Harry C. Bishop

Surgical management of alimentary tract duplications

Alimentary tract duplications are unusual anomalies that may require surgical intervention in the neonate, infant, and occasionally in the older child. The clinical presentation of patients with alimentary tract duplications includes bleeding, abdominal pain, intussusception, and respiratory distress, or it may be an incidental finding on either abdominal examination or chest x-ray. A review of 96 patients with 101 duplications seen over the last 37 years is reported herein. Twenty-one duplications were confined to the thorax; three were thoracoabdominal, and 77 were abdominal. Seventy-four patients presented as infants less than 2 years of age, and 22 patients were older. Ectopic gastric mucosa was found in 21 duplications, and pancreatic tissue was found in five. Seventy-five duplications were cystic and 26 were tubular. Ultrasonography, computed tomography (CT), and myelography are helpful diagnostic tools. Ninety-four of the 96 patients underwent surgical management for their duplications. One duplication was found at necropsy, and one patient was asymptomatic and did not undergo operation. A single death occurred in a 2-day-old infant who had intrauterine volvulus and meconium peritonitis. Management was based on the age and condition of the patient, the location of the lesion, whether it was cystic or tubular and communicating with the true intestinal lumen, and whether it involved one or more anatomic locations. Generally, total excision was preferred, but staged approaches were sometimes necessary.

Total parenteral nutrition in infants with catastrophic gastrointestinal anomalies

Abstract Intensive nutritional support is essential for survival and surgical rehabilitation of newborn infants with gastrointestinal anomalies. Oral feedings can usually be established after correction of a single congenital defect. At times, a feeding tube or gastrostomy may be required to provide nutrients to poor risk infants. However, all too often, enteral feeding is impossible and parenteral support inadequate, so that babies with multiple or complex lesions of the gastrointestinal tract die from the complications of starvation. The laboratory demonstration that normal growth and development can be achieved by supplying basic nutrients exclusively by the intravenous route 1 prompted use of the technic in surgical patients. More than 200 adults with chronic complicated gastrointestinal disease have been supported exclusively by the intravenous route with 2400–5000 calories per day for 10 to 200 days. Weight gain was observed in all patients associated with wound healing, fistula closure, positive nitrogen balance, and increased strength and activity. 2 Preliminary data on the first infant to be nourished by this intravenous feeding technic demonstrated normal growth, development, and metabolism despite near total absence of the small bowel and gastrointestinal dysfunction. 3 This is the first group of infants in whom total parenteral nutrition was used in conjunction with surgical treatment of catastrophic gastrointestinal anomalies.

Morbidity and mortality of short-bowel syndrome acquired in infancy: An update

The advent of total parenteral nutrition (TPN) has made survival beyond infancy possible for large numbers of patients who have sustained massive small intestinal loss due to a variety of intraabdominal catastrophes. However, the quantity and quality of life have been limited by the development of late sequelae due both to the protracted use of TPN and the long-term complications of foreshortening of the gut. To determine to what extent the morbidity and mortality of short-bowel syndrome (SBS) may have improved over the last 10 years, we reviewed our experience since 1973 with patients losing more than 50% of total small intestinal mass in infancy. The etiologies of SBS in the 16 study patients were necrotizing enterocolitis (6), midgut volvulus (5), multiple atresias (3), gastroschisis (1), and congenital SBS (1). Overall survival was 81%; total small intestinal length (SIL) at the time of diagnosis was 44.2 +/- 7.9 cm in survivors and 30.3 +/- 7.8 cm in nonsurvivors, probability values not significant. Although no patient survived without an ileocecal valve whose total SIL was greater than 20 cm, the three deaths in this series were not related directly to the SIL, but to end-stage liver disease resulting from TPN-associated cholestasis. Among the survivors, adaptation to enteral feedings required 13.8 +/- 2.5 mo, during which time weaning from TPN occurred; weight at adaptation was 6.87 +/- 1.32 kg.(ABSTRACT TRUNCATED AT 250 WORDS)

SURGICAL MANAGEMENT OF DUPLICATIONS OF THE ALIMENTARY TRACT.

