TY - JOUR
T1 - Anophthalmia in the Focal Dermal Hypoplasia Syndrome
AU - Marcus, Dennis M.
AU - Shore, John W.
AU - Albert, Daniel M.
PY - 1990/1
Y1 - 1990/1
N2 - We examined an orbital exenteration specimen from an anophthalmic patient with focal dermal hypoplasia. Eyelid angiofibromas were evident and immunoperoxidase studies for human papilloma virus were negative. Orbital tissue contained a ductal cyst, chronic inflammation of the lacrimal duct and sac, rudimentary conjunctival fornices, lacrimal gland, striated muscle, and adipose tissue. Microscopic examination revealed a posteriorly located cystic structure with uveal and lens remnants. Neuroectodermal structures consistent with retina, optic nerve, or meninges were not observed, thus representing true anophthalmia. These findings remain as the only histopathologic description of ocular tissues in patients with focal dermal hypoplasia.
AB - We examined an orbital exenteration specimen from an anophthalmic patient with focal dermal hypoplasia. Eyelid angiofibromas were evident and immunoperoxidase studies for human papilloma virus were negative. Orbital tissue contained a ductal cyst, chronic inflammation of the lacrimal duct and sac, rudimentary conjunctival fornices, lacrimal gland, striated muscle, and adipose tissue. Microscopic examination revealed a posteriorly located cystic structure with uveal and lens remnants. Neuroectodermal structures consistent with retina, optic nerve, or meninges were not observed, thus representing true anophthalmia. These findings remain as the only histopathologic description of ocular tissues in patients with focal dermal hypoplasia.
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U2 - 10.1001/archopht.1990.01070030102038
DO - 10.1001/archopht.1990.01070030102038
M3 - Article
C2 - 2404489
AN - SCOPUS:0025038065
SN - 0003-9950
VL - 108
SP - 96
EP - 100
JO - Archives of ophthalmology
JF - Archives of ophthalmology
IS - 1
ER -