospital and general anaesthetic to have the stents removed endoscopically. We describe a technique whereby the stents are attached to the suprapubic catheter and are therefore removed prior to the patient's discharge from hospital, thus obviating the need for a second admission and second anaesthetic for the stent removal. We have studied 23 patients with this technique and find that it is a reliable and safe method to use.

The incidence and investigation of acute scrotal problems in children.

The true incidence of the various causes of acute scrotum in children is unclear; epididymo-orchitis (EO) is thought to be uncommon. Investigation for underlying urological abnormality in children with EO is recommended. To determine the incidence of the various pathologies in boys presenting to the emergency department with an acute scrotal condition and assess the value of further investigation of the urinary tract in a subgroup of boys with EO, a retrospective review of 100 consecutive patients admitted with a diagnosis of testicular pain was performed. Seventy had torsion of an appendix testis (TAT) and 12 had torsion of the testicle (TT). Ten boys were admitted with 11 episodes of EO; 7 had other pathologies including incarcerated hernia, varicocoele, and idiopathic scrotal oedema. The diagnosis of EO was made at operation in all 11 episodes. Escherichia coli was cultured in 4 patients; none were found to have underlying urinary tract abnormality. TAT was thus commonest causes of the acute scrotum. EO is not rare in infants, the incidence in this study being equal to the of TT. Subsequent urological investigation did not disclose any underlying abnormality. However, based on the current published literature futher investigation is recommended in selected cases.

A new complication of the Malone antegrade continence enema.

The Malone antegrade continence enema now commonly known as the ACE procedure was described originally for the treatment of intractable fecal incontinence in 1990. Since this time it has been used widely in many centers for the treatment of constipation and faecal soiling in both adult and pediatric practice. This case report describes a previously unreported complication of an ACE that developed in a disused appendicocecostomy in a child.

Continent catheterizable conduits: which stoma, which conduit and which reservoir?

OBJECTIVE: To assess the outcome of the various methods used in creating continent catheterizable conduits. PATIENTS AND METHODS: The case notes were reviewed from 89 patients who underwent the formation of 112 continent catheterizable conduits. RESULTS: Sixty-five conduits were Mitrofanoff and 47 were antegrade colonic enema (ACE); 21 patients had both. At a mean follow-up of 34 months, 95 (85%) conduits were still in use. There was no difference in complications between the Mitrofanoff and ACE conduits; 109 (97%) conduits were continent and stomal stenosis occurred 35 (31%). There was no significant difference relating to the conduit used, the reservoir, the stoma type or the stoma site. Only 39% of patients required no revisional surgery. CONCLUSION: Although urinary and fecal continence can be achieved in most patients there is a high burden of complications and revisional surgery. All patients should be counselled accordingly.

The effects of intravenous epidermal growth factor on bacterial translocation and central venous catheter infection in the rat total parenteral nutrition model.

Sepsis is a major complication of total parenteral nutrition (TPN) in children. Gut mucosal atrophy (GMA) and bacterial translocation (BT) occur in patients receiving TPN, and the translocated enteric organisms may cause central venous catheter (CVC) infection. Epidermal growth factor (EGF) has a trophic effect on the gut mucosa and may reduce BT, thereby reducing catheter infection. Using a rat TPN model, the relationship between GMA, BT, and catheter sepsis was examined and the effect on these of intravenous EGF was studied. There were four experimental groups. Group 1 had no CVC, Groups 2, 3, and 4 had a continuous central venous infusion as follows: group 2, saline; group 3, TPN; group 4, TPN with EGF. Groups 1 and 2 had free access to chow, groups 3 and 4 had no enteral feeds. After killing at 1 week, blood, tissue, and catheter specimens were cultured and mucosal morphology analysed. BT was defined as the presence of the same organism in cultures from the gut lumen and mesenteric lymph nodes (MLN). TPN only (group 3) resulted in GMA and BT, and 5 of 12 animals with BT had the same gut bacteria in blood and/or catheter cultures. The addition of EGF to the TPN significantly reduced GMA, BT to the MLN, and blood and/or catheter infections (P = <0.05). In animals carrying enterococci, there was a significant reduction in translocation of enterococci (group 3: 8/14; group 4: 0/11; P<0.05) and catheter infection by enterococci was prevented (group 3: 3/14; group 4: 0/11). EGF thus reduced GMA, BT, and blood and/or catheter infection when given IV to rats receiving TPN. Enterococcal translocation and subsequent blood and/or catheter infection was completely prevented, suggesting a selective effect of EGF.

Intravenous glutamine or short-chain fatty acids reduce central venous catheter infection in a model of total parenteral nutrition.

BACKGROUND/PURPOSE: In children receiving total parenteral nutrition (TPN) translocated enteric organisms are a possible cause of central venous catheter infection. The aim of this study was to determine the relationship between gut mucosal atrophy, bacterial translocation, and catheter sepsis in rats receiving TPN alone or supplemented with intravenous short chain fatty acids (SCFA) or glutamine. METHODS: Mature Wistar rats were studied. Groups 2 to 5 had a central venous catheter inserted. Group 1 (n = 12) had enteral feeds only, group 2 (n = 14) had enteral feeds and intravenous saline, group 3 (n = 15) had TPN only, group 4 (n = 13) had TPN with SCFA, and group 5 (n = 15) had TPN with glutamine. At 1 week, blood, tissue, and catheter specimens were obtained for culture and mucosal morphometry. RESULTS: Villus height and crypt depth were reduced significantly in group 3 compared with group 1 (P < .05). Glutamine and SCFA significantly ameliorated the mucosal atrophy. Significant bacterial translocation and catheter infection occurred in group 3. Reduced translocation with SCFA was not significant, but catheter infection was (P < .05). Glutamine significantly reduced translocation and catheter infection. CONCLUSIONS: There is an association between bacterial translocation and central venous catheter infection. The risk of catheter infection is reduced by supplementing TPN with SCFA or glutamine.