Abstract Duplications may be found along any portion of the alimentary tract. They may or may not communicate and share a common wall with adjacent bowel; they may be cystic, tubular or multiple, may involve several body cavities, may be associated with other anomalies and may lead to secondary complications. Proper surgical management requires a thorough knowledge of the anatomic varieties and various surgical technics that have been successfully used.

Management of esophageal atresia and tracheoesophageal fistula in the neonate with severe respiratory distress syndrome

In a 10-year period, 22 neonates with esophageal atresia (EA) and tracheoesophageal fistula (TEF) required high pressure ventilatory support soon after birth because of respiratory distress syndrome (RDS). Eleven of the 22 or 50% survived overall, but if the 5 patients who died before definitive surgical repair could be attempted are excluded, 11 of 17 or 65% survived. More importantly, 4 of 7 (57%) patients who had gastrostomy performed first survived while 7 of 10 (70%) who had fistula ligation performed first survived. The difficulties with intraoperative management of those who had gastrostomy performed first were even more impressive. Our experience leads us to conclude that patients with EA and TEF with severe RDS who require high pressure ventilation preoperatively represent a group of patients who require special consideration. The danger to such patients with increased pulmonary resistance is not gastric distention but sudden loss of intragastric pressure. In the presence of poor lung compliance, the upper gastrointestinal tract functions in continuity with the tracheobronchial tree. A sudden loss of intragastric pressure, as with placement of a gastrostomy tube, results in an acute loss of effective ventilating pressure. Resuscitation of such a patient is not possible until leakage from the esophagus is controlled by ligation of the fistula or transabdominal occlusion of the distal esophagus. Placement of a Fogarty catheter into the fistula via a bronchoscope is effective but may not be feasible in every case. Early thoracotomy and ligation of the fistula in patients with progressive RDS provides immediate improvement in ventilatory efficiency and relief of gastric distention.

Total intestinal aganglionosis: A new technique for prolonged survival

Total small bowel aganglionosis is uniformly fatal; and prolonged nutritional treatment for the resulting severe short bowel syndrome in the absence of a therapy designed to achieve a functional bowel length has not been warranted. We report an operative technique, long segment small bowel myectomymyotomy, which has produced a functioning length of intestine capable of supporting ever increasing amounts of enteral nutrition. A term female was noted to have neonatal intestinal obstruction, and two laparotomies proved total colonic and near-total small bowel aganglionosis. At 2 months of age reoperation was done and the aganglionosis was proved to extend to 7 cm below the ligament of Treitz. From this transition zone to 10 cm distally, a myectomy was done removing a 1 cm wide length of seromuscular tissue to the level of the submucosa. From the distal end of the myectomy, another 40 cm of bowel received an antimesenteric border myotomy cutting to the submucosal level followed by spreading of the cut surface to a width of 1 cm. This left the patient with 55 to 60 cm of small bowel from the ligament of Treitz to the end of the myotomy at which point an end ostomy was created. The remainder of the small bowel was excised and the colon exteriorized as a mucous fistula. The patient was continued on total parenteral nutrition alone for ten days at which time small volume enteral feeds were introduced. By 5 months of age, 25% of calories were enteral; by 6 months, 33% of calories were enteral; and by 8 months, 45% of intake was enteral.(ABSTRACT TRUNCATED AT 250 WORDS)

Mongolism with mosaic chromosome pattern

Summary A Mongoloid infant is presented with a mosaic chromosome pattern in both blood and skin. Unusual immune responses were also manifested by a delayed homograft reaction and by little response to infection. Approximately one third of the white blood cells in the mothers peripheral blood belonged to the male type. It is felt that this most likely represents a prenatal transfusion of male cells into the mothers circulation from the twin of the patient here described, but other mechanisms are also considered.

