Pediatr Surg Int. [31] Mechanical obstruction can be diagnosed with digital rectal examination and barium enema. Han-Geurts IJ, Hendrix VC, de Blaauw I, Wijnen MH, van Heurn EL. J Laparoendosc Adv Surg Tech A. 2018 Aug. 53(8):1555-9. Images courtesy of Pratap A, Gupta DK, Tiwari A, et al. J Pediatr Surg. J Pediatr Surg. [Medline]. PubMed comprises more than 26 million citations for biomedical literature from MEDLINE, life science journals, and online books. Steven L Lee, MD Chief of Pediatric Surgery, Harbor-UCLA Medical Center; Associate Clinical Professor of Surgery and Pediatrics; University of California, Los Angeles, David Geffen School of Medicine Chen D, Mi J, Wu M, Wang W, Gao H. Expression of dishevelled gene in Hirschsprung's disease. [Full Text]. Semin Pediatr Surg. 10:30-2. [Medline]. The most common complications after pull-through are anastomotic stricture, long obstructing seromuscular cuff, long spur after a Duhamel procedure, twisted pull-through, or retained … 2013 Nov. 48(11):2281-8. [Medline]. Laparoscopic Swenson pull-through: a comparison with the open procedure. F: Contrast enema showing a CETZ at descending colon. [Medline]. Am J Gastroenterol. Potential complications for the complex operations associated with Hirschsprung disease encompass the entire spectrum of gastrointestinal surgical complications. 2017 Apr. [Medline]. Eur J Pediatr Surg. 48(3):642-51. 2017 Feb. 52(2):277-80. This website also contains material copyrighted by 3rd parties. [Medline]. Clinical features' diagnostics and treatment of Hirschsprung's disease in adults. Stem Cell Reports. Retinoic acid accelerates the specification of enteric neural progenitors from in-vitro-derived neural crest. It is the failure of the large intestine to populate with the … [Medline]. 8:184. A prospective study of botulinum toxin for internal anal sphincter hypertonicity in children with Hirschsprung's disease. Your feedback has been submitted successfully. 2013. However, up to 10 percent may have constipation, and less than 1 percent may … [Medline]. Steven L Lee, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American College of Surgeons, American Pediatric Surgical Association, Association for Academic Surgery, Society of Laparoendoscopic Surgeons, International Pediatric Endosurgery Group, Pacific Association of Pediatric Surgery, Society of American Gastrointestinal and Endoscopic SurgeonsDisclosure: Nothing to disclose. 1995 Jul. Hirschsprung disease enterocolitis can occur preoperatively (up to 50% incidence) or as a postoperative complication (up to 22% incidence).15,16Patients present with abdominal distension, fever, vomiting, and explosive diarrhea. [Medline]. Colonic motility after surgery for Hirschsprung's disease. Patients may present postoperatively with abdominal distention, emesis, or constipation indicative of ongoing obstruction. Hirschsprung disease is a relatively common condition managed by pediatric surgeons.Significant advances have been made in understanding its etiologies in the last decade, especially with the explosion of molecular genetic techniques and early diagnosis. All material on this website is protected by copyright, Copyright © 1994-2021 by WebMD LLC. [Medline]. Choose one of the access methods below or take a look at our subscribe or free trial options. 2000 Dec. 35(12):1733-6. 1990 Apr. J Pediatr Surg. The large intestine becomes blocked with stool. Hirschsprung disease always involves the RE c T um and is often associated with RET mutations. 1995. [Medline]. Long-segment disease is associated with an increased incidence of enterocolitis. Chen Y, Nah SA, Laksmi NK, et al. If you log out, you will be required to enter your username and password the next time you visit. Bowel perforation, septic shock, and death can result if enterocolitis goes untreated. [Medline]. 