Sacrococcygeal teratoma: A 33-year experience

During the years 1941 through 1973, 48 patients, 16 males and 32 females, with sacrococcygeal teratoma were seen at the Childrens Hospital of Los Angeles. Forty-four patients have been followed, three are lost to follow-up, and one patient died 2 wk after excision of teratoma.Of the 44 patients with follow-up, 26 had teratoma with mature tissues only, all these patients are living. Six patients had tumor containing mature and embryonic tissues. Of these, five are living and one died with metastases of malignant teratoma 1 yr after excision of the primary tumor. Of the remaining 12 patients, 11 have died during the first 4 yr of life due to malignant teratoma and only one is living without recurrence 15 yr after excision of teratoma containing frankly neoplastic tissues. Recurrence and/or metastasis of malignant sacrococcygeal teratoma was lethal in all instances.     

Ovarian cysts and timors in infancy and childhood.

Cysts and tumors of the ovary are not common in children. At the large children's hospitals only one to four patients are seen annually with ovarian enlargement.^1–11 This paper summarizes our experience with 99 patients with ovarian cysts or tumors seen at the Childrens Hospital of Los Angeles during the years 1951 through 1973.     

High-calorie parenteral alimentation: serum lactic and pyruvic acid levels in immature dogs during infusion with concentrated glucose solutions

Solutions of glucose of varying concentrations (10%–25%) were administered through central venous catheters to immature dogs in volumes greater than those employed in clinical total parenteral infusion therapy. Blood lactate and pyruvate levels were not raised during the course of the infusion, irrespective of the glucose concentration employed. Acidosis was not produced by these infusions. Glucose concentrations of approximately 20% were utilized most effectively, as demonstrated by total glucose retention. Clinical lactic acidemia is probably not adversely affected by continued infusion of high concentrations of glucose.     

The stomach after cholecystectomy

Removal of the gallbladder results in an accelerated enterohepatic circulation of bile salts. It has been suggested that reflux of bile into the stomach after cholecystectomy may predispose a patient to the development of gastric lesions. To examine this hypothesis, 349 patients with endoscopically proved gastric ulcers or mucosal lesions were age and sex matched with 349 patients with upper gastrointestinal symptoms who had normal endoscopic findings. The frequency of cholecystectomy was determined to be 4.3 percent in the gastric ulcer group (control 3.7 percent) and 3.8 percent in the mucosal lesions group (control 4.2 percent). There was no statistical difference between patients with gastric ulcer and the control subjects, mucosal lesions and controls in the frequency of cholecystectomy using individually matched case-control comparison. The relative risk for gastric ulcer-matched control comparison was 1.3 (95 percent confidence limit 0.3 to 6.3). The relative risk for mucosal lesion-matched control comparison was 0.85 (95 percent confidence limit 0.2 to 2.9). The data did not support the hypothesis that cholecystectomy increases the propensity to develop gastric lesions.     

Hepatic “intravenous fat pigment” in infants and children receiving lipid emulsion

In 23 patients who received fat emulsion (Intralipid) intravenously and had subsequent necropsy, the deposition of pigment in the liver was evaluated quantitatively. Pigment was found in hepatic cells (HC) in 14 of 23 patients as well as reticuloendothelial cells (REC) in 22 of 23 patients. There was more pigment deposition in HC in younger children. HC pigment deposition occurred most frequently in patients with acute inflammatory processes within the abdomen. Patients infused with emulsion at rates less than 0.5 g/kg body wt/hr had less pigment deposition than patients infused at faster rates. Neonates infused at rates less than 0.2 g/kg body wt/hr for 24 hr had less HC pigment than neonates infused at higher rates. The quantity of REC pigment bore no apparent relationship to age, clinical diagnosis, rate or total dose of fat emulsion, but was increased in groups infused more than 14 days.     

