eMedicine Specialties > Pediatrics: Surgery > General Surgery

Atresia, Stenosis, and Other Obstruction of the Colon: Follow-up

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
Coauthor(s): Jeffrey Zitsman, MD, Director, Minimal Access Surgery, Associate Clinical Professor, Department of Surgery, Division of Pediatric Surgery, Children's Hospital of New York, New York Presbyterian Medical Center; Jeremy T Aidlen, MD, Fellow in Pediatric Surgery, North Shore-Long Island Jewish Health System, Schneider's Children's Hospital
Contributor Information and Disclosures

Updated: Apr 28, 2008

Outcome and Prognosis

Survival of patients with colonic atresia and stenosis is related to the patient's condition before surgery, technical difficulties with colonic anastomosis, sepsis, and associated anomalies.44,11

Whereas older series reported high mortality rates for colonic atresia, modern series report survival of all patients, except those with significant life-threatening comorbidities.

Patients with Hirschsprung disease and colonic atresia have more complicated courses and a mortality rate of 10%.29  

Colonic stenosis outcomes have also improved significantly since Gross reported the death of the single patient treated at the Boston Children's Hospital before 1952.10 Improvement in resuscitation and perioperative care presently results in survival rates of approximately 90%.45,1

Outcomes of Surgery in Colonic Atresia and Stenosis

Open table in new window

[ CLOSE WINDOW ]
Table
Author (Year)Number of PatientsProcedureSurvival Rate
Gross (1952) 10 6Ostomy*33%
Sturim (1966) 46 2Ostomy50%
Coran (1969) 47 9Ostomy66%
Pohlson (1988) 21 11Ostomy (6), anastomosis (4)†, resection diaphragm (1)‡ 73%
Smith (1989) 48 2Not specified100%
Davenport (1990) 5 11Ostomy (6); anastomosis (4)91%
Barrack (1993) 49 2Anastomosis100%
Dalla Vecchia (1998) 11 21Ostomy (18); anastomosis (3)100%
Abu-Judeh (2001) 45 1Anastomosis100%
Author (Year)Number of PatientsProcedureSurvival Rate
Gross (1952) 10 6Ostomy*33%
Sturim (1966) 46 2Ostomy50%
Coran (1969) 47 9Ostomy66%
Pohlson (1988) 21 11Ostomy (6), anastomosis (4)†, resection diaphragm (1)‡ 73%
Smith (1989) 48 2Not specified100%
Davenport (1990) 5 11Ostomy (6); anastomosis (4)91%
Barrack (1993) 49 2Anastomosis100%
Dalla Vecchia (1998) 11 21Ostomy (18); anastomosis (3)100%
Abu-Judeh (2001) 45 1Anastomosis100%

*Ostomy, resection and staged anastomosis months later
† Resection with primary anastomosis
‡ Cecotomy, resection of diaphragm

Future and Controversies

Summary

Colonic atresia and congenital stenosis are uncommon lesions. Infants present with abdominal distension and vomiting and often fail to pass meconium. Associated anomalies are common, and their severity directly affects outcome. The combination of Hirschsprung disease and colonic atresia remains rare; however, missing the association prior to reconnecting the intestinal tract can lead to reoperations, poor outcome, and increased mortality. 

Surgical correction is the mainstay of therapy for atresia and stenosis. In the absence of significant comorbidity, primary resection and anastomosis is the recommended surgical treatment and appears to have an equivalent outcome to resection with creation of a colostomy and subsequent closure.

Controversies
The question of the necessity for routine rectal biopsy prior to establishing intestinal continuity is not so much controversial as it is difficult to definitively answer. The small number of cases that associate Hirschsprung disease with colonic atresia is such that the association is greater than random but still exceedingly rare. The downside to taking a staged approach if Hirschsprung disease is a possibility is minimal when compared with the risk of multiple operations and increased mortality when the association is missed prior to anastomosis. Knowing both sides of this issue allows the surgeon to choose the approach that best serves their patient.

 
Acknowledgments

The authors would like to acknowledge Richard Glick, MD, for the images of his patient with distal transverse colonic atresia.



