Timing of colonic necrosis in hemolytic uremic syndrome.

Hemolytic uremic syndrome (HUS) consists of an acute onset of microangiopathic hemolytic anemia, thrombocytopenia, and renal dysfunction. HUS-associated colitis can be seen in up to 100% of patients and is usually associated with severe abdominal pain and distention. Colonic perforation is a complication of HUS that has a reported incidence of 1%-2%, and although there are several case reports in the literature describing perforation of the colon, it is still very difficult to discern the abdominal symptoms associated with HUS colitis from perforation.

Pouchitis in young patients after the ileal pouch-anal anastomosis.

: The purpose of this study was to determine the incidence of pouchitis after the ileal pouch-anal anastomosis in young patients, and to identify possible predisposing factors. The study design included long-term follow-up of young patients after closure of their temporary diverting ileostomy, through regular visits, a detailed questionnaire, or phone call. The Kaplan-Meier method was used to summarize survival free of pouchitis in several subgroups.

Differentiated and medullary thyroid cancer in childhood and adolescence.

Thyroid cancer is rare in childhood and consists of several different histopathologic groups with widely varying clinical behavior. Major categories include differentiated, medullary, and anaplastic thyroid cancer. Non-Hodgkin's lymphoma and various sarcomas also can arise in the thyroid.

Transanal mucosectomy: a modification of the Soave procedure for Hirschsprung's disease.

In the Soave procedure for Hirschsprung's disease, the mucosectomy of the aganglionic segment of colon is performed transabdominally. The authors describe an innovative modification of the standard Soave procedure, in which a transanal mucosectomy is performed as the abdominal incision is made. The transanal mucosectomy technique was compared with the conventional approach.

Surgical management of Crohn's disease in children.

Crohn's disease is a chronic, transmural inflammatory disease of the intestinal tract most frequently involving the terminal ileum and colon. It is a disorder of undetermined etiology that shares many pathophysiologic clinical aspects with chronic ulcerative colitis. Surgical treatment of Crohn's disease continues to be generally limited to the treatment of the complications of the disease.

Medullary thyroid carcinoma in children.

Medullary cancer of the thyroid (MCT) in children, although uncommon, represents not only a most intriguing problem but also one that is a model of early cancer detection that results in cure. MCT in children is usually a part of the multiple endocrine neoplasia (MEN) syndrome (MCT, pheochromocytoma, and hyperparathyroidism) and is detected by screening studies. MCT arises from C cells that secrete calcitonin, with C-cell hyperplasia representing the precancerous state.

Papillary carcinoma of the thyroid in children.

Papillary carcinoma of the thyroid is the most common thyroid cancer in children. It usually occurs in the teenage female and presents as a 3-cm mass within the thyroid itself. Although the tumor is well differentiated, almost 80% of all children will present with local lymph node metastasis.

Thyroglossal duct cysts and branchial cleft anomalies.

Thyroglossal duct cysts and branchial cleft anomalies are important in the differential diagnosis of cervical lesions. Both lesions are common and are seen more commonly in the child than in the adult. Considerable discomfort and morbidity may be associated with severe infections occurring in both of these lesions.

Current surgical management of Crohn's disease in childhood.

Crohn's disease is a chronic transmural inflammatory disease that may involve any portion of the gastrointestinal tract. It is being reported with increasing frequency in children of all ages. The diagnosis of Crohn's disease in children is dependent on the clinical, radiological, endoscopic, and pathological material that excludes other acute inflammatory disease of the gastrointestinal tract.

Rectal mucosectomy: a definitive approach to extensive hemangiomas of the rectum.

Major rectal bleeding may occur in children and adults who have extensive hemangiomatous involvement of the pelvis and rectosigmoid colon, as in the Kleppel-Trenaunay syndrome (KTS). Conventional surgical techniques such as bowel resection and colostomy have often been associated with large blood loss and/or incontinence. We have used a new approach to this problem utilizing rectal mucosectomy to eliminate the bleeding rectal mucosa and to preserve anal function.

Review of head and neck lesions in infancy and childhood.

Common congenital and infectious lesions of the head and neck in infancy and childhood have been discussed. Those that are present at birth, asymptomatic, and frequently cystic are clearly benign lesions that require operative management for the potential complications of enlargement and infection. Solid lesions, particularly those of the lymph nodes, must be differentiated from neoplasms, particularly Hodgkin's disease and non-Hodgkin's lymphoma.

Giant epignathus. A case report.

We report the successful treatment of a 2.8 kg female infant born with a giant epignathus, and we present our current prenatal and neonatal recommendations for managing this problem. We recommend that the delivery be done by cesarean section, that an adjacent operating room be ready for the baby, and that a neonatologist, anesthesiologist, and pediatric surgeon be standing by.