95(7):1759-64. 2003 May. [Medline]. Friedmacher F, Puri P. Residual aganglionosis after pull-through operation for Hirschsprung's disease: a systematic review and meta-analysis. 229(5):678-82; discussion 682-3. Vorobyov GI, Achkasov SI, Biryukov OM. [37, 38], Internal sphincter achalasia may result in persistent obstruction. Transl Res. J Pediatr Surg. 2011. Holschneider AM, Puri P, eds. Transitions in care from pediatric to adult general surgery: Evaluating an unmet need for patients with anorectal malformation and Hirschsprung disease. Most patients treated for Hirschsprung’s disease do not have complications. 2001 Jul. Long-term outcome of bowel function for 110 consecutive cases of Hirschsprung's disease: Comparison of the abdominal approach with transanal approach more than 30 years in a single institution - is the transanal approach truly beneficial for bowel function?. Hirschsprung disease in the U.S. associated Pacific Islands: more common than expected. 2000 Jun. Wu XJ, Zhang HY, Li N, et al. In: Hirschsprung Disease and Allied Disorders. [Medline]. Postoperative complications after pull-through for Hirschsprung’s disease can occur in a considerable number of patients. 2003 Oct. 238(4):569-83; discussion 583-5. 4. [39, 40], Functional megacolon may be present due to stool-holding behavior. Children with Hirschsprung’s disease are unable to defecate or pass stool, which can cause bowel obstruction. [Medline]. Histopathology. 2017 Mar. Hirschsprung disease. [Full Text]. One-stage Soave pull-through for Hirschsprung's disease: a comparison of the transanal and open approaches. De la Torre L, Ortega A. Transanal versus open endorectal pull-through for Hirschsprung's disease. 107-13. [Full Text]. Acquired aganglionosis following surgery for Hirschsprung's disease: a report of five cases during a 33-year experience with pull-through procedures. [Guideline] Gosain A, Frykman PK, Cowles RA, et al, for the American Pediatric Surgical Association Hirschsprung Disease Interest Group. Orphanet J Rare Dis. [Medline]. Ann Surg. 1999 Oct. 13(10):972-4. Cohen MC, Moore SW, Neveling U, Kaschula RO. 35(6):820-2. Pediatr Surg Int. Role in diagnosis and management. J Pediatr Surg. 2016 Dec. 40(12):1637-46. Transanal endorectal pull-through versus transabdominal approach for Hirschsprung's disease: a systematic review and meta-analysis. Hirschsprung’s disease affects one in … 64(4):96-8, 100-1. Hirschsprung-associated enterocolitis often presents with fever, explosive diarrhea, abdominal swelling, lethargy, and vomiting. 23(2):94-102. [Medline]. Frykman PK, Short SS. ERNICA guidelines for the management of rectosigmoid Hirschsprung's disease. 34(7):1152-60. Pathophysiology. [Medline]. Semin Pediatr Surg. [Medline]. This process may occur in either aganglionic or ganglionic segments of the bowel. The barium fills and coats the lining of the bowel, creating a clear silhouette of the colon and rectum. J Pediatr Gastroenterol Nutr. Muller CO, Rossignol G, Montalva L, et al. [Medline]. 1995 May. 1992 Jan. 27(1):76-81. Children with other congenital conditions, such as Down syndrome and heart defects, are more likely to have the disease. Am J Surg Pathol. 2003 Jun. Pediatr Dev Pathol. J Clin Invest. 1994 Jan. 83(1):68-71. PLoS One. Management of 30 cases. Hirschsprung disease is a birth defect in which nerve cells are missing at the end of a child’s bowel. Okamoto E, Takashi U. Embryogenesis of intramural ganglia of the gut and its relation to Hirschsprung's disease. Arch Intern Med (Chic). The most prominent symptom is constipation. 2001 Sep. 281(3):G602-11. 