Surgical management of jaw-winking synkinesis and ptosis in Marcus Gunn syndrome: a systematic outcomes analysis

Abstract

Aim: Marcus Gunn jaw-winking synkinesis (MGJWS) is characterized by congenital ptosis in conjunction with rapid and involuntary elevation of the affected upper eyelid upon contraction of the ipsilateral external pterygoid muscle. Selecting an approach to the surgical management of eyelid malposition in this syndrome is challenging and requires careful discussion with each patient’s family. In this systematic review, we describe reported surgical approaches, assess outcomes data, and attempt to identify areas of consensus in the management of MGJWS. Methods: Twenty-seven peer-reviewed studies were identified, describing a variety of interventions. Results: The most commonly-used surgical techniques included: bilateral levator excision with bilateral frontalis sling, unilateral levator excision with bilateral or unilateral frontalis sling, the Neuhaus/Lemagne method, and levator plication surgery. However, no clear outcomes-based consensus regarding choice of surgical approach was identified, highlighting the ongoing role of surgeon and family preference in the selection of management strategy. Further, there was considerable variability in the literature for reporting outcome measures, including grading schemes for ptosis and jaw-wink. Conclusion: The existing literature on management of MGJWS does not enable the development of an evidence-based consensus algorithm regarding the selection of an appropriate surgical technique. The disorder is treated according to a case-by-case approach governed by surgeon and family preference. Standardization of nomenclature and outcome measures is crucial for obtaining higher-quality, generalizable data in futures studies.