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The umbilical fold incision for infantile hypertrophic pyloric stenosis provides a convenient exposure and cosmetically appealing scar. This study investigates the possible difference in infection rates between traditional and supraumbilical approaches for pyloromyotomy.All patients who underwent pyloromyotomy for infantile hypertrophic pyloric stenosis at a tertiary pediatric hospital were reviewed. Baseline wound infection rate was determined through review of patients with right upper quadrant incisions (group 1). A nonrandomized comparison was performed between patients with a supraumbilical approach (group 2) and those undergoing supraumbilical incisions after prophylactic antibiotic administration (group 3).Complete records were reviewed on 384 patients over a 6-year period. Demographics and preoperative factors were similar among groups. The rate of infection in group 1 (n = 258) was 2.3%. With introduction of the supraumbilical approach, there was a statistically significant increase in wound infection rate to 7.0% (chi 2 ; group 1 vs group 2, P.05). The use of prophylactic antibiotics with a supraumbilical approach reduced this rate of infection back to 2.3% (chi 2 ; group 1 vs group 3, P1.0 and group 2 [n = 85] vs group 3 [n = 42], P.3).The risk of wound infection by classic pyloromyotomy of 2.3% is significantly increased with an open supraumbilical approach. The use of prophylactic antibiotics reduces this risk of wound infection.

Background Many children with chronic constipation and fecal incontinence have benefited from the antegrade colonic enema (ACE) procedure. Routine antegrade colonic lavage often allows such children to avoid daytime soiling. This report describes 2 children in whom the ACE procedure was complicated by a cecal volvulus. Methods A retrospective review of 164 children with an ACE procedure was conducted. Two instances of cecal volvulus were identified. Results The first child presented with abdominal pain and difficulty intubating the ACE site. Over the subsequent day, his pain worsened, and radiographs depicted a colonic obstruction. At laparotomy, a cecal volvulus resulting in bowel necrosis was observed, and resection of the affected bowel and appendix (in the right lower quadrant) and end ileostomy was required. He subsequently had the stoma closed and a new ACE constructed with a colon flap. The second child presented with shock and evidence of an acute abdomen. At laparotomy, a cecal volvulus was noted, and ileocolic resection including the ACE stoma (located at the umbilicus) and an ileostomy and Hartmann pouch was performed. He had a protracted hospital course requiring ventilator and inotropic support. He currently is well and still has an ileostomy stoma. Conclusions A high index of suspicion for a potentially life-threatening cecal volvulus should be maintained in children undergoing an ACE procedure who present with abdominal pain, evidence of bowel obstruction, or difficulty in advancing the ACE irrigation catheter.

Background. The goal of this study was to review current injury characteristics, severity, intervention, and outcome of duodenal injuries from a single, pediatric trauma facility. Methods. A retrospective review was performed of duodenal injuries in children less than 16 years of age from 1990 to 2000. Results. Twelve children had duodenal injuries as a result of blunt abdominal trauma. Six injuries were the result of motor vehicle crashes. Nonaccidental trauma (2) and contact injury (4) provided the remaining cases. Diagnosis was achieved by abdominal computed tomography. Severity of duodenal injury included grade I (1), II (8), and III (3). Seven patients had associated visceral or neurologic injuries. Average Injury Severity Score was 18. Duodenal repair was required in 9 of the 10 patients explored. Treatment included observation (3); primary repair, alone, (2) or with proximal decompression (4); and pyloric exclusion with gastrojejunostomy (3). Exclusion techniques had fewer complications (0% vs 57%) and fewer hospital days (19 vs 23). Conclusions. Blunt abdominal trauma remains the most prevalent mechanism for pediatric duodenal injuries. Patients undergoing pyloric exclusion for severe duodenal trauma had a lesser morbidity and a shorter hospital stay in this small series. Pyloric exclusion remains an alternative for the treatment of severe duodenal injuries in selected children. (Surgery 2002;132:748-53.)

