CT of cerebral venous sinus thrombosis in a child with homocystinuria

Abstract

We describe a case of cerebral venous sinus thrombosis in a child with homocystinuria. We present both the classic CT findings of cerebral sinus thrombosis, and also the first report of the CT visualization of transdural venous collateral circulation.

Cerebral venous sinus thrombosis in infancy and childhood: role of genetic and acquired risk factors of thrombophilia

“…It has been commonly associated with underlying systemic conditions such as dehydration, sepsis, malignancy [26,31] or trauma [22,46], and occurs in rare cases of systemic lupus erythematosus [48], homocystinuria [12,40], or Behcet disease. In addition, local dural sinus thrombosis is a known complication of subclavian or jugular vein catheterisation [17,45].…”

“…In addition, the following factors considered to be``trigger factors'' for thrombosis were de®ned [12,17,22,27,31,40,45,46,48]: severe bacterial or viral infection with CNS involvement (meningitis, encephalitis, orbital infection, otitis), cancer and polychemotherapy, auto-immune diseases, renal diseases (haemolytic-uraemic syndrome, nephrotic syndrome), peripartal asphyxia, dehydration, nicotine abuse, central lines placed in the internal jugular or subclavian vein, prothrombotic medication (steroids, oral contraceptives) and vascular malformations.…”

Epidemiology of Cerebral Vein and Sinus Thrombosis

“…During pregnancy or puerperium, a significant increase in the risk of cerebral venous thrombosis was associated with caesarian delivery (OR 3.1), increasing maternal age (OR 2.5), presence of comorbidities including hyperhemesis (OR 14.2), intercurrent infections (OR 3.45) and maternal hypertension [18] Neurosurgery [19] Meningitides [20] Arterovenous malformation [21] Sepsis (sinusitis, mastoiditis, cellulitis) [22,23] Stroke and hemorrhage Space-occupying lesions Jugular catheterization Systemic Dehydration (diabetic ketoacidosis) Septicemia [24] Pregnancy and puerperium [5] Inflammatory bowel disease [25] Malignancy [26] Sarcoidosis [27] Collagen disease (Behcet's syndrome, SLE, Sjögren's syndrome) [28][29][30] Homocysteinuria [31] Nephrotic syndrome [32] Autoimmune thyroiditis Drugs…”

Homocystinuria Presenting as Sagittal Sinus Thrombosis

“…Angiography may be dangerous in such patients, since thrombosis may be precipitated by intravenous con trast agents in the presence of homocystinuria. Accord ingly, homocystinuria should be considered in the differ ential diagnosis of venous or arterial thrombosis regard less of the age of the patient and even in the absence of other features of homocystinuria [1][2][3][7][8][9][10][11][12][13][14][15][16][17],…”

“…He is also unique in being pyridoxine unresponsive without mental handicap, lens dislocation, marfanoid habitus, or osteoporosis -all usually seen in pyridoxine-unresponsive patients [2], Thromboembolic disease is said to be infrequent in patients with homozygous homocystinuria before the age of 20 years [2], We speculate that a number of patients with thromboses and atypical homocystinuria may be misdiagnosed as idiopathic thrombosis [2,4,5,9,12,13] or as other neurologic disease such as multiple sclerosis [14]. Angiography may be dangerous in such patients, since thrombosis may be precipitated by intravenous con trast agents in the presence of homocystinuria.…”