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Erosion of peritoneal dialysis (PD) catheters into the intestine is a rare complication of PD. Herein, we convey the first reports of 2 pediatric patients undergoing PD who were found to have the catheter eroding into their intestines. They were treated minimally with catheter removal and antibiotics. Definitive repair of the intestinal injury was not performed. These are the first pediatric patients reported with PD catheter erosion. Perforating injuries may be self-limiting, and therefore a more minimal approach may be considered in certain patient populations who do not express overt signs of peritonitis or illness.

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.

Splenectomy is frequently required in children with various hematologic disorders. The reported advantages of laparoscopic splenectomy (LS) include less pain, shorter hospital stay, and improved cosmesis. This report evaluates the outcome of children undergoing LS at a single children's facility. One hundred twelve children underwent LS by the lateral approach between August 1995 and February 2001. Indications for LS were hereditary spherocytosis in 58, idiopathic thrombocytopenic purpura in 21, sickle cell disease in 19, and other conditions in 14. LS alone was completed in 89 children and LS and cholecystectomy (LSC) in 20. Three required conversion to open splenectomy. Accessory spleens were identified in 19. Complications included ileus (four), acute chest syndrome (four), bleeding (two), pneumonia (one), and diaphragm perforation (one). There was no mortality. An accessory spleen was missed in one child with recurrent anemia. Average operative time for LS was 106 minutes and for LSC 135 minutes. Operative time for LS decreased with experience but the difference was not significant. Average length of stay was 1.51 days (range 1–11) and was longer in sickle cell disease (2.47 days) versus hereditary spherocytosis (1.29 days) and idiopathic thrombocytopenic purpura (1.16 days). We conclude that LS is safe and effective in children with hematologic disorders and is associated with minimal morbidity, zero mortality, and a short length of stay.

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.

Objective The objective of the study was to determine the incidence of oophorectomy in girls presenting with precocious puberty and vaginal bleeding who are subsequently diagnosed with McCune-Albright syndrome (MAS). Study Design Medical records of girls diagnosed with MAS between 1988 and 2005 were reviewed. Variables analyzed included presenting features, presence of cafe au lait macules, presence of fibrous dysplasia, radiographic studies, estradiol levels, tumor markers, surgery, and pathology reports. Results Nine girls with MAS were identified. Average age at initial presentation was 3.2 ± 2.1 years (range, 0.6-7 years). All patients presented with sudden onset of vaginal bleeding. Eight (88%) also had breast development and 2 (22%) had associated pubic hair. Four (44%) girls underwent salpingo-oophorectomy before the diagnosis of MAS was made. Of these, 3 had cafe au lait macules on initial presentation, and 3 were later diagnosed with fibrous dysplasia. Surgical pathology revealed benign ovarian cysts in all 4 patients. Conclusion Unnecessary oophorectomy is common in girls with MAS who are taken to the operating room for a presumed ovarian tumor. This highlights the need for increased awareness of MAS among pediatricians, pediatric surgeons, and emergency room physicians. Distinguishing features, which can be helpful in differentiating these 2 conditions, are often present.

Aim of Study Long-term outcome studies in survivors with stage IV neuroblastoma (NB) are sparse. This review evaluates late complications and long-term outcomes in stage IV NB survivors. Methods A retrospective review of stage IV NB survivors was performed to analyze outcomes, including long-term morbidity, recurrence, and survival. Main Results Of 153 patients with stage IV NB, 52 (34%) survived (male-female, 26:26). Age at diagnosis was 29.1 ± 31.7 months in survivors. Eighteen were 1 year or younger and 34 were older than 1 year compared with 10 nonsurvivors 1 year or younger and 91 older than 1 year ( P = .0003, Fisher's Exact test). Primary tumor sites were adrenal (35), retroperitoneal (11), mediastinal (3), pelvic (2), and no primary with tumor metastases identified (1). Ten survivors had favorable and 16 had unfavorable histology compared with 1 favorable and 18 unfavorable in nonsurvivors ( P = .01). Four survivors had MYCN amplification (≥10 copies) and 2 deletions of 1p and 11q. Sites of metastasis in survivors and nonsurvivors were similar. Treatment in survivors included surgery in 51 (75% [39/51] complete tumor resection [CTR]); chemotherapy, 50; radiation, 17; stem cell transplantation, 20; and bone marrow transplant, 1. In nonsurvivors, 13 (25%) of 53 ( P ≪ .0001) had CTR, 18 stem cell transplantation, and 12 bone marrow transplant. Six patients had tumor recurrence but survived (mean, 9.3 ± 8.3 years; range, 6 months–24 years). Recurrence was local (1), distant (2), and both (3) and was treated by resection, chemotherapy, and radiation. The mean age of survivors was 12.4 ± 8.3 years (range, 2-34 years). In all stage IV cases, event-free survival was 30% and overall survival was 34%. Long-term complications occurred in 23 (44%) survivors, including endocrine disturbances (7), orthopedic (5), cataracts (2), adhesive bowel obstruction (2), hypertension (1), bronchiolitis (1), blindness (1), peripheral neuropathy (1), nonfunctioning kidney (1), cholelithiasis (1), and thyroid nodule (1). Conclusion Only 34% of patients with stage IV NB survived despite aggressive multimodal therapy. Age of younger than 1 year, favorable pathology, CTR, and no recurrence were the only statistically significant factors that favored survival. Forty-four percent of survivors experienced late morbidity, and tumor recurred in 6 (11.5%) of 52. Patients should be monitored for tumor recurrence and long-term sequelae. New methods of treatment are required to achieve better outcomes.

