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Clin Colon Rectal Surg 2018; 31(02): 051-060
DOI: 10.1055/s-0037-1604034
Review Article
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Hirschsprung Disease beyond Infancy

Casey M. Calkins
1   Division of Pediatric General and Thoracic Surgery, Department of Surgery, Medical College of Wisconsin, Milwaukee, Wisconsin
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Publication Date:
25 February 2018 (online)

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Abstract

Hirschsprung disease (HD) is a common cause of neonatal intestinal obstruction in which a variable segment of the distal intestinal tract lacks the normal enteric nervous system elements. Affected individuals present with varying degrees of obstructive symptoms, but today most patients are diagnosed within the first several months of life owing to the well-recognized symptoms and the ease of making the diagnosis by way of the bedside suction rectal biopsy. Thus, for the adult general or colorectal surgeon, the vast majority of patients who present for evaluation will have already undergone surgical treatment within the first year of life by a pediatric surgeon. Despite several safe operative interventions to treat patients with HD, the long-term results are far from perfect. These patients may reach adult life with ongoing defecation disorders that require a systematic evaluation by a multidisciplinary group that should be led by a surgeon with a thorough knowledge of HD operations and the potential problems. The evaluation of these patients will form the basis for the majority of this review—however, some patients manage to escape diagnosis beyond the infant and childhood period—and a section herein will briefly address the case of an older patient who is suspected of having HD.

Keywords

Hirschsprung - complications - reoperation - incontinence - constipation
 

  • References

  • 1 Sergi C. Hirschsprung's disease: historical notes and pathological diagnosis on the occasion of the 100(th) anniversary of Dr. Harald Hirschsprung's death. World J Clin Pediatr 2015; 4 (04) 120-125
    CrossrefPubMedSearch in Google Scholar
  • 2 Swenson O, Rheinlander HF, Diamond I. Hirschsprung's disease; a new concept of the etiology; operative results in 34 patients. N Engl J Med 1949; 241 (15) 551-556
    CrossrefPubMedSearch in Google Scholar
  • 3 De La Torre L, Langer JC. Transanal endorectal pull-through for Hirschsprung disease: technique, controversies, pearls, pitfalls, and an organized approach to the management of postoperative obstructive symptoms. Semin Pediatr Surg 2010; 19 (02) 96-106
    CrossrefPubMedSearch in Google Scholar
  • 4 Levitt MA, Dickie B, Peña A. The Hirschsprungs patient who is soiling after what was considered a “successful” pull-through. Semin Pediatr Surg 2012; 21 (04) 344-353
    CrossrefPubMedSearch in Google Scholar
  • 5 Granström AL, Husberg B, Nordenskjöld A, Svensson PJ, Wester T. Laparoscopic-assisted pull-through for Hirschsprung's disease, a prospective repeated evaluation of functional outcome. J Pediatr Surg 2013; 48 (12) 2536-2539
    CrossrefPubMedSearch in Google Scholar
  • 6 Jarvi K, Laitakari EM, Koivusalo A, Rintala RJ, Pakarinen MP. Bowel function and gastrointestinal quality of life among adults operated for Hirschsprung disease during childhood: a population-based study. Ann Surg 2010; 252 (06) 977-981
    CrossrefPubMedSearch in Google Scholar
  • 7 Heikkinen M, Rintala R, Luukkonen P. Long-term anal sphincter performance after surgery for Hirschsprung's disease. J Pediatr Surg 1997; 32 (10) 1443-1446
