1/238. Functional intestinal obstruction due to deficiency of argyrophil neurones in the myenteric plexus. Familial syndrome presenting with short small bowel, malrotation, and pyloric hypertrophy. In 3 infants functional intestinal obstruction, associated with a short small intestine, malrotation, and pyloric hypertrophy, was shown to be due to failure of development of the argyrophil myenteric plexus, with the absence of ongoing peristalsis. 4 infants with similar clinical features have been described previously, and there is evidence for an autosomal recessive mode of inheritance of this syndrome. ( info) |
2/238. Simultaneous pyloric and colonic obstruction associated with hiatus hernia in a weightlifter: a case report. Hiatus hernia is usually attributed to conditions that cause a chronic increase in intra-abdominal pressure such as multiple pregnancies and obesity. A 30-year-old man, a weightlifter, had a massive hiatus hernia causing both high and low gastrointestinal obstruction but no involvement of the gastroesophageal junction or fundus. The onset of the obstruction is attributed to an extreme increase in intra-abdominal pressure caused by the action of lifting weights. ( info) |
3/238. Pyloric channel stricture secondary to high-dose ibuprofen therapy in a patient with cystic fibrosis. OBJECTIVE: To describe a case of pyloric channel stricture secondary to high-dose ibuprofen therapy in a pediatric patient with cystic fibrosis. CASE SUMMARY: A 12-year-old white girl started taking high-dose ibuprofen to treat the pulmonary manifestations of cystic fibrosis. The peak plasma concentration at dose initiation was within the accepted therapeutic range. Approximately one month later, the patient developed emesis and intolerance of solid foods, which persisted for several months and resulted in a weight loss of seven kilograms. The patient was referred to a pediatric gastroenterologist, who performed an upper endoscopy and subsequently diagnosed a pyloric channel stricture. The patient's pyloric channel was successfully dilated with two balloons. It is felt that the pyloric stricture developed from healing antral/pyloric channel ulcers. ibuprofen was discontinued and omeprazole therapy was begun. Over the course of the following year, the patient was asymptomatic. Follow-up upper gastrointestinal barium swallows were normal. DISCUSSION: When used for analgesia and fever in the pediatric population, ibuprofen has been shown to be a relatively safe drug. While it is known that ibuprofen may cause gastrointestinal adverse effects, the pediatric population is at lower risk; however, large doses of ibuprofen increase the risk of gastrointestinal adverse effects. The use of large doses of ibuprofen in the treatment of cystic fibrosis is a relatively new therapy. Limited data thus far in cystic fibrosis patients do not suggest increased risk of gastrointestinal complications. CONCLUSIONS: Limited data to date indicate that ibuprofen, when used in large doses to treat the pulmonary manifestations of cystic fibrosis, is relatively safe. However, because of the potential risks to the gastrointestinal tract of high-dose ibuprofen therapy, clinicians should be aware of its possible complications. ( info) |
4/238. Recurrent vomiting after successful pyloromyotomy. Persistent vomiting after pyloromyotomy is rare. We report the case of a boy with recurrent vomiting after a successful pyloromyotomy, who required repyloromyotomy. ( info) |
5/238. jaundice with hypertrophic pyloric stenosis as an early manifestation of Gilbert syndrome. jaundice associated with hypertrophic pyloric stenosis was recognised in three patients; previous reports have suggested that this is a possible early manifestation of Gilbert syndrome. Most patients with Gilbert syndrome are homozygous for a (TA)(7)TAA polymorphism in the gene promoter coding for bilirubin glucuronosyltransferase. Two of the reported patients were homozygous for the (TA)(7)TAA polymorphism whereas the third was heterozygous for the same polymorphism. Furthermore, no other factors contributing to jaundice in the three patients were found. These results suggest that jaundice associated with hypertrophic pyloric stenosis is due to molecular defects within the gene promoter. ( info) |
