Detrimental effects of standard medical therapy in congenital diaphragmatic hernia.

OBJECTIVE: To evaluate the impact of a nonstandard ventilation strategy on survival in congenital diaphragmatic hernia (CDH). BACKGROUND: Despite recent advances, including nitric oxide, CDH remains an unsolved problem with a mortality rate of 35% to 50%. Hyperventilation and alkalization remain common therapies. METHODS: In 1992, the authors prospectively abandoned hyperventilation and alkalization. Patients are lightly sedated and ventilated with the lowest pressure providing adequate chest movement, and the rate is set to patient comfort. Nitric oxide and extracorporeal membrane oxygenation (ECMO) are reserved for life-threatening instability. Surgical repair is delayed 1 to 5 days. Sixty consecutive patients are compared with 29 previous patients treated with hyperventilation and alkalization, 13 before and 16 after the availability of ECMO. RESULTS: Overall, 47 of 60 patients (78%) in study era 3 survived compared with 2 of 13 (15%) in the hyperventilation era and 7 of 16 (44%) in the hyperventilation/ECMO era (p < 0.0001). The disease severity and the incidence of associated anomalies did not differ between groups. To compare management strategies, patients who had treatment withheld because of lethal associated conditions were then removed from analysis. Peak inspiratory pressure and arterial pH were lower (p < 0.0001) and Paco2 was higher (p < 0.05) in era 3 than in the previous eras. The rate of pneumothorax (1.9%) decreased (p < 0.0001). In era 3, survival was 47 of 53 (89%) treated patients, and 23 of 25 inborn patients with isolated CDH survived (92%). CONCLUSIONS: Nonstandard ventilatory support of patients with CDH has led to significantly improved survival rates. This study sets a survival benchmark and strongly suggests the negative effects of hyperventilation and alkalization.

Gastrointestinal myoelectric activity in a child with gastroschisis and ileal atresia.

Gastroschisis is frequently associated with intestinal atresia and alterations in gastrointestinal function. The authors studied gastric and small bowel myoelectric activity in a child who had a complex course and prolonged inability to tolerate oral intake after staged repair of gastroschisis and an associated ileal atresia. The child remained unable to tolerate oral intake after repair of the atresia and was reexplored 3 months later to rule out a partial small bowel obstruction, with simultaneous placement of serosal electrodes on the stomach and proximal small bowel. Persistent gastric dysrhythmias were observed postoperatively, and the child was unable to tolerate gastrostomy tube feedings. Abnormalities were also seen in small bowel motility, including retrograde propagation of activity fronts of the migrating myoelectric complex. However, the intestine converted to a fed myoelectric pattern with tube feedings, and the child was subsequently able to tolerate feedings via a tube placed directly into the small bowel. The authors conclude that myoelectric recordings via implanted electrodes are safe and feasible in children, and may give information regarding underlying motility alterations. The ultimate clinical role of myoelectric recordings in treating children with suspected motility disorders will require further study.

Unilateral pulmonary agenesis, esophageal atresia, and distal tracheoesophageal fistula.

A small-for-gestational-age, premature infant with the combination of unilateral pulmonary agenesis, esophageal atresia, and distal tracheoesophageal fistula was treated successfully by early gastrostomy and delayed fistula division with esophagoesophagostomy. Only 2 other successfully treated cases have been reported previously. Both were full-term infants treated with early division of the tracheoesophageal fistula and esophagoesophagostomy. Gestational age, size, and associated medical problems need to be considered when planning operative therapy for these babies.

Long-term complications of laparotomy in Hodgkin's disease.

OBJECTIVE: The authors determined the incidence of complications in 133 patients who had undergone staging laparotomy with splenectomy before treatment for Hodgkin's disease (stages I-IV). METHODS AND MATERIALS: Medical records were reviewed, and the patients or their relatives were interviewed. Median follow-up after laparotomy was 15.7 years (range = 2.5-28 years). RESULTS: Ten episodes of overwhelming postsplenectomy infection (OPSI) were documented in nine patients (6.8%). None of 25 patients who received pneumococcal vaccine before splenectomy developed OPSI. Patients with advanced (stages III-IV) or recurrent Hodgkin's disease were at higher risk of OPSI than those with early disease, and those who received combined modality oncologic therapy were at greater risk than those receiving less intensive treatment. Surgical complications included small bowel obstruction in 13 patients (9.8%), necessitating repeat laparotomy in 9 patients (6.8%), atelectasis in 17 patients, abscess in 3 patients, and 1 wound dehiscence. No deaths occurred as a result of surgical complications. Causes of death in the 29 patients who died included Hodgkin's disease (12 patients), acute treatment-related morbidity (1 patient), leukemia (5 patients), bone marrow failure (3 patients), solid malignancy (2 patients), intercurrent disease (4 patients), unknown causes (1 patient), and OPSI (1 patient). CONCLUSION: With presplenectomy pneumococcal vaccination and modern surgical techniques, the long-term risks of laparotomy with splenectomy are acceptable if knowledge of the pathologic extent of abdominal Hodgkin's disease would alter treatment regimens.

