Skip to main content
Top
Published in: Pediatric Surgery International 5/2016

01-05-2016 | Original Article

The mid-term outcomes of TRM–PIAS, proctocolectomy and ileoanal anastomosis for total colonic aganglionosis

Authors: Qi Li, Long Li, Qian Jiang, Zhen Zhang, Ping Xiao

Published in: Pediatric Surgery International | Issue 5/2016

Login to get access

Abstract

Aims

The present study aimed to evaluate the mid-term outcomes of total colonic aganglionosis (TCA) after transanal rectal mucosectomy and partial internal anal sphincterectomy (TRM–PIAS), proctocolectomy and ileoanal anastomosis.

Patients and methods

From 2012 to 2014, 12 patients (7 boys; 58.3 %) diagnosed with TCA and treated with the TRM–PIAS, proctocolectomy and ileoanal anastomosis. Seven TCA patients who underwent laparotomy-assisted endorectal pull-through (LEPT) between 2010 and 2012 were used as control group. Demographic features and complication of the two groups were evaluated. The functional outcomes were assessed by using a score system.

Results

The procedure was successfully performed in all patients. The incidence of postoperative HAEC in the TRM–PIAS group was significantly lower (25.0 vs 85.7 %; p < 0.05) than control group within the second postoperative year. The number of bowel movement after 3, 12 and 24 months postoperatively, was 8.5 ± 3.5, 5.3 ± 2.9 and 3.1 ± 1.4 (p < 0.05) per day, respectively, in the TRM–PIAS group. The soiling was noted in 50.0 % (n = 6) of the patients in the 6th postoperative month, and 25.0 % (n = 3) in the 24th postoperative month in the TRM–PIAS group. There was no significant difference in overall functional outcome between two groups, but the TRM–PIAS group was better in terms of bowel movement and soiling.

