Endoscopic treatment of congenital H-Type and recurrent tracheoesophageal fistula with electrocautery and histoacryl glue.

OBJECTIVE: Congenital H-Type tracheoesophageal fistulae (H-Type TEF) and recurrent fistulae after primary repair of esophageal atresia represent a difficult problem in diagnosis and management. The treatment traditionally involved an open technique via a cervical or thoracic route, approaches with high morbidity and mortality rates of up to 50%. Endoscopic closure of fistulae has been reported with various techniques such as tissue adhesives, electrocautery, sclerosants and laser. However, the published case series contain a small number of patients with usually short-term follow-up. The aim of this paper is to present the experience of a decade at Toronto's Hospital for Sick Children, using diathermy and histoacryl tissue adhesive and discuss the indications and limitations of this technique. METHODS: Since 1995, 192 patients have been managed in this institution with tracheoesophageal fistulae of which 10 patients have been treated endoscopically. The fistulae were both of H-Type and recurrent tracheoesophageal fistulae following surgery for esophageal atresia and fistula division. One fistula occurred following trauma. The procedure was undertaken under general anesthesia in the image guided therapy suite under fluoroscopic control. Flexible ball electrocautery and injection of histoacryl glue were used either on their own or in combination. RESULTS: Fistula closure was achieved in 9 out of 10 fistulae. Four patients had a second endoscopic procedure. No major respiratory or other complications were encountered in association with the procedure. Follow-up has been between 3 months and 9 years. CONCLUSION: We conclude, endoscopic treatment of tracheoesophageal fistulae with electrocautery and histoacryl glue has been a safe and successful technique of managing H-Type and recurrent tracheoesophageal fistulae. In this paper, we discuss the indications and the surgical steps of the procedure. We highlight that diathermy should be carefully controlled and applied preferably in the small non-patulous fistulae. A fistula that has not closed after two endoscopic attempts is not suitable for further endoscopic treatment and therefore an external approach should be recommended.

Prenatal ultrasonography and neonatal imaging of complete cleft sternum: a case report.

The prenatal diagnosis of a complete cleft sternum was established in a fetus of a twin pregnancy at 22 weeks' gestation. We present the prenatal imaging and correlation with postnatal magnetic resonance imaging and high-resolution ultrasonography. Thinned and depressed midline anterior chest wall transmitting the cardiac pulsation was the clue to the diagnosis, and the defective sternum could be identified on close sonographic observation. Successful surgical correction was undertaken at 2 months of age. There were no major associated abnormalities such as ectopia cordis or midline abdominal wall defects.

Correlation between radiographic transition zone and level of aganglionosis in Hirschsprung's disease: Implications for surgical approach.

BACKGROUND/PURPOSE: The anticipated level of aganglionosis can influence the surgical approach to Hirschsprung's disease. The aim of this study was to determine the accuracy of the contrast enema in predicting this level. METHODS: Over a 6-year period (1995 through 2000), 88 patients with Hirschsprung's disease underwent surgical correction. Preoperative contrast enema findings were available for 75 of these patients and were compared with operative and pathology reports. Data were analyzed by chi(2). RESULTS: The contrast enema showed a transition zone suggestive of Hirschsprung's disease in 67 of 75 patients (89%). In 59 of 67 (88%), the pathologic and radiographic transition zones were concordant. Seven of the 8 patients with discordant studies had total colonic (n = 5) or long-segment (n = 2) disease. Contrast enema correctly predicted the level of aganglionosis in 55 of 62 (89%) patients with rectosigmoid disease but only 4 of 13 (31%) of those with long-segment or total colonic disease (P <.01). Of the patients with a radiographic transition zone in the rectosigmoid, 54 of 60 (90%) had a matching level of aganglionosis. CONCLUSIONS: In rectosigmoid Hirschsprung's disease, the location of the radiographic transition zone correlates accurately with the level of aganglionosis in 90% of cases. However, the small incidence of discordance between anticipated level of aganglionosis and operative findings should be recognized, particularly when planning a one-stage transanal pull-through.

Corrosive esophagitis in children: a 30-year review.