Progress in childhood cancer care in Florida. 1970-1992.

Pediatric cancer has been a priority in Florida since 1970. That year physicians established a statewide network of children's tumor programs, the Florida Association of Pediatric Tumor Programs (FAPTP), which has grown from the initial two programs to 10 in 1992 and now maintains a registry with follow-up to monitor incidence and other indicators. State-of-the-art care is provided through affiliation with the Pediatric Oncology Group. The incidence of pediatric cancer in Florida is equivalent to national rates, but the number of children followed in 1991 had grown to approximately 2,000. The number receiving care has increased an average of 13% annually since 1981. Services for these patients should be reviewed on a continuing basis to assure access to specialized programs.

Pseudomembranous colitis following resection for Hirschsprung's disease.

Enterocolitis is the most common cause of significant morbidity and death in Hirschsprung's disease. Although most cases respond to nasogastric decompression, antibiotics, and colonic evacuation, some children have an unusually fulminant or protracted clinical course. Four cases are reported of pseudomembranous colitis (PMC) that developed 1 to 18 months (mean, 8 months) after definitive surgery for Hirschsprung's disease (Soave endorectal pull-though, 2; Duhamel procedure, 2). While all children presented with fever, abdominal distention, and diarrhea, indistinguishable from typical Hirschsprung's enterocolitis, the clinical course was fulminant in two cases, both of whom died of septic shock. Postmortem examination in both showed extensive colonic pseudomembranes despite identification of Clostridium difficile toxin and subsequent vancomycin therapy (initiated late in the clinical course). Two children in the series had protracted hospitalizations and eventually required diverting enterostomy despite recognition of C difficile toxin and treatment with enteral vancomycin, in one child necessitating multiple courses of antibiotic therapy. Awareness of the virulence of PMC associated with Hirschsprung's disease (even after definitive resection) should prompt submission of stool specimens from any child who presents with enterocolitis for both C difficile culture and toxin levels. On the basis of our experience it is our policy to initiate a prompt course of vancomycin by rectal lavage or nasogastric tube in all children with Hirschsprung's enterocolitis, pending culture results, in view of the significant morbidity and mortality exemplified by cases in this review.

Does patient age or intestinal pathology influence the bacteria found in cases of necrotizing enterocolitis?

To evaluate the relationship between bacterial colonization in neonatal peritonitis and patient age or intestinal pathology, we retrospectively reviewed peritoneal culture results from 96 infants having surgery for necrotizing enterocolitis. Microorganisms recovered mirrored those usually found as fecal flora within the neonatal intensive care unit. These reflect, in turn, the abnormal colonization intrinsic to care in the intensive care nursery. Analysis revealed no significant correlation between the disease and either age of the infant or intestinal site of disease.

Balloon dilatation of long-segment tracheal stenoses.

Although a rare anomaly, congenital tracheal stenosis is associated with a high mortality. Surgical approaches proposed for this life-threatening lesion have included resection of localized stenoses; tracheoplasty involving interposition of cartilage, periosteum, or pericardium via an anterior tracheal split; or esophageal anastomosis to a posterior tracheal split. None of these techniques have provided uniformly satisfactory results, and all entail significant morbidity associated with the tracheal anastomosis. We have used endoscopic balloon dilatation in four children with critical airway obstruction from long-segment tracheal stenoses. In each of these patients, symptoms began early in life (from 2 weeks to 6 months of age) and consisted of respiratory distress from upper airway obstruction or recurrent episodes of stridor and chest retractions requiring hospitalization. The location of the tracheal stenoses varied, involving the upper third, middle third, middle half, and distal half of the trachea in the patients encountered. At bronchoscopy, the lumen was judged to be less than or equal to 1 mm in three of these patients. Dilatation was accomplished using Gruentzig balloon catheters in sizes ranging from 3 mm to (eventually) 8 mm, and produced a posterior split in the complete tracheal rings responsible for the stenosis in all patients, resulting in great increases in airway lumen. All children were improved following balloon dilatation, and three are currently doing well at 2.5, 3.0, and 3.0+ years postdilatation. One infant eventually died of airway obstruction incurred by severe bronchomalacia distal to the tip of an endotracheal tube that had been passed to the carina after sequential balloon dilatations.(ABSTRACT TRUNCATED AT 250 WORDS)

The effect of initial operative repair on the recovery of intestinal function in gastroschisis.