More on Atresia, Stenosis, and Other Obstruction of the Colon

Overview: Atresia, Stenosis, and Other Obstruction of the Colon
Workup: Atresia, Stenosis, and Other Obstruction of the Colon
Treatment: Atresia, Stenosis, and Other Obstruction of the Colon
Follow-up: Atresia, Stenosis, and Other Obstruction of the Colon
Multimedia: Atresia, Stenosis, and Other Obstruction of the Colon
References
[ CLOSE WINDOW ]

References

  1. Oldham KT. Atresia, stenosis and other obstructions of the colon. In: O' Neill JA, Jr, Rowe MI, Grosfeld JL, Fonkalsrud EW, Coran AG, eds. Pediatric Surgery. St. Louis, Mo: Mosby; 1998:1361-68.

  2. Gaub OC. Congenital stenosis and atresia of the intestinal tract above the rectum; with a report of an operated case of atresia of the sigmoid in an infant. Tr Am S A. 1922;40:582.

  3. Potts WJ. Congenital atresia of the intestine and colon. Surg Gynecol Obstet. 1947;85:14.

  4. Evans CH. Collective review: atresias of the gastrointestinal tract. Surg Gynecol Obstet. 1955;92:1-8.

  5. Davenport M, Bianchi A, Doig CM, Gough DC. Colonic atresia: current results of treatment. J R Coll Surg Edinb. Feb 1990;35(1):25-8. [Medline].

  6. Webb CH, Wangensteen OH. Am J Dis Child. 1931;4:232.

  7. Benson CD, Lotfi MW, Brogh AJ. Congenital atresia and stenosis of the colon. J Pediatr Surg. Apr 1968;3(2):253-7. [Medline].

  8. Powell RW, Raffensperger JG. Congenital colonic atresia. J Pediatr Surg. Apr 1982;17(2):166-70. [Medline].

  9. Freeman NV. Congenital atresia and stenosis of the colon. Br J Surg. 1966;53:595-7.

  10. Gross RE. The surgery of infancy and childhood. Philadelphia, Pa: WB Saunders & Co; 1953:150-74.

  11. Dalla Vecchia LK, Grosfeld JL, West KW, et al. Intestinal atresia and stenosis: a 25-year experience with 277 cases. Arch Surg. May 1998;133(5):490-6; discussion 496-7. [Medline].

  12. Kim S, Yedlin S, Idowu O. Colonic atresia in monozygotic twins. Am J Med Genet. Mar 20 2000;91(3):204-6. [Medline].

  13. Janik JS, Ein SH, Mancer K. Intestinal stricture after necrotizing enterocolitis. J Pediatr Surg. Aug 1981;16(4):438-43. [Medline].

  14. Kosloske AM, Burstein J, Bartow SA. Intestinal obstruction due to colonic stricture following neonatal necrotizing enterocolitis. Ann Surg. Aug 1980;192(2):202-7. [Medline].

  15. Virjee JP, Gill GJ, Desa D, et al. Strictures and other late complications of neonatal necrotising enterocolitis. Clin Radiol. Jan 1979;30(1):25-31. [Medline].

  16. Millar AJW, Rode H, Cywes S. Intestinal atresia and stenosis: colonic atresia. In: Ashcraft KW, ed. Pediatric Surgery. 3rd ed. Philadelphia, Pa: WB Saunders Company; 2000.

  17. 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].

  18. Nitta K, Iwafuchi M, Ohsawa Y, et al. A case of congenital colonic atresia associated with atresia ani. J Pediatr Surg. Nov 1987;22(11):1025-6. [Medline].

  19. Asabe K, Nagasaki A. Double atresia of hindgut with ileal stenosis: a case report. Asian J Surg. Jan 2004;27(1):49-51. [Medline].

  20. Digray NC, Mengi Y, Goswamy HL, Thappa DR. Colorectal perforations in neonates with anorectal malformations. Pediatr Surg Int. 2001;17(1):42-4. [Medline].

