Jordan J. Weitzman

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 Downs syndrome, the patients 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.

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 (omphalocoele—9, gastros-chisis—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%.

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 complete 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.

Jejunal atresia with agenesis of the dorsal mesentery: With “Christmas tree” deformity of the small intestine*

Summary Four cases of jejunal atresia with agenesis of the dorsal mesentery associated with a “Christmas tree” deformity of the small intestine are presented. Evidence that this anomaly is the result of a fetal vascular accident is presented, and the treatment of this unusual anomaly is briefly discussed.

Rectal suction biopsy for the diagnosis of Hirschsprung's disease

The diagnosis of Hirschsprungs 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 Hirschsprungs 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 Hirschsprungs 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.

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.

The significance of right aortic arch in repair of esophageal atresia and tracheoesophageal fistula

Abstract Of 130 infants with esophageal atresia who were operated on during the past 12 yr, 7 (5.4%) had a right aortic arch. In 1 infant, preoperative recognition of the right arch allowed successful primary repair of the esophagus through the left chest. In 6 infants, an unsuspected right arch was found at the time of initial right thoracotomy. In 2, repair from the right side was difficult but successful. In 4, however, the right arch markedly altered the clinical course, leading to the death of 1 infant, abandonment of esophageal repair and colon interposition in another, and bilateral thoracotomies for successful repair in 2 more infants. From this experience, we conclude that the presence of a right aortic arch is of considerable surgical significance in infants with esophageal atresia. We believe preoperative localization of the arch is important in every infant with esophageal atresia and tracheoesophageal fistula (TEF) not only because a right aortic arch poses a definite technical problem during right-sided repair, but also because a right aortic arch is frequently associated with congenital heart disease or vascular compression of the trachea and esophagus. The following recommendations arise from this review: (1) Infants with esophageal atresia should be screened preoperatively by noninvasive techniques for the presence of a right aortic arch. If these studies raise the possibility of a right aortic arch or there is any other evidence of cardiac disease, angiography should be performed. (2) Surgical repair of esophageal atresia is best performed from the side opposite the aortic arch. (3) If a previously unsuspected right aortic arch is found at the time of right thoracotomy for repair of esophageal atresia, the alternatives in management are a matter of surgical judgment. Our experience suggests that in many cases it is safest to close the right thoracotomy without further dissection of the esophageal pouches or with simple ligation of the TEF, and to perform the definitive esophageal repair through a subsequent left thoracotomy.

Renal transplantation in young children

Abstract Thirty-one children, aged one and a half to twelve years, received thirty-six renal allografts from fourteen living related donors, one living unrelated donor, and twenty-one cadaver donors during a five year period of study. Twenty-five of the thirty-one children (81 per cent) are presently alive with functioning allografts (twenty first and five second transplants); five children (16 per cent) have died and one child (3 per cent) is undergoing repetitive hemodialysis while awaiting a subsequent graft. The medical and surgical complications and rehabilitative aspects of the treatment program are delineated in detail to emphasize the acceptability of young children as candidates for renal transplantation.

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.

dl‐α‐TOCOPHEROL, IRON, AND LIPOFUSCIN IN THALASSEMIA*

In 1969 we started a multidisciplined longitudinal study of the physiologic effects of preventing intertransfusion anemia in 1 3 patients with @-thalassemia major in whom the pretransfusion hemoglobin level was increased from approximately <7 g % to > 11 g %. The protocol included annual biopsies of skin, liver, thyroid, and testes to determine whether there was acceleration of iron deposition or other pathologic changes in these tissues. The biopsy material was examined with a variety of stains, including Sudan Black, and was also examined for autofluorescence. In this way, the observation was made by one of us (B.L.) that lipofuscin was present in abnormal amounts in many of the specimens. The distribution of the lipofuscin deposits, including presence in the smooth muscle of small blood vessels as well as in epithelial and reticuloendothelial cells, resembles that seen in vitamin E deficiency.’ This is a preliminary report of a study designed to determine whether these patients are deficient in vitamin E, and to evaluate the effects, if any, on their tissues of chronic administration of vitamin E in pharmacologic doses.