Effectiveness of enteral and parenteral nutrition in the nutritional management of children with Wilms' tumors.

The effectiveness of enteral and parenteral feeding in supporting a satisfactory nutrition status and/or reversing protein-energy malnutrition was evaluated in nine children, ages 1 to 7 years (eight female), with Wilms' tumors. At the onset of treatment, eight patients received comprehensive enteral nutrition (CEN) which included intense nutritional counseling and oral supplements while one received total parenteral nutrition (TPN). Despite CEN, the initial, intense treatment period was associated with a decreased energy intake (64 +/- 27% Recommended Dietary Allowances), dramatic weight loss (22 +/- 7% by 26 +/- 17 days from the beginning of treatment), decreased skinfold thickness (< 10th percentile), and decreased albumin concentrations (< 3.2 g/dl). Four of those who initially received CEN subsequently required TPN. A total of five patients received TPN for a mean of 31 days (range 11 to 60); kcal averaged 105 +/- 9% Recommended Dietary Allowances during weight gain. At onset of TPN, the mean albumin, transferrin, total lymphocyte count were 3.02 +/- 0.45 g/dl, 155 +/- 40 mg/dl, and, 655 +/- 437/mm3, respectively; all children had abnormal anthropometric measurements and anergy to recall skin test antigens. TPN for 28 or more days supported weight gain (+ 2.44 kg), increased serum albumin (+ 0.58 +/- 0.47 g/dl) and transferrin (+ 76 +/- 34 mg/dl), and reversed anergy despite low total lymphocyte counts. During maintenance treatment, nutritional status was maintained or restored with CEN in the group who responded. These preliminary data document the severity of protein-energy malnutrition which accompanies initial, intense treatment of children with Wilms' tumors, the nutritional and immunological benefits of TPN during continuing intense treatment and the effectiveness of CEN in maintaining a satisfactory nutritional status during maintenance treatment.

Congenital rectal stenosis: a sign of a presacral pathologic condition.

Congenital rectal stenosis may be detected in the newborn during the initial physical examination. Failure of conservative therapy (dilatation) should alert the physician to the presence of an associated pathologic condition in the presacral space. Presacral teratoma, anterior sacral meningocele, or bony anomalies may be the underlying extrinsic causes of congenital rectal stenosis. Prompt recognition and appropriate operative management directed at the presacral lesion will relieve obstructive symptoms and minimize morbidity.

Pyloromyotomy: Does surgery in infancy increase allergy?

One recently published article and several presentations at international meetings have suggested that there is an increased incidence of allergic and asthmatic manifestations, as compared with the general population, if a child undergoes surgery before the age of 2 years. The study explores the incidence of asthma and allergy in a group of patients who presented with pyloric stenosis. All of these underwent surgery. The technique of the study was a retrospective telephone review and the response rate was approximately 40%. The data show that there is no increase in the incidence of allergy or asthma in this population as compared with nationally reported figures.

Postoperative necrotizing fasciitis in children.

Necrotizing fasciitis is a rapidly progressive soft tissue infection, involving the skin, subcutaneous tissue, and superficial fascia. It is a rare but life-threatening complication in the postoperative patient. In the last 7 years, we have treated four children in whom necrotizing fasciitis developed after appendectomy for ruptured appendix, bilateral inguinal herniorrhaphy, or gastrostomy closure. These four patients and seven well-described children from the literature with necrotizing fasciitis following surgery form the basis of this review. The ages ranged from six days to 15 years (mean 4.5 years). There were eight boys and three girls. There were five clean, five clean-contaminated, and one contaminated surgical procedures. No patient had evidence of malignancy or diabetes. Two of our four patients had evidence of failure to thrive. Only one patient had an intraabdominal abscess. In ten, the infection started in the abdominal wall; in one, the infection started in the chest wall. In our four patients, three had neutropenia and fever, four had tachycardia, and two had wound crepitation and radiographic evidence of subcutaneous gas. Cultures of all ten wounds were positive for bacteria; six were positive for more than one organism. Blood culture results were positive in five of five patients who died and in only two of five patients who survived. All survivors had wide surgical debridement and were treated with broad-spectrum antibiotics. The mortality rate was 45% in the whole series.(ABSTRACT TRUNCATED AT 250 WORDS)

Esophageal atresia and tracheoesophageal fistula: Effect of delayed thoracotomy on survival.

