ANTERIOR PERSISTENT HYPERPLASTIC PRIMARY VITREOUS AND ATYPICAL IRIS COLOBOMA A CASE REPORT
C. Klais, K. Vanselow, M. Hacker, A. Zubcov, N. Stärk
Background: Persistent hyperplastic primary vitreous (PHPV) refers to a spectrum of abnormalities. The anterior type is rare. We present a child with atypical anterior PHPV and atypical iris coloboma.
Case report: A 3-year-old girl was admitted to our department because of a newly diagnosted lens opacification of the right eye. Since birth a horizontal nasal iris colomboma was known on the same eye. At the initial examination an atypical iris colomboma between 2.30h and 5.30h was observed on her right eye. Moreover, there was a transparent fibrovascular tissue adherent to the anterior lens capsule. This tissue extended laterally to the iris stroma at the border of the colomboma. The central lens was clear. This fibrovascular membrane was a result of an atypically incomplete regression of the tunica vasculosa lentis. In ultrasound a microphthalmos was verified. Funduscopy showed no abnormalities. Examination of the fellow eye was unremarkable. Visual acuity was 3/60 on the right eye and 6/12 Sheridan-Gardiner-test (SGT) on the leff eye. There was no binocular function. Retinoscopy was performed (OD -1,0 ± 7,0/90º, OS -1,75 ± 1,75/110º). With appropriate correction and therapy of amblyopia visual acuity improved after three months (OD 6/24 SGT, OS 6/6 SGT).
Conclusion: A PHPV patient whose diagnosis is only established at age 3 may receive surgical or conservative therapy. If conservative treatment is chosen with regard to the lack of binocular function therapy of amblyopia and appropriate correction may provide good functional results.
Universitäts-Augenklinik, Theodor Stern Kai 7, 60590 Frankfurt/Main