Oesophageal substitution with free and pedicled jejunum: short- and long-term outcomes.

In children, the indications for oesophageal substitution are principally, long gap oesophageal atresia (OA), severe anastomotic disruption following primary repair of OA and severe caustic or peptic strictures. We present an outcome review of eight cases who underwent oesophageal substitution with jejunum at our institution between 1986 and 2001. The purpose of this study was to evaluate our experience with free/pedicled jejunal grafts and its long-term outcome as an oesophageal substitute. Operative and postoperative outcome with free and pedicled jejunal grafts in four cases of pure OA, two cases of OA and distal tracheo-oesophageal fistula (TOF), one patient with a high retrolaryngeal oesophageal web and one case of severe caustic oesophageal stricture. Six patients had an oesophagostomy and a gastrostomy fashioned previously. Eleven free jejunal grafts were performed in six patients (three intraoperative redo interpositions for immediate graft loss, three separate grafts in one patient and two free grafts in two patients). One patient's pedicled jejunal graft proximally required microvascular anastomosis while the other had a pedicled graft without microvascular anastomosis. Early postoperative complications included four upper anastomotic leaks (three free grafts, one pedicled with microvascular support), pneumothorax requiring prolonged ventilation and Horner's syndrome. Recurrent laryngeal nerve injury occurred in the patient who had a high retrolaryngeal oesophageal web. During follow up (5-18 years) late complications of upper anastomotic stricture in four patients and graft redundancy with subsequent kinking of the lower anastomosis were observed in one patient. Three patients established a complete oral diet; a further three patients relied on supplemental gastrostomy feeds and one patient is entirely gastrostomy fed. There were two late deaths, one from aspiration and the other from a severe asthmatic attack (5 and 7 months postoperatively, respectively). Our results indicate that there are significant complications related to the use of free jejunal grafts. Early recognition and treatment are of paramount importance in the ultimate achievement of a successful technical outcome.

Duodenal duplication cyst: beware of the lesser sac collection.

A 11-month-old boy presented with a 4-month history of failure to thrive. His initial presentation was highlighted by fever, postprandial abdominal pain and lethargy. The diagnosis was elusive despite extensive investigations. A contrast enhanced abdominal computerised tomogram (CT) suggested the presence of a pancreatic pseudocyst. At laparotomy, a lesser sac collection was drained and the patient's general condition improved. Three weeks postoperatively, the symptoms recurred and a second contrast enhanced abdominal CT revealed a duodenal duplication cyst. A 6.5-cm duodenal duplication cyst communicating with the fourth part of the duodenum was resected in its entirety with resolution of the patients' symptoms and establishment of adequate growth.

The primary management of testicular maldescent in gastroschisis.

Gastroschisis (GS) is the commonest abdominal-wall defect in the Western world. The conventional practice has been reduction of the viscera and closure of the abdominal wall as an emergency procedure. The testis is often a part of the prolapsed viscera along with the bowel loops, stomach, fallopian tube, etc. The primary management of prolapsed (PT) (3) and intra-abdominal (5) testes (IAT) in this condition was studied in 16 consecutive male babies with GS, each was managed by simple reposition of the testes and closure of the abdominal wall. The babies were followed up for spontaneous descent of the testes. At 18-month follow-up, all five IAT had descended into the scrotum spontaneously and were palpably normal. Of the three extra-abdominal PT, two had descended into the scrotum and were normal in size and on palpation. One was palpable in the superficial inguinal pouch. Simple reposition of the testes into the abdomen and closure of the abdominal defect is the correct approach for primary management of PT or IAT in a newborn with GS.

Central-venous access through the peripheral route in surgical neonates: an audit of 125 consecutive lines from a regional neonatal centre.