Caustic ingestion with resultant corrosive esophagitis in the pediatric population comprises a heterogeneous group in terms of offending agent, location of burn and the degree of damage. Consequently, the treatment of these injuries has also varied over the years and the optimal management remains controversial. Another area of concern that is seldom reported in the otolaryngology literature is the socioeconomic impact of such injuries both on the child and on the family. We report a 30-year retrospective review of aerodigestive tract caustic injuries at The Hospital for Sick Children. Eighty patients were identified with an age range from 1 month to 16 years. Early and late complications are reviewed including 23 patients (29%) that developed medical complications and 16 patients (20%) that developed severe esophageal strictures. Five of these children required repeat prograde dilatation, while 11 children required gastrostomy and stringing with subsequent retrograde dilatation. All the 11 children required esophageal replacement surgery. The economic and social consequences to the child and family were also noted with particular attention to hospital costs, parental absenteeism from work, and the need for job relocation. A partial cost analysis using a child with a severe injury is presented as an example. Psychological aspects such as attempted murder, jail terms and attempted suicides are also documented. We also present an analysis of those children requiring esophageal replacement surgery, and examine the possible role of esophageal stents in recalcitrant strictures.

A longitudinal analysis of the pediatric surgeon workforce.

OBJECTIVE: To describe the trends in the pediatric surgeon workforce during the last 25 years and to provide objective data useful for planning graduate medical education requirements. SUMMARY BACKGROUND DATA: In 1975, the Study on U.S. Surgical Services (SOSSUS) was published, including a model to survey staffing. A pediatric surgeon workforce study was initiated in conjunction with SOSSUS as a population, supply, and need-based study. The study has been updated every 5 years using the same study model, with the goals of determining the number and distribution of pediatric surgeons in the United States, the number needed and where, and the number of training programs and trainee output required to fill estimated staffing needs. This is the only such longitudinal workforce analysis of a surgical specialty. METHODS: Questionnaires were sent to 100 pediatric surgeons representing the 62 standard metropolitan statistical areas (SMSAs) in the United States with a population of 200,000 or more to verify the names and locations of all active pediatric surgeons and to gain information about the 5-year need for new pediatric surgeons by region. A program was developed to predict the number of pediatric surgeons relative to the total population and the 0-to-17-year-old population in the subsequent 30 years using updated data on the present number and ages of pediatric surgeons, age-specific death and retirement rates, projections of U.S. population by age group, and varying numbers of trainees graduated per year. As each 5-year update was done, previous projections were compared with actual numbers of pediatric surgeons found. The trends during the last 25 years were analyzed and compared and additional information regarding the demographics of practice, trends in reimbursement, and volume and scope of surgery was obtained. RESULTS: The birth rate has been stable since 1994. The 0-to-17-year-old population has been increasing at 0.65% per year; a 0.64% annual rate is projected to 2040. At present, 661 pediatric surgeons are distributed in every SMSA of 200,000 or more population, with an average age of 45 and an average age of retirement 65. The actual number of pediatric surgeons in each 5-year survey has consistently validated previous projections. Trainee output has increased markedly in the past 10 years. The rate of growth of the pediatric surgeon workforce at present is 50% greater than the forecasted rate of increase in the pediatric age group, and during the past 25 years the rate of growth of the pediatric surgeon workforce has been double that of the pediatric population growth. Nationally, significant changes in reimbursement, volume of surgery, and demographics of practice have occurred.

Traumatic diaphragmatic injuries in infants and children: imaging findings.

OBJECTIVES: Traumatic diaphragmatic injuries (DI) in infants and children are uncommon and are often associated with multiple severe injuries. Delayed presentation can be life threatening due to organ herniation and strangulation. We present the imaging findings in a relatively large population of children who experienced this rare injury. METHODS: Medical records of all patients admitted to our Trauma Service from 1977 to 1998 with DI were retrospectively reviewed recording imaging, clinical and surgical or autopsy findings. RESULTS: Of sixteen patients with DI (7 females, 9 males; age 3 weeks to 15 years), 14 suffered from blunt trauma secondary to high-energy impact, and 2 from penetrating injuries. Unilateral DI occurred equally on each side, with one bilateral injury. Associated injuries, present in 81%, included severe head injuries, visceral, mesenteric and vascular injuries and multiple fractures. Six patients died from multiple organ failure (3), head injury (2), and shock (1). Findings in the initial chest X-ray suggested the diagnosis in 13 (81%) of 16 injuries, and CT demonstrated irregularity and thickening of the diaphragm in 4 out of 7. CONCLUSIONS: Plain film findings suggested the diagnosis in most; CT and MR were useful adjuncts. High index of suspicion and awareness of the mechanism of injury can lead to prompt diagnosis, early repair, and decreased morbidity and mortality.

