Microphthalmia with Orbital Cyst: Case Presentation.

Cho, Ho Kyun; Moon, Yeon Sung; Hong, Nae Sun

Original Article

Journal of the Korean Ophthalmological Society 1991;32(11):1009-1014.
Published online November 1, 1991.

Microphthalmia with Orbital Cyst: Case Presentation.

Ho Kyun Cho, Yeon Sung Moon, Nae Sun Hong

Department of Ophthalmology, Chung-Ang University Hospital, Seoul, Korea.

낭종을 가진 소안구증 ( 증례보고 )

홍래선(Nae Sun Hong),조호균(Ho Kyun Cho),문연성(Yeon Sung Moon)

Abstract

A microphthalmia with orbital cyst resulting from a failure in the involution of primary optic vesicle or defective closure of embryonic cleft is rare congenital anomaly. Clinical features are variable and mainly depend upon the time of arrest in development of the embryonic eyeball The authors have experienced a case of microphthamia with1uge orbital cyst in left orbit. The patient was 4 months of age and it was found at the time of birth. At radiologic study an area of suspicious defect in the orbital roof was shown. The orbital cyst and small eyeball were removed by intracranial approach and histopathologic examination was performed with serial section. We could find a suspicous site of defective closure of embryonic cleft and also ectopic retinal tissue in the cystic wall. A disorganized ocular tissues forming tumor like mass filled with microphthalmic eyeball. No cornea, iris or anterior chamber was found in the slide. In view of those histopathologic findings we can suspect that the defective closure of embryonic cleft occured at 7 to 14 mm embryo stage.

Key Words: Microphthalmia;Orbital cyst;Primary optic vesicle;Embryonic cleft;Ectopic retinal tissue