J Korean Ophthalmol Soc.  1991 Nov;32(11):1009-1014.

Microphthalmia with Orbital Cyst: Case Presentation

• Cho HK
• Moon YS
• Hong NS

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.

Keyword

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

MeSH Terms

Anterior Chamber
Cornea
Embryonic Structures
Humans
Iris
Microphthalmos*
Orbit*
Parturition
Retinaldehyde
Retinaldehyde