Purpose This study tests the hypothesis that steroid administration improves the outcome of biliary atresia (BA) by evaluating the efficacy of postoperative steroid use on surgical outcomes in infants with BA. Methods Steroid use and outcomes in patients with BA were retrospectively analyzed at a tertiary pediatric hospital. Institutional review board approval was obtained. Results Kasai portoenterostomy (PE) was performed in 43 patients with BA treated from 1992 to 2004 (16 boys and 27 girls). Twenty-one PE patients received steroids and 22 did not. Portoenterostomy was successful in 24 patients (55.8%) with consistent serum bilirubin less than 2 mg/dL. Sixteen (66%) received postoperative steroids. A normal postoperative bilirubin was achieved at 6 months in 16 (76%) of 21 patients with steroids compared with 8 (37%) of 22 in untreated controls (Fisher's Exact test, P = .01). Of the 43 patients, 19 (44%) required liver transplantation, including 7 (37%) of 19 with steroids vs 12 (63%) of 19 without ( P = .2). Twenty-eight infants developed cholangitis (fever with and without changes in hepatic function): 25 after PE and 3 after transplant. Of the 25, 12 (48%) received steroids. Seven died (16%) (range, 7 months to 4 years): 2 while awaiting transplantation (received steroids) and 5 after transplantation (1 received steroids and 4 were untreated). Survival was 86% (18/21) in patients with steroids and 82% (18/22) in those without. Transplant survival (74%) was comparable to previously reported historical controls (82%). Conclusions The Kasai PE continues to be the procedure of choice in infants with BA younger than 3 months. A significantly improved clearance of postoperative jaundice and lower serum bilirubin levels were observed in patients receiving steroids. However, steroids had no effect on the incidence of cholangitis, need for liver transplantation, and overall survival. A prospective study with standardized dose and length of steroid administration and longer period of follow-up is necessary to more accurately assess the effectiveness of steroids after PE.

Background/Methods A 32-year retrospective review from 1972 to 2004 analyzed complications and long-term outcomes in children with total colonic aganglionosis (TCA) as they relate to the procedure performed. Results Thirty-six patients (27 boys, 9 girls) had TCA. The level of aganglionosis was distal ileum (26), mid–small bowel (8), midjejunum (1), and entire bowel (1). Enterostomy was performed in 35 of 36. Eight developed short bowel syndrome. Twenty-nine (81%) had a pull-through at 15 ± 6 months (modified Duhamel 20, Martin long Duhamel 4, and Soave 5). Six had a Kimura patch. Postoperative complications (including enterocolitis) were more common after long Duhamel and Soave procedures. Seven (19%; 2 with Down's syndrome) died (3 early, 4 late) from pulmonary emboli (1), sepsis (1), fluid overload (1), viral illness (1), liver failure (1), arrhythmia (1), and total bowel aganglionosis (1). Mean follow-up was 11 ± 9 years (range, 6 months-29 years). Twenty-four (83%) of 29 patients exhibited growth by weight of 25% or more, 21 (91%) of 23 older than toddler age had 4 to 6 bowel movements per day, and 17 (81%) of 21 were continent. In 5 of 6, the Kimura patch provided functional benefit with proximal disease. Conclusion Long-term survival was 81%. The highest morbidity occurred with long Duhamel or Soave procedures. The modified Duhamel is our procedure of choice in TCA. Bowel transplantation is an option for TCA with unadapted short bowel syndrome.

