Bronchogastric fistula, pulmonary sequestration, malrotation of the intestine, and Meckel's diverticulum--a new association.

Two female children, each who had a bronchogastric fistula and pulmonary sequestration (communicating bronchopulmonary foregut malformation, CBPFM) and associated malrotation of the intestine and Meckel's diverticulum are presented. Each child also presented with severe gastroesophageal reflux. The association of malrotation of the intestine and Meckel's diverticulum with a CBPFM never has been reported as a distinct entity. The concept of association of anomalies is discussed briefly.

Hirschsprung's disease: a possible cause of anastomotic failure following repair of intestinal atresia.

Despite several reported cases, the association of Hirschsprung's disease and intestinal atresia is not widely recognized. We describe three patients with jejunoileal atresia who all had a preoperative barium enema that failed to show a transition zone. All three patients developed an anastomotic leak of the atresia repair, and required a temporary diverting enterostomy. Two patients had total colonic Hirschsprung's disease and in one, the transition zone was in the midtransverse colon. Histological examination of the appendix at the time of repair may be helpful in patients with small intestinal atresia. In patients who develop an anastomotic breakdown, rectal biopsy should be performed to rule out Hirschsprung's disease.

Malrotation of the intestine in children.

Intestinal malrotation may be complicated by volvulus and intestinal necrosis. One hundred two children (64 male, 38 female) undergoing surgical abdominal exploration from 1977 to 1987 had malrotation. Fifty-two patients were less than 7 days of age, 13 from 8 to 30 days, 26 from 31 to 365 days, and 11 were older than 1 year of age. Of infants, 39 of 65 had 40-week gestations, 18 of 65 had 36- to 39-week gestations, and 8 of 65 had less than 36-week gestations. Chief symptomatology included: bilious emesis (47), intestinal obstruction (19), abdominal pain (11), and bloody stools (7). Seventy patients had congenital anomalies (50 single, 20 multiple). Diagnostic evaluations included 56 upper gastrointestinal series and 27 barium enemas. Each patient underwent correction of malrotation and appendectomy, and correction of congenital anomalies (omphalocele-9, gastroschisis-6, diaphragmatic hernia-7). Complications included short gut (2), sepsis (5), feeding difficulties (2), pneumonia (3), small bowel obstruction (2), and other (15). Nine patients (8.8%) died (trisomy 18-1, trisomy 13-1, intestinal necrosis-3, hepatic failure-1, prematurity-1, other sepsis-2). Two hundred sixteen children with intestinal malrotation have been treated from 1937 to 1987. Mortality rate has improved from 23% to 2.9%.

Successful late Kasai portoenterostomy.

This article describes an infant with extrahepatic biliary atresia who underwent a successful hepatoportoenterostomy at age 132 days in spite of a delay in diagnosis. Contributing to the delay in diagnosis of this case were the lack of appreciation of alcoholic stools, the presence of a gallbladder and distal common bile duct on ultrasound examination, and insufficient biopsy material for interpretation. This case emphasizes the variability in presentation and potential difficulties in diagnosis and the need for intraoperative cholangiogram and exploratory laparotomy in such cases. An approach to the patient with extrahepatic biliary atresia who is diagnosed late is discussed. A successful outcome can be achieved for such patients.

Transanal mucosal sleeve resection for the treatment of rectal prolapse in children.

This is a report of a simple transanal operation performed on six patients (age range, 19 months to 18 years), who underwent unsuccessful nonoperative management of complete rectal prolapse for at least 1 month (range, 1 month to 13 years). All patients had normal sweat chloride levels, normal chest radiographs, and normal barium enemas. None of the patients were neurologically compromised. At the time of surgery, all but one patient had occurrence of reducible prolapse with minor straining or with every bowel movement. No severe mucosal ulcerations were present. Surgical therapy consisted of the transanal mucosal sleeve resection described herein. In this series, there were no anastomotic leaks, no clinically evident strictures and no recurrence of prolapse in 1.5- to 19-year follow-up. Surgical therapy for rectal prolapse in infants and children is rarely necessary. Various complicated or ineffective operations for the treatment of this condition have been recommended in the past. This technique offers a simple, safe, and effective method of treating complete, medically intractable rectal prolapse in children.

A 40-year multinational retrospective study of 880 Swenson procedures.

