Limb scalp and skull defects
Overview
Adams Oliver syndrome is a rare disorder identified in 1945 by Dr F H Adams and Dr C P Oliver. The syndrome is characterised by congenital scalp and skull defects. These defects can range from mild, with some skin and hair defect, through to severe when there may be defects of the underlying bone.
Symptoms
The syndrome is characterised by congenital scalp and skull defects. These defects can range from mild, with some skin and hair defect, through to severe when there may be defects of the underlying bone. Rarely, bleeding occurs from dilated veins that are sometimes associated with severe scalp defects. Other features that may occur include eye anomalies, accessory nipples cutis marmorata telangiectatica congenita, haemangiomas, skin tags and woolly hair. Intellectual development is generally within normal limits, even in individuals with large cranial bone defects, although learning disabilities have been reported. Limb defects, ranging from small fingers and toes through to severe involvement of long bones, can also occur. In very severe cases, the lower limb can be absent below mid-calf. However, it is common for people with the affected gene to have minimal scalp or limb abnormality.