• Archives of Plastic Surgery
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• 제34권3호
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• pp.325-329
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• 2007

Purpose: Mobius syndrome is a rare congenital disorder characterized by facial diplegia and bilateral abducens palsy, which occasionally combines with other cranial nerve dysfunction. The inability to show happiness, sadness or anger by facial expression frequently results in social dysfunction. The classic concept of cross facial nerve grafting and free muscle transplantation, which is standard in unilateral developmental facial palsy, cannot be used in these patients without special consideration. Our experience in the treatment of three patients with this syndrome using transfer of muscles innervated by trigeminal nerve showed rewarding results. Methods: We used bilateral temporalis muscle elevated from the bony temporal fossa. Muscles and their attached fascia were folded down over the anterior surface of the zygomatic arch. The divided strips from the attached fascia were passed subcutaneously and anchored to the medial canthus and the nasolabial crease for smiling and competence of mouth and eyelids. For the recent 13 years the authors applied this method in 3 Mobius syndrome cases- 45 year-old man and 13 year-old boy, 8 year-old girl. Results: One month after the surgery the patients had good support and already showed voluntary movement at the corner of their mouth. They showed full closure of both eyelids. There was no scleral showing during eyelid closure. Also full closure of the mouth was achieved. After six months, the reconstructed movements of face were maintained. Conclusion: Temporalis muscle transfer for Mobius syndrome is an excellent method for bilateral reconstruction at one stage, is easy to perform, and has a wide range of reconstruction and reproducibility.

• 대한영상의학회지
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• 제81권4호
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• pp.979-984
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• 2020

묘성증후군은 드문 유전자 결손 증후군으로, 환아는 후두 및 후두개의 기형과 신경학적 구조적 이상으로 인해 특징적인 높은 톤의 단조로운 울음소리를 내고 반복적인 흡인성 폐렴을 앓는다. 이전 보고된 증례들의 뇌 자기공명영상 소견을 정리한 결과 교뇌 저형성이 가장 뚜렷하였고, 소뇌 저형성이 동반되기도 하여 주로 후두개와의 이상 소견을 보였다. 천막상부 구조물의 위축도 자주 관찰되었는데 이것은 교뇌 저형성에 의한 이차적인 변화로 생각되었다. 본원에서 확진된 3개월 환아 또한 교뇌 저형성이 두드러져 이전 보고된 증례들과 거의 유사하였으나 수초화 양상에서 내섬유막 전완의 수초화 감소가 아니라 전반적인 수초화의 지연이 관찰되었다는 점에서 타 증례와 차이가 있었다. 후두, 교뇌, 소뇌는 비슷한 척삭에서 유래하므로, 묘성증후군에서 생기는 후두 및 뇌의 구조적 이상은 발생 초기의 이상임을 시사한다.