6/238. The development of hypertrophic pyloric stenosis in a patient with prostaglandin-induced foveolar hyperplasia. BACKGROUND: Hypertrophic pyloric stenosis (HPS) has been described in association with several obstructive antropyloric lesions including idiopathic foveolar hyperplasia (gastric mucosal hypertrophy), feeding tubes, eosinophilic gastroenteritis, and hypertrophic antral polyps. Non obstructive antral webs have also been described with HPS. PATIENT AND methods: We present a case of gastric-outlet obstruction in association with HPS, namely, prostaglandin-induced foveolar hyperplasia. This entity has been previously described, but rarely in association with HPS. We report a female infant requiring prostaglandin therapy for pulmonary atresia who developed dose-related prostaglandin-induced foveolar hyperplasia and symptoms of progressive non-bilious vomiting. RESULTS: Initially, ultrasonography demonstrated evidence of antral mucosal hypertrophy as the cause for gastric-outlet obstruction. The patient subsequently developed progressive thickening of the antropyloric muscle, resulting in sonographic appearances of hypertrophic pyloric stenosis. Pyloromyotomy was eventually required for treatment of HPS. CONCLUSION: A common denominator of most of the above-described entities is thickening and/or hypertrophy of the antral mucosa. We suggest that the antropyloric musculature may hypertrophy in an effort to overcome the gastric-outlet obstruction caused by the adjacent thickened antral mucosa. In other words, these entities may represent examples of "secondary" hypertrophic pyloric stenosis. ( info) |
7/238. Hypertrophic pyloric stenosis in infants following pertussis prophylaxis with erythromycin--Knoxville, tennessee, 1999. In February 1999, pertussis was diagnosed in six neonates born at hospital A in Knoxville, tennessee. Because a health-care worker at hospital A was most likely the source of exposure, the local health department recommended on February 25, 1999, that erythromycin be prescribed as postexposure prophylaxis for the approximately 200 infants born at hospital A during February 1-24, 1999. In March 1999, local pediatric surgeons noticed an increased number of cases of infantile hypertrophic pyloric stenosis (IHPS) in the area, with seven cases occurring during a 2-week period. All seven IHPS cases were in infants born in hospital A during February who were given erythromycin orally for prophylaxis following possible exposure to pertussis, although none had pertussis diagnosed. The tennessee Department of health and CDC investigated the cluster of IHPS cases and its possible association with use of erythromycin. This report summarizes the results of the investigation, which suggest a causal role of erythromycin in this cluster of IHPS cases. ( info) |
8/238. A rare coexistence of two gastric outlet obstructive lesions: infantile hypertrophic pyloric stenosis and organoaxial gastric volvulus. Infantile pyloric stenosis is one of the most common conditions requiring surgery during the first few weeks of life. The association of infantile pyloric stenosis with gastric volvulus in an extremely uncommon occurrence. A 10-month-old male infant operated for infantile pyloric stenosis at two months of age is presented. His current problem was recurrent pulmonary infections and he was diagnosed to have organoaxial gastric volvulus and gastroesophageal reflux. The common features of presentation, radiological findings, surgical procedures and possible mechanisms of gastric volvulus associated with infantile pyloric stenosis are discussed. ( info) |
9/238. Gastric antral stricture in a patient with chronic granulomatous disease. Chronic granulomatous disease (CGD) is a rare disorder of phagocytic cell oxidative metabolism. patients have recurrent infections with catalase-positive organisms and granulomatous lesions throughout the body. Gastric antrum can be an occult site of involvement. We describe a four-year old boy with chronic granulomatous disease who was admitted with the complaints of persistent vomiting and weight loss. Gastric antral narrowing was diagnosed according to radiological findings. Treatment with steroid and antibiotics yielded a good clinical response in 15 days with a relief of the obstruction. This case report emphasizes the beneficial effect of this form of therapy in preventing life-threatening obstruction of vital organs in CGD. ( info) |
| Although infantile hypertrophic pyloric stenosis following esophageal atresia repair is known, infantile hypertrophic pyloric stenosis following H-type tracheoesophageal fistula has not been encountered previously. A case of H-type tracheoesophageal fistula and infantile hypertrophic pyloric stenosis is presented. The patient, operated on for H-type fistula, a rare congenital anomaly of the esophagus, on the tenth day of life was readmitted 19 days later because of continuous vomiting after every feeding. The clinical findings and physical and radiological examinations revealed infantile hypertrophic pyloric stenosis which required surgical treatment. It is suggested that the association of H-type tracheoesophageal fistula with infantile hypertrophic pyloric stenosis is coincidental, given the estimated incidence of one in every 84,375,000 males and 337,500,000 females. ( info) |