Near-total intestinal aganglionosis: long-term follow-up of a morbid condition.

PURPOSE: The aim of this study was to understand the long-term outcome of near-total intestinal aganglionosis (NTIA). METHODS: The study was an institutional review of all patients with NTIA between 1974 and 1999, inc!usive. Seven full-term babies were identified (4 boys, 3 girls). Mean birth weight was 3.2 kg (range, 2.7 to 4.1). Patients included 1 pair of siblings and 1 Down's syndrome baby. All 7 had obstruction within 5 days of life. One patient experienced bowel perforation. All 7 had NTIA diagnosed within the first 2 months of life. In 2 of 7, aganglionosis began 10 cm distal to the ligament of Treitz (LOT). The longest segment of normal small bowel was 130 cm from the LOT. Five of 7 babies underwent a stoma between day of life (DOL) 1 and 15. RESULTS: Two of three "long-term" survivors (>2 years) had corrective surgery by 13 months. The first received extended jejuno-ileal myotomy and myectomy with multiple postoperative problems. She died at 8 years of age of complications of total parenteral nutrition (TPN). The second underwent myotomy, resection and patch graft to jejunum, and several jejunostomy revisions. All of the involved bowel eventually was resected. The 3 of 7 who lived more than 1 year all took some nutrition orally. Three died, between 3 months and 8 years of age, of complications of TPN. There are 2 survivors at 3 and 7 years of age. Survival has not correlated with the length of aganglionic bowel. CONCLUSIONS: Aganglionosis involving most of the bowel has a high morbidity and mortality rate. Since 1990 a more aggressive surgical approach has resulted in improved survival rates but with significant morbidity. For children surviving beyond 3 months of age, outcome was less dismal. Some patients may benefit from extended jejunal myotomy or myectomy. However, postoperative complications are the rule, not the exception.

The late nonfunctioning duodenal atresia repair--a second look.

BACKGROUND/PURPOSE: In 1986, the authors reported on 3 newborns who had repair of their duodenal atresia, and between 6 and 18 months postoperatively an anastomotic obstruction developed in each suddenly. After prolonged medical and surgical treatments it became apparent that the duodenal atresia repair was functionally obstructed and plication of the dilated atonic proximal duodenum was curative. Since then, 2 more patients became so obstructed at 5 and 24 years postoperatively. The aim of this study was to report the very late occurrence of a functional obstruction of a newborn duodenal atresia repair. METHODS: The 2 additional histories and surgical repairs were reviewed. RESULTS: The 5-year old boy was cured immediately with plication only of his dilated proximal duodenum. The 24-year-old nurse had a very stormy 2-year course with several bypass operations, which did not relieve her abdominal pain and bile vomiting until they were taken down and her dilated proximal duodenum was plicated, after which she made a good recovery. Both remain well. CONCLUSION: An uncommon, very late, sudden, apparently anastomotic, postoperative, newborn, duodenal atresia repair obstruction caused by proximal, dilated, duodenal atony, and dysfunction can occur many years later and responds to duodenal plication alone.

The management of nonpigmented gallstones in children.

BACKGROUND/PURPOSE: An increasing number of children are diagnosed with gallstones today. The best management of nonpigmented gallstones in children without hematologic disorders is not known. METHODS: The authors prospectively studied 74 children with cholelithiasis diagnosed with ultrasonography. Clinical presentation, natural history, complications, and indications for cholecystectomy were examined. The follow-up (mean, 21 months) consisted of routine clinic visits, chart reviews, and telephone questionnaires with the children or their parents. RESULTS: Of the 74 children, 33 required cholecystectomies, and 41 were followed. The average age was similar in the 2 groups (11.7 v 11.0 years). Children with risk factors for cholelithiasis required earlier surgical treatment (P < .001). In the operative group, 8 presented acutely and 25 electively. There were 2 complications, a wound infection and a retained common duct stone. In the group that underwent follow-up, 34 of 41 children remained asymptomatic or had symptoms improve with dietary manipulation. No complications developed during the follow-up period. CONCLUSIONS: Children with gallstones and typical symptoms of right upper quadrant or epigastric pain with food intolerance should undergo cholecystectomy. Eighty-two percent of children with cholelithiasis and atypical symptoms had improvement with dietary manipulation. Pediatric patients with gallstones that are asymptomatic or associated with atypical symptoms can be safely followed without complications.