  21. Pohlson EC, Hatch EI Jr, Glick PL, Tapper D. Individualized management of colonic atresia. Am J Surg. May 1988;155(5):690-2. [Medline].

  22. 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. Apr 1993;28(4):635-6. [Medline].

  23. Kim PC, Superina RA, Ein S. Colonic atresia combined with Hirschsprung's disease: a diagnostic and therapeutic challenge. J Pediatr Surg. Aug 1995;30(8):1216-7. [Medline].

  24. 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].

  25. Williams MD, Burrington JD. Hirschsprung's disease complicating colon atresia. J Pediatr Surg. Apr 1993;28(4):637-9. [Medline].

  26. 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. Mar 2001;17(2-3):218-20. [Medline].

  27. Croaker GD, Harvey JG, Cass DT. Hirschsprung's disease, colonic atresia, and absent hand: a new triad. J Pediatr Surg. Sep 1997;32(9):1368-70. [Medline].

  28. 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. Dec 1998;8(6):378-9. [Medline].

  29. 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. Feb 2007;42(2):e5-8. [Medline].

  30. Applegate KE, Sargent SK. Spontaneous colonic ischemia in a patient with Riley-Day syndrome. Pediatr Radiol. 1995;25(4):312-3. [Medline].

  31. Patten BM. Human Embryology. New York, NY: McGraw-Hill; 1953.

  32. Louw JH, Barnard CN. Congenital intestinal atresia. Lancet. 1955;ii:1065-7.

  33. Fairbanks TJ, Kanard RC, Del Moral PM, et al. Colonic atresia without mesenteric vascular occlusion. The role of the fibroblast growth factor 10 signaling pathway. J Pediatr Surg. Feb 2005;40(2):390-6. [Medline].

  34. Hollinshead WH. Anatomy for Surgeons. Vol 2. New York, NY: Hoeber-Harper; 1956.

  35. Gornall P. Management of intestinal atresia complicating gastroschisis. J Pediatr Surg. Jun 1989;24(6):522-4. [Medline].

  36. Wang NL, Yeh ML, Chang PY, et al. Prenatal and neonatal intussusception. Pediatr Surg Int. Apr 1998;13(4):232-6. [Medline].

  37. Al-Wafi A, Morris-Stiff G, Lari A. Colonic atresia secondary to a choledochal cyst. Pediatr Surg Int. Jul 1998;13(5-6):422-3. [Medline].

  38. Louw JH. Investigations into the etiology of congenital atresia of the colon. Dis Colon Rectum. 1964;7:471-478.

  39. Werler MM, Sheehan JE, Mitchell AA. Association of vasoconstrictive exposures with risks of gastroschisis and small intestinal atresia. Epidemiology. May 2003;14(3):349-54. [Medline].

  40. de Jesus LE, Marques AM, Rocha MS, et al. Left colon stenosis caused by tuberculosis. J Pediatr Surg. Oct 2004;39(10):e5-7. [Medline].

  41. Bland-Sutton JD. Imperforate ileum. Am J Med Sci. 1889;98:457-62.

  42. 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. Oct 2005;21(10):813-8. [Medline].

  43. Fishman SJ, Islam S, Buonomo C, Nurko S. Nonfixation of an atretic colon predicts Hirschsprung's disease. J Pediatr Surg. Jan 2001;36(1):202-4. [Medline].

  44. 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].

  45. Abu-Judeh HH, Methratta S, Ybasco A, et al. Congenital colonic stenosis. South Med J. Mar 2001;94(3):344-6. [Medline].

  46. Sturim HS, Ternberg JL. Congenital atresia of the colon. Surgery. Mar 1966;59(3):458-64. [Medline].

  47. Coran AG, Eraklis AJ. Atresia of the colon. Surgery. May 1969;65(5):828-31. [Medline].

  48. Smith GH, Glasson M. Intestinal atresia: factors affecting survival. Aust N Z J Surg. Feb 1989;59(2):151-6. [Medline].

  49. Barrack SM, Kyambi JM, Ndungu J, et al. Intestinal atresia and stenosis as seen and treated at Kenyatta National Hospital, Nairobi. East Afr Med J. Sep 1993;70(9):558-64. [Medline].

