Long-term analysis of children with esophageal atresia and tracheoesophageal fistula.

BACKGROUND/PURPOSE: For children with esophageal atresia (EA) or tracheoesophageal fistula (TEF), the first years of life can be associated with many problems. Little is known about the long-term function of children who underwent repair as neonates. This study evaluates outcome and late sequelae of children with EA/TEF. METHODS: Medical records of infants with esophageal anomalies (May 1972 through December 1990) were reviewed. Study parameters included demographics, dysphagia, frequent respiratory infections (> 3/yr), gastroesophageal reflux disease (GERD), frequent choking, leak, stricture, and developmental delays (weight, height < 25%, < 5%, respectively). RESULTS: Over 224 months, 69 infants (37 boys, 32 girls) were identified: type A, 10 infants; type B, 1; type C, 53; type D, 4; type E, 1. Mean follow-up was 125 months. During the first 5 years of follow-up, dysphagia (45%), respiratory infections (29%), and GERD (48%) were common as were growth delays. These problems improved as the children matured. CONCLUSIONS: Children with esophageal anomalies face many difficulties during initial repair and frequently encounter problems years later. Support groups can foster child development and alleviate parent isolationism. Despite growth retardation, esophageal motility disorders, and frequent respiratory infections, children with EA/TEF continue to have a favorable long-term outcome.

Gastric tonometry and direct intraabdominal pressure monitoring in abdominal compartment syndrome.

BACKGROUND/PURPOSE: Abdominal compartment syndrome (ACS) may complicate abdominal closure in patients with abdominal wall defects, abdominal trauma, intraperitoneal bleeding, and infection. Increased intraabdominal pressure (IAP) leads to respiratory compromise, organ hypoperfusion, and a high mortality rate. This study evaluates the efficacy of continuous direct monitoring of IAP and gastric tissue pH in detecting impending ACS. METHODS: Ten mongrel puppies weighing 2.8 to 6.4 kg underwent general endotracheal anesthesia, placement of an intraabdominal inflatable balloon to simulate ACS and a Swan-Ganz catheter to measure direct IAP. A gastric tonometer, nasogastric tube, foley catheter, and arterial catheter also were inserted. Half-hourly inflation's of the intraabdominal balloon were used to simulate the development of ACS. Direct intraabdominal (IAP), gastric (GP), bladder (BP), and peak airway pressures (PAP) were measured. Gastric tonometry fluid and arterial blood gas levels were obtained during inflation, and the gastric tissue pH level was calculated. Data were statistically analyzed using Pearson's correlation coefficients. RESULTS: Baseline pressures were 2 to 5 cm H(2)O in the stomach and bladder catheters, 1 to 3 mm Hg in the intraabdominal catheter, and correlated with a gastric tissue pH level of 7.4. Significantly high correlation coefficients (cc) were observed between IAP versus BP (cc, 0.77; P <.002). IAP versus GP (cc, 0.79; P <.002) and IAP versus PAP (c, 0.83; P <.0004). A high negative correlation coefficient was noted between gastric pH and IAP (cc, 0.61; P <.026). The pH level dropped to 7.0 with BP and GP of 20 cm H(2)O and IAP of 10 mm Hg, to 6.8 at 30 cm H(2)O and 20 mm Hg, and 6.5 at 40 cm H(2)O and 30 mm Hg, respectively. However, correlation coefficients between gastric tissue pH and BP, GP, or PAP were not significant. CONCLUSIONS: These data suggest that continuous direct intraabdominal pressure monitoring is a simple and effective method that correlates well with indirect bladder or gastric pressure measurement. Changes in gastric tissue pH in association with increased intraabdominal pressure may be an early indicator of impending abdominal compartment syndrome. These observations indicate that these techniques may be more sensitive than current methods of indirect measurement, which may be associated with delayed recognition of ACS.

Portal venous air: the poor prognosis persists.

