• Title/Summary/Keyword: Mobius syndrome

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The first Korean case of poland-Mobius syndrome associated with dextrocardia (Poland-Mobius syndrome 신생아 1례)

  • Jung, Jiyoung;Kim, Han Gyu;Ahn, Hae-Mi;Cho, Su Jin;Park, Eun Ae
    • Clinical and Experimental Pediatrics
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    • v.52 no.12
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    • pp.1388-1391
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    • 2009
  • Poland syndrome is characterized by unilateral absence or hypoplasia of the pectoralis muscle and variable degree of ipsilateral hand anomalies. Mobius syndrome is a congenital neurological disorder characterized by complete or partial facial paralysis. Although the pathogeneses of these diseases are not well-characterized, diminished blood flow to the affected side is thought to play a role. A male infant weighing 2.670 g was born at 38+3 weeks of gestation with left facial paralysis, left chest wall defect with dextrocardia, and symbrachydactyly between the second and third fingers. The combination of Poland-Mobius syndrome is rare, and only 2 cases associated with dextrocardia have been reported worldwide. Here, we report the first case of Poland-Mobius syndrome associated with dextrocardia in Korea.

Dynamic Reconstruction with Temporalis Muscle Transfer in Mobius Syndrome (뫼비우스 증후군에서 측두근 전위술을 이용한 역동적 재건)

  • Kim, Baek Kyu;Lee, Yoon Ho
    • Archives of Plastic Surgery
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    • v.34 no.3
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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.