Morbus Hirschsprung

Zusammenfassung

Der Morbus Hirschsprung ist ein häufiges kinderchirurgisches Krankheitsbild.

Auch wenn eine Vielzahl von genetischen Mutationen beschrieben wurde, besteht die Trias der Diagnosestellung weiterhin in der sorgfältigen Anamnese, Kolon-Kontrastmitteluntersuchung und Rektumbiopsie.

Die Durchzugsoperation hat zum Ziel, den aganglionären Darm zu entfernen und die intestinale Passage durch Anastomosierung des innervierten Darms mit dem Analkanal unmittelbar oberhalb der Linea dentata zu rekonstruieren, sodass der Analkanal und die Sphinkterfunktion erhalten bleiben. Zu den häufigsten Techniken gehören die Operationen nach Swenson, Soave (de la Torre) und Duhamel. Diese können auch laparoskopisch assistiert durchgeführt werden.

Obwohl der Morbus Hirschsprung durch Resektion des aganglionären Darmes primär gut zu behandeln ist, ist die Langzeitmorbidität hoch. Diese wird vor allem durch die fort-bestehende Obstipation bis hin zur Stuhlinkontinenz oder rezidivierende Episoden einer Enterokolitis bestimmt. Durch eine zielgerichtete Diagnostik und multidisziplinäre Behandlung können diese Symptome meist im Verlauf mehrerer Jahre erfolgreich behandelt werden.

Der Pädiater spielt beim Erkennen und Management dieser Kinder eine wichtige Rolle.

Summary

The treatment of Hirschsprung disease is a common in pediatric surgery. Although a variety of genetic mutations have been described the triad of diagnosis consists of a thorough history, contrast enema and rectal biopsy.

The pull-through operation aims to resect aganglionic bowel and reestablish the intestinal passage by connecting healthy bowel to the anal canal just above the dentate (pecti-nate) line. While doing this the anal canal itself and the sphincter muscles have to be preserved by all means. Most common surgical techniques include the Swenson, Soave (de la Torre) and Duhamel pull-through. They can all be carried out by the help of laparoscopy.

In Hirschsprung disease the resection of aganglionic bowel is highly feasible, however long-term morbidity remains high. In later life ongoing constipation, fecal incontinence, and recurrent episodes of enterocolitis may be a problem. With a multidisciplinary approach these sequelae can be tackled successfully.

The pediatrician plays an important role in managing these children.

Schlüsselwörter

Aganglionose - Megakolon - Enterokolitis - Morbus Hirschsprung - Durchzugsoperation

Keywords

Aganglionosis - congenital megacolon - enterocolitis - Hirschsprung disease - pull-through

• Literatur

• 1 Arts E, Botden SM, Lacher M. et al. Duhamel versus transanal endorectal pull through (TERPT) for the surgical treatment of Hirschsprung’s disease. Tech Coloproctol 2016; 20: 677-682.
• 2 Bischoff A, Levitt MA, Peña A. Bowel management for the treatment of pediatric fecal incontinence. Pediatr Surg Int 2009; 25: 1027-1042.
• 3 De la Torre L, Ortega A. Transanal versus open endorectal pull-through for Hirschsprung’s disease. J Pediatr Surg 2000; 35: 1630-1632.
• 4 Friedmacher F, Puri P. Hirschsprung’s disease associated with Down syndrome: a meta-analysis of incidence, functional outcomes and mortality. Pediatr Surg Int 2013; 29: 937-946.
• 5 Georgeson KE, Fuenfer MM, Hardin WD. Primary laparoscopic pull-through for Hirsch-sprung’s disease in infants and children. J Pediatr Surg 1995; 30: 1017-1021.
• 6 Goldstein A, Hofstra R, Burns A. Building a brain in the gut: development of the enteric nervous system. Clin Genet 2013; 83: 307-316.
• 7 Gosain A, Frykman PK, Cowles RA. et al; American Pediatric Surgical Association Hirschsprung Disease Interest Group. Guidelines for the diagnosis and management of Hirschsprung-associated enterocolitis. Pediatr Surg Int 2017; 33: 517-521.
• 8 Gosemann JH, Friedmacher F, Ure B. et al. Open versus transanal pull-through for Hirschsprung disease: a systematic review of long-term outcome. Eur J Pediatr Surg 2013; 23: 94-102.
• 9 Keyzer-Dekker CM, Sloots CE, Schokker-van Linschoten IK. et al. Effectiveness of Rectal Suction Biopsy in Diagnosing Hirschsprung Disease. Eur J Pediatr Surg 2016; 26: 100-105.
• 10 Lacher M, Fitze G, Helmbrecht J. et al. Hirsch-sprung-associated enterocolitis develops independently of NOD2 variants. J Pediatr Surg 2010; 45: 1826-1831.
• 11 Langer JC. Laparoscopic and transanal pull-through for Hirschsprung disease. Semin Pediatr Surg 2012; 21: 283-290.
• 12 Langer JC, Rollins MD, Levitt M. et al; American Pediatric Surgical Association Hirschsprung Disease Interest Group. Guidelines for the management of postoperative obstructive symptoms in children with Hirschsprung disease. Pediatr Surg Int 2017; 33: 523-526.
• 13 Lawal TA, Rangel SJ, Bischoff A. et al. Laparoscopic-assisted Malone appendicostomy in the management of fecal incontinence in children. J Laparoendosc Adv Surg Tech A 2011; 21: 455-459.
• 14 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: 344-353.
• 15 Lewis NA, Levitt MA, Zallen GS. et al. Diagnosing Hirschsprung’s disease: increasing the odds of a positive rectal biopsy result. J Pediatr Surg 2003; 38: 412-416.
• 16 Moore SW. Genetic impact on the treatment & management of Hirschsprung disease. J Pediatr Surg 2017; 52: 218-222.
• 17 Muise ED, Cowles RA. Rectal biopsy for Hirsch-sprung’s disease: a review of techniques, pathology, and complications. World J Pediatr 2016; 12: 135-141.
• 18 Nakamura H, Henderson D, Puri P. A meta-analysis of clinical outcome of intestinal transplantation in patients with total intestinal aganglionosis. Pediatr Surg Int 2017; 8: 837-841.
• 19 Pini Prato A, Rossi V, Avanzini S. et al. Hirsch-sprung’s disease: what about mortality?. Pediatr Surg Int 2011; 27: 473-478.
• 20 Putnam LR, John SD, Greenfield SA. et al. The utility of the contrast enema in neonates with suspected Hirschsprung disease. J Pediatr Surg 2015; 50: 963-966.
• 21 Rowan-Legg A. Managing functional constipation in children. Paediatr Child Health 2012; 16: 661-670.
• 22 Santos-Jasso KA, Arredondo-García JL, Maza-Vallejos J. et al. Effectiveness of senna vs polyethylene glycol as laxative therapy in children with constipation related to anorectal malformation. J Pediatr Surg 2017; 52: 84-88.
• 23 Thomson D, Allin B, Long AM. et al. Laparoscopic assistance for primary transanal pull-through in Hirschsprung’s disease: a systematic review and meta-analysis. BMJ Open 2015; 5: e006063.
• 24 Puri P, Gosemann JH. Variants of Hirschsprung disease. Semin Pediatr Surg 2012; 21: 310-318.