Figure Legend
Figure 1: Prevalence of NIH diagnostic signs (criteria) among
patients presenting to ophthalmology clinic with Neurofibromatosis in a
black African population
Figure 2: Distribution of plexiform neurofibromas in patients presenting
with Neurofibromatosis in a black African population
Figure 3: Pigmentation in black African patients with NF1.
a-b : Café sans laît (CSLM) macules some of which are almost
imperceptible (black arrow) against the dark skinned complexion.
c : Café au laît macules on the buttocks of 10 year old boy with
NF1. A hypopigmented macule (laît) is indicated in this photograph,
using double black arrows.
d : Hyperpigmented, somewhat hypertrophic, CALMs on the scalp of
a 10-year-old boy with NF1. The scalp is an unusual location for CALMs
in Caucasians and is usually spared (Friedmann, J.M., 2002)4
Figure 4- Hematoxylin and eosin stained sections- Histopathological
findings of a typical plexiform neurofibroma from an eyelid nodule in an
11 year old female
(X40) Skin with enlarged dermal nerves, containing large nerve
bundles.
B and C. (X100) Irregularly contoured and enlarged nerves.
D. (X400) Enlarged nerve with a cellular matrix containing fibroblasts,
Schwann cells, collagen and mucin in the involved nerve.
Figure 5: a. Plexiform neurofibroma of the nose in a 12-year-old male.
b. Orbito-cranial plexiform neurofibroma causing proptosis in a
15-year-old female (below) notice also the plexiform neurofibroma of the
left pinna and hyperpigmented macules.
c,d : Pseudoarthrosis and deformity of interphalangeal joint of
5th digit (digiti minimi quintis), of 15-year-old
female with NF 1. Note: notice also unusual freckling (hyperpigmented
macules) on both palms , this is also an rare site for CALMs in
Caucasians (Friedmann, J.M., 2002)
e . Bone dysplasia causing lateral bowing of the tibia (above)
notice the cutaneous neurofibroma on the contralateral leg f .
Absence of the left lesser wing of the sphenoid (below) in the same
15-year-old female with NF1
Figure 6: a: Lisch nodules in the iris appearing as multiple raised
flat-topped yellow spots in 12-year old female (seen without slit lamp)
b: Lisch nodules seen with magnification, using slit lamp in a 45-year
old woman, first degree relative. Notice the irregular but well
circumscribed borders, this woman also has a nasal pterygium.
Figure 6 c,d: Fundus photograph showing bilateral optic atrophy in a
17-year-old male presenting with clinical features of neurofibromatosis
1, Brain MRI (Figure 9) showed bilateral optic-hypothalamic pathway
glioma
Figure 6 e,f: Optic-hypothalamic pathway glioma in the 17 year old male
presenting with neurofibromatosis and bilateral optic atrophy
Note : Abnormal hyperintensity and expansion of the optic tracts
(6e ) and enlargement of the optic chiasm (6f ), worse
on the left, are shown on low-field magnetic resonance imaging (0.4
Tesla) of the brain.
Table 1: Neuro-imaging diagnoses of patients with
neurofibromatosis type1, showing recommendations for multidisciplinary
management.