Characterizing Pediatric Familial Adenomatous Polyposis in Patients Undergoing Colectomy in the United States

Colleen B. Flahive*, Amanda Onwuka, Lee M. Bass, Suzanne P. MacFarland, Peter C. Minneci, Steven H. Erdman

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

Abstract

Objective: To characterize a multi-institutional cohort of pediatric patients who underwent colectomy for familial adenomatous polyposis (FAP). Study design: In this retrospective cohort study, diagnosis and procedure codes were used to identify patients who underwent colectomy for FAP within the Pediatric Health Information System (PHIS). The inclusion criteria were validated at 3 children's hospitals and applied to PHIS to generate a cohort of patients with FAP between 2 and 21 years who had undergone colectomy between 2009 and 2019. Demographics, clinical and surgical characteristics, and endoscopic procedure trends as identified through PHIS are described. Descriptive and comparative statistics were used to analyze data. Results: Within the PHIS, 428 pediatric patients with FAP who underwent colectomy were identified. Median age at colectomy was 14 years (range 2-21 years); 264 patients (62%) received an ileal pouch anal anastomosis and 13 (3%) underwent ileorectal anastomosis. Specific anastomotic surgical procedure codes were not reported for 151 patients (35%). Endoscopic assessment at the surgical institution occurred in 40% of the cohort before colectomy and in 22% of the cohort following colectomy. Conclusions: In this cohort, colectomy took place at an earlier age than suggested in published guidelines. Ileal pouch anal anastomosis is the predominant procedure for pediatric patients with FAP who underwent colectomy in US pediatric centers. Endoscopic assessment trends before and after surgery suggest that the surgical institution plays a limited role in the care of this population.

Original languageEnglish (US)
JournalJournal of Pediatrics
DOIs
StateAccepted/In press - 2021
Externally publishedYes

Keywords

  • cancer predisposition syndromes
  • ileal-pouch anal anastomosis (IPAA)
  • ileorectal anastomosis (IRA)
  • polyposis

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

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