Top

Published in:

01-05-2019 | Enterostomy | Original Article

Nationwide survey of outcome in patients with extensive aganglionosis in Japan

Authors: Satoshi Obata, Satoshi Ieiri, Takashi Akiyama, Naoto Urushihara, Hisayoshi Kawahara, Masayuki Kubota, Miyuki Kono, Yuji Nirasawa, Shohei Honda, Masaki Nio, Tomoaki Taguchi

Published in: Pediatric Surgery International | Issue 5/2019

Abstract

Purpose

Hirschsprung’s disease-related short bowel syndrome (HDSBS) is characterized by aganglionosis that extends orally to 75 cm from Treitz’s band. The condition is reported be associated with a high mortality rate of 50–80%. This retrospective study aimed to survey the current trends in HDSBS treatment in Japan.

Methods

Patient data were extracted from the results of a nationwide survey we conducted, resulting in the retrospective collection of the data of 1087 HD patients from 2008 to 2012 in Japan.

Results

A total of 11 (0.9%) cases of HDSBS were noted. All patients underwent jejunostomy as neonates. Radical procedures performed in five patients (A-colon patch method in four, Duhamel’s procedure in one). Ziegler’s myotomy-myectomy and serial transverse enteroplasty (STEP) were performed in each patient as palliative procedures. No radical operations were performed in 4 of the 11 cases. The mortality rate was 36.4%. Four patients died, 1 patient who underwent STEP and 3 patients who received no radical procedures. The causes of death were sepsis due to enterocolitis or central intravenous catheter infection, and hepatic failure. All patients who underwent radical procedures survived and showed satisfactory outcomes.

Conclusion

HDSBS still showed a high mortality rate, although surgical approaches such as the A-colon patch method resulted in satisfactory outcomes.

Taguchi T, Obata S, Ieiri S (2017) Current status of Hirschsprung’s disease: based on a nationwide survey of Japan. Pediatric Surg Int 33(4):497–504. https://doi.org/10.1007/s00383-016-4054-3 CrossRef

Hukkinen M, Koivusalo A, Merras-Salmio L, Rintala RJ, Pakarinen MP (2015) Postoperative outcome and survival in relation to small intestinal involvement of total colonic aganglionosis. J Pediatric Surg 50(11):1859–1864. https://doi.org/10.1016/j.jpedsurg.2015.05.017 CrossRef

Ruttenstock E, Puri P (2009) A meta-analysis of clinical outcome in patients with total intestinal aganglionosis. Pediatric Surg Int 25(10):833–839. https://doi.org/10.1007/s00383-009-2439-2 CrossRef

Ieiri S, Suita S, Nakatsuji T, Akiyoshi J, Taguchi T (2008) Total colonic aganglionosis with or without small bowel involvement: a 30-year retrospective nationwide survey in Japan. J Pediatric Surg 43(12):2226–2230. https://doi.org/10.1016/j.jpedsurg.2008.08.049 CrossRef

Wales PW, de Silva N, Kim JH, Lecce L, Sandhu A, Moore AM (2005) Neonatal short bowel syndrome: a cohort study. J Pediatric Surg 40(5):755–762. https://doi.org/10.1016/j.jpedsurg.2005.01.037 CrossRef

Amerstorfer EE, Fasching G, Till H, Huber-Zeyringer A, Höllwarth ME (2015) Long-term results of total colonic agangliosis patients treated by preservation of the aganglionic right hemicolon and the ileo-cecal valve. Pediatric Surg Int 31(8):773–780. https://doi.org/10.1007/s00383-015-3743-7 CrossRef

Pakarinen MP, Kurvinen A, Koivusalo AI, Ruuska T, Mäkisalo H, Jalanko H, Rintala RJ (2013) Surgical treatment and outcomes of severe pediatric intestinal motility disorders requiring parenteral nutrition. J Pediatric Surg 48(2):333–338. https://doi.org/10.1016/j.jpedsurg.2012.11.010 CrossRef

Ueno T, Wada M, Hoshino K, Sakamoto S, Furukawa H, Fukuzawa M (2013) A national survey of patients with intestinal motility disorders who are potential candidates for intestinal transplantation in Japan. Transpl Proc 45(5):2029–2031. https://doi.org/10.1016/j.transproceed.2013.01.092 CrossRef

Bond GJ, Reyes JD (2004) Intestinal transplantation for total/near-total aganglionosis and intestinal pseudo-obstruction. Semin Pediatric Surg 13(4):286–292. https://doi.org/10.1053/j.sempedsurg.2004.10.016 CrossRef

10.

Schwartz MZ (2013) Novel therapies for the management of short bowel syndrome in children. Pediatric Surg Int 29(10):967–974. https://doi.org/10.1007/s00383-013-3404-7 CrossRef

11.

Doğan SM, Kılınç S, Kebapçı E, Tuğmen C, Gürkan A, Baran M, Kurtulmuş Y, Ölmez M, Karaca C (2014) Mesenchymal stem cell therapy in patients with small bowel transplantation: single center experience. World J Gastroenterol WJG 20(25):8215–8220. https://doi.org/10.3748/wjg.v20.i25.8215 CrossRefPubMed

12.

El-Nachef W, Grikscheit T (2014) Enteric nervous system cell replacement therapy for hirschsprung disease: beyond tissue-engineered intestine. Eur J Pediatric Surg 24 (03):214–218. https://doi.org/10.1055/s-0034-1376311 CrossRef

Title: Nationwide survey of outcome in patients with extensive aganglionosis in Japan
Authors: Satoshi Obata
Satoshi Ieiri
Takashi Akiyama
Naoto Urushihara
Hisayoshi Kawahara
Masayuki Kubota
Miyuki Kono
Yuji Nirasawa
Shohei Honda
Masaki Nio
Tomoaki Taguchi
Publication date: 01-05-2019
Publisher: Springer Berlin Heidelberg
Keywords: Enterostomy
Enterostomy
Jejunostomy
Published in: Pediatric Surgery International / Issue 5/2019
Print ISSN: 0179-0358
Electronic ISSN: 1437-9813
DOI: https://doi.org/10.1007/s00383-019-04462-9

At a glance: The STEP trials

Springer Medicine

Nationwide survey of outcome in patients with extensive aganglionosis in Japan

Abstract

Purpose

Methods

Results

Conclusion

At a glance: The STEP trials

Springer Medicine

Abstract

Purpose

Methods

Results

Conclusion

Please log in to get access to this content

Other articles of this Issue 5/2019

Close relationship between the short round ligament and the ovarian prolapsed inguinal hernia in female infants

Independent risk factors for contralateral patent processus vaginalis undetected by pre-operative ultrasonography in boys with unilateral inguinal hernia

Repair of type IV laryngotracheoesophageal cleft (LTEC) on ECMO

Quality of life and functional outcome in Swedish children with low anorectal malformations: a follow-up study

Thoracoscopic thymectomy for juvenile myasthenia gravis

Adolescent gallstones—need for early intervention in symptomatic idiopathic gallstones