Antenatal thrombosis of torcular herophili presenting with anemia, consumption coagulopathy and high-output cardiac failure in a preterm infant

Abstract

Dural sinus malformation occasionally results in the development of giant venous lakes involving torcular herophili. Such dilatation can result in the formation of a massive venous thrombus leading to ventriculomegaly/ hydrocephalus. Although, majority of patients have an unremarkable clinical course owing to spontaneous resolution of the thrombus, significant morbidity and mortality has been documented. We report the first case of torcular thrombosis in a preterm infant who survived severe anemia, consumption coagulopathy and high-output cardiac failure during the neonatal period and had a normal neurological outcome.

Thrombosed Dural Sinus Malformation in a Fetus: A Case Report

“…In addition, there was no sign of increased intracranial pressure, and cranial circumference decreased to within the normal range soon after birth. We believe that the increased cranial circumference in the present patient may be a result of thrombosis, since other reports have described a persistent cranial enlargement even after the regression of thrombosis . Five patients who exhibited macrocephaly have been previously reported ,.…”

“…Clinical data on these patients are summarized in Table 1. [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15] The clinical course of live birth cases with thrombosed DSM is variable, ranging from death and neurological complications to normal neural development. However, many patients have a favorable outcome, as indicated in Table 1.…”

“…We believe that the increased cranial circumference in the present patient may be a result of thrombosis, since other reports have described a persistent cranial enlargement even after the regression of thrombosis. 15 Five patients who exhibited macrocephaly have been previously reported. 1,7,8,15 Two patients with AVF died due to cardiac decompensation and respiratory failure, and 2 other cases with a bulging fontanelle experienced mild hemiparesis.…”

“…15 Five patients who exhibited macrocephaly have been previously reported. 1,7,8,15 Two patients with AVF died due to cardiac decompensation and respiratory failure, and 2 other cases with a bulging fontanelle experienced mild hemiparesis. 7 These findings, together with those of the patient case who had favorable prognosis, suggest that macrocephaly alone may not be a negative prognostic factor.…”

Prenatal Diagnosis of Torcular Herophili Thrombosis

“…Sonographically, it appears as an anechoic triangular or elongated structure inferior to the occipital bones and posterior to the cerebellum, just inside the cranial vault 2 . Ectasia of the torcular herophili is a variant of a dural sinus malformation, which presents as a huge venous lake with very slow flow communication with the other sinuses and the cerebral veins 3 , 4 . A dural sinus malformation is a type of congenital dural arteriovenous fistula and is classified into 2 groups: midline with giant pouches and lateral involving the jugular bulb 5 .…”