2016 Dec 1. Diagn Pathol. for: Medscape. Whitehouse FR, Kernohan JW. Totally transanal LESS pull-through colectomy: a novel approach for avoiding abdominal wall incision in children with long-segment intestinal aganglionosis. Most commonly diagnosed in the first year of life. 2009 Oct. 22(10):1379-84. Why do the patients with Hirschsprung disease get redo pull-through operation?. 2009 Jan-Feb. 12(1):6-15. [Full Text]. Stricture of the resected segment - a late complication. What are the complications of Hirschsprung disease in a child? Orphanet J Rare Dis. [Medline]. J Pediatr Surg. J Pediatr Surg. Gosemann JH, Friedmacher F, Ure B, Lacher M. Open versus transanal pull-through for Hirschsprung disease: a systematic review of long-term outcome. Dr. The bowel and abdomen swell as a result. Kubota M, Suita S, Kamimura T, Ito Y, Szurszewski JH. A subscription is required to access all the content in Best Practice. C: Plain abdominal radiograph showing a PARTZ at the descending colon. 8(6):466-79. Association of Program Directors in Surgery, International Pediatric Endosurgery Group, Society of American Gastrointestinal and Endoscopic Surgeons, American Association for the Study of Liver Diseases, American Society for Gastrointestinal Endoscopy. [Medline]. 82(1):75-111. 3rd ed. Levitt MA, Martin CA, Olesevich M, Bauer CL, Jackson LE, Pena A. Hirschsprung disease and fecal incontinence: diagnostic and management strategies. J Pediatr Surg. J Pediatr Surg. As the disease progresses, the intestinal lumen fills with a fibrinous exudate, and the risk of perforation increases. Guidelines for the management of postoperative soiling in children with Hirschsprung disease. [Medline]. Shayan K, Smith C, Langer JC. 2. [Medline]. 1999 Jul. 2000 Nov. 35(11):1630-2. Hirschsprung disease (HD) is a motor disorder of the colon caused by the failure of neural crest cells (precursors of enteric ganglion cells) to migrate completely during intestinal development. One-stage transanal Soave pullthrough for Hirschsprung disease: a multicenter experience with 141 children. Guidelines for the diagnosis and management of Hirschsprung-associated enterocolitis. Failure to pass out feces can lead to fecal impaction and obstruction of the colon. Smith GH, Cass D. Infantile Hirschsprung disease - is barium enema useful?. Kapur RP, Reed RC, Finn LS, Patterson K, Johanson J, Rutledge JC. [Medline]. Radiology. Hirschsprung disease (HSCR) is a disease of the large intestine or colon.People with this disease do not have the nerve cells in the intestine required to pass stools from the body normally. 2004 Dec. 41(12):942-88. Qin KW, Shi H, Zhang L, Liu PF, Cai WL, Wu KH. [Medline]. [Full Text]. [Medline]. J Pediatr Surg. Han JW, Youn JK, Oh C, Kim HY, Jung SE, Park KW. Complications may include enterocolitis, megacolon, bowel obstruction and intestinal perforation. Pediatr Surg Int. Rectal irrigations for the prevention of postoperative enterocolitis in Hirschsprung's disease. [Medline]. Li N, Zhang W, Yu D, et al. Keywords: Hirschsprung disease, children, diagnosis, management, complications and outcome Introduction Hirschsprung disease (HSCR) is defined as a functional intestinal obstruction that results from the congenital deficiency of the normal myenteric plexus parasympathetic ganglion cells in the distal portion of the large intestine. Dev Biol. [Medline]. Up to 40% of children with Hirschsprung disease develop enterocolitis. BMC Pediatr. Constipation: Hirschsprung's disease is one that occurs in utero. [Medline]. This condition is inflammation of the small and large intestines. Calretinin immunohistochemistry versus acetylcholinesterase histochemistry in the evaluation of suction rectal biopsies for Hirschsprung Disease. Rectovesical fistulas have also been reported in the literature. hirschsprung disease complications. Belin B, Corteville JE, Langer JC. [Medline]. 36(7):1032-5. Others experience severe symptoms that can be life-threatening. Leakage of the anastomosis. Pediatr Dev Pathol. 