Nadir bir orbital patoloji: Persistan hiperplastik primer vitreus

Year 2025, Volume: 30 Issue: 1, 48 - 53, 29.01.2025

Muhammed Akif Deniz , Aydın Aslan

https://doi.org/10.21673/anadoluklin.1453554

Abstract

Amaç: Persistan hiperplastik primer vitreus (PHPV), embriyolojik primer vitreus ve hyaloid damar sisteminin gerilememesi nedeniyle ortaya çıkan konjenital bir anomalidir. Etkilenen gözlerde anatomik ve visual sonuç üzerinde önemli etkisi olabilir. Amacımız PHPV tanısı konulmuş hastaların Manyetik Rezonans Görüntüleme (MRG) özelliklerini sunmak ve literatürle karşılaştırmaktır.

Yöntemler: Çalışmamız retrospektif bir arşiv çalışması olup Ocak 2010-Ocak 2020 tarihleri arasında merkezimizde orbital MR çekimi yapılan ve görüntülemelerinde PHPV tanısı bulunan 9 hasta çalışmaya dahil edilmiştir. Hastalar yaş, cinsiyet, etkilenen orbita (sağ-sol, unilateral-bilateral), eşlik eden ek anomaliler açısından değerlendirildi.

Bulgular: Çalışmaya alınan 9 hastanın 8’i erkek 1’i kız idi. Hastaların yaş aralığı 3 ay-56 yaş olup yaş ortalaması 10,8 idi. Bilateral tutulum 2 (%22) hastada, unilateral tutulum 7 (%78) hastada (5 hastada sağ, 2 hastada sol) izlendi. Mikroftalmi 5 hastada, dismorfik orbita 2 hastada, retinal hemoraji-dekolman 3 hastada izlenmiştir. PHPV olgularına eşlik eden ek kranial patolojiler değerlendirildiğinde; 1 hastada lizensefali, 1 hastada porensefalik kavitasyon ve 2 hastada optik sinir periferinde sıvı artışı izlendi.

Sonuçlar: Sonuç olarak tek taraflı tutulum, mikroftalmi ve ön kamaranın kollabe olması PHPV ayırıcı tanısında en önemli klinik ipuçlarıdır. PHPV’den şüphelenildiğinde retrolental kitle ve huni şeklinde retina dekolmanı MR görüntülemede tanı koymada son derece önemli bulgulardır.

Keywords

Manyetik rezonans görüntüleme, orbita hastalıkları, persistan hiperplastik primer vitreus, retinal hemoraji

A rare orbital pathology: Persistent hyperplastic primary vitreous

Abstract

Aim: Persistent hyperplastic primary vitreous (PHPV) is a congenital anomaly that occurs due to failure of the embryological primary vitreous and hyaloid vascular system to regress. It may have a significant impact on the anatomical and visual outcome in the affected eyes. Our aim is to present the Magnetic Resonance Imaging (MRI) features of patients diagnosed with PHPV and compare them with the literature.

Methods: Our study is a retrospective archive study and 9 patients who underwent orbital MRI in our center between January 2010 and January 2020 and were diagnosed with PHPV on their images were included in the study. Patients were evaluated in terms of age, gender, affected orbit (right-left, unilateral-bilateral), and accompanying additional anomalies.

Results: Of the 9 patients included in the study, 8 were boys and 1 was a girl. The age range of the patients was 3 months-56 years and the average age was 10.8 years. Bilateral involvement was observed in 2 (22%) patients, and unilateral involvement was observed in 7 (78%) patients (right in 5 patients, left in 2 patients). Microphthalmia was observed in 5 patients, dysmorphic orbita in 2 patients, and retinal hemorrhage-detachment in 3 patients. When additional cranial pathologies accompanying PHPV cases are evaluated; lissencephaly was observed in 1 patient, porencephalic cavitation in 1 patient, and increased fluid in the optic nerve periphery was observed in 2 patients.

Conclusions: As a result, unilateral involvement, microphthalmia and anterior chamber collapse are the most important clinical clues in the differential diagnosis of PHPV. When PHPV is suspected, retrolental mass and funnel-shaped retinal detachment are extremely important findings in making the diagnosis on MR imaging.

Keywords

Magnetic resonance imaging, orbital disease, persistent hyperplastic primary vitreous, retinal hemorrhage

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