John M. Templeton

Morbidity and mortality of short-bowel syndrome acquired in infancy: An update

The advent of total parenteral nutrition (TPN) has made survival beyond infancy possible for large numbers of patients who have sustained massive small intestinal loss due to a variety of intraabdominal catastrophes. However, the quantity and quality of life have been limited by the development of late sequelae due both to the protracted use of TPN and the long-term complications of foreshortening of the gut. To determine to what extent the morbidity and mortality of short-bowel syndrome (SBS) may have improved over the last 10 years, we reviewed our experience since 1973 with patients losing more than 50% of total small intestinal mass in infancy. The etiologies of SBS in the 16 study patients were necrotizing enterocolitis (6), midgut volvulus (5), multiple atresias (3), gastroschisis (1), and congenital SBS (1). Overall survival was 81%; total small intestinal length (SIL) at the time of diagnosis was 44.2 +/- 7.9 cm in survivors and 30.3 +/- 7.8 cm in nonsurvivors, probability values not significant. Although no patient survived without an ileocecal valve whose total SIL was greater than 20 cm, the three deaths in this series were not related directly to the SIL, but to end-stage liver disease resulting from TPN-associated cholestasis. Among the survivors, adaptation to enteral feedings required 13.8 +/- 2.5 mo, during which time weaning from TPN occurred; weight at adaptation was 6.87 +/- 1.32 kg.(ABSTRACT TRUNCATED AT 250 WORDS)

Nonoperative management of blunt pediatric major renal trauma

Although algorithms exist for the management of renal trauma in adults, guidelines have not been established in children. Of 1,175 patients entered into our Trauma Registry between 1987 and 1991, 61 (5.2%) presented with gross or microscopic hematuria. Eight of the 58 patients (13.8%) who had blunt abdominal trauma had major renal injuries. Gross hematuria (n = 10) was a significant predictor of major renal injury (n = 5) (p < 0.001). All 3 patients with microscopic hematuria and a major renal injury also had evidence of multisystem trauma. Admission blood pressure, hemoglobin, and trauma score were not predictors of major renal trauma. All cases were managed nonoperatively except for 1 patient who required a partial nephrectomy for continued hemorrhage. These data suggest that hematuria of any degree should be evaluated in the pediatric population, since major injuries can occur with even microscopic hematuria or in the absence of shock. Nonoperative management in this series resulted in no morbidity or delayed complications and suggests that surgical exploration be reserved for ongoing bleeding.

Short-term ν long-term quality of life in children following repair of high imperforate anus*

In children with high imperforate anus, their quality of life (QOL) is directly related to their success or failure in attaining fecal continence (FC). At the Childrens Hospital of Philadelphia, 120 patients were treated for high imperforate anus. Sixty-one patients were available for long-term follow-up ranging from 2.5 to 24 years. The patients were analyzed in three separate time periods. The purpose of the study was to (1) establish a reproducible quantitative and qualitative scoring system for evaluating QOL; (2) identify clinical techniques for maximizing fecal continence; and (3) develop an algorithm for long-term management of children with persistent fecal incontinence. QOL scores and FC scores were similar for males and females. Patient age, however, proved to be a particularly important factor in QOL. As the length of follow-up increased, there was a significant difference in the percentage of those patients with a QOL score higher than FC score; 57% for the youngest patients v 15% and 7% for the older patients (P less than .01). Ninety-two percent of patients who were 10 years old or less had very supportive families. These families used a number of stratagems to minimize incontinent problems: liners in underpants, enemas, meticulous perineal hygiene, and avoidance of certain foods. After 10 years of age, a childs FC score became a major determinant of his QOL. Older children with fecal incontinence could no longer be shielded by parents and were not well tolerated by teachers and peers. From this study, an algorithm is proposed for children with impaired FC.(ABSTRACT TRUNCATED AT 250 WORDS)

Antenatal diagnosis and early surgery for choledochal cyst

The pathogenesis and optimal treatment of choledochal cyst have long remained questions of considerable speculation and dispute. Because the pregnancy of a 37-year-old woman was felt to be at risk, five antenatal ultrasound examinations were made. The fourth examination at 31.5 weeks of gestation demonstrated a choledochal cyst. Following birth, the child was studied with repeat ultrasound examinations and scintigraphy. The results of these studies plus the findings at surgery contributed new evidence regarding the possible pathogenesis and optimal treatment of choledochal cysts. Although the time at which the cyst originated is compatible with the concept of reflux of pancreatic juice into the common duct, no abnormal junction of the pancreatic and common duct was identified. No evidence of obstruction as part of the pathogenesis could be demonstrated. Progressive changes in the choledochal cyst in the first ten days of life suggested that delay in diagnosis and treatment of a choledochal cyst may contribute to early complications such as cholangitis. Early excision of the cyst in the newborn is considered to be the optimal treatment and may pose less risk to the patient than delayed surgical exploration.