Primary hyperparathyroidism in infancy

Primary hyperparathyroidism in the neonate is a rare and often fatal disorder. These infants typically display severe hypercalcemia, respiratory distress, muscular hypotonia, and skeletal demineralization. They are usually diagnosed within the first three months of life and have hyperplasia of the four parathyroid glands. Twenty-nine infants with primary hyperparathyroidism are reported in the literature. Mortality is 87.5% in medically managed patients and 24% in surgically managed patients. Surgical management has not been satisfactory, in that recurrent hypercalcemia has been encountered in most patients undergoing subtotal parathyroidectomy, and total parathyroidectomy has resulted in the need for lifelong calcium and vitamin D supplementation. We have recently cared for a term newborn female in whom the diagnosis of primary hyperparathyroidism was made clinically on the second day of life, and later was confirmed biochemically. The baby underwent neck exploration on the 11th day of life and was successfully treated with total parathyroidectomy and parathyroid autotransplantation. Although initially rendered eucalcemic, the infant subsequently developed recurrent hypercalcemia requiring the removal of some of the autograft. Currently, the child is more than 2 years following surgery, growing well, and off all medication. The world literature is reviewed in this report of one of the first and the youngest infants, to our knowledge, to undergo parathyroid autotransplantation. In view of its success in avoiding the complication of repeated neck exploration for recurrent hyperparathyroidism or the creation of permanent hypoparathyroidism, we recommend this surgical approach for the rare neonate with primary hyperparathyroidism.

The usefulness of open-lung biopsy in the pediatric bone marrow transplant population

From October 1976 to October 1986, 126 children had bone marrow transplants at the Childrens Hospital of Philadelphia. The indications were acute lymphocytic leukemia (ALL) (30), nonlymphocytic leukemia (24), aplastic anemia (15), solid tumors (47), and miscellaneous conditions (10). Of these, 21 (17%) underwent 22 open-lung biopsies. Fourteen of these patients showed no causative microorganism. When a cause was found it was viral (usually cytomegalovirus [CMV]) in three, fungal in one, Pneumocystis carinii alone in two, both viral and pneumocystis in one, and a combination of viral, bacterial, and pneumocystis in one. Thirteen patients died due to continued deterioration after the biopsy. In only two patients was there a significant change in antimicrobial therapy as a result of the biopsy. Both had Pneumocystis (one in combination with virus and bacteria). One patient with chronic infiltrates showed a lymphocytic interstitial pneumonia, which responded well to steroids. Open-lung biopsy is currently of limited value in this patient population. Survival is dismal unless the patient has Pneumocystis. We believe that prospective studies should be set up to compare open-lung biopsy with empiric antimicrobial therapy. A major emphasis must be on prevention.

Surgical management of reflux strictures of the esophagus in childhood.

The etiology of gastroesophageal reflux (GER) in infancy is related to developmental factors, and there is a high incidence of associated conditions such as neurologic syndromes and esophageal atresia (60%). This is different from the situation in adults. Experience with 18 consecutive children with peptic esophageal strictures is reviewed to determine if conservative surgical management is effective. Eighteen children 14 months to 13 years (mean 6.3 years) of age took an average of 3.5 years from the time of onset of symptoms of GER to develop tight strictures diagnosed by esophagography and esophagos-copy. The incidence of stricture in patients with GER was approximately 15%. Preoperative dilatation or direct surgical management prior to correction of reflux is ineffective. All 18 children were managed by intraopcrative dilatation, Nissen fundoplication, and guided dilatation after operation. More aggressive surgical procedures were not required nor were associated operations such as pyloroplasty; they are rarely necessary. An average three-year follow-up indicates that this conservative surgical approach is effective in the management of peptic esophageal strictures in childhood with relief of symptoms and gratifying improvement in growth.