Thomas V.N. Ballantine

Operative management of intestinal atresia and stenosis based on pathologic findings

This report describes the individualized operative therapy in 75 patients with intestinal atresia or stenosis based on pathological findings (1972–1978). Location of obstruction was duodenal in 30, jejunoileal in 38, and colonic in 7. Duodenal atresia was noted in 23 infants and stenosis in 7. Associated anomalies were observed in 23 patients, prematurity in 11, polyhydramnios in 11, and Downs Syndrome in 10. Operative treatment included duodenoduodenostomy (DD) in 19 patients, duodenotomy and web excision in 9, and side-to-side duodenojejunostomy (DJ) in 2. Both (DJ) cases needed revision to (DD) because of obstruction. A gastrostomy was used in all cases. Jejunoileal atresia occurred in 33 patients and stenosis in 5. Operations included wide proximal resection and endto-end anastomosis in 18 patients with normal bowel length, minimal resection with antimesenteric tapering enteroplasty (using autostaples) and anastomosis in 9 patients with foreshortened bowel, and resection and double barrel enterostomy in 6 with peritonitis or questionable bowel viability. Seventy percent received total parenteral nutrition (TPN). Six patients with colon atresia and one infant with stricture of the rectosigmoid survived initial colostomy and subsequent anastomosis at 3 to 6 mo of age. Survival for duodenal cases was 84%, jejunoileal 90%, and colonic 100%. Individualizing the operative management according to pathologic findings and TPN support when indicated improve operative survival in infants with intestinal atresia.

Juvenile secretory carcinoma of the breast

Juvenile secretory carcinoma of the breast, a rare tumor in infants and children, has an unusual histological appearance and clinical behavior. Isolated case reports and small series have appeared since this lesion was first described by McDivitt and Stewart in 1966. Our case of a 3-year-old boy with axillary metastasis and 17 cases in children, from the literature, provide the basis of this review. Of the 18 patients, there were three boys and 15 girls. Their ages ranged from 3 to 17 years (mean 9.8 years). All patients presented with an asymptomatic mass in the breast. None had nodes which were clinically involved. Eight patients had excisional biopsy only. Two patients had quadrantectomy. Four had simple mastectomy; one of whom received postoperative axillary irradiation, and one of whom had axillary nodal sampling (our patient). One patient had a modified radical mastectomy and three had radical mastectomy. Two of eight children who had excisional biopsy alone developed local recurrences. In the first patient, the recurrences occurred at 2 and 8 years following initial therapy. In the second, they occurred at 4 and 21 years. Axillary nodal metastases were found in three of the six patients in whom nodal biopsies were performed. In only one patient were estrogen receptors measured and they were negative. No deaths have been reported in children during a follow-up period ranging from 0 to 22 years (mean 6.5 years). Secretory carcinoma of the breast in this group of patients appears to be a slow growing, locally recurring malignancy. Adults with histologically similar tumors also have a good prognosis. Excisional biopsy is probably inadequate therapy.(ABSTRACT TRUNCATED AT 250 WORDS)

Reversal of Protein-energy Malnutrition in Children During Treatment of Advanced Neoplastic Disease

The effectiveness of enteral and parenteral feeding in supporting a satisfactory nutritional status and/or reversing protein-energy malnutrition was evaluated in 28 children, ages 1-19 (14 female) with advanced malignant disease (21 solid tumors, 7 leukemia-lymphoma). At the onset of treatment, 21 patients received intensive nutritional counseling (INC) and oral supplementation while seven received total parenteral nutrition (TPN). Sixteen of 21 patients who received INC had a decreased intake (x 48 +/- 24%) Recommended Dietary Allowances (RDA) for kilocalories and dramatic weight loss (x 16.4 +/- 12.4%). A total of 18 patients received TPN for a mean of 24 days (7-60); kcal averaged 90 +/- 26% RDA during weight gain. At onset of TPN, the mean serum albumin, transferrin and total lymphocyte counts were 3.06 +/- 0.38 g/dl, 175 +/- 62 mg/dl, and 1102 +/- 966/mm3 respectively, 15/18 children had subnormal anthropometric measurements and 17/18 patients were anergic to recall skin test antigens. TPN for less than 9-14 days neither repleted weight, skinfold reserves, nor serum albumin concentrations (greater than 3.2 g/dl) although an early increase (p less than .02) in transferrin concentration was observed. However, TPN for 28 days supported weight gain (3.27 kg, 16 +/- 6%), increased serum albumin (0.62 +/- 0.43 g/dl, p less than .001) and transferrin (62 +/- 42, p less than .002) to normal concentrations and reversed anergy in 7/11 patients retested. This study documents the severity of protein energy malnutrition which accompanies intense treatment of children with cancer and the nutritional and immunological benefits of a 28 day course of TPN.