Background/Purpose Recognition of appendicitis in the child with hematologic malignancy may be difficult particularly in the setting of neutropenia and multiple medications causing an altered inflammatory response. Typhilitis may produce a similar constellation of clinical findings causing further diagnostic confusion. This review compares the relative frequency of these two conditions in children with hematologic malignancy with a focus on the clinical presentation, distinguishing features, surgical management, and outcome for patients with appendicitis. Methods This institutional review board–approved retrospective study evaluated 464 pediatric patients treated for hematologic malignancy at our institution from 1997 to 2003. From this cohort, we identified all children with a diagnosis of appendicitis or typhilitis. Data include demographics, clinical presentation, laboratory studies, and computed tomography (CT) scan findings. Groups were compared using the Fisher exact test. Significance was defined as P Results Eight (1.7%) of 464 children were diagnosed with typhilitis and 7 (1.5%) with appendicitis. There were no demographic differences between patients with appendicitis and typhilitis. Distinguishing clinical features in children with typhilitis included presence of fever and diarrhea. Clinical presentation in children with appendicitis was atypical in 5 of 7 cases yielding an incorrect preoperative diagnosis in all 5. Radiographic evaluation by CT scan accurately defined typhilitis, but not appendicitis. An operation was performed on all 7 children with appendicitis with no operative morbidity or mortality. Conclusions Appendicitis and typhilitis occur with similar frequency in children with leukemia and lymphoma. Typhilitis is accurately diagnosed with clinical findings of fever, diarrhea, abdominal pain, and typical CT scan findings. Appendicitis tends to present with atypical findings, but can be successfully managed with standard surgical care.

Background Duodenal atresia and stenosis is a frequent cause of congenital, intestinal obstruction. Current operative techniques and contemporary neonatal critical care result in a 5% morbidity and mortality rate, with late complications not uncommon, but unknown to short-term follow-up. Methods A retrospective review of patients with duodenal anomalies was performed from 1972 to 2001 at a tertiary, children's hospital to identify late morbidity and mortality. Results Duodenal atresia or stenosis was identified in 169 patients. Twenty children required additional abdominal operations after their initial repair with average follow-up of 6 years (range, 1 month to 18 years) including fundoplication (13), operation for complicated peptic ulcer disease (4), and adhesiolysis (4). Sixteen children underwent revision of their initial repair: tapering duodenoplasty or duodenal plication (7), conversion of duodenojejunostomy to duodenoduodenostomy (3), redo duodenojejunostomy (3), redo duodenoduodenostomy (2), and conversion of gastrojejunostomy to duodenoduodenostomy (1). There were 10 late deaths (range, 3 months to 14 years) attributable to complex cardiac malformations (5), central nervous system bleeding (1), pneumonia (1), anastomotic leak (1), and multisystem organ failure (2). Conclusions Late complications occur in 12% of patients with congenital duodenal anomalies, and the associated late mortality rate is 6%, which is low but not negligible. Follow-up of these patients into adulthood is recommended to identify and address these late occurrences.

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. J Pediatr Surg 36:1302-1303. Copyright © 2001 by W.B. Saunders Company.

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. J Pediatr Surg 36:51-55. Copyright © 2001 by W.B. Saunders Company.

Background/Purpose: Minimal access surgery (MIS, Nuss Procedure) is gaining acceptance rapidly as the preferred method for pectus excavatum repair. This shift in operative management has followed a single institution's evaluation of the procedure. This report describes an additional experience with the Nuss procedure. Methods: Twenty-one patients with pectus excavatum underwent repair by the Nuss Procedure. The patients ranged in age from 5 to 15 years (average, 8.2 years). There were 19 boys and 2 girls. Results: In 1 patient (age 5 years) the MIS procedure was aborted because of persistence of chest wall asymmetry. The other 20 patients had completion of their procedure without intraoperative complication. The operating times ranged from 45 to 90 minutes; however, there was an additional anesthetic set-up time (average, 45 minutes). All cases utilized a single support bar (11 to 17 inches). Patients underwent extubation in the operating room and were admitted to a ward bed with an epidural catheter in place for pain control and received intravenous analgesia. The hospital stay ranged from 4 to 11 days and averaged 4.9 days. Early postoperative complications included ileus (n = 1), bilateral pleural effusion (n = 2), atelectasis (n = 1), fungal dermatitis (n = 1), pneumothorax (n = 1), and flipped pectus bar (n = 2). Delayed complications included flipped pectus bar (n = 2), marked pectus carinatum requiring bar removal (n = 1), mild carinatum (n = 1), mild bar deviation (n = 1), progressive chest wall asymmetry (n = 3) with 1 requiring bar removal and open pectus repair, pleural effusion (n = 1), and chronic persistent pain requiring bar removal (n = 1). The length of follow-up is 3 to 20 months with an average of 12.3 months. Conclusions: The Nuss Procedure is quick, minimally invasive, and a technically easy method to learn; however, our data indicate there is a significant learning curve. Although previous reports suggest that few complications occur, we believe further assessment of patient selection regarding age, presence of connective tissue disorder, and severe chest wall asymmetry are still needed. Long-term follow-up also will be required to assure both health professionals and the public that this is the procedure of choice for patients with pectus excavatum. J Pediatr Surg 35:246-251. Copyright © 2000 by W.B. Saunders Company.