Background/Purpose: Initially described in 1937, inflammatory pseudotumor (IPT) inflammatory myofibroblastic tumor (IMT) or plasma cell granulomas are synonymous for an inflammatory solid tumor that contains spindle cells, myofibroblasts, plasma cells, and histocytes. Common sites of presentation include lung, mesentary, liver, and spleen; intestinal presentations are rare, and the etiology remains obscure. This report details the clinical and surgical experiences in 4 children with alimentary tract IPT at a single institution. Methods: A retrospective chart review was conducted of pediatric patients with the pathologic diagnosis of IPT. Results: Between 1990 and 1999, 4 patients (4 girls, ages 5 to 15 years) were identified with gastrointestinal tract origins of IPT. Symptoms at presentation included anemia (n = 4), intermittent abdominal pain (n = 3), fever (n = 3), weight loss (n = 2), diarrhea (n = 2), dysphagia (n = 1). Two patients had comorbid conditions of juvenile rheumatoid arthritis and mature B cell lymphoma. Three of 4 patients had elevated sedimentation rates. The sites of origin were the gastroesophageal junction, the colon, the rectum, and the appendix, with the referral diagnosis achalasia, perforated appendix, inflammatory bowel disease, and recurrent lymphoma, respectively. All were treated with aggressive surgical resection, and 3 girls have had no recurrences since the initial surgery. One patient had 3 recurrences within 8 months of presentation; she remains disease free 8 years later. Conclusions: IPT, although rare in the gastrointestinal tract, mimics more common problems. Successful surgical management is possible even in cases of multiple recurrences. J Pediatr Surg 36:169-173. Copyright © 2001 by W.B. Saunders Company.

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. J Pediatr Surg 35:134-138. Copyright © 2000 by W.B. Saunders Company.

Purpose: Congenital diaphragmatic hernia (CDH) is a complex anomaly requiring intensive pulmonary and hemodynamic management. Survival has increased in this population placing them at risk for subsequent morbidities including surgery. The purpose of this study is to review the need for subsequent surgeries in the CDH population. Methods: After receiving institutional review board approval, a retrospective chart review of all CDH patients between 1980 and 2007 was conducted noting subsequent surgeries and the impact of extracorporeal membrane oxygenation (ECMO) on the types of surgical procedures. Comparison of groups was done by Fisher's Exact test or nonparametric Wilcoxon rank-sum test where appropriate. A P value of less than .05 was considered significant. Results: Data were analyzed for 227 of 294 patients during this period. Extracorporeal membrane oxygenation support was used in 45% of patients. Subsequent surgery was required in 117 patients. Seventy patients in the ECMO group (69%) required a subsequent operation. The most common operative procedures included inguinal hernia/orchiopexy, antireflux, and recurrent diaphragmatic hernias. Conclusion: In this series, ECMO survivors are at a high risk for requiring subsequent surgeries compared to the total CDH group. This information can be used as an education tool for referring physicians and parents as they care for this group of children.

Purpose Esophageal atresia is known to be associated with a variety of additional congenital anomalies in multiple organ systems. Emphasis on cardiovascular anomalies has been focused on aortic arch and intrinsic cardiac malformations. Persistent left superior vena cava (PLSVC) is the most common venous thoracic anomaly in the general population and creates a problem when central venous access is required. This review was undertaken to define the incidence of PLSVC in infants with esophageal atresia and to determine if any subgroup of associated anomalies poses additional risk. Methods A retrospective, institutional review board–approved review of all children treated for esophageal atresia from 1993 to 2002 at Riley Hospital for Children was undertaken. Of 118 children, 89 had sufficient data for inclusion. Charts were reviewed for gestational age, weight, type of atresia, echocardiogram, and associated anomalies. Statistical analysis was performed using the Fisher's Exact test. Results Of 89 children, 8 (9.9%; confidence interval, 4%-17%) had PLSVC compared with the reported incidence of 0.3% in the general population. Presence of additional organ system anomalies did not significantly increase relative risk for PLSVC. Conclusion The incidence of PLSVC is significantly increased in children with esophageal atresia when compared with the general population. This increased incidence of PLSVC is not influenced by the presence of cardiac or other associated anomalies. This finding should be kept in mind when central venous access is required in this patient population.

