CHARGE and esophageal atresia.

CHARGE association was diagnosed in 61 infants, 20 of whom died, mainly during the first 2 years of life. Esophageal atresia and/or tracheoesophageal fistula were present in 10 neonates. Axial skeletal anomalies occurred in 7 of the 10, but none had preaxial limb defects typical of the VATER association.

Morbidity and mortality in 46 patients with the VACTERL association.

During a 10-year period, 313 infants were treated at the Hospital for Sick Children for esophageal atresia, of whom 46 had features of the VACTERL association. These 46 patients had a mortality rate of 24%, most deaths being caused by cardiovascular abnormalities. Infants weighing less than 2,050 g had a mortality rate of 26%, double that of heavier infants.

The role of lymphatics in the pathogenesis of pneumatosis in experimental bowel ischemia.

Twelve spontaneously delivered, normally suckled, small-for-gestational-age (weighing 756 to 1,213 g) neonatal piglets were used to assess the role of the mesenteric vasculature in the pathogenesis of neonatal necrotizing enterocolitis (NEC) by producing intestinal ischemia. Component vessels (arteries, veins, lymphatics) of the mesenteric vascular arcades were variously occluded by ligation for 48 hours. Nine adjacent vessels of the same type or nine adjacent combinations of vessels were occluded in piglets 12 to 18 hours postpartum.

The ontogeny and distribution of neuropeptides in the human fetal and infant esophagus.

The innervation and neuropeptide expression of fetal and infant human esophagus were studied. Esophageal samples (n = 30) from 8 weeks' gestation to 28 months of age were immunostained using antisera to general and specific neuronal antigens, and the results were quantified using computer-assisted image analysis. Nerve protein (protein gene peptide 9.

Gastric transposition for esophageal substitution in children.

Fifty-four gastric transposition procedures have been carried out for esophageal substitution in the 10-year period 1981 through 1990. The indication for esophageal replacement was esophageal atresia in 36 (19 long-gap atresia with distal fistula and 17 isolated atresia), caustic stricture in 9, intractable peptic reflux stricture in 3, 2 achalasia and 1 each of prolonged foreign body impaction, diffuse leiomyoma, congenital esophageal stenosis, and congenital short esophagus. Eight patients had previously undergone an unsuccessful colonic replacement procedure.

Surgical treatment of hyperinsulinaemic hypoglycaemia in infancy and childhood.

Despite a greater awareness of hyperinsulinaemic hypoglycaemia, one in three patients has some degree of mental retardation by the time the diagnosis is made. The diagnosis is established by demonstrating high plasma insulin concentrations during an episode of hypoglycaemia. Twenty one hyperinsulinaemic infants and children were referred for surgical treatment after failing to respond to medical management.

Quantitative study of the development and maturation of human oesophageal innervation.

By 8 wk gestation, the human fetal oesophagus is identifiable as a hollow epithelium-lined tube with primitive nerve and muscle precursors present. From 8-16 wk gestation, the muscle layers and innervation mature until fetal swallowing commences at 16 wk. This study examines quantitatively the development and maturation of nerve fibres and cell bodies within the oesophagus using histochemistry.

Collateral blood flow in the distal ileum of neonatal piglets: a clue to the pathogenesis of necrotizing enterocolitis.

Normal blood flow was measured in two regions of the ileum (distal and proximal) of normal birth weight (NBW) and low birth weight (LBW) neonatal piglets. Compensatory collateral blood flow in response to occlusion of vessels in the mesenteric vascular arcades was also measured in distal and proximal ileum of NBW and LBW neonatal piglets. Under normal control conditions the blood flow in the distal ileum of NBW piglets is reduced (40% less than proximal) and in LBW animals this reduction is greater (55% less than proximal).

Necrotizing enterocolitis induced by local circulatory interruption in the ileum of neonatal piglets.

Occlusion of groups of vessels in the mesenteric vascular arcades of distal ileum for 48 h induced necrotizing enterocolitis lesions in low birth weight, spontaneously delivered, neonatal piglets. Lesion severity increased with numbers of adjacent groups of vessels occluded and with proximity to the ileocecal junction. A previously undescribed feature, "prepneumatosis," was confined to the lymphatic vessels of the submucosa and serosa.

Anastomotic leakage following surgery for esophageal atresia.

Of 199 neonates undergoing primary or delayed primary repair of esophageal atresia, 34 (17%) developed anastomotic leakage, 7 of which (3.5%) were major anastomotic disruptions. Infants with major leaks developed signs within 5 days and all required early reoperation, necessitating abandonment of the esophagus in 6.

