Roux-en-Y jejunostomy button in infants.

Establishing enteral feeding in high-risk infants with significant gastroesophageal reflux is a difficult challenge. Some patients are considered at very high risk for fundoplication and gastrostomy due to unstable medical conditions, dense upper-abdominal adhesions due to previous surgical procedures, or unfavorable anatomy. We describe a less invasive operation that provides jejunal feeding in a way that is safe, reliable, and convenient for the family.

Transanal one-stage Soave procedure for infants with Hirschsprung's disease.

PURPOSE: Many centers perform a one-stage pull-through procedure for Hirschsprung's disease (HD) diagnosed in infancy. The authors have developed a one-stage pullthrough procedure using a transanal approach that eliminates the need for intraabdominal dissection. METHODS: Nine children aged 3 weeks to 18 months with biopsy-proven HD underwent a transanal pull-through procedure over a 13-month period. A rectal mucosectomy was performed starting 0.5 cm proximal to the dentate line, and extending proximally to the level of the intraperitoneal rectum. In the first eight children, intraperitoneal position was confirmed with a laparoscope placed through a 3- to 5-mm port in the base of the umbilicus. The muscular sleeve was divided circumferentially to allow full-thickness mobilization of the rectosigmoid junction. Manual transanal traction permitted direct visualization and division of mesenteric vessels with transanal mobilization above the transition zone. Ganglion cells were confirmed by frozen section, and the bowel was transected. The rectal muscular cuff was divided longitudinally, and the anastomosis was completed. The laparoscope confirmed orientation and adequate hemostasis. In a ninth patient, the identical procedure was performed, but with the laparoscope used only for confirmation at the end of the procedure. RESULTS: Operative time, including frozen sections, averaged 194 minutes (range, 169 to 250 minutes), and the average length of bowel resected was 12 cm (range, 7.5 to 22 cm). Four of the nine patients were discharged on postoperative day (POD) 1, four on POD 2, and one patient with Down's syndrome was discharged on POD 6. Median follow-up was 6 months (range, 3 to 14 months). One death occurred 2.5 months postoperatively secondary to sudden infant death syndrome. Complications included postoperative apnea spells (n = 1), mild enterocolitis (n = 2), constipation (n = 1), anastomotic stricture(n = 1), and muscularcuff narrowing (n = 1); each responded to nonoperative management. Stool output has ranged from four to eight per day. CONCLUSION: A one-stage pull-through for HD can be performed successfully using a transanal approach without intraperitoneal dissection. This procedure is associated with excellent clinical results and permits early postoperative feeding, early hospital discharge, and no visible scars.

Management of uncomplicated skull fractures in children: is hospital admission necessary?

OBJECTIVE: This study was undertaken to determine the necessity for routine hospital admission of children with skull fractures, a normal neurological exam, a normal head CT, and no other injuries ('uncomplicated skull fracture'). METHODS: A prospective study of closed-head injuries in children was done over a 2-year period at St. Louis Children's Hospital. All patients with closed head injuries underwent skull radiographs and a head CT scan. From this cohort, children with uncomplicated skull fractures were identified and studied. For comparison, a retrospective analysis was also performed of the hospital admission records of children admitted over a 5-year period (1990-1994) with the diagnosis of epidural hematoma (EDH) to identify the typical time intervals between injury and documentation of the lesion in these cases. RESULTS: Forty-four patients with uncomplicated skull fractures were identified; all had been admitted for observation. Mean age was 1.8 years. Average time between injury and hospital admission was 6.35 h with half of this time being spent in the emergency room. Average LOS was 35 h, but 50% of patients were hospitalized less than 24 h. No patient in this study group suffered a complication related to their inury. Twenty-three patients with EDH had been admitted during the 5-year review period. Slightly more than one-half of patients had their EDH detected within 6 h of injury. The others were diagnosed more than 6 h after injury due to a delay in medical evaluation or a delay in obtaining a computed tomographic (CT) scan after an initial medical evaluation. CONCLUSIONS: Patients with uncomplicated skull fractures, in the absence of recurrent emesis and/or evidence of child abuse, can be considered for discharge home. The definition of an uncomplicated skull fracture requires that a head CT be performed on these patients.

