Prenatal Diagnosis of Torcular Herophili Thrombosis

Abstract

Prenatally diagnosed thrombosis of the torcular herophili is very rare, and it is sometimes misdisgnosed due to unfamiliarity. Sonography with color Doppler imaging is the key imaging modality for prenatal diagnosis of torcular herophili thrombosis. Typical prenatal sonographic findings include a well-defined triangular anechoic collection in the occipital region and an echogenic structure within the collection, which represents the thrombus. Fetal magnetic resonance imaging is usually used as an adjunctive modality for prenatal diagnosis, as it confirms the diagnosis by providing more precise anatomic information and better characterization of the lesion. We present 2 cases of thrombosis of an ectatic torcular herophili with serial sonographic and magnetic resonance imaging examinations, as well as a review of the literature regarding the prenatal diagnosis of torcular herophili thrombosis.

Thrombosis of the torcular herophili in the fetus: a series of eight cases

“…Although no data on the prevalence of this condition is available, thrombosis of the torcular herophili is the most frequently diagnosed cerebral thrombotic event during fetal life. [6][7][8][9] As demonstrated by this report and other reviews available in the literature, [6][7][8][9] this condition seems to have a benign antenatal course in a significant number of cases. It presents with a variable sonographic pattern depending on the particular stage of evolution of the blood clot, frequently undergoes partial or complete spontaneous resolution prior to delivery, and does not appear to be associated with chromosomal defects or environmental exposures.…”

“…It presents with a variable sonographic pattern depending on the particular stage of evolution of the blood clot, frequently undergoes partial or complete spontaneous resolution prior to delivery, and does not appear to be associated with chromosomal defects or environmental exposures. 6,9 However, the lesion may cause ischemic brain damage due to cerebral hypoperfusion caused by retrograde venous hypertension and compression by the mass effect, which may be associated with a worse long-term neurodevelopmental outcome. Differential diagnoses that should be considered include intracranial tumors of the posterior fossa such as teratoma or lipoma, intracranial bleeding affecting the cerebellum or occipital lobes, and thrombosis of a vein of Galen malformation.…”

“…3 In the vast majority of the reported prenatal cases, a relation with environmental exposures or thrombophilic conditions such as antiphospholipid syndrome has not been demonstrated. [6][7][8][9] Therefore, a vascular anatomical malformation affecting the drainage into the torcular herophili seems to be the most common condition associated with these cases. Indeed, dural sinus dilatation involving the torcular herophili is almost invariably present.…”

“…4,5 However, dural sinus thrombosis in the fetus is a rare cerebrovascular condition invariably affecting the torcular herophili, with fewer than 50 cases reported in the English literature. [6][7][8][9] In these cases, the most striking findings include the presence of a large intracranial thrombus within a distended torcular herophili. Despite this very dramatic sonographic presentation, it can be associated with spontaneous resolution with good perinatal outcome when the thrombosis is an isolated event.…”

“…Despite this very dramatic sonographic presentation, it can be associated with spontaneous resolution with good perinatal outcome when the thrombosis is an isolated event. However, although several authors have recommended expectant management throughout pregnancy and in the neonatal period, [6][7][8][9][10][11][12] the pregnancy termination rate still remains high. 9 In view of the limited information available on this condition, we aimed to describe the prenatal and postnatal features in eight cases of thrombosis of the torcular herophili managed by the authors in a period of 5 years.…”