Omphalocele and gastroschisis. Trends in survival across two decades

During the decade from July 1970 through June 1980, 57 patients with omphalocele and 64 with gastroschisis were treated at the Childrens Hospital of Los Angeles. Among the patients with omphalocele, the mortality was not significantly different between those with an abdominal wall defect smaller than 4 cm (5 of 24 patients) and those with a larger defect (6 of 33 patients); between those with a birth weight of less than 2,500 g (3 of 13 patients) and those with a higher birth weight (8 of 44 patients); between patients who had part of their liver in the omphalocele sac (6 of 29 patients) and those who did not (5 of 28 patients); and between patients who had primary fascial closure of the abdominal wall defect (3 of 24 patients) and those who had staged closure (4 of 25 patients). The overall mortality of 19 percent (11 of 57 patients) is not significantly different from that seen in patients treated during the preceding decade, 1960 through 1970 (23 percent, 5 of 22 patients), in our institution. Major chromosomal and other associated anomalies adversely affected the survival rate in these patients. In contrast, the overall survival rate of gastroschisis patients has markedly increased over the past two decades (91 percent in 1975 to 1980). In these patients, the difference in survival between those who had primary fascial closure (73 percent) and those who had staged closure by skin flaps or silon chimney (81 percent) was not statistically significant. Prematurity, bowel complications, and candida septicemia associated with the use of total parenteral nutrition contributed to the mortality.     

Congenital tracheoesophageal fistula without esophageal atresia: A 22 year experience

Experience in managing the patients with congenital tracheoesophageal fistula without esophageal atresia led to the following conclusions: (1) The pulmonary and gastrointestinal manifestations of this lesion may be subtle and similar to those of other disorders. A high index of suspicion is required to recognize the fistula. (2) Skillful and persistent efforts should be made to establish the diagnosis and localize the fistula before any surgical attempt at correction. (3) Adequate preoperative preparation, cannulation of the fistula and careful surgical dissection will minimize surgical complications.     

Improved survival in infants and children with primary malignant liver tumors

Forty-six infants and children with primary malignant liver tumors were studied. Thirty-three had hepatoblastomas, 9 hepatocellular carcinomas, and 4 had sarcomas. Thirty-eight patients underwent liver biopsy or excision of the tumor. Nineteen patients were treated between 1952 and 1971 (mean survival 5.5 months) and the remaining 19 were treated between 1972 and 1981 (mean length of survival 30 months, 2 year survival rate 53 percent). In 21 patients with an unresectable tumor, biopsy alone was performed (mean survival 7 months). Ten patients underwent primary resection of their liver tumor (mean length of survival 23 months, 2 year survival rate 37.5 percent). Seven other patients with an unresectable tumor were treated with chemotherapy alone or in combination with radiotherapy for a mean period of 6 months before second-look celiotomy was carried out. Six of these patients (all treated after 1975) had significant reduction in the size of their tumors. Mean length of survival in this group was 4 years 2 months, and the 2 year survival rate was 100 percent; at present, 5 of these patients are living without the disease 2.5 to 7 years after diagnosis. Improved techniques in liver resection and chemotherapy before second-look celiotomy is undertaken for an unresectable lesion have improved prognosis in children with a malignant liver tumor.     

Benign and malignant ovarian tumors in children and adolescents: A review of 63 cases

Sixty-three patients (15 months to 17 years of age) with ovarian tumors were seen. Fifty-six patients had germ cell tumors and 7 epithelial tumors, 6 patients had bilateral tumors, 47 patients had teratomas (41 benign, 3 with embryonic tissue, and 3 malignant), 6 germinomas, 1 endodermal sinus tumor, and 2 mixed germ cell tumors. Of the patients with epithelial tumors, six had cystadenomas and one cystadenocarcinoma. Abdominal pain was the most common symptom, and an abdominal mass the most common sign. A calcification on abdominal films was seen in 29 patients with benign teratoma. At surgery, the opposite ovary was bivalved or biopsied in 31 patients with benign tumors. Two patients had teratomas burried in the ovarian tissue. Among 47 patients with benign tumors, 37 have remained well after the operation and 10 were lost to follow-up. Two girls with embryonic teratoma are well 71 and 19 months after diagnosis but the third died with embryonal carcinoma. Of the 13 patients with malignant tumors, 8 are alive disease free $\text{4}\text{1}\text{2}$ to 17 years after diagnosis, 4 are dead, and 1 is lost to follow-up (disease free 15 months after diagnosis).     