A long Percutaneous silastic IV line is frequently used in surgical neonates for infusion of hyperosmolar parenteral nutrition fluid into a central vein for several days without the need for operative insertion of a Broviac catheter or risks of direct puncture of a central vein. Our study was aimed at auditing the performance of 125 consecutive lines over a 2-year period. During this period, insertion was attempted in 125 babies; in 13 cases the line could not be inserted because of technical problems. The gestational ages varied between 25 and 41 weeks and weights between 630 g and 4.2 kg. Success did not appear to be related to the age or weight of the baby. The mean duration of complication-free performance was 22.4 days. There was a significant difference between the complication rate of lines inserted in the operating theatre versus those on the ward (P < 0.05). There was no significant increase in complications in lines used for over 4 weeks. The technique adopted by us for inserting these lines is likely to succeed in the majority of cases, including premature and small-for-dates babies. In our experience, lines inserted in the controlled theatre environment either before or after abdominal surgery performed better. The manufacturer's recommendation to electively change the line every 4 weeks needs further prospective evaluation.

Dysgerminoma associated with hypercalcemia: A case report.

Hypercalcemia is an extremely rare paraneoplastic manifestation of malignancy in children. The authors report a case of dysgerminoma in a 14-year-old girl associated with severe hypercalcemia that only resolved after excision of the tumor. There are only 6 previously reported cases of dysgerminoma associated with hypercalcemia. Serum calcium levels should be checked in all children with solid ovarian tumors. Hypercalcemia in such circumstances may be resistant to medical management, and surgical resection of the tumor results in the normalization of calcium levels.

Pyloric atresia: five new cases, a new association, and a review of the literature with guidelines.

BACKGROUND/PURPOSE: Pyloric atresia is an uncommon condition occurring in 1 of 100,000 live births. When occurring in isolation, the clinical course usually is uncomplicated after surgical treatment. However, it may occur in association with other congenital abnormalities. The authors present 5 new cases, 3 of associated abnormalities including 1 of esophageal atresia and 2 of agenesis of the gall bladder and malrotation. Agenesis of the gall bladder has not been described previously in combination with pyloric atresia. The literature has been reviewed and guidelines are suggested for the management. METHODS: The case records of 4 neonates who presented to the author's institution between January 1998 and June 1999 and 1 who presented at another center in 1991 were reviewed. A Medline literature search was performed, and guidelines were developed for the management of this condition based on our cases and the literature review. RESULTS: Patients 1 and 5 had no associated anomalies. Patient 2 had associated esophageal atresia, tracheoesophageal fistula, atrial septal defect, crossed renal ectopia, malrotation, and absent gall bladder. Patient 3 had a rectovestibular fistula, vaginal atresia, atrial septal defect, malrotation absent gallbladder, and absent extrahepatic portal vein. Patient 4 had epidermolysis bullosa. Patients 2 and 5 had unremarkable recoveries, patients 2 and 3 had markedly delayed gastric emptying that responded to cisapride. Patient 3 has portal hypertension and remains under close follow-up. Patient 4 died at 22 days of age of pseudomonas sepsis. CONCLUSIONS: Based on our cases and literature review, we have adopted the following guidelines: (1) All children with pyloric atresia should be screened for multiple anomalies. (2) Delayed gastric emptying should be considered early and may respond to prokinetic agents. (3) Association with Epidermolysis bullosa should not preclude surgical treatment. (4) A skin biopsy specimen should be taken at the time of surgery for electron microscopy if there is a family history of epidermolysis bullosa.

Backache: a rare diagnosis and unusual complication.

We report an adolescent girl who presented with an acute exacerbation of a chronic backache of 6 months duration. She complained of dribbling micturition, constipation, and an irresistible urge to strain for 72 h. In the waiting area of the local hospital she developed sudden, severe chest pain with progressive swelling of the upper torso. This proved to be the mode of presentation of a haematocolpos due to an imperforate hymen with the unusual complication of mediastinal emphysema. Imperforate hymen is a rare diagnosis, but should be considered when dealing with an adolescent girl with lower abdominal symptoms or backache.

Clinical features and prognosis of children assessed for isolated small bowel or combined small bowel and liver transplantation.