BACKGROUND/PURPOSE: The prognostic importance of portal vein air (PVA) in babies with necrotizing enterocolitis (NEC) has been controversial. This study compares the outcome in babies with NEC and PVA treated surgically versus those with medical management. METHODS: Forty neonates in the neonatal intensive care unit (NICU; 1995 through 1999) had (PVA) during their hospitalization. Babies were analyzed for gestational age (GA), birth weight (BW), and survival after operative versus medical management. RESULTS: The average GA was 26 weeks, average BW was 1,173 g. Twenty-three patients (57.5%) tolerated full feedings and 8 (20%) partial feedings at diagnosis. All 40 babies required intubation at birth with 23 (57.5%) requiring reintubation with onset of PVA. In all cases, PVA was present within 24 hours of onset of abdominal distension, feeding intolerance, or heme-positive stools. Two cases of PVA "resolved" only to recur later in the patients' courses. Thirty-two patients (80%) manifested pneumatosis intestinalis on abdominal radiographs, and 8 (20%) had perforations. Acidosis was present in 25 (63%) patients, and vasopressor support (dopamine) was required in 15 (38%), with 2 patients requiring support only preoperatively. Initial management consisted of bowel rest, fluid resuscitation, orogastric decompression, and broad-spectrum antibiotics. Operation was performed in 31 (78%). Seventeen underwent resection with ostomy formation with 6 deaths and 11 survivors. Four underwent resection using the clip and drop back method, with one death and 3 requiring an ostomy at second look laparotomy. Ten had NEC totalis and closure of the abdomen only. Overall operative mortality rate was 17 of 31 (54%). Nine seemingly stable patients were treated nonoperatively. Six had progressed disease and died before salvage laparotomy could be performed, whereas 3 (33%) survived without further therapy. CONCLUSIONS: PVA has been a relative indication for operation. This view has been challenged by the survival of some patients without laparotomy. Although nonoperative therapy seems appealing in hemodynamically stable patients without acidosis, our data confirm the poor prognosis of infants with PVA and NEC.

The utility of lung biopsy in recipients of stem cell transplantation.

BACKGROUND/PURPOSE: Pulmonary infiltrates in recipients of stem cell transplantation often present as diagnostic dilemmas. Although lung biopsy may establish the diagnosis of parenchymal disease, it remains unclear whether such a procedure results in a significant change in the patient's treatment and outcome. This study evaluates the efficacy of lung biopsy in recipients of stem cell transplantation. METHODS: The medical records of 15 stem cell transplant recipients who underwent 18 lung biopsies were reviewed. The indications for stem cell transplantation were leukemia in 10 patients, lymphoma in 2, histiocytosis in 1, neuroblastoma in 1, and Ewing's sarcoma in 1. The results of the lung biopsies were correlated to the clinical management and outcomes. RESULTS: The overall mortality rate was 67% (10 patients). Eight of the 9 patients who required mechanical ventilatory support at the time of lung biopsy died. The pathologic diagnoses were pneumonitis in 6 biopsies, fibrosis in 6, brochiolitis obliterans organizing pneumonia in 3, hemorrhage in 2, and infarction in 1. Therapy was changed in 1 patient who improved after a course of steroids for bronchiolitis obliterans organizing pneumonia. Lung biopsy cultures were positive in 6 patients but rarely resulted in changes in antibiotic therapy. CONCLUSIONS: Results of very few lung biopsies performed in stem cell transplant recipients redirected therapy. Furthermore, the ultimate outcome of these patients were not improved by the results of lung biopsies.

Patch esophagoplasty using AlloDerm as a tissue scaffold.

BACKGROUND/PURPOSE: Anastomotic leak and stricture are common causes of morbidity after esophageal repair. The authors describe a technique of patch esophagoplasty using decellularized human skin. METHODS: Twelve conditioned dogs underwent a cervical 2.0- x 1.0-cm esophagoplasty with AlloDerm. A gastrostomy tube was used for feedings until an esophagram was performed on the 10th to 14th postoperative day. Dogs were then given oral chow and followed up for leak and dysphagia. Animals were killed at 1-, 2-, and 3-month intervals and evaluated for stricture, diverticula formation, and patch histology. RESULTS: All animals survived, and none had sepsis or dysphagia. All esophagrams were without evidence of leak or stricture. At death there were no strictures or diverticula. Histologic examination of 1-month specimens showed partial reepithelialization of the patch with neovascularization. Control staining of AlloDerm was strongly positive for elastin. This was decreased in the region of the patch at 1 month. Two-month specimens showed intact epithelium and an increase in the caliber of new blood vessels. Three-month specimens showed no significant variation from 2-month animals. CONCLUSION: Decellularized human skin (AlloDerm) provides a temporary collagen framework on which esophageal healing can occur and function can be maintained.

Pectus excavatum repair: experience with standard and minimal invasive techniques.