2009 Apr. New York, NY: Springer; 2008. Predicting sexual problems in young adults with an anorectal malformation or Hirschsprung disease. Complications can include: 1. [Medline]. 30(5):652-4. Barium or another contrast dye is placed into the bowel through a special tube inserted in the rectum. 15(8):553-8. B: Plain abdominal radiograph showing a PARTZ at the midsigmoid. Percutaneous cecostomy: updates in technique and patient care. 123(3):1182-91. Eur J Pediatr Surg. Birth Defects Res A Clin Mol Teratol. Functional outcomes in Hirschsprung disease: a single institution's 12-year experience. BS Anand, MD is a member of the following medical societies: American Association for the Study of Liver Diseases, American College of Gastroenterology, American Gastroenterological Association, American Society for Gastrointestinal EndoscopyDisclosure: Nothing to disclose. In Hirschsprung disease a part of the large intestine lacks normal nerve cells. The surgical treatment of toxic megacolon in Hirschsprung disease. Abdominal X-ray using a contrast dye. Outcome after anal intrasphincteric Botox injection in children with surgically treated Hirschsprung disease. After intestinal diversion, patients may also develop enterostomal complications, such as prolapse, herniation, or stricture. [Medline]. Vivek V Gumaste, MD is a member of the following medical societies: American College of Gastroenterology, American Gastroenterological AssociationDisclosure: Nothing to disclose. 2006 Dec. 22(12):987-90. Tran VQ, Lam KT, Truong DQ, et al. Acetylcholinesterase histochemistry (AChE)--a helpful technique in the diagnosis and in aiding the operative procedures of Hirschsprung disease. Minkes RK, Langer JC. PMID: 17257439, PMCID: PMC1790893. 6. [Medline]. Thapar N. New frontiers in the treatment of Hirschsprung disease. [29]. A: Plain abdominal radiograph showing a transition zone (PARTZ) at the rectosigmoid. de Lagausie P, Berrebi D, Geib G, Sebag G, Aigrain Y. Laparoscopic Duhamel procedure. J Pediatr Surg. 2013 Dec. 23(12):1020-3. 30(7):1023-6; discussion 1026-7. Tang CS, Gui H, Kapoor A, et al. J Pediatr Gastroenterol Nutr. 2013 May. Pediatr Surg Int. 2005 Apr. 1948 Jul. Myenteric plexus in congenital megacolon; study of 11 cases. 2016 Dec. 26(12):1032-5. In Hirschsprung disease, the nerves are missing from a part of the bowel. Diagnostic value of rectal suction biopsies using calretinin immunohistochemical staining in Hirschsprung's disease. The distribution of NCAM in the chick hindlimb during axon outgrowth and synaptogenesis. Expression and significance of neuroligins in myenteric cells of Cajal in Hirschsprung's disease. 33(4):455-60. Justin P Wagner, MD is a member of the following medical societies: American College of Surgeons, American Pediatric Surgical Association, Association for Academic Surgery, Association for Surgical Education, Association of Program Directors in SurgeryDisclosure: Nothing to disclose. The most frequent postoperative complications include enterocolitis after the Swenson procedure, constipation following the Duhamel repair, and diarrhea and incontinence after the Soave pull-through procedure. 227(1):246-50. Pediatr Surg Int. 2002 Jan. 131(1 suppl):S288-93. Di Lorenzo C, Solzi GF, Flores AF, Schwankovsky L, Hyman PE. Vivek V Gumaste, MD Associate Professor of Medicine, Mount Sinai School of Medicine of New York University; Adjunct Clinical Assistant, Mount Sinai Hospital; Director, Division of Gastroenterology, City Hospital Center at Elmhurst; Program Director of GI Fellowship (Independent Program); Regional Director of Gastroenterology, Queens Health Network [Medline]. Eur J Pediatr Surg. Am J Physiol Gastrointest Liver Physiol. 