Massive osteolysis of the chest in children: an unusual cause of respiratory distress.

Massive osteolysis, also called Gorham's disease, is an uncommon disease in which bone virtually disintegrates and is replaced by vascular fibrous connective tissue. The authors treated two children with massive osteolysis of the chest. Both conditions responded well to radiation therapy; one even showed evidence of reossification. Massive osteolysis should be considered in children who have spontaneous hemothorax. The bony structures should be examined for evidence of lytic lesions.

Unique anomalies in cephalothoracopagus janiceps conjoined twins with implications for multiple mechanisms in the abnormal embryogenesis.

The anatomic features of female conjoined twins with the Janiceps type of cephalothoracopagus are described. Abnormalities included bilateral clefts of the alveolar arches, shared rudimentary mandible, high, arched clavicles, multiple rib deformities, single shared foregut and small intestine, absent large intestines, omphalocele, multicystic kidneys, hypoplastic lungs, interconnected aortas and neck vessels, single ovary with elongated uterus in each twin, displaced labia, abnormal segmentation of the vertebrae, spinal dysraphism, diastasis of the symphysis pubis, malrotated lower extremity, bilateral posterior dislocation of the hips, and club feet. There were two hearts with internal anomalies. Both spinal cords had a myelocele in the lumbar region. The abnormalities noted in previous reports of conjoined twins of this type are reviewed and compared. We propose that factors associated with conjoining, dysgenetic (developmental) defects, and deformations resulting from crowding in utero all may have been important in the abnormal development in this case.

Congenital left atrial aneurysm in an infant.

Congenital left atrial aneurysm, without associated cardiac abnormalities, is a rare defect. We report the case of a large left atrial aneurysm filling almost the entire left hemothorax. This is the first report of this anomaly occurring in an infant less than 1 year of age.

Ideal treatment for total colonic Hirschsprung's disease.

Five children were treated for total colonic Hirschsprung's disease between 1982 and 1986. Three girls and two boys underwent total colectomy and straight ileoanal endorectal pull-through, with creation of a suprapelvic side-to-side anastomosis of 10 cm of cecum and ascending colon to ileum (Boley procedure). All patients have been followed for periods of greater than 1 year, and they have been free of any postoperative complication. Stool frequency on an unrestricted diet has been between one and five semiformed per day. Their growth and development have been excellent. An advantage of this procedure is excellent water absorption without the risks of mechanical problems encountered with a variety of pelvic pouch creations in the young growing child.

Falls in urban children. A problem revisited.

Falls in urban setting are a common cause for emergency room visits in children and adolescents. In a retrospective review, the charts of 48 patients admitted between 1980 and 1985 with a history of a vertical fall from a height were examined. In comparison, a previous review from the same institution disclosed that 66 patients were admitted because of a vertical fall from a height between 1965 and 1974, suggesting an increase of 37.5%. Most children fell from heights of 12 ft or less, although an increasing proportion of children in our series (33%) fell from heights of 36 ft or less. Sites included windows, walls, and roofs. The peak age of incidence has increased from 2 to 6 years; however, the mean age of children in whom significant injury occurred was 7.5 years, with only 27% of children under 3 years of age suffering a documented injury, as opposed to 67% of children over 3 years of age. Children are more apt to suffer a fracture than any other injury, most likely a fracture of the ulna and/or radius. Although hospital costs are high, mortality rates (2%) and the incidence of long-term sequelae (4%) are low. In conclusion, falls in the urban setting continue to be a significant public health problem, particularly in the 6- to 7-year age group.

Identical twins with malrotation and type IV jejunal atresia.

Identical twins are reported, who associated with maternal anaphylactic shock at 101/2 weeks gestation, acquired a type IV jejunal atresia with total absorption of the small bowel beyond the third portion of the duodenum and persistence of the most terminal length of ileum in the ileocolic artery in an apple peel configuration. Both infants survived a tapered jejunoileostomy and have undergone a successful transition to a regular diet. This accidental occurrence in two infants at the same time and in an identical fashion is a rare event.

Transduodenal feedings: a superior route of enteral nutrition.

