Randomized clinical trial of glutamine-supplemented versus standard parenteral nutrition in infants with surgical gastrointestinal disease.

BACKGROUND: Addition of glutamine to parenteral nutrition in surgical infants remains controversial. The aim of this trial was to determine whether glutamine supplementation of parenteral nutrition in infants requiring surgery would reduce the time to full enteral feeding and/or decrease the incidence of sepsis and septicaemia. METHODS: A prospective double-blind multicentre randomized clinical trial was performed in surgical infants less than 3 months old who required parenteral nutrition. Patients were allocated to treatment or control groups by means of minimization. Infants received either 0·6 g per kg per day alanyl-glutamine (treatment group) or isonitrogenous isocaloric parenteral nutrition (control group) until full enteral feeding was achieved. Primary outcomes were time to full enteral feeding and incidence of sepsis. Cox regression analysis was used to compare time to full enteral feeding, and to calculate risk of sepsis/septicaemia. RESULTS: A total of 174 patients were randomized, of whom 164 completed the trial and were analysed (82 in each group). There was no difference in time to full enteral feeding or time to first enteral feeding between groups, and supplementation with glutamine had no effect on the overall incidence of sepsis or septicaemia. However, during total parenteral nutrition (before the first enteral feed), glutamine administration was associated with a significantly decreased risk of developing sepsis (hazard ratio 0·33, 95 per cent confidence interval 0·15 to 0·72; P = 0·005). CONCLUSION: Glutamine supplementation during parenteral nutrition did not reduce the incidence of sepsis in surgical infants with gastrointestinal disease. REGISTRATION NUMBER: ISRCTN83168963 (http://www.controlled-trials.com).

Roux-en-Y feeding jejunostomy in infants and children.

Roux-en-Y jejunostomy (RYJ) permits enteral feeding in children unable to tolerate oral or intragastric feeds. It avoids many of the complications of nasojejunal and gastrojejunal tubes. Here we report our experience of intubated RYJ. By retrospective review of patient notes from 1998 - 2003, thirty-five children were identified. The median age was seven months (1 month - 16 years) and median follow-up was twenty-five months (1 - 55 months). There were no early postoperative complications or deaths. There were eighteen (51 %) late complications: peristomal leak (6), peristomal infection (2), bilious vomiting (5), tube displacement (3), abscess (1) and jejuno-colic fistula (1). Five children progressed to full oral feeds and had the RYJ resected. Fourteen of the twenty-eight children still alive remain fed by RYJ. Nine infants subsequently underwent fundoplication and gastrostomy with RYJ resection. Seven children died during the study period. RYJ is a straightforward procedure which can be performed safely in the face of poor nutrition and significant comorbidity. We recommend RYJ as a medium-term measure for enteral feeding.

Exomphalos major, oesophageal atresia and tracheooesophageal fistula--a rare combination of major malformations.

Exomphalos major, oesophageal atresia and tracheooesophageal fistula are rare but well described birth defects. Both may be associated with other anomalies but rarely have been described as occurring together. We present a neonate born both premature and with these anomalies who presented major management difficulties.

Tailgut cyst in a neonate with anal stenosis.

Tailgut cysts, embryological remnants of the hindgut, are rare retrorectal tumours. They have been described in adults but are rare in children, especially neonates. We report a case of a neonate, who presented with anal stenosis and an incidental ultrasonographic finding of a presacral mass. Excision and histological examination of the mass confirmed the appearance of a tailgut cyst. There were no postoperative complications and no evidence of recurrence of the presacral mass over one year after excision. The clinical, pathological, and radiological findings of the tailgut cyst are described with a review of the literature.

Complications with shunts in adults with spina bifida.

OBJECTIVE: To assess the incidence of malfunction of shunts in adults with spina bifida who have shunts to control hydrocephalus. DESIGN: A retrospective review of the medical notes and contact by questionnaire of adults with spina bifida to assess symptoms, function of shunts, frequency of operative procedures, and follow-up. SUBJECTS: 110 patients with shunts who attended Lord Mayor Treloar College for the physically disabled between 1978 and 1993. RESULTS: The average (range) number of revisions of shunts per person was 3.6 (0-28). Although 37 patients underwent an emergency operation for revision in their first year of life, there was a continuing low incidence, increasing in the early teenage years, which persisted into the third decade. Intervals between emergency revisions varied: 202/320 occurred within one year of the last shunt operation, 56 occurred after five years, 24 after 10 years, and 15 after 15 or more years. Fifteen patients had chronic intermittent headaches, of whom four died and three suffered severe morbidity. Thirteen died; three had raised intracranial pressure, and four died suddenly; these deaths were presumed to be related to their shunts. Up to the age of 16 there was 100% hospital follow up, but after that only 40% of young adults underwent review, including review of their shunt function. CONCLUSION: Shunts to control hydrocephalus may fail after many years without symptoms. This is difficult to diagnose and if missed may lead to chronic morbidity and death. As hospital follow up of this group is falling, both general practitioners and hospital doctors must be aware that a shunt may malfunction after prolonged quiescent periods.

