Evaluation of 28 years of surgical treatment of children and young adults with familial adenomatous polyposis

Booij, Klaske A.C.; Mathus-Vliegen, Elisabeht M.H.; Taminiau, Jan A.J.M.; Ten Kate, Fibo J.W.; Slors, J. Frederick M.; Tabbers, Merit M.; Aronson, Daniel C.

doi:10.1016/j.jpedsurg.2009.06.017

« Previous Next »Journal of Pediatric Surgery
Volume 45, Issue 3 , Pages 525-532, March 2010

Evaluation of 28 years of surgical treatment of children and young adults with familial adenomatous polyposis

Klaske A.C. Booij
- Department of Pediatric Surgery, Emma Children's Hospital AMC, Amsterdam 1100 DD, The Netherlands
- Corresponding author. Academic Medical Center, University of Amsterdam, 1105 AZ Amsterdam, The Netherlands. Fax: +20 5669243.
Elisabeht M.H. Mathus-Vliegen
- Department of Gastroenterology and Hepatology, Academic Medical Center, University of Amsterdam, Amsterdam 1100 DD, The Netherlands
Jan A.J.M. Taminiau
- Department of Pediatric Gastroenterology and Nutrition, Emma Children's Hospital AMC, Amsterdam 1100 DD, The Netherlands
Fibo J.W. Ten Kate
- Department of Pathology, Academic Medical Center, University of Amsterdam, Amsterdam 1100 DD, The Netherlands
J. Frederick M. Slors
- Department of Surgery, Academic Medical Center, University of Amsterdam, Amsterdam 1100 DD, The Netherlands
- Deceased.
Merit M. Tabbers
- Department of Pediatric Gastroenterology and Nutrition, Emma Children's Hospital AMC, Amsterdam 1100 DD, The Netherlands
Daniel C. Aronson
- Department of Pediatric Surgery, Emma Children's Hospital AMC, Amsterdam 1100 DD, The Netherlands

Received 12 September 2008; received in revised form 5 June 2009; accepted 12 June 2009.

Abstract

Background

In this retrospective study, 28 years of surgical treatment of children and young adults with familial adenomatous polyposis (FAP) was analyzed.

Methods

Forty-three patients were operated on before the age of 26 years. Endoscopic aspects, operative data, and complications were analyzed, and the resection specimens were reevaluated. Functional outcome was assessed by telephone questionnaire.

Results

Primary ileorectal anastomosis (IRA) was performed in 34 patients with a mean age of 16 years (range, 7-25 years). Primary ileal-pouch anal anastomosis (IPAA) was performed in 9 patients at a mean age of 19 years (range, 15-24 years). Secondary excision of the rectum was performed in 7 patients. Overall, rectal carcinoma was present in 4 patients, at the age of 35, 36, 37, and 38 years. Two patients, aged 39 and 40 years, died because of invasive carcinoma with distant metastasis. The functional outcome and postoperative complications after both procedures were similar to those described in literature for children with FAP. Most patients did not experience alterations in lifestyle, and there was no urinary incontinence.

Conclusions

In this retrospective study, both IRA and IPAA showed to be feasible techniques in young patients with FAP. A prospective study with a sufficient follow-up is needed to compare both techniques in this specific group of patients.

Key words: Familial adenomatous polyposis coli, Pediatric patients, Children, Teenagers, Ileal pouch-anal anastomosis, Ileorectal anastomosis