Our recent study comparing gastric with jejunal feedings in neonatal piglets demonstrated better weight gain, less diarrhea, and less fat malabsorption in animals fed gastrically. These differences were postulated to be due either to loss of duodenal osmoregulation and absorption, and/or to loss of the combined effects of salivary enzymes, gastric emptying, and gastric secretions on jejunal feedings. The objective of this investigation was to determine the relative contributions of gastric and duodenal function to the differences between gastric and jejunal feedings. Seven piglets (3 to 5 days old, 1.5 to 2.0 kg) underwent operative transgastric insertion of a duodenostomy tube placed just distal to the pylorus. Intravenous fluids and antibiotics were administered for two days postoperatively, then Premature Enfamil Formula (24 kcal/oz; Mead Johnson, Evansville, IN) was administered continuously through the feeding tube. The animals were maintained on 115 kcal/kg/d as in the previous study. Weight, fecal fat analysis, and stool characteristics were determined throughout the 2-week period. The resulting data were compared with data from our previous study comparing gastric with jejunal feedings. These data suggest the vital importance of gastroduodenal contributions of absorption, osmoregulation, and regulation of nutrient concentrations in improving the nutritional status of experimental animals. Duodenal feedings may be preferred to either gastric or jejunal feedings because they provide equivalent nutritional support without the gastroesophageal reflux and aspiration reported with gastric feedings in the neonatal population.

Intestinal lymphangiectasia and bilateral pleural effusions: effect of dietary therapy and surgical intervention on immunologic and pulmonary parameters.

This article describes the immunologic and pulmonary abnormalities and the chemical composition of pleural effusion fluid in a patient with intestinal lymphangiectasia as they are effected by therapeutic measures during a 7-year period. Lymphedema was first noticed in the patient at 3 years of age, and pleural effusions developed 7 years later. Thoracentesis demonstrated that the right pleural fluid was yellow, clear, and had the composition of lymph. The left pleural fluid was milky and had a higher triglyceride and lymphocyte content than the right pleural fluid. Complete removal of pleural fluid transiently increased total lung capacity to a maximum of 52% predicted. Strict dietary management with a low-fat and high-protein diet resulted in a transient partial reversal of circulating lymphopenia and low T cell concentration. This was accompanied by a decrease in lymphocyte and T cell concentration in the pleural fluid. Unstimulated mononuclear cells from pleural fluid synthesized increased amounts of DNA, and added mitogens or antigens further increased DNA synthesis. Dietary therapy had a minimal effect on this DNA synthesis. Despite circulating hypogammaglobulinemia, normal antibody activity was detected. The proportion of B cells in pleural fluid was greater than that in the circulation, and dietary therapy did not alter this difference. Pulmonary physiology improved during the initial 9-month period of diet therapy, but then the rate of fluid accumulation increased, causing respiratory compromise. Stability was achieved by a right-sided pleurodesis, followed 18 months later by a left pleurodesis with the addition of a shunt to provide internal lymph drainage.

The microvascular response of the neonatal mesentery to hypertonic feedings.

Using intravital microscopy we studied the responses of the jejunal and ileal mesenteric microcirculation to hypertonic meals in neonatal piglets. Test meals were infused through a cannulated segment of proximal jejunum. The intestinal segments were observed under conditions of controlled temperature and humidity, and the velocity of red blood cells through mesenteric arterioles was measured and blood flow calculated. Jejunal flow was not altered from baseline by saline or hypertonic mannitol infusion, while significant increases in flow were observed during hypertonic glucose infusion (3.8 X 10(-6) versus 10.7 X 10(-6) ml/s, p less than 0.01). Jejunal glucose infusion did not cause shunting from the ileal microcirculatory bed. We conclude that the infusion of hypertonic glucose causes an increase in jejunal blood flow, but does not result in a change in ileal flow. The increase in flow is a local phenomenon related to glucose absorption in neonatal piglets.

A comparison of intragastric and intrajejunal feedings in neonatal piglets.

