Congenital Ptosis May Stem From Fibrotic Changes to the Levator Aponeurosis

Fibrotic changes in levator aponeurosis may play a role in the etiology of simple congenital ptosis and suggest a complex pathogenesis, according to study findings published in Ophthalmic Plastic and Reconstructive Surgery. 

The precise muscle pathogenesis of the levator palpebrae superioris is still debated although there is general agreement on the myogenic etiology of simple congenital ptosis.

“The effect of [levator aponeurosis] fibrotic changes on the [levator function] was assessed in different [levator aponeurosis] fibrotic changes with or without levator palpebrae superioris (LPS) muscle fatty infiltration,” according to the report.

The study included 56 eyelids from 49 pediatric patients (57.1% boys; mean age, 6.7 years) with simple congenital ptosis who underwent skin approach levator aponeurosis resection as the primary intervention with an intraoperative photographic documentation of fibrotic changes in levator aponeurosis. Bilateral simple congenital ptosis was noted in 7 patients. Severe, moderate, and mild ptosis were noted in 39 patients, 16 patients, and 2 patients, respectively. 

Among patients with severe ptosis, levator function was good (8 mm to 9 mm) in 10 eyelids, fair (5 mm to 7 mm) in 18 eyelids, and poor (3 mm to 4 mm) in 11 eyelids. Variable presentation was noted upon review of intraoperative photographic documentation with normal levator aponeurosis appearing white, smooth, and with a shiny sheet seen just below the preaponeurotic fat. Abnormal fibrotic changes in levator aponeurosis were noted as either a sheet of fibrosis (19 eyelids) or fibrous bands (23 eyelids). In addition, a thin levator aponeurosis was noted in 4 eyelids. Fatty infiltrations of LPS was noted in a total of 28 eyelids, 10 of which were without fibrotic changes to levator aponeurosis and 18 of which were associated with variable shapes and degrees of fibrosis. 

All patients underwent anterior approach levator aponeurosis resection. Compared with levator aponeurosis with fibrotic sheets,  preoperative levator function was diminished in patients with LPS fatty infiltration (P =.026). Additionally, postoperative levator function was significantly improved in both levator aponeurosis fibrotic sheets and fibrotic bands compared with LPS with fatty infiltration (P =.004). Finally, significant improvements in postoperative levator function was noted in patients with fibrotic bands without LPS fatty infiltration compared with fibrotic bands with LPS fatty infiltration (P <.001). 

“The data in our study may raise a possible role of the LA developmental changes as an additional etiology of simple congenital ptosis. To rationalize this possibility, it is worthy to address the sequences of LPS development,” according to the research. “Further embryologic and histopathologic studies are warranted.”

Reference 

Al-Faky YH. Surgical observations of the levator aponeurosis fibrotic changes in simple congenital ptosis suggest complex pathogenesis. Ophthalmic Plast Reconstr Surg. 2021;37(4):329-333. doi:10.1097/IOP.0000000000001860