BACKGROUND/PURPOSE: The Nuss procedure is a minimally invasive pectus repair that helps avoid cartilage resection and osteotomy. This report compares outcomes in patients undergoing a standard pectus repair to patients with the Nuss procedure. METHODS: One hundred three children (ages 5 to 20 years) with severe pectus excavatum underwent repair. Patients were evaluated for type of repair performed, associated anomalies, cardiopulmonary function, operating time, analgesia requirements, complications, length of hospital stay, hospital and operative charges, and cosmetic result. Statistical analysis was performed using the Mann-Whitney rank sum test. RESULTS: There were 68 patients (average age, 12.6 years) in the standard group and 35, (average age, 9.5 years) in the Nuss group. Associated anomalies were found in 6 standard group and 2 Nuss group patients. Average operating time in Nuss was 3.3 hours and in open procedures, 4.7 hours. Postoperative complications occurred in 13 (20%) standard repair patients and 15 (43%) after the Nuss. In the standard group, 14 patients received intrathecal and 3 received epidural analgesia, while 35 (52%) required an intravenous patient-controlled anesthetic device (PCA; average, 1.8 days). In the Nuss group, 25 patients (71%) received epidural anesthesia (average, 3 days), and 31 (89%) utilized PCA (average 3.8 days). Four (6%) standard patients and 8 Nuss patients (29%) required reoperation. Length of stay averaged 4.0 days (range 2 to 30) in the standard group and 4.8 days (range, 2 to 11) in the Nuss group. Average operating room charge was $8,325 in the standard group and $9,480 in the Nuss group. Average hospital charge was $4,137 for the standard patient and $4,044 for the Nuss group. Analgesic requirements and length of hospital stay were increased (P <.05). The complication rate and operative and hospital charges were similar between groups. CONCLUSIONS: Although the Nuss repair is associated with shorter operating time, smaller incisions, and less dissection, early results indicate few other advantages. Drawbacks of the Nuss procedure include high complication and reoperation rates and lack of efficacy in older teenagers and those with connective tissue disorders. Long-term follow-up will be necessary to determine final cosmetic and functional outcomes and define the overall risks and benefits of this procedure as compared with the standard technique.

Gastroschisis: a plea for risk categorization.

BACKGROUND: The incidence of gastroschisis has increased in the past decade. A differing clinical course between "complex" (those with atresias, perforation, or stenosis) and "simple" cases has prompted a review of risk assessment factors. METHODS: A retrospective chart review was conducted of 103 infants with gastroschisis over 5 years (1992 to 1997). RESULTS: Of 103 infants, 52 were girls and 51 were boys. Seventy-one infants (69%) had a simple defect, and 32 (31%) were complex. The simple group had an average estimated gestational age of 37.5 weeks (range, 26 to 40), and a birth weight of 3.0 kg (range, 1.7 to 3.8). A total of 71% underwent primary repair, whereas 29% required a silo. Mechanical ventilation averaged 6.8 days (range, 1 to 19). Enteral feedings were initiated at 15 days (range, 3 to 27) with full enteral intake achieved by 22.4 days (range, 5 to 40). Three infants required home parenteral nutrition. The average length of stay (LOS) was 26.4 days (range, 10 to 57). Complications occurred in 26 infants (36%), including intravenous catheter sepsis (n = 15), pneumatosis (n = 2), pneumonia (n = 1), bowel obstruction (n = 7), wound infection (n = 5), and SVC thrombosis (n = 1). Survival rate was 100%. Thirty-two infants had complex defects; 27 patients had atresias, stenosis, or perforations; and 3 had volvulus. The average estimated gestational age was 34 weeks (range, 26 to 38), and birth weight was 2.0 kg (range, 0.9 to 4.0). Primary repair was performed in 65% and silo placement in 35%. Mechanical ventilation was required for 22.3 days (range, 2 to 14). Enteral feedings were initiated at 22.5 days (range, 6 to 56) with full feedings achieved at 50 days (range, 21 to 113). Fourteen infants required home total parenteral nutrition (TPN). The LOS was 85.4 days (range, 24 to 270). A total of 47 complications occurred in the complex group including catheter sepsis (n = 15), short bowel syndrome (n = 7), pneumatosis (n = 3), bowel obstruction (n = 4), pneumonia (n = 2), superior vena cava thrombosis (n = 1), enterocutaneous fistula (n = 1), and 9 deaths (28% mortality rate). CONCLUSIONS: These data indicate gastroschisis can be divided into low-risk (simple) and high-risk (complex) categories. These 2 groups have significant differences in clinical behavior, postsurgical complications, LOS, and mortality rate (0 v 28%). Although the overall survival rate was 91% (94 of 103), parents, referring physicians, and insurers must be made aware of the impact of risk categorization on the estimated cost, LOS, and outcomes.

