Acute necrotizing enterocolitis in infancy: a review of 64 cases.

Sixty-four cases of necrotizing enterocolitis are reviewed. The diagnosis was based on tissue examination in 57 and on the clinical syndrome, including pneumatosis, in 7. Three factors are important in the development of the disease: injury to the intestinal mucosa, bacteria, and feedings. The indications for surgical intervention are pneumoperitoneum, signs of peritonitis, and intestinal obstruction. The importance of stress in the etiology of the disease is confirmed by the high incidence of perinatal complications, particularly hypoxia. The mortality was high, but results are improving with the institution of early aggressive treatment.

Major hepatic resections for neoplasia in children.

In a consecutive series from 1968 to 1978, 11 hepatic resections encompassing at least two hepatic segments were carried out for neoplasia in pediatric patients varying in age from 7 days to 14 years. There were no operative deaths. These resections consisted of four right lobectomies, three extended right lobectomies, one right lobectomy with right nephrectomy and inferior vena caval resection, two left lobectomies, and one left extended lobectomy. Diagnoses were hepatoblastoma in six patients, Wilms' tumor invading the liver in two, hemangioendothelioma in two, and malignant mesenchymoma in one. Selective angiography and technetium Tc 99m sulfur colloid scintigraphy were important preoperative aids. Complications were infrequent and there were no major infections or biliary fistulas. There was one death eight months postoperatively due to recurrent hepatoblastoma. Vigorous hepatic regeneration occurred in all instances. Major hepatic resections are well tolerated in children and allow good subsequent development.

Birth trauma.

Although significant birth injury accounts for few neonatal deaths and stillborns in the United States, it still occasionally and unavoidably occurs. This article reviews soft tissue, peripheral nerve, intrathoracic, and intra-abdominal injury.

Inflammatory esophagogastric polyps: resolution following antireflux surgery.

Inflammatory gastroesophageal polyps are a rare manifestation of reflux esophagitis. In this report, an inflammatory gastroesophageal polyp resolved following antireflux surgery. The literature of inflammatory polyps is reviewed.

Diagnosis and management of gastrointestinal perforations in childhood dermatomyositis with particular reference to perforations of the duodenum.

Four children with dermatomyositis were recently seen with gastrointestinal perforations. The sites of perforation in the four cases were: (1) the duodenum, esophagus, and colon; (2) the duodenum; (3) the distal stomach; and (4) the traverse colon. The gastric and transverse colon perforations were intraperitoneal and easily diagnosed. The gastric perforation was treated successfully by partial gastrectomy. The patient with the colon perforation underwent exteriorization; death occurred from cerebral complications possibly related to vasculitis. Both duodenal perforations were posterior in the distal descending portion. Enzymatic dissection into the right lower quadrant produced confusing clinical and radiographic signs and extensive retroperitoneal necrosis. Successful treatment was obtained by partial gastrectomy, sump drainage of the perforation, and parenteral nutrition. Gastrointestinal perforation is a well-recognized complication of vasculitis in childhood dermatomyositis. In particular, perforations of the distal duodenum, as reported by others, are associated with delay in diagnosis and high mortality.

The suture fistula technique in the repair of selected cases of esophageal atresia.

Correction of esophageal atresia with tracheoesophageal fistula can present a difficult problem when distance and tension between upper and lower esophageal segments are too great for primary anastomosis. Alternatives have included staged procedures with upper pouch elongation or interpositions. In 1974, Shafer and David described an operation in which the lower segment was divided, closed, and then merely apposed to the unopened upper pouch. A central "necrosing suture" was placed through the lumina of both segments. Spontaneous fistulization between the two segments occurred postoperatively, and the resultant channel was then progressively dilated with woven silk bougies. This technique has been employed at Babies Hospital in five children. The results were excellent in four. One child failed to fistulize, and following a complicated postoperative course, died at home from accidental dislodgement of his tracheostomy tube. All five children required antireflux procedures but ultimately did well with regard to esophageal function.

Selective management of iatrogenic esophageal perforation in the newborn.

Since 1974 nine neonates have been treated for iatrogenic esophageal perforation. They ranged in weight from 480 to 3900 g. Four of them had been resuscitated for meconium aspiration, four were being treated for respiratory distress syndrome (RDS), and one had received only routine postpartum suctioning and gastric aspiration. In five infants, esophageal perforation was suspected following traumatic intubation of difficulty in the passage of a catheter or tube. Three neonates passed formula from chest tubes placed for pneumothorax and one child presented with persistent pneumothorax. Esophageal perforation was documented in each case by direct visualization and/or radiographic studies. Three infants were treated with antibiotics and placement of a silastic nasogastric feeding tube. An additional two infants were treated with a silastic tube, antibiotics and chest tube drainage. Four children underwent operation: gastrostomy and drainage (2), gastrostomy and closure of perforation (1), gastrostomy only (1). There were no complications of deaths. Management of iatrogenic perforation of the esophagus depends upon the extent and location of the injury. A nonoperative approach can be successful in those infants with limited injury of short duration. Operation is required in cases with extensive extravasation or delay in diagnosis.

