Purpose: 
To report a case of asymmetric, non-hereditary pigmented paravenous retinochoroidal atrophy (PPRCA) in an 8-month-old female.

Study Design: 
Observational case report and literature review.

Methods: 
The patient’s clinical records were reviewed including history, clinical examinations, multimodal imaging and investigations. A thorough review of the literature was performed.

Results: 
An 8-month-old female of Caucasian and Asian ethnicity was referred for acute onset left exotropia (XT) first noticed at 7.5 months of age. Past medical history was unremarkable and family history was non-contributory. Initial examination revealed unmaintained fixation OS, visual acuity 20/63 OD, 20/130 OS and 15PD left XT at distance, progressing to 25PD over 5 months. Hypermetropic anisometropia (+1.50OD, +3.50OS) and strong objection to occlusion OD was noted. Macular chorioretinal scar was discovered OS on fundus examination. A thorough infectious workup revealed non-reactive TORCH titers and negative serology for Syphilis, Bartonella, Lyme, Histoplasmosis and Tuberculosis. CRB1 gene mutation testing is pending. Incidental finding of microcytic anemia resulted in a referral to pediatric hematology and a diagnosis of iron deficiency anemia  (presumed to be diet related) and likely thalassemia trait. An examination under anaesthesia confirmed macular chorioretinal scar with peripheral pigmentation and pigmented retinochoroidal atrophy in a perivenous distribution OS. Fluorescein angiography (IVFA) showed window defect corresponding to atrophic areas with staining along the borders OU. Window defects corresponding to the atrophic macular scar was also noted with no leakage. Fundoscopic examination, including IFVA, of the patient’s parents was normal. 

Conclusions: 
PPRCA is a rare, often bilateral disorder, uncommon in females, which is not well understood or characterized. Although its etiology remains unknown, reports have suggested possible post-inflammatory etiologies including tuberculosis, rubeola and syphilis.  Mutations of CRB1 gene have also been implicated in its pathogenesis. To the best of our knowledge, this is the first case of PPRCA associated with thalassemia trait and only one of three cases of non-hereditary PPRCA in an infant. Further, this is the youngest reported PPRCA case with colour fundus photographs and angiography.

Pigmented paravenous retinochoroidal atrophy in an infant with thalassemia trait: A case report and literature review

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