History and Physical Examination

Patients with colonic atresia or congenital stenosis may sometimes have characteristic findings on antenatal ultrasonography (US), such as dilated bowel loops or the presence of polyhydramnios. Initial physical examination findings are normal in the absence of associated conditions. The anus usually appears normal. Progressive abdominal distention develops. Rectal examination reveals white or pale mucus rather than pigmented meconium.

Failure to pass meconium completely suggests atresia, whereas delayed passage of meconium (>24 hours) suggests Hirschsprung disease. Patients with colonic atresia may pass meconium normally because the incident that caused the atresia may have occurred after the colon had become filled with meconium.

Colonic stenosis usually follows some form of injury to the colon, sustained either in utero or postnatally, and ischemia is considered central to the insult. The infant or child may present with symptoms similar to atresia with high-grade stenosis; less stenotic lesions may not become apparent until feeding is undertaken. In those instances, the child’s abdomen may become distended with feeding, and stool production is scant, if present.

Babies with necrotizing enterocolitis may show signs of acquired stenosis after their acute episode. When the septic signs of the illness resolve and the child is doing well, feeding is often attempted. If a stenosis has formed, the baby usually will not tolerate feedings, and the abdomen will become distended. Studies may be performed to confirm the diagnosis and to try to localize the site of narrowing, at the discretion of the surgeon.

