Familial Adenomatous Polyposis: A Generational Disease Managed by Generations of Surgeons with an Interesting Indication for Intersphincteric Proctectomy

Author(s): Nicholas K. Lago, Tara Lewandowski, Stacey L. Tannenbaum, Charles P. Lago

Background: Classic Familial Adenomatous Polyposis (FAP) is an inherited disorder in which hundreds to thousands of adenomatous polyps develop in the gastrointestinal (GI) tract, predominantly in the colon and rectum. Most patients with classic FAP initially present with nonspecific GI symptoms such as diarrhea, abdominal discomfort, or rectal bleeding. The American Society of Colon and Rectal Surgeons recommends that polyposis syndromes be considered in patients with greater than 20 lifetime adenomas, a personal history of desmoid tumor or other extracolonic manifestations of FAP, or in individuals with known family history of FAP or other subtypes of FAP. Screening individuals with a family history of Classic Familial Adenomatous Polyposis is essential to survival, as colorectal cancer is inevitable with a diagnosis of FAP.

Case Presentation: We present multiple examples of cases of classic FAP across several generations of one family including an uncommon indication for intersphincteric proctectomy, all of whom are being treated by their original surgeon Dr. Charles Lago along with his son, Dr. Nicholas Lago.

Conclusion: The mainstay treatment of FAP is colectomy with or without proctectomy. Therefore, an intersphincteric proctectomy should be considered a safe and suitable option for patients with retained rectum and non-oncologic indications for resection.