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.

Importance of multiple episodes of hypoxia or cold stress on the development of enterocolitis in an animal model.

Necrotizing enterocolitis, a highly lethal disease in the newborn infant characterized by ischemic necrosis of the gastrointestinal tract frequently leading to perforation, is seen primarily in low birth weight infants who have undergone stress, such as hypoxia. In an animal model it was demonstrated that cold stress was as effective as hypoxia in producing the disease in formula-fed newborn rats. Breast milk was completely protective in both cold- and hypoxic-stressed animals. Presumably cold stress produces the same selective circulatory ischemia as does hypoxia. The experiment further supports the concept that any insult or stress which decreases mesenteric blood flow may initiate the changes leading to necrotizing enterocolitis. It was shown also that the incidence of the disease in formula-fed rats was related directly to the number of episodes of either cold or hypoxic stress. These results suggest that a critical amount of ischemia is necessary to initiate these changes and may help to explain the fact that not all infants exposed to hypoxia or cold stress developthe disease.

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.

Urologic problems associated with imperforate anus.

A total of 120 children with both high, intermediate, and low types of imperforate anus were evaluated urologically. Genitourinary anomalies were associated with all types of imperforate anus but were found more often in children with high rectal deformities. Because the over-all incidence of genitourinary anomalies was high, early and complete urologic evaluation is necessary to achieve the best functional result.

Ultrasonic evaluation of the fetal human heart.

The fetal echocardiogram has demonstrated its utility for visualizing the fetal human heart during the second and third trimesters of pregnancy. The potential utility of the technique for establishing accurate anatomic diagnoses of major congenital cardiac malformations has been established. These data have been utilized to counsel prospective parents and formulate management plans for the remainder of pregnancy and the intrapartum and immediate postpartum periods. Diagnoses have been established as early as the 16th to 18th week of gestation, raising in some cases the moral and ethical question of termination of pregnancy.

Iliofemoral thrombophlebitis associated with central nervous system pathology.

It is very uncommon for acute thrombophlebitis to develop in children without an identifiable predisposing cause. During a ten year period at a children's hospital, central nervous system disease was observed in four of eight children with iliofemoral thrombophlebitis. This association suggests that a thorough neurologic evaluation should be carried out in any child with acute thrombophlebitis.

Fetal echocardiography: assessment of cardiovascular anatomy and function.

Echocardiography is used to assist the prenatal diagnosis group in understanding and identifying normal and abnormal structure, function, and rhythm in the fetal heart, normal variations, and change during development. Detailed segmental analysis of the heart's complex structure can be performed by experienced observers and the information used to aid in management decisions and to better understand the complex relationships of fetal cardiovascular anatomy and function, and fetal outcome. When used prudently, the technology appears to be safe to fetus and mother, but all who undergo examination will want to know of potential benefits and risks.

Angiographic diagnosis and treatment of bleeding by selective embolization following pelvic fracture in children.

Angiography has been used successfully in adults for evaluation following major trauma and to subsequently control hemorrhage associated with pelvic fracture by selective embolization. This report illustrates that the technique is applicable in the evaluation and treatment of children with similar injuries.

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.

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.

Effects of total parenteral nutrition and semi-starvation on the liver of beagle puppies.

The effects on hepatic growth of fat-free TPN and semistarvation have been studied in newborn beagle puppies. Our data indicate that liver growth during TPN is characterized by normal deposition of protein and minerals, with increased glycogen and decreased lipid content. While the lipid pattern of TPN livers is normal, the phospholipid fraction contains an abnormal fatty acid pattern, consistent with the development of essential fatty acid deficiency. The exact physiologic significance of this is unknown, but it is tempting to speculate that changes in integrity of the hepatic cell membrane as a result of this abnormal fatty acid pattern might account for some of the clinical abnormalities of hepatic function during TPN.

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.

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.

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.