Cerebral Thromboembolism Due to Antithrombin III Deficiency in Two Children

Abstract

Despite numerous well-described causes of stroke in infancy and childhood, a significant proportion remains unexplained. Venous thromboembolism is a common complication in adult patients undergoing surgery, and after severe trauma, but not in otherwise healthy children less than 10 years old. However, it may also occur spontaneously without recognizable cause. It has been known for a long time that some patients are particularly prone to venous thrombosis and in recent years great efforts have been made to identify the risk factors. The attention of haematologists has been focused on the possibility that certain abnormalities of coagulation may be associated with a tendency to thrombosis, but only in a few instances a clear causal relationship has been established. One such example is a deficiency of antithrombine III, but such a deficiency has hitherto been recognized as a cause for thrombosis in children only in very particular circumstances. We present two young children with stroke of which one was purely ischemic and the other ischemic with secondary hemorrhage. Both our patients showed an AT III deficiency. Patient one also had a cyanotic congenital heart disease with right to left shunting which made cerebral embolism originating from a thrombus in the iliac vein possible to occur. We consider her hematocrit values too low to be a predisposing factor for this thrombosis. AT III deficiency may be caused by several different mechanisms. Either it exists as a congenital (hereditary) or as an acquired disorder. In patient two the family history was positive for hereditary AT III deficiency.(ABSTRACT TRUNCATED AT 250 WORDS)

Hereditary and Acquired Risk Factors for Childhood Stroke

“…There are reports of arterial strokes in children (mainly neonates) linked to low plasma concentrations of physiologic anticoagulants (8,9,10,22,29,60). However, when age-appropriate controls are used, it becomes apparent that, in most, if not all, of these children, values are normal for age (3).…”

Cerebral venous thrombosis in a child with iron deficiency anemia and thrombocytosis.

“…15 - 21 The former includes deficiencies of the physiologic inhibitors of coagulation, proteins C and S, plasminogen, antithrombin m , and the lupus anticoagulant Of these, antithrombin III deficiency (inherited or acquired) is increasingly recognized as a risk factor for stroke in children. 18 - 19 Thrombotic tendencies may also occur in children with primary thrombocytosis (myeloproliferative disorders). 15 A review of the literature, however, suggests that strokes are uncommon in children with these disorders; only five cases have been reported.…”

An unusual cause of cerebral venous thrombosis in a four-year-old child.

“…1 -4 Central thrombosis has also been described in various hypercoagulable states associated with nephrotic syndrome, acute lymphoblastic leukemia, and congenital antithrombin HI deficiency. [5][6][7][8] We report the case of a 4-year-old boy with cerebral venous thrombosis after a trivial head injury; he had none of the previously reported associated conditions. The search for a factor to explain this clinical event led to the suggestion that protein S deficiency may also be associated with childhood superior sagittal sinus thrombosis.…”