Children with Beckwith-Wiedemann syndrome (BWS) have an increased risk of developing Wilms' tumors, hepatoblastomas, and adrenal tumors. This study evaluates disease-free survival in children with BWS and intraabdominal tumors. Sixteen tumors occurred in 13 children with BWS (8 boys, 5 girls). Diagnoses included Wilms' tumor (10) (2 bilateral, 20%), hepatoblastoma (2), bladder rhabdomyosarcoma (1), and adrenal cortical tumor (1). In the 10 children with Wilms' tumor, the average age at diagnosis was 3.5 years (range, 7 months to 5 years). Nine of 10 had initial tumor resection, chemotherapy, and radiation therapy (when indicated). One child with bilateral disease had tumor biopsy, chemotherapy, and partial nephrectomy. Tumors were classified as stage I (5), stage II (2), stage IV (1) and stage V (2), all with favorable histology. Disease-free survival rate was 100% with median follow-up of 9 years (range, 4 to 22 years). One patient had a left adrenal tumor detected during screening sonography 11 years after Wilms' tumor resection. Two infants with advanced-stage hepatoblastoma responded to chemotherapy, allowing subsequent complete hepatic resection. Both tumors had unfavorable histology. Both completed postoperative chemotherapy and have no evidence of disease (NED) with normal alpha-fetoprotein levels at 21 and 12 months, respectively, after tumor detection. One patient with stage III (group 3) bladder rhabdomyosarcoma underwent partial cystectomy following chemoradiation and is alive (NED) after 20 months. Children with BWS should be screened at regular intervals (every 3 to 6 months) for renal, adrenal, and hepatic tumors. The exact duration of screening is not yet determined. Despite an increased risk of tumor occurrence, this study suggests that children with BWS have an improved survival rate (100%). All 13 children are currently alive with NED. BWS may be a favorable biological characteristic for survival.

Between 1980 and 1993, 74 children and adolescents were referred for surgical evaluation of palpable breast masses. Thirty-two were managed nonoperatively for unilateral thelarche (26), fibroadenoma (3), gynecomastia (2), or hemorrhagic cyst (1). The other 42 children had surgical intervention for giant or painful fibroadenomas (19), breast abscesses (5), painful gynecomastia (6), metastatic disease (4), or other conditions (8). No instances of primary breast malignancy were noted. Physical examination and minimal (selective) diagnostic testing can conserve health care dollars in cases of pediatric patients with breast masses.

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. J Pediatr Surg 36:1143-1145. Copyright © 2001 by W.B. Saunders Company.

This report analyzes factors associated with 71 recurrent inguinal hernias in 62 children treated between 1976 and 1988. Cases were evaluated for sex, age, type of initial repair, interval to recurrence, the presence of comorbid conditions, and type of reoperation. There were 57 boys and 5 girls. Sixty percent of patients were less than 6 months old and 72% were less than 1 year of age at the time of the initial repair. Recurrence was on the right in 74%, left in 24%, and bilateral in 2%. Recurrence was noted by 6 months in 50%, by 2 years in 76%, and by 5 years in 96%. Comorbid conditions were present in 60% of cases, including increased intraabdominal pressure (ventriculoperitoneal [VP] shunts), growth failure, prematurity, chronic pulmonary disease, bladder exstrophy, connective tissue disorders, crytorchism, seizure disorder, and malnutrition. Incarceration was a factor in four of the 62 cases. Seven patients had multiple recurrences. Fifty-one recurrences were indirect, whereas 20 were direct inguinal hernias. Inadequate high ligation (three with chromic catgut), wound infection, and groin hematoma were other findings. The direct hernias may be related to injury to the floor of the canal during initial repair. Recurrent repair included high ligation of the sac alone (20), high ligation plus snugging of a large internal ring (11), and high ligation with repair of the iliopubic tract in patients with VP shunts, connective tissue disorder, or weak floor (20). All direct hernias had a Cooper's ligament (McVay) repair. Two direct hernias recurred again and were successfully repaired using a preperitoneal approach. Adequate high ligation at the internal ring, snugging of a large internal ring, avoidance of injury to the canal floor, and closure of the internal ring in girls are important operative considerations in preventing indirect hernia recurrence. Selected patients with connective tissue disorders, poor nutrition, and increased intraabdominal pressure (ascites, VP shunts) associated with weak floor tissues should undergo iliopubic tract repair to prevent recurrence. Cooper's ligament repair is usually successful in cases of direct inguinal hernia; the preperitoneal repair is advantageous in patients with multiple recurrences.

Recent reports have documented the successful use of percutaneous drainage (PD) in the management of traumatic pancreatic pseudocysts in children. This study presents four cases of pancreatic pseudocyst in which percutaneous catheter drainage was performed. In one instance, no operative therapy was required. However, in the other three cases PD failed to resolve the problem and distal pancreatectomy with splenic salvage was performed when contrast studies (endoscopic retrograde cholangiopancreatography or catheter injection) demonstrated disruption of the main pancreatic duct. This report suggests that children with pancreatic pseudocysts unresponsive to PD require prompt investigation of ductal anatomy to rule out transection or other major injury.

