Abstract
Abstract
Background
Moebius syndrome is a rare congenital disorder characterized by non-progressive palsy of the abducens (VI) and facial (VII) cranial nerves. Its common features include dysfunctions associated with other cranial nerves, orofacial abnormalities, skeletal muscle hypotonia, and other systemic disorders of differing severities. There are several concerns in the perioperative management of patients with Moebius syndrome.
Case presentation
We present a report on the management of general anesthesia of a 14-year-old male patient with Moebius syndrome who was scheduled for mandibular cystectomy. The patient was diagnosed with Moebius syndrome at the age of 7 years based on his clinical manifestations of nerve palsy since birth and cranial nerve palsy of the trigeminal (V), facial (VII), glossopharyngeal (IX), vagus (X), and sublingual nerves (XII). The patient’s oral morphological abnormalities made intubation difficult. He also experienced dysphagia and aspiration pneumonia on a daily basis. Oral secretions were frequently suctioned postoperatively. However, after discharge, the patient developed aspiration pneumonia and was readmitted to the hospital.
Conclusions
The main problem arising when administering general anesthesia to patients with this syndrome is difficult airway management. The oral abnormalities in these patients, such as small jaw and extreme dental stenosis, make mask ventilation and intubation difficult. Furthermore, this syndrome often involves respiratory impairment and dysphagia due to cerebral nerve palsy, so there is a high risk of postoperative respiratory complications. Since multiple organs are affected in patients with Moebius syndrome, appropriate perioperative management strategies must be prepared for these patients.
Publisher
Springer Science and Business Media LLC
Subject
Anesthesiology and Pain Medicine
Reference29 articles.
1. Möbius PJ. Ueber angeborene doppelseitige Abducens-Facialis-Lähmung. Mnch Wochenschr. 1888;35:91–4.
2. Möbius PJ. Uber infantilen Kernschwund. Munchen Wochnschr. 1892;39:17–21 41–3, 55–8.
3. Bell C, Nevitt S, McKay VH, Fattah AY. Will the real Moebius syndrome please stand up? A systematic review of the literature and statistical cluster analysis of clinical features. Am J Med Genet A. 2019;179:257–65.
4. Stabile M, Cavaliere ML, Scarano G, Fels A, Valiani R, Ventruto V. Abnormal B.A.E.P. in a family with Moebius syndrome: evidence for supranuclear lesion. Clin Genet. 1984;25:459–63.
5. Briegel W, Schimek M, Kamp-Becker I, Hofmann C, Schwab KO. Autism spectrum disorders in children and adolescents with Moebius sequence. Eur Child Adolesc Psychiatry. 2009;18:515–9.
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