Vol. 29 No. 4 Case Series PDF

Mobius sequence with varied strabismus presentations in Filipino patients

Alvina Pauline D. Santiago, MD, Christopher Sebastian J. Uy, MD

Objective
To report various presentations of patients diagnosed with Möbius sequence, discuss theoretical basis for the findings, and present treatment options.

Methods
Consecutive cases of patients meeting the minimum criteria of VI and VII cranial-nerve diplegia seen from January 2003 to June 2003 were included in this case series. Their strabis-mus presentations and associated systemic findings were presented. All patients underwent a comprehensive ophthalmologic examination.

Results
Seven patients aged six months to eight years, five males and two females, were identified. Patients were born to mothers 28 to 38 years old with varying parities. First trimester insults in the form of tobacco and alcohol exposure, upper respiratory and varicella infections were seen in three patients. While all patients had bilateral abduction deficit consistent with bilateral VI cranialnerve palsy, the strabismus deviations varied. Four patients had large-angle esotropia exceeding 40 PD, one of whom had dissociated vertical deviation (DVD), ptosis, and lid fissure narrowing on attempted adduction. The others had 20 PD of exotropia, 10 PD of intermittent esotropia with DVD, and one was orthotropic. Four patients had limb abnormalities, including three with talipes equinovarus or clubfoot and one with absent distal phalanges. Four patients, two of them females, suffered from mental retardation. Two patients had seizure disorder.

Conclusion
Patients with typical Möbius sequence may have varying strabismus presentations despite bilateral VI cranial-nerve involvement. Standard strabismus management principles apply. These patients require a multidisciplinary team for optimal care.

Keywords: Cranial-nerve-VI and -VII diplegia, Hanhart syndrome; Möbius sequence/syndrome, Moebius/Möbius, Poland-Möbius syndrome, Strabismus