Timing of video-assisted thoracoscopic debridement for pediatric empyema.

BACKGROUND: Video-assisted thoracoscopic debridement (VATD) is a new method of managing pediatric empyema. The purpose of this retrospective study was to determine the relation between the timing of VATD and its success in avoiding the need for open decortication. STUDY DESIGN: Twenty-one children aged 3 to 16 years (mean, 8 years) with symptomatic, loculated, parapneumonic empyema were treated with VATD at two tertiary pediatric centers between 1994 and 1997. The preoperative duration of symptoms, hospitalization, and previous need for thoracostomy drainage were compared between patients having VATD only and those who subsequently required a thoracotomy and decortication. Statistical analysis used the Wald chi-square test or Fisher's exact test with p < 0.05 considered significant. RESULTS: Video-assisted thoracoscopic debridement was successful in 15 patients (group 1) and unsuccessful in six patients (group 2), who required a thoracotomy and decortication. Group 1 had a shorter mean duration of preoperative symptoms (13 versus 27 days; p=0.03), a shorter median duration of preoperative hospitalization (6 versus 18 days; p=0.04), and a lower incidence of previous thoracostomy drainage (4/15 versus 5/6; p=0.05). CONCLUSIONS: The technique of VATD is most likely to be successful when used within one week of diagnosis of a loculated parapneumonic empyema. A prospective trial comparing VATD with intrapleural fibrinolytic agents for the initial treatment of pediatric empyema is needed.

Histopathologic analysis of interval appendectomy specimens: support for the role of interval appendectomy.

The treatment of appendiceal abscess is controversial. For patients initially treated "conservatively" with antibiotics with or without drainage, the role of interval appendectomy is an area of considerable debate. Without interval appendectomy, the true risks of recurrent disease and missed pathological findings are uncertain, and large, long-term, prospective studies are unavailable. To evaluate the role of interval appendectomy, the authors reviewed the histopathologic specimens from patients with presumed appendiceal abscess treated by interval appendectomy. Over a 7-year period, 162 children presented with a clinical diagnosis of perforated appendicitis. Eighteen patients had localized abscesses treated conservatively, followed by interval appendectomy. Standard histopathologic sections of 17 of the 18 appendices were examined by one pathologist who was blinded to the clinical data and to the interpretation of the original pathologist. Of the 11 boys and seven girls (mean age, 7.4 +/- 3.4 years), eight underwent percutaneous drainage and one underwent operative drainage. All received intravenous antibiotics for a mean of 8.6 +/- 3.2 days with a hospital stay of 10.4 +/- 8.3 days. Interval appendectomy was performed at a mean of 92.7 +/- 20.7 days after initial admission, with discharge at a mean of 2 +/- 1.3 days after surgery. There were no complications or deaths. Histopathologic review showed normal appendix (n = 4), normal appendix with mild serositis (n = 6), normal appendix with unsuspected resolved Meckel's diverticulitis (n = 1), appendiceal duplication (n = 1), granulomatous appendicitis (n = 3), and acute appendicitis (n = 2). All appendices had patent lumens, and 15 were documented to be present to the tip. There was no correlation between the histopathologic findings and the interval between abscess and interval appendectomy. Interval appendectomy was performed with no morbidity and a short hospital stay. Two patients had histopathologic recurrent acute appendicitis, five had unsuspected pathological findings (appendiceal duplication, Meckel's diverticulitis, granulomatous inflammation), and none of the appendices had an obliterated lumen, suggesting that all patients were at long-term risk for recurrent disease. These data support the role of interval appendectomy in cases of perforated appendicitis treated conservatively.

Diagnosis of Hirschsprung's disease.