The hepatic histology and clinical status of 37 children on long-term parenteral nutrition (PN) referred for consideration of small bowel transplantation were determined. Seventy five percent of the children had splenomegaly and plasma bilirubin level of greater than 100 mumol/L. All of 21 children who underwent liver biopsy, had increased fibrosis, but only half had established cirrhosis. Thirty-one children were considered to be in need of transplantation (combined liver and bowel transplant, 29; isolated bowel transplant, 2), but only 13 were stable enough to be placed on the transplant list. Seven out of the thirteen children waiting have died because of lack of size-matched organs, and the overall mortality rate of the 37 children was 70%. The main risk factors for death within 6 months were bilirubin level of greater than 100 mumol/L, splenomegaly, and cirrhosis (P = .01). The natural history of PN-associated liver disease is that of progressive liver failure and death 6 to 12 months after onset of cholestasis, defined as bilirubin level of greater than 100 mumol/L. The development of cirrhosis occurs after the onset of jaundice, so early referral may also permit some children to be offered isolated bowel transplantation, which has better outcome than combined liver and bowel transplantation.

Cisapride reduces neonatal postoperative ileus: randomised placebo controlled trial.

AIM: To assess the efficacy of cisapride in reducing ileus persisting to the tenth postoperative day after neonatal abdominal surgery. METHODS: A prospective, randomised, double blind trial comparing rectal cisapride (1.4-2.3 mg/kg/day) with placebo over seven days was undertaken in 33 neonates. RESULTS: Seven of 12 (58%) patients receiving placebo and eight of 11 (73%) receiving cisapride achieved a first sustained feed during treatment. Of those receiving cisapride, the first sustained feed occurred at 2.3 days (SEM 0.6) compared with 4.7 days (SEM 0.8) with placebo. By the seventh day the mean daily net enteral balance was 69 (SEM 18) ml/kg in the cisapride subgroup and 17 (SEM 8) ml/kg for those receiving placebo. Stool was passed on 6.3 (SEM 0.4) treatment days in the cisapride subgroup compared with 4.1 (SEM 1.0) treatment days in the placebo subgroup. CONCLUSION: Cisapride is effective in neonates with a prolonged ileus after abdominal surgery.

Parenteral nutrition-related cholestasis in postsurgical neonates: multivariate analysis of risk factors.

The medical records of 74 neonates dependent on parenteral nutrition for at least 21 days after emergency abdominal surgery (performed between 1988 and 1992) were reviewed respectively. The role of enteral starvation, prematurity, composition and duration of parenteral nutrition, and sepsis in the evolution of parenteral nutrition-related cholestasis was evaluated by multiple regression analysis. The most important factors for cholestasis were low gestational age (median, 34 weeks), early exposure to parenteral nutrition, and sepsis. Episodes of sepsis were associated with a 30% increase in the bilirubin level. Enteral starvation and composition and the duration of parenteral nutrition solutions did not correlate significantly with the development of cholestasis. Prevention of sepsis should be the priority in minimising cholestasis in postsurgical neonates who are dependent on parenteral nutrition.

Duodenal atresia: a comparison of three modes of treatment.

To determine the most successful mode of treatment, 33 consecutive cases of duodenal atresia treated by duodenoduodenostomy and not associated with other gastro-intestinal anomalies were analysed retrospectively. These patients have been placed in a nonrandomised fashion into one of three groups: Group A: Duodenostomy (side to side) with gastrostomy and transanastomotic feeding tube (n = 12); Group B: Duodenoduodenostomy (diamond shape) with jejunostomy feeding tube (n = 12); Group C: Duodenoduodenostomy (diamond shape) only (n = 9). A nasogastric tube was used in all cases. There was no difference between the groups for gestational age, birthweight, and age at operation. The outcome measures used to compare these groups were the time taken to achieve full preanastomotic feeds and the duration of hospital stay. There was no difference in time taken to achieve full pre-anastomotic feeds between Group A and Group B. Patients in Group C took significantly less time to achieve full pre-anastomotic feeds than either of the other two groups (p < 0.05, Mann-Whitney U). The duration of hospital stay was also significantly shorter for patients in Group C (median = 12 days) than for patients in either Group A or B (median = 24, 20 days respectively) (p < 0.05, Mann-Whitney U).