This report reviews the experience of pediatric surgeons in seven cities in North American and Western Europe where the Swenson procedure was performed on 880 patients. Information on the diagnosis, treatment, complications, and long-term results was collected by reviewing the hospital records, the treating physicians' office records, and by interviewing the patients in person or by telephone. A follow-up evaluation was obtained on 814 patients. The patients' ages at the time of the resection ranged from four days to 50 years. The length of follow-up averaged 10.3 years, while the longest follow-up was 39.5 years. The overall postoperative mortality was 2.4% during the entire 40 years of the study. The postoperative mortality has decreased to 1.25% for the last 20 years. Significant factors influencing postoperative mortality included Down's syndrome, the patient's age at the time of the operation, and leak of the distal colonic anastomosis. Most of the patients followed for over 5 years have normal bowel habits, report one to three bowel movements per day, and have no soiling. No patient has urinary incontinence or impotence.

Esophageal replacement with colon interposition in children.

During a 21-year period, 39 colon interposition operations were performed on 37 children at the UCLA Medical Center and the Childrens Hospital of Los Angeles. The average age at the time of operation was 5.8 years. The indications for operation were esophageal atresia in 23 patients and other benign strictures in 14 patients. The duration of patient follow-up ranged from 6 months to 21 years (mean: 9.7 years). The most common complications were esophagocolonic anastomotic leak (12), esophagocolonic anastomotic stricture (14), pneumonia, and pneumothorax. Fourteen of the 25 patients with retrosternal colon interposition had complications (56%), whereas 10 of 14 patients with left thoracic colon interposition had complications (71%). One patient died (mortality: 3%) after left thoracic interposition because of severe respiratory distress associated with other malformations. Each of the 18 patients with isoperistaltic colon interposition showed rapid transit and emptying, provided that obstruction or extensive dilatation did not occur; reverse colon segments were more dilated and emptied more slowly. The 25 patients with retrosternal colon segments had less colonic distension with better emptying than did the 14 patients with left thoracic interposition. Thirty-two of the 36 children increased their weight percentile after colon interposition. Within 2 years after cervical anastomotic stricture or leak, 78% of these children were asymptomatic and gaining weight. Thirty-one of the 37 patients (84%) had excellent results with colon interposition, with a mean follow-up of 9.7 years. Most of the major postoperative complications occurred within the first few weeks and were corrected during the first few months after operation. Preservation of the esophagus should be the surgeon's first priority; however, prolonged attempts to elongate the esophagus for anastomosis in certain patients with long-gap esophageal atresia have been more hazardous in our experience than has colon interposition.

Preoperative chemotherapy for unresectable primary hepatic malignancies in children.

Eight children presenting with unresectable primary hepatic malignancies were treated with chemotherapy in an attempt to decrease the size of the tumor. Adriamycin was used in all drug regimens, usually in combination with cyclophosphamide, vincristine, and 5-fluorouracil. Seven children exhibited a pronounced, clinical response with marked reduction in the size of the primary tumor as well as any pulmonary metastases present. Four children were able to have complete, uncomplicated surgical excision of residual disease, and three are alive and well off therapy. One patient with hepatocellular carcinoma had compete disappearance of all disease with chemotherapy alone. An approach utilizing preoperative chemotherapy for extensive hepatic malignancies may permit eventual resection of initially inoperable lesions, with long-term survival for these highly lethal malignancies.

Metastatic medullary thyroid carcinoma in young children with mucosal neuroma syndrome.

Multiple endocrine neoplasia, type 2b (MEN 2b) is a disorder characterized by a distinct phenotype and a predisposition for medullary thyroid carcinoma (MTC) and pheochromocytoma. Two siblings aged 3 and 6 years with MEN 2b, who had elevated plasma calcitonin levels suggesting the presence of MTC are described. Microscopic foci of MTC were found in their thyroid glands and the glands were removed. In the younger child, a metastatic focus was present in a cervical lymph node. Of 12 previously reported children with MEN 2b and MTC who were less than 10 years of age at diagnosis, five had metastases. One of the present patients is the youngest described with this complication. The high frequency of metastases in very young patients with this syndrome has not been emphasized previously. These findings indicate the need for early diagnosis of MEN 2b and the importance of thyroidectomy at the earliest possible age when MTC is suspected by calcitonin screening tests.

Rectal suction biopsy for the diagnosis of Hirschsprung's disease.

The diagnosis of Hirschsprung's disease is at times difficult, particularly in the young patient. Since 1972 we have used rectal suction biopsy as a screening technique in neonates and infants with failure to pass meconium or evidence of obstruction. In addition, it is used to confirm the diagnosis of Hirschsprung's disease when suspected by barium enema study. This technique has been used in 444 patients, 302 of whom were less than one year of age. No anesthesia is necessary, and there have been no associated complications. Only one patient early in the study had an initial misdiagnosis. There have been no false-positive or false-negative specimens since this initial problem, and no patients have undergone inappropriate pull-through procedures for suspected Hirschsprung's disease. It is recommended that all neonates who do not pass meconium in the first 48 hours of life undergo rectal suction biopsy to establish the diagnosis of congenital megacolon.