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.

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.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.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.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.

The relationship between percutaneous endoscopic gastrostomy (PEG) and subsequent development of gastroesophageal reflux (GER) is complex and not well understood. The authors retrospectively reviewed 82 children over a 5-year period who underwent PEG tube (n = 64) or PEG button (n = 18) placement. Children were evaluated preoperatively for clinical evidence of GER (C-GER) or radiographic GER (R-GER) with upper gastrointestinal contrast study or Tc99m gastric scinitiscan. Seventy-five patients were evaluated for clinical evidence of postoperative GER by direct family contact. Eleven of 39 (28%) patients with no GER preoperatively developed GER postoperatively, eight (20%) of whom required Nissen fundoplication (NF) or gastrojejunostomy (GJ) tube. Ten of 19 (53%) with preoperative C-GER but no R-GER continued to have GER after PEG, but only three required NF or GJ. Only one of nine children who had R-GER only developed clinical GER after PEG placement. Of the eight children with both C-GER and R-GER, only two (25%) required NF or GJ and two (25%) had no postoperative GER. The authors conclude that PEG tubes are useful in infants and children and are associated with a relatively low incidence of postoperative GER. If C-GER is absent, a PEG is a reasonable procedure to consider even in the presence of R-GER.

Colonic strictures are rare in patients who have cystic fibrosis, but recently have developed in those who have been treated with delayed-release high-dose pancreatic enzyme supplements. Colonic strictures from eight such pediatric patients showed neural abnormalities consisting of ganglion cell hyperplasia and ectopia, and intermyenteric plexus hyperplasia. Cholinergic and adrenergic stains of mucosal nerve fibers were more prominent in histological sections of the cystic fibrosis strictures than in sections from colons of children without cystic fibrosis. The mean grade of staining with acetylcholinesterase in the lamina propria of the strictured cystic fibrosis colons was 2.38 +/- 1.25, compared with .93 +/- .93 (P.055) in bowels from children without cystic fibrosis. The mean grade for tyrosine hydroxylase staining in the lamina propria was 2 +/- .97 in the strictures and was .79 +/- .81 (P.05) in the bowels of children who did not have cystic fibrosis. Vasoactive intestinal peptide staining in bowels from children with cystic fibrosis with and without stricture did not differ significantly from that of children without cystic fibrosis. Vasculopathy consisting of fibrointimal hyperplasia in submucosal veins and mesenteric arteries was found only in colonic strictures owing to cystic fibrosis. Colonic strictures in patients with cystic fibrosis who received high-dose pancreatic enzyme supplements contain ganglion cell abnormalities, and mucosal cholinergic and adrenergic activity may be increased in these strictures. The stricture vasculopathy may be drug-related and/or related to increased catecholamine activity.

Necrotizing enterocolitis (NEC) and midgut volvulus (MGV) often are associated with extensive bowel necrosis. These cases may require extensive enterectomy and the formation of high or multiple stomas, and frequently are complicated by short bowel syndrome, excessive fluid losses, fistulas, stenosis, and skin breakdown. This report describes a "clip and drop-back" technique, followed by delayed anastomosis performed 48 to 72 hours later. The technique was successful in five severely ill infants (3 NEC, 2 MGV) with extensive necrosis, bowel perforation(s), and peritonitis, who required either a high stoma near the ligament of Treitz or multiple resections and enterostomies. This method removes obvious necrotic perforated bowel, controls contamination, avoids stomas (and their inherent complications in this age group), and preserves bowel length. All five babies survived. The technique is a useful addition to the pediatric surgeon's operative armamentarium in selective cases.