From June 1986 to May 1988, there were 1,931 childhood deaths recorded in Indiana. Eight hundred six children (0 to 18 years old) died as a result of trauma (41.4% of all deaths). Seventy percent of all traumatic deaths occurred in boys. Blunt trauma accounted for 54% of deaths, asphyxia or drowning 26%, penetrating trauma 15%, electrocution 3%, and burns 1%. Sixty percent of deaths occurred in rural areas and 40% occurred in urban centers; however, state-wide demographics define the population as 70% urban. The percentage of deaths due to trauma within a given race was: hispanic 71%, caucasian 42%, black 35%, and others 50%. However, when deaths occurring in infants less than 30 days of age were eliminated, the percentages changed: hispanics 70%, caucasian 45%, black 45%, and others 50%. Traumatic deaths were 1.6 times as likely to occur during the months of June through October (n = 85 deaths/mo) as opposed to November through May (n = 53 deaths/mo) (P less than .05). Mortality from burns was limited to children less than 5 years of age and penetrating trauma mortality was twice as likely to occur in children over 15 years (10% v 20%). Fifty-two percent of all traumatic deaths in children occurred between 15 and 18 years of age. Major burns account for only 1% of traumatic deaths in this state. Asphyxia and drowning were more common in young children, and blunt traumatic deaths more common in older children. In 1988, the first state-wide accident awareness program was instituted.(ABSTRACT TRUNCATED AT 250 WORDS)

Hirschsprung's disease (HD) is a neurogenic form of intestinal obstruction characterized by a congenital absence of ganglion cells in the submucosal and myenteric plexuses. Acquired aganglionosis (AAG) is a rare condition that may occur following various pull-through procedures for HD. This report describes five boys with acquired aganglionosis. In all cases, the presence of normal ganglion cells was confirmed on review of biopsies of the pull-through segments at the initial operation. The original pull-through procedure included Soave (2), Duhamel (2), and Swenson (1) operations. Three procedures were initially performed at other institutions. Recurrent symptoms including abdominal distention, obstipation, enterocolitis, and failure to thrive developed 7, 11, 12, 18, and 30 months postoperatively (mean, 15.6 months). The diagnosis of AAG was delayed 1.5 to 9 years after the onset of recurrent symptoms. Full column barium enema studies revealed a transition zone or narrow area in the rectosigmoid or descending colon in four children. Repeat full thickness rectal biopsies at 3.0 cm above the anal verge in the pull-through segment confirmed the absence of ganglion cells in each case. Two children (post Swenson and Duhamel) were successfully revised with a Swenson procedure. Two additional children (post Soave and Duhamel) were successfully treated with extended posterior anomyomectomy procedures. The remaining boy now has a preliminary colostomy and is awaiting a second procedure. Vascular compromise of the distal bowel segment at the time of the initial pull-through procedure may contribute to the selective loss of ganglion cells postoperatively as neural tissues are most sensitive to hypoxia. This rare finding must be considered when recurrent obstructive symptoms develop following corrective surgery for HD and mechanical causes have been excluded.

This report concerns 60 children with documented Staphylococcus epidermidis sepsis. There were 34 boys and 26 girls, ages 2 weeks to 15 years. The primary diagnosis included malignancy (13), congenital (13) or acquired (11) gastrointestinal disorders, prematurity (7), cardiac defect (5), hydrocephalus (2) and miscellaneous (9). Clinical presentation included fever (54), tachycardia (15), lethargy (20), hypotension (8), irritability (6), increased gastric residuals (6) and apnea/bradycardia (3). A documented source of sepsis was noted in 56 patients, including percutaneous central venous catheters (23), Broviac catheters (17), umbilical arterial catheters (6), wound (3), V-P shunt (2), cardiac defect (2), cholangitis (1), chest tube (1) and peripheral arterial line (1). There were six sepsis-related deaths, four in premature infants. Two of six infected subclavian catheters were treated successfully with vancomycin. Infection was successfully cleared in 20 of 23 infected Broviac catheters with vancomycin through the line. However, six were eventually removed for tract infection (1), persistent fever (2), and Candida sp . infection (3). Although once considered a nonpathogenic skin contaminant, S. epidermidis has emerged as a serious pathogen in hospitalized, immunosuppressed, premature and malnourished pediatric patients. Indwelling catheters enhance the likelihood of infection in these patients. Agressive antimicrobial therapy is vital in this potentially lethal infection. Vancomycin proved efficacious in this series.

