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Intestinal Atresia and Stenosis a 25-Year Experience with 277 Cases

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Intestinal Atresia and Stenosis a 25-Year Experience with 277 Cases PAPER Intestinal Atresia and Stenosis A 25-Year Experience With 277 Cases Laura K. Dalla Vecchia, MD; Jay L. Grosfeld, MD; Karen W. West, MD; Frederick J. Rescorla, MD; L. R. Scherer, MD; Scott A. Engum, MD Objective: To evaluate the causes, clinical presenta- excision in 5 (4%), and the Bianchi procedure in tion, diagnosis, operative management, postoperative care, 1(0.8%). Patients with colon atresia were managed and outcome in infants with intestinal atresia. with initial ostomy and delayed anastomosis in 18 (86%) of 21 patients and resection with primary anas- Design: Retrospective case series. tomosis in 3 (14%). Short-bowel syndrome was noted in 32 neonates. Setting: Pediatric tertiary care teaching hospital. Main Outcome Measures: Morbidity and early and Patients: A population-based sample of 277 neonates late mortality. with intestinal atresia and stenosis treated from July 1, 1972, through April 30, 1997. The level of obstruction Results: Operative mortality for neonates with duode- was duodenal in 138 infants, jejunoileal in 128, nal atresia was 4%, with jejunoileal atresia, 0.8%, and with and colonic in 21. Of the 277 neonates, 10 had obstruc- colonic atresia, 0%. The long-term survival rate for chil- tion in more than 1 site. Duodenal atresia was as- dren with duodenal atresia was 86%; with jejunoileal atre- sociated with prematurity (46%), maternal poly- sia, 84%; and with colon atresia, 100%. The Bianchi pro- hydramnios (33%), Down syndrome (24%), annular cedure (1 patient, 0.8%) and growth hormone, glutamine, pancreas (33%), and malrotation (28%). Jejunoileal atre- and modified diet (4 patients, 1%) reduced total paren- sia was associated with intrauterine volvulus, (27%), gas- teral nutrition dependence. troschisis (16%), and meconium ileus (11.7%). Conclusions: Cardiac anomalies (with duodenal atre- Interventions: Patients with duodenal obstruction sia) and ultrashort-bowel syndrome (,40 cm) requir- were treated by duodenoduodenostomy in 119 (86%), ing long-term total parenteral nutrition, which can be of 138 patients duodenotomy with web excision in 9 complicated by liver disease (with jejunoileal atresia), are (7%), and duodenojejunostomy in 7 (5%) A duode- the major causes of morbidity and mortality in these pa- nostomy tube was placed in 3 critically ill neonates. tients. Use of growth factors to enhance adaptation and Patients with jejunoileal atresia were treated with advances in small bowel transplantation may improve resection in 97 (76%) of 128 patients (anastomosis, 45 long-term outcomes. [46%]; tapering enteroplasty, 23 [24%]; or temporary ostomy, 29 [30%]), ostomy alone in 25 (20%), web Arch Surg. 1998;133:490-497 NTESTINAL ATRESIA is a well- RESULTS recognized cause of bowel ob- struction in the newborn. The DUODENAL ATRESIA management of neonates with in- AND STENOSIS testinal atresia has improved in Irecent decades due to refinements in Duodenal obstruction was found in 138 neonatal intensive care, operative tech- (50%) of the 277 neonates enrolled in the nique, use of total parenteral nutrition study, including 79 females (57%) and 59 (TPN), and neonatal anesthesia. This males (43%). Prematurity, defined as a ges- review evaluates the causes, clinical pre- tational age of 37 weeks or less, was noted From the Department of sentation, diagnosis, operative manage- in 64 neonates (46%). Maternal polyhy- Pediatric Surgery, James ment, postoperative care, and outcomes dramnios was noted in 45 neonates (33%) Whitcomb Riley Hospital for of 277 patients with intestinal atresia and evidence of duodenal obstruction on Children, Indiana University treated at a pediatric tertiary facility for a prenatal ultrasonograms was observed in Medical Center, Indianapolis. 25-year period. 22 (16%). In addition, the conditions of ARCH SURG/ VOL 133, MAY 1998 490 ©1998 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/23/2021 Type III Atresia (12%) Type II Atresia (12%) Type I Atresia (43%) Stenosis (33%) Figure 2. Pathological types of duodenal atresia and stenosis noted in the current series. Table 2. Associated Operative Findings With Duodenal Atresia and Stenosis Figure 1. Abdominal radiograph demonstrating the characteristic Pathologic Type No. (%) of Cases “double-bubble” sign associated with duodenal atresia. Annular pancreas 46 (33) Malrotation 39 (28) Anterior portal vein 7 (5) Table 1. Congenital Anomalies Associated Jejunoileal atresia 5 (3.6) With Duodenal Atresia Second distal web 2 (1.5) Colonic atresia 2 (1.5) Type No. (%) of Cases Ectopic pancreatic rests 2 (1.5) Meckel diverticulum 2 (1.5) Cardiac 53 (38) Jejunal duplication cysts 1 (,1) Renal 