Wilm's Tumor: An Update.

Major advances have been made against Wilms' tumor as a result of treatment methods developed by single institutions that then have been confirmed and extended by national cooperating groups. Better survival rates have been achieved, and therapy has been refined so that treatment can be reduced in early stage disease without jeopardizing tumor control. This results in fewer short- and long-term complications, an especially important consideration in children. Their organs and tissues are vulnerable to anti-mitotic treatments such as chemo- and radiotherapy, that can produce disabling if not lethal dysfunctions. This progress has been the result of the cooperative efforts by multiple specialists, and provides evidence of the value of such integrated studies. They have changed the outlook from a 90% death rate in the early years of this century to the 90% survival rate now possible with modern management.

Report of a case of gross thymic hyperplasia in a child.

A large thoracic mass and a mediastinal lymph node were excised from an infant with a peripheral blood and bone marrow lymphocytosis. The 224-mass was composed of histologically normal thymus, and the lymph node architecture was partially effaced. Hypogammaglobulinemia was detected two years after thymectomy. The enormous thumus in this case fits the classic gross pathologic definition of hyperplasia. The possibility of associated thymic hyperfunction in this case is discussed.

Nonoperative treatment of duodenal hematomata in childhood.

Blunt abdominal trauma, often apparently trivial in nature, may produce intramural hematoma causing complete duodenal obstruction. A child with abdominal pain, persistent vomiting and upper abdominal tenderness should be carefully questioned for a history of trauma. Prompt upper gastrointestinal x-rays will allow early and definitive diagnosis. Previously recommended treatment varies from immediate surgical intervention to observation followed by exploratory laparotomy at a later date. We have treated nine patients with duodenal obstruction caused by intramural hematoma of which eight were successfully managed with nasogastric suction and parenteral fluid administration. One patient had the hematoma evacuated at surgery. This report reviews our experience.

Congenital bronchobiliary fistula: management of the involved hepatic segment.

We present three children with congenital bronchobiliary fistula (CBBF). The first infant underwent resection of the fistula at age 3 weeks and was reported as a success. She returned 7 years later with bilious expectoration for which a second thoracotomy was performed. One year thereafter a bile collection in the right chest required open drainage. Subsequently, the left hepatic lobe was resected. The second child was initially diagnosed as suffering from gastroesophageal reflux. He underwent a fundoplication and a gastrostomy at age 9 months. Persistence of bilious sputum led to the diagnosis of CBBF at age 23 months. The fistula was excised through a right thoracotomy. The distal end was injected demonstrating an arborization involving the entire left hepatic lobe. Since there was no communication with the remaining biliary tree or the intestine, a left hepatectomy was performed. The third child was reported in a communication focusing on the diagnosis of CBBF by hepatobiliary scintigraphy. Her history is almost identical to the second patient. However, instead of a hepatectomy, she underwent a Roux-en-Y drainage of the fistula. The three children had uneventful recoveries and are well. We believe that an abnormal biliary tract should be studied by contrast injection. If no decompressing communication with the normal biliary ducts or the duodenum can be demonstrated, the involved hepatic segment should be resected or internally drained. Children who underwent simple fistula excision must have close long-term follow-up.

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.

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)

Pancreatic adenomas in infants and children: current surgical management.

Islet cell adenomas are an important consideration in infants and children with hypoglycemia due to hyperinsulinism. Between 1965 and 1977, 32 patients with hyperinsulinism were seen at the Children's Hospital of Philadelphia. Sixteen of these patients underwent surgery. Eight patients had single pancreatic adenomas, 5 of whom were infants under 1 yr of age. In 3 infants an adenoma was recognized and removed at the time of surgery. In 4 patients a subtotal pancreatectomy was successful in removing the adenoma. One infant is whom the adenoma was not included in the subtotal pancreatectomy died postoperatively with intractable hypoglycemia. Of the 7 patients cured by surgery, 6 are normal and 1 has brain damage due to delay in treatment of hypoglycemia. One child has mild diabetes. A combined medical and surgical protocol has been developed that permits rapid diagnosis of hyperinsulinism and selection of candidates for surgery. Infants under 1 yr of age who fail to respond to diazoxide should be explored. In children whose hyperinsulinism appears beyond 1 yr of age, surgery is indicated, since an adenoma is likely.

Massive gastrointestinal hemorrhage in children with posterior fossa tumors.

Over a 12-month period (September 1984 to September 1985), 64 children with newly diagnosed brain tumors were admitted to the Neurosurgical Service at The Children's Hospital of Philadelphia. Of these children, 29 had posterior fossa tumors. Of this population of children with posterior fossa tumors, three patients aged 4 months, 22 months, and 4 years old developed massive exsanguinating upper gastrointestinal hemorrhage within seven days of their primary neurosurgical procedure. In each instance, large posterior duodenal ulcers were encountered and were treated with oversewing of the duodenal ulcer and vagotomy-pyloroplasty. Follow-up currently ranges from 18 to 26 months. All three children have survived and none have had any gastrointestinal bleeding since then. Massive exsanguinating hemorrhage was not seen in children with brain tumors in locations other than the posterior fossa. In this population of patients, we advocate the use of prophylactic cimetidine and titration of gastric acidity with antacids.