Conclusion

TRM–PIAS, proctocolectomy and ileoanal anastomosis might be an effective treatment for TCA. More prospective studies evaluating the TRM–PIAS technique over longer period and with greater sample size are needed to confirm the findings in this study.
Literature
1.
go back to reference Laughlin DM, Friedmacher F, Puri P (2012) Total colonic aganglionosis: a systematic review and meta-analysis of long-term clinical outcome. Pediatr Surg Int 28(8):773–779CrossRefPubMed Laughlin DM, Friedmacher F, Puri P (2012) Total colonic aganglionosis: a systematic review and meta-analysis of long-term clinical outcome. Pediatr Surg Int 28(8):773–779CrossRefPubMed
2.
go back to reference Hukkinen M et al (2015) Postoperative outcome and survival in relation to small intestinal involvement of total colonic aganglionosis. J Pediatr Surg 50:1859–1864 Hukkinen M et al (2015) Postoperative outcome and survival in relation to small intestinal involvement of total colonic aganglionosis. J Pediatr Surg 50:1859–1864
3.
go back to reference Zhang JS et al (2014) Transanal rectal mucosectomy and partial internal anal sphincterectomy for Hirschsprung’s disease. J Pediatr Surg 49(5):831–834CrossRefPubMed Zhang JS et al (2014) Transanal rectal mucosectomy and partial internal anal sphincterectomy for Hirschsprung’s disease. J Pediatr Surg 49(5):831–834CrossRefPubMed
4.
go back to reference Pastor AC et al (2009) Development of a standardized definition for Hirschsprung’s-associated enterocolitis: a Delphi analysis. J Pediatr Surg 44(1):251–256CrossRefPubMed Pastor AC et al (2009) Development of a standardized definition for Hirschsprung’s-associated enterocolitis: a Delphi analysis. J Pediatr Surg 44(1):251–256CrossRefPubMed
5.
go back to reference Holschneider AM, Puri P (2008) Hirschsprung’s disease and allied disorders. Springer, New York Holschneider AM, Puri P (2008) Hirschsprung’s disease and allied disorders. Springer, New York
6.
go back to reference Wildhaber BE, Teitelbaum DH, Coran AG (2005) Total colonic Hirschsprung’s disease: a 28-year experience. J Pediatr Surg 40(1):203–206 (discussion 206–7) Wildhaber BE, Teitelbaum DH, Coran AG (2005) Total colonic Hirschsprung’s disease: a 28-year experience. J Pediatr Surg 40(1):203–206 (discussion 206–7)
7.
go back to reference Austin KM (2012) The pathogenesis of Hirschsprung’s disease-associated enterocolitis. Semin Pediatr Surg 21(4):319–327CrossRefPubMed Austin KM (2012) The pathogenesis of Hirschsprung’s disease-associated enterocolitis. Semin Pediatr Surg 21(4):319–327CrossRefPubMed
8.
go back to reference Anupama B, Zheng S, Xiao X (2007) Ten-year experience in the management of total colonic aganglionosis. J Pediatr Surg 42(10):1671–1676CrossRefPubMed Anupama B, Zheng S, Xiao X (2007) Ten-year experience in the management of total colonic aganglionosis. J Pediatr Surg 42(10):1671–1676CrossRefPubMed
9.
go back to reference Escobar MA et al (2005) Long-term outcomes in total colonic aganglionosis: a 32-year experience. J Pediatr Surg 40(6):955–961CrossRefPubMed Escobar MA et al (2005) Long-term outcomes in total colonic aganglionosis: a 32-year experience. J Pediatr Surg 40(6):955–961CrossRefPubMed
10.
go back to reference Martin LW (1968) Surgical management of Hirschsprung’s disease involving the small intestine. Arch Surg 97(2):183–189CrossRefPubMed Martin LW (1968) Surgical management of Hirschsprung’s disease involving the small intestine. Arch Surg 97(2):183–189CrossRefPubMed
12.
go back to reference Kimura K et al (1981) A new surgical approach to extensive aganglionosis. J Pediatr Surg 16(6):840–843CrossRefPubMed Kimura K et al (1981) A new surgical approach to extensive aganglionosis. J Pediatr Surg 16(6):840–843CrossRefPubMed
13.
go back to reference Hoehner JC et al (1998) Long-term morbidity in total colonic aganglionosis. J Pediatr Surg 33(7):961–965 (discussion 965–6) Hoehner JC et al (1998) Long-term morbidity in total colonic aganglionosis. J Pediatr Surg 33(7):961–965 (discussion 965–6)
14.
go back to reference Davies MR, Cywes S (1983) Inadequate pouch emptying following Martin’s pull-through procedure for intestinal aganglionosis. J Pediatr Surg 18(1):14–20CrossRefPubMed Davies MR, Cywes S (1983) Inadequate pouch emptying following Martin’s pull-through procedure for intestinal aganglionosis. J Pediatr Surg 18(1):14–20CrossRefPubMed
15.
go back to reference Shen C et al (2009) A comparison of the effectiveness of the Soave and Martin procedures for the treatment of total colonic aganglionosis. J Pediatr Surg 44(12):2355–2358CrossRefPubMed Shen C et al (2009) A comparison of the effectiveness of the Soave and Martin procedures for the treatment of total colonic aganglionosis. J Pediatr Surg 44(12):2355–2358CrossRefPubMed
16.
go back to reference Bischoff A, Levitt MA, Pena A (2011) Total colonic aganglionosis: a surgical challenge. How to avoid complications? Pediatr Surg Int 27(10):1047–1052CrossRefPubMedPubMedCentral Bischoff A, Levitt MA, Pena A (2011) Total colonic aganglionosis: a surgical challenge. How to avoid complications? Pediatr Surg Int 27(10):1047–1052CrossRefPubMedPubMedCentral
17.
go back to reference Tsuji H et al (1999) Management and long-term follow-up of infants with total colonic aganglionosis. J Pediatr Surg 34(1):158–161 (discussion 162) Tsuji H et al (1999) Management and long-term follow-up of infants with total colonic aganglionosis. J Pediatr Surg 34(1):158–161 (discussion 162)
18.
go back to reference Levitt MA, Dickie B, Pena A (2010) Evaluation and treatment of the patient with Hirschsprung disease who is not doing well after a pull-through procedure. Semin Pediatr Surg 19(2):146–153CrossRefPubMed Levitt MA, Dickie B, Pena A (2010) Evaluation and treatment of the patient with Hirschsprung disease who is not doing well after a pull-through procedure. Semin Pediatr Surg 19(2):146–153CrossRefPubMed
19.
go back to reference Ralls MW, Coran AG, Teitelbaum DH (2012) Reoperative surgery for Hirschsprung disease. Semin Pediatr Surg 21(4):354–363CrossRefPubMed Ralls MW, Coran AG, Teitelbaum DH (2012) Reoperative surgery for Hirschsprung disease. Semin Pediatr Surg 21(4):354–363CrossRefPubMed
20.
go back to reference Dickie BH et al (2014) The problematic Soave cuff in Hirschsprung disease: manifestations and treatment. J Pediatr Surg 49(1):77–80 (discussion 80–1) Dickie BH et al (2014) The problematic Soave cuff in Hirschsprung disease: manifestations and treatment. J Pediatr Surg 49(1):77–80 (discussion 80–1)
21.
go back to reference Ralls MW et al (2014) Redo pullthrough for Hirschsprung disease: a single surgical group’s experience. J Pediatr Surg 49(9):1394–1399CrossRefPubMed Ralls MW et al (2014) Redo pullthrough for Hirschsprung disease: a single surgical group’s experience. J Pediatr Surg 49(9):1394–1399CrossRefPubMed
Metadata
Title
The mid-term outcomes of TRM–PIAS, proctocolectomy and ileoanal anastomosis for total colonic aganglionosis
Authors
Qi Li
Long Li
Qian Jiang
Zhen Zhang
Ping Xiao
Publication date
01-05-2016
Publisher
Springer Berlin Heidelberg
Published in
Pediatric Surgery International / Issue 5/2016
Print ISSN: 0179-0358
Electronic ISSN: 1437-9813
DOI
https://doi.org/10.1007/s00383-016-3870-9

Other articles of this Issue 5/2016

Pediatric Surgery International 5/2016 Go to the issue