Cold Orbital Abscess associated with Hyperimmunoglobulinemia E syndrome

Introduction:

The hyperimmunoglobulin E syndrome (HIES) is a rare immunodefiency that is typically characterized by the symptom triad of recurrent abscesses, broncho-pulmonary infections, and elevated serum IgE levels.

Material/Methods:

Clinical case report, literature search

Case report:

A 31-year-old female patient was admitted to the emergency department through the eye clinic in Tübingen. She reports of progressive swelling and redness of the left upper lid for 2 weeks with complete obstruction of the orbital opening. Upon palpation the swelling was firm, elastic and completely free of pain. There was also onychomycosis of the finger nails. HIES was the only known pre-existing condition. Her medical history showed a lung resection several years ago, likely because of abscess formation.

The eye clinic reported regular visual acuity and impaired eye elevation.

CT Scans showed an inhomogenous mass of the left eye lid with infiltration of the cranial orbita. MRI imaging showed a centrally necrotic mass lying in the extraconal space. On the right side there were multiple mucoceles arroding the maxillar, and ethmoidal sinuses as well as the orbita, with questionable breach of the cavernous sinus.

The patient had emergency surgery with external decompression of the eye lid to drain the abscess formation with endoscopic pansinus surgery to drain the mucoceles.

Clincial followup 4 weeks later showed no irritation of the eye, with slight residual impairment of elevation.

Conclusion:

Abscesses related to HIES can be clinically misinterpreted because of the underlying immunodefiency and suppressed inflammation.

Consequent diagnostics, antibiotic treatment, and surgical intervention must be performed in a timely manner, even if clinical symptoms are subacute.

Publication History

Publication Date:
23 April 2019 (online)