[ CLOSE WINDOW ]

Further Reading

[ CLOSE WINDOW ]

Keywords

colonic atresia, colonic stenosis, colonic stricture, colonic obstruction, congenital colon obstruction, colonic narrowing, abdominal distention, failure to pass meconium, narrowed colon, Hirschsprung disease, small left colon syndrome, obturation obstruction, meconium ileus, meconium plug, abdominal distention, failure to pass meconium, congenital stenosis, acquired stenosis, stricture, necrotizing enterocolitis, intestinal blockage, bowel obstruction, feeding intolerance
 
failure to thrive, colon atresia, small intestinal atresia, dilated bowel loops, polyhydramnios, abdominal wall defects, genitourinary tract abnormalities, anal atresia, imperforate anus, colonic perforation, omphalocele, aganglionosis, mesenteric vascular incident, cryptophthalmia syndrome, cleft lip and palate, dysplastic kidneys, proximal jejunal atresia, arthrogryposis, proximal intestinal atresia, neoplasm, malrotation, Riley-Day syndrome, spontaneous colon ischemia, coloboma, cataracts, facial hemihypertrophy

facial asymmetry, exophthalmia, bilateral optic nerve hypoplasia, vascular interruption, thrombosis, volvulus, herniation, bowel necrosis, duodenal atresia, bowel ischemic injury, internal hernia, choledochal cyst, volvulus, bowel infarction, Crohn disease, tuberculosis

[ CLOSE WINDOW ]

Contributor Information and Disclosures

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, Canadian Association of Pediatric Surgeons, and Eastern Association for the Surgery of Trauma
Disclosure: Nothing to disclose.

Coauthor(s)

Jeffrey Zitsman, MD, Director, Minimal Access Surgery, Associate Clinical Professor, Department of Surgery, Division of Pediatric Surgery, Children's Hospital of New York, New York Presbyterian Medical Center
Jeffrey Zitsman, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Surgeons, American Pediatric Surgical Association, and International Pediatric Endosurgery Group
Disclosure: Nothing to disclose.

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
Disclosure: Nothing to disclose.

Medical Editor

Kurt D Newman, MD, Vice Chairman, Department of Pediatric Surgery, Children's National Medical Center; Professor, Departments of Surgery and Pediatrics, George Washington University School of Medicine
Kurt D Newman, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Surgeons, American Pediatric Surgical Association, and Society of Surgical Oncology
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine.com, Inc
Disclosure: Pfizer Inc Stock Investment from broker recommendation; Avanir Pharma Stock Investment from broker recommendation

Managing Editor

Deborah F Billmire, MD, Associate Professor, Department of Surgery, Indiana University Medical Center
Deborah F Billmire, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American College of Surgeons, American Pediatric Surgical Association, Phi Beta Kappa, and Society of Critical Care Medicine
Disclosure: Nothing to disclose.

CME Editor

H Biemann Othersen Jr, MD, Professor of Surgery and Pediatrics, Emeritus Head, Division of Pediatric Surgery, Medical University of South Carolina
H Biemann Othersen Jr, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Association for the Surgery of Trauma, American Burn Association, American Cancer Society, American College of Surgeons, American Medical Association, American Pediatric Surgical Association, American Society for Parenteral and Enteral Nutrition, American Surgical Association, American Thoracic Society, British Association of Paediatric Surgeons, Society for Surgery of the Alimentary Tract, Society of Critical Care Medicine, South Carolina Medical Association, Southeastern Surgical Congress, Southern Medical Association, Southern Society for Pediatric Research, and Southern Thoracic Surgical Association
Disclosure: Nothing to disclose.

Chief Editor

Marleta Reynolds, MD, Professor of Surgery, Feinberg School of Medicine, Northwestern University; Interim Head, Division of Pediatric Surgery, Department of Surgery, Children's Memorial Hospital of Chicago
Marleta Reynolds, MD is a member of the following medical societies: American Pediatric Surgical Association
Disclosure: Nothing to disclose.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.