근이영양증 환자에서의 사시수술 2예
Two Cases of Strabismus Surgery in Myotonic Dystrophy
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목적: 근이영양증 환자에서의 사시수술 2예를 경험하였기에 이를 보고하고자 한다. 증례요약: 근이영양증을 진단 받은 49세 여자 환자가 50프리즘 디옵터 이상의 외사시와 내측 및 하측 주시 시 양안 눈운동 제한으로 내원하였다. 우안 9.25 mm 외직근 후전술 및 좌안 8.75 mm 외직근 후전술을 시행하였으나 술 후에 40프리즘 디옵터의 외사시가 남았다. 39세 여자 환자가 근이영양증 1형을 진단받은 후 외사시를 주소로 내원하였다. 프리즘 가림-안가림 검사에서 40프리즘 디옵터의 근거리 외사시, 25프리즘 디옵터의 원거리 외사시 소견으로 좌안 6.0 mm 외직근 후전술 및 좌안 4.5 mm 내직근 접치기를 시행하였고 수술 3개월 후 프리즘 가림-안가림 검사에서 10프리즘 디옵터의 근거리 간헐외사시 및 6프리즘 디옵터의 원거리 외사시를 보였다. 결론: 이 질환은 백내장 및 망막 이상 이외에도 외안근 약화로 인한 눈운동 이상 및 사시가 나타날 수 있으므로 이에 대한 적절한 평가와 치료가 필요하다고 본다.
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.
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