Recent reports concerning the treatment of gastroschisis suggest that primary closure results in more rapid return of gastrointestinal function, a shortened hospitalization, diminished perinatal complications, and improved long-term survival. A 4-year retrospective review of infants treated for gastroschisis at the University of Florida yielded 30 infants requiring repair of this abdominal wall defect. The series included 19 males and 11 females, and the average abdominal wall defect measured 4 cm in its greatest dimension. Nine infants (mean weight, 2,275 gm) were repaired using a staged closure using a silastic (six) or cutaneous (three) silo. Complete fascial closure was accomplished in an average of 8 days in the silastic group and 15 days in the infants with skin flaps. Mean age at start of enteral feeds was 23 days, with complete oral feedings at 43 days. Twenty-one infants (mean weight, 2,127 gm) underwent primary fascial closure. Three deaths occurred in the perioperative period: one from acute renal failure and two from sepsis secondary to a segment of necrotic intestine. An additional infant developed postoperative necrotizing entercolitis but recovered. Two infants in this group also had jejuno-ileal atresia requiring extensive small bowel resection. In the remaining 15 infants, oral alimentation was initiated for an average of 23 days, with full oral alimentation at 46 days. The data suggest that the recovery of the gastrointestinal system, adequate enough to support total oral alimentation, is unrelated to the initial surgical procedure chosen to obtain fascial closure in the newborn with gastroschisis. In addition, vigorous attempts at primary fascial closure may jeopardize intestinal viability, diminish ventilatory function, and result in unnecessary morbidity and mortality.

Successful cryotherapy of a benign tracheal neoplasm.

A 10-year-old black male with a 6-month history of progressive dyspnea and stridor was found to have a submucosal mass occupying 75% of the subglottic airway. Biopsy specimens showed a pleomorphic adenoma of minor salivary gland origin--a tumor with a predilection for local recurrence after resection. A tracheostomy was performed for airway control and the lesion was treated with endotracheal cryotherapy. Two months later, the tracheostomy was removed and the patient has remained asymptomatic for 9 years. Pulmonary function studies 2 years following cryotherapy demonstrated a forced vital capacity (FVC) of 81% predicted, and a forced expiratory volume-one second (FEV-1) of 73% predicted. Bronchoscopy with biopsy at 5 years showed no evidence of recurrent airway obstruction or persistent tumor. This represents the first reported case of successful treatment of an airway tumor in a child utilizing profound cryotherapy. The case illustrates the utility of endotracheal cryotherapy in the treatment of certain benign and malignant obstructing lesions of the airway in children.

Impact of emergency room laboratory studies on the ultimate triage and disposition of the injured child.

One hundred sixty six surviving pediatric trauma patients were retrospectively studied to assess the contribution of initial laboratory evaluations to clinical decision making in the emergency department (ED). All laboratory tests ordered, the results of those tests and the number of test results available before patient disposition from the ED were reviewed. A total of 626 laboratory studies were ordered in 166 patients. Results from only 68.5 per cent of the laboratory tests were available in the ED prior to the patient's transfer from the ED. Only 6.4 per cent of these results were abnormal. This low incidence of abnormal test results and preadmission availability questions the utility of extensive initial routine laboratory evaluations of the pediatric trauma patient. Based on this data, the current protocol has been revised to include hemoglobin/hematocrit determinations, type and screen, and urinalysis. Additional blood is obtained and appropriately labeled so that further studies can be performed if the patient's management should require baseline laboratory evaluations. This approach is more efficient, cost-effective, and no less sensitive in the initial evaluation and management of the injured child.

The microbiology of neonatal peritonitis.