Home bowel preparation for elective colonic procedures in children: cost savings with quality assurance and improvement.

BACKGROUND/PURPOSE: The current health care environment pressures providers to lower cost and demands quality care that is measured by outcomes and patient satisfaction. Most insurers will not approve bed days for in-hospital preoperative bowel preparations for elective colorectal procedures. This policy does not take into account that infants and children are unable to tolerate large volumes of enteral preparation, which adversely affects outcome because of an inadequate preparation. This report describes a prospective evaluation of a standard home bowel preparation regimen utilizing local and regional home health care agency support. METHODS: For an elective colorectal procedure, pediatric patients underwent a home bowel preparation using GoLYTELY (100 mL/kg) via a nasogastric tube infused over 4 hours by a pediatric home health nurse trained in this technique. During the bowel preparation, the nurse educated the family members about the service and performed physiological monitoring to insure safety. At the completion of the preparation, any unusual events were transmitted to the staff surgeon for further instructions. Our initial 30 patients were treated by our hospital home health agency personnel to insure safety. Since then, 41 additional bowel preparations have been performed by statewide agencies. RESULTS: Seventy-one patients underwent complete home bowel preparation (45 boys; 26 girls). The age range was 3 months to 9 years (average, 5 months). There was one complication caused by incorrect mixing of GoLYTELY causing gastrointestinal cramping. All 71 home bowel preparations were recorded as good at the time of the colorectal procedure by the staff pediatric surgeon. The average cost for home bowel preparation was $300 in network, and $350 out of network. This compares with an inpatient hospital day cost of greater than $800 ($36,000 savings). CONCLUSIONS: This technique offers the pediatric surgeon an opportunity to maintain a high standard of quality care while using home health agency personnel to minimize cost. This program is safe, effective, and associated with a good outcome and a high degree of family satisfaction.

Prehospital triage in the injured pediatric patient.

BACKGROUND/PURPOSE: Identifying major trauma patients in the prehospital setting is essential in determining management, destination, and best utilization of emergency department resources. Few methods of trauma triage have been accepted unanimously. This study prospectively evaluates the efficacy of comprehensive field triage using 12 criteria (simplified version of the American College of Surgeon's guidelines) in 1,285 pediatric trauma patients. METHODS: Major trauma was defined as occurring in those who died in the emergency room, had major surgery (penetrating injury involving surgery of the head, neck, chest, abdomen, or groin), or were admitted directly to the intensive care unit. The correlation between trauma triage criteria, hospital disposition, and triage accuracy were determined prospectively and compared in the pediatric patients (36 months) with an adult cohort of patients (12 months). RESULTS: A total of 1,285 pediatric trauma patients were evaluated and compared with 1,326 adult trauma patients. The most accurate trauma triage criterion for major injury was a blood pressure < or = 90 mmHg (systolic) with an accuracy of 86%. This was followed by burn greater than 15% total body surface area (79%), Glasgow Coma Scale score < or = 12 (78%), respiratory rate less than 10/min or greater than 29/min (73%), and paralysis (50%). Less accurate criteria included a fall from greater than 20 feet (33%); penetrating injury to head, neck, chest, abdomen, or groin (29%); ejection from vehicle (24%); pedestrian struck at greater than 20 mph (16%); paramedic judgement (12%); rollover (3%); and extrication (0%). The Glasgow Coma Scale score was a more accurate indicator of major injury in children than adults, and paramedic judgement was less accurate in children when compared with adults. Of the 379 major pediatric trauma victims, the Revised Trauma Score and Pediatric Trauma Score missed 36% and 45% of these major trauma victims, respectively. The overtriage rate for children was 71% with a sensitivity of 100% (no missed major trauma patients). CONCLUSIONS: Physiological variables, anatomic site, and mechanism of injury provide a sensitive and safe system of triage. Continued education of prehospital personnel regarding pediatric trauma and stratification of the current triage tools are necessary to minimize overtriage in an era of shrinking resources.

Efficacy of primary and secondary video-assisted thoracic surgery in children.