OBJECTIVE: Our aims were to determine the validity of using low-osmolality water-soluble contrast enemas (WSCE) in neonates and infants with suspected Hirschsprung's disease (HD) and to devise a scoring system that uses a checklist of radiologic signs to determine the probability of HD. MATERIALS AND METHODS: The records of all patients referred by pediatric surgeons from 1988 through 1992 for the radiologic investigation of possible HD were retrospectively reviewed. Thirty-eight patients who were from 2 days to 9 months old were studied; 20 of them were neonates (less than 1 month old). Of all the patients, 24 underwent WSCE and the other 14 underwent barium enema. For all patients, HD had been diagnosed by rectal biopsy or excluded by biopsy, clinical follow-up, or both. Radiographs were read by a gastrointestinal radiologist who used a checklist of diagnostic criteria reported in the literature. The sensitivity had specificity of the findings were compared with those in the literature. RESULTS: Of the 18 patients with HD, 12 were neonates. All reported radiologic diagnostic criteria were seen; the frequency, sensitivity, and specificity of the findings were reported. Twenty percent (n = 2) of HD patients in the WSCE group (n = 10) had negative findings. Two of the 12 neonates developed colonic perforation, one during the enema and the other within 24 hr of the procedure. CONCLUSION: WSCE has a sensitivity and specificity equivalent to those of the barium enema for the detection of HD. For the two patients with perforation, the use of WSCE was of considerable benefit, avoiding the problems associated with barium spillage into the peritoneal cavity. A scoring system for diagnostic enemas is feasible.

One-stage versus two-stage Soave pull-through for Hirschsprung's disease in the first year of life.

Several investigators have reported good results after a one-stage Soave procedure without a stoma for infants with Hirschsprung's disease. The authors reviewed their concurrent experience with the one- and two-stage approaches, comparing the two groups with respect to rate of complications and clinical outcome. Over a 3-year period, 36 infants with colonic Hirschsprung's disease presenting in the first year of life were treated with a Soave pull-through. Thirteen had a one-stage pull-through, and 23 had a two-stage procedure using an initial stoma. There was no difference with respect to median age at time of diagnosis, median follow-up period, length of aganglionosis, or male:female ratio between the groups. The incidences of major complications such as small bowel obstruction, segmental or acquired aganglionosis, anastomotic leak, and malabsorption were equal between the two groups. However, 13% of the two-stage patients required revision of the stoma. All major complications in the one-stage group were in those who weighed less than 4 kg at the time of surgery. Minor complications such as wound infection, perianal excoriation, and need for repeated dilatation were similar between the groups, but minor stoma-related complications (prolapse or retraction) occurred in 26% of the two-stage infants. When complications were stratified using a more sophisticated scale of severity, no significant difference was found between the groups. The overall complication rate was 1.5 events per patient in the one-stage group and 2.0 events per patient in the two-stage group. This small difference was related to the presence of a stoma in the two-stage group. Overall, 10 of 12 survivors in the one-stage group and 22 of 23 in the two-stage group were doing well, with normal bowel function noted on long-term follow-up (mean period, of 14 and 19 months, respectively). Both one- and two-stage approaches were associated with a significant complication rate, although long-term outcome was excellent in both groups. The higher complication rate in the two-stage group was attributable to the presence of a stoma. For small infants, it may be beneficial to delay the one-stage pull-through until weight exceeds 4 kg.

Fryns syndrome: a rare familial cause of congenital diaphragmatic hernia.

Fryns syndrome is a rare autosomal recessive disorder characterized by diaphragmatic hernia and multiple anomalies. Almost all infants have died at birth, and survivors have had severe mental retardation. The authors report on a family in which three children had diaphragmatic hernia diagnosed prenatally. The first child died of severe pulmonary hypoplasia in the neonatal period. The second survived after diaphragmatic hernia repair, and was found to have Fryns syndrome based on the spectrum of associated anomalies and the family history. He has done well except for significant gastroesophageal reflux, mild developmental delay, and mild hypotonia. The third child's diaphragmatic hernia was diagnosed early during fetal life, and the parents chose to terminate the pregnancy. These cases illustrate the spectrum of Fryns syndrome and the importance of a family history in patients with congenital diaphragmatic hernia. This is the first report of survival of a patient with Fryns syndrome without severe mental retardation.

Pneumatic reduction of intussusception using carbon dioxide.