    CrossrefPubMedSearch in Google Scholar
  • 8 Diseth TH, Bjørnland K, Nøvik TS, Emblem R. Bowel function, mental health, and psychosocial function in adolescents with Hirschsprung's disease. Arch Dis Child 1997; 76 (02) 100-106
    CrossrefPubMedSearch in Google Scholar
  • 9 Baillie CT, Kenny SE, Rintala RJ, Booth JM, Lloyd DA. Long-term outcome and colonic motility after the Duhamel procedure for Hirschsprung's disease. J Pediatr Surg 1999; 34 (02) 325-329
    CrossrefPubMedSearch in Google Scholar
  • 10 Rintala RJ, Pakarinen MP. Long-term outcomes of Hirschsprung's disease. Semin Pediatr Surg 2012; 21 (04) 336-343
    CrossrefPubMedSearch in Google Scholar
  • 11 Aworanti OM, McDowell DT, Martin IM, Quinn F. Does functional outcome improve with time postsurgery for Hirschsprung disease?. Eur J Pediatr Surg 2016; 26 (02) 192-199
    Thieme ConnectPubMedSearch in Google Scholar
  • 12 Reding R, de Ville de Goyet J, Gosseye S. , et al. Hirschsprung's disease: a 20-year experience. J Pediatr Surg 1997; 32 (08) 1221-1225
    CrossrefPubMedSearch in Google Scholar
  • 13 Bai Y, Chen H, Hao J, Huang Y, Wang W. Long-term outcome and quality of life after the Swenson procedure for Hirschsprung's disease. J Pediatr Surg 2002; 37 (04) 639-642
    CrossrefPubMedSearch in Google Scholar
  • 14 Yanchar NL, Soucy P. Long-term outcome after Hirschsprung's disease: patients' perspectives. J Pediatr Surg 1999; 34 (07) 1152-1160
    CrossrefPubMedSearch in Google Scholar
  • 15 Catto-Smith AG, Trajanovska M, Taylor RG. Long-term continence in patients with Hirschsprung's disease and Down syndrome. J Gastroenterol Hepatol 2006; 21 (04) 748-753
    CrossrefPubMedSearch in Google Scholar
  • 16 Keckler SJ, Yang JC, Fraser JD. , et al. Contemporary practice patterns in the surgical management of Hirschsprung's disease. J Pediatr Surg 2009; 44 (06) 1257-1260 , discussion 1260
    CrossrefPubMedSearch in Google Scholar
  • 17 Soave F. Hirschsprung's disease: a new surgical technique. Arch Dis Child 1964; 39: 116-124
    CrossrefPubMedSearch in Google Scholar
  • 18 Boley SJ. New modification of the surgical treatment of Hirschsprung's disease. Surgery 1964; 56: 1015-1017
    PubMedSearch in Google Scholar
  • 19 Yokoi A, Satoh S, Takamizawa S, Muraji T, Tsugawa C, Nishijima E. The preliminary study of modified Swenson procedure in Hirschsprung disease. J Pediatr Surg 2009; 44 (08) 1560-1563
    CrossrefPubMedSearch in Google Scholar
  • 20 Duhamel B. A new operation for the treatment of Hirschsprung's disease. Arch Dis Child 1960; 35: 38-39
    CrossrefPubMedSearch in Google Scholar
  • 21 Canty TG. Modified Duhamel procedure for treatment of Hirschsprung's disease in infancy and childhood: review of 41 consecutive cases. J Pediatr Surg 1982; 17 (06) 773-778
    CrossrefPubMedSearch in Google Scholar
  • 22 Suita S, Taguchi T, Yanai K, Kamimura T, Nakao M, Ikeda K. Longterm outcomes and quality of life after Z-shaped anastomosis for Hirschsprung's disease. J Am Coll Surg 1998; 187 (06) 577-583
    CrossrefPubMedSearch in Google Scholar
  • 23 Martin LW. Total colonic aganglionosis preservation and utilization of entire colon. J Pediatr Surg 1982; 17 (05) 635-637
    CrossrefPubMedSearch in Google Scholar
  • 24 Steichen FM, Talbert JL, Ravitch MM. Primary side-to-side colorectal anastomosis in the Duhamel operation for Hirschsprung's disease. Surgery 1968; 64 (02) 475-483
    PubMedSearch in Google Scholar
  • 25 Georgeson KE, Robertson DJ. Laparoscopic-assisted approaches for the definitive surgery for Hirschsprung's disease. Semin Pediatr Surg 2004; 13 (04) 256-262
    CrossrefPubMedSearch in Google Scholar
