Objective:

Present the multifactorial etiologies of complicating stroke with management implications in a pediatric case of Lemierre’s syndrome.

Background:

Lemierre’s syndrome is a rare septic thrombophlebitis of the head and neck with increasing prevalence among healthy, young individuals.  While internal jugular vein thrombophlebitis is common, implication of carotid arteries and consequent ischemic stroke is rare and likely multifactorial.

Design/Methods:

Case report and literature review

Results:

A previously healthy 10-year-old male presented with five days of unilateral ear and neck pain progressing to swelling, stiffness, and fevers. Initial imaging demonstrated bilateral internal jugular vein thrombophlebitis, bilateral cavernous sinus thromboses, maxillary sinusitis, orbital cellulitis, skull base osteomyelitis, and numerous brain abscesses. Subsequent vessel imaging demonstrated narrowing of the bilateral internal carotid arteries with suspected arteritis and vasospasm. Empiric antibiotics and anticoagulation were started. Reversal of anticoagulation was required for retropharyngeal abscess drainage. No complications were noted during surgery, though intermittent hypotension below the first percentile was noted. While recovering from anesthesia, the patient developed unilateral weakness and horizontal gaze palsy. MRI brain demonstrated acute ischemic strokes of the bilateral ACA-MCA watershed areas. Anticoagulation was resumed after two weeks given risk of hemorrhagic conversion. Focal deficits gradually improved during admission and subsequent imaging revealed decreased thrombi and abscess burden and expected evolution of watershed infarcts.

Conclusions:

Lemierre’s syndrome, a rare complication of oropharyngeal infection characterized by septic thrombophlebitis, is well-known for its venous complications. There remains much controversy surrounding anticoagulation and the pathophysiology related to uncommon but devastating cerebral infarct. This patient demonstrates a multifactorial etiology with extensive thrombophlebitis contributing to vasospasm, extensive arteritis impairing cerebral perfusion auto-regulation, and periods of hypotension culminating in bilateral, symmetric ACA-MCA watershed infarcts. In addition to therapeutic anticoagulation for venous thrombi and stroke prevention, early management of vasospasm, arteritis and hypotension are imperative given vessel disease and impaired auto-regulation.