19 (14) Esophageal atresia or tracheoesophageal fistula 8 (6) Imperforate anus 7 (5) Skeletal 8 (6) Central nervous system 4 (3) distal duodenal atresia. Duodenoduodenostomy was per- Other* 11 (8) formed in 119 neonates (86%), duodenotomy with web excision in 9 (7%), and duodenojejunostomy in 7 (5%). *Other indicates additional anomalies. Three neonates underwent placement of a duodenos- tomy tube because of their critical condition. Addi- tional intraoperative findings included malrotation, an- 1 patient with omphalocele and 2 patients with gastros- nular pancreas, and anterior portal vein (Table 2). Ladd chisis had been diagnosed prenatally by ultrasonogram. procedure with appendectomy was performed in pa- Down syndrome (trisomy 21) was present in 33 neo- tients with evidence of malrotation. A concomitant gas- nates (24%). Clinical presentation included bilious eme- trostomy tube was placed in 50 neonates, most in the first sis or aspirates in 126 neonates, upper abdominal dis- 10 years of this series. In patients with esophageal atre- tension in 13, and feeding intolerance in 13. Diagnosis sia, the duodenal repair was performed prior to treating was achieved in most instances by plain abdominal ra- the esophageal atresia. Neonates with high imperforate diographs, which demonstrated the characteristic “double- anus also had a colostomy performed at the time of their bubble” sign in 108 neonates (Figure 1). No gas was duodenal atresia repair. observed beyond the second bubble in instances of atre- Postoperative complications included anastomotic sia. Upper gastrointestinal contrast-enhanced examina- obstruction in 4 neonates (3%), congestive heart failure tion showed partial duodenal obstruction in 48 neo- in 13 (9%), prolonged adynamic ileus in 6 (4%), pneu- nates with duodenal stenosis. A variety of associated monia in 7 (5%), and superficial wound infection in 4 congenital anomalies was noted, with cardiac or renal ab- (3%). Late complications included adhesive bowel ob- normalities most frequently documented (Table 1). struction in 13 children (9%), late duodenal dysmotility Surgery was performed after administration of in- resulting in megaduodenum that required tapering duo- travenous fluids to correct hypovolemia and electrolyte denoplasty in 5 (4%), and gastroesophageal reflux dis- abnormalities. Findings at the time of operation in- ease unresponsive to medical management that re- cluded atresia in 92 neonates (67%) (mucosal web [type quired antireflux surgery (Nissen fundoplication) in 7 I] in 59 [64%], fibrous cord [type II] in 16 [17%], or com- (5%). A late-appearing choledochal cyst that required ex- plete separation [type III] in 17 [18%]) and stenosis in cision and Roux-en-Y hepaticoenterostomy occurred in 46 (33%) (Figure 2). Two patients had a second, more 2 teenagers (1%). ARCH SURG/ VOL 133, MAY 1998 491 ©1998 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/23/2021 graphs usually showed an unused microcolon, a finding that reveals the obstruction is limited to the small intes- tine. The barium enema also documented the location of the cecum, alerting us to instances of associated dis- orders of intestinal rotation and fixation. Free air was ob- served on plain abdominal radiographs in 2 neonates I II (1.6%). Associated congenital anomalies included om- phalocele in 2 neonates (1.6%), cardiac anomalies in 10 (8%), skeletal anomalies in 3 (2%), gastroschisis in 21 (16%), renal abnormalities in 5 (4%), concurrent duo- denal atresia in 5 (4%), associated colonic atresia in 3 (2%), and central nervous system malformations in 4 (3%). At the time of surgery, 29 (23%) of the jejunoileal IIIa IIIb atresias were classified as type I (mucosal), 35 (27%) as type II (atretic fibrous cord), 23 (18%) as type IIIa (V- shaped mesenteric defect), 9 (7%) as type IIIb (“apple peel” atresia), and 31 (24%) as type IV (multiple atre- sias) (Figure 3). Other intraoperative findings in- cluded volvulus in 34 neonates (27%), malrotation in 24 (19%), meconium peritonitis in 10 (8%), Meckel diver- ticulum in 3 (2%), and intrauterine intussusception in 2 IV (1.6%). Additional atresias were noted in the colon in 3 Figure 3. Classification of jejunoileal atresia describes the pathology as patients and in the duodenum in 5 (4%). Operative man- type I (mucosal web), type II (fibrous cord), type IIIa (mesenteric gap agement included resection of the atresia with primary defect), type IIIb (“apple peel”), or type IV (multiple atresias). Reprinted from Pediatric Surgery1(p843) with permission from Mosby-Year Book Inc, formerly bowel anastomosis in 45 neonates (35%), resection with Year Book. tapering enteroplasty in 23 (18%), temporary ostomy in 54 (42%) (with intestinal resection in 29 [57%] of those), enterotomy with web excision in 5 (4%), and a Bianchi The operative
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