Injury to the common iliac artery during suction rectal biopsy.

A case of injury to the common iliac artery during suction rectal biopsy is described. Factors that might influence the incidence of complications after suction rectal biopsy are considered. Suction rectal biopsy, although invaluable in establishing the diagnosis of Hirschsprung's disease, is a potentially dangerous technique and should not be delegated to an inexperienced operator. In neonates it is unwise to take biopsy specimens more than 4 cm from the anal verge. A posterior approach is likely to be safer than an anterior or lateral one.

Xanthogranulomatous pyelonephritis in childhood: pre-operative diagnosis is possible.

OBJECTIVE: To diagnose xanthogranulomatous pyelonephritis (XGP) in childhood pre-operatively. PATIENTS AND METHODS: Eleven cases of XGP presenting to Birmingham Children's Hospital over a 10-year-period are reported. Nine (82%) were diagnosed preoperatively on the clinical and radiological findings. RESULTS: The characteristic clinical findings were urinary tract infection (9 cases), palpable renal mass (8 cases) and anaemia (8 cases). The most common radiological findings were enlarged kidney (9 cases), renal calculi (7 cases) and non-functioning kidney (6 cases). Renal ultrasound typically demonstrated a central echoic area (6 cases) and multiple hypoechoic areas in the parenchyma (7 cases). A computed tomography (CT) scan was performed in three cases. This showed characteristic multiple, low attenuation, unenhanced areas within the parenchyma with extension of the inflammatory process into peri-nephric fat (two cases). Ten cases (91%) were of the diffuse type. There was extension into the peri-nephric fat in eight cases (73%). Three cases (27%) were associated with congenital urological abnormalities. Nephrectomy was performed in 10 cases and a partial nephrectomy in one. CONCLUSION: XGP is uncommon in childhood but should always be considered in the differential diagnosis of renal masses, especially in the presence of anaemia. Nephrectomy usually results in a permanent cure.

Is specialist centre delivery of gastroschisis beneficial?

This study aims to establish the usefulness of delivering neonates with gastroschisis in a regional obstetric and neonatal centre without the facility of on site surgery. A retrospective analysis was performed on the notes of 43 consecutive neonates with gastroschisis referred to Birmingham Children's Hospital over a 10 year period. Two groups were compared: those delivered at the regional obstetric centre (n = 9) and those delivered peripherally (n = 34). Both groups underwent postnatal transfer. There were no significant differences with regard to gestational age, birth weight, caesarean section rate, time to operation, and mortality. Primary closure rates were 89% for the regional centre group and 94% for the peripheral hospital group. Mean time to full enteral feeding was 24 days for the regional centre group and 23 days for those delivered peripherally. These data show that good results can be achieved with postnatal transfer. If on site surgery is not available, neonatal services are adequate peripherally, and the transfer distance is not too great, then delivery in a regional obstetric centre with subsequent postnatal transfer offers no advantage.

Impalpable testis: testicular vessel division in treatment.

Between 1984 and 1989, 110 boys with 130 impalpable testes were investigated and treated. One hundred and six boys underwent laparoscopy. Twenty had bilateral impalpable testes and 13 had a palpable, but maldescended, testis on the other side. Forty-nine testes were absent and one atrophic testis was found in the scrotum. Twelve orchidectomies were performed. One child had a microvascular transfer procedure. The remaining 67 testes were treated by orchidopexy: 28 single-stage and 39 two-stage procedures. One of the single-stage and 29 of the two-stage orchidopexies included division of the testicular vessels. Twenty-three testes examined 12 or more months after staged testicular vessel division revealed a good result in 15.

