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Review
. 2018 Mar;31(2):132-142.
doi: 10.1055/s-0037-1609029. Epub 2018 Feb 25.

Current Approaches to Pediatric Polyposis Syndromes

Aodhnait S Fahy  1 , Christopher R Moir  1
Affiliations
Review

Current Approaches to Pediatric Polyposis Syndromes

Aodhnait S Fahy et al. Clin Colon Rectal Surg. 2018 Mar.

Abstract

Colorectal adenomatous polyposis syndromes encompass a diverse group of disorders with varying modes of inheritance and penetrance. Children may present with overt disease or within screening programs for families at high risk. We provide an overview of the array of pediatric polyposis syndromes, current screening recommendations, and surgical indications and technical considerations. Optimal disease management for these pediatric patients is still evolving and has implications for screening, surveillance, pediatric surgical management, and transition of care gastroenterologic neoplasia physicians and surgeons.

Keywords: IPAA; pediatric polyposis; restorative proctocolectomy.

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Figures

Fig. 1
Fig. 1
Stapled ileoanal anastomosis. ( A ) The anastomosis is undertaken with the circular stapler as indicated. ( B ) Shorter cuff lengths are possible with this technique (©Mayo Clinic).
Fig. 2
Fig. 2
Maneuvers to increase mesenteric reach in ileal pouch anal anastomosis. ( A ) Initial mobilization of the ileal mesentery away from the retroperitoneum by optimal dissection to the mesenteric root of the SMA (IC artery, ileocolic artery; RC, right colic artery; –MC, middle colic artery). ( B ) This exposes the aorta (ao), duodenum (D), right ureter (Ur), and gonadal vessels (GV). ( C ) The mesenteric reach can be further extended by scoring the peritoneum stepwise. ( D and E ) Ligation of the ileocolic with preservation of the distal SMA can provide further reach for the ileal J pouch (©Mayo Clinic).
See this image and copyright information in PMC

References

    1. Burn J, Chapman P, Delhanty J et al. The UK Northern region genetic register for familial adenomatous polyposis coli: use of age of onset, congenital hypertrophy of the retinal pigment epithelium, and DNA markers in risk calculations. J Med Genet. 1991;28(05):289–296. - PMC - PubMed
    1. Kerr S E, Thomas C B, Thibodeau S N, Ferber M J, Halling K C. APC germline mutations in individuals being evaluated for familial adenomatous polyposis: a review of the Mayo Clinic experience with 1591 consecutive tests. J Mol Diagn. 2013;15(01):31–43. - PubMed
    1. Bussey H. Baltimore, MD: Johns Hopkins University Press; 1975. Familial Polyposis Coli: Family Studies, Histopathology, Differential Diagnosis, and Results of Treatment.
    1. Vasen H F, Möslein G, Alonso A et al. Guidelines for the clinical management of familial adenomatous polyposis (FAP) Gut. 2008;57(05):704–713. - PubMed
    1. Burt R W, Leppert M F, Slattery M L et al. Genetic testing and phenotype in a large kindred with attenuated familial adenomatous polyposis. Gastroenterology. 2004;127(02):444–451. - PubMed