Adult Outcomes After Newborn Respiratory Failure Treated With Extracorporeal Membrane Oxygenation.

OBJECTIVE: To describe the outcome of young adults treated for hypoxemic respiratory failure with extracorporeal membrane oxygenation as neonates. DESIGN: The study was designed as a multisite, cross sectional survey. SETTING: The survey was completed electronically or on paper by subjects and stored in a secure data base. SUBJECTS: Subjects were surviving neonatal extracorporeal membrane oxygenation patients from eight institutions who were18 years old or older. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: A questionnaire modified from the 2011 Behavioral Risk Factor Surveillance System and the 2011 National Health Interview Survey with additional unique questions was completed by subjects. Results were compared to age-matched national Behavioral Risk Factor Surveillance System and National Health Interview Survey data. One hundred and forty-six subjects participated (8.9% of eligible candidates). The age at questionnaire submission was 23.7 ± 2.89 years. Subjects differed statistically from national cohorts by being more satisfied with life (93% vs 84.2%); more educated (some college or degree; 80.1% vs 57.7%); more insured for healthcare (89.7% vs 72.3%); less frequent users of healthcare in the last 12 months (47.3% vs 58.2%); more limited because of physical, mental, and developmental problems (19.9% vs 10.9%); and having more medical complications. Furthermore, learning problems occurred in 29.5% of the study cohort. The congenital diaphragmatic hernia group was generally less healthy and less well educated, but equally satisfied with life. Perinatal variables contributed little to outcome prediction. CONCLUSIONS: Most young adult survivors in this study cohort treated with extracorporeal membrane oxygenation as neonates are satisfied with their lives, working and/or in college, in good health and having families. These successes are occurring despite obstacles involving health issues such as asthma, attention deficit disorder, learning difficulties, and vision and hearing problems; this is especially evident in the congenital diaphragmatic hernia cohort. Selection bias inherent in such a long-term study may limit generalizability, and it is imperative to note that our sample may not be representative of the whole.

A single histrelin implant is effective for 2 years for treatment of central precocious puberty.

We investigated whether a "yearly" histrelin implant would provide pubertal suppression when left in place for 2 years. Equivalent suppression was observed when comparing 12 and 24 months in 33 children with central precocious puberty. A single implant for 2 years reduces cost and number of implant procedures.

Choledochoceles: are they choledochal cysts?

OBJECTIVE: The aim of this analysis was to report a multidisciplinary series comparing choledochoceles to Todani Types I, II, IV, and V choledochal cysts. SUMMARY BACKGROUND DATA: Choledochoceles have been classified as Todani Type III choledochal cysts. However, most surgical series of choledochal cysts have reported few choledochoceles because they are managed primarily by endoscopists. METHODS: Surgical, endoscopic, and radiologic records were reviewed at the Riley Children's Hospital and the Indiana University Hospitals to identify patients with choledochal cysts. Patient demographics, presenting symptoms, radiologic studies, associated abnormalities, surgical and endoscopic procedures as well as outcomes were reviewed. RESULTS: A total of 146 patients with "choledochal cysts" including 45 children (31%) and 28 with choledochoceles (18%) were identified, which represents the largest Western series. Patients with choledochoceles were older (50.7 vs. 29.0 years, P < 0.05) and more likely to be male (43% vs. 19%, P < 0.05), to present with pancreatitis (48% vs. 24%, P < 0.05) rather than jaundice (11% vs. 30%, P < 0.05) or cholangitis (0% vs. 21%, P < 0.05), to have pancreas divisum (38% vs. 10%, P < 0.01), and to be managed with endoscopic therapy (79% vs. 17%, P < 0.01). Two patients with choledochoceles (7%) had pancreatic neoplasms. CONCLUSIONS: Patients with choledochoceles differ from patients with choledochal cysts with respect to age, gender, presentation, pancreatic ductal anatomy, and their management. The association between choledochoceles and pancreas divisum is a new observation. Therefore, we conclude that classifications of choledochal cysts should not include choledochoceles.

Management of Peritoneal Dialysis Catheters That Erode Into Bowel: Two Pediatric Case Reports and a Review of the Literature.

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.

Prophylactic Antibiotics for Prevention of Cholangitis in Patients With Biliary Atresia Status Post-Kasai Portoenterostomy: A Systematic Review.

PURPOSE: To determine effectiveness of prophylactic antibiotics in preventing cholangitis, we conducted a systematic review comparing cholangitis occurrence in biliary atresia patients after Kasai portoenterostomy (KP) with and without antibiotics. METHODS: We searched online bibliographic databases from April 1, 2013, using search terms "biliary atresia" OR "cholangitis" AND "antibiotics," selecting studies with control group data. RESULTS: Four of 509 titles met inclusion criteria, yielding a total of 319 patients from 3 countries. Three studies were retrospective cohorts and one was a randomized clinical trial. Two cohort studies concluded that prophylactic antibiotics reduced incidence of cholangitis and one did not. The randomized clinical trial supported prophylaxis after comparing the prospective randomized groups to a historical control group. CONCLUSION: Few published studies measure the effect of prophylactic antibiotics after Kasai portoenterostomy. We identified 4 studies and they presented contradictory results. Prospective research is needed.

