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August 1999

Parry-Romberg Syndrome

Arch Ophthalmol. 1999;117(8):1099. doi:

Miedziak et al1 report a case of Parry-Romberg syndrome and evidence of intracranial vascular malformations. They cite no previous reports of a similar association. The authors acknowledge that the cause of this syndrome is unknown, but they favor the concept that Parry-Romberg syndrome may be the result of an arrested angiogenic process affecting the central nervous system during growth and development. Lending some support to their concept, and yet not mentioned by them, is the occurrence of retinal telangiectasis, Coats disease, and exudative neuroretinopathy in some patients with Parry-Romberg syndrome.2-4 None of the 4 cases reported with retinal and optic nerve involvement had clinical evidence of intracranial vascular malformations. Only 1 of the 4 cases, however, was studied with modern scanning techniques (computed tomography). The authors' use of the term "malformation" implies a focal dysgenesis of the vascular system of the central nervous system, and yet the vascular changes in the brain and eye may be acquired secondary to some other pathological process underlying the Parry-Romberg syndrome.

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