Greig cephalopolysyndactyly syndrome

Greig cephalopolysyndactyly syndrome (GCPS) is a rare inherited disorder characterized by bodily abnormalities affecting the fingers and toes (digits) and the head and facial (craniofacial) area. The skin between the fingers and toes may be fused (cutaneous syndactyly). Greig Cephalopolysyndactyly Syndrome is caused by a gene with a change or mutation that is inherited from a parent. The gene that causes these abnormalities is named GLI3 and is situated on chromosome 7.

This is an autosomal main disease, which means that a child will grow the condition if he or she inherits one copy of a tainted gene from either one of the parents. However, Greig Cephalopolysyndactyly Syndrome can also arise spontaneously and without any previous person in the family having Greig Cephalopolysyndactyly Syndrome. Several people with Greig Cephalopolysyndactyly Syndrome do not require any surgery to right the abnormalities. Surgical intervention may be required if there is an extra thumb since it may affect the hand grip.

Surgical restore of polydactyly in GCPS is performed on a possible basis; preaxial polydactyly of the thumbs is a superior priority for surgical alteration than postaxial polydactyly of the hand or polydactyly of the foot because of the significance of the thumbs for prehensile grasp. Syndactyly of the fingers is repaired if it is more than minimal. Surgical correction of the feet must be carefully considered, as cosmetic benefits and easier fitting of shoes can be outweighed by potential orthopedic complications. Cosmetic benefits and easier fitting of shoes can be outweighed by potential orthopedic complications. Seizures are treated symptomatically.