Surgical considerations in cystic fibrosis: a 32-year evaluation of outcomes.

BACKGROUND: Information concerning long-term operative outcomes in patients with cystic fibrosis (CF) is relatively sparse in the operative literature. METHODS: A retrospective review of CF patients with operative conditions was performed (1972-2004) at a tertiary children's hospital to analyze outcomes including long-term morbidity and survival. RESULTS: A total of 226 patients with CF presented with an operative diagnosis (113 men, 113 women). A total of 422 operations were performed in 213 patients (94%). The mean age at operation was 4.1 +/- 6.2 years (range, 1 d to 26 y) and 109 were neonates. Fifteen of 42 (36%) babies with simple meconium ileus (MI) were treated nonoperatively with hypertonic enemas, 27 of 42 and all 45 patients with complicated MI required operation, including 15 with jejunoileal atresia (17%). Seventeen of 27 (63%) patients with meconium ileus equivalent had MI as neonates; 7 of 27 (26%) required operation. Eight of 9 (89%) with fibrosing colonopathy required operation. Organ transplantation was required in 21 patients. Follow-up evaluation was possible in 204 of 213 (96%) patients. The duration of follow-up evaluation was 14.9 +/- 8.5 years (range, 2 mo to 35 y). Operative morbidity was 11% at 1 year, 2% at 2 to 4 years, 1% at 5 to 10 years, and less than 1% at more than 10 years. There were 24 deaths (11%); 22 followed CF-related pulmonary complications and included 8 of 16 (50%) children with pneumothorax. CONCLUSIONS: Long-term survival in CF patients has improved significantly (89%), with many surviving into the fourth decade. MI may predispose to late complications including meconium ileus equivalent and fibrosing colonopathy. Pneumothorax in CF patients is an ominous predictor of mortality. Children with CF are living longer and are good candidates for operation, but require long-term follow-up evaluation because of ongoing exocrine dysfunction.

Surgical management of duodenal injuries in children.

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.

Laparoscopic splenectomy has become the gold standard in children.

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.

Influence of extracorporeal membrane oxygenation on subsequent surgeries after congenital diaphragmatic hernia repair.

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.

Oophorectomy in McCune-Albright syndrome: a case of mistaken identity.

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 café 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 café 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.

Laparoscopic splenic procedures in children: experience in 231 children.

OBJECTIVES: The purpose of this report is to evaluate the efficacy of and complications observed after laparoscopic splenic procedures in children. METHODS: Review of a prospective database at a single institution (1995-2006) identified 231 children (129 boys; 102 girls; average age 7.69 years) undergoing laparoscopic splenic procedures. RESULTS: Two hundred twenty-three children underwent laparoscopic splenectomy (211 total; 12 partial) by the lateral approach. Indication for splenectomy was hereditary spherocytosis (111), immune thrombocytopenic purpura (36), sickle cell disease (SCD) (51), and other (25). Four (2%) required conversion to an open procedure. Eight additional laparoscopic splenic procedures were performed: splenic cystectomy for epithelial (4) or traumatic (2) cyst, and splenopexy for wandering spleen (2). Average length of stay was 1.5 days. Complications (11% overall, 22% in SCD patients) included ileus (5), bleeding (4), acute chest syndrome (5), pneumonia (2), portal vein thrombosis (1), priapism (1), hemolytic uremic syndrome (1), diaphragm perforation (2), colonic injury (1), missed accessory spleen (1), trocar site hernia (1), subsequent total splenectomy after an initial partial (1), and recurrent cyst (1). Subsequent operations were open in 3 (colon repair, hernia, and missed accessory spleen) and laparoscopic in 2 (completion splenectomy, and cyst excision). There were no deaths, wound infections, or instances of pancreatitis. CONCLUSIONS: Laparoscopic splenic procedures are safe and effective in children and are associated with low morbidity, higher complication rate in SCD, low conversion rate, zero mortality, and short length of stay. Laparoscopic splenectomy has become the procedure of choice for most children requiring a splenic procedure.

Long-term outcomes in patients with stage IV neuroblastoma.

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 (> or = 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.

Effect of corticosteroid therapy on outcomes in biliary atresia after Kasai portoenterostomy.

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.

Incidence of persistent left superior vena cava in esophageal atresia.

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.

Long-term outcomes in total colonic aganglionosis: a 32-year experience.

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.

Supraumbilical pyloromyotomy: a unique indication for antimicrobial prophylaxis.

BACKGROUND: 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. METHODS: 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). RESULTS: 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, P < 1.0 and group 2 [n = 85] vs group 3 [n = 42], P < .3). CONCLUSIONS: 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.

Appendicitis in childhood hematologic malignancies: analysis and comparison with typhilitis.

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 < .05. 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.

Duodenal atresia and stenosis: long-term follow-up over 30 years.

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.