Pediatric Reversible Cerebral Vasoconstriction Syndrome: Two Unique Cases with a Review of all Reported Children

Abstract

Reversible cerebral vasoconstriction syndrome (RCVS) has been well described in adults, but pediatric cases are yet under recognized. We describe two children with RCVS and review similar already published pediatric cases. The first patient was a 10-year-old girl who presented with severe headaches and seizures 3 days after blood transfusion. Brain magnetic resonance imaging (MRI) showed changes compatible with posterior reversible encephalopathy syndrome and subarachnoid hemorrhage. Magnetic resonance angiogram showed diffuse vasoconstriction of multiple cerebral arteries. The second patient was a 9-year-old boy who presented with severe thunderclap headaches. Brain MRI showed isolated intraventricular hemorrhage. Computed tomography/MR angiogram and digital subtraction angiogram were normal. A week later, he developed focal neurological deficits. Repeated MR angiogram showed diffuse vasospasm of multiple intracranial arteries. Both children recovered completely. A clinico-radiological review of previously reported childhood RCVS is provided.

Reversible Cerebral Vasoconstriction Syndrome with Intracerebral Hemorrhage in a Thalassemic Child—An Extremely Rare Complication

“…This has been attributed to higher androgen levels in peripubertal males. 7 Hypertension, which was reported in 55% of children, 4 was not seen in our case; 85% of children had identifiable triggers. The maximum number of cases were triggered by drugs (41%), followed by exercise, including swimming (30%), which is similar to adults ( Tables 1 and 2 ).…”

“…►Table 2 summarizes the differences between pediatric and adult RCVS cases. [4][5][6] Unlike in adults, there is male preponderance (1.6:1) among the pediatric cases (►Tables 1 and 2). This has been attributed to higher androgen levels in peripubertal males.…”

“…Apart from vasoconstrictor drugs (topical oxymetazoline and phenylephrine, eletriptan, and epinephrine), antihistamines, steroid inhaler, sertraline, immunomodulators such as cyclosporine (in aplastic anemia), mycophenolate, rituximab, and cyclophosphamide (in three systemic lupus erythematosus patients) and chemotherapeutic drugs such as intrathecal cytarabine and pegylated L-asparaginase (used in acute lymphoblastic leukemia) were also followed by the development of RCVS. 4 8 9 The most recent article describes two children with coronavirus disease 2019-associated multisystem inflammatory syndrome who developed RCVS with multifocal ischemic changes. 5 Although well described in adults, blood transfusion triggering RCVS has hitherto been described only in two children.…”

“…Higher volume and faster transfusion are associated with a shorter latency between the breakdown of cerebrovascular autoregulation and symptom onset. 4 11 Thus, maintaining a higher pretransfusional hemoglobin level prevents significant posttransfusional changes in blood viscosity and reduces the risk of RCVS.…”

“…[1][2][3] In the written literature from 2006 till date, there are 29 reported cases of pediatric RCVS. 4 To the best of the authors' knowledge, the index case is the first reported case of RCVS in a thalassemic child.…”