Background and Objective:

Möbius syndrome is a congenital disorder that affects facial and abducens cranial nerves, resulting in uni or bilateral facial palsy and strabismus. The treatment is facial reanimation with free functional muscle transfer. Typically, facial reanimation has been realized in 2 or more stages with controversial issues, such as the amount of time between the stages and the ideal donor nerve (masseter or spinal accessory).

We describe our one-stage facial reanimation surgery for complete Möbius Syndrome using 2 free gracilis muscle flaps, and bilateral trigeminal or spinal nerves as a donor. We evaluate our outcomes and determine if this is an effective and safe procedure.

Methods:

Between March 2014 and October 2016, 4 patients with complete Möbius Syndrome were operated using bilateral gracilis free muscle transfers in one surgical time. Detailed physical and neurologic examinations were performed with special emphasis on which cranial nerves were involved. Traditionally our first choice of nerve donor has been the trigeminal nerve, but it was involved in 2 patients, so in theme we used the spinal nerve as a donor to innervate 2 free gracilis flaps for smile reconstruction.

The mean postoperative follow-up time was 17 months. Outcomes were assessed using the Terzis functional and aesthetic grading system, Chuang's smile excursion score, and a questionnaire to evaluate patient satisfaction.

Results:

A total of 4 patients were included: 2 male and 2 female. We performed 8 free gracilis muscle transfers in 4 surgical times. We used the facial vessels in every flap, and the trigeminal nerve as a donor in 2 patients and in the 2 remaining, the spinal nerve. We have no complications nor flap failures. All patients achieved at least score of III using the Terzis functional and aesthetic grading system, and IV in those with spinal nerve as a donor. Likewise, the minimum score using Chuang's smile excursion score was 2, but in those with spinal nerve as a donor we got a score of 3. Finally the mean satisfaction score was 3, but the patient with the longest follow-up time gave a 4.

Conclusions:

One-stage facial reanimation surgery for complete Möbius Syndrome using 2 free gracilis muscle flaps is an effective and safe procedure. Careful patient selection, adequate physical evaluation, motor nerve donor selection, flawless surgery performance, and adequate postoperative rehabilitation program, are mandatory in order to obtain success.

        · abstract in Spanish     · text in Spanish     · Spanish ( pdf )