Intact omphalocele. Perennial dilemma of operative or "conservative" management

A critical analysis of twenty-seven patients with intact omphalocele is presented. Surgically correctable intra-abdominal anomalies were found in ten patients. Among these ten, three (11 per cent) had associated diaphragmatic hernia, four (14.8 per cent) had atresia of the small bowel, and seven (25.9 per cent) had malrotation with associated congenital duodenal bands.It appears that in patients with intact omphalocele, life-threatening, but surgically correctable, intra-abdominal anomalies occur frequently enough to warrant abdominal exploration and concomitant correction.     

Aldosteronoma in a child with localization by adrenal vein aldosterone: collective review of the literature.

Hyperaldosteronism due to aldosteronoma is a rare but potentially curable form of pediatric hypertension. We have presented a patient who had symptoms of enuresis and fatigue, and in whom the diagnosis was suggested by low serum potassium and persistent hypertension. Diagnosis was confirmed by increased plasma and urinary aldosterone and decreased plasma renin. The tumor was localized with the aid of adrenal venography and catheterization, which showed greatly increased plasma aldosterone levels in the right adrenal vein. The pathologic findings were totally reversed by right adrenalectomy. The clinical picture and results following surgical removal of aldosterone-producing tumors in six children are reviewed.     

Surgical closure of patent ductus arteriosus in the premature infant with respiratory distress

During the period from February, 1971 to February, 1973, 30 premature infants underwent surgical ligation of patent ductus arteriosus. The gestational ages ranged from 25 to 36 wk (mean 30), and the birth weights ranged from 760 to 2010 g (mean 1274). The patients were divided into two groups on the basis of the indications for assisted ventilation. Group I consisted of 21 patients with severe hyaline membrane disease who required assisted ventilation during the first 2 days of life and could not be weaned off the respirator by 10 days of age. Group II was composed of nine infants who required intermittent positive-pressure breathing after a mean age of 8 days because of repeated apneic spells secondary to uncontrollable heart failure. All infants in Group II survived the operation and left the hospital well. Fourteen of the 21 patients in Group I survived; the seven deaths were all due to underlying severe pulmonary disease (bronchopulmonary dysplasia). The value of PDA ligation in premature infants with uncontrollable heart failure is demonstrated in this study; this procedure also appears to be beneficial in neonates with severe respiratory distress syndrome.     

The acceleration of hepatic regeneration by prior laparotomy

Laparotomy, carried out 6 hr prior to partial hepatectomy, accelerates the initial rise and first peak of DNA synthesis by approximately 3 hr and the second peak by approximately 6 hr in the rat model. The effect is most striking in the 16-hr postresection liver in which [³H]thymidine incorporation in animals stressed by prior laparotomy was >25 times that found in unstressed animals. These studies suggest that an initial phase of the response to hepatic resection may be initiated by nonspecific trauma which is of sufficient magnitude and administered at an appropriate interval prior to hepatectomy.     

Esophageal atresia and tracheoesophageal fistula: 1939 to 1979

During the past 40 years, the survival of patients with esophageal atresia and tracheoesophageal fistula has increased from zero to approximately 85 percent. With the increased survival rate, the surgeon has the opportunity to follow up the patient into adult life. By so doing, the early and late complications can be anticipated and treated appropriately.     

Modalities of preservation of the traumatized spleen.

Postsplenectomy septicemia has been seen to occur not only in children but in adults as well. The reason splenectomy is still practiced when the spleen is traumatized is the fear of development of subcapsular hematoma leading to delayed rupture and massive bleeding. The aim of therapy at present is early recognition of the type of splenic injury by abdominal paracentesis, radioisotope scan, ultrasound, and/or arteriography. Four alternative methods of management are presented and discussed that lead to the preservation of splenic tissue in patients with injured spleen.