Differential Diagnoses

Lupo PJ, Isenburg JL, Salemi JL, Mai CT, Liberman RF, Canfield MA, et al. Population-based birth defects data in the United States, 2010-2014: A focus on gastrointestinal defects. Birth Defects Res. 2017 Nov 1. 109 (18):1504-1514. [Medline]. [Full Text].
Fairbanks TJ, Kanard RC, Del Moral PM, Sala FG, De Langhe SP, Lopez CA, et al. Colonic atresia without mesenteric vascular occlusion. The role of the fibroblast growth factor 10 signaling pathway. J Pediatr Surg. 2005 Feb. 40 (2):390-6. [Medline].
Mirza B, Iqbal S, Ijaz L. Colonic atresia and stenosis: our experience. J Neonatal Surg. 2012 Jan-Mar. 1 (1):4. [Medline].
Patten BM. Human Embryology. New York, NY: McGraw-Hill; 1953.
Louw JH, Barnard CN. Congenital intestinal atresia; observations on its origin. Lancet. 1955 Nov 19. 269(6899):1065-7. [Medline].
Bland-Sutton JD. Imperforate ileum. Am J Med Sci. 1889. 98:457-62.
Louw JH. Investigations into the etiology of congenital atresia of the colon. Dis Colon Rectum. 1964 Nov-Dec. 7:471-8. [Medline].
Davenport M, Bianchi A, Doig CM, Gough DC. Colonic atresia: current results of treatment. J R Coll Surg Edinb. 1990 Feb. 35(1):25-8. [Medline].
Hollinshead WH. Anatomy for Surgeons. New York, NY: Hoeber-Harper; 1956. Vol 2:
Gornall P. Management of intestinal atresia complicating gastroschisis. J Pediatr Surg. 1989 Jun. 24(6):522-4. [Medline].
Wang NL, Yeh ML, Chang PY, Sheu JC, Chen CC, Lee HC, et al. Prenatal and neonatal intussusception. Pediatr Surg Int. 1998 Apr. 13 (4):232-6. [Medline].
Al-Wafi A, Morris-Stiff G, Lari A. Colonic atresia secondary to a choledochal cyst. Pediatr Surg Int. 1998 Jul. 13 (5-6):422-3. [Medline].
Werler MM, Sheehan JE, Mitchell AA. Association of vasoconstrictive exposures with risks of gastroschisis and small intestinal atresia. Epidemiology. 2003 May. 14 (3):349-54. [Medline].
de Jesus LE, Marques AM, Rocha MS, Brom BR, Siqueira RR. Left colon stenosis caused by tuberculosis. J Pediatr Surg. 2004 Oct. 39 (10):e5-7. [Medline].
Evans CH. Collective review: atresias of the gastrointestinal tract. Surg Gynecol Obstet. 1955. 92:1-8.
Webb CH, Wangensteen OH. Webb CH, Wangensteen OH. Am J Dis Child. 1931. 4:232.
Benson CD, Lotfi MW, Brogh AJ. Congenital atresia and stenosis of the colon. J Pediatr Surg. 1968 Apr. 3 (2):253-7. [Medline].
Powell RW, Raffensperger JG. Congenital colonic atresia. J Pediatr Surg. 1982 Apr. 17(2):166-70. [Medline].
Freeman NV. Congenital atresia and stenosis of the colon. Br J Surg. 1966. 53:595-7.
Gross RE. The Surgery of Infancy and Childhood. Philadelphia, Pa: WB Saunders & Co; 1953. 150-74.
Ishii D, Miyagi H, Hirasawa M, Miyamoto K. Congenital multiple colonic atresias with intestinal malrotation: a case report. Surg Case Rep. 2020 Mar 30. 6 (1):60. [Medline]. [Full Text].
Dalla Vecchia LK, Grosfeld JL, West KW, Rescorla FJ, Scherer LR, Engum SA. Intestinal atresia and stenosis: a 25-year experience with 277 cases. Arch Surg. 1998 May. 133 (5):490-6; discussion 496-7. [Medline].
Kim S, Yedlin S, Idowu O. Colonic atresia in monozygotic twins. Am J Med Genet. 2000 Mar 20. 91 (3):204-6. [Medline].
Elisa Z, Cinzia C, Sergio S, Giuseppe LV, Fortunato S. Multiple Congenital Colonic Stenosis: A Rare Gastrointestinal Malformation. Case Rep Pediatr. 2016. 2016:6329793. [Medline].
Janik JS, Ein SH, Mancer K. Intestinal stricture after necrotizing enterocolitis. J Pediatr Surg. 1981 Aug. 16 (4):438-43. [Medline].
Kosloske AM, Burstein J, Bartow SA. Intestinal obstruction due to colonic stricture following neonatal necrotizing enterocolitis. Ann Surg. 1980 Aug. 192 (2):202-7. [Medline].
Virjee JP, Gill GJ, Desa D, Somers S, Stevenson GW. Strictures and other late complications of neonatal necrotising enterocolitis. Clin Radiol. 1979 Jan. 30 (1):25-31. [Medline].
Ameh EA, Nmadu PT. Intestinal atresia and stenosis: a retrospective analysis of presentation, morbidity and mortality in Zaria, Nigeria. West Afr J Med. 2000. 19:39-42. [Medline].
Draus JM Jr, Maxfield CM, Bond SJ. Hirschsprung's disease in an infant with colonic atresia and normal fixation of the distal colon. J Pediatr Surg. 2007 Feb. 42(2):e5-8. [Medline].
Abu-Judeh HH, Methratta S, Ybasco A, Garrow E, Ali S. Congenital colonic stenosis. South Med J. 2001 Mar. 94 (3):344-6. [Medline].
Oldham KT. Atresia, stenosis and other obstructions of the colon. O'Neill JA, Jr, Rowe MI, Grosfeld JL, Fonkalsrud EW, Coran AG, eds. Pediatric Surgery. St. Louis, Mo: Mosby; 1998. 1361-8.