Situs inversus: The complex inducing neonatal intestinal obstruction

Situs inversus (SI) complicating neonatal bowel obstruction presents a challenging complex, and to facilitate rational decision making for treatment, we have reviewed 23 cases of abdominal SI seen in our hospital over the last 25 years. Preoperative roentgenographic studies most always predicted SI, the specific patient groups including: 12 abdominal SI with dextrocardia, 10 abdominal SI with levocardia, and 1 with partial heterotaxia. Major intraabdominal anomalies produced surgical emergencies in 7 neonates in the first year, 6 of these 7 being in the first month of life. In these 7 patients, multiple anomalies occurred including 1 child with a rotational anomaly with reversible ischemia secondary to midgut volvulus, and 4 with a rotational anomaly without volvulus, all being treated with a modified Ladd procedure. One of these children had an unrecognized intraluminal duodenal membrane, 1 an operatively diagnosed intraluminal membrane, 1 had annular pancreas, and 1 had a discontinuous jejunal atresia. A preduodenal portal vein was present in 4 of the 7 children, a branch being divided in 1 and the full vein bypassed in 2 of the other 3 patients. Two patients had biliary atresia, one of whom also had a diaphragmatic hernia. Five of the 7 neonates had associated major congenial heart disease accounting for 2 of the 3 deaths in this series. This review emphasizes the protean nature of abdominal SI, especially as it may cause or contribute to neonatal intestinal obstruction; and it is this understanding which is a prerequisite to optimal operative management.

Substance abuse in adolescent trauma.

STUDY OBJECTIVE To determine if there is a significant prevalence of drug or alcohol use among adolescents evaluated for significant acute trauma. DESIGN A prospective, age-matched controlled study over a 20-month period. SETTING Urban pediatric emergency department in a Level I pediatric trauma center. PARTICIPANTS Patients between 13 and 19 years of age requiring admission to the trauma service following evaluation in a pediatric ED and an age-matched control group of asthmatic patients. RESULTS A total of 134 patients (mean age, 14.8 years) were admitted for trauma-related injuries, and 22 of 65 (34%) were positive for alcohol or drugs of abuse. The mean age of patients with a positive toxicology screen was 15.4 years. Most commonly detected drugs were alcohol (eight), benzodiazepines (eight), cocaine (five), and cannabinoids (four). The number of positive screens in the trauma group (22 of 65) was significantly higher than controls (one of 49) (P < .001). This remained statistically significant even when those trauma patients not screened were assumed to have a negative toxicology screen (22 of 134 versus one of 49) (P < .01). There was also a significantly higher number of positive toxicology screens among adolescents with an intentional versus unintentional mechanism of injury (21 of 71 versus one of 63) (P < .001). CONCLUSION A significant number of adolescents admitted to the hospital for trauma-related injuries have a toxicology screen positive for alcohol or drugs of abuse. A toxicology screen should be a standard laboratory test in adolescents involved in significant trauma, especially if the mechanism was intentional.

Management of esophageal atresia and tracheoesophageal fistula in the neonate with severe respiratory distress syndrome

In a 10-year period, 22 neonates with esophageal atresia (EA) and tracheoesophageal fistula (TEF) required high pressure ventilatory support soon after birth because of respiratory distress syndrome (RDS). Eleven of the 22 or 50% survived overall, but if the 5 patients who died before definitive surgical repair could be attempted are excluded, 11 of 17 or 65% survived. More importantly, 4 of 7 (57%) patients who had gastrostomy performed first survived while 7 of 10 (70%) who had fistula ligation performed first survived. The difficulties with intraoperative management of those who had gastrostomy performed first were even more impressive. Our experience leads us to conclude that patients with EA and TEF with severe RDS who require high pressure ventilation preoperatively represent a group of patients who require special consideration. The danger to such patients with increased pulmonary resistance is not gastric distention but sudden loss of intragastric pressure. In the presence of poor lung compliance, the upper gastrointestinal tract functions in continuity with the tracheobronchial tree. A sudden loss of intragastric pressure, as with placement of a gastrostomy tube, results in an acute loss of effective ventilating pressure. Resuscitation of such a patient is not possible until leakage from the esophagus is controlled by ligation of the fistula or transabdominal occlusion of the distal esophagus. Placement of a Fogarty catheter into the fistula via a bronchoscope is effective but may not be feasible in every case. Early thoracotomy and ligation of the fistula in patients with progressive RDS provides immediate improvement in ventilatory efficiency and relief of gastric distention.

