Developmental Disturbances

1

INI CET 2021 May

Question

Syndrome which develops from mutation of all three germ layers?

OPTION 2

 

•            Gardner syndrome is an interesting disease complex, reviewed by Fader and his associates and by Duncan and his associates.

•            Gardner's syndrome is a dominant autosomal non sex-linked hereditary condition resulting from lesions of all three primary germ layers : ectoderm, entoderm and mesoderm.

•            It therefore includes a variety of symptoms which concern E.N.T. specialists, dermatologists, maxillo-facial surgeons and gastroenterologists, although the present tendency is to recognize the existence of partial or dissociated Gardner's syndromes.

•            The finding of an isolated symptom of the Gardner's series should lead to a systematic search for polyposis of the large bowel, as the latter carries a short- or long-term risk of malignant degeneration which has considerable bearing on prognosis.

 

•            It is also characterized by the occurrence of multiple impacted supernumerary teeth. This syndrome consists of:

1.           Multiple polyposis of the large intestine

2.           Osteomas of the bones, including long bones, skull, and jaws

3.           Multiple epidermoid or sebaceous cysts of the skin, particularly on the scalp and back

4.           Occasional occurrence of desmoid tumors

5.           The impacted supernumerary and permanent teeth