Sturim HS, Ternberg JL. Congenital atresia of the colon. Surgery. 1966 Mar. 59(3):458-64. [Medline].
Coran AG, Eraklis AJ. Atresia of the colon. Surgery. 1969 May. 65(5):828-31. [Medline].
Pohlson EC, Hatch EI Jr, Glick PL, Tapper D. Individualized management of colonic atresia. Am J Surg. 1988 May. 155(5):690-2. [Medline].
Smith GH, Glasson M. Intestinal atresia: factors affecting survival. Aust N Z J Surg. 1989 Feb. 59(2):151-6. [Medline].
Barrack SM, Kyambi JM, Ndungu J, Wachira N, Anangwe G, Safwat S. Intestinal atresia and stenosis as seen and treated at Kenyatta National Hospital, Nairobi. East Afr Med J. 1993 Sep. 70 (9):558-64. [Medline].
[Guideline] Dassinger M, Jackson R, Smith S. Management of colonic atresia with primary resection and anastomosis. Pediatr Surg Int. July 2009. 25(7):579-582. [Medline].
Millar AJW, Rode H, Cywes S. Intestinal atresia and stenosis: colonic atresia. Ashcraft KW, ed. Pediatric Surgery. 3rd ed. Philadelphia, Pa: WB Saunders Company; 2000.
Landes A, Shuckett B, Skarsgard E. Non-fixation of the colon in colonic atresia: a new finding. Pediatr Radiol. 1994. 24 (3):167-9. [Medline].
Nitta K, Iwafuchi M, Ohsawa Y, Uchiyama M, Yamagiwa I, Hirota M, et al. A case of congenital colonic atresia associated with atresia ani. J Pediatr Surg. 1987 Nov. 22 (11):1025-6. [Medline].
Asabe K, Nagasaki A. Double atresia of hindgut with ileal stenosis: a case report. Asian J Surg. 2004 Jan. 27(1):49-51. [Medline].
Digray NC, Mengi Y, Goswamy HL, Thappa DR. Colorectal perforations in neonates with anorectal malformations. Pediatr Surg Int. 2001. 17(1):42-4. [Medline].
Akgur FM, Tanyel FC, Buyukpamukcu N, Hicsonmez A. Colonic atresia and Hirschsprung's disease association shows further evidence for migration of enteric neurons. J Pediatr Surg. 1993 Apr. 28(4):635-6. [Medline].
Kim PC, Superina RA, Ein S. Colonic atresia combined with Hirschsprung's disease: a diagnostic and therapeutic challenge. J Pediatr Surg. 1995 Aug. 30 (8):1216-7. [Medline].
Siu KL, Kwok WK, Lee WY, Lee WH. A male newborn with colonic atresia and total colonic aganglionosis. Pediatr Surg Int. 1999. 15(2):141-2. [Medline].
Williams MD, Burrington JD. Hirschsprung's disease complicating colon atresia. J Pediatr Surg. 1993 Apr. 28(4):637-9. [Medline].
Sarsu SB, Karakus SC, Erdem AU. Colon perforation caused by a foreign body in Hirschsprung disease. J Pediatr Surg. 2012 Oct. 47(10):e15-7. [Medline].
Wang KS, Cahill JL, Skarsgard ED. Omphalocele, colonic atresia, and Hirschsprung's disease: an unusual cluster of malformations in a single patient. Pediatr Surg Int. 2001 Mar. 17 (2-3):218-20. [Medline].
Croaker GD, Harvey JG, Cass DT. Hirschsprung's disease, colonic atresia, and absent hand: a new triad. J Pediatr Surg. 1997 Sep. 32 (9):1368-70. [Medline].
Akgur FM, Olguner M, Hakguder G, Ozer E, Aktug T. Colonic atresia associated with Hirschsprung's disease: it is not a diagnostic challenge. Eur J Pediatr Surg. 1998 Dec. 8(6):378-9. [Medline].
Singh S, Das A, Chawla AS, Arya SV, Chaggar J. A rare presentation of midgut malrotation as an acute intestinal obstruction in an adult: Two case reports and literature review. Int J Surg Case Rep. 2012 Oct 12. 4(1):72-75. [Medline].
Applegate KE, Sargent SK. Spontaneous colonic ischemia in a patient with Riley-Day syndrome. Pediatr Radiol. 1995. 25(4):312-3. [Medline].
Lee SH, Cho YH, Kim HY, Park JH, Byun SY. Clinical experience of complex jejunal atresia. Pediatr Surg Int. 2012 Nov. 28(11):1079-83. [Medline].
Carroll AG, Kavanagh RG, Ni Leidhin C, Cullinan NM, Lavelle LP, Malone DE. Comparative Effectiveness of Imaging Modalities for the Diagnosis of Intestinal Obstruction in Neonates and Infants:: A Critically Appraised Topic. Acad Radiol. 2016 May. 23 (5):559-68. [Medline].
Cox SG, Numanoglu A, Millar AJ, Rode H. Colonic atresia: spectrum of presentation and pitfalls in management. A review of 14 cases. Pediatr Surg Int. 2005 Oct. 21(10):813-8. [Medline].
Stanescu AL, Liszewski MC, Lee EY, Phillips GS. Neonatal Gastrointestinal Emergencies: Step-by-Step Approach. Radiol Clin North Am. 2017 Jul. 55 (4):717-739. [Medline].
Fishman SJ, Islam S, Buonomo C, Nurko S. Nonfixation of an atretic colon predicts Hirschsprung's disease. J Pediatr Surg. 2001 Jan. 36(1):202-4. [Medline].
Hamzaoui M, Ghribi A, Makni W, Sghairoun N, Gasmi M. Rectal and sigmoid atresia: transanal approach. J Pediatr Surg. 2012 Jun. 47(6):e41-4. [Medline].