Eighty-four infants with esophageal atresia and/or tracheosophageal (TE) fistula were treated from 1972 to 1977. Twenty-eight percent were premature and 24% weighed less than 2.0 kg. Major symptoms included excess salivation (56 patients), respiratory distress (28 patients), cyanosis (26 patients), and choking (nine patients). Pneumonia and or atelectasis occurred in 58% and associated anomalies in 68%. Seventy-three of 84 patients (87%) had proximal esophageal atresia and distal TE fistula (type C defect). Operation was carried out in 79 patients. Gastrostomy was performed in 75 patients, often under local anesthesia with subsequent delayed extrapleural thoractomy (mean, 3.9 days), when the infant's pulmonary condition was improved. Primary anastomosis was performed in 55 patients, division TE fistula and esophagostomy in ten, staged anastomosis in seven, cervical esophagostomy alone in three, division H fistual in two, ligation TE fistula alone in one, and gastrostomy alone in one. Complications were frequent, including need for ventilator support in 28 patients, atelectasis in 28, pneumonia in 18, jaundice in 13, heart failure in 11, anastomotic leak in 10, and stricture in four. Operative mortality was 5% (four of 79). Two deaths followed immediate thoracotomy and two were premature with anomalies. There were eight late deaths 4 to 39 months after operation. Seven had multiple anomalies. The overall mortality was 15%. Management of high-risk cases by preliminary gastrostomy and delayed extrapleural thoracotomy is associated with improved survival (67 to 79) (85%). Neonatal intensive care, detection of associated anomalies, and long-term follow-up are essential factors in reducing mortality.

Benign and malignant teratomas in children: analysis of 85 patients.

Teratomas were observed in 85 infants and children. Fifty-eight (68%) were girls and 27 (32%) boys. Site of origin was sacrococcygeal in 55 patients (64.8%), mediastinal in ten (11.7%), gonadal in ten (11.7%), presacral in four (4.8%), retroperitoneal in three (3.5%), and cervical in three (3.5%). Sixty-seven (78.8%) teratomas were benign and 18 (21.1%) malignant. Malignant tumors were noted in 11 of 55 sacrococcygeal (20%), two of ten mediastinal (20%), three of eight ovarian (37.5%), and both testicular lesions. Cervical, retroperitoneal, and presacral tumors were benign. Age at diagnosis (greater than 1 month), presence of symptoms (urinary and colonic obstruction), and serum positive for alpha fetoprotein were indicators of malignancy in sacrococcygeal cases. Age was not a factor for teratomas at other sites. Tumor size, presence of calcification, and gross appearance (cystic or solid) did not relate to the tumor's benign or malignant nature. Three of four presacral tumors were associated with anorectal anomalies. Operative resection is the treatment of choice. Four deaths were related to operative hemorrhage. Four of five survivors with malignant teratoma received radiation and chemotherapy. A more aggressive role for combined adjunctive measures is suggested in cases of malignancy.

Posterior sagittal anorectoplasty for adults. A sphincter-saving operation.

We performed posterior sagittal anorectoplasty to treat two adult patients. One patient had an idiopathic stricture extending from the proximal sigmoid colon to the anus. The second patient was born with a high imperforate anus and, following a pull-through procedure as an infant, remained totally incontinent of feces. Both patients now have excellent fecal continence following this operation. The posterior sagittal anorectoplasty may be used to preserve rectal continence in selected adults.

Gastroesophageal reflux in infants and children. Diagnosis and management.

Eighty-three infants and children underwent surgical correction of gastroesophageal reflux (GER) from 1973 to 1978. Fifty-four patients had coexistent brain damage (most commonly due to cerebral palsy), eight were previously treated for esophageal atresia, and four had gastroschisis or omphalocele repair. Clinical presentation included failure to thrive in 64 patients, vomiting in 59, and recurrent bouts of aspiration pneumonitis in 43. Barium roentgenography showed GER in 61 patients, whereas additional tests (particularly pH monitoring) were required for detection of GER in 22 patients. After failure of medical management, transabdominal Nissen fundoplication was performed in 80 cases and a Hill repair in three cases. The surgical mortality was zero, but there were five late deaths. Results were considered excellent in 54 patients, good in 22 patients, and poor in seven. Ten of 12 patients with preoperative stricture responded to dilation after fundoplication. Nissen fundoplication was a safe and effective antireflux procedure in 76 of the 83 cases.

Colostomy complications in children. An analysis of 146 cases.

This report analyzes the course of 146 pediatric patients with colostomies in reference to problems with colostomy formation, management, and subsequent closure. Colostomy was performed predominantly for Hirschsprung's disease (70 cases) and imperforate anus (46 cases). A transverse colostomy was done in 120 patients (82%), and a sigmoid colostomy in the remaining patients. Loop colostomies were five times more frequent than divided colostomies. Early major complications occurred in 24 patients (16%). Stomal complications occurred in 69 patients and were more frequent after loop colostomies. Colostomy revision was required in 24 cases. Sigmoid colostomy had a significantly lower complication rate (P less than .01). One hundred nine patients underwent colostomy closure. Major complications occurred in 16 cases (15%). There were no deaths related to colostomy closure. The use of a sigmoid colostomy when possible and close attention to technical details, principles of stomal care, and proper parental instruction should minimize morbidity.

