• Clinical and Experimental Pediatrics
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• 제52권12호
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• pp.1388-1391
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• 2009

Poland 증후군은 일측 대흉근의 부재나 저형성과 다양한 동측 손기형을 특징으로 한다. Mobius 증후군은 완전 혹은 불완전의 안면마비를 특징으로 하는 선천적 신경학적 이상을 말한다. 이들의 병인은 명확히 밝혀지지 않았지만, 이환된 측의 혈류가 감소됨으로서 발생된다 여겨지고 있다. 2.670 g 남자아이가 38주 3일에 왼쪽 안면마비와 우심증이 동반된 왼쪽 흉벽 결손에 2번째, 3번째 손가락 합지증을 가지고 태어났다. Poland-Mobius 증후군은 드물며, 세계적으로 우심증과 관련된 증례는 단지 2례 밖에 없었다. 이에 우리는 우심증이 동반된 Poland-Mobius 증후군의 국내 첫 증례를 보고하는 바이다.

• 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.