BACKGROUND/PURPOSE: Video-assisted thoracic surgery (VATS) is used commonly for diagnostic and therapeutic procedures in children. The purpose of this study was to determine the accuracy, efficacy, and complications associated with primary and secondary VATS in children. METHODS: Eighty-seven infants, children, and adolescents underwent 104 VATS procedures between March 1993 and April 1999. There were 47 boys and 40 girls with an age range of 6 months to 19 years. VATS was performed for excision of pulmonary nodule (n = 51), biopsy of infiltrate (n = 14), excision or biopsy mediastinal mass (n = 12), decortication of empyema (n = 16), pleurodesis and bleb excision for pneumothorax (n = 5), pleurolysis for P32 administration (n = 3), esophageal myotomy (n = 2), and thymectomy (n = 1). In 6 children a contralateral thoracic procedure was performed along with VATS (3 VATS, 3 thoracotomies). Secondary VATS was performed in 20 after prior thoracic procedures. RESULTS: VATS was efficacious for diagnostic or therapeutic purposes in 93 cases. Overall, 11 (11%) VATS required conversion to open thoracotomy. Average length of thoracostomy tube drainage (CTD) was 2.2 days, and average length of stay (LOS) was 3.7 days. Complications included prolonged air leak (> 7 days) in 3 (2 empyema, 1 nodule). Two children with malignancy and pulmonary infiltrates died within 30 days of progressive respiratory failure. There were no bleeding complications or deaths related to VATS. CONCLUSIONS: VATS is a safe and effective primary and secondary procedure in children resulting in a short length of CTD and LOS. Duration of CTD and LOS are prolonged if empyema is associated with a bronchopleural fistula, and VATS may not be of value in this setting.

The use of pleuroperitoneal shunts in the management of persistent chylothorax in infants.

BACKGROUND/PURPOSE: The development of chylothorax is a serious and often life-threatening clinical entity that may cause profound respiratory, nutritional, and immunologic complications and has become increasingly common in recent years. Optimal management of this problem has not been well defined because medical therapy has a significant failure rate. Surgical treatment of complicated chylothorax has become a mainstay of care. METHODS: Over the last 36 months, seven infants had a pleuroperitoneal shunt placed for the management of refractory chylothorax. Ages ranged from 10 to 66 days with a weight between 1,000 to 4,850 g. Five of the seven infants were ventilator dependent. The etiologies were congenital in four and acquired in three with one related to a cardiothoracic procedure, one related to superior vena caval thrombosis, and one postoperative diaphragmatic hernia repair with superior vena caval thrombosis. Associated conditions included a left congenital diaphragmatic hernia, asplenia, isolated renal agenesis, bronchopulmonary dysplasia, and a patent ductus arteriosus. Each patient was unresponsive to thoracentesis, tube thoracostomy, and dietary manipulation with preoperative volume of chest tube output ranging from 50 to 162 cc/kg/d. The duration of preoperative therapy in congenital occurrences ranged from 10 to 46 days (average, 22 days). A Denver double-valved shunt system was used and catheters were implanted under general anesthesia. Manual pumping was required postoperatively on an hourly basis. RESULTS: All seven patients had excellent results with the elimination of the chylothorax and resolution of symptoms. There were two complications. Shunt survival rate was six of seven (86%). Shunt removal ranged from 24 to 79 days (average, 44 days). Patient survival rate was five of seven (71%) with one infant dying of progressive pulmonary disease and one infant dying from viral sepsis; both had functioning shunts. One patient remains ventilator dependent secondary to chronic lung disease from prematurity. CONCLUSIONS: Pleuroperitoneal shunting is safe, simple, and an effective treatment of chylothorax in infants despite their size, age, or degree of prematurity.

A case controlled comparison of open and laparoscopic splenectomy in children.

