Abstract
Gardner’s syndrome (GS) is a very rare autosomal dominant multisystem disease. Osteomas, skin and soft tissue tumors are present with gastrointestinal polyposis. The polyps have very high malignancy potentials. If prophylactic resection is not performed, colorectal cancer development is inevitable in all patients with GS. Polyposis is usually asymptomatic. Therefore, careful evaluation of extraintestinal findings of the disease is very important for early diagnosis. In this article, diagnosis and treatment of GS are presented in monozygotic twins, which have not been previously described in the literature. The diagnostic process, which started with dental complaints of one case, was carried out in an effective manner and then, prophylactic surgery was performed in twins. This article aimed to make clinicians and dentists attentive for early diagnosis of disease and to review treatment options.
Publisher
Turkish Journal of Surgery
Subject
General Chemical Engineering
Reference15 articles.
1. Gardner EJ, Richards RC. Multiple cutaneous and subcutaneous lesions occurring simultaneously with hereditary polyposis and osteomatosis. Am J Hum Genet 1953; 5(2): 139-47.
2. Nandakumar G, Morgan JA, Silverberg D, Steinhagen RM. Familial polyposis coli: Clinical manifestations, evaluation, management and treatment. Mt Sinai J Med 2004; 71(6): 384-91.
3. Gardner EJ. A genetic and clinical study of intestinal polyposis: A predisposing factor for carsinoma of the colon and rectum. Am J Hum Genet 1951; 3(2): 167-76.
4. Vaswani BA, Shah M, Shah PM, Parikh BJ, Anand AS, Sharma GL. Giant mesenteric fibromatosis in Gardner’s syndrome. Indian J Cancer 2011; 48(1): 140-2. https://doi.org/10.4103/0019-509X.76652
5. Agrawal D, Newaskar V, Shrivastava S, Nayak PA. External manifestations of Gardner’s syndrome as the presenting clinical entity. BMJ Case Reports 2014; 2014: bcr2013200293. https://doi.org/10.1136/ bcr-2013-200293