Intestinal malrotation: the role of small intestinal dysmotility in the cause of persistent symptoms.

To investigate the possibility that small intestinal dysmotility is a cause of long-standing and persistent symptoms in patients with malrotation, we retrospectively reviewed 94 patients operated on for intestinal malrotation. In 50 patients operated on during the neonatal period, associated abnormalities were common (24%) and all presented with obstructive symptoms; only three (6%) had continuing mild symptoms the postneonatal period (aged 1 month to 1 year), five of whom (22%) had other abnormalities. Recurrent vomiting was the most common presentation (11/23); only 5 patients (21%) had acute obstruction. Response to operation was good in 18 (78%) and two had persistant symptoms; both died. Twenty-one patients presented beyond infancy (aged greater than 1 year), only 19 of whom had symptoms of less than 2 months' duration. Eight (47%) of those with long-standing symptoms had no relief from operation. Small bowel motility was recorded manometrically in 4 patients with long-standing symptoms. The findings suggest that small intestinal dysmotility may be common in patients with malrotation and persistant symptoms.

Plasma chloride and alkalaemia in pyloric stenosis.

Fifty infants with infantile hypertrophic pyloric stenosis were studied prospectively to evaluate the reliability of plasma chloride estimation in the assessment of the acid-base status on admission and during correction of alkalaemia. Four cases were subsequently excluded because of a breach of the study protocol, leaving 46 cases in the study. Seventeen (37 per cent) were normoacidaemic on admission; 13 had plasma chloride concentrations of greater than or equal to 106 mmol/l and four had chloride concentrations of 100-105 mmol/l on admission. Twenty-nine (63 per cent) were alkalaemic on admission; six cases had chloride concentrations of 100-105 mmol/l and 23 cases had concentrations of less than 100 mmol/l. Of those 29 cases requiring correction of alkalaemia, normoacidaemia was achieved at a plasma chloride concentration of greater than or equal to 106 mmol/l in 21 cases (72 per cent) and at a concentration of 100-105 mmol/l in eight cases (28 per cent). Plasma chloride is a reliable parameter in the assessment and correction of alkalaemia in infantile hypertrophic pyloric stenosis provided that a concentration of at least 106 mmol/l is the goal.

The long-term sequelae of ileocolic anastomosis in childhood: a retrospective survey.

The long-term effects of ileocolic anastomosis in children are not known. We therefore carried out a survey of all such patients treated at our hospital between 1971 and 1985. Thirty-seven patients were identified and invited to attend for follow-up. Twenty-seven (73 per cent) were examined and included in the survey. Mean age at survey was 7.8 years (range 1.6-17.6 years) and mean duration since operation was 6.3 years. Necrotizing enterocolitis and intussusception were the commonest indications for operation. As judged by height, weight and skin fold thickness, all patients were well nourished. Seven (26 per cent) had loose stools, five had chronic folic acid deficiency and one was vitamin B12 deficient. We conclude that resection of the ileocaecal region is well tolerated in childhood and does not affect growth. Loose stools are directly related to the length of ileal resection.

Growth and feeding problems after repair of oesophageal atresia.

Of 230 families belonging to a support group for parents of children born with oesophageal atresia, 124 returned a detailed questionnaire on feeding history and growth. Being slow to feed, refusing meals, coughing or choking during eating, and vomiting at meal times were significantly more common than in 50 healthy control children. Anthropometric analysis indicated that almost one third of patients were growth retarded, although those with a primary oesophageal anastomosis were less likely to be stunted or wasted compared with children who had an oesophageal substitution preceded by an oesophagostomy. Feeding problems tended to resolve spontaneously but slowly, with half of all children still reporting some difficulties at 7 years of age. Parents were considerably worried by feed related symptoms and families benefited from mutual support. There is a need for additional help and advice to be provided both by hospital staff interested in nutrition and feeding disorders and those professionals involved with primary care.