Intrajejunal feedings by continuous infusion of nutrients into the proximal jejunum have been advocated for premature and low-birthweight infants. This technique eliminates the risks of gastric distention and aspiration associated with intragastric feedings. However, intrajejunal feedings disregard the normal physiologic responses to gastric feedings and completely bypass the stomach and duodenum, thus eliminating the important contributions to intestinal function of salivary enzymes, pancreatic secretions, and duodenal osmoregulation and absorption. The objective of this investigation is the comparison in neonatal piglets of differences in nutritional status and intestinal absorption between animals administered intrajejunal nutrients with those given intragastric formula. A total of 14 three- to five-day-old 1.5 to 2.5 kg domestic strain piglets were studied for two weeks. Seven animals underwent operative insertion of a jejunostomy tube into the proximal jejunum, while seven animals had a gastrostomy tube inserted. Intravenous antibiotics and fluids were given for two days postoperatively, then Premature Enfamil (24 kcal/oz) was administered continuously through the feeding tube. The animals were maintained on 115 kcal/kg/d for 10 to 14 days with daily measurements of weight, height, length, stool volume, and weekly determinations of electrolytes, blood counts, and fecal fat analysis. Student's unpaired t-test analysis of the data reveals significantly greater weight gain in the gastrostomy animals (2.5% weight gain/d +/- 0.3% v 1.5% +/- 0.3%, P less than 0.05). These animals also had less diarrhea (14% v 71%), and less fat malabsorption (0.15g/48 hr v 0.9g/48 hr).(ABSTRACT TRUNCATED AT 250 WORDS)

Thoracic trauma in children.

A 20-year experience at The University of Chicago in dealing with chest injuries in pediatric patients has been reviewed. The clinical presentation, including results of diagnostic measures, and the initial and definitive management of thoracic trauma in children are discussed. The condition of the majority of patients was stable at time of presentation. The most frequent surgical intervention performed was intercostal tube placement. Thoracotomy was infrequently required and laparotomy for associated injuries was more common. Trends in the causes and epidemiology of thoracic trauma in an inner city environment over this 20-year period were noted: Blunt trauma, which is increasing in frequency, most commonly involves the younger age group and is usually related to automobile accidents. Penetrating injuries are generally seen in older patients within the pediatric age groups and are similar to those seen in the adult population.

Intestinal osmoregulation of single component meals in neonatal piglets.

The ability of the neonatal stomach and duodenum to modify the osmolality of hypotonic and hypertonic meals containing a single nutrient was studied in 3- to 5-day-old piglets. Test meals were administered through a gastrostomy and samples of gastric content and all proximal jejunal effluent were collected every 30 minutes for 2 hours. Hypotonic meals (149 mosm/L) and hypertonic meals (497 mosm/L) of glucose, crystalline amino acids, or lipids were used. Results were similar after all meals. There were significant changes in intragastric osmolality at each postprandial time period. However, osmolality never returned to basal levels. During duodenal transit there were further increases in osmolality after hypotonic meals and decreases after hypertonic meals but isotonic levels were never achieved. As a result, chyme of widely divergent osmolality entered the jejunum until late in the observation periods. Influx of sodium, potassium, and chloride were similar after all meals and were independent of original meal osmolality. These data indicate that, in awake, unstressed neonatal piglets, the stomach and duodenum are capable of effective but incomplete osmoregulation of hypotonic and hypertonic single component meals.

Duodenal osmoregulation after total parenteral nutrition in neonatal piglets.

The physiologic effects of intestinal rest on the efficiency of gastroduodenal osmoregulation of hypertonic and hypotonic carbohydrate meals were studied in neonatal piglets 3 to 5 days old. Sixteen piglets underwent laparotomy, gastrostomy, and end jejunostomy just distal to the ligament of Treitz. Sixteen littermates had insertion of a central venous catheter and received 2 weeks of total parenteral nutrition (TPN) before laparotomy, gastrostomy, and end jejunostomy. Experiments were performed 2 days after laparotomy in all groups. Each piglet received intragastric infusions of two test meals (25 ml/kg), half the pigs receiving glucose (494 +/- 11, 147 +/- 3 mOsm/L) and half mannitol (493 +/- 11, 150 +/- 4 mOsm/L) (SD). Gastric samples and jejunal effluent were collected every 30 minutes for 2 hours. TPN did not influence duodenal osmoregulation. Significant changes in osmolality occurred during duodenal transit after all meals, and these changes were similar for all groups. However, jejunal osmolality did not approach basal levels until late in the observation period. These results demonstrate significant but incomplete intraduodenal osmoregulation of carbohydrate meals in neonatal piglets that is unaffected by 2 weeks of intestinal rest.