Ninety-two children with pancreatic disorders were treated over a 10-year period. Thirty-three had blunt trauma, while 69 had medical, metabolic, or neoplastic diseases. Children with trauma had either duct disruption (3), gland fracture (4), or pseudocysts formation (26). Operation was required in 30. Pseudocysts were treated with observation alone in three cases, ultrasound-guided percutaneous aspiration in three, surgical external drainage in two, distal pancreatectomy in four, cyst gastrostomy in ten, and cyst-Roux-en-Y jejunostomy in six. Other disorders included pancreatitis (44), neoplasms (10), nesidioblastosis (4), and pancreaticosplenic abscess (2). Treatment for neoplasms included surgical excision in nine and biopsy in one (adenocarcinoma). Patients with nesidioblastosis underwent 95% (near total) pancreatic resection (two after previous unsuccessful 80% resection). Pancreatitis was familial in two cases, necrotizing in two, idiopathic in 11, and secondary to medications in six cases (steroids, 2; L-asparaginase, 4), gallstones in 17, and choledochal cysts in 6. Pancreatitis resolved after observation and conservative therapy in ten idiopathic cases, 4/6 medication-related cases, and following correction of biliary tract disease (15/17) or choledochal cysts (6). Pancreatic resection or drainage was required in the remaining cases. Pancreatic disorders can be accurately detected with computed tomography (CT) scan in most cases (excluding insulinoma). Ultrasound (US) is useful in cases of biliary tract disease and pseudocyst formation. Traumatic pseudocysts can resolve spontaneously or with US-guided percutaneous drainage (in the presence of normal ducts). Children with neoplasms, abnormal pancreatic ducts, or recurrent pancreatitis require resection or appropriate drainage procedures. Overall survival was 95%.

Fifty-three of 177 infants with necrotizing enterocolitis (NEC) developed portal vein air (PVA). These infants were evaluated for prenatal, perinatal, therapeutic, clinical, laboratory, radiographic, and operative factors as they related to infants with and without PVA. A significantly higher incidence of PVA was seen in infants with NEC who weighed less than 2400 g (P less than 0.025). The use of Vitamin E (P less than 0.01), aminophylline, and high-density premature formula (P less than 0.001) was associated with an increased incidence of PVA. NEC totalis was seen in 55% of the infants with NEC and PVA (P less than 0.001). The presence of PVA was associated with a 58% mortality rate (P less than 0.05). Infants with NEC and PVA operated on prior to 1983 had a 71% mortality. Since 1983, seven of nine infants with NEC and PVA survived early operation using PVA as an indication for surgical intervention. In infants at risk for this highly lethal form of NEC, the avoidance of enteral feedings or altered dietary intake, cautious placement of arterial catheters and the judicious application of pharmacologic agents (eg, Vitamin E, aminophylline) is of importance in regard to prevention. These observations suggest that PVA is a sign of advanced disease and that these high-risk patients should be considered candidates for early surgical intervention.

Hydrocephalus is a frequently encountered problem in infancy and is most commonly treated by placement of ventriculoperionteal (VP) or ventriculoatrial (VA) shunts. Other sites for insertion of the distal shunt have included the stomach, ureter, and fallopian tube. This report describes an experience with ventricular gallbladder shunts (VGB) in 25 children performed from 1970 to 1985. There were 13 girls and 12 boys ranging in age from 6 months to 16 years. Diagnosis included meningomyelocoele (7), congenital hydrocephalus (7), postmeningitic complications (5), intracranial tumor (4), and intraventricular hemorrhage (2). Indications for operation included VP shunt infection (15), massive ascites following VP shunt (3), VA shunt infection (4), and distal shunt malfunction due to fibrinous adhesions or cysts (secondary to infection; (3). Three patients had early shunt failure due to proximal obstruction (2) and gallbladder atony (1). Shunt revision was required in two and the atony was successfully treated with cholecystokinin in one. Fourteen shunts remain in place, two patients have been lost to follow-up, and three children died from unrelated causes. Seventy percent of the 20 patients available for long-term follow-up have functional shunts in place. The VGB shunt procedure remains an attractive alternative for patients with hydrocephalus in whom intraperitoneal and intravascular shunts are no longer feasible.