Nutritional care of the surgical neonate.

The function of the nutritionist was to identify and anticipate nutritional problems, to facilitate complete care for each infant, and to serve as an educational resource to staff, patients, and families. Neonatal nutrition is an open and challenging field for nutritionists. An expanded role for the nutritionist as a member of the physician-nutritionist team, as demonstrated in this article, may provide direction for the organization of such a team in other health care facilities with neonatal intensive care units.

Survey of nutritional problems encountered in children with neuromotor disorders.

A survey of 200 patients ranging in age from 5-21 yr was undertaken in 3 facilities in central Pennsylvania: state- (100), county- (13), and privately-operated (66), together with 21 children being cared for at home. Their diagnoses included chromosomal, metabolic, and anatomic abnormalities, and other encephalopathies. The following nutritional problems were identified: 1) inadequate nutrient intake due to feeding technique, swallowing difficulties, or regurgitation; 2) obesity and low activity level; 3) constipation; 4) nutrient-drug interactions and allergies; and 5) inadequate standards with which to compare growth and adequacy of nutrient intake. The nutritional problems encountered in these pediatric patients with neuromotor disorders warrant management by a physician-nutritionist team skilled in nutritional assessment and techniques of providing nutritional support.

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.

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)

Long-term outpatient amphotericin B therapy via a silicone central alimentation catheter.

A 6-yr-old boy with systemic histoplasmosis received intravenous amphotericin B for two months as an outpatient utilizing techniques developed for home parenteral nutrition. The therapy was administered on alternate days in the emergency room by nurses trained to use aseptic technique. No complications occurred. The merits of this technique include avoidance of prolonged hospitalization with its high cost and risk of nosocomial infection and better patient acceptance with the patient living at home and continuing many of his usual activities.

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.

The relationship between peak inspiratory pressure and positive end expiratory pressure on the volume of air lost through a bronchopleural fistula.

A bronchopleural fistula (BPF) may complicate the management of patients with major pulmonary disease or thoracic surgery. Neonates with idiopathic respiratory distress syndrome and requiring ventilation are especially susceptible to pulmonary barotrauma, which may result in a BPF. Morbidity and mortality are consistently high. In ventilating patients with BPF, the effects of peak inspiratory pressure (PIP) and positive and expiratory pressure (PEEP) on air leak have not been documented. These relationships were studied in rabbits prepared by thoracotomy and creation of a standardized BPF. Randomized trials of various levels of PIP and PEEP were applied, and the percent of inspired tidal volume lost through the BPF calculated. The percent of inspired volume lost does not increase significantly from 10 to 30 cm H2O PIP (p greater than 0.05). Percent leak does increase significantly when increasing PEEP frm 0 to 16 cm H2O (p less than 0.001). Any PEEP greater than 6 cm H2O results in more air loss through the BPF than any level of PIP (p less than 0.01). Linear regressions through a common origin were calculated to illustrate the relationship of PIP versus leak and PEEP versus leak. The slopes of these lines (0.572 and 3.97, respectively) are significantly different (p less than 0.001). When using equal increments of PIP and PEEP, PEEP will have over a sixfold greater effect on air leak than doses PIP. These data suggests that PIP should be increased preferentially when ventilating patients with BPF in order to minimize air leak. PEEP less than 6 cm H2O can be used without any significant increase in the volume of air lost.

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.

A critical assessment of fluid requirements in gastroschisis.

This report critically evaluates the fluid requirements of 30 infants with gastroschisis. Fluid requirements were unrelated to gestational age, birth weight, or type of repair. Twenty-five percent of the fluid was colloid needed to restore intravascular volume and oncotic pressure, the balance consisting of Ringer's lactate solution. These data support the conclusion that the fluid requirements of the newborn with gastroschisis are significantly increased over those of the normal neonate. This is a reflection of antenatal peritonitis, third space fluid shifts and loss of water and electrolytes during transport to the pediatric surgical center. Recognition of these needs and appropriate replacement should reduce the perioperative morbidity and mortality in these seriously ill neonates.

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.

Assessment of pulmonary wedge resection for the treatment of lung metastases.

Wedge resection for pulmonary metastases was performed on 18 children suffering from a variety of malignant tumors. The overall survival rate was 39%. However, tumor recurred at or near the site of resection in half the patients. A more extensive resection, such as lobectomy, should be considered for most patients. Wedge resection should be reserved for tumor involving many lobes or for neoplasms having a demonstrated sensitivity to chemotherapy and irradiation.