Facial diplegia with deficient abduction was first described in the German literature in 1880 by von Graefe1 and in 1881 by Harlan.2 The eponymic syndrome was coined in 1888 by Paul Julius Möbius.3 It is characterized by hypoplasia of the pons during development leading to sixth and seventh cranial nerve palsies. Previously, strabismus surgery for patients with Möbius syndrome has consisted of bilateral medial rectus recession.4 The few cases that have been reported have demonstrated mixed results.4 We describe a patient who underwent superior rectus transposition for treatment of Möbius syndrome with successful results.
A 22-month-old boy with a history of Möbius syndrome presented for management of his strabismus. He had been born via cesarean section at full term to healthy parents after a normal pregnancy. Two days after birth, the genetics service diagnosed him as having Möbius syndrome, which was distinguished from Duane syndrome due to presence of bilateral facial diplegia. When evaluated by the authors, he had −5 abduction limitation bilaterally, a variable A-pattern esotropia, rotary nystagmus, and bilateral seventh nerve palsy with decreased blink and lagophthalmos (Figure 1A). There was mild limitation of adduction bilaterally. The external examination was otherwise normal. Anterior and posterior examinations were also unremarkable, and cycloplegic retinoscopy measured +2.50 diopters sphere in both eyes. The patient had full vertical gaze, but assumed a chin-up head posture with a slight right head tilt to achieve orthotropia. His esotropia at near measured up to 25 prism diopters; the deviation could not be measured at distance because horizontal versions were so poor as to prevent refixation. At age 26 months, surgery was recommended.
(A) Preoperative photograph showing moderate angle esotropia. (B) Postoperative photograph showing correction of esotropia following bilateral superior rectus transposition and medial rectus recession.
Bilateral medial rectus recession and bilateral superior rectus transposition were performed using the technique previously described by Johnston et al.5 and others. Briefly, both medial rectus muscles were recessed to a position near the equator. Each superior rectus muscle was then transposed temporally so that the nasal pole of the superior rectus muscle was located adjacent to the previous temporal insertion of the superior rectus muscle, and the lateral pole of the superior rectus was located to the superior pole of the lateral rectus muscle insertion. No posterior fixation sutures were used (Figure 2).
Diagram of the superior rectus transposition with medial rectus recession.
The patient did well postoperatively. At the 2-month postoperative visit, the patient had no tropia in primary position with −3 bilateral abduction deficits and −2 bilateral adduction deficits and no abnormal head position.
The efficacy of superior rectus transposition with ipsilateral medial rectus recession for treatment of esotropia in Duane syndrome has been widely reported,6,7 but has not been described in the treatment of Möbius syndrome. Mehendale et al. described significant reduction in esotropia in primary position, increased abduction, and improvement in head position with few changes in adduction for patients with Duane syndrome.6 Yang et al.7 also described the effectiveness of the combination of superior rectus transposition and medial rectus recession in improving abduction, alignment, and the compensatory head posture in a comparative study against medial rectus recession or bilateral medial rectus recession. An advantage of superior rectus transposition with medial rectus recession over full tendon vertical rectus muscle transfer with medial rectus recession is that of decreased risk of anterior segment ischemia.8,9
Because our surgical procedure consisted of medial rectus recession in combination with superior rectus transposition, we cannot be certain of the relative contributions of each procedure. Nonetheless, in our hands, bilateral medial rectus muscle recession alone has been ineffective in correcting the primary position deviation present in patients with Möbius syndrome. However, we also believe that the severe restriction caused by tight medial rectus muscles made isolated transpositions untenable. This was why we performed both procedures concomitantly, and why we believe the combination of superior rectus transposition with medial rectus recession should be considered as the primary treatment for patients with bilateral Möbius syndrome.
To our knowledge, our patient is the first described in the literature with successful superior rectus transposition and medial rectus recession for Möbius syndrome. The surgery worked remarkably well, with complete improvement of esotropia and head turn and significant reduction in abduction deficits (−5 preoperatively to −3 at 2 months postoperatively) (Figure 1B). However, this improvement was associated with some additional reduction in adduction (−2 postoperatively).
Given the success of this procedure in this case, we believe that superior rectus transposition with medial rectus recession is an effective treatment for patients with bilateral Möbius syndrome.
- Von Graefe A. Graefe-Saemisch's Handbuch der Gesammten Augenheilkunde. Leipzig: Engelman; 1880:6.
- Harlan GC. Congenital paralysis of both abducens and both facial nerves. Trans Am Ophthalmol Soc. 1881;3:216–218.
- Möbius PJ. About congenital bilateral abducens and facialis palsy (1888). Strabismus. 2008;16:39–44. doi:10.1080/09273970801946737 [CrossRef]
- Spierer A, Barak A. Strabismus surgery in children with Möbius syndrome. J AAPOS. 2000;4:58–59. doi:10.1016/S1091-8531(00)90014-4 [CrossRef]
- Johnston SC, Crouch ERC Jr, Crouch ER. An innovative approach to transposition surgery is effective in treatment of Duane's syndrome with esotropia. Invest Ophthalmol Vis Sci. 2006;47:E-Abstract 2475.
- Mehendale RA, Dagi LR, Wu C, Ledoux D, Johnston S, Hunter DG. Superior rectus transposition and medial rectus recession for Duane syndrome and sixth nerve palsy. Arch Ophthalmol. 2012;130:195–201. doi:10.1001/archophthalmol.2011.384 [CrossRef]
- Yang S, MacKinnon S, Dagi LR, Hunter DG. Superior rectus transposition vs medial rectus recession for treatment of esotropic Duane syndrome. JAMA Ophthalmol. 2014;132:669–675. doi:10.1001/jamaophthalmol.2014.358 [CrossRef]
- Murdock TJ, Kushner BJ. Anterior segment ischemia after surgery on 2 vertical rectus muscles augmented with lateral fixation sutures. J AAPOS. 2001;5:323–324. doi:10.1067/mpa.2001.118668 [CrossRef]
- Saunders RA, Phillips MS. Anterior segment ischemia after three rectus muscle surgery. Ophthalmology. 1988;95:533–537. doi:10.1016/S0161-6420(88)33154-4 [CrossRef]