Unusual hepatic tumor with features of mesenchymal hamartoma and congenital solitary nonparasitic cyst.

We report a hepatic tumor in an adolescent that does not fit into any of the described categories of liver tumors. The patient presented with hepatomegaly, abdominal pain, and normal liver function test; the tumor was cystic in imaging studies. The resected specimen, result of a partial hepatectomy, measured 21 cm and was multicystic with solid areas. Microscopically, the cysts were lined by a mucous-producing or intestinal-type epithelium, associated with smooth muscle and small mucous-producing glands. The solid component contained fibrous and adipose tissue, smooth muscle and thick-walled vessels. Aneuploidy was demonstrated by flow cytometry. We interpreted the tumor as having features of a mesenchymal hamartoma and congenital solitary nonparasitic cyst. It is conceivable that the lesions originated with small peribiliary glands with dilatation and intestinal metaplasia.

Bilateral eventration of the diaphragm with perforated gastric volvulus in an adolescent.

Bilateral congenital eventration of the diaphragm almost uniformly presents in infancy with respiratory compromise and is associated with a high mortality rate. Delayed presentation of diaphragmatic eventration in older children and adults may be associated with acute gastric volvulus. Thus, any patient with abdominal pain, vomiting, or nonspecific gastrointestinal symptoms in association with abnormal diaphragmatic findings on chest x-ray should undergo further diagnostic workup with upper gastrointestinal series or computed tomography (CT) scan. Treatment of gastric volvulus requires immediate surgical repair to prevent subsequent necrosis and perforation. The authors describe a case report of bilateral congenital diaphragmatic eventration complicated by a perforated gastric volvulus in a 13-year-old boy. Emergent reduction of the volvulus, closure of the perforated stomach, plication of the diaphragm, and placement of gastrostomy was performed successfully.

Eighteen years' experience with neonatal Hirschsprung's disease treated by endorectal pull-through without colostomy.

METHODS: In the past 18 years, the authors have treated 84 patients with Hirschsprung's disease. Of these, 43 patients were under 1 month of age and underwent endorectal pull-through without colostomy. Some have undergone follow-up for as long as 18 years. RESULTS: Thirty-four of these 43 (79%) newborn patients were available for follow-up. Twenty-two were totally continent. The remaining 12 have normal sphincter tone. Of the 41 patients above 1 month of age, 34 (83%) were available for follow-up. Some have undergone follow-up for as long as 18 years. CONCLUSION: Twenty-two of this latter group (79%) have normal bowel control.

Papillary-cystic neoplasm of the pancreas.

Papillary-cystic neoplasm of the pancreas is a rare, nonfunctioning low-grade malignant tumor seen in young patients, most often female. Ultrasound and CT show a circumscribed, solid nonhomogeneous mass with cystic areas, with peripheral but not central enhancement and occasional calcification. Prognosis after excision is usually excellent. We describe a case of the papillary-cystic neoplasm of the pancreas in a 13-year-old girl to illustrate the radiological findings.

Idiopathic infant pyoceles.

Three infants presented with acute scrotal swelling, erythema, and a tender irreducible firm mass within the scrotum. All patients were operated upon with the preoperative diagnosis of testicular torsion. At operation, grossly purulent fluid was found in a hydrocele sac. In all the cases, the testes were normal and no source of the scrotal infection was identified. A variety of bacterial organisms were cultured and responded readily to antibiotics. Follow-up has documented survival of the testes without apparent atrophy. "Idiopathic" infant pyocele is rarely described and consequently, not included in the differential diagnosis of patients with acute surgical conditions of the inguinoscrotal region.

Gastroesophageal fundoplication and gastrostomy in familial dysautonomia.

Gastric and esophageal dysfunction are components of familial dysautonomia. The limited success of various medical management programs, has led to two types of surgical intervention. Experience with nine patients who had gastrostomy alone and 12 patients who had gastroesophageal fundoplication is reviewed. Both surgical procedures decreased frequency of vomiting and pneumonias and had positive effects on weight gain. Although "dysautonomic crises" are not eliminated, sufficient modification in character occurs so that associated risks are lessened. It is suggested that if medical management cannot control recurrent pneumonia, postprandial vomiting, esophageal bleeding, and/or inadequate weight gain, then the patient should be evaluated for fundoplication and/or gastrostomy.

CT in B-thalassemia: iron deposition in the liver, spleen, and lymph nodes.

The authors report the striking increase in lymph node density due to hemochromatosis observed with computed tomography (CT) in nine patients with Cooley anemia treated with multiple blood transfusions. The CT appearance and pathologic findings of hemochromatosis of the liver and spleen in three of these patients were also observed and correlated with pathologic specimens. CT density of the liver seemed to relate to the degree of hepatic fibrosis or cirrhosis, rather than the amount of iron. Previous reports have not emphasized dense ferritinized lymph nodes in treated Cooley anemia patients.

The umbilical polyp.

Anomalies of the omphalomesenteric duct (OMD) present a variety of surgical problems. The most readily apparent of these lesions is the umbilical polyp. One-third of patients explored at our hospital and 56% of patients reviewed had an additional OMD anomaly when explored for an umbilical polyp. The preferred treatment for this lesion should be a mini-laparotomy after the presence of intestinal mucosa at the umbilicus is confirmed.

Villous tumors of the duodenum.

Villous tumors of the duodenum are rare, but treatment may be problematic because of their association with invasive adenocarcinoma. Two cases of villous tumor of the duodenum are described and 39 other reported cases are reviewed. Presenting symptoms were bleeding 27%; obstruction 24%; jaundice 22% and vague dyspepsia 20%. Diagnosis may be made by radiographic barium contrast evaluation of the duodenum, especially with the addition of air contrast hypotonic studies and by fibro-optic endoscopy. Twenty-seven per cent of villous tumors of the duodenum are associated with adenocarcinoma. Invasive tumor is more common in patients over 50 years old (35%), in tumors of the third and fourth portions of the duodenum (44%) and in tumors over 4 cm in diameter (30%). Local excision is the treatment of choice for benign lesions. Pancreatico-duodenectomy is recommended for tumors which include invasive carcinoma in patients without distal metastases.