  • 26 de Lagausie P, Bruneau B, Besnard M, Jaby O, Aigrain Y. Definitive treatment of Hirschsprung's disease with a laparoscopic Duhamel pull-through procedure in childhood. Surg Laparosc Endosc 1998; 8 (01) 55-57
    CrossrefPubMedSearch in Google Scholar
  • 27 Curran TJ, Raffensperger JG. The feasibility of laparoscopic swenson pull-through. J Pediatr Surg 1994; 29 (09) 1273-1275
    CrossrefPubMedSearch in Google Scholar
  • 28 Langer JC, Minkes RK, Mazziotti MV, Skinner MA, Winthrop AL. Transanal one-stage Soave procedure for infants with Hirschsprung's disease. J Pediatr Surg 1999; 34 (01) 148-151 , discussion 152
    CrossrefPubMedSearch in Google Scholar
  • 29 Rassouli R, Holschneider AM, Bolkenius M. , et al. Long-term results of Rehbein's procedure: a retrospective study in German-speaking countries. Eur J Pediatr Surg 2003; 13 (03) 187-194
    Thieme ConnectPubMedSearch in Google Scholar
  • 30 State D. Physiological operation for idiopathic congenital megacolon (Hirschsprung's disease). J Am Med Assoc 1952; 149 (04) 350-355
    CrossrefPubMedSearch in Google Scholar
  • 31 Aworanti OM, Mcdowell DT, Martin IM, Hung J, Quinn F. Comparative review of functional outcomes post surgery for Hirschsprung's disease utilizing the paediatric incontinence and constipation scoring system. Pediatr Surg Int 2012; 28 (11) 1071-1078
    CrossrefPubMedSearch in Google Scholar
  • 32 Bradnock TJ, Walker GM. Evolution in the management of Hirschsprung's disease in the UK and Ireland: a national survey of practice revisited. Ann R Coll Surg Engl 2011; 93 (01) 34-38
    PubMedSearch in Google Scholar
  • 33 Dodero P, Magillo P, Scarsi PL. Total colectomy and straight ileo-anal soave endorectal pull-through: personal experience with 42 cases. Eur J Pediatr Surg 2001; 11 (05) 319-323
    Thieme ConnectPubMedSearch in Google Scholar
  • 34 Cheung ST, Tam YH, Chong HM. , et al. An 18-year experience in total colonic aganglionosis: from staged operations to primary laparoscopic endorectal pull-through. J Pediatr Surg 2009; 44 (12) 2352-2354
    CrossrefPubMedSearch in Google Scholar
  • 35 Mir E, Karaca I, Günsar C, Sencan A, Fesçekoglu O. Primary Duhamel-Martin operations in neonates and infants. Pediatr Int 2001; 43 (04) 405-408
    CrossrefPubMedSearch in Google Scholar
  • 36 van der Zee DC, Bax NM. Duhamel-Martin procedure for Hirschsprung's disease in neonates and infants: one-stage operation. J Pediatr Surg 1996; 31 (07) 901-902
    CrossrefPubMedSearch in Google Scholar
  • 37 Bischoff A, Levitt MA, Peña A. Total colonic aganglionosis: a surgical challenge. How to avoid complications?. Pediatr Surg Int 2011; 27 (10) 1047-1052
    CrossrefPubMedSearch in Google Scholar
  • 38 Heath AL, Spitz L, Milla PJ. The absorptive function of colonic aganglionic intestine: are the Duhamel and Martin procedures rational?. J Pediatr Surg 1985; 20 (01) 34-36
    CrossrefPubMedSearch in Google Scholar
  • 39 Nishijima E, Kimura K, Tsugawa C, Muraji T. The colon patch graft procedure for extensive aganglionosis: long-term follow-up. J Pediatr Surg 1998; 33 (02) 215-219
    CrossrefPubMedSearch in Google Scholar
  • 40 Sauer H, Fasching G. Preservation of the ileocecal valve and right colon in total colonic aganglionosis. J Pediatr Surg 1993; 28 (12) 1640-1643
    CrossrefPubMedSearch in Google Scholar
  • 41 Shandling B. Total colon aganglionosis--a new operation. J Pediatr Surg 1984; 19 (05) 503-505
    CrossrefPubMedSearch in Google Scholar
  • 42 Hukkinen M, Koivusalo A, Rintala RJ, Pakarinen MP. Restorative proctocolectomy with J-pouch ileoanal anastomosis for total colonic aganglionosis among neonates and infants. J Pediatr Surg 2014; 49 (04) 570-574