In vitro demineralization of enamel by F-sensitive and F-resistant mutans streptococci in the presence of 0, 0.05, or 0.5 mmol/L NaF.

Lactate production and accompanying enamel demineralization by fluoride-sensitive and fluoride-resistant mutans streptococci were studied in an in vitro demineralization model in the presence of 0, 0.05, or 0.5 mmol/L NaF.

Primary anastomosis in apple-peel bowel syndrome.

During a 15-year period, 12 infants with apple-peel bowel were treated; the first 4 by initial enterostomy with delayed anastomosis and the remaining 8 by primary anastomosis. All four infants in the enterostomy group required parenteral nutrition, compared with only six of the eight who had a primary anastomosis. Seven of the eight infants treated by primary anastomosis were in hospital for less than 50 days, (however, the remaining child was an in-patient for over 2 years, but did suffer from short-bowel syndrome with only 17 cm of small intestine).

Esophageal atresia with obstructed tracheoesophageal fistula and gasless abdomen.

Five neonates with esophageal atresia and absence of gas on abdominal radiographs were each found to have an apparently obliterated distal tracheoesophageal fistula without a long gap between the esophageal ends. Four were treated by primary repair of the esophagus, and one underwent esophageal replacement, although it is conceivable that primary repair could have been achieved if the correct diagnosis had been made at presentation. This variant of tracheoesophageal fistula can be expected in at least 14% of patients presenting with a gasless abdomen.

Interactions of sanguinarine and zinc on oral streptococci and Actinomyces species.

Sanguinaria extract, which contains benzophenanthridine alkaloids, has been used as a folk medicine for many years. Minimum inhibitory and minimum bactericidal concentrations (MIC and MBC values) for sanguinarine were determined for common and etiologically important plaque bacteria. Because the efficacy of sanguinarine is believed to be enhanced by zinc, isobolograms were assessed to determine their mode(s) of interaction.

Xiphoomphaloischiopagus tripus conjoined twins: successful separation following abdominal wall expansion.

A case of xiphoomphaloischiopagus tripus conjoined twins is reported. Particular emphasis was paid to the large abdominal wall defect that would be produced by separation, and the complications resulting from the use of intraperitoneal Silastic tissue expanders are described. As a result of adequate tissue expansion and the use of a vascularized pedicle graft from the fused limb, primary abdominal wall and pelvic closure was possible without complications of wound healing.

Tracheo-oesophageal fistula and pre-operative mechanical ventilation.

Twelve patients with tracheo-oesophageal fistula (TOF) and restrictive lung disease necessitating pre-operative ventilation are reported. Eight patients had respiratory distress syndrome, four had aspiration pneumonia, and 11 had associated oesophageal atresia. Two patients in whom a preliminary gastrostomy was performed died.

The functional sequelae of sacrococcygeal teratoma.

Twenty-seven patients with sacrococcygeal teratoma were followed for a mean period of 5 years (range, 2 to 12 years). Eleven (41%) had some form of functional impairment. Fecal and/or urinary incontinence was present in nine children and two had weakness of the lower limbs secondary to sciatic nerve palsy.

Anastomotic stricture following repair of esophageal atresia.

Anastomotic strictures developed in 74 (37.2%) of 199 patients undergoing primary or delayed primary repair of esophageal atresia with or without tracheoesophageal fistula. Significant predisposing factors included the use of braided silk sutures (relative risk 1.

Developmental progress of newborns undergoing neonatal surgery.

The development of 30 full-term infants undergoing surgery soon after birth was compared with a matched group of 29 healthy newborn infants in a prospective longitudinal study. At 1 year of age the infants who underwent surgery were performing within the normal range, but significantly less well than the controls in almost all areas of development. Of the various neonatal and perinatal factors studied, the length of hospital admission was the one most strongly associated with developmental progress at 1 year of age.

Recurrent tracheo-oesophageal fistula: experience with 24 patients.

Over an 11 year period, 275 infants with oesophageal atresia and tracheo-oesophageal fistula were treated. Of these, 22 (8%) developed a recurrent fistula. An additional two patients with an established recurrent fistula were referred for secondary surgery.

Primary malignant liver tumors in childhood: assessment of resectability with high-field MR and comparison with CT.

Nine children (mean age 20 months), with proven primary malignant hepatic tumors have been examined prospectively by high-field magnetic resonance (MR) imaging to assess tumor resectability. All patients had comparative ultrasonography (US), 8 patients had X-Ray computed tomography (CT), and surgical correlation was available in 8 patients. The hepatic and portal veins and the inferior vena cava were visualized in all patients on MR and in 4 of 8 patients on CT.