Experience with hepatic hemangioendothelioma in infancy and childhood.

This report describes our experience with 16 infants and children with a mean age of 7 months and a median age of 6 weeks with hepatic hemangioendothelioma. Fifteen patients presented with hepatomegaly. Seven had congestive heart failure and four had associated cutaneous lesions. Although diagnosis was clinically evident in 15 of the 16 patients, arteriography and computerized tomography (CT) were diagnostic. A variety of treatment approaches were used including radiation, resection, systemic steroids, hepatic artery ligation, angiographic embolization, and various combinations of these modalities. The following information was gained from this experience. CT with enhancement is as specific a diagnostic tool as hepatic arteriography. Angiographic and CT appearances do not correlate with prognosis. Most patients can be treated successfully with steroids; those who do not respond should have other approaches tried such as embolization or ligation. The angiographic appearance determines whether embolization therapy is worthwhile, as we found that hemangioendotheliomatosis with portal as well as hepatic arterial supply will not respond to embolization. The survival rate in this series was 80%.

Survival in bilateral Wilms' tumor--review of 30 National Wilms' Tumor Study cases.

Bilateral tumors were found in 33 patients or 5.4% of 606 children enrolled in the National Wilms' Tumor Study. Twenty six of 30 patients (87%) for whom detailed clinical information is available survived 2 yr after conservative surgery and radiation therapy. All patients were given one or more chemotherapeutic agents. A variety of surgical procedures were used including biopsy only, nephrectomy, and partial nephrectomies. Radiation therapy doses ranged widely, but between 1000 and 2000 rad apparently sufficed for local control in most cases. Recommendations regarding management by surgery, radiation therapy, and chemotherapy are given.

Microdiverticulitis: a rare but distinct cause of small bowel obstruction in early infancy.

During a 20-year period, eight term infants admitted to a large children's hospital for treatment of dehydration associated with diarrhea (6) and vomiting (2), and who ranged in age from 10 to 60 days at onset of symptoms and 18 to 75 days at presentation, developed distal small bowel obstruction following apparently successful rehydration. With the exception of a single infant who was dead on arrival, and another whose obstruction went unrecognized, all infants came to operation shortly after obstruction was confirmed. In each case, a severely inflamed area of distal ileum (7) or proximal colon (1) was found at autopsy (2) or operation (6) to be the cause; perforation was present in four of the cases. Resection of the diseased segments of intestine, and primary anastomosis, were performed in all six operated cases; reoperation was required in four of the six for leaks (3) and adhesions (1). All but two survived. Pathologically, the resected intestinal segments showed a unique pattern of injury: numerous punctate ulcers were apparent, which undermined the muscularis mucosae, without evidence of necrosis. Regenerating epithelium extended through these defects, resulting in the presence of glandular invaginations, which were surrounded by a brisk inflammatory response: hence the term "microdiverticulitis." We believe this lesion represents a beginning or furtive attempt at repair of severely inflamed, but viable intestine, and that it is a rare but true cause of small bowel obstruction in early infancy, separate and distinct from necrotizing enterocolitis.

Partial splenectomy: the preferred alternative for the treatment of splenic cysts.

We report six pediatric patients who had a partial splenectomy for splenic cysts and a seventh patient who had a total splenectomy. There were six epidermoid cysts and one traumatic cyst. All patients had benign postoperative courses. All patients who underwent partial splenectomy had a significant postoperative rise in platelet count that subsequently returned toward baseline. This rise implies a loss of splenic function, although the exact meaning of this is unclear. All patients had late postoperative liver-spleen scans that showed mean spleen size and function to be normal. We have described several methods of partial splenectomy used at Children's Hospital of Philadelphia. Recommendations for patients undergoing partial splenectomy include preoperative pneumococcus and Hemophilus influenzae vaccinations and prophylactic antibiotics postoperatively. The antibiotics are terminated if a liver-spleen scan and platelet count at the end of 3 months' time are normal.

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.

Resolution of intractable cholestasis associated with total parenteral nutrition following biliary irrigation.

Although the protracted use of total parenteral nutrition (TPN) in infants is known to predispose to cholestasis, which in certain instances may not be reversible, failure to understand the pathogenesis of this condition has hindered the development of an effective medical treatment. That surgical treatment may reverse this process in selected patients is the subject of the present report. Two premature infants and one term infant, with short bowel syndrome acquired in infancy, developed conjugated hyperbilirubinemia following institution of balanced TPN. Infectious and metabolic etiologies for the jaundice were ruled out. No excretion of 99mTc-labeled DISIDA from the biliary tree was demonstrable 24 hours following injection by nuclear scintigraphy, despite the absence of extrahepatic biliary obstruction by abdominal sonography. When all conventional medical methods of managing the problem failed, exploration was performed. Intraoperative cholangiography showed normal intrahepatic and extrahepatic biliary ducts in each instance. Irrigation of the biliary tree with normal saline solution was then performed. In all cases, liver biopsies were obtained that were consistent with TPN-associated cholestasis and in all cases, jaundice resolved promptly following operation. We conclude that intractable cholestasis associated with TPN, regardless of its etiology, is a functional abnormality of biliary excretion that may respond in selected patients to irrigation of the biliary tree with radiographic dye and/or normal saline solution.

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)