Pediatrics & Neonatology
Volume 51, Issue 1 , Pages 57-60, February 2010

Successful Weaning of a Laryngeal Mask Airway After a Tongue-lip Adhesion Operation in a Case With Cerebro-costo-mandibular Syndrome

  • Chi-Yung So

      Affiliations

    • Division of Genetics, Department of Pediatrics, Chung Shan Medical University Hospital, Taichung, Taiwan
    • ,
  • Yan-Yan Ng

      Affiliations

    • Division of Genetics, Department of Pediatrics, Chung Shan Medical University Hospital, Taichung, Taiwan
    • ,
  • Chih-Yu Peng

      Affiliations

    • Department of Oral and Maxillofacial Surgery, Chung Shan Medical University, Taichung, Taiwan
    • ,
  • Jui-Ming Hu

      Affiliations

    • Division of Genetics, Department of Pediatrics, Chung Shan Medical University Hospital, Taichung, Taiwan
    • ,
  • Suh-Jen Chen

      Affiliations

    • Division of Genetics, Department of Pediatrics, Chung Shan Medical University Hospital, Taichung, Taiwan
    • ,
  • Jia-Yuh Chen

      Affiliations

    • School of Medicine, Chung Shan Medical University, Taichung, Taiwan
    • ,
  • Pen-Hua Su

      Affiliations

    • Division of Genetics, Department of Pediatrics, Chung Shan Medical University Hospital, Taichung, Taiwan
    • School of Medicine, Chung Shan Medical University, Taichung, Taiwan
    • Corresponding Author InformationCorresponding author. Division of Genetics, Department of Pediatrics, Chung Shan Medical University Hospital, 110 Chien-Kuo North Road, Section 1, Taichung, Taiwan

Received 29 December 2008; received in revised form 23 March 2009; accepted 24 April 2009.

Article Outline

Cerebro-costo-mandibular syndrome (CCMS) consists of severe micrognathia, glossoptosis, posterior rib-gap defects and developmental delay. It may cause upper airway obstruction and flail chest, resulting in neonatal hypoxia, and possibly death. Early airway management or surgical intervention to maintain a patent airway is critical to avoid hypoxia in CCMS patients. We report a newborn with CCMS who was successfully weaned from a laryngeal mask after undergoing a tongue–lip adhesion operation at 164 days of age.

Key Words:  airway obstruction , cerebro-costo-mandibular syndrome , tongue-lip adhesion

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References 

  1. Flodmark P , Wattsgård C . Cerebro-costo-mandibular syndrome . Pediatr Radiol . 2001;31:36–37
  2. Hennekam RC , Goldschmeding R . Complete absence of rib ossification, micrognathia and ear anomalies: extreme expression of cerebro-costo-mandibular syndrome? . Eur J Hum Genet . 1998;6:71–74
  3. McNicholl B , Egan-Mitchell B , Murray JP , Doyle JF , Kennedy JD , Crome L . Cerebro-costo-mandibular syndrome: a new familial developmental disorder . Arch Dis Child . 1970;45:421–424
  4. James PA , Aftimos S . Familial cerebro-costo-mandibular syndrome: a case with unusual prenatal findings and review . Clin Dysmorphol . 2003;12:63–68
  5. Wilcox BK , Tatum SA . Cerebro-costo-mandibular syndrome presenting as Pierre Robin Sequence . Am J Otolaryngol . 2004;25:258–262
  6. Huang F , Lo LJ , Chen YR , Yang JC , Niu CK , Chung MY . Tongue-lip adhesion in the management of Pierre Robin Sequence with airway obstruction: technique and outcome . Chang Gung Med J . 2005;28:90–96
  7. Tibesar RJ , Price DL , Moore EJ . Mandibular distraction osteogenesis to relieve Pierre Robin airway obstruction . Am J Otolaryngol . 2006;27:436–439
  8. Meyer AC , Lidsky ME , Sampson DE , Lander TA , Liu M , Sidman JD . Airway interventions in children with Pierre Robin Sequence . Otolaryngol Head Neck Surg . 2008;138:782–787
  9. Douglas B . The treatment of micrognathia associated with obstruction by a plastic procedure . Plast Reconstr Surg . 1946;1:300–308
  10. McCarthy JG , Schreiber J , Karp N , Thorne CH , Grayson BH . Lengthening the human mandible by gradual distraction . Plast Reconstr Surg . 1992;89:1–8

PII: S1875-9572(10)60011-3

doi:10.1016/S1875-9572(10)60011-3

Pediatrics & Neonatology
Volume 51, Issue 1 , Pages 57-60, February 2010

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