Pneumatic reduction using air has recently become popular for the initial non-surgical management of intussusception. Since carbon dioxide (CO2) is rapidly absorbed from body surfaces, it should theoretically result in less cramping and distension following reduction. We reviewed our recent experience with the pneumatic reduction of intussusception using CO2 in 26 children. In 22 of these the intussusception was reduced (85%). There was one performation with CO2; the patient did not suffer any postoperative complications. Five additional children who had been treated unsuccessfully with barium had intussusception subsequently reduced with CO2. Following CO2 reduction, most children were fed within hours, and there were no instances of significant abdominal distension or cramping. We conclude that pneumatic reduction of intussusception using CO2 is safe and effective, and has the theoretical advantage of more rapid absorption from the gastrointestinal tract than air.

The single-subject randomized trial. A useful clinical tool for assessing therapeutic efficacy in pediatric practice.

The purpose of a single-subject randomized trial is to assess objectively the efficacy of a specific therapeutic intervention in an individual patient. Treatment is randomly alternated with placebo over a number of study periods. Specific outcome measures are recorded blindly and later compared via paired statistical analysis. Single-subject trials have long been successfully performed in adults, but rarely in children. We present single-subject trials of two pediatric patients done to assess the effect of cisapride on symptoms arising from gastroesophageal reflux. In the first patient, the drug affected neither vomiting nor gagging, although stool frequency increased. Since the symptoms of concern were unaffected, cisapride was discontinued. In the second patient, use of cisapride led to a significant decrease in vomiting and wheezing; the drug was therefore incorporated into the therapeutic regimen. Single-subject randomized trials are inexpensive and simple and can be used by the family physician, pediatrician, or pediatric surgeon in daily practice. They permit the rational use of effective therapy and the abandonment of ineffective measures.

Optimal management of patent ductus arteriosus in the neonate weighing less than 800 g.

Between January 1988 and December 1990, 132 neonates weighing < 800 g were admitted to our neonatal intensive care unit. Of the 76 who survived initial resuscitation, 42 had developed a hemodynamically significant patent ductus arteriosus (PDA) (mean +/- SD): gestational age 25.3 +/- 1.9 weeks, birth weight 650 +/- 93 g. Two infants were referred for primary surgical ligation because of contraindications to indomethacin. Forty infants were initially treated with indomethacin. Seventeen of 40 (43%) were subsequently referred for surgical ligation because of indomethacin failure. Infants requiring surgical duct closure were a lower gestational age (24.6 +/- 1.3 v 25.7 +/- 2.0 weeks, P = 0.49) and had a greater left atrial-aortic (LA/Ao) ratio on echocardiography (1.71 +/- 0.28 v 1.46 +/- 0.26, P = .04) compared with those treated successfully with indomethacin. There were 6 deaths (15%), all of which occurred in infants receiving indomethacin (5 indomethacin alone, 1 indomethacin+ligation). Indomethacin was directly associated with intestinal perforation in 3 patients, and acute renal failure in 1; all 4 died. Surgery was associated with minimal morbidity (intraoperative transfusion in 1, postoperative pneumothorax requiring chest tube in 1). These data suggest that in the extremely premature neonate with a hemodynamically significant PDA: (1) indomethacin therapy is associated with a high failure rate and significant complications; (2) PDA associated with a large LA/Ao ratio is unlikely to close with indomethacin therapy; and (3) surgical duct closure is associated with minimal morbidity. We conclude that primary surgical ligation may provide the optimal management for PDA in carefully selected patients.

Home esophageal self-dilatation in children.

Home esophageal self-dilatation permits greater independence and more frequent treatments than intermittent hospital procedures for patients with esophageal strictures. The article describes an innovative program for teaching esophageal self-dilatation to preadolescent children, using a form of creative visualization. The child focuses on a 2-minute mental image (e.g., video game, a popular song, or swimming a race) from his/her own experience while passing a Maloney bougie. A nasal breathing technique is used, and active encouragement is provided. Topical lidocaine and oral diazepam can be used initially to assist relaxation and are stopped when the child gains confidence with the technique. The authors taught esophageal self-dilatation to three children aged 12 to 13 years with esophageal strictures. All three learned the technique within 1 week and continued it at home for between 4 and 6 months. Two of the children were taught in hospital by a nurse, and the third was taught as an outpatient by a peer who had already mastered the technique. Home esophageal self-dilatation can be successfully taught to children in the preadolescent age group. The teaching program is simple and effective and can be easily applied to other tasks such as pain control, nasogastric tube feeding, and self-injection.