162(1):1-15. Interstitial cells of Cajal in human colon and in Hirschsprung's disease. Pini Prato A, Rossi V, Mosconi M, Holm C, Lantieri F, Griseri P. A prospective observational study of associated anomalies in Hirschsprung's disease. Pediatr Surg Int. 98(5):1052-7. Menezes M, Corbally M, Puri P. Long-term results of bowel function after treatment for Hirschsprung's disease: a 29-year review. [Medline]. Caniano DA, Teitelbaum DH, Qualman SJ. Hirschsprung-associated enterocolitis is the most frequent complication of HSCR occurring in 30-40% of individuals with HSCR and can be mild to severe in nature. Millar AJ, Steinberg RM, Raad J, Rode H. Anal achalasia after pull-through operations for Hirschsprung's disease -- preliminary experience with topical nitric oxide. [Medline]. Troy Reyna answered. Normally, the bowel contains many nerve cells all along its length that control how the bowel works. Am J Gastroenterol. J Laparoendosc Adv Surg Tech A. Smooth muscle from aganglionic bowel in Hirschsprung's disease impairs neuronal development in vitro. 1989 Dec. 24(12):1271-7. 2013 Apr. Int J Clin Exp Pathol. [Medline]. J Pediatr Surg. 1986 Apr. Our aim was to define predictive factors of the main postoperative complications: obstructive symptoms (OS) … Any time a person has a general anesthesia and surgery, however, there is a possibility of complications. The influence of Down's syndrome on the management and outcome of children with Hirschsprung's disease. 1996 Oct. 111(4):901-10. Agrawal RK, Kakkar N, Vasishta RK, Kumari V, Samujh R, Rao KL. [Medline]. For any urgent enquiries please contact our customer services team who are ready to help with any problems. Histology of the transition zone in Hirschsprung disease. Moore SW. Advances in understanding functional variations in the Hirschsprung disease spectrum (variant Hirschsprung disease). Int J Colorectal Dis. Persisting constipation (~ 10%)[14]. Other symptoms may include vomiting, abdominal pain, diarrhea and slow growth. Some children have mild inflammation. Justin P Wagner, MD Resident Physician, Department of Surgery, University of California, Los Angeles, David Geffen School of Medicine Mod Pathol. [28] Causes of redo included pathologic problems (aganglionosis, hypoganglionosis, immature ganglion cell) and anatomic problems (stricture, fistula and/or abscess at anastomosis), with those who had anatomic problems undergoing third redo procedures more than the group with pathologic problems. Meza-Valencia BE, de Lorimier AJ, Person DA. 2013 Mar. [Guideline] Saadai P, Trappey AF, Goldstein AM, et al, for the American Pediatric Surgical Association Hirschsprung Disease Interest Group. 1999. Hirschsprung disease (HSCR) is a special type of enteric neuropathy, which typically leads to intestinal obstruction in newborns, and has attracted wide attention from pediatric surgeons throughout the world. [32] If a rectal biopsy does not show ganglion cells, revision of the pull-through must be done. Hirschsprung disease (HD) is a congenital disorder defined by the absence of ganglion cells (GC) at the Meissner’s plexus of the submucosa and Auerbach’s plexus of the muscularis in the terminal rectum that extends in a variable distance proximally. Hirschsprung's disease (HD or HSCR) is a birth defect in which nerves are missing from parts of the intestine. 44 years experience Pediatric Surgery. 2020 Aug 11. [Medline]. Most cases resolve by age 5 years. [Medline]. In a retrospective (1979-2016) study of medical chart information from 657 Korean patients with Hirschsprung disease who underwent pull-through surgeries, investigators found 49 patients (7.5%) had redo pull-through procedures. Intestinal contents build up behind the blockage. Onishi S, Nakame K, Yamada K, et al. Acta Paediatr. J Pediatr Urol. 1994 Apr. 2019 Aug. 35(8):829-34. 2013 Mar. Am J Surg.
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