Tailored Surgical Management of Marcus Gunn Jaw-Winking Syndrome with Congenital Ptosis Using Autologous Fascia Lata and Palmaris Longus Sling: A Five-Case Series

A 7-year-old boy presented with congenital severe left upper eyelid ptosis since birth associated with marked upper lid retraction during chewing and contralateral jaw movement. The ptosis had progressively worsened and completely obscured the visual axis. Examination revealed visual acuity of 6/9 in B/L eyes. Levator excursion was 1 mm, lid crease was absent, and prominent jaw-winking was present. Bell’s phenomenon was preserved. Orbital MRI and routine investigations were normal. Levator excision with staged autologous fascia lata frontalis suspension was performed. Fascia lata harvested from the right thigh was fashioned into a pentagonal sling. Because postoperative edema interfered with lid assessment, sling adjustment was performed on postoperative day 4. At discharge on day 8, jaw-winking had completely disappeared, lid position was satisfactory with mild undercorrection, and donor-site healing was uneventful.

Case 2

Case 3

A 48-year-old male presented with congenital right upper eyelid ptosis and longstanding jaw-winking since childhood, which became functionally bothersome after prior blunt orbital trauma. Examination demonstrated moderate ptosis, levator excursion below 3 mm, intact Bell’s phenomenon, preserved ocular movements, and obvious jaw-winking during lateral mandibular movement. Levator excision was performed followed by palmaris longus tendon frontalis suspension harvested from the left forearm. Postoperative recovery was uneventful with complete synkinesis abolition and stable lid contour.

Case 4

A 12-year-old boy presented with congenital right upper eyelid ptosis associated with pronounced lid elevation during chewing, speaking, and mouth opening. Levator function was approximately 2 mm with jaw-winking excursion near 5 mm. Bell’s phenomenon was good and visual acuity was 6/6 bilaterally. Levator excision followed by autologous fascia lata frontalis suspension was performed using pentagonal sling technique. By postoperative day 3, lid symmetry was satisfactory, jaw-winking had disappeared completely, and no complications were observed.

Case 5

A 55-year-old male presented with a lifelong history of left upper eyelid ptosis and intermittent jaw-winking, which had progressively worsened over the past decade, leading to visual field obstruction and cosmetic concern. He reported no history of trauma but noted increased fatigue and compensatory head tilt. On examination, he exhibited severe ptosis with levator function measuring 2 mm, intact Bell’s phenomenon, full extraocular movements, and pronounced synkinesis during mastication. Surgical intervention involved levator aponeurosis resection combined with a frontalis suspension using a harvested fascia lata graft from the right thigh. Postoperatively, the patient demonstrated complete resolution of synkinesis, improved lid height, and symmetrical palpebral fissures at 3-month follow-up.

DISCUSSION

The cases illustrate the diverse spectrum of blepharoptosis management tailored to etiology, severity, patient age, and specific functional and cosmetic requirements. In severe congenital Marcus Gunn jaw-winking synkinesis (Case 1), levator excision with autologous fascia lata frontalis sling effectively abolished synkinesis and achieved optimal lid height through edema-accommodating adjustment, highlighting its utility in pediatric cases with poor levator function. Similarly, in the 12-year-old boy with congenital right upper eyelid ptosis and pronounced jaw-winking (Case 4), levator excision followed by autologous fascia lata frontalis suspension using the pentagonal sling technique provided excellent early postoperative symmetry, complete elimination of synkinesis, and preservation of good Bell’s phenomenon without complications. This approach reinforces the value of extirpative surgery combined with reliable autologous sling material in young patients, where robust long-term lid elevation and prevention of aberrant movements are critical for visual development and facial symmetry. In the 55-year-old male with lifelong left upper eyelid ptosis and progressively worsening intermittent jaw-winking (Case 5), levator aponeurosis resection combined with frontalis suspension using harvested fascia lata effectively resolved synkinesis, improved lid height, and restored symmetrical palpebral fissures at 3-month follow-up. This intervention addressed both the mechanical ptosis and synkinetic component in an older adult with visual field obstruction and compensatory head tilt, demonstrating that similar extirpative and suspensory principles remain effective across age groups when tailored to disease chronicity and patient-reported fatigue. In contrast, severe post-traumatic non-synkinetic ptosis in an adult (Case 3) was successfully corrected using a palmaris longus tendon sling, demonstrating its advantages of simpler forearm harvest, reduced donor morbidity, and reliability when Bell’s phenomenon is intact. Mild acquired ptosis with preserved levator function (Case 2) was appropriately managed conservatively, prioritizing observation or minor procedures for cosmetic concerns without functional impairment. Together, these cases emphasize individualized approaches—extirpative strategies in synkinetic congenital or longstanding disease (Cases 1, 4, and 5), sling material selection guided by morbidity, patient age, and staging feasibility, and restraint in mild presentations—while underscoring the importance of multidisciplinary care for optimal functional, cosmetic, and long-term outcomes across age groups and etiologies.[1] The pathophysiology of MGJWS involves congenital miswiring during ocular motor development, where axons from the lateral pterygoid nerve (CN V3) aberrantly innervate the levator palpebrae superioris, bypassing CN III. This synkinesis manifests as eyelid retraction proportional to jaw excursion amplitude (typically 2-15 mm), exacerbated in severe ptosis (>7 mm) due to levator hypoplasia. Supporting evidence from histopathological studies shows normal levator histology, underscoring the neural rather than myopathic basis. Genetic factors, including incomplete penetrance in autosomal dominant patterns, contribute in 6-10% of familial cases, though most are sporadic. Differential diagnoses include Duane retraction syndrome or third nerve palsy, but the pathognomonic jaw trigger distinguishes MGJWS. [2,3,5,6,10,11]

Table.1 summarizes 10 published cases or series of Marcus Gunn jaw-winking synkinesis with ptosis, spanning 1987–2024, and reveals predominantly unilateral congenital presentations (90%) in pediatric patients (mean ages 5–12 years where specified), with surgical interventions like levator excision and frontalis sling achieving 84–92% success in reducing synkinesis and improving lid height, though residual winking persisted in severe cases; non-surgical or observational approaches were common in mild, bilateral, or diagnostic-focused reports, emphasizing variability in management and outcomes.

Table 1: Comparison of Similar Published Cases