Recurrent vitreous haemorrhage and epidural haematoma in a child with hypofibrinogenaemia

Abstract

SummaryA 14-month-old male infant was brought by parents for redness of the right eye of 18 days duration. Exam and B-scan ultrasonography revealed total hyphema, dense vitreous haemorrhage and lens subluxation in the right eye while CT disclosed right small epidural haematoma. The left eye had neither retinal haemorrhage nor disc oedema. There was no sign of shaken baby syndrome. Fibrinogen level in the blood was very low. The parents are first-degree cousins with two family members having hypofibrinogenaemia. Vitreous haemorrhage recurred after surgical intervention resulting in phthisis and loss of vision. Hypofibrinogenaemia needs to be included in the differential diagnosis of ocular haemorrhage and vision loss. BACKGROUND

Spontaneous intracranial extradural hematoma secondary to hypofibrinogenemia: A rare case report

“…It is inherited through an autosomal recessive trait, and parental consanguinity is common among affected females. [10] According to a study conducted by Peyvandi et al , there were no cases of CNS bleeding reported in patients with mild fibrinogen deficiency (fibrinogen levels of 10 mg/dl or more) who usually presented with menorrhagia, hemarthrosis, muscle hematoma, GI bleeds, and epistaxis. [11] Prolonged umbilical stump bleeding at birth is highly suggestive of such bleeding disorders.…”

Vitreous hemorrhage – Causes, diagnosis, and management

“…Hematological disorders such as idiopathic thrombocytopenic purpura, hypo-fibrinogenemia, disseminated intra-vascular coagulation, von Willebrands syndrome, and hemophilia may present as VH, which may be bilateral and massive in some cases. [ 28 29 30 31 32 ] Characteristically, the hematological disorders cause intra-retinal hemorrhages and vascular occlusion but may rarely present as VH. Vitreous veils in the setting of juvenile X-linked retinoschisis is also an important cause of VH.…”