    CrossrefPubMedSearch in Google Scholar
  • 43 Ein SH, Shandling B, So H. A new look at an old operation for aganglionosis. J Pediatr Surg 1994; 29 (09) 1228-1230
    CrossrefPubMedSearch in Google Scholar
  • 44 Escobar MA, Grosfeld JL, West KW. , et al. Long-term outcomes in total colonic aganglionosis: a 32-year experience. J Pediatr Surg 2005; 40 (06) 955-961
    CrossrefPubMedSearch in Google Scholar
  • 45 Wildhaber BE, Teitelbaum DH, Coran AG. Total colonic Hirschsprung's disease: a 28-year experience. J Pediatr Surg 2005; 40 (01) 203-206 , discussion 206–207
    CrossrefPubMedSearch in Google Scholar
  • 46 Barrena S, Andres AM, Burgos L. , et al. Long-term results of the treatment of total colonic aganglionosis with two different techniques. Eur J Pediatr Surg 2008; 18 (06) 375-379
    Thieme ConnectPubMedSearch in Google Scholar
  • 47 Blackburn S, Corbett P, Griffiths DM, Burge D, Beattie RM, Stanton M. Total colonic aganglionosis: a 15-year single center experience. Eur J Pediatr Surg 2014; 24 (06) 488-491
    Thieme ConnectPubMedSearch in Google Scholar
  • 48 Shen C, Song Z, Zheng S, Xiao X. A comparison of the effectiveness of the Soave and Martin procedures for the treatment of total colonic aganglionosis. J Pediatr Surg 2009; 44 (12) 2355-2358
    CrossrefPubMedSearch in Google Scholar
  • 49 Marquez TT, Acton RD, Hess DJ, Duval S, Saltzman DA. Comprehensive review of procedures for total colonic aganglionosis. J Pediatr Surg 2009; 44 (01) 257-265 , discussion 265
    CrossrefPubMedSearch in Google Scholar
  • 50 Neilson IR, Yazbeck S. Ultrashort Hirschsprung's disease: myth or reality. J Pediatr Surg 1990; 25 (11) 1135-1138
    PubMedSearch in Google Scholar
  • 51 Foroutan HR, Hosseini SM, Banani SA. , et al. Comparison of botulinium toxin injection and posterior anorectal myectomy in treatment of internal anal sphincter achalasia. Indian J Gastroenterol 2008; 27 (02) 62-65
    PubMedSearch in Google Scholar
  • 52 Friedmacher F, Puri P. Comparison of posterior internal anal sphincter myectomy and intrasphincteric botulinum toxin injection for treatment of internal anal sphincter achalasia: a meta-analysis. Pediatr Surg Int 2012; 28 (08) 765-771
    CrossrefPubMedSearch in Google Scholar
  • 53 Peña A, Elicevik M, Levitt MA. Reoperations in Hirschsprung disease. J Pediatr Surg 2007; 42 (06) 1008-1013 , discussion 1013–1014
    CrossrefPubMedSearch in Google Scholar
  • 54 Harrison MW, Deitz DM, Campbell JR, Campbell TJ. Diagnosis and management of Hirschsprung's disease. A 25.  year perspective. Am J Surg 1986; 152 (01) 49-56
    CrossrefPubMedSearch in Google Scholar
  • 55 Langer JC, Birnbaum E. Preliminary experience with intrasphincteric botulinum toxin for persistent constipation after pull-through for Hirschsprung's disease. J Pediatr Surg 1997; 32 (07) 1059-1061 , discussion 1061–1062
    CrossrefPubMedSearch in Google Scholar
  • 56 Han-Geurts IJ, Hendrix VC, de Blaauw I, Wijnen MH, van Heurn EL. Outcome after anal intrasphincteric Botox injection in children with surgically treated Hirschsprung disease. J Pediatr Gastroenterol Nutr 2014; 59 (05) 604-607
    CrossrefPubMedSearch in Google Scholar
  • 57 Wester T, Granström AL. Botulinum toxin is efficient to treat obstructive symptoms in children with Hirschsprung disease. Pediatr Surg Int 2015; 31 (03) 255-259
    CrossrefPubMedSearch in Google Scholar
  • 58 Peeraully MR, Lopes J, Wright A. , et al. Experience of the MACE procedure at a regional pediatric surgical unit: a 15-year retrospective review. Eur J Pediatr Surg 2014; 24 (01) 113-116
    Thieme ConnectPubMedSearch in Google Scholar
