Neurofibromas
The typical plexiform (84.8%), cutaneous and subcutaneous (71.4%) neurofibromas were the second and third most prevalent systemic manifestations observed. These are the most striking clinical manifestations of the disorder and are more noticeable than the CALMs. The distribution of plexiform neurofibromas is shown in Figure 3. The commonest site of occurrence for plexiform neurofibroma was in the craniofacial region, affecting the lids in half of all cases and other parts of the head and neck in another 25%. Figure 4 shows typical histopathological finding of plexiform neurofibroma from the eyelid of an 11 year old female. The forearms and upper limbs were the least common locations (3%). A plexiform neurofibroma of the nose is shown in Figure 5a and proptosis from an orbitocranial neurofibroma in Figure 5b. There was no significant difference in prevalence of plexiform neurofibromas with age (p= 0.05) or sex (p= 0.79).