What have we learned about traumatic diaphragmatic hernias in children?

BACKGROUND/PURPOSE: Diaphragmatic injuries have been reported to be a predictor of serious associated injuries in trauma and a marker of severity. The aim of this retrospective study was to identify pitfalls in the diagnosis and treatment of these injuries in children. METHODS: Data were collected from all patients admitted to the trauma service with traumatic diaphragmatic hernias for the period of January 1977 to August 1998. The authors evaluated 15 cases of traumatic diaphragmatic rupture (6 girls and 9 boys). RESULTS: Mean age was 7.5 years (range, 3 weeks to 15 years). Thirteen patients suffered from blunt trauma, and 2 patients suffered from penetrating trauma. The right and left hemidiaphragms were injured equally (7 patients each), with 1 additional patient suffering from bilateral injuries. All but 1 patient had laparotomies for trauma (n = 14). The diagnosis was made preoperatively in 8 patients (53%) with just a chest radiograph. Computed tomography (CT) scan, magnetic resonance imaging (MRI), and oral contrast studies were used as ancillary tests to diagnose traumatic diaphragmatic rupture. There were 3 missed injuries. Associated injuries included liver lacerations (47%), pelvic fractures (47%), major vessels tear (40%), bowel perforations (33%), long bone fractures (20%), renal lacerations (20%), splenic lacerations (13%), and closed head injuries (13%). The mean hospital stay was 20 days (range, 7 to 60 days). Complications were observed most commonly in those patients with multiple injuries and included postoperative ileus (40%), pneumonia (30%), pancreatitis (20%), wound infection (20%), intestinal obstruction (20%), cholestasis (10%), and renal failure (6%). Five deaths (33%) were caused by hemorrhagic shock, respiratory failure, coagulopathy, and refractory acidosis. CONCLUSIONS: Traumatic diaphragmatic hernias usually are associated with serious injuries in children. It is important to combine a high index of suspicion with radiological diagnostic tests in patients at risk. Palpation and/or visualization of both diaphragms at laparotomy is extremely important in detecting these injuries when they are not suspected preoperatively.

Gastric perforation attributable to liquid nitrogen ingestion.

Despite the widespread use of liquid nitrogen in medicine and industry, there are only a few reports of injuries associated with its use. We report a case of a 13-year-old boy who developed gastric perforation after liquid nitrogen ingestion. This is a previously unreported complication.

Posttraumatic torsion of accessory lobe of the liver and the gallbladder.

Torsion of an accessory lobe of the liver and of the gallbladder is a rare etiology for acute abdominal pain in children and infants. We report a case of an 8-year-old girl who was admitted with acute epigastric pain and vomiting, after her brother had jumped on her back. Physical examination revealed an afebrile child with a nontender right upper quadrant (RUQ) mass. Color Doppler ultrasound and contrast-enhanced CT demonstrated a heterogeneous, avascular mass with displacement of a thickened-wall gallbladder. A contorted, congested accessory lobe of the liver and the gallbladder were resected at laparotomy. Imaging and operative findings are presented and a differential diagnosis is discussed in order to increase awareness of this rare condition.

The morbidity and mortality of pediatric splenectomy: does prophylaxis make a difference?

PURPOSE: The aim of this study was to analyze the incidence of postsplenectomy sepsis morbidity and mortality after prophylaxis, in comparison with our previous 13-year study (1958 to 1970, inclusive). METHODS: All patients who had splenectomy at the Hospital for Sick Children, Toronto, between 1971 and 1995, inclusive (to give a minimum of 2 years for follow-up), were reviewed for infection and mortality. The criterion for classifying a patient as "infected" was the recovery of an invading encapsulated organism from the blood culture in a patient admitted to the hospital. RESULTS: Of the 264 patients studied, 10 had a postsplenectomy infection (3.8%); nine occurred in patients who underwent splenectomy between the ages of 0 and 5 years. Infection took place within 2 +/- 3 years (mean +/- SD) after splenectomy for the immunized patients and 11 +/- 5 days (mean +/- SD) for the nonimmunized children. A significant number of patients were admitted for an apparent respiratory infection, but no serum organisms were isolated. One died of overwhelming sepsis, but the responsible organism was not identified. CONCLUSION: Although there has not been a decrease in the number of splenectomies performed per year, the incidence of infection and mortality has decreased by 47% and 88%, respectively, with prophylaxis.

