Rapunzel syndrome: a rare form of trichobezoar in the stomach with some extension into the small intestine.

Trichobezoars are gastric foreign bodies rarely found in children. They are mainly composed of swallowed hair. Rapunzel syndrome is a very rare form of trichobezoar characterized by extension of the ingested hair from the stomach into the small intestine or even beyond. Clinical presentation can range from an asymptomatic abdominal mass to severe symptoms: gastric outlet obstruction, perforation of the stomach, and acute pancreatitis. Trichobezoars are usually treated with surgical removal. Here we present the case of an 8-year-old girl in whom a firm non-tender mass in the epigastric region was palpated during a regular outpatient clinic visit. The girl reported some slight abdominal discomfort and early satiety during meals. Subsequently, focal alopecia was detected. A trichobezoar in the stomach with some extension into the duodenum was diagnosed by ultrasound, and later confirmed by a barium swallow study and CT scan. The trichobezoar was successfully surgically removed.

Inflammatory myofibroblastic tumor of the pancreatic head - a case report of a 6 months old child and review of the literature.

BACKGROUND: Inflammatory myofibroblastic tumors are rare in the pediatric population. Most common localizations were reported in the lungs. A localization in the pancreas needs differentiation from other tumors and chronic pancreatitis. Treatment is surgical resection, although there are reports of treatment with oral steroids and radiation therapy. CASE REPORT: A 6-month-old child was treated due to a tumor in the head of the pancreas. On admission he was jaundiced with pruritus. US and MRI confirmed pancreatic tumor. Preoperative biopsy wasn't conclusive regarding the nature of the tumor. Duodenopancreatectomy was performed. Postoperative course was uneventful. Histologic examination confirmed the diagnosis of inflammatory myofibroblastic tumor. On follow up, he remained with no evidence of recurrence. CONCLUSIONS: A literature review revealed 10 cases of pancreatic inflammatory myofibroblastic tumors in the pediatric age group. Our patient is the youngest reported. Despite major resection, there were no complications. However, management of this child might be possible with steroids, but conservative treatment might be insufficient, especially in aggressive forms of tumors.

Prolonged prostaglandin E1 therapy in a neonate with pulmonary atresia and ventricular septal defect and the development of antral foveolar hyperplasia and hypertrophic pyloric stenosis.

Prostaglandin E1 (alprostadil) is widely used for maintaining the patency of ductus arteriosus in ductus-dependent congenital heart defects in neonates to improve oxygenation. Among more common side effects are fever, rash, apnoea, diarrhoea, jitteriness, and flushing. More severe side effects are brown fat necrosis, cortical hyperostosis, and gastric outlet obstruction, most commonly the result of antral foveolar hyperplasia or hypertrophic pyloric stenosis. We report on an infant with a ductus-dependent congenital heart defect who developed symptoms and sonographic evidence of focal foveolar hyperplasia and hypertrophic pyloric stenosis after prolonged treatment with prostaglandin E1. Gastrointestinal symptoms persisted after corrective cardiac surgery, and pyloromyotomy was required. Study of the case and of available literature showed an association between the total dose of prostaglandin E1 administered and duration of treatment and the development of gastric outlet obstruction. We conclude that if patients are treated with a prostaglandin E1 infusion, careful monitoring for symptoms and signs of gastric outlet obstruction is required.

Giant T-shaped duplication of the transverse colon. A case report.

A case of long diverticular colonic duplication producing acute abdominal pain in a 6-year-old girl is presented. Physical examination showed no signs of acute abdomen at the initial presentation. After a pain-free interval, there was a sudden onset of severe abdominal pain and a large tumor in the lower abdomen was observed. A plain x-ray showed an enormously dilated colonic pouch filled with gas. Excision of the T-shaped duplication and small part of the transverse colon was successful. Because of extensive fibrotic changes in the colon near the opening of duplication, a resection margin of at least 2 cm is recommended.

Meckel's diverticulum perforation with intraabdominal hemorrhage.

Perforation of Meckel's diverticulum in children is a rare and serious complication. The authors report a case of a 3-year-old boy with perforation and hemoperitoneum caused by Meckel's diverticulum. Difficulty of preoperative diagnosis is discussed, indication for incidental diverticulotomy is established, and the literature is reviewed.

Anatomical variations of the arterial pattern in the left hemiliver.

The arterial supply to the left hemiliver was studied in 70 liver casts. The arteries were divided into 15 groups according to their origin and branching pattern. The left hemiliver was supplied by one artery in 53% of cases, by two arteries in 40% and by three arteries in 7%. The left hepatic artery, which originated from the proper hepatic artery, supplied all three left segments in 39% of specimens. The replacing left hepatic artery, which originated from the left gastric artery, supplied the whole left hemiliver in 3% of cases. The incomplete, replacing left hepatic artery supplied segments 2, 3 and a part of segment 4 in 6% of cases, and only segments 2 and 3 in 11%. There was one segmental artery for segment 2 in 86%, and two in 14%. Segment 3 was supplied by one artery in 87%, and by two in 13%. Segment 4 was supplied by one artery in 39% of cases, by two arteries in 43%, by three in 14% and by four arteries in 4%.