Is there a role for high jejunostomy in the management of severe necrotising enterocolitis?

Necrotising enterocolitis (NEC) remains a common and severe condition affecting both preterm and term infants. Treatment when NEC involves a large proportion of the gastrointestinal tract remains controversial. We present one surgeon's experience of high jejunostomy (HJ) as the primary procedure in this group of children. HJ was performed in the presence of severe panintestinal disease. In those who survived, a second-look procedure and reconstruction was performed after 6 to 8 weeks. Over a 16-year period, 10 of 113 patients with NEC had a HJ constructed. Two died within 1 day due to persisting instability; the others survived to undergo a second-look laparotomy. Intestinal continuity was restored in all cases with one to five anastomoses. Three patient's died within 1 year from total parenteral nutrition (TPN) related cholestasis and cirrhosis. Five became long-term TPN-free survivors. The HJ as an initial procedure is a useful surgical option in neonates with severe NEC affecting the majority of the intestine. In this high-risk group, we achieved 50% survival from NEC.

Mixed gonadal dysgenesis and cell line differentiation. Case presentation and literature review.

A male patient with mixed gonadal dysgenesis, involving a streak gonad on the right and a histologically normal testis in the left, was found to have a 45,X/46,X,dic(Yp) chromosome constitution on peripheral blood cultures. Fibroblasts grown from both gonads showed the "normal" testicular tissue to have e 45,X/46,X,dic(Yp), whereas the cells from the streak gonad were all 45,X. The structure of the dic(Yp) chromosome was confirmed using non-isotopic in situ hybridization with Y centromere and Yp specific probes. On hormonal stimulation, testosterone levels rose by 50%. The "normal" testis was left in situ, but close follow up will be required in view of the malignant potential.

Radiation dosage during AO locking femoral nailing.

Radiation dosages to both the patient and the surgeon were measured during 10 consecutive AO locking nail procedures. The total screening time for each procedure averaged 12 min 8 s; the average screening time for distal locking was 6 min 52 s. Radiation dosages to surgeons' hands, thyroid and gonads and to the patients were well within permitted safety levels.

Tubularization of the incised urethral plate (Snodgrass procedure) for primary hypospadias surgery.

OBJECTIVE: To evaluate the Snodgrass procedure for patients with hypospadias who have not undergone previous surgery and whose urethral plate is too narrow for tubularization alone. PATIENTS AND METHODS: Thirty-two boys (mean age 18 months) underwent primary hypospadiac surgery performed by one surgeon (P.S.M.). Twenty-five boys had a distal hypospadias (coronal, subcoronal) and seven had a more proximal defect (penile shaft, penoscrotal). The operation involved incision of the urethral plate, which was then tubularized (Snodgrass procedure). The neourethra was then covered with a de-epithelialized pedicled dartos flap from the inner prepuce before glans and skin closure. RESULTS: With a mean follow-up of 10 months (range 2-14) there were two complications; one child with a coronal hypospadias developed a fistula whist one with a penile shaft defect had complete breakdown of the neourethra. The cosmetic appearance in the other 30 patients is that of a normal slit-like terminal meatus. CONCLUSION: Tubularization of the incised urethral plate is a safe advance in the surgery of hypospadias. We recommend it for both distal and proximal defects, in patients where the urethral plate is insufficient for tubularization alone.

Transversely tubularized ileal segments for the Mitrofanoff or Malone antegrade colonic enema procedures: the Monti principle.

OBJECTIVE: To assess the use of a transverse tubularized segment(s) of ileum in the Mitrofanoff or Malone antegrade colonic enema (MACE) procedures. PATIENTS AND METHODS: Eleven patients in three centres underwent the formation of a continent conduit to bowel (MACE, eight patients) and/or bladder (Mitrofanoff, four) using either a single segment of transverse tubularized ileum (10 patients) or two segments of ileum anastomosed and tubularized into a single conduit (two). RESULTS: Within a follow-up of 8 weeks to 6 months, all conduits were continent and catheterized easily. One stomal stenosis required a revision procedure. CONCLUSION: This method for forming a continent catheterizing conduit, based on the Mitrofanoff principle, appears to be effective and is recommended in cases where the appendix cannot be used or where a second conduit is required.

Bacteriological state of the urine in symptom-free adult males.

Mid-stream urine specimens from 120 symptom-free males aged between 15 and 60 years were examined for cells and bacteria. Only 3 specimens showed pyuria, all of which yielded fastidious organisms. No specimen yielded a pure growth of an aerobe. Fastidious organisms in pure or mixed growth were isolated from 26% of the specimens, none in a count greater than 10(8)/l and the majority in counts less than 10(7)/l. It was concluded that pure growths of aerobes in any count in patients with symptoms are indicative of infection. Fastidious organisms should be sought in the urine of patients with symptoms or pyuria before more intensive and expensive investigations are pursued.