Congenital segmental dilatation of the colon.

Segmental dilatation of the colon with bowel wall muscle hypertrophy has been described in children 6 mo and older presenting with constipation since birth. A case of segmental dilatation of the colon without muscle hypertrophy in a newborn argues for the congenital origin of the dilatation and for the muscle hypertrophy being acquired.

Respiratory hydrogen secretion: a simple test of bowel adaptation in infants with short gut syndrome.

The prime goal in the management of infants with short gut syndrome is the smooth transition from total parenteral alimentation to total gastrointestinal alimentation. Such transition is dependent upon early feeding to actively promote the intestinal mucosal hyperplasia that is the key to eventual oral alimentation. A modular hyposmolar formula was previously reported to be successful in promoting the intestinal adaptation in several infants with short gut syndrome. Progression of the daily diet was dependent upon the day's clinical response, i.e., whether a change in volume or substrate composition resulted in increased stooling or diarrhea. Diarrhea occurs when the substrate load exceeds the absorptive capacity of the intestine and may be severe enough to require a return to parenteral alimentation until intestinal recovery is complete. Monitoring of daily breath H2 has provided an early warning mechanism to indicate carbohydrate overload in the adapting bowel before clinical signs of malabsorption occur. It provides an accurate daily measure of the bowel's ability to utilize the specific volume and caloric density it is being presented, thereby allowing diet advancement in a controlled fashion. The use of the breath H2 test in three infants with short gut syndrome has assisted in the transition to oral alimentation in 3 to 20 wk without the complications of severe diarrhea.

Tracheostomy in neonates and small infants: problems and pitfalls.

Newborn babies and small infants who require tracheostomy often follow a complicated clinical course characterized by frequent sepsis, altered ventilatory dynamics, and eventual respiratory decompensation. Many of these problems are avoidable with use of a properly placed endotracheal tube during tracheostomy, a special surgical technique in the performance of the tracheostomy, and silicone rubber tracheostomy tubes. Seventy-four babies underwent tracheostomy at our Children's Center between 1963 and 1976. A review of this experience demonstrates the benefits of unhurried, standardized technique and management. In the 48 newborns requiring tracheostomy, no technical difficulties were encountered, and no complications occurred as a result of the tracheostomy. In the group of 26 older infants, however, there were significant complications, especially in children undergoing emergency tracheostomy without a previously placed endotracheal tube. Although there were no deaths directly related to tracheostomy, one case of pirulent tracheitis and one case of interstitial thyroid hemorrhage were noted at autopsy. Among survivors, there was one case of bilateral pneumothoraces, two cases of severe subcutaneous emphysema, and three cases of postoperative bleeding. Review of the long-term complications in this series demonstrates the benefits of the silicone rubber polymer tube. Since its routine use, problems with stomal granulation have almost disappeared. There have been no problems in extubating the very young babies. Our operative technique and intensive care management of these babies will be emphasized as the keys to the improved outcome.

Continent ileoanal endorectal pull-through.

Five patients with familial polyposis coli, ages 13 through 21 yr, were given the choice of permanent reservoir-type ileostomy or ileoanal endorectal pull-through to prevent cancer of the colon. Total colectomy and an ileoanal endorectal pull-through was uniformly chosen and was performed using the principles described by Boley and Soavè. The post-operative courses were characterized by a transition from 8 to 10 liquid stools per day and up to 3 incontinent nocturnal stools, to a bowel pattern of 2 to 4 continent semi-liquid stools without nocturnal soiling by 1 mo. Defecograms at 1 mo revealed excellent sphincter function and mild reservoir dilatation of the ileum. These observations have convinced us that this is the operative management of choice for familial polyposis.

Hyperinsulin hypoglycemia in the neonate: therapeutic choices.

Lack of elevated serum insulin in 3 patients with nesidioblastosis following 95% pancreatectomy leaves one with the theoretical possibility that the recurrent hypoglycemia is not due to excess insulin secretion, but rather an iatrogenic hypoglucagon state. If this explanation is correct, progression to a "total" pancreatectomy may not be the appropriate form of therapy. It seems to us that further evaluation of other hormones secreted by the endocrine portion of the pancreas must be clarified before continuing extensive or total pancreatic resections for nesidioblastosis in infancy.