BACKGROUND: This case controlled study compares the efficacy, safety, and cost of laparoscopic splenectomy (LS) and open splenectomy (OS) for hematologic disorders in children. METHODS: The records of 82 consecutive children and adolescents undergoing splenectomy for hematologic disorders between August 1994 and September 1997 were reviewed retrospectively. RESULTS: Fifty patients underwent LS by a lateral approach and 32 underwent OS through a left subcostal incision. Mean age was 7.76 years for LS and 6.9 years for OS. Patient weights were similar: (LS, mean 30.5 kg; OS, mean 27.6 kg). Hematologic indications included hereditary spherocytosis in 43 children (LS 26, OS 17), sickle cell anemia with sequestration in 13 (LS 7, OS 6), immune thrombocytopenic purpura in 14 (LS 8, OS 6), and 12 with other disorders (LS 9, OS 3). Concomitant cholecystectomy was performed in 10 of 50 LS and 6 of 32 OS cases. Accessory spleens were identified in 8 of 32 (25%) OS and 9 of 50 (18%) LS cases (P = .578). No LS procedures required conversion to OS. The mean estimated blood loss was 54.4 mL for LS and 49.0 mL for OS (P = .233). LS required a longer operative time (115 vs 83 minutes, P = .002), less need for postoperative intravenous narcotic (51% vs 100%, P < .0001), lower total narcotic doses (0.239 vs 0.480 mg/kg morphine, P = .006), shorter length of hospital stay (1.4 +/- 0.97 vs 2.5 +/- 1.43 days, P = .0001), and lower average total hospital charges ($5713 vs $6564) than OS. There were no deaths or major complications in either group. CONCLUSIONS: Laparoscopic splenectomy is a safe and effective procedure in children with hematologic disorders resulting in longer operative times, less narcotic administration, shorter length of stay, and lower total hospital charge.

Long-term follow-up after bowel resection for necrotizing enterocolitis: factors affecting outcome.

BACKGROUND: Necrotizing enterocolitis (NEC) is the most common surgical emergency among newborns and is associated with a high morbidity and mortality. This study evaluates the long-term survival of infants requiring surgical intervention for NEC and factors affecting outcome. METHODS: A retrospective review of infants requiring surgery for complications of NEC at a tertiary care, pediatric hospital over a 16-year period was performed. Patients were evaluated for early and late morbidity and mortality, length of intestinal resection, presence of the ileocecal valve (ICV), days of parenteral nutrition (PN), and growth. RESULTS: Two hundred forty-nine patients were included, with an average gestational age of 30 +/- 5 (+/- SD) weeks and birth weight of 1.50 +/- 0.89 kg. The surgical mortality rate was 45%, with survivors (137) being larger (P < .001) and older (P < .001) at time of birth than nonsurvivors. Mortality rates varied inversely with gestational age and birth weight. Surgical survivors had an average of 21 +/- 26 cm of intestinal length resected. The ileocecal valve was preserved in 45% of infants. Growth was similar between infants with or without an ICV. Stratification of length of intestine resected showed that infants with larger resections had greater requirements for parenteral nutrition, but this had no influence on long-term growth at follow-up. CONCLUSIONS: Survivors of NEC are characterized by greater gestational age, greater birth weight, and older postgestational age at surgery. Infants who underwent greater intestinal resections required longer periods of PN. The length of intestine resected or presence of the ileocecal valve had no overall bearing on long-term outcome.

Necrotizing enterocolitis.

Necrotizing enterocolitis is a relatively common disorder of unknown etiology that primarily affects premature newborns. The majority of babies with necrotizing enterocolitis respond to nonsurgical management, and, despite an increase in the number of premature infants, the surgical mortality rate has improved. This review provides an overview of the current literature covering new developments in etiology, risk factors, pathogenesis, diagnosis, therapy, outcome, and preventable measures related to necrotizing enterocolitis.

Intestinal atresia and stenosis: a 25-year experience with 277 cases.

