Abstract
Patients with familial adenomatous polyposis require surgical intervention at some point in their lives. The diagnosis is often apparent from their phenotype and family history, however, this is not always the case. Many factors can influence the surgical strategy although the polyposis burden and distribution remain the main consideration. While prophylactic removal of the rectum and colon is often required, sparing the rectum at the index surgery is safe in select patients. This article aims to dispel misconceptions in the diagnosis and treatment of patients with familial adenomatous polyposis.
Keywords:
colorectal surgery; familial adenomatous polyposis; total proctocolectomy.
© 2017 Royal Australasian College of Surgeons.
MeSH terms
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Abdominal Neoplasms / complications*
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Abdominal Neoplasms / diagnosis
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Adenomatous Polyposis Coli / complications
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Adenomatous Polyposis Coli / diagnosis*
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Adenomatous Polyposis Coli / mortality
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Adenomatous Polyposis Coli / surgery
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Adult
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Anastomosis, Surgical / standards
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Clinical Decision-Making
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Colorectal Neoplasms / diagnosis*
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Colorectal Neoplasms / surgery
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Cost of Illness
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Female
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Fibromatosis, Aggressive / complications*
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Fibromatosis, Aggressive / diagnosis
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Genotype
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Humans
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Laparoscopy / methods
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Male
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Proctocolectomy, Restorative / adverse effects
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Proctocolectomy, Restorative / methods*
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Prophylactic Surgical Procedures / ethics
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Quality of Life
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Rectum / surgery
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Risk Factors
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Therapeutic Misconception / ethics*
Supplementary concepts
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Desmoid disease, hereditary