Recognition of pancreatic injuries is frequently delayed, and optimal treatment is often controversial. The use of endoscopic retrograde cholangiopancreatography (ERCP) has allowed accurate delineation of pancreatic ductal injuries; however, the small size of children and the concern with inducing pancreatitis and/or lesser sac contamination have limited its use in children. In 1988, the authors began using ERCP for selected pancreatic injuries. This report describes their experience with this technique and examines the role of ERCP in pediatric pancreatic injuries. Six children with pancreatic transections resulting from blunt trauma were treated between 1988 and 1993. The age range was 2 1/2 to 8 years, and the weight range was 13.6 to 27.9 kg. The average period from injury to referral to the hospital was 14 days (range, 2 to 30 days). All six children presented with chemical evidence of pancreatitis and had an initial computed tomography (CT) scan; five scans were interpreted as being normal. Five of the six patients had subsequent CT scans, which showed lesser-sac fluid collection. Three patients were treated with drainage (2 percutaneous, 1 open [outside hospital]), and when this failed, ERCP was performed, at 13.6 days (average) after presentation. These three patients underwent ERCP relatively early in the course (an average of 3 days after presentation). All six children had major ductal transections documented through ERCP. After ERCP, the serum amylase level remained elevated in three, increased in one, and normal in one. (It was not measured in one of the recent cases taken for immediate operation.)(ABSTRACT TRUNCATED AT 250 WORDS)

Adenocarcinoma arising in Barrett's esophagus has recently been described in two children aged 11 and 14 years. The long-term follow-up of Barrett's esophagus in children is not well described. We evaluated 16 cases of Barrett's esophagus in children treated at this institution during the last 16 years. Ages ranged from 1.2 to 16 years (mean, 10.3 years). There were 11 boys and 5 girls. Barrett's esophagus was documented by endoscopy in 14 instances and at autopsy in 2 patients with secretory diarrhea and tetralogy of Fallot who died of sepsis. Two children had cancer (neuroblastoma, leukemia) and died of their malignant disease. Five patients had cerebral palsy, 1 esophageal atresia, 1 Fanconi's anemia, and 5 were otherwise normal children. Six were treated medically. Eight patients underwent Nissen fundoplication for complications of gastroesophageal reflux (GER). Five patients were available for follow-up endoscopy (mean, 2 years; range, 1.1 to 5.4 years). Endoscopy was performed on a yearly basis, obtaining biopsy specimens from multiple levels of the esophagus. Four children had satisfactory clinical response to an antireflux procedure including the resolution of a stricture in one case. However, in all 5 cases persistent metaplastic epithelium was documented and showed no evidence of regression. Although there has been speculation that Barrett's esophagus in children may be more likely to revert to normal squamous epithelium than in the adult, there has been only one case of regression in 180 cases of Barrett's esophagus occurring in children described in 37 reports in the literature. These cases and our experience suggest that: (1) Barrett's esophagus is relatively common in children; (2) metaplasia almost always persists despite treatment; and (3) given the risk of carcinoma, a child with Barrett's esophagus should be followed yearly, regardless of symptoms, and endoscopic biopsy specimens should be obtained to rule out dysplasia or early malignancy.

Pancreatic tumors are rare in children. Over a 20-year period we have treated 13 children with pancreatic neoplasms. There were eight boys and five girls (age range, 4 months to 12 years). Seven tumors were benign, including five insulinomas, and two cystadenomas. Six lesions were malignant (rhabdomyosarcoma, 2; pancreatic carcinoma, 4). Children with insulinoma presented with hypoglycemia and irrational behavior. Three had abnormal insulin:glucose ratios ( greater than 1.0). The tumor was detected by computed tomography scan in three cases, at the time of surgery in one, and with intraoperative ultrasound in one. Surgical treatment included tumor enucleation in four cases and 80% pancreatectomy in one. Mucinous cystadenomas were observed in two patients, ages 4 months and 10 months. Tha latter infant underwent cyst excision alone, resulting in malignant recurrence at 18 months of age and death. The 4-month-old child had a distal pancreatectomy and is alive at 6 years. Two of the four children with pancreatic cancer had unresectable tumors at diagnosis, and were treated by biopsy (ductal adenocarcinoma), irradiation, and chemotherapy. Length of survival was 6 months and 9 months. Two others (ages 4 and 12 years) underwent 85% distal pancreatic resection for pancreatoblastoma and a pancreatoduodenectomy for papillary carcinoma, respectively. The latter is alive and tumor-free at 20 years of follow-up. The former underwent hepatic lobectomy for a 3.0 x 3.0 cm solitary liver metastases and is alive at 6 years with no evidence of disease. One child with rhabdomyosarcoma died of progressive disease, the other is alive with residual disease despite resection and chemotherapy. Most insulinomas can be treated by enucleation.(ABSTRACT TRUNCATED AT 250 WORDS)

Extracorporeal membrane oxygenation (ECMO) is an accepted form of therapy in the treatment of neonates with otherwise lethal persistent pulmonary hypertension related to meconium aspiration, congenital diaphragmatic hernia, and sepsis. This report concerns two neonates with congenital cystic lesions of the lung who developed severe pulmonary hypertension and were salvaged with lobectomy and ECMO. These cases present an additional group of patients in whom ECMO may be a life-saving measure.