OBJECTIVE: To evaluate the causes, clinical presentation, diagnosis, operative management, postoperative care, and outcome in infants with intestinal atresia. DESIGN: Retrospective case series. SETTING: Pediatric tertiary care teaching hospital. PATIENTS: A population-based sample of 277 neonates with intestinal atresia and stenosis treated from July 1, 1972, through April 30, 1997. The level of obstruction was duodenal in 138 infants, jejunoileal in 128, and colonic in 21. Of the 277 neonates, 10 had obstruction in more than 1 site. Duodenal atresia was associated with prematurity (46%), maternal polyhydramnios (33%), Down syndrome (24%), annular pancreas (33%), and malrotation (28%). Jejunoileal atresia was associated with intrauterine volvulus, (27%), gastroschisis (16%), and meconium ileus (11.7%). INTERVENTIONS: Patients with duodenal obstruction were treated by duodenoduodenostomy in 119 (86%), of 138 patients duodenotomy with web excision in 9 (7%), and duodenojejunostomy in 7 (5%) A duodenostomy tube was placed in 3 critically ill neonates. Patients with jejunoileal atresia were treated with resection in 97 (76%) of 128 patients (anastomosis, 45 [46%]; tapering enteroplasty, 23 [24%]; or temporary ostomy, 29 [30%]), ostomy alone in 25 (20%), web excision in 5 (4%), and the Bianchi procedure in 1 (0.8%). Patients with colon atresia were managed with initial ostomy and delayed anastomosis in 18 (86%) of 21 patients and resection with primary anastomosis in 3 (14%). Short-bowel syndrome was noted in 32 neonates. MAIN OUTCOME MEASURES: Morbidity and early and late mortality. RESULTS: Operative mortality for neonates with duodenal atresia was 4%, with jejunoileal atresia, 0.8%, and with colonic atresia, 0%. The long-term survival rate for children with duodenal atresia was 86%; with jejunoileal atresia, 84%; and with colon atresia, 100%. The Bianchi procedure (1 patient, 0.8%) and growth hormone, glutamine, and modified diet (4 patients, 1%) reduced total parenteral nutrition dependence. CONCLUSIONS: Cardiac anomalies (with duodenal atresia) and ultrashort-bowel syndrome (<40 cm) requiring long-term total parenteral nutrition, which can be complicated by liver disease (with jejunoileal atresia), are the major causes of morbidity and mortality in these patients. Use of growth factors to enhance adaptation and advances in small bowel transplantation may improve long-term outcomes.

Reoperation after Nissen fundoplication in children with gastroesophageal reflux: experience with 130 patients.

OBJECTIVE: The authors evaluate reoperation for recurrent gastroesophageal reflux (GER) after a failed Nissen fundoplication. SUMMARY BACKGROUND DATA: Nissen fundoplication is an accepted treatment for GER refractory to medical therapy. Wrap failure and recurrence of GER are noted in 8% to 12%. METHODS: Medical records of 130 children undergoing a second antireflux operation for recurrent GER from January 1985 to June 1996 retrospectively were reviewed. RESULTS: One hundred one patients (78%) were neurologically impaired (NI), 74 (57%) had chronic pulmonary disease, and 8 had esophageal atresia. Recurrent symptoms included vomiting (78%), growth failure (62%), choking-coughing-gagging (38%), and pneumonia (25%). Gastroesophageal reflux was confirmed by barium swallow, gastric scintigraphy, and endoscopy. Operative findings showed wrap breakdown (42%), wrap-hiatal hernia (30%), or both (21%). A second Nissen fundoplication was performed in 128 children. Complications included bowel obstruction (18), wound infection (10), pneumonia (6) and tight wrap (9). There were two postoperative (<30 days) deaths (1.5%). Of 124 patients observed long term, 89 (72%) remain symptom free. Eight were converted to tube feedings. Twenty-seven required a third fundoplication, and 19 (70%) were successful outcome. Two with repetitive wrap failure due to gastric atony underwent gastric resection and esophagojejunostomy. CONCLUSION: Nissen fundoplication was successful in 91% of patients. In 9% with wrap failure, a second Nissen fundoplication was successful in 72%. Reoperation is justified in properly selectedpatients. Conversion to jejunostomy feedings is suggested for neurologically impaired after two wrap failures and a partial wrap in those with esophageal atresia and severe esophageal dysmotility. Repeated wrap failure due to gastric atony requires gastric resection and esophagojejunostomy.

The "other side" of pediatric hernias: the role of laparoscopy.

The role of contralateral inguinal exploration in neonates, infants, and children presenting with unilateral hernias is controversial. Factors considered by surgeons include the patient's age, sex, and side of the clinically apparent hernia. The purpose of this study was to evaluate the role of diagnostic laparoscopy performed through the clinically apparent hernia sac to identify a contralateral patent processus vaginalis (CPPV) in children and limit contralateral exploration to CPPV-positive patients. One hundred neonates, infants, and children underwent laparoscopic evaluation for a CPPV through the ipsilateral hernia sac. There were 79 boys and 21 girls. Forty-eight of 100 (48%) had a CPPV identified, which was confirmed operatively. Thirty-one of 68 patients (46%) with a right-sided and 18 of 32 (56%) with a left-sided hernias had a CPPV (P = 0.39). Thirty-six of 56 (64%) patients younger than 6 months of age had a CPPV compared to 13 of 44 (30%) older than six months (P = 0.001). Fourteen of 21 (67%) girls had a CPPV compared to 35 of 79 (44%) boys (P = 0.087). Laparoscopy through the hernia sac is a safe and effective means of identifying the presence of a CPPV and avoiding unnecessary contralateral inguinal exploration. Infants (< 6 months) are much more likely to have a CPPV.

