Robert M. Filler

Liver tumors in children in the particular reference to hepatoblastoma and hepatocellular carcinoma: American Academy of pediatrics surgical section survey — 1974

A review of 227 cases of hepatoblastoma, hepatic cell carcinoma in children seen in the United States over a 10-yr period is presented. Both tumors were seen most commonly in infancy, but the hepatocellular carcinoma shows a second peak of incidence around puberty. Males predominated in both diseases more so in hepatoblastoma. Presenting symptoms in both diseases were very similar, most commonly an upper abdominal mass or abdominal enlargement associated with anorexia and weight loss. In the preoperative evaluation the presence of alpha-feto protein was one of the most helpful diagnostic tests. Disturbances of liver function were usually mild but were more marked in those children with hepatocellular carcinoma. Preoperative x-rays were abnormal in a large percentage of cases with the hepatic arteriogram and vena cavagram being the most useful diagnostic x-rays for liver tumors. Liver scans were positive for liver tumor in 95% of the children when this test was carried out. The follow-up for these patients ranged from 2 to 10 yr. The size of the primary tumor did not appear to correlate with survival but bilateral location of the tumor, 33% in hepatoblastoma and 45% in hepatocellular carcinoma, made many of these tumors inoperable. Multicentric tumors were also found in a large number of patients, being more common in hepatocellular carcinoma. There was a high rate of local recurrence or local extension after operation in both diseases, and metastatic spread was similar being most common to the lungs and abdomen. A wide variety of surgical procedures were carried out in these patients from biopsy only to extended hepatic lobectomy. When incomplete excision or biopsy only was carried out no patient survived in either group. Among the hepatoblastoma patients, 45 of 78 patients who had complete excision are surviving. In the hepatocellular carcinoma patients where the operability rate was much lower 12 of 33 patients are surviving when tumor was completely excised. Complications were frequent, the most common being excessive blood loss at operation. There were eight operative deaths and 17 postoperative deaths in the combined group. There was no evidence that radiation therapy or chemotherapy controlled disease which could not be completely excised surgically. The only direct evidence of a favorable effect of radiation and chemotherapy were three cases of hepatoblastoma in which the tumor changed from inoperable to operable by a combination of radiation therapy and multiple drug chemotherapy. Both tumors are highly malignant, and 90% of the children who died of hepatoblastoma died within 12 mo of diagnosis. In the hepatocellular carcinoma 80% of the deaths occurred within 1 yr of diagnosis. At this time it seems that operative excision offers the only chance of cure in children with these tumors and cure rates of 60% can be expected with hepatoblastoma and 33% in hepatocellular carcinoma if the tumor can be completely excised.

Congenital diaphragmatic hernia—A tale of two cities: The Toronto experience☆

PURPOSE The optimal therapy for congenital diaphragmatic hernia (CDH) is evolving. This study analyzes the results of treatment of CDH in a large tertiary care pediatric center using conventional and high-frequency oscillatory ventilation (HFOV) without extracorporeal membrane oxygenation (ECMO) contrasting these with a parallel study from a similar large urban center using conventional ventilation with ECMO. METHODS Between 1981 and 1994, 223 consecutive neonates who had CDH diagnosed in the first 12 hours of life were referred for treatment before repair. Conventional ventilation was used with conversion to HFOV for refractory hypoxemia or hypercapnia, and a predicted near 100% mortality rate. ECMO was used in only three patients, all of whom died. A retrospective database was collected. Thirty-one clinical variables were tested for their association with the outcome. Common ventilatory and oxygenation indices were tested for their prognostic capability. RESULTS Apgar scores, birth weight, right-sided defects, pneumothorax, total ventilatory time, and the use of high frequency oscillatory ventilation were the only variables associated with outcome. A modified ventilatory index and postductal A-aDo2 were strong prognostic indicators. From 1981 to 1984 surgery was performed on an emergency basis. Since 1985 surgery was deferred until stabilization had been achieved. This resulted in a shift in the mortality from postoperative to preoperative with no change in total survival. HFOV did not alter the overall survival. Results of autopsies performed (70%) showed significant pulmonary hypoplasia and barotrauma as the primary causes of death. The survival was 54.7%. CONCLUSION Conventional ventilation with HFOV produced equal survival to conventional ventilation with ECMO in two comparable series. Pulmonary hypoplasia was the principle cause of death. This continued high mortality at both centers suggests that new therapies are required to improve outcomes.