Plain film detection of right aortic arch in infants with esophageal atresia and tracheoesophageal fistula.

The aortic arch can be clearly demonstrated in neonatal chest radiographs by use of a high kilovoltage-filtered film with air-gap magnification. This allows identification of the presence of right aortic arch in patients with esophageal atresia and tracheoesophageal fistula; awareness of this anomaly may influence choice of surgical approach.

Hypothyroidism caused by topical povidone-iodine in a newborn with omphalocele.

A case of physiologic hypothyroidism caused by the topical application of povidone-iodine (PVPI) in a newborn with an omphalocele is presented. The literature on systemic absorption and effects of PVPI is reviewed. A management strategy is offered.

Neuroblastoma with intraspinal (dumbbell) extension.

Twenty-three patients with neurogenic tumors having extradural extension (dumbbell tumors) were treated over a 30-year period. Three had ganglioneuroma, 6 had ganglioneuroblastoma, and 14 had neuroblastoma. Nineteen patients with malignant tumors had symptoms of spinal-cord compression; three patients with ganglioneuroma and one with a malignant tumor had no neurologic symptoms. Delays in diagnosis were frequent ranging from 6 weeks to 3 years in 10 patients. Sixteen patients had abnormal x-rays of the spine. All patients with malignant tumors had positive myelograms. Three patients with ganglioneuroma and 13 with malignant tumors are alive and free of disease. Seven patients with malignant tumors died: five with stage IV and 1 with stage III tumors from disease, and 1 with stage II tumor during laminectomy. Age and stage were important prognostic factors; location was not. Treatment consisted of excision and radiation or radiation only. Patients receiving lower-dose (less than 2000 rad) radiation did as well as those receiving much larger doses. Morbidity in patients with malignant tumors was high; 4 have spinal deformity (3 severe) and 8 of 13 surviving patients have residual neurologic deficits. Earlier diagnosis, a surgical approach which does not introduce morbidity, and low-dose radiation in younger patients are important factors in reducing morbidity.

Complications at menarche of urogenital sinus with associated anorectal malformations.

Persistent urogenital sinus is frequently associated with a wide spectrum of complex anatomic abnormalities involving the urinary, genital, and gastrointestinal tracts. Failure to accurately define these abnormalities can result in serious complications. One such group of complications occurs relatively late in the clinical course of these children and has received little attention. These are the complications related to menarche. This report reviews the anatomy, complications, and management in five such patients. In this group the onset of menses was associated with hydrosalpinx, pyosalpinx, hematocolpos, hematometrocolpos tubo-ovarian abscess, ruptured ovarian endometrioma, endometriosis, and peritonitis. Anatomic abnormalities included double and septate vagina, vaginal atresia and stenosis, uterus didelphys, and uterus bicornis unicollis, all predisposing to inadequate menstrual flow. Each of these children required surgical intervention. These cases stress the need for an awareness of not only the urinary but the vaginal and uterine abnormalities. Careful early definition of the anatomy and long-term follow-up of children with urogenital sinus malformation is important in order to avoid potential future complications.

Epidermoid cysts of the liver.

To the four recorded cases of epidermoid cysts of the liver are added two cases recently seen in children. One was in a 4-yr-old girl and associated with biliary cirrhosis. The other was in a 5-mo-old boy. The former was treated by roux-en-y cystjejunostomy, the latter by enucleation. The origin of these cysts is unknown. One theory suggests derivation from accessory foregut buds. Because of their malignant potential, treatment should be by excision. Where this is impossible, roux-en-y cystjejunostomy offers satisfactory palliation.

Calcific thrombi of the inferior vena cava in infants and children.

Calcified caval thrombus should be considered in any infant or child where calcifications are noted in the high right retroperitoneal area on plain x-rays of the abdomen. Although typically bullet-shaped in configuration, the calcium distribution in the neonate may be atypical or incompletely developed, suggesting neuroblastoma. Definitive diagnosis can be made by inferior vena cavagram. As no deaths or complications have been attributed to the lesion in the cases thus far reported, no specific treatment is recommended.

Down's syndrome and the gastrointestinal tract.

Gastrointestinal (GI) abnormalities are frequent in patients with Down's syndrome. In a 12-year retrospective review, we identified 187 patients with Down's syndrome admitted to the Columbia-Presbyterian Medical Center. Twenty-seven had major GI disorders, the most common being duodenal stenosis (DS, nine), gastroesophageal reflux (GER, five), imperforate anus (five), and Hirschsprung's disease (four). The mortality for the whole group was 11% (20 patients). The mortality in the small group of patients with duodenal stenosis was particularly high (five out of nine, or 56%). Associated congenital heart disease, especially endocardial cushion defects, and the frequent occurrence of pneumonia contributed to this high mortality rate.