To review the bacteriology of neonatal intra-abdominal sepsis, we reviewed peritoneal cultures from 86 newborns undergoing operation for necrotizing enterocolitis (NEC) for the type and incidence of microorganism recovered. As a control, we conducted a similar review in 59 children with perforated appendicitis during the same period. Necrotizing enterocolitis was characterized by a lower incidence of polymicrobial contamination (1.7 organisms per patient vs 2.4 organisms per patient, NEC vs appendicitis) and an uncharacteristic pattern of isolates. Although enteric gram-negative bacilli were recovered in 80% of newborns, the incidence of Escherichia coli was only 21% in the NEC group vs 69% in the appendicitis group, while Klebsiella and Enterobacter species represented the most common gram-negative isolates recovered (63% vs 17%). More than 50% of neonatal cultures yielded gram-positive cocci, most frequently coagulase-negative staphylococci (30% vs 0%) and enterococci (17% vs 5%), as compared with more frequent streptococcal isolates in the appendicitis group (50% vs 10%). Anaerobes were seldom recovered in NEC cases (6%), but they were present in 50% of appendicitis cases. Additionally, Candida isolates were recovered in 10% of NEC cases (0% of appendicitis group). These results indicate the unique bacteriology of peritonitis in the critically ill newborn and probably reflect abnormal colonization in the neonatal intensive care unit.

Abdominal complications of ventriculoperitoneal shunts. Case reports and review of the literature.

Ventriculoperitoneal (VP) shunting of cerebrospinal fluid (CSF) is the standard therapy for the management of hydrocephalus. Before the advent of silastic, early abdominal complications were frequent and finally led to the abandonment of this technique for management of hydrocephalus. With the use of silastic shunt tubing, VP shunts have once again gained favor as the procedure of choice. Although there are now considerably fewer complications from VP shunts, the presence of an intraperitoneal catheter can still initiate various complications. Abdominal complications of VP shunts are reported to be from 10-30 per cent, thus remaining clinically important for early recognition and treatment in patient management. An awareness of these complications is necessary in creating an index of suspicion for the primary physician whose patients harbor a VP shunt and present with abdominal symptoms. This report presents five cases of children with abdominal complications of VP shunts (four pseudocysts and one umbilical granuloma with spontaneous drainage of CSF). Additional abdominal complications of VP shunts are discussed, as well as diagnostic and therapeutic alternatives in order to improve and expedite accuracy in diagnosis and provide simplicity and efficiency in treatment.

The role of coagulase-negative Staphylococcus in neonatal necrotizing enterocolitis.

Coagulase-negative Staphylococcus has emerged as a prominent pathogen in the neonatal intensive care unit and a recent report has implicated this organism in necrotizing enterocolitis (NEC). This same study suggests that Staphylococcus epidermidis is most commonly associated with a "mild form of enterocolitis." This prompted a review of the role of coagulase-negative Staphylococcus in the surgical complications of NEC. Between 1982 and 1986, 86 newborns underwent operation for perforation or intestinal necrosis secondary to NEC. Blood cultures, obtained within 72 hours of surgery, were positive in nine of 71 infants (13%). One third of these grew coagulase-negative Staphylococcus. Peritoneal cultures obtained at the time of operation were positive in 71 neonates. The incidence of Staphylococcus epidermidis was 30%. In a third of these cases, coagulase-negative Staphylococcus was the only organism covered. Overall mortality within the group was 33%. Coagulase-negative Staphylococcus was the single most frequent organism recovered from those infants who expired (35%). This data clearly indicates that Staphylococcus epidermidis must be considered as a significant pathogen in NEC. It is associated with both morbidity and mortality and, therefore, warrants appropriate aggressive therapy when recovered from the neonate with enterocolitis.

Nutritional support by intraperitoneal dialysis in the rat: maintenance of body weight with normal liver and plasma chemistries.

A rat model was developed to study intraperitoneal (ip) dialysis as a means of total nutritional support. Rats (200 g) were implanted ip with a catheter device and connected to a rodent infusion assembly. An automated system exchanged 10-ml volumes of a 37 degrees C solution containing 10% dextrose, 2% amino acid solution plus micronutrients. Rats were adapted over 3 days to a schedule of 16 1-hr cycles/day, and continued on this regimen for another 4 days. Rats subjected to this program maintained similar body weight, nitrogen balance, plasma chemistries, and liver tests in comparison to control animals fed per os in isocaloric and isonitrogenous amounts. Efficiency of peritoneal absorption for both glucose and amino acid was 95%. Histological examination of intraabdominal organs revealed only mild inflammation. This model is applicable to studies involving nutritional support via the peritoneal cavity, a technique which may be of value in patients with sensitive fluid balances (cardiac, renal, or pulmonary failure).

Caustic ingestion and its sequelae in children.