Central venous access has become a frequent requirement in the management of seriously ill or injured infants and children with a wide variety of conditions. This report evaluates the complications observed with the use of central venous catheters in 1,378 cases. Central venous catheters (n = 2,281) were placed in 1,378 children (728 boys:650 girls). There were 1,012 temporary catheters (noncuffed/percutaneously placed) while 1,268 were inserted operatively, including 37 portacaths and 1,231 with dacron cuffs (Hickman, Broviac) for long-term use. A single catheter was inserted in 542 cases and multiple catheters in 836. Indications for catheter insertion included emergency resuscitation or access (501), malignancy (462), and intestinal dysfunction (415). Catheter infection occurred in 430 cases (18.8%). Of 219 infected temporary access catheters, 123 were removed while 96 were changed over a guide wire. Of 211 infected permanent catheters, 18 were immediately removed while 193 were treated with i.v. (vancomycin, gentamicin) antibiotics. Forty-seven of 193 (24%) catheters were eventually removed because of persistent or recurrent infection (16 cases) or subsequent fungal sepsis due to Candida albicans (31 cases). Only 3 of 37 portacaths were removed because of infection. Bacterial isolates were single in 125 cases and multiple in 86. Organisms included Staphylococcus epidermidis in 104 cases, Staph. aureus in 65, Klebsiella pneumoniae in 51, Escherichia coli in 51, and others in 18. Catheter complications occurred in 107 (5%) cases, including symptomatic vessel thrombosis in 49, pneumothorax in 26, catheter migration in 25, vessel injury in 5, and catheter embolus in 2. Despite the relatively high complication rate there were no catheter-related deaths. Multiple-lumen catheters had twice the complication rate and infection rate of single lumen catheters (P < 0.05). Temporary percutaneous catheters had a complication/infection rate 1.5 times greater than permanent catheters (P < 0.05) that were operatively placed. Bacterial infection cleared with antibiotics in 76% of cases with catheter sepsis, however secondary fungal infection necessitated prompt catheter removal. These data indicate that percutaneously placed catheters and multilumen catheters are associated with significantly higher complication and infection rates. Surgeons should balance the risks of convenience vs. complications in their choice of catheters and methods of insertion.

This report describes a 7-year experience with acute peritoneal dialysis in 31 neonates and infants less than 60 days of age. There were 20 boys and 11 girls, ages 3 to 60 days. Tenckhoff catheters of modified length were placed in the newborn intensive care unit (ICU), pediatric ICU, or surgery suites, and hourly exchanges (20 cc/kg) were started immediately postoperatively. Diagnoses included congenital metabolic disorders (11), acute tubular necrosis (6), postcardiopulmonary bypass with renal failure (5), renal cortical necrosis (5), obstructive uropathy (2), renal agenesis (1), and bilateral renal dysplasia (1). Complications included: peritonitis (4), bowel perforation (1), exit site infection (3), leaking dialysate (4), catheter obstruction (2), inguinal hernias (3), umbilical hernia (1), and retroperitoneal hemorrhage (1). There were 19 deaths (61.3%) from 1 to 90 days postinsertion in this high risk group. The (1), and post liver transplant (1). Effective dialysis (lowering of blood urea nitrogen (BUN) or ammonia, correction of acidosis, decrease in fluid overload) was possible in all cases. Five of the 12 survivors remain on chronic dialysis awaiting renal transplantation. Peritoneal dialysis is effective in the newborn period in the management of metabolic disturbances as well as renal failure. Morbidity and mortality (61.3%) is related to the near-morbid condition of the baby at the time of insertion and the severity of the complex underlying diagnosis often associated with multiorgan failure.

The increased use of child safety seats and seat belt restraints has significantly reduced the incidence of severe head injuries associated with motor vehicular accidents. However, an increase in the number of both acutely recognized intestinal perforations and delayed obstructions due to ischemic strictures has been noted. This report describes two children with delayed onset of intestinal obstruction related to the "seat belt syndrome" who presented with bilious emesis 3 to 6 weeks following an unrecognized lap belt injury. At laparotomy, a volvulus around an omental band adherent to a resolving traumatic mesenteric hematoma was the basis of the obstruction in both cases. The volvulus resulted in a stricture in each instance that required resection and end-to-end anastomosis. The diagnosis of posttraumatic intestinal obstruction should be suspected in children who develop nausea and bilious emesis following motor vehicular accidents in which they were wearing lap belts.