Alternate approach to the management of acute perforating appendicitis in children.

The results of this study support the conservative management of acutely ill patients with the clinical diagnosis of perforating appendicitis. An interval appendectomy should be performed four to six weeks later. If the patient does not respond after a clinical trial of 12 to 24 hours, there is no increased morbidity from performing an appendectomy at that time. Selection of antibiotic therapy should be appropriate to cover the usual intestinal flora of aerobes and anaerobes. Ampicillin, gentamycin or tobramycin, and clindamycin currently are the recommended antibiotics at this institution. The patient is seen in the office each week until interval appendectomy is performed. Any recurrence of fever or abdominal pain warrants immediate hospital admission and appendectomy. This approach in selected patients had proved safe when adequate follow-up study is assured.

Hyperthyroidism in children. A reevaluation of treatment.

The results of medical and surgical therapy were determined in 107 hyperthyroid children. After surgery, 85% of patients were rendered free of hyperthyroidism; however, 62% became hypothyroid. After medical treatment, 30% of patients were euthyroid and 2% became hypothyroid. The relapse rate, however, was higher after medical (22%) than after surgical (9%) therapy. Serious drug-related complications (arthritis-, hepatitis-, and collagen disease-like syndromes) occurred in 14% of patients. Complications occurred in 9% of surgically treated patients, but recurrent laryngeal nerve injury or permanent hypoparathyroidism did not occur. In medically treated patients, both a goiter size less than three times normal prior to treatment and a reduction in goiter size to less than two times normal at the completion of therapy correlated with a successful outcome.

Thyroidectomy for hyperthyroidism in children.

Between November 1964 and August 1978, 66 patients underwent subtotal thyroidectomy for hyperthyroidism. Fifty-seven of these patients have been followed for more than 2 yr postoperatively and form the basis for this report. The mean age of these patients was 11 7/12 yr. There wre no deaths in this series and no recurrent laryngeal nerve injuries. Hyperthyroidism recurred in 4 patients from 10 to 60 mo following surgery (mean of 30 mo). Patients with relapse had a significantly larger gland at operation, but no difference in estimated thyroid remnant. Those patients with larger glands at exploration need a relatively larger percentage of the gland removed to prevent recurrent hyperthyroidism.

Preoperative chemotherapy for hepatoblastoma in children: report of six cases.

Surgical excision has been the primary treatment for hepatoblastoma; however, at presentation, only one-third of such tumors are surgically resectable. Without operation, the disease is universally fatal. Six children with initially unresectable hepatoblastoma (two with pulmonary metastases) were treated with chemotherapy, which included Adriamycin. Four of the six children showed a significant reduction in tumor size, in three delayed resection of the primary lesion was possible, and the fourth patient died of Adriamycin cardiotoxicity. Two patients did not respond and developed pulmonary metastases after 2 and 16 mo of chemotherapy, respectively. Adriamycin alone, or in combination with other agents, has proven effective in primary and metastatic childhood hepatoblastoma. This preoperative chemotherapy regimen permits resection of previously unresectable hepatoblastoma at "second look" operation and reduces the morbidity and mortality of an otherwise extensive operation.

Operative technique for the correction of congenital obstruction of the duodenum in the neonate.

More than 30 newborn infants with a congenital obstruction of the duodenum were managed by the technique described. There have been no deaths or anastomotic complications. None of the patients has experienced any problems related to the respoitioning of the midgut at long term follow-up study.

Splenoportography in the pediatric age group.

Eleven splenoportograms were performed in 10 patients between the ages of 2.5 and 17 yr. A definitive diagnosis was made in every instance. There were no complications despite the fact that 6 patients had platelet counts less than 100,000/mm3. Fear of potential complications is unwarranted, and splenoportography should be an essential part of the workup of a child suspected of having portal hypertension. Splenoportography, when combined with measurement of splenic pulp pressure, provides precise information regarding the presence of and/or change in portal hypertension and its underlying cause. With this information, therapy for the pediatric patient with portal hypertension can be individualized and managed in a logical fashion.

Primary chemotherapeutic management of unresectable and metastatic hepatoblastoma in children: report of four cases.

Four children presenting with unresectable hepatoblastomas and one with metastatic disease are reported. Following initial biopsy all were treated with chemotherapy which included Adriamycin. Three of the four children showed a significant reduction in tumor size, and in two, delayed resection of the primary lesion was possible. Chemotherapy including Adriamycin represents effective initial cytoreductive therapy for childhood hepatoblastoma, thereby reducing the morbidity and mortality associated with the extensive hepatic resection usually required for an untreated lesion.