  • 59 DeFreest L, Smith J, Whyte C. Laparoscopic-assisted percutaneous cecostomy for antegrade continence enema. J Laparoendosc Adv Surg Tech A 2014; 24 (04) 261-264
    CrossrefPubMedSearch in Google Scholar
  • 60 Tsuji H, Spitz L, Kiely EM, Drake DP, Pierro A. Management and long-term follow-up of infants with total colonic aganglionosis. J Pediatr Surg 1999; 34 (01) 158-161 , discussion 162
    CrossrefPubMedSearch in Google Scholar
  • 61 Anupama B, Zheng S, Xiao X. Ten-year experience in the management of total colonic aganglionosis. J Pediatr Surg 2007; 42 (10) 1671-1676
    CrossrefPubMedSearch in Google Scholar
  • 62 Zaslavsky C, Loening-Baucke V. Anorectal manometric evaluation of children and adolescents postsurgery for Hirschsprung's disease. J Pediatr Surg 2003; 38 (02) 191-195
    CrossrefPubMedSearch in Google Scholar
  • 63 Kuwahara M, Iwai N, Yanagihara J, Tokiwa K, Fukata R. Endosonographic study of anal sphincters in patients after surgery for Hirschsprung's disease. J Pediatr Surg 1999; 34 (03) 450-453
    CrossrefPubMedSearch in Google Scholar
  • 64 Levitt MA, Martin CA, Olesevich M, Bauer CL, Jackson LE, Peña A. Hirschsprung disease and fecal incontinence: diagnostic and management strategies. J Pediatr Surg 2009; 44 (01) 271-277 , discussion 277
    CrossrefPubMedSearch in Google Scholar
  • 65 Teitelbaum DH, Coran AG. Enterocolitis. Semin Pediatr Surg 1998; 7 (03) 162-169
    CrossrefPubMedSearch in Google Scholar
  • 66 Menezes M, Puri P. Long-term outcome of patients with enterocolitis complicating Hirschsprung's disease. Pediatr Surg Int 2006; 22 (04) 316-318
    CrossrefPubMedSearch in Google Scholar
  • 67 Murphy F, Puri P. New insights into the pathogenesis of Hirschsprung's associated enterocolitis. Pediatr Surg Int 2005; 21 (10) 773-779
    CrossrefPubMedSearch in Google Scholar
  • 68 Pontarelli EM, Ford HR, Gayer CP. Recent developments in Hirschsprung's-associated enterocolitis. Curr Gastroenterol Rep 2013; 15 (08) 340
    CrossrefPubMedSearch in Google Scholar
  • 69 Hackam DJ, Filler RM, Pearl RH. Enterocolitis after the surgical treatment of Hirschsprung's disease: risk factors and financial impact. J Pediatr Surg 1998; 33 (06) 830-833
    CrossrefPubMedSearch in Google Scholar
  • 70 Wang X, Li Z, Xu Z, Wang Z, Feng J. Probiotics prevent Hirschsprung's disease-associated enterocolitis: a prospective multicenter randomized controlled trial. Int J Colorectal Dis 2015; 30 (01) 105-110
    CrossrefPubMedSearch in Google Scholar
  • 71 Starling JR, Croom III RD, Thomas Jr CG. Hirschsprung's disease in young adults. Am J Surg 1986; 151 (01) 104-109
    CrossrefPubMedSearch in Google Scholar
  • 72 Haricharan RN, Seo JM, Kelly DR. , et al. Older age at diagnosis of Hirschsprung disease decreases risk of postoperative enterocolitis, but resection of additional ganglionated bowel does not. J Pediatr Surg 2008; 43 (06) 1115-1123
    CrossrefPubMedSearch in Google Scholar
  • 73 Ammar SA, Ibrahim IA. One-stage transanal endorectal pull-through for treatment of Hirschsprung's disease in adolescents and adults. J Gastrointest Surg 2011; 15 (12) 2246-2250
    CrossrefPubMedSearch in Google Scholar
  • 74 Jarry J, Faucheron JL. Laparoscopic rectosigmoid resection with transanal colonic pull-through and delayed coloanal anastomosis: a new approach to adult Hirschsprung disease. Dis Colon Rectum 2011; 54 (10) 1313-1319
    CrossrefPubMedSearch in Google Scholar
  • 75 Vorobyov GI, Achkasov SI, Biryukov OM. Clinical features' diagnostics and treatment of Hirschsprung's disease in adults. Colorectal Dis 2010; 12 (12) 1242-1248
    CrossrefPubMedSearch in Google Scholar