Wednesday, June 12, 2024
HomePediatrics: General MedicinePediatric Gardner Syndrome

Pediatric Gardner Syndrome

Background

Gardner syndrome is a familial polyposis syndrome, better classified as a variant of familial adenomatous polyposis (FAP). In Gardner syndrome, the symptoms of classic FAP syndrome are present; this consists of the development of approximately 500-2500 colonic adenomas that blanket the surface of the colonic mucosa. Multiple adenomas are also often present throughout the GI tract, especially in the periampullary region and the stomach.

In addition to the classic findings of FAP, other findings include multiple osteomas (commonly of the skull, mandible, and long bones), dental abnormalities (including supernumerary teeth and odontomas), epidermal cysts, thyroid carcinoma, pancreatic adenocarcinoma, congenital hypertrophy of the retinal pigmented epithelium (CHRPE), and fibromatosis or desmoid tumors (commonly in the abdominal wall and retroperitoneum).
Less commonly, lipomas, leiomyomas, neurofibromas, hepatoblastomas, and pigmented skin lesions are also seen.

RELATED ARTICLES
- Advertisment -

Most Popular