Retinopathy Revealing Cerebral Venous Thrombosis in Sickle Cell Disease in Niger

Nouhou Diori Adam,Saley Ali,Saley Idrissa,Amadou Moussa Salia,Yacoubou Soumana

doi:10.4236/ojoph.2019.93014

Abstract

Cerebral venous thrombosis (CVT) in sickle cell disease has been rarely described in the literature. Some authors consider sickle cell disease as a risk factor for CVT. We report the case of a 20-year-old boy, known as sickle cell, followed at the National Reference Center for Sickle Cell Disease in Niger. Admitted in consultation for a decrease of acuity with the right eye with perception of a black spot evolving since about three (3) days, ophthalmological examination revealed sickle cell retinopathy associated with cerebral venous thrombosis on MRI. The rest of the balance showed normochromic anemia, ionic disturbance and a D-dimer at 1500 μg/l. Rehydration, strict rest, antibiotic therapy and analgesics were started with an internal medicine opinion for further treatment. There was an improvement in visual acuity of 6/10 in 48 hours with disappearance of black spot perception, after three weeks, an ad integrum recovery of retinal lesions to control imaging. Despite the ocular complications of sickle cell disease involving visual prognosis, cerebral venous thrombosis is another complication to be investigated in any sickle cell retinopathy.

Highlights

Sickle cell disease is the most common hereditary genetic pathology in the world
Cerebral venous thrombosis (CVT) in sickle cell disease has been rarely described in the literature
We report the case of a 20-year-old boy, known as sickle cell, followed at the National Reference Center for Sickle Cell Disease in Niger

Summary

Introduction

Sickle cell disease is the most common hereditary genetic pathology in the world. The gene frequency of the disease is 5% to 20% in Africa and 40% in Central Africa [1]. It is a real public health problem in Niger. A cerebral venous thrombosis is a rare form of cerebral attack in sickle cell patients which is the most difficult diagnosis [6]. We report the case of cerebral venous thrombosis in an SS sickle cell with retinopathy, which has the particularity of the severity of both visual and vital prognosis

Observation

Discussion

Conclusion