Biopsy-proven sarcoidosis lesion of the levator palpebrae superioris causing myogenic blepharoptosis

Abstract

Purpose: We describe a case of myogenic blepharoptosis due to sarcoidosis directly involving the levator palpebrae superioris, confirmed with biopsy.

Observations: A 43-year-old male with hypertension and hyperlipidemia presented with four months of progressive right blepharoptosis and two months of dry cough. Workup revealed elevated serum muramidase, hilar adenopathy, and bilateral lacrimal gland and right levator MRI enhancement. Both lacrimal gland biopsy and later levator biopsy were consistent with sarcoidosis.

Conclusions and importance: Most sarcoidosis-related blepharoptosis is secondary to Horner syndrome or mass effect from lacrimal gland enlargement. On literature review, we did not identify prior biopsy-confirmed direct involvement of the levator as a cause of sarcoidosis-related blepharoptosis. No cases were identified with isolated levator enhancement without involvement of other extraocular muscles. In this case, both the lacrimal gland and levator biopsies were consistent with sarcoidosis. It is possible that prior reports of sarcoidosis-related blepharoptosis secondary to lacrimal gland enlargement with positive biopsy were in fact due to direct co-involvement of the levator. The exact mechanism of sarcoidosis-related blepharoptosis impacts the proposed surgical intervention if indicated after failed medical therapy, and direct levator involvement should be considered prior to surgical planning.

Keywords: Blepharoptosis; Orbital inflammation; Orbital sarcoidosis; Ptosis; Sarcoidosis.

Fig. 1

External photographs. There was minimal right levator function with an MRD1 of 0 mm at presentation (top), which worsened six months later to complete blepharoptosis (second from the top). Postoperative day 1 after the first frontalis sling procedure is shown in the photo second from the bottom. The bottom photo shows the postoperative day 1 photo after sling revision.

Fig. 2

Orbital MRI. Subtle enhancement of the right levator is indicated by the arrow in the top panel. Bilateral lacrimal glands show enlargement with asymmetric enhancement as indicated by the arrows in the panel second from the top.

Fig. 3

Right lacrimal gland and orbital soft tissue biopsies. The arrow indicates granulomatous inflammation and multinucleated giant cells (left). Arrows indicate multinucleated giant cells in levator, connective tissue (top left of right images), orbital septum adipose tissue (top right of right images), orbital fat (middle left and right of right images), and preaponeurotic fat (lower right of right images). The lower left panel of the right images highlights a large perivascular inflammatory cuff in levator and orbital fat.