Colon interposition for the short bowel syndrome

A 5-mo-old male had disabling diarrhea and malabsorption following massive small bowel resection. His transit time was 10 min. After 9 mo of conservative treatment, a 24-cm isoperistaltic segment of colon was interposed 6.5 cm from the ligament of Treitz. Transit time was increased to 105 min, and he was eventually able to be maintained on an enteric diet without significant diarrhea. Colon interposition requires little manipulation of the remaining small intestine and does not rely on active intestinal obstruction to effect an increased transit time.

The usefulness of open-lung biopsy in the pediatric bone marrow transplant population

From October 1976 to October 1986, 126 children had bone marrow transplants at the Childrens Hospital of Philadelphia. The indications were acute lymphocytic leukemia (ALL) (30), nonlymphocytic leukemia (24), aplastic anemia (15), solid tumors (47), and miscellaneous conditions (10). Of these, 21 (17%) underwent 22 open-lung biopsies. Fourteen of these patients showed no causative microorganism. When a cause was found it was viral (usually cytomegalovirus [CMV]) in three, fungal in one, Pneumocystis carinii alone in two, both viral and pneumocystis in one, and a combination of viral, bacterial, and pneumocystis in one. Thirteen patients died due to continued deterioration after the biopsy. In only two patients was there a significant change in antimicrobial therapy as a result of the biopsy. Both had Pneumocystis (one in combination with virus and bacteria). One patient with chronic infiltrates showed a lymphocytic interstitial pneumonia, which responded well to steroids. Open-lung biopsy is currently of limited value in this patient population. Survival is dismal unless the patient has Pneumocystis. We believe that prospective studies should be set up to compare open-lung biopsy with empiric antimicrobial therapy. A major emphasis must be on prevention.

Hypertonic saline improves brain resuscitation in a pediatric model of head injury and hemorrhagic shock

INTRODUCTION Brain injury accompanied by hypovolemic shock is a frequent cause of death in multiply injured children. Hypertonic saline (HTS) has been shown to return hemodynamics to normal in adult models, without increasing intracranial pressure (ICP) as seen with crystalloids. To assess fluid resuscitation, the authors evaluated HTS versus lactated Ringers solution (LR) with respect to hemodynamics and cerebrovascular hemoglobin oxygen saturation (Sco2) in anesthetized, head-injured, 1-month-old piglets. METHODS Group 1 (n = 6) was studied for 3.5 hours after a cryogenic brain injury and no shock. Groups 2 and 3 had cryogenic brain injury followed by hemorrhagic shock, in which mean arterial pressure (MAP) was reduced to 40 to 50 mm Hg and maintained for 30 minutes. Group 2 (n = 5) was then resuscitated with 1 mL of 7.5% HTS per 1 mL of blood loss. Group 3 (n = 6) was resuscitated with 3 mL of LR per 1 mL of blood loss. Sco2 was determined by near-infrared spectroscopy in the injured region of the brain. All data were analyzed using analysis of variance with repeated measures. RESULTS MAP, ICP, temperature, serum sodium, and cardiac output (CO) were similar in all groups during baseline and between groups 2 and 3 during shock. After resuscitation, MAP, CO, and core temperature were similar in all three groups, and serum sodium was increased in the HTS group (by 29%). Sco2 increased transiently after cryogenic injury in all groups, then gradually decreased to below baseline. After shock, Sco2 decreased precipitously in group 2 and 3. After resuscitation, Sco2 was different in the two resuscitation groups, increasing in the HTS group, above baseline values, but remaining below baseline values in the LR group (P < .002). ICP was lowered by HTS resuscitation and increased by LR resuscitation (P < .002) CONCLUSION In our model of head injury and shock, resuscitation with either HTS or LR restored MAP and CO to control levels. However, during shock, the injured brain was severely deoxygenated, and administration of HTS restored cerebral oxygenation whereas LR did not, reflecting improved cerebral resuscitation by HTS without elevating ICP. The data suggest that HTS is a better resuscitation fluid than LR in head-injured children with hemorrhagic shock.