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      Möbius Syndrome: Surgical Treatment for Eyelid Dysfunction

      case-report

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          Abstract

          Introduction: Möbius syndrome is a heterogeneous congenital disorder that is linked to bilateral palsies of the cranial nerves VI and VII, resulting in congenital facial paralysis sometimes associated with impaired ocular abduction. Case Report: We present the case of a 44-year-old woman with Möbius syndrome and inferior recurrent keratitis secondary to scleral show in both eyes. We decided to use a cartilage graft from the ear in the inferior eyelid to avoid eyelid retraction and scleral show. Discussion: Patients with Möbius syndrome have a severe dysfunction of their facial mimic. Their treatment must be individualized, depending on their age, clinical examination and symptoms.

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          Free tissue transfer for the treatment of facial paralysis.

          David Chuang (2008)
          Long-standing facial paralysis requires the introduction of viable, innervated dynamic muscle to restore facial movement. The options include regional muscle transfer and microvascular free tissue transfer. There are advantages and disadvantages of each. Briefly, the regional muscle transfer procedures are reliable and provide immediate return of movement. However, the movement is not of a spontaneous mimetic nature. Free tissue transfer, in contrast, offers the possibility of synchronous, mimetic movement. It does, however, require a prolonged healing time in comparison with that of regional muscle transfer. The choice is made by physician and patient together, taking into account their preferences and biases. Muscle-alone free tissue transfer is our preferred option for reanimation of uncomplicated facial paralysis without skin or soft tissue deficits. Combined muscle and other tissue (most are skin flap) is another preferred option for more challenging complex facial paralysis with skin or soft tissue deficits after tumor excision. Gracilis flap is the author's first choice of muscle transplantation for both reconstructions. From 1986 to 2006, gracilis functioning free muscle transplantation (FFMT) was performed at Chang Gung Memorial Hospital for facial reanimation in 249 cases of facial paralysis. The main etiology is postoperative complication and Bell's palsy. The innervating nerve comes mostly from contralateral facial nerve branches, few from ipsilateral facial nerve due to tumor ablation, and from ipsilateral motor branch to masseter or spinal accessory nerve due to Möbius syndrome. We have evolutionally used a short nerve graft (10 to 15 cm) to cross the face in the first stage; after a 6- to 9-month waiting period, gracilis FFMT was performed for the second stage of the reconstruction. The technique of evolution has shown encouraging results to achieve the goal of rapid restoration and fewer scars on the donor leg.
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            Congenital abnormalities of cranial nerve development: overview, molecular mechanisms, and further evidence of heterogeneity and complexity of syndromes with congenital limitation of eye movements.

            Elias Traboulsi (2003)
            The clinical and molecular genetic classification of syndromes with congenital limitation of eye movements and evidence of cranial nerve dysgenesis continues to evolve. This monograph details clinical and molecular genetic data on a number of families and isolated patients with congenital fibrosis of the extraocular muscles (CFEOM) and related disorders, and presents an overview of the mechanisms of abnormal patterns of motor and sensory cranial nerve development in these rare syndromes. Clinical examination of one patient with CFEOM1, one family with clinical features of CFEOM2, one family with recessive CFEOM3, one family with horizontal gaze palsy and progressive scoliosis (HGPPS), and four patients with various combinations of congenital cranial nerve abnormalities. Genotyping of families with CFEOM and HGPPS for polymorphic markers in the regions of the three known CFEOM loci and in the HGPPS region, and mutation analysis of the ARIX and KIF21A genes in patients with CFEOM were performed according to standard published protocols. The patient with CFEOM1 had the second most common mutation in KIF21A, a 2861 G>A mutation that resulted in an R954Q substitution. The family with CFEOM2 phenotype did not map to the CFEOM2 locus. The family with recessive CFEOM3 did not map to any of the known loci. The HGPPS family mapped to 11q23-q25. One patient had optic nerve hypoplasia and fifth nerve dysfunction. Two patients had the rare combination of Möbius syndrome and CFEOM. One patient had Möbius syndrome and fifth nerve dysfunction. There is genetic heterogeneity in CFEOM2 and CFEOM3. Abnormalities in sensory nerves can also accompany abnormalities of motor nerves, further substantiating the effect of individual mutations on developing motor as well as sensory cranial nerve nuclei.
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              Surgical correction of unilateral and bilateral facial palsy.

              Rhett Harrison (2005)
              Unilateral and bilateral facial palsies are debilitating and depressing conditions for the patient. For the past 30 years attempts have been made to improve the reanimation of these patients. The ability to transfer axons over significant distances with nerve grafts and the transfer of muscle that can be revascularised by microvascular surgery greatly improves results of this surgery. The revascularisation of muscle has been the important step forward but the re-focusing of interest in this condition has brought about a number of peripheral advances.
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                Author and article information

                Journal
                Journal ID (publisher-id): COP
                Journal ID (pmc): COP
                Journal ID (doi): 10.1159/issn.1663-2699
                Title: Case Reports in Ophthalmology
                Publisher: S. Karger AG
                ISSN (Electronic): 1663-2699
                Publication date Subscription-year: 2013
                Publication date Collection: September – December 2013
                Publication date (Electronic): 05 November 2013
                Volume: 4
                Issue: 3
                Pages: 229-233
                Affiliations
                Servicio de Oftalmología, Sección de Oculoplástica y Dacriología, Hospital Universitario Ramón y Cajal, Madrid, Spain
                Author notes
                *Gloria Lopez-Valverde, MD, Calle Obispo Orberá 55, 9°-3, ES-04001 Salamanca (Spain), E-Mail gloria_lpz@hotmail.com
                Article
                Publisher ID: 356528 Pmcid ID: PMC3843936 Other ID: Case Rep Ophthalmol 2013;4:229-233
                DOI: 10.1159/000356528
                PMC ID: PMC3843936
                PubMed ID: 24348407
                SO-VID: db40db0b-2f73-47a9-81e9-3f271dc72667
                Copyright © © 2013 S. Karger AG, Basel
                License:

                Open Access License: This is an Open Access article licensed under the terms of the Creative Commons Attribution-NonCommercial 3.0 Unported license (CC BY-NC) ( http://www.karger.com/OA-license), applicable to the online version of the article only. Distribution permitted for non-commercial purposes only. Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug. Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.

                History
                Page count
                Figures: 3, Pages: 5
                Categories
                Subject: Published: November 2013

                ScienceOpen disciplines: Vision sciences,Ophthalmology & Optometry,Pathology
                Keywords: Scleral show,Inferior recurrent keratitis,Möbius syndrome,Eyelid dysfunction
                Data availability:
                ScienceOpen disciplines: Vision sciences, Ophthalmology & Optometry, Pathology
                Keywords: Scleral show, Inferior recurrent keratitis, Möbius syndrome, Eyelid dysfunction

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