J Korean Ophthalmol Soc.  2017 Apr;58(4):482-487. 10.3341/jkos.2017.58.4.482.

Two Cases of Strabismus Surgery in Myotonic Dystrophy

• Kim S
• Park MR

• KMID: 2376608
• DOI: http://doi.org/10.3341/jkos.2017.58.4.482

Abstract

PURPOSE
We report two cases of strabismus on myotonic dystrophy treated with a surgical procedure.
CASE SUMMARY
A 49-year-old female with myotonic dystrophy presented with exotropia above 50 prism diopters and limitation of eye movement at nasal and inferior gaze in both eyes. We performed 9.25 mm lateral rectus recession in her right eye and 8.75 mm lateral rectus recession in her left eye. After surgery, she had 40 prism diopter residual exotropia. A 39-year-old female with myotonic dystrophy type 1 had outward deviation of her left eye. Prism cover-uncover test revealed 40 prism diopter exotropia at near distance and 25 prism diopter exotropia at far distance. We performed 6.0 mm lateral rectus recession and 4.5 mm medial rectus tucking in her left eye. Three months after surgery, the prism cover-uncover test showed 10 prism diopter residual intermittent exotropia at near distance and 6 prism diopter residual exophoria at far distance.
CONCLUSIONS
Not only cataract and retinal lesion, but also abnormal ocular movement and strabismus due to weakened extraocular muscles can occur in myotonic dystrophy patients. We recommend examination for functions of extraocular motility to diagnose and to treat for maintaining relatively straight alignment.

Keyword

Myotonic dystrophy; Strabismus surgery

MeSH Terms

Adult
Cataract
Exotropia
Eye Movements
Female
Humans
Middle Aged
Muscles
Myotonic Dystrophy*
Retinaldehyde
Strabismus*
Retinaldehyde

Figure

• 

  Figure 1. Preoperative fundus color photographs, optical coherence tomography (OCT) images of both eyes and photographs of the nine cardinal positions of gaze. (A) In fundus color photograph, the cataracts of both eyes obscured the view of the posterior pole. (B) OCT image showed epiretinal membrane on both eyes. (C) Preoperative 9 cardinal photographs showed exotropia in the primary position. The limitation of adduction of -4 in the right eye and -3.5 in the left eye were observed. Bilateral ptosis were showed, especially ptosis in the left eye was aggravated when attempted to look upward.

• 

  Figure 2. Preoperative photographs of facial appearance and the nine cardinal positions of gaze and Hess chart. (A) She had frontal bald-ness and prominent zygoma due to muscle wasting, which are characteristic facial appearance of myotonic dystrophy. (B) Photographs demonstrated adduction limitation -1 in the left eye. (C) The Hess chart at initial visit showed exotropia and left hypertropia.

• 

  Figure 3. Postoperative three-months the nine cardinal positions of gaze. Photographs demonstrated that the limitation of adduction in left eye was improved to -0.5.

Reference

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