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Research Letters | Nov 2012

Orbital Bands in Gomez-Lopez-Hernandez Syndrome

Michel J. Belliveau, MD; Brian W. Arthur, MD

Arch Ophthalmol. 2012;130(11):1496-1497. doi:10.1001/archophthalmol.2012.703.

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Orbital bands are anomalous muscle tissue connections between recti muscles or between a rectus muscle and the globe. Also known as supernumerary extraocular muscles, they have been identified by magnetic resonance imaging in 0.8% of orthotropic and 2.4% of strabismic patients.¹ Gomez-Lopez-Hernandez syndrome (OMIM 601853) is a rare sporadic syndrome reported in only 27 patients worldwide to date.² This syndrome, also known as cerebellotrigeminal dermal dysplasia, has not received any attention in the ophthalmic literature. The characteristic triad is rhombencephalosynapsis, trigeminal anesthesia, and bilateral alopecia in the parietal or parieto-occipital regions. Additional features are listed in the Table.

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Figure 1. Temporoparietal alopecia and low-set, posteriorly rotated ears, which are features of Gomez-Lopez-Hernandez syndrome.

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Figure 2. Magnetic resonance images. A, T2-weighted coronal view showing thin bands (arrows) joining the lateral aspects of the superior and inferior recti bilaterally. B, T1-weighted coronal view of the left orbital band. C, Sagittal view of a magnetization-prepared rapid gradient echo sequence of the left orbital band (arrow) showing the anterior-posterior dimension. D, Coronal view of a magnetization-prepared rapid gradient echo sequence showing a fused cerebellum without distinct hemispheres.

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Belliveau MJ, Arthur BW. Orbital Bands in Gomez-Lopez-Hernandez Syndrome. Arch Ophthalmol. 2012;130(11):1496-1497. doi:10.1001/archophthalmol.2012.703.

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