Gastrointestinal perforation and peritonitis in infants and children: experience with 179 cases over ten years.

BACKGROUND: Premature infants continue to have a high mortality after gastrointestinal perforation. This report describes 179 patients with gastrointestinal perforation and peritonitis and compares etiologic factors, mortality, and causes of death in premature infants and older children in an attempt to predict outcome. METHODS: The 113 boys (63.1%) and 66 girls (36.9%) had an age range of newborn (n = 139, 77.6%) to 17 years. Site of perforation was gastric in 16, duodenal in 9, small bowel in 105, colon in 37, and undesignated in 12. Eighteen had multiple perforations. Etiologic factors in newborns (younger than 2 months) included necrotizing enterocolitis (NEC) (75, 41.9%), isolated ileal perforations (30, 21.5%), malrotation/volvulus (8), iatrogenic causes (5), and others (6). Gestational age was 29.6 +/- 4.3 weeks for NEC versus 31.4 +/- 5.4 weeks for non-NEC. Birth weight for patients with NEC was 1.45 +/- 0.8 gm and 1.81 +/- 1.0 gm for non-NEC babies. Etiologic factors in 33 older children (older than 2 months to 17 years) were trauma (10), Meckel's diverticulum (4), intussusception (2), pseudomembranous colitis (2), adhesions (2), stomal leak (2), others (4), and nondesignated (7). Gastric perforations (n = 16) were iatrogenic in 7, idiopathic in 5, and caused by an ulcer in 4. RESULTS: Mortality for NEC was 36 of 75 (48%), 15 of 55 (27.2%) for non-NEC infants (p < 0.05 versus NEC), 15.1% (5 of 33) for older children (p < 0.05 versus NEC), and 4 of 16 (25%) for gastric perforation. Infant deaths were related to overwhelming sepsis, immaturity of systems, and multiorgan failure. Deaths for older children were a result of sepsis, multiorgan failure, and immunodeficiency. CONCLUSIONS: Gastrointestinal perforation is more common in premature infants with the highest mortality (48%) noted in NEC. Despite surgical intervention and advances in neonatal intensive care unit care, premature low birth weight infants (especially NEC) continue to have a high mortality.

Increased risk of necrotizing enterocolitis in premature infants with patent ductus arteriosus treated with indomethacin.

OBJECTIVE: The authors evaluated the risk of necrotizing enterocolitis (NEC) in very low birth weight infants receiving indomethacin (INDO) to close patent ductus arteriosus (PDA). BACKGROUND DATA: Controversy exists regarding the best method of managing very low birth weight infants with PDA and whether to employ medical management using INDO or surgical ligation of the ductus. METHODS: Two hundred fifty-two premature infants with symptomatic PDA were given intravenously INDO 0.2 mg/kg every 12 hours x 3 in an attempt to close the ductus. Patients were evaluated for sex, birth weight, gestational age, ductus closure, occurrence of NEC, bowel perforation, and mortality. RESULTS: There were 135 boys and 117 girls. The PDA closed or became asymptomatic in 224 cases (89%), whereas 28 (11%) required surgical ligation. Ninety infants (35%) developed evidence of NEC after INDO therapy. Fifty-six were managed medically; surgical intervention was required in 34 of 90 cases (37.8%) or 13% of the entire PDA/INDO study group. Bowel perforation was noted in 27 cases (30%). Factors associated with the onset of NEC included gestational age < 28 weeks, birth weight < 1 kg, and prolonged ventilator support. The overall mortality rate was 25.5%, but was higher in infants with NEC versus those without. The highest mortality was noted in perforated NEC cases. The PDA/INDO patients were compared with a control group of 764 infants with similar sex distribution, birth weights, and gestational ages without PDA who did not receive INDO. Necrotizing enterocolitis occurred in 105 of 764 control patients (13.7%), including 13 (12.3%) with perforation. The overall mortality rate of controls was 25%, which was similar to the overall 25.5% mortality rate in the PDA/INDO study group. CONCLUSION: These data indicate that there is increased risk of NEC and bowel perforation in premature infants with PDA receiving INDO. Mortality was higher in the PDA/INDO group with NEC than those PDA/INDO infants without NEC.