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Purpose: The purpose of this study was to determine which imaging study, upper gastrointestinal series (UGI) or abdominal ultrasonography (US), is more cost-effective in diagnosing infantile hypertrophic pyloric stenosis (IHPS) using a decision analysis model., Methods: Probabilities were calculated from a review of the records of all infants less than 6 months of age referred for UGI or US to rule out IHPS over a 3-year period from January 1992 to December 1995. Cost-effectiveness was determined from hospital charges for each imaging study and its possible outcomes., Results: The positive predictive value of UGI was 1.0 and US was 0.98 in the 246 infants evaluated for possible IHPS. In patients who had an initially normal study finding (UGI or US), 25% of patients undergoing US first required a second study for persistent symptoms, whereas only 6% of patients who had a negative initial UGI finding required a second study., Conclusions: Cost analysis found UGI to be more cost-effective than US because fewer secondary studies were required. UGI provides information regarding other pathological conditions as compared with US.

Peutz-Jeghers syndrome is characterized by hamartomatous polyposis of the small and large bowel and mucocutaneous pigmentation. The authors describe a 9-year-old girl with small bowel obstruction related to duodenal intussusception caused by polyposis in the fourth portion of the duodenum. Operative reduction of the intussusception and excision of the polyps were performed, at which time the pancreas appeared to have mild pancreatitis. A liver biopsy specimen showed mild portal fibrosis and ductal proliferation. The patient did well postoperatively, but later presented with symptoms consistent with biliary obstruction. Percutaneous transhepatic cholangiography showed pancreatic and biliary duct dilatation as well as obstruction of the common bile duct, which extended into the left upper quadrant. Exploration showed ampullary obstruction several centimeters proximal to the line of resection. Sphincteroplasty was performed. The postoperative course was uncomplicated. The authors conclude that Peutz-Jeghers syndrome with polyps in the duodenum can markedly distort duodenal and ductal anatomy and can lead to ampullary obstruction.

Recent advances in biochemical and imaging studies have improved the diagnostic accuracy of Cushing's syndrome. To better define roles for these studies in children, the authors reviewed their experience with this rare group of patients. Fifteen children, aged 11 weeks to 17 years, were treated for noniatrogenic Cushing's syndrome over a 33-year period. All children presented with signs of hypercortisolism. Nineteen different diagnostic tests were used, reflecting changes in how these patients are evaluated. Pathological diagnoses were adrenal cortical carcinoma (3), primary adrenocortical nodular dysplasia (PAND) (2), and pituitary adenoma (10). Children with adrenal cortical carcinoma presented with an adrenal mass and at a younger age (mean, 22.3 months). Key diagnostic features of patients with PAND were a low plasma adrenocorticotrophin hormone (ACTH) and no suppression with high-dose dexamethasone. Children with a pituitary cause of Cushing's syndrome presented at an older age (mean, 15.7 years) and were diagnosed using a combination of high-dose dexamethasone testing, simultaneous inferior petrosal sinus sampling, and/or ovine corticotrophin-releasing hormone stimulation test. A strategy for the diagnosis of Cushing's syndrome in children is presented.

Four children were treated at the Oregon Health Sciences University for perforated peptic ulcer disease over the last 15 years. All children were being treated for other severe underlying diseases at the time of perforation. All of the children were receiving corticosteroids; one was receiving ulcer prophylaxis. Presenting symptoms were those of a perforated viscus, and pneumoperitoneum was present in all patients. Simple closure and omental patching of the ulcer resulted in no operative mortality or morbidity. None of the ulcers have recurred. In reviewing the literature, corticosteroids were associated with perforated peptic ulcer disease in 17.8% of children with perforated peptic ulcer. Despite controversy regarding the role of corticosteroids in the causation of peptic ulcer, we recommend prophylaxis for all children receiving corticosteroids.

An infant who presented with signs of duodenal atresia had proximal duodenal obstruction by upper gastrointestinal barium study, but had a small amount of air in the distal bowel. Exploration showed an annular pancreas and microperforation of a duodenal diaphragm. Prior to concluding that pancreaticobiliary duct anomalies are the path of air into the distal bowel in patients with duodenal atresia, microperforation of a duodenal diaphragm must be excluded.

Two newborn infants with neurenteric cysts have been evaluated with recently developed imaging techniques. Magnetic resonance imaging or computed tomography can provide the evaluations needed to facilitate removal of the entire cyst at one operation. Appreciation of the extent of the problem allows for expeditious evaluation and early definitive surgical intervention.

A rare type of congenital diaphragmatic hernia occurs in which there is a large opening in the anterior diaphragm between the pericardial and peritoneal cavities without a sac. This hernia is invariably associated with defects in the anterior abdominal wall and with sternal defects. More commonly, it is associated with cardiac anomalies as in the Pentalogy of Cantrell. The etiology of this hernia is undoubtedly different from the more common hernia of Morgangni, which has a sac and few associated anomalies. The etiology may be failure of fusion of the pars sternis area of the septum transversum. Only five other cases have been described.

Myasthenia gravis is considered to be an autoimmune disorder of neuromuscular transmission. Thymectomy is an effective mode of treatment which produces either remission or stabilization of symptoms especially in young female patients with short duration of disease. The present study, records the experience in ten pediatric patients with early thymectomy done via the transcervical approach. There were no operative deaths or serious complications. Follow-up for a mean of 37 months has shown one to be in complete remission requiring no medication and eight to have improved or stable symptoms. Thymectomy via the transcervical approach is an effective mode of treatment for patients with myasthenia gravis. Since this approach involves a small dissection and avoids sternotomy, the transcervical approach results in a very short postoperative recovery period. It is ideal for those patients not in respiratory failure. It can also be safely done in patients requiring intubation for acute respiratory failure preoperatively.

Hypoxia and hypovolemia result in similar ultrastructural changes. These changes are characterized by aggregation of platelets and leukocytes in the vascular space, focal endothelial cell disruption, and appearance of increased amounts of perivascular edema, and loss of ultrastructural organization of the mucosal cell. These changes appear to relate to variations in blood flow to the mucosa of different areas of the bowel and to follow the distribution observed for necrotizing enterocolitis in the human infant.

Six children have undergone lung biopsy for evaluation of fine structure. Four children demonstrated increased DAVO2 preoperatively and had marked ultrastructural changes in their lungs. Such changes appear to result from embolization of microthrombi or microaggregates to the lung from under-oxygenated tissue.

Previous studies have suggested that delayed gastric emptying occurs in severely mentally retarded patients with gastroesophageal reflux. Based on this data, pyloroplasty was employed in such patients. A retrospective analysis of 99 consecutive patients who underwent primary fundoplication for GER was performed. Gastric emptying, as measured by successful removal of the nasogastric tube or elevation of the gastrostomy tube, was studied. Children with neurologic disorders had no clinically significant difference in gastric emptying after fundoplication (3.31 days) when compared with neurologically normal patients (2.21 days). When added to Nissen fundoplication, pyloroplasty did not hasten the return of gastrointestinal function in the severely impaired patients (4.91 days). A prospective study employing gastric isotope bolus feedings before and after Nissen fundoplication will determine if pyloroplasty improves gastric emptying when used in conjunction with Nissen fundoplication for patients with severe neurologic disorders.

The present study demonstrates that a 2 hr period of sustained hypoxia results in a focally destructive process, a process which resolves by 5 days. That the changes described in earlier work are not overwhelming to the animal suggest that an early, focal, and reversible stage of the development of acute enterocolitis in the puppy can be described with the aid of electron microsocpy.