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).