Cerebral infarction following intracranial hemorrhage in pediatric Moyamoya disease - A case report and brief review of literature.

Moyamoya disease is a clinical entity characterized by progressive cerebrovascular occlusion with spontaneous development of a collateral vascular network called Moyamoya vessels. This disease mainly manifests as cerebral ischemia. Intracranial bleeding is another major presentation of patients with Moyamoya disease. We report here a 12-year-old male child who presented with severe headache, vomiting and meningismus. Initial neuroimaging study with noncontrast computed tomography scan revealed fresh intraventricular hemorrhage in right-sided lateral ventricle. Magnetic resonance imaging with angiography of brain was done 5 days later when the child developed right-sided hemiparesis, and the diagnosis of Moyamoya disease was confirmed along with lacunar infarction of right posterior peri and paraventricular area and in the left paraventricular area and centrum semiovale. Simultaneous presence of cerebral infarction along with intraventricular hemorrhage in adult with bleeding-type Moyamoya disease is reported in literature, but it is a rare entity in a child.

Introduction

Moyamoya disease is a chronic occlusive cerebrovascular disease characterized by bilateral stenosis or occlusion at the terminal portion of the internal carotid artery (ICA), at the proximal portion of the anterior (ACA) and middle cerebral arteries (MCA) and abnormal net-like vessels at the base of the brain.[1] The idiopathic form was first reported in 1957 in Japan,[2] where the incidence is believed to be the highest. Moyamoya disease is a known cause of transient ischemic attack (TIAs), cerebral infarction, and intracranial hemorrhage including intracerebral hemorrhage (ICH), intraventricular hemorrhage (IVH), and subarachnoid hemorrhage (SAH).[1] ICH is common in adult patients with Moyamoya disease, but it is an infrequent entity in the pediatric age group.[3] The risk of ischemic complications in patients with bleeding-type Moyamoya disease is poorly understood with a few cases reported, mainly in adults.[4] But we are presenting a case of post-ICH cerebral infarction which is a rare entity in children with Moyamoya disease.

Case Report

A 12-year-old male child got admitted in our hospital with severe headache and projectile vomiting for 15 days. He did not have any history of fever, altered sensorium, suggestive history of focal neurological deficit or cranial nerve involvement. He did not have any significant past and family history. General physical examination did not reveal any abnormality like neurocutaneous markers and facial dysmorphism. His blood pressure was 98 (systolic)/62 (diastolic) mmHg and pulse rate was within 90–100 beats per minute. On central nervous system examination, he was conscious and oriented and the cranial nerves and papillary examination were within normal limit. This patient did not reveal any focal neurological deficit but had meningeal signs at admission. Fundoscopy did not reveal any abnormality and central venous pressure (CVP) or intracranial pressure (ICP) could not be measured. The routine investigations did not show any abnormalities (Hb% 12 gm%; leukocyte count 9400/mm3; platelets 254,000/mm3, urea 22 mg/dl; creatinine 0.5 mg/dl; sodium 139 meq/l; potassium 4 meq/l, normal coagulation profile). Peripheral blood smear was normal and sickling test was found to be negative. Lumbar puncture was done and cerebrospinal fluid (CSF) analysis revealed the presence of red blood cells with mild elevation of protein and CSF sugar was within normal range. As he was having severe headache with meningismus, a neuroimaging was planned and the noncontrast Computed tomography (CT) scan of head [Figure 1] showed IVH of the right lateral ventricle. He was treated conservatively with intravenous bolus of isotonic electrolyte solution followed by N/2 in 5% dextrose solutions as maintenance fluid and oral glycerol was used as hyperosmolar solution to reduce the raised ICP. After admission, intake–output chart was maintained and he was passing urine adequately. Though the patient was kept nothing per oral for the first 24 hours after admission, he was advised to take orally when vomiting subsided. Magnetic Resonance (MR) imaging with angiography of brain, which was done 5 days after doing CT scan when the patient had developed sudden weakness of right side of the body though it was not associated with vomiting, altered sensorium and hypotension at that time, revealed the features of Moyamoya disease with lacunar infarcts (acute) in right and left posterior paraventricular white matter and centrum semiovale along with IVH [Figures 2 and 3]. Digital subtraction angiography (DSA) was not performed. Neurosurgical opinion was taken and the patient was planned to be treated with stenting of intracranial arteries. He neither suffered from hypotension, hypovolemia or dehydration nor did he receive injection Mannitol. Till the last follow-up, he was doing well and he has no neurological deficit at present.

Figure 1

CT scan of head showing intraventricular hemorrhage in right lateral ventricle

Figure 2

T2 and FLAIR images are displayed

Figure 3

MR angiogram of brain reveals occlusion of distal most ends of internal carotid artery and its bifurcation as well as in the proximal portion of anterior cerebral artery and middle cerebral artery on both sides.

Discussion

Moyamoya disease is categorized as either ischemic and hemorrhagic type or true mixed type and the pathogenesis of this disease is unknown.[5] ICH is rare in children with Moyamoya disease.[6] The incidence of ischemic complication in the acute phase of bleeding-type Moyamoya disease is still unclear.[4] In Medline search, it is seen that so far 16 cases of concomitant cerebral infarction and ICH in Moyamoya disease have been reported in the literature.[4] Among these cases, only six patients were from pediatric age group [Table 1].

Table 1

Summary of case reports of cerebral infarction following intracranial hemorrhage in children with Moyamoya disease

The effects of ICH on cerebral hemodynamics are increased ICP, IVH resulting in hydrocephalus followed by increased ICP. In patients with hemodynamic insuffiency, increase in ICP may cause ischemia.[7] Even administration of hyperosmolar drug for reduction of ICP sometimes results in dehydration.[8] Hypotension, dehydration and even vasospasm in patients with ICH may cause irreversible cerebral ischemia in Moyamoya disease as cerebral hemodynamic conditions may be critical after ICH.[9] The mechanism of ischemia may be varied in each individual patient.[89] However, in our patient, there was no clinical record of hypotension or dehydration. Our patient had features of increased ICP (vomiting, headache and irritability), so it could be the underlying mechanism of cerebral ischemia. The risk factors for progression to cerebral ischemia are complex and cerebral hemodynamics are difficult to maintain in the acute phase of bleeding type of Moyamoya disease.[9]

Patients with Moyamoya disease with ICH should be managed with great caution and monitoring. Dehydration and hypotension must be avoided. Hyperosmolar agents must be used with caution. If features of increased ICP are present, then surgical intervention like ventricular drainage should be performed. In our case, as the patient improved and features of increased ICP settled down, he was managed conservatively though intracranial arterial stenting was planned to do later. Adequate and judicious management of ICP and systemic circulation is an essential part for the management of bleeding-type Moyamoya disease.