Unsuccessful air-enema reduction of intussusception: is a second attempt worthwhile?

Pneumatic reduction of idiopathic intussusception is successful in about 80% of cases, while 60% of the failures are reduced at surgery without resection. To determine whether delayed, repeated attempts at enema reduction of failures would reduce the need for operation in selected cases, over a 2-year period (1994-1996 inclusive), 17 infants with idiopathic intussusception underwent delayed repeat enemas 2-19 h following the first failed attempt at reduction. Clinical parameters and radiologic findings were evaluated with respect to outcome. Ten intussusceptions were successfully reduced after the second attempt in 9 and after the fourth attempt in 1. Seven children underwent a laparotomy, 5 because of failure of progressive reduction at air enema (AE). Two were taken to surgery early in the series, 1 because of perforation during a second attempt and 1 while awaiting a third reduction attempt. The 10 successful reductions all showed progressive movement of the intussusceptum on each AE; the 2 who perforated failed to show progressive reduction on their second AE. Because of these cases, the remaining 5 were referred to surgery because of failure of progressive reduction of the intussusceptum on the second attempt. At laparotomy, of the 7 unsuccessful reductions, 4 required resection and 3 had difficult manual reduction. The presence of vomiting, a mass, and/or bloody stools were not predictors of outcome. Failures had higher body temperatures (38.1 +/- 0.3 vs 37.4 +/- 0.1 degrees C, P = 0.07), heart rates (153.7 +/- 8 vs 136.9 +/- 2.1 min, P = 0.03), and longer duration of symptoms (36.8 +/- 4 vs 21.3 +/- 3.6 h; P = 0.01) than successes. Delayed repeat AEs may be safe and effective in selected cases of idiopathic intussusception, but should be considered only if significant movement of the intussusceptum is noted at each attempt. The ideal time for repeat AE reduction prior to surgery is not established, but 2-4 h appears appropriate. Pyrexia, tachycardia, and duration of symptoms greater than 36 h are relative contraindications to this course of management.

Nonocclusive small bowel infarction in familial dysautonomia syndrome.

The authors report a case of a 14-year-old boy with familial dysautonomia (FD) in whom a small-bowel infarction developed during a dysautonomic crisis. Atypical features of the presentation included hypotension with prolonged fever and abdominal distension. The authors postulate that the bowel infarction was caused by prolonged hypoperfusion. It is recognized that the small bowel in normal subjects can adapt to periods of ischemia without irreversible injury. The authors speculate that the known abnormal systemic cardiovascular regulation in patients with FD may adversely affect splanchnic blood flow, which led to the catastrophic consequences in this case. This report draws attention to the risk of significant ischemic complications during a dysautonomic crisis, especially in the face of atypical features, and emphasizes the challenging cardiovascular management of such patients.

The role of peritoneal drains in treatment of perforated necrotizing enterocolitis: recommendations from recent experience.

BACKGROUND/PURPOSE: Intraperitoneal drains have been used in the treatment of perforated necrotizing enterocolitis (NEC), especially in infants less than 1,000 g, yet their role is still debated. The authors wished to examine their more recent experience in the treatment of NEC to make recommendations for operative management. METHODS: The authors reviewed the records of all infants seen between 1989 and 1995 who had clinical and radiological evidence of NEC at the Hospital for Sick Children, Toronto, Ontario. One hundred sixty-seven infants were treated for NEC and 73 (44%) infants required surgical intervention. RESULTS: Forty-five patients had perforated NEC; 23 were treated initially by peritoneal drainage and 22 by primary laparotomy. The 23 newborns who had peritoneal drainage were of significantly lower birth weight, and 19 (83%) of these infants required subsequent laparotomy for worsening disease. The overall mortality rate for perforated NEC was 36%. CONCLUSION: Insertion of a peritoneal drain is still useful in resuscitating small critically ill infants with NEC; however, the majority of these infants will also require laparotomy.