Delayed primary anastomosis for esophageal atresia: 18 months' to 11 years' follow-up.

In 1977 we started treating babies with isolated esophageal atresia by delayed primary anastomosis and in 1981 reported our early experience in five cases treated between 1977 and 1979. Since 1979, 11 further consecutive cases have been managed by initial gastrostomy followed by delayed primary esophageal anastomosis. Their mean gestation was 35 weeks (range, 28 to 40 weeks) and mean birth weight was 2,040 g (range, 1,140 to 2,720 g). The esophageal gap between the two ends when assessed initially at fluoroscopy ranged from 2.2 to 4.5 cm (mean, 3.2 cm). Age at delayed primary anastomosis ranged from 6 to 20 weeks. Anastomotic leak occurred in three babies in the immediate postoperative period and all were successfully managed conservatively. Eight of the 11 patients developed anastomotic strictures; seven cases required 1 to 5 esophageal dilatations. One patient who did not respond to multiple esophageal dilatations required resection of an esophageal stricture. One patient died at 15 months of age of unrelated causes. The 10 surviving patients have been followed-up from 18 months to 11 years. At follow-up, seven patients were eating normally. Three patients had swallowing difficulties and all three were found to have esophageal strictures on barium swallow, two of them also had gross esophageal reflux and hiatus hernia. The height and weight in the 10 patients varied from 3rd centile to 75th centile. Delayed primary anastomosis is feasible in cases of isolated esophageal atresia and the patient's own esophagus is the best. A more aggressive approach should be applied to gastroesophageal reflux in these patients.

Management of pancreatic pseudocyst in childhood: an increasing role for percutaneous external drainage.

A total of four patients with a major pancreatic pseudocyst were treated by percutaneous pigtail catheter drainage. All patients recovered with complete resolution of the pseudocyst. There were no complications in relation to catheter insertion. Mean(s.d.) hospital stay was 56(15) days with a mean(s.d.) duration of catheter drainage and total parenteral nutrition of 21(14) and 35(9) days respectively. Percutaneous drainage is an appropriate method of first line therapy for non-resolving or enlarging pancreatic pseudocyst in children.

An alternative approach to pyogenic hepatic abscess in childhood.

Childhood pyogenic hepatic abscess is rare and remains a difficult diagnostic problem. Seven cases in six children were treated over a 13 year period. There was a recurrence in one patient 7 years after the first presentation. Four patients had identified predisposing factors, namely, chronic granulomatous disease, acute appendicitis, previous abdominal surgery and umbilical vein catheterization, while the other two were cryptogenic. There were no specific symptoms and signs but the combination of unexplained pyrexia, upper abdominal tenderness, hepatomegaly and leucocytosis should raise the suspicion of hepatic abscess. Four cases were diagnosed by ultrasound, one by radionuclide scan and the other two at laparotomy. Treatment in the earlier years was by transabdominal drainage. In the later part of the series, percutaneous catheter drainage using ultrasound guidance was achieved with satisfactory result. Ultrasonography can provide an early diagnosis and effective treatment can be achieved with percutaneous catheter drainage in combination with appropriate antibiotic therapy.

Atlantoaxial instability in Down's syndrome: clinical and radiological screening.

One hundred and thirty children with Down's syndrome were screened for the presence of atlantoaxial instability, using both clinical examination and radiographs of the cervical spine taken in flexion and hyperextension views. Seven children were found to have radiological evidence of atlantoaxial instability, with an atlanto-dens interval greater than 5.0 mm in one or all positions. Although a full clinical history was obtained from the attending parent and each child underwent a complete neurological examination, there were no factors detected which differentiated between those with radiological evidence of atlantoaxial instability and those with a normal atlantodens distance. It is recommended that children with Down's syndrome be screened twice, at the ages of 5-10 years and at 15 years.

Endoscopic correction of vesico-ureteric reflux by subureteric Teflon injection: follow-up ultrasound and voiding cystography.

The technique of subureteric Teflon injection for the correction of vesico-ureteric reflux was first described by Puri and O'Donnell in 1984. The Teflon granuloma that occurs after the injection anchors the submucosal ureter, thereby preventing reflux. This paper reviews the ultrasound and cystographic findings in 88 patients with 115 refluxing ureters followed-up for periods ranging from 3 months to 3 years. Reflux disappeared in 91 ureters (79.1%) and the degree of reflux improved in 22 ureters (19.1%). In this follow-up study, the size of the granuloma on ultrasound was assessed in relation to the number of injections, the total volume of Polytef paste used and the outcome of the procedure. A well circumscribed granuloma was seen at the orifices of 92 ureters. There was no ureteric granuloma evident in 23 ureters and Teflon plaques were found at the trigone in six patients. Complications were rare. One large granuloma caused severe obstruction requiring re-implantation. The size of the granuloma appears to depend on individual tissue reaction rather than on the other factors. The presence or the size of the granuloma bears no direct relationship to the success of the procedure.

Electro-spinal stimulation in children with adolescent and juvenile scoliosis.

A series of forty-one patients with adolescent or juvenile idiopathic scoliosis who were treated with electro-spinal stimulation is reported. Nineteen children were removed from the program because of curve progression or unacceptable deformity. Sixteen of these (39%) required surgery as a first alternative. Three were given a brace. Thirteen children have reached skeletal maturity without significant deterioration. Those who did well were in a lower risk category. The efficacy of this form of treatment has not been demonstrated in this series, and it is being discontinued.

Status of a group of spina bifida children not managed by early surgery.

At Our Lady's Hospital for Sick Children, Crumlin, the policy towards neonates with Spina Bifida is one of individualised care. This means that those children who, experience has shown, will not be long term survivors are not subjected to procedures that are distressing and traumatic to the infant and parents. They are given full nursery care. A small number of children treated on this basis thrive. The question arises whether or not they have been further handicapped by this approach. Since this is a problem which causes concern to both parents and clinicians, this study reviews the present state of these survivors. In the five year period 1979-1983, there was a total of 192 infants admitted to Our Lady's Hospital with open myelomeningocoele. 66 were treated with early surgical intervention. 75 died without any surgical procedure at a mean of 46 days. 51 children underwent late intervention, despite which 29 died. A group of 22 are alive, aged 3-7 years. The mean first admission of these 22 patients was 77 days and was uneventful. However, all 22 are severely handicapped; all are paraplegic, doubly incontinent and have some degree of mental handicap. All have hydrocephalus graded moderate to gross and 17 have been shunted. Amongst these 22 surviving patients, we have been unable to identify, in the initial period, specific events or developments which have clearly caused additional damage to the infant's intellectual or physical well-being.

Diastematomyelia: a critical review of the natural history and treatment.

Twenty-eight children whose diastematomyelia had been diagnosed at Our Lady's Hospital, Crumlin, were reviewed with respect to presenting symptoms, treatment, and outcome. Two children suffered significant neurologic damage during prophylactic removal of their bone spurs. Three children underwent spinal correction under traction with their bony spurs in situ without suffering any neurologic sequelae. The morbidity and mortality of surgical removal of bone spurs was considered and the traditional approach to this condition questioned.

Beak sign in duodenal duplication cyst.

Duodenal duplication cyst is an uncommon congenital anomaly of the alimentary tract and preoperative diagnosis is important in relation to the surgical approach. This paper describes a beak-like projection of the duodenum in contrast studies which enabled a confident preoperative diagnosis of duodenal duplication cyst in four infants.