Media Gallery

Colonic atresia, type 1 (sigmoid colon). Dilated colon abruptly tapers to unused distal segment.
Contrast enema showing microcolon with dilated proximal colon (colonic atresia of sigmoid colon).
Contrast enema revealing colonic stenosis at hepatic flexure.
Abdominal radiograph of baby with colonic atresia at distal transverse colon. Colon proximal to atresia is visible as large dilated loop of intestine running obliquely across abdomen. Courtesy of Richard Glick, MD.
Intraoperative photograph displays proximal dilated segment in patient with type 3 atresia of distal transverse colon. Courtesy of Richard Glick, MD.
Small portion of bowel held on right looks like appendix but is actually distal segment for patient with type 3 atresia at distal transverse colon. Courtesy of Richard Glick, MD.

of 6

Author

Nelson G Rosen, MD, FACS, FAAP Assistant Professor of Surgery and Pediatrics, Albert Einstein College of Medicine; Attending Pediatric Surgeon and Director, Pediatric Trauma Center, Department of Pediatric General Surgery, Schneider Children's Hospital

Nelson G Rosen, MD, FACS, FAAP is a member of the following medical societies: American Academy of Pediatrics, American College of Surgeons, American Pediatric Surgical Association, American Trauma Society, Association of Military Surgeons of the US, Eastern Association for the Surgery of Trauma, Canadian Association of Pediatric Surgeons

Disclosure: Nothing to disclose.

Chief Editor

Eugene S Kim, MD, FACS, FAAP Professor of Surgery and Pediatrics, Division of Pediatric Surgery, Division of Hematology, Oncology, Blood and Marrow Transplantation, Keck School of Medicine, University of Southern California; Attending Pediatric Surgeon, Children's Hospital Los Angeles

Eugene S Kim, MD, FACS, FAAP is a member of the following medical societies: American Academy of Pediatrics, American Association for Cancer Research, American College of Surgeons, American Medical Association, American Pediatric Surgical Association, American Surgical Association, Association for Academic Surgery, Children's Oncology Group, Society of Laparoendoscopic Surgeons, Society of University Surgeons, Texas Medical Association

Disclosure: Nothing to disclose.

Acknowledgements

Jeremy T Aidlen, MD Fellow in Pediatric Surgery, North Shore-Long Island Jewish Health System, Schneider's Children's Hospital

Jeremy T Aidlen, MD is a member of the following medical societies: American College of Surgeons and International Pediatric Endosurgery Group