Big shunts for small patients with portal hypertension: a bit of history.

This is a brief resume of my personal experiences that led to the development of two portosystemic shunt procedures designed to be used in small patients with bleeding varices and portal hypertension. Both enable one to decompress the congested protal venous system in the preschool-aged group when about two thirds of such patients begin to suffer their first life threatening bleeding episodes.

Robert E. Gross.

Born in 1905, Gross graduated with honors from Carleton College and the Harvard Medical School. After spending 2 years in pathology, he entered his surgical training at the Peter Brent Brigham Hospital and at the Boston Children's Hospital with Dr. William E. Ladd, who occupied the first Chair of Pediatric Surgery in the United States. After 3 years of basic training with a wide variety of surgical problems in both adults and children, he decided to devote his considerable talents toward solving some of the problems of children with congenital malformations. After having returned to Harvard to assume the Chief Residency in Surgery at the Boston Children's Hospital, he worked out a surgical approach to the closure of the patent ductus arteriosus, and he performed the first successful ligation of this structure. Two years later, Gross co-authored with Dr. Ladd Abdominal Surgery of Infancy and Childhood. In the laboratory Gross was actively pursuing the treatment of anomalies of the heart and great vessels. With Dr. Charles Hufnagel, he developed a practical method of preserving, sterilizing, and using aortic homografts to bridge damaged aortic areas, and thus introduced modern reconstructive vascular surgery. In 1947 Gross was named Professor of Children's Surgery at Harvard Medical School and Surgeon-in-Chief of the Boston Children's Hospital. His contributions to the literature included the classic textbook Surgery of Infancy and Childhood. Gross was elected President of the American Association for Thoracic Surgery in 1964, and served as the first President of the newly formed American Pediatric Surgical Association in 1970.(ABSTRACT TRUNCATED AT 250 WORDS)

Esophageal sclerotherapy: an effective modality in children.

During the past five years, sclerotherapy has been used at our institution in 13 children for the management of recurrent major variceal bleeding. The varices were secondary to extrahepatic portal hypertension in seven patients and to intrahepatic portal hypertension in the remaining six. Sclerotherapy was performed under direct vision using either rigid or flexible endoscopic equipment, and the sclerosing agents were injected directly into the varices. The average age at initiation of sclerotherapy was 9 years (range: 1 to 19 years). The follow-up has ranged from 2 to 4 1/2 years with a mean of 3 1/2 years. Complete obliteration of all varices was obtained in eight of these patients. Two children have minimal residual varices, in one of whom 17 sclerotherapy procedures have been performed to date. One additional patient had a severe episode of bleeding during esophagoscopy, and transesophageal ligation of varices was required for control. Two patients have died following initiation of sclerotherapy. In neither case was the death the result of bleeding esophageal varices or a complication of endosclerosis. Bleeding from varices was the major clinical problem in all of these children, and this problem has been largely corrected by the sclerotherapy program. With one exception, there have been no episodes of variceal bleeding requiring transfusion in these patients following initiation of this therapy. One child developed an esophageal ulcer postinjection, but none have developed esophageal strictures. One patient developed an allergic reaction to the sclerosant that was treated during subsequent injections with prior administration of an antihistamine (diaphenhydramine chloride) and steroids.(ABSTRACT TRUNCATED AT 250 WORDS)

Carcinoma in a colon conduit urinary diversion.

In recent years urinary diversion by means of the colon conduit has gained popularity because of the failure to exhibit reflux and the lower incidence of stomal stenosis. However, colon conduit diversion may be associated with adenocarcinoma, as is ureterosigmoidostomy. We report the first occurrence of adenocarcinoma in a colon conduit and, perhaps more important, in a colon conduit in which there had never been a fecal stream.

Omphalocele: a prognostic classification.

A classification for infants born with an omphalocele is proposed that is based on the recognition of four syndromes and two common associated anomalies. The recognition of these syndromes and anomalies in our newborns with an omphalocele provided a better estimate of expected mortality and morbidity than the size of the omphalocele, preoperative rupture, delay in treatment, or low birth weight. This classification should aid the physician in determining priorities regarding the timing and type of treatment for the omphalocele itself.

The pediatric patient with sarcoma.

The results of the treatment of the commonest soft tissue sarcoma in childhood have improved dramatically during the past decade. The combination of reasonable surgery, radiotherapy and multiagent chemotherapy has been demonstrated to provide effective control of both the primary tumor and distant metastases in patients with rhabdomyosarcoma. Similar progress with the less common soft tissue malignancies will be more difficult to attain. Current therapeutic trends appear to favor a similar multimodal approach with a conservative but complete surgical excision combined with varying regimens of radiation and chemotherapy. Because of the infrequent occurrence of these unusual tumors (450 cases per year in the United States) no single institution can possibly attract a significant number of new cases to enter on treatment protocols. Substantial improvement in our current therapeutic techniques will be possible in the future only if our combined multicenter studies are continuously refined, re-examined, and extended. A plea is made for early referral of these difficult patients to regional study centers.

Successful spleno-renal shunt and splenectomy in two patients with alpha-1-antitrypsin deficiency.

