Palpebral ecchymosis and cerebral venous thrombosis in a near term infant

Cerebral venous thrombosis (CVT) is rarely diagnosed in newborn babies.¹ Seizures, haemorrhagic venous infarcts, and, in particular, intraventricular haemorrhage are the most common signs of CVT in term newborns.² Little is known about the neuroradiological and clinical presenting findings of CVT in preterm babies.

We observed bilateral palpebral ecchymosis in a 35 week gestation preterm baby (fig 1A) with major bleeding in the posterior fossa on an ultrasound brain scan (first day of life). Computed tomography imaging on the second day confirmed the haemorrhage and showed an unexpected venous thrombosis (“empty delta sign”, a triangle of decreased density caused by the contrast enhanced blood flowing around the clot) of the torcular Herophili (fig 1B). The haematoma was surgically drained and an intraventricular reservoir was inserted to treat the acute obstructive hydrocephalus.

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Figure 1

 (A) Bilateral palpebral ecchymosis. (B) Computed tomography scan showing venous thrombosis (“empty delta sign” of torcular Herophili), posterior fossa bleeding, and ventriculomegaly.

Factor V Leiden, Factor II, and MTHFR mutations were negative; motor and cognitive impairments were observed at 1 year of age.

The association between palpebral ecchymosis and CVT is intriguing, as palpebral veins empty, throughout the ophthalmic vein, into the sinus cavernous and thereafter into the transverse sinus. A clot in the major cerebral veins is likely to cause increased venous pressure predisposing to major or minor bleeding similarly to those affecting palpebrae.

Spontaneous palpebral ecchymosis is an extremely rare finding which can be associated with CVT, as recently observed in an adult patient.³