Long-Term Total Parenteral Nutrition in Infants

Abstract An intravenous amino acid, hypertonic glucose, electrolyte and vitamin solution was used in 14 infants under two months of age in whom oral intake was completely withheld for as long as 60 days. Included were infants with complicated intestinal obstruction, bowel fistulas, intraperitoneal sepsis and chronic diarrhea, as well as three with prenatal rupture of an omphalocele. In the past, a high mortality has been observed in such critically ill infants. In this series, all infants survived. Studies of metabolic balance revealed that the intravenous mixture was capable of maintaining satisfactory nutrition in spite of repeated surgical procedures, sepsis and enteric losses. No serious glycosuria, osmotic diuresis or adverse side effects were detected.

Complications of indwelling venous access lines in the pediatric hematology patient : a prospective comparison of external venous catheters and subcutaneous ports

Since 1984, 316 subcutaneous ports (SP) and 339 external venous catheters (EC) [Roko Catheter, The Hospital for Sick Children (HSC)] have been inserted in hematology/ oncology patients at HSC. During a 22-month period (July 1987 to April 1989), a committed central line nurse (J.I.) prospectively collected clinical and microbiologic data on 144 consecutive SPs and 130 consecutive ECs. Children with the SP had 0.6 infected lines and 0.7 infectious episodes per 1,000 patient days compared to 2.9 infected lines and 4.3 infectious episodes per 1,000 patient days with the EC (p < 0.001). This lower infectious complication rate with SP was demonstrated in the entire group of unselected patients and in a cohort of children with acute lymphoblastic leukemia (ALL) receiving intensive chemotherapy, and it was evident in all age groups. In view of the other advantages of SP–normal activity, absence of the need for home maintenance, improved body image, less expense–these data suggest that SPs are the preferred device in pediatric patients and provide effective venous access with acceptable complication rates.

A cross-sectional study of catheter-related thrombosis in children receiving total parenteral nutrition at home

We performed a cross-sectional evaluation of deep vein thrombosis (DVT) related to the use of central venous lines (CVLs) in all pediatric patients receiving home total parenteral nutrition at our institution (N = 12). All children (5 months to 17 years of age) were examined with bilateral upper limb venography. All CVLs were flushed daily with heparin (200 units). At the time of evaluation, 49 CVLs had been placed in the 12 children. Of the 39 CVLs removed, 27 (66%) were blocked; venograms had not been previously obtained except of one child. Eight children had clinical evidence of superficial collateral circulation in the upper portion of the chest and the upper extremities; five had intermittent symptoms of superior vena cava obstruction. On venography, 8 of the 12 children had extensive evidence of DVT; two were unilateral and six bilateral. Five children were treated with warfarin (0.12 to 0.28 mg/kg per day) to achieve an international normalized ratio of 1.4 to 1.8. Neither bleeding nor further CVL-related DVT has occurred. We conclude that the risk of CVL-related DVT in children requiring home total parenteral nutrition is high, and that venography should be performed early in the event of CVL blockage. A multicenter, controlled trial assessing optimal warfarin therapy in this patient population is indicated.

The use of expandable metallic airway stents for tracheobronchial obstruction in children

Expandable metallic angioplasty stents (Palmaz stent) have been implanted in the trachea and/or bronchi of seven children. Three children had severe tracheal stenosis after tracheoplasty for congenital tracheal stenosis repair, and four had tracheomalacia or bronchomalacia with or without vascular compression. The mean age at stenting was 9.7 months (range, 2 to 15 months). Balloon expandable stents were inserted into the trachea or bronchus through a 3.5-mm bronchoscope under fluoroscopic control. Initially a single tracheal stent was used for all patients except for one with obstruction in the trachea and both bronchi, in whom three stents were implanted. Three children had recurrent airway obstruction 1 month later; one was cured with a second stent; one child died 1 year later; and the other is being treated for heart disease. The others have no serious respiratory problems. The stents in all have been in place for 1 to 25 (mean, 11) months. No immediate complications were noted. Early and late bronchoscopy showed incomplete epithelialization of the stent and patches of granulation tissue on it. Two stents were removed bronchoscopically, one at the completion of treatment for tracheomalacia and the other at the time of recurrent airway obstruction. This preliminary experience indicates that expandable metallic stents have a useful role in the treatment of selected lower airway obstructions.

Timing of surgery for congenital diaphragmatic hernia: Is emergency operation necessary?