Cloacal exstrophy: prenatal diagnosis before rupture of the cloacal membrane.

Embryologically, cloacal exstrophy is thought to result from persistence and subsequent rupture of the infraumbilical cloacal membrane during the fifth embryonic week. We report a case of cloacal exstrophy in which a prenatal diagnosis was made prior to rupture of the cloacal membrane. A routine ultrasound at 17 weeks' gestation demonstrated monoamniotic twins. One twin was normal, but the other was found to have a sacral myelomeningocele, "rocker-bottom" feet, splaying of the pubic rami, and a large cystic mass protruding from the infraumbilical anterior abdominal wall. A repeat ultrasound was performed at 22 weeks, with the same findings. At 26 weeks, further examination showed disappearance of the abdominal cyst, a small omphalocele, no demonstrable bladder, and the suggestion of prolapsed bowel inferior to the umbilical cord insertion. After delivery at 34 weeks, the abnormal twin was found to have the typical findings of cloacal exstrophy, myelomeningocele, bilateral lower limb anomalies, and extremely foreshortened small bowel. Rupture of the presumed cloacal membrane after 22 weeks in this case is inconsistent with our current understanding of the embryology of this anomaly, and should stimulate a reexamination of the current concepts. If the characteristic features are recognized, cloacal exstrophy can be diagnosed by prenatal ultrasound, permitting prenatal counseling and appropriate perinatal management.

False diagnosis of intestinal obstruction in a fetus with congenital chloride diarrhea.

Intestinal obstruction is often diagnosed prenatally by ultrasound, providing an opportunity for prenatal counseling, genetic investigation, and planned delivery at a perinatal center. We describe a patient with typical features of fetal bowel obstruction, who was found at birth to have congenital chloride diarrhea. A 25-year-old white woman had marked polyhydramnios; multiple dilated, fluid-filled loops of intestine were seen in the fetal abdomen on prenatal ultrasound. However, postnatally, there was no evidence of bowel obstruction. The infant girl passed large amounts of watery stools, but tolerated feeds well. A rectal biopsy showed normal ganglion cells. On the fourth day of life her serum sodium and chloride were markedly decreased, and stool chloride levels were diagnostic of congenital chloride diarrhea. She was placed on sodium chloride and potassium chloride supplements, and her serum electrolytes normalized. Congenital chloride diarrhea is a rare, inherited condition caused by an abnormality of intestinal electrolyte transport. This case illustrates that it may present prenatally with a picture similar to that seen with intestinal obstruction.

Heterotopic gastric mucosa in the gallbladder: a cause of chronic abdominal pain in a child.

A 12-year-old boy presented with a 7-year history of intermittent colicky epigastric pain. Serial abdominal ultrasound studies showed a contracted gallbladder, and single and double dose oral cholecystograms demonstrated nonfunction. All other investigation results were normal. At cholecystectomy, there was a fibrotic stricture at the junction of the gallbladder neck and cystic duct. The small, thin-walled gallbladder contained white bile. Histologically, the gallbladder was lined with normal mucous secreting columnar epithelium. The area of stricture showed diffuse fibrosis with islands of heterotopic gastric mucosa containing all gastric mucosal cell types. One year postoperatively, there have been no further episodes of abdominal pain. This is the seventh reported case of heterotopic gastric mucosa in the gallbladder of a child causing symptoms necessitating cholecystectomy. We recommend that a child with abdominal pain and a nonfunctioning gallbladder be considered for cholecystectomy even in the absence of cholelithiasis.

A prospective evaluation of the Button gastrostomy in children.