Recent evidence suggests that the cirrhosis of alpha-1-antitrypsin deficiency is not invariably fatal as it was previously thought. Portal hypertension is often the major determinant of survival. The few reports of porta-systemic venous anastomosis in this disorder have shown poor results or uncertain outcome. Thus, doubts exist as to whether porta-systemic shunts should be performed in alpha-1-antitrypsin deficiency. Two patients with alpha-1-antitrypsin deficiency (PiZZ) and associated portal hypertension, cirrhosis, and hypersplenism underwent splenorenal shunt and splenectomy 8 yr ago, and both have done well. One of the patients has chronic severe headaches, diarrhea, exudative enteropathy, sinusitis, and hematuria, all uncommon in alpha-1-antitrypsin deficiency but possibly related to the antienzyme deficiency. She also has a higher trypsin inhibitory capacity than is generally reported in ZZ individuals. Based on the experience with these 2 patients, it appears that alpha-1-antitrypsin deficiency with cirrhosis is not a valid contraindication to the performance of a portasystemic shunt.

Calcium and phosphate requirements of preterm infants who require prolonged hyperalimentation.

Preterm infants require large amounts of calcium and phosphate to maintain skeletal mineralization during prolonged hyperalimentation. It is impossible to supply the amount of calcium and phosphate normally acquired transplacentally (150 mg/kg/day of calcium and 80 mg/kg/day of phosphate) with reasonable volumes of a single hyperalimentation solution of a physiological pH. We recently encountered calcium phosphate precipitates plugging our hyperalimentation catheters as we tried to raise the calcium concentration above 20 mEq of calcium per liter of hyperalimentation solution. Such a solution supplies 50 mg/kg/day of calcium when run at 125 mL/kg/day. In an attempt to circumvent this problem, we are currently using two hyperalimentation solutions--one high in calcium and the other high in phosphates--run alternately for 12-hour periods. This regimen supplies 80 mg/kg/day of calcium.

Appendiceal rupture: a continuing diagnostic problem.

The mortality rate for appendicitis in children has remained relatively unchanged since the 1940s, when antibiotics were introduced in the treatment of appendiceal peritonitis. However, since this time the incidence of appendiceal rupture has increased appreciably, presumably owing to a failure of early recognition and treatment. At Columbus Children's Hospital, one half of all patients undergoing appendectomy for ruptured appendix in 1975 had been seen by another physician before admission, but the correct diagnosis had not been made. The history obtained by the primary physician and that given on admission were similar, yet differed from the histories given by patients whose disease had been correctly diagnosed. Findings on in-hospital physical examination of incorrectly diagnosed patients differed from those recorded by the primary physician, but were similar to those of patients whose disease had been correctly diagnosed. Since it is unlikely that the natural history of the disease has changed, the increased incidence of rupture must result either from early misinterpretation of physical findings or from greater delay by parents in responding to the child's illness. Physicians and parents must share the responsibility equally for the increasing incidence of appendiceal rupture in children.

Reimplantation of the ampulla of Vater after total pancreatectomy for nesidioblastosis.

Total pancreatectomy is often required to control hypoglycemic seizures in infants with nesidioblastosis. If duodenal devascularization results, reconstruction is difficult because standard biliary-intestinal anastomoses may reflux, or, in infants, become strictured. Therefore, surgeons are loath to perform such procedures. To avoid these complications, the common bile duct and the ampulla of Vater were implanted into the reconstructed duodenum.

Reasonable surgery for thoracic neuroblastoma in infants and children.

Neuroblastoma is the most commonly encountered soft tissue malignant tumor of childhood. Over the past 30 years we have treated 180 patients with neuroblastoma. Sixty-five percent had primary abdominal tumors and 20 percent (41 patients) had primary chest tumors. For the 22 patients under the age of 2 years, the 2 year survival rate was 87 percent. There were 19 patients who were 2 years of age or older, and of these only seven patients have survived 2 years after the diagnosis was made. The vast majority of these patients were treated with surgery (debulking type procedure) and postoperative radiation and chemotherapy. Patients with the most differentiated tumors had a remarkably good survival rate, with no deaths. However, the tumors with lesser differentiation did not stratify enough focus to draw conclusions as to survival. Staging correlated the least with survival when compared to age or grading. The 2 year survival rates for patients with Stage I, II, III, IV, and IV-S disease were 75, 82, 100, 17, and 80 percent, respectively. In conclusion, 41 cases of documented primary thoracic neuroblastoma are reviewed, with follow-up from 2 to 27 years (average 9.3 years). We have concluded from this experience that age is the main determining factor influencing survival. Heroic and/or radical surgery is contraindicated in this disease.

Presinusoidal extrahepatic portal hypertension: a review of thirty-five cases variously treated.

Thirty-five cases of proven preinusoidal extrahepatic portal hypertension in children have been reviewed with particular reference to etiology, clinical course, therapy, and long-term follow up results. Thirty-three patients has significant gastrointestinal bleeding and 31 were operated upon. There were no operative deaths. Portosystemic venous shunts were successful in 12 of 21 patients. A variety of procedures were used for 15 of the 17 "unshuntable" individuals. Some form of esophagectomy and proximal partial gastrectomy with colon substitution appears to be the most effective operation for control of hemorrhage in the "unshuntable" patients. Expectant treatment is preferred in the infant and younger child, but the older child should have a definitive shunt or direct attack on the varices since recurrent bleeding is usually the rule.

Is the risk of morbidity and rare mortality worth the cure?

Many more children with cancer are being cured, often as a result of aggressive treatment regimens. These employ several modalities and multiple chemotherapeutic agents. The acute complications resulting from this type of treatment are relatively well known. Those that develop later are becoming better understood; some are formidable. The challenge in pediatric oncology lies in developing better treatment strategies with a view to minimizing normal tissue damage while achieving higher cure rates. The cooperative clinical trial mechanism is well-suited to testing new treatment regimens designed to achieve these goals.