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.

Streptokinase was infused through the partially thrombosed Tenckhoff catheters of four children on continuous ambulatory peritoneal dialysis (CAPD). Radiographic contrast studies performed before and after the infusions demonstrated dramatic dissolution of the fibrin thrombi associated with restoration of normal catheter function. The catheters subsequently functioned for an average of 12.5 months. No allergic or hemorrhagic complications were noted in association with the infusions. Coagulation studies revealed no evidence for systemic streptokinase absorption. We suggest that streptokinase, when given in the manner described, is a safe and effective treatment of thrombosed Tenckhoff catheters in children.

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.

One hundred twenty-five infants underwent surgical intervention for necrotizing enterocolitis between 1972 and 1984. Sixty-three infants, who survived more than 30 days postoperatively, were evaluated for long-term complications. There were 28 girls and 35 boys (mean birth weight 1,725 +/- 890 g; gestational age 32 +/- 4 weeks). Associated problems included hyaline membrane disease (43), cardiac anomalies (25), and trisomy 21(2). Thirty-six survivors required long-term ventilatory support. Fifty-nine infants underwent bowel resection and enterostomy, 3 decompressing enterostomies without resection, and 1, exploratory laparotomy only. Enterostomies were closed at four months. Twenty four had short bowel syndrome. Fifteen infants subsequently died for a late mortality rate of 23%. Mortality was related to sepsis (3), respiratory failure (5), cardiac anomalies (3), cardio-respiratory arrest (2), and TPN related liver failure (2), and was common with gestational age less than 31 weeks and birth weight less than 1,000 g. Medical problems included cholestasis (17), TPN induced cirrhosis (3), meningitis (3), seizures (8), and nutritional rickets (6). Significant developmental and intellectual delays were observed.

Twenty one children with achalasia of the esophagus were treated from 1970 to 1986. There were 11 girls and ten boys (average age, 10.9 years; range, 6 months to 16 years). Diagnosis was established by barium swallow in 21 cases and confirmed by manometrics and motility studies in 14. Four children had unsuccessful dilatation (range, 1 to 16 dilatations/pt). All 21 children underwent modified anterior Heller esophagomyotomy (transabdominal in 15 and transthoracic in six). Concomitant Nissen fundoplication was performed in three. Follow-up from 1 to 14 years (mean, 6.3 years) showed complete relief of obstruction in 18 patients (86%), while three required additional procedures for persistent dysphagia. One child improved after a single dilatation, but two others eventually required a second esophagomyotomy. Three additional patients subsequently developed gastroesophageal reflux (GER), and two were managed with Nissen fundoplication; the third responded to medical management. The mortality for this series was zero. Postoperative complications occurred in nine children (42%) and was due to atelectasis and postoperative fever. Modified Heller esophagomyotomy is safe and effective in children with achalasia (mortality, 0%; relief of obstruction, 86%). Results were similar after a transabdominal or transthoracic approach. Esophageal dilatation was not an effective method of treatment. Although postsurgical barium swallow showed relief of obstruction, abnormal esophageal motility persisted, suggesting that long-term follow-up is important.

Approximately 10% of blunt traumatic injuries in children involved the thorax. Among these cases, traumatic diaphragmatic hernia is a relatively uncommon injury. This report describes the clinical findings and supportive and operative management in four children with traumatic diaphragmatic hernia. The hernia was on the right side in 2 patients, the left side in 1, and was bilateral in 1. There were 2 boys and 2 girls, ages 11 mo, 3.5 yr, 4.5 yr, and 6 yr. Trauma was related to vehicular-pedestrian (3) and passenger (1) injuries. Despite the fact that chest x-ray was diagnostic or highly suspicious, delay in diagnosis was observed in 2 patients (3 days, 3 mo) initially managed elsewhere. Acute respiratory distress was observed in 2 patients (pneumothorax, bilateral diaphragmatic hernias); however, all four had hypoxemia (pO 2