Congenital diaphragmatic hernia (CDH) is considered by most researchers to be a surgical emergency. However, early repair does not necessarily improve respiratory function or reverse fetal circulation, and many patients deteriorate postoperatively. As a result, in 1985, we began to employ a protocol in which surgery was delayed until the PCO2 was maintained below 40 and the child was hemodynamically stable; children in whom these criteria could not be achieved died without surgical repair. Sixty-one consecutive infants with CDH were managed over 4 years; 31 from 1983 to 1984 (group 1) and 30 from 1985 to 1986 (group 2). The groups were similar with respect to sex, side of the defect, birth weight, gestational age, incidence of pneumothorax, and blood gases. High frequency oscillation was used with increasing frequency during the study period, for patients with refractory hypercarbia (13% in group 1, 30% in group 2). All patients were initially paralyzed and ventilated. Mean time from admission to surgery was 4.1 hours in group 1 and 24.4 hours in group 2 (P less than .05). In group 1, 87% of patients had surgical repair (77% within eight hours of admission, 10% after eight hours), and in group 2 only 70% of patients had surgery (10% within eight hours, 60% after eight hours). All patients who were not operated on died. Overall mortality was 58% in group 1 and 50% in group 2; this difference was not statistically significant. These data indicate that our current approach has not increased overall mortality.(ABSTRACT TRUNCATED AT 250 WORDS)

A 20-year review of pediatric pancreatic tumors

Pancreatic tumors are rare surgical problems in infants and children. A 20-year audit (1971 to 1991) of this institution showed six patients ranging in age from 3 weeks to 16 years who were operated on for pancreatic neoplasms. Five of these tumors were malignant, bringing the reported experience to 71 cases. This series of malignancies included three solid cystic tumors, one insulin-secreting tumor, and one pancreatoblastoma. The clinical presentations varied: three had abdominal pain, one developed hypoglycemia, and one had an abdominal mass with jaundice. In five of the six patients pancreatic pathology was suspected preoperatively. All were treated primarily with pancreatic resection including one pancreatoduodenectomy. No radiotherapy or chemotherapy was used. The perioperative mortality was 0% with a morbidity of 50%. The long-term results are encouraging, with all patients alive after a mean follow-up of 7.8 years. These data suggest that aggressive surgical therapy is warranted in the management of pediatric pancreatic tumors.

Validation of Doubly Labeled Water for Assessing Energy Expenditure in Infants

ABSTRACT: Previous studies show that the doubly labeled water method is accurate for measuring energy expenditure in the adult human. To validate this method in infants, carbon dioxide production rate and energy expenditure were measured for 5 to 6 days by doubly labeled water (DLW) and periodic open circuit respiratory gas exchange (RGE) in 10 blinded studies in nine infants following abdominal surgery. Infants were maintained on consistant oral or parenteral nutrition prior to and during study. This avoided diet-related changes in baseline isotopic enrichment of body water which could theoretically contribute to significant errors in calculation of carbon dioxide production rate. For DLW, insensible water loss was assumed to be proportional to respiratory volume and body surface area, where the former was predicted from carbon dioxide production rate. Insensible water loss thus calculated averaged 18% of water turnover. Rates of carbon dioxide production measured by DLW were not significantly different from that of RGE (10.4 ± 1.1 and 10.5 ± 0.9 1/kg/day, mean ± SD, respectively). Energy expenditure was calculated using respiratory quotients from dietary intake (DLW:DIET) and RGE (DLW:RGE) data. There was no significant difference between energy expenditure determined by DLW (DLW:DIET and DLW:RGE) and that measured by RGE (58.5 ± 6.1, 56.8 ± 6.1, and 57.3 ± 5.1 kcal/kg/day, mean ± SD, respectively). Rate of carbon dioxide production, DLW:diet, and DLW:RGE calculated by DLW differed from corresponding RGE values by - 0.9 ± 6.2, -1.1 ± 6.1, and 1.6 ± 6.2%, mean ± SD, respectively. These findings demonstrate the validity of the doubly labeled water method for determining energy expenditure in infants without concurrent water balance studies.

Ruptured spleen—When to operate?

Sixty-three patients with splenic injuries were treated during a 5-yr period from 1974-1979. The decision to operate was based on the patients clinical course, not on the presence of splenic injury alone. Those who were stable on admission or after initial resuscitation were treated nonoperatively. This consisted of strict bed rest, nasogastric suction, and i.v. fluids--including blood--as required. Those who bled massively were operated on promptly. At operation, the spleen was repaired if possible or excised if damaged beyond repair. Forty patients were treated nonoperatively. Sixteen of these required blood transfusions (mean 31.2 +/- 5.3 ml/kg). One patient in this group developed a large defect on spleen scan at 3 wk post injury. There was no other morbidity and no mortality following nonoperative treatment. Nineteen required operation all within 16 hr of admission. Fifteen underwent splenectomy, 2 partial splenectomy, and 1 splenorrhaphy. In 1 the bleeding had stopped. All required blood before operation (mean 80.4 +/- 10.1 ml/kg). Seven in this group died (6 from head injuries and 1 from bleeding). Thus surgery was avoided in 2 out of 3 and the spleen saved in 3 out of 4 patients with documented splenic injuries. We believe that where adequate facilities exist nonoperative treatment of splenic injuries is both safe and effective. When bleeding is massive from the beginning or replacement requirements exceed 40 ml/kg, operation is indicated.