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Case Reports
. 2015 Oct 9;10(4):61-4.
doi: 10.1016/j.radcr.2015.08.003. eCollection 2015 Dec.

Visual and otologic manifestation of Camurati-Engelmann's disease: a case report

Tariq Alam  1 , Muhammad Khurram  2 , Hidayatullah Hamidi  1 , Asif Alam Khan  3
Affiliations
Case Reports

Visual and otologic manifestation of Camurati-Engelmann's disease: a case report

Tariq Alam et al. Radiol Case Rep. .

Abstract

Camurati-Engelmann's disease (CED) is a rare disorder worldwide with just over 200 cases reported. No case of CED has been reported in Afghanistan till date. Most patients of CED (also known as progressive diaphyseal dysplasia and oeteopathica hyperostotica multiplex infantalis) present with extremity pain, muscle weakness, and waddling gait. It tends to be bilateral and symmetrical and can affect any bone but has greater affinity for long bones e.g., humerus, femur, tibia, ulna, and radius. Other common sites include skull and pelvis. Symptomatology relating to cranial nerve impingement is secondary to amorphous increase in the density of skull bones resulting in stenosis of various foramina/spaces within skull.

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Figures

Fig. 1
Fig. 1
T2-weighted coronal image of the brain revealed marked hyperostosis of the entire calvarium (arrow), temporal bone (arrow), and visualized cervical vertebra (arrow).
Fig. 2
Fig. 2
T2-weighted axial image of the brain showing marked narrowing of bilateral optic canals (arrows) due to excessive hyperostosis.
Fig. 3
Fig. 3
Radiograph of right femur anterioposterior view revealed diffuse diaphyseal hyperostosis.
See this image and copyright information in PMC

References

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