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Ординатура / Офтальмология / Английские материалы / Essentials in Ophthalmology Pediatric Ophthalmology Neuro-Ophthalmology Genetics_Lorenz, Brodsky_2010.pdf
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706 Neuroanatomical Strabismus

Antagonists of hypoplastic EOMs become secimpairment. Moebius syndrome is a heterogeneous

 

 

 

ondarily sti .

clinical disorder whose clinical definition has evolved in

 

 

Neuromuscular features may vary between orbits

the recent literature. Minimum criteria include congeni-

 

 

 

of the same patient, and among patients with

tal facial palsy with impairment of ocular abduction

6

 

 

 

 

identical genetic CCDDs.

[54–56]. The wide clinical spectrum and multiple areas

 

 

High-resolution imaging of EOMs and their

of brainstem involvement in patients with Moebius syn-

 

 

 

peripheral innervation can be clinically valuable

drome have led to its early conceptualization as a devel-

 

 

 

for strabismus management in CCDDs.

 

opmental disorder of the brainstem, rather than an

 

 

 

 

 

 

 

 

 

isolated cranial nerve developmental disorder [56].

 

 

 

 

 

 

 

 

 

 

However, Moebius syndrome may present with total

 

 

 

 

 

facial paralysis and complete external ophthalmoplegia,

 

 

 

 

 

where MRI demonstrates a normal brainstem and suba-

 

 

6.6.5 Moebius Syndrome

 

 

rachnoid portions of motor cranial nerves innervating

 

 

 

 

 

 

 

Moebius syndrome typically presents as a sporadic trait

the orbit, but marked hypoplasia of the deep portions of

 

 

with

congenital facial (CN7) palsy and abduction

the EOMs.

Fig. 6.8 Coronal T2-weighted MRI of both orbits in left SO palsy demonstrating marked reduction in SO cross-section, as well as reduction in normal contractile increase in cross-section from up to down gaze