Abstract

The most common cause of arterial ischemic stroke (AIS) in a previously healthy child is a large vessel cerebral arteriopathy. A clear relationship has been commonly noted between childhood infections and the cerebral arteriopathy in previously healthy children presenting with a first AIS. This rare outcome of common childhood infections has been described in the VIPS 1 study conducted from 2009-2016. Post-infectious arteriopathies can be subdivided into large, medium and small vessel vasculitis. Large vessel disease can further be subdivided into arterial dissection, unilateral focal cerebral arteriopathy (includes inflammatory and dissection types), primary/secondary Moya Moya disease, genetic/syndromic arteriopathies, iatrogenic and others types. A recent upper respiratory tract infection (the most common implicated cause) had 2.8 times higher odds for idiopathic arterial stenosis in children versus those with other arteriopathies (e.g., arterial dissection, moyamoya) or no arteriopathy. However, multifocal large vessel affection following orbital cellulitis is a rarely described event. Today we describe 2 cases of large vessel disease following orbital cellulitis, both presenting with monophasic illness, but with 2 different outcomes- resolution in one case and progressive arterial occlusion in another.

Case 1- A 2.5-year-old girl with moderate malnutrition, presented with right forehead abscess followed by ipsilateral orbital cellulitis. She also developed a left hydropneumothorax within a week. Subsequently she had left focal onset seizures with secondary generalised status epilepticus, followed by development of left hemiparesis. Neuroimaging showed cortical laminar necrosis and luxury hyperperfusion in the right cerebral hemisphere (in gyral pattern) and basal ganglia with infarcts of varying ages. Angiography revealed right internal carotid artery pseudoaneurysm. Vessel wall imaging showed concentric thickening and contrast enhancement of bilateral ICAs. Blood and pus cultures grew methicillin resistant staphylococcus aureus (MRSA). Investigations for immunodeficiency were negative. The child received injection vancomycin for 6 weeks and intercostal tube drainage for hydropneumothorax. She was discharged with an antiepileptic drug, and aspirin. There was complete resolution of the orbital cellulitis, and hydropneumothorax and also the pseudoaneurysm on follow-up angiography 3 months later.

Case 2- A 3.5-year-old child presented with right orbital cellulitis, which was unresponsive to the initial oral antibiotic. She subsequently developed left focal clonic seizure followed by generalisation 4 days after onset of illness. Later she had left hemiparesis, and convergent squint, chemosis and proptosis of left eye too. MRI brain showed right fronto-parietal cortical, high frontal subcortical, bilateral superficial and deep watershed infarcts. MR angiography showed obliterated left internal carotid artery (ICA), fusiform dilatation of the cavernous segment of right ICA. Thrombi were seen in the cavernous segments of bilateral ICAs. The repeat angiography 5 months later showed retrograde obliteration of bilateral ICAs beyond the carotid bulbs. Renal angiography showed normal renal arteries, thereby excluding Takayashu disease. Cerebral venogram was normal. She was put on long-term aspirin and antiepileptic therapy. She did not have stroke recurrence over the next one year. Repeat angiography showed persistent obliteration of both ICAs.

Keywords

Arteriopathy