The effects of microwave induced hyperthermia, papaverine, and chemotherapy were evaluated in transplanted CI300 murine neuroblastoma. One hundred forty-five Ajax mice received axillary subcutaneous implants of CI300 round cell neuroblastoma (1.25 X 10(6) cells). Animals were divided into 8 treatment groups: Group 1 (n = 14) received no therapy (controls); Group 2 (n = 20) received radiofrequency microwave hyperthermia (2450 MHz at 40 milliwatts/cm2 for 4 min at 41.5 decrees C); Group 3 (n = 14) received papaverine (65 mg/kg for 3 days); Group 4 (n = 14) received a single intraperitoneal injection of cyclophosphamide (CPM) (50 mg/kg) and vincristine (VCR) (0.2 mg/kg); Group 5 (n = 22) received papaverine, cyclophosphamide, and vincristine as above; Group 6 (n = 20) received hyperthermia and papaverine; Group 7 (n = 18) received hyperthermia, cyclophosphamide, and vincristine; and Group 8 (n = 19) received hyperthermia, papaverine, cyclophosphamide, and vincristine. Therapy was instituted at 21 days after implant (tumor size 1 cm2), continuing for 2 weekly cycles. Animals were assessed for tumor regression, survival and relapse free rate at 1 and 4 wk. Tumor regression was seen only in animals receiving chemotherapy. Initial response rate (1 week) was 50% (group 4), 55% (group 5), 89% in (group 7), and 100% in mice receiving chemotherapy, papaverine, and hyperthermia (group 8) (p less than 0.05 7 and 8 versus 4 and 5). At 4 wk however, tumor relapse was noted in 6 of 7 group 4 responders, 10 of 12 in group 5, 13 of 16 in group 7, but only 4 of 19 group 8 responders (p less than .001 group 8 versus 4, 5, 7). These data indicate a synergistic effect of papaverine, microwave induced hyperthermia and chemotherapy in murine neuroblastoma. These observations suggest such treatment may be useful in clinical instances of neuroblastoma, especially in nonresponders to conventional therapy.

The distal small intestine and its mesentery contain abundant lymphoid tissues rich in B cells and IgM. An increased mortality due to sepsis has been observed after extensive distal bowel resection (80%) presumably related to excision of gut lymphoid tissue and resultant impairment of humoral immunity. This study compares the effect of live E. coli and endotoxin on mortality following massive distal bowel resection. One-hundred ninety Sprague-Dawley rats (200 g) underwent sterile midline laparotomy. Fifty animals had a sham operation (CONTROL) and the remainder an 80% distal small bowel resection (DBR). At seven days after operation, rats were divided into four groups and received an intraperitoneal challenge with either 10 8 organisms of live E. coli or 10 mg/kg of E. coli endotoxin (ENDO) and were evaluated for mortality at 72 hr. Mortality was 9% in controls+ENDO rats (2/25) (group I), 12% in CONTROL+ E. coli rats (3/25) (group II), 56% with DBR+ENDO (39/70) (group III) and 36% with DBR+ E. coli (25/70) (group IV). Mortality was increased in animals with DBR (groups III, IV) compared to controls regardless of the method of challenge ( p E. coli ( p E. coli sepsis and/or endotoxin challenge in animals with massive distal bowel resection. An LD 10 of endotoxin had a greater influence on mortality than live E. coli . These results confirm the observation that massive distal bowel resection increases the risk of mortality related to gram negative sepsis probably due to decreased humoral immunity. Mortality is adversely affected by release of endotoxin and its ultimate effect on vital organs.

Over a 10 year period, 429 Nissen fundoplications were performed on children with gastroesophageal reflux. Postoperative complications occurred in 69 children (16 percent), including wrap herniation or breakdown in 29; postoperative bowel obstruction in 18; stricture in 10; intraabdominal abscess and enterocutaneous fistula in 3 patients each; and wound infection, wound dehiscence, and inadvertent splenectomy in 2 patients each. The postoperative mortality rate was 0.9 percent (4 of 429 patients) and was related to sepsis in 1 patient, a metabolic disorder in 1 patient, and underlying pulmonary disease in 2 patients. All four patients were neurologically impaired. Fundoplication successfully controlled symptoms of gastroesophageal reflux in 395 children (92 percent) over a follow-up period ranging from 6 months to 10 years. Thirty-eight patients (8.8 percent) required a second antireflux operation because of recurrent symptoms. Twenty-nine patients had severe neurologic impairment (76 percent), 5 had associated congenital malformations (13 percent), and 3 had significant pulmonary problems (8 percent). Only one child requiring reoperation was considered otherwise normal. Indications for reoperation included wrap breakdown or herniation (28 patients), stricture (6 patients), and inadequate wrap (4 patients). Twenty-four of 28 children with wrap herniation or breakdown had neurologic impairment. A second fundoplication was successful in 35 of 38 patients (92 percent). A second procedure failed in three children, who required subsequent resection and colon interposition.