Management of caustic ingestion in children remains a difficult challenge, with the outcome ranging from an asymptomatic state to intractable esophageal strictures. We reviewed the cases of 56 children ranging in age from 10 months to 5 years treated from 1973 to 1984 at the University of Florida. For children seen primarily at our institution, initial management consisted of prompt endoscopy and early institution of steroids and antibiotics. Esophageal burns were confirmed in 37 patients, 21 (56.75%) of whom subsequently had esophageal strictures of varying severity. In seven patients, limited esophageal strictures were managed successfully by dilation, but 14 children with multiple strictures required eventual esophageal replacement. Substernal right colonic interposition was tolerated well by these patients, with few complications, and their growth and development have been satisfactory over follow-up periods ranging from nine months to 13 years. A review of these cases suggests that the character of the ingested caustic material is the most important determinant of the severity of esophageal injury, with preparations containing lye being the most injurious; that in the presence of full-thickness esophageal injury, there is a high potential for stricture formation, regardless of the type of initial management, including early use of steroids; and that multiple esophageal strictures are refractory to dilation, and esophageal replacement should be an early consideration.

Decreased lung metastasis and tumor growth in parenterally fed mice.

The influence of alternate forms of nutritional support on primary tumor growth rate, tumor DNA synthesis rate, and number of lung metastases was examined in Swiss mice bearing subcutaneously implanted Lewis lung carcinoma (LLC). From Day 14 through 22 postimplant, mice were fed by continuous intravenous infusion of dextrose/amino acid (TPN), were offered the same solution from a feeding bottle (PO), were offered a casein-based, solid diet (CASEIN), or were infused with an electrolyte (ELECT) solution while energy and nitrogen were provided from the casein diet. Tumor weight and doubling time were decreased in the PO group compared to CASEIN; however, host weight decreased by 22% in the PO group. Tumor weight and DNA synthesis were decreased in the TPN group compared to CASEIN, and host weight increased by 4.6%. The decreased rate of tumor growth in the PO group was not reflected in a decrease in DNA synthesis, perhaps a result of the circadian pattern of DNA synthesis as previously reported for LLC. The number of metastatic lung nodules was significantly decreased in both the TPN and ELECT groups compared to PO and CASEIN, suggesting that intravenous fluid load rather than nutrient intake was the causative factor. In this host-tumor system, parenteral feeding was associated with a decrease in primary tumor weight and DNA synthesis rate, maintenance of host weight, and a decrease in pulmonary metastatic disease compared to mice fed a conventional diet.

The pediatric trauma score as a predictor of injury severity in the injured child.

The ability of the Pediatric Trauma Score (PTS) to accurately predict the degree of injury severity of the injured child was assessed by comparing two separate groups of pediatric trauma victims. The first group consisted of 110 patients evaluated at a regional pediatric trauma center whose data was collected and assessed by a single investigator. The second group consisted of a similar matched cohort of 120 patients from the National Pediatric Trauma Registry whose data was collated from multiple participating institutions. In both cases, a linear relationship between PTS and Injury Severity Score (ISS) was documented that was statistically significant to P less than .001. The linear regression coefficients of each group were similar as was the distribution of PTS and ISS. This study documents the validity of the PTS as an initial assessment tool and confirms its reliability as a predictor of injury severity.

The effect of energy substrate manipulation on tumor growth and metastasis and intermediary metabolism in the parenterally fed mouse.

The effect of N-free energy substrate manipulation on tumor growth and metastasis, host maintenance, and intermediary metabolism was studied in parenterally fed Swiss mice bearing subcutaneously implanted Lewis lung carcinoma. Non-N energy was provided from dextrose (CHO), lipid emulsion (FAT), or a 75:25 balanced (BAL) solution, infused from day 14 through day 22 postimplant. Control mice were offered equivalent energy and N from a balanced, casein-based solid diet (CAS). Tumor-doubling time was significantly prolonged in the CHO group compared to FAT and CAS. Pulmonary metastatic nodules were decreased in number in all parenterally fed mice compared to CAS, suggesting that the route of administration altered pulmonary physiology in such a way that the transmissability and/or growth of the tumor cells was inhibited. Tumor-free body weight was maintained in the CHO (+ 1.3%) and BAL (+ 0.3%) groups. However, significant weight loss occurred, despite equal intake, in the FAT (-4.7%) and CAS (-7.5%) groups. The energy appeared to be channeled into nonoxidative pathways, reflected by an increase in hepatic and adipose tissue lipogenesis and hepatic glycogen content. During the period studied, parenteral dextrose/amino acid infusion in this host-tumor system resulted in a decrease in primary tumor growth and optimal host maintenance compared to fat-based TPN and enteral feeding of a balanced, solid diet. Tumor metastasis was decreased in all parenterally fed mice, a phenomenon related to the route of administration and apparently independent of energy substrate.