The surgical management of children with ulcerative colitis. The old vs. the new.

The authors' experience with children who have chronic ulcerative colitis was reviewed to compare their current surgical approach (ileoanal anastomosis) with earlier methods of management. Between 1960 and 1984, 137 children with chronic ulcerative colitis underwent surgery (mean duration of follow-up, 7.1 years).

Carcinoid tumor of the appendix in the first two decades of life.

Carcinoid tumor of the appendix is the most common neoplasm of the gastrointestinal tract in childhood and adolescence. Sufficient long-term follow-up data after surgical treatment are not currently available for patients diagnosed during the first two decades of life. From 1936 to 1988, 23 patients were observed at this institution with histologically confirmed carcinoid tumors involving the vermiform appendix.

Long-term follow-up of the ileoanal anastomosis in children and young adults.

The purpose of this study was to carry out a long-term study of the ileoanal anastomosis (IAA) in children and young adults, comparing the straight IAA to the J pouch. One hundred twenty-one young people who had undergone IAA were studied, with 114 available for long-term follow-up. One hundred one were 18 years and under.

Evaluation of children with multiple endocrine neoplasia type IIb following thyroidectomy.

Medullary thyroid carcinoma (MTC) develops in all patients with multiple endocrine neoplasia type IIb (MEN IIb), a rare syndrome that either occurs sporadically or is inherited in an autosomal dominant pattern. The MTC in patients with MEN IIb has been reported to be biologically aggressive with onset at a young age and rapid progression as evidenced by widespread metastases and death, frequently in the teenage years. Seven children, aged 2 to 11 years (mean, 7 years), from three kindreds with MEN IIb were evaluated for evidence of tumor recurrence 3 to 10 years following thyroidectomy.

Exercise cardiorespiratory function in adolescents with pectus excavatum. Observations before and after operation.

We studied 12 children with pectus excavatum (mean age, 13.8 years) using pulmonary function and exercise testing. Eight patients had surgical repair and were studied before and after repair.

Pediatric urological complications with intraoperative radiation therapy.

Intraoperative radiotherapy with variable energy electrons has been used as a supplemental boost to treat 6 children with locally advanced retroperitoneal malignancies. Of the patients 4 had treatment-related injuries to portions of the urinary tract within the intraoperative and external radiation fields. Three patients had significant renal impairment requiring surgical correction.

Medullary carcinoma in children. Results of early detection and surgery.

Early diagnosis and surgical treatment of medullary carcinoma of the thyroid (MCT) in children is essential to decrease the likelihood of metastatic spread. To determine the optimal timing of screening and surgery, we reviewed all children younger than 16 years old who were undergoing surgical treatment. From 1970 to 1988, 33 children 6 months old to 15.

Clinical outcome in children with acute cerebellar encephalopathy and neuroblastoma.

Acute cerebellar encephalopathy (ACE)--ataxia often associated with opsoclonus, polymyoclonus, and irritability--may be associated with neuroblastoma and should be suspected in a child who presents with ACE. The survival in ten children with ACE associated with neuroblastoma was 100%. Most of the tumors were ganglioneuroblastomas.

Distribution, quantitation, and origin of immunoreactive neuropeptide Y in the human gastrointestinal tract.

A radioimmunoassay for measurement of immunoreactive neuropeptide Y has been developed using antiserum from a rabbit (221) immunized with porcine neuropeptide Y. Antibody 221 has been characterized for both sensitivity and specificity. To determine the distribution of neuropeptide Y in the human gastrointestinal tract, fresh tissue specimens were separated by microdissection into the muscularis externa and the mucosa-submucosa.

Localization of pancreatic insulinoma: comparison of pre- and intraoperative US with CT and angiography.

Methods of preoperative radiologic localization of insulinoma were compared in 52 patients, 44 of whom had solitary tumors. Examinations performed in these 44 patients were preoperative ultrasonography (US) in 28, angiography in 26, and computed tomography in 23. Prospective sensitivities were 61%, 54%, and 30%, respectively.

The endorectal pull-through procedure in children and young adults: a follow-up study.

At our institution, proctocolectomy with rectal mucosectomy and ileoanal anastomosis with endorectal pull-through is now the procedure of choice for young patients with chronic ulcerative colitis and familial polyposis. We have followed up 66 patients (37 male and 29 female, with a median age of 16 years) for at least 6 months after closure of the temporary ileostomy. The patients were seen regularly in follow-up or were sent a comprehensive questionnaire to assess their condition.