Wydanie 3/2010
str. 91

Wrodzona dysgenezja mezenchymalna przedniego odcinka gałki ocznej

Congenital Anterior Segment Mesenchymal Dysgenesis – Case Report

Monika Modrzejewska, Ewelina Lachowicz, Danuta Karczewicz

Katedra i Klinika Okulistyki Pomorskiego Uniwersytetu Medycznego w Szczecinie
Samodzielny Publiczny Szpital Kliniczny nr 2 w Szczecinie
Kierownik: prof. dr hab. n. med. Danuta Karczewicz


Summary: In this article we present rare case of congenital anterior segment mesenchymal dysgenesis. Blepharophimosis and symblepharon, binocular blepharoptosis, microphthalmia, microcornea, sclerocornea, anterior corneal staphyloma, iris hipoplasia with partial defect, anterior chamber deepening were characteristic for this anomaly. Intraocular pressure values remained within normal ranges. Analysis of genetic, familial, infectious, traumatic, metabolic and toxic risk factors, including medicines intake, in perinatal period were not confirmed to be contributing in this pathology development. Congenital polydysplasia including central nervous system (aplasia and agenesia of the corpus callosum and ventricles pathology), organic heart disease (Pfo), dermal defect (telangiectasis), neurological symptoms (hypotony) and dysmorphic disorders (hypodontia, microdontia, maxillary bone hipoplasia, wide and flat root of the nose, telecanthus and hypertelorism) were confirmed in diagnostic investigation (X-Ray, MRI, US, EMG, EEG). The follow-up ophthalmological examination carried out in 4th year of the child’s age revealed useful visual acuity of the right eye and amblyopia of the left eye, despite advanced anomaly of the anterior segment.
This case suggests that ophthalmological anomaly in form of iridocorneal dysgenesis and other forms of polydysplasia requires continuous multispecialistic care.

Słowa kluczowe: dysgenezja rogówkowo tęczówkowa, wrodzone wady przedniego odcinka gałki ocznej, diagnostyka różnicowa.

Keywords: iridocorneal dysgenesis, congenital anterior segment abnormalities, differential diagnosis.


powrót

REDAKCJA NIE UDZIELA PORAD MEDYCZNYCH I NIE POŚREDNICZY W KONSULTACJACH PACJENTÓW Z LEKARZAMI