The Button gastrostomy has become popular for patients requiring long-term enteral feeding, because it is considered less irritating, more stable and more esthetically acceptable than the traditional tube gastrostomy. By a standardized questionnaire and personal interview, the authors evaluated prospectively the efficacy and complication rate in 19 children who had a Button gastrostomy inserted during a 6-month period. In 15 children the Button replaced a standard tube gastrostomy, and in 4 the Button was inserted surgically initially. Thirteen children had severe neurologic disabilities, and 6 required supplemental enteral feeding as part of the nutritional management of another chronic disease. In all 19 children, the Button was esthetically more acceptable and produced less skin irritation than the standard tube gastrostomy. All but one caregiver thought that the Button gastrostomy was preferable to a tube gastrostomy. This was especially apparent in the six neurologically normal children who were able to be more active and had an improved self-image. Cost analysis showed that, despite the higher initial cost of the Button, elimination of the need for frequent tube changes and hospital visits made it ultimately more cost effective than the standard tube gastrostomy. The authors conclude that the Button gastrostomy is a useful alternative to the standard tube gastrostomy in selected patients. Close long-term follow-up is extremely important to ensure a good result.

The diagnosis of pneumonia in the immunocompromised child: use of bronchoalveolar lavage.

Between January 1987 and December 1988, 26 immunocompromised children (aged 15 months to 17 years) underwent bronchoalveolar lavage (BAL) for evaluation of pneumonia (chemotherapy for malignancy, 12; orthotopic liver transplantation, 9; other hematologic disease, 5). Bilateral diffuse pulmonary disease was present in 25 children. All were receiving broad spectrum antibiotics. In addition, five were receiving antiviral therapy and two were receiving antifungal therapy. Sixteen patients underwent rigid and 10 underwent flexible bronchoscopy. Two lavages of 10 to 20 mL of normal saline were obtained from involved subsegmental bronchi of both lungs in each patient. Second wash samples from each lung were sent for bacterial and viral cultures, silver staining for pneumocystis, and direct electronmicroscopy analysis for viral particles. Samples were considered satisfactory if they contained an abundance of alveolar macrophages and only small numbers of upper respiratory tract epithelial cells. Alveolar macrophages were present in 21 (81%) of the BAL samples. A specific infectious agent was identified in 15 of these patients (cytomegalovirus [CMV], 6; Pneumocystis carinii, 4; gram-positive cocci, 3; Candida albicans, 2), and therapy was modified in 12. In the five patients in whom BAL samples were contaminated with upper respiratory tract cells no infectious agents were isolated. Because of continued clinical deterioration, open-lung biopsies were performed in three patients in whom BAL had identified CMV and in three patients in whom no organisms had been obtained. Lung biopsies did not identify any new infectious agents, although in the latter group specific histological diagnosis of a noninfectious process was made (hemorrhagic infarct, bronchiolitis obliterans, and lymphoma).(ABSTRACT TRUNCATED AT 250 WORDS)

Diagnosis and incidence of cardiac injury in children with blunt thoracic trauma.

Over a 20-month period, we prospectively studied 41 children with blunt thoracic trauma (BTT) to determine the incidence and morbidity of cardiac injury in this population. Four patients died, and the data were incomplete in 10, leaving 27 for analysis. Serial electrocardiograms (ECG) were abnormal in 36% of the patients studied, serial creatine phosphokinase isoenzyme (CK-MB) ratios were elevated in 31%, echocardiogram showed septal dysfunction in 14%, and pyrophosphate scan showed grade 1 uptake in 14%. There was poor correlation among tests, since only four patients had more than one abnormal test. There was no significant difference in Injury Severity Score or Thoracic Abbreviated Injury Scale between patients with and without abnormal investigations. No patient in the study developed arrhythmias or cardiac failure. In 13 trauma-related deaths undergoing autopsy over the same period, including the four with thoracic trauma, none had evidence of cardiac injury. These results indicate a striking lack of consistency in the diagnosis of posttraumatic cardiac injury in children using standard investigations. The absence of adverse cardiac events in surviving patients and the lack of autopsy evidence of cardiac injury in the trauma deaths suggest that the actual incidence and clinical significance of these lesions in children is lower than generally reported. Children with BTT should be followed clinically, and reliance on screening tests should be avoided.