Pneumatosis intestinalis (PI) is a well-recognized manifestation of necrotizing enterocolitis (NEC) in the new-born—a condition that oftern requires surgical intervention for infarcted bowel. However, little information is available concerning PI in older children or its management. Sixteen older infants and children (>2 months) had x-ray findings of PI (intramural air). There were eight girls and eight boys ranging in age from 2 months to 8 years. Associated conditions included short bowel syndrome (SBS) (8), congenital heart disease (2), iron ingestion (1), nesidioblastosis (1), hemolytic anemia (1), rheumatoid arthritis (1), bronchopulmonary dysplasia (BPD) (1), and malrotation (1). Clinical presentation included abdominal distension (13), bloody diarrhea (12), bilious emesis (5), and lethargy (5). Two patients on steroids had unsuspected PI identified as an incidental operative finding during pancreatectomy for nesidioblastosis (1) and splenectomy for hemolytic anemia (1), respectively. Only four other children (iron toxicity, postcardiac catheterization, rheumatoid arthritis, and BPD required surgical intervention. Each manifested peritioneal irritation, acidosis, and hypotension or had pneumoperitoneum on abdominal x-ray. In ten of 14 patients, PI was managed nonoperatively with neasogastric suction, fluid resuscitation, intravenous (IV) antibiotics (seven to ten days), and repeated abdominal x-ray and physical examinations. Children with SBS comprised 50% of the total number of patients and eight of ten treated by observation. All had associated viral syndromes (rotavirus) or rhotozyme-positive stools and developed bloody diarrhea. There were two deaths (12.5%) in patients with iron toxicity and congenital heart disease who required resection of gangrenous bowel. All of the other patients survived. These observations suggest that in older infants and children, careful nonoperative management and antibiotic therapy can be safely employed in instances of pneumatosis intestinalis. In 75% of these children (including all with SBS), PI was successfully managed nonoperatively. In older children, pneumatosis intestinalis alone is not an indication for operation. Operation is indicated, however, in patients with pneumoperitoneum and/or signs of progressive clinical deterioration (acidosis, peritoneal irritation, etc) as is currently practiced in the management of the neonatal PI group. The relationship of steroid administration and pneumatosis intestinalis is unclear. Rotavirus infection frequently occurs in infants and children with SBS and might be implicated in the development of their pneumatosis intestinalis. None of the patients with SBS required operative intervention. Intestinal adaptation, however, was significantly delayed in these cases. PI has not been previously reported as a late complication of SBS. Loss of mucosal integrity and subsequent bacterial invasion might be etiologic factors in these cases.

This report concerns 60 infants and children with short bowel syndrome, most commonly caused by necrotizing enterocolitis in this study. Resection of atretic or gangrenous bowel was performed in 53 patients, tapering enteroplasty and primary anastomosis was performed in 13 patients, and temporary enterostomies were performed in 40 patients. Second-look laparotomy was useful in two of four cases of questionable bowel viability. The ileocecal valve was resected in 33 patients and remained intact in 27. The mean length of remaining bowel was 58.4 cm (range 13 to 150 cm). Seven patients with total aganglionosis and mid to proximal small bowel extension were managed with an initial enterostomy, whereas three had a pull-through procedure with an aganglionic patch enteroplasty. All patients received total parenteral nutrition and early enteral feedings. Home hyperalimentation was attempted when 50 percent of the calorie intake was enteral. Intestinal adaptation required from 3 to 14 months. Frequent setbacks were related to catheter sepsis, rotavirus infection, carbohydrate intolerance, and liver dysfunction. The overall survival rate was 85 percent, with mortality due to liver failure and sepsis associated with total parenteral nutrition.

This report describes a 7-year-old boy presenting with watery diarrhea, hypokalemia, and hypochlohydria associated with vasoactive intestinal polypeptide (VIP)-secreting ganglioneuromatosis involving the entire colon and rectum. The child's symptoms resolved following proctocolectomy, and the VIP levels returned to normal. Although 55 previous children have been reported with VIP-secreting tumors, this case is the first involving the entire colon and recturm.