European Paediatric Surgeons' Association Survey on the Management of Hirschsprung Disease

Abstract

Aim This study aims to define patterns of Hirschsprung disease (HD) management.

Methods An online questionnaire was sent to all European Paediatric Surgeons' Association (EUPSA) members.

Results A total of 294 members (61 countries) answered (response rate: 61%). Diagnosis: All respondents perform rectal biopsies (61% rectal suction [RSBs], 39% open full-thickness), 96% contrast enema, and 31% anorectal manometry. At RSB, 17% take the most distal biopsy 1 cm above the dentate line, 34% take 2 cm, 30% take 3 cm, and 19% take > 3 cm. Rectal biopsy staining's are hematoxylin/eosin (77%), acetylcholinesterase (74%), calretinin (31%), S100 (2%), nicotinamide adenine dinucleotide-tetrazolium reductase (2%), succinate dehydrogenase (1%), and neuron-specific enolase (1%). A total of 85% respondents recognize entities including hypoganglionosis (69%), intestinal neuronal dysplasia (55%), and ultrashort segment HD (50%). Surgery: Pull-through (PT) is performed at diagnosis by 33% or delayed by 67% (4 months or > 5 kg). Awaiting definitive surgery, 77% perform rectal irrigations, 22% rectal dilatation/stimulations, and 33% perform a stoma. The preferred type of PT is the Soave approach (65%), performed with transanal technique by 70% respondents. If symptoms persist after PT, most opt for conservative approach (enemas/laxatives = 76%; botulinum toxin = 27%), 30% would redo the PT. Total colonic aganglionosis: PT is performed in neonates (4%), at 1 to 6 months (29%), 6 to 12 months (37%) or older (30%). If required, a stoma is sited in the ileum (31%), according to intraoperative biopsies (54%), macroscopic impression (13%), and radiology (2%). Duhamel PT is performed by 52%, Soave by 31%, and Swenson by 17%. Overall, 31% would perform a J-pouch.

Conclusions Most aspects of HD management lack consensus with wide variations in obtaining a diagnosis. Transanal Soave PT is the most common technique in standard segment HD. Guidelines should be developed to avoid such variability in management and to facilitate research studies.

Keywords

congenital megacolon - aganglionosis - Soave - Duhamel - transanal

• References

• 1 Grosfeld JL. Hirschsprung's disease: A historical perspective—1691–2005. In: Holschneider A, Puri P, , eds. Hirschsprung's Disease and Allied Disorders. Berlin, Germany: Springer-Verlag; 2008: 1-12
• 2 Kleinhaus S, Boley SJ, Sheran M, Sieber WK. Hirschsprung's disease–a survey of the members of the Surgical Section of the American Academy of Pediatrics. J Pediatr Surg 1979; 14 (5) 588-597
• 3 Keckler SJ, Yang JC, Fraser JD , et al. Contemporary practice patterns in the surgical management of Hirschsprung's disease. J Pediatr Surg 2009; 44 (6) 1257-1260 , discussion 1260
• 4 Huddart SN. Hirschsprung's disease: present UK practice. Ann R Coll Surg Engl 1998; 80 (1) 46-48
• 5 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 (1) 34-38
• 6 Ikeda K, Goto S. Diagnosis and treatment of Hirschsprung's disease in Japan. An analysis of 1628 patients. Ann Surg 1984; 199 (4) 400-405
• 7 Suita S, Taguchi T, Kamimura T, Yanai K. Total colonic aganglionosis with or without small bowel involvement: a changing profile. J Pediatr Surg 1997; 32 (11) 1537-1541
• 8 Suita S, Taguchi T, Ieiri S, Nakatsuji T. Hirschsprung's disease in Japan: analysis of 3852 patients based on a nationwide survey in 30 years. J Pediatr Surg 2005; 40 (1) 197-201 , discussion 201–202
• 9 Zani A, Zani-Ruttenstock E, Eaton S, Pierro A. The value of surveys in pediatric surgery. Eur J Pediatr Surg 2015; 25 (6) 500-503
• 10 Noblett HR. A rectal suction biopsy tube for use in the diagnosis of Hirschsprung's disease. J Pediatr Surg 1969; 4 (4) 406-409
• 11 Aldridge RT, Campbell PE. Ganglion cell distribution in the normal rectum and anal canal. A basis for the diagnosis of Hirschsprung's disease by anorectal biopsy. J Pediatr Surg 1968; 3 (4) 475-490
• 12 Barshack I, Fridman E, Goldberg I, Chowers Y, Kopolovic J. The loss of calretinin expression indicates aganglionosis in Hirschsprung's disease. J Clin Pathol 2004; 57 (7) 712-716
• 13 Kapur RP, Reed RC, Finn LS, Patterson K, Johanson J, Rutledge JC. Calretinin immunohistochemistry versus acetylcholinesterase histochemistry in the evaluation of suction rectal biopsies for Hirschsprung Disease. Pediatr Dev Pathol 2009; 12 (1) 6-15
• 14 Morris MI, Soglio DB, Ouimet A, Aspirot A, Patey N. A study of calretinin in Hirschsprung pathology, particularly in total colonic aganglionosis. J Pediatr Surg 2013; 48 (5) 1037-1043
• 15 Martucciello G, Pini Prato A, Puri P , et al. Controversies concerning diagnostic guidelines for anomalies of the enteric nervous system: a report from the fourth International Symposium on Hirschsprung's disease and related neurocristopathies. J Pediatr Surg 2005; 40 (10) 1527-1531
• 16 Chen JZ, Jamieson DH, Skarsgard ED. Does pre-biopsy contrast enema delay the diagnosis of long segment Hirschsprung's disease?. Eur J Pediatr Surg 2010; 20 (6) 375-378
• 17 Muller CO, Mignot C, Belarbi N, Berrebi D, Bonnard A. Does the radiographic transition zone correlate with the level of aganglionosis on the specimen in Hirschsprung's disease?. Pediatr Surg Int 2012; 28 (6) 597-601
• 18 Singh SJ, Croaker GD, Manglick P , et al. Hirschsprung's disease: the Australian Paediatric Surveillance Unit's experience. Pediatr Surg Int 2003; 19 (4) 247-250
• 19 Swenson O, Bill Jr AH. Resection of rectum and rectosigmoid with preservation of the sphincter for benign spastic lesions producing megacolon; an experimental study. Surgery 1948; 24 (2) 212-220
• 20 Duhamel B. Retrorectal and transanal pull-through procedure for the treatment of Hirschsprung's disease. Dis Colon Rectum 1964; 7: 455-458
• 21 Soave F. A new original technique for treatment of Hirschsprung's disease. Surgery 1964; 56: 1007-1014
• 22 Rintala RJ, Lindahl HG. Proctocolectomy and J-pouch ileo-anal anastomosis in children. J Pediatr Surg 2002; 37 (1) 66-70
• 23 Lal DR, Nichol PF, Harms BA, Go LL, Lund DP. Ileo-anal S-Pouch reconstruction in patients with total colonic aganglionosis after failed pull-through procedure. J Pediatr Surg 2004; 39 (7) e7-e9