Metastatic neuroblastoma: Factors influencing survival

Ninety of 142 patients (63%) with neuroblastoma had metastatic disease at the time of diagnosis. Seventy-four (52%) patients had stage IV disease and 16 (11%) had stage IV-S. Survival in stage IV-S was 75% (12/16). Four deaths occurred in infants under 6 wk of age, three of whom had bone marrow involvement. Deaths were related to respiratory insufficiency and sepsis rather than progression of disease. All patients over 6 wk of age survived with resection of primary tumor and skin metastases. Survivors had slow, spontaneous regression of tumor over a 6–15 mo period regardless of treatment. Bone marrow involvement with tumor reduces the otherwise favorable outlook for patients with stage IV-S disease. This suggests that patients with bone marrow metastases be excluded from stage IV-S classification. Of 74 patients with stage IV disease, 49 were boys and 25 were girls, with a mean age of 37 mo. Site of primary tumor was adrenal in 40 patients, paraspinal in 27, and mediastinal in seven. Prior to 1965, the mean survival was 3 mo. Since that time, with chemotherapy programs, the mean survival is 20 mo with 18% of patients surviving more than 2 yr. While there is little objective evidence that chemotherapy increases the cure rate, it can reduce tumor size, cause histologic maturation of tumor, and result in considerable palliation. There are six survivors (8%) disease-free longer than 2 yr. Two were under 13 mo of age and three over 6 yr. All had delayed primary or second-look tumor resections, and five had bone marrow involvement, but only two had bone cortex metastases. These observations suggest that resection of bulk tumor and histologic evidence of tumor maturation, particularly in patients with only bone marrow involvement, may result in an occasional survival. These data further suggest a modification in the staging of metastatic cases into three groups: stage IV-M, bone marrow involvement only; stage IV-B, metastatic disease including bone cortex; and stage IV-S, metastatic disease involving liver and skin only.

High-frequency ventilation at rates of 375 to 1800 cycles per minute in four neonates with congenital diaphragmatic hernia

Neonates with congenital diaphragmatic hernias (CDH) often die because of pulmonary hypoplasia and high pulmonary vascular resistance (PVR). Pulmonary hypertension and right-to-left shunting precedes progressive hypoxia and death. PVR is increased by acidosis and by high airway pressures. High-frequency oscillation (HFO) is a new technique which may improve the outcome for such infants. Gas exchange in HFO is achieved by directing rapid pulsations of small volumes of gas down the trachea, typically at rates greater than 200 cycles per minute, volumes less than 25% of dead space, and low airway pressures. Gas transport results from augmented diffusion, not from bulk flow. Four neonates with CDH deteriorated on conventional mechanical ventilation and required hand ventilation at rates above 200 per minute. HFO at frequencies from 375 to 1800 cycles per minute was then initiated using a flow-interrupter type of oscillator. A marked fall in PaCO2 and a rise in pH resulted. The elimination of CO2 was very efficient with low mean airway pressures (less than 15 mm Hg). The initial improvement during HFO probably resulted from a decrease in PVR due to reversal of the acidosis. However, all four babies died after 13 to 80 hours of HFO. Neonates with CDH who remain hypercapneic despite conventional mechanical ventilation can be successfully ventilated by HFO. Use of HFO produces respiratory alkalosis which may stabilize PVR in the normal range and improve survival rate.

Respiratory paralysis to improve oxygenation and mortality in large newborn infants with respiratory distress

The nonsynchronous respiratory efforts of neonates with surgically correctable disorders may inhibit effective mechanical ventilation. The records of 25 infants treated with metocurine for muscular paralysis to improve mechanical ventilation were reviewed. All patients were greater than 35 (37.6 +/- 2.1) weeks gestation and 2.27 (2.98 +/- .47) kg. All required ventilatory support with an FiO2 of 100%. The mortality rate of this group of infants was 20% as compared with 73% (p < .001) in a similar group of 26 infants managed without paralysis. In 10 of the 25 infants treated with metocurine, pre- and 1 hr postparalysis paO2 values were available. The mean paO2 prior to paralysis was 62 (45--111) mm Hg and the mean post-paralysis paO2 was 144 (75--227) mm Hg, representing at 132% increase in paO2 (p < .001). The mean dosage for metocurine was 3.5 (1.45--6.79) mg/kg/day; however, those requiring paralysis for greater than 7 days showed a dramatically increasing requirement. These preliminary data suggest that respiratory paralysis reduces right-to-left shunting, improves paO2 and decreases mortality in large infants with severe respiratory distress requiring ventilatory support.

Transabdominal Esophagomyotomy for Achalasia in Children

Achalasia is an uncommon esophageal problem in children. Nine cases accumulated over 7 yr at one institution are reviewed. Infants present with failure to thrive and may be misdiagnosed as suffering from chalasia. older children demonstrate dysphagia, nocturnal regurgitation, and failure to thrive. Contrast studies and esophageal manometry are diagnostic of the disease. Transabdominal esophagomyotomy is advocated, especially if Nissen fundoplication is also felt to be indicated to prevent reflux. All nine patients were treated successfully with this variation of the operation.

Hepatic mesenchymal hamartoma causing heart failure in the neonate

This is the first report of an hepatic mesenchymal hamartoma causing congestive heart failure in an infant. These benign tumors usually present as a bulky abdominal mass but rarely can present with congestive failure when the angiomatous component is prominent.

Esophageal atresia, distal tracheoesophageal fistula, and an air shunt that compromised mechanical ventilation

Infants with esophageal atresia and a distal tracheoesophageal fistula are predisposed to respiratory failure on the basis of prematurity, respiratory distress syndrome, aspiration of saliva, and reflux of gastric contents into the tracheobronchial tree. Thoracotomy and primary repair may be delayed to allow time for complete evaluation of the infant and respiratory stabilization. Poorly compliant lungs and a large distal fistula can result in selective passage of ventilatory gases into the gastrointestinal tract with resultant hypercarbia. Fogarty balloon occlusion of the distal esophageal segment halts this air shunt and facilitates effective mechanical ventilation.

Abscess of the falciform ligament in a child with a ventriculoperitoneal shunt

Falciform ligament abscess resulting from an infected ventriculoperitoneal shunt has not previously been described. This unusual type of abscess should be considered in a patient with an infected ventriculoperitoneal shunt who presents with epigastric tenderness. Signs of peritonitis may not be present if the abscess is localized. Imaging studies such as ultrasound or CT scan may frequently establish the diagnosis.