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Published in: BMC Anesthesiology 1/2022

Open Access 01-12-2022 | Aspiration Pneumonia | Case report

Difficult intubation and postoperative aspiration pneumonia associated with Moebius syndrome: a case report

Authors: Aya Oda, Kana Oue, Yuki Oda, Shima Taguchi, Tamayo Takahashi, Akari Mukai, Mitsuru Doi, Yoshitaka Shimizu, Masahiro Irifune, Mitsuhiro Yoshida

Published in: BMC Anesthesiology | Issue 1/2022

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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.
Literature
1.
go back to reference Möbius PJ. Ueber angeborene doppelseitige Abducens-Facialis-Lähmung. Mnch Wochenschr. 1888;35:91–4. Möbius PJ. Ueber angeborene doppelseitige Abducens-Facialis-Lähmung. Mnch Wochenschr. 1888;35:91–4.
2.
go back to reference Möbius PJ. Uber infantilen Kernschwund. Munchen Wochnschr. 1892;39:17–21 41–3, 55–8. Möbius PJ. Uber infantilen Kernschwund. Munchen Wochnschr. 1892;39:17–21 41–3, 55–8.
3.
go back to reference 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.CrossRef 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.CrossRef
4.
go back to reference 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.CrossRef 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.CrossRef
5.
go back to reference 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.CrossRef 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.CrossRef
6.
go back to reference Chowdhury S, Sarkar S, Guha D, Dasgupta MK. Moebius syndrome: A rare entity or a missed diagnosis? J Pediatr Neurosci. 2020;15:128–31.CrossRef Chowdhury S, Sarkar S, Guha D, Dasgupta MK. Moebius syndrome: A rare entity or a missed diagnosis? J Pediatr Neurosci. 2020;15:128–31.CrossRef
7.
go back to reference Carta A, Favilla S, Calzetti G, Casalini MC, Ferrari PF, Bianchi B, et al. The epidemiology of Moebius syndrome in Italy. Orphanet J Rare Dis. 2021;16:162.CrossRef Carta A, Favilla S, Calzetti G, Casalini MC, Ferrari PF, Bianchi B, et al. The epidemiology of Moebius syndrome in Italy. Orphanet J Rare Dis. 2021;16:162.CrossRef
8.
go back to reference Sarnat HB. Watershed infarcts in the fetal and neonatal brainstem. An aetiology of central hypoventilation, dysphagia, Möbius syndrome and micrognathia. Eur J Paediatr Neurol. 2004;8:71–87.CrossRef Sarnat HB. Watershed infarcts in the fetal and neonatal brainstem. An aetiology of central hypoventilation, dysphagia, Möbius syndrome and micrognathia. Eur J Paediatr Neurol. 2004;8:71–87.CrossRef
9.
go back to reference Graziadio C, Lorenzen MB, Rosa RF, Pinto LL, Zen PR, Travi GM, et al. New report of a familial case of Moebius syndrome presenting skeletal findings. Am J Med Genet A. 2010;152A:2134–8.CrossRef Graziadio C, Lorenzen MB, Rosa RF, Pinto LL, Zen PR, Travi GM, et al. New report of a familial case of Moebius syndrome presenting skeletal findings. Am J Med Genet A. 2010;152A:2134–8.CrossRef
10.
go back to reference Verzijl HT, van der Zwaag B, Cruysberg JR, Padberg GW. Möbius syndrome redefined: a syndrome of rhombencephalic maldevelopment. Neurology. 2003;61:327–33.CrossRef Verzijl HT, van der Zwaag B, Cruysberg JR, Padberg GW. Möbius syndrome redefined: a syndrome of rhombencephalic maldevelopment. Neurology. 2003;61:327–33.CrossRef
11.
go back to reference Ferguson S. Moebius syndrome: a review of the anaesthetic implications. Paediatr Anaesth. 1996;6:51–6.CrossRef Ferguson S. Moebius syndrome: a review of the anaesthetic implications. Paediatr Anaesth. 1996;6:51–6.CrossRef
12.
go back to reference Ha CY, Messieha ZS. Management of a patient with Möbius syndrome: a case report. Spec Care Dentist. 2003;23:111–6.CrossRef Ha CY, Messieha ZS. Management of a patient with Möbius syndrome: a case report. Spec Care Dentist. 2003;23:111–6.CrossRef
13.
go back to reference Ames WA, Shichor TM, Speakman M, Zuker RM, McCaul C. Anesthetic management of children with Moebius sequence. Can J Anaesth. 2005;52:837–44.CrossRef Ames WA, Shichor TM, Speakman M, Zuker RM, McCaul C. Anesthetic management of children with Moebius sequence. Can J Anaesth. 2005;52:837–44.CrossRef
14.
go back to reference Hobaika AB, Neves BS, Fernandes ML, Guedes VC. Anesthesia in a patient with Moebius sequence: case report. Rev Bras Anestesiol. 2009;59:341–3.CrossRef Hobaika AB, Neves BS, Fernandes ML, Guedes VC. Anesthesia in a patient with Moebius sequence: case report. Rev Bras Anestesiol. 2009;59:341–3.CrossRef
15.
go back to reference Telich-Tarriba JE, Guevara-Valmaña O, Ruiz-Beltran S, Aguiar-Rosas S, Cardenas-Mejia A. Difficult airway identification and management in patients with Moebius syndrome. J Craniofac Surg. 2020;31:183–5.CrossRef Telich-Tarriba JE, Guevara-Valmaña O, Ruiz-Beltran S, Aguiar-Rosas S, Cardenas-Mejia A. Difficult airway identification and management in patients with Moebius syndrome. J Craniofac Surg. 2020;31:183–5.CrossRef
16.
go back to reference Costa MMB. Neural control of swallowing. Arq Gastroenterol. 2018;55(Suppl 1):61–75.CrossRef Costa MMB. Neural control of swallowing. Arq Gastroenterol. 2018;55(Suppl 1):61–75.CrossRef
17.
go back to reference Florie MGMH, Pilz W, Dijkman RH, Kremer B, Wiersma A, Winkens B, et al. The effect of cranial nerve stimulation on swallowing: a systematic review. Dysphagia. 2021;36:216–30.CrossRef Florie MGMH, Pilz W, Dijkman RH, Kremer B, Wiersma A, Winkens B, et al. The effect of cranial nerve stimulation on swallowing: a systematic review. Dysphagia. 2021;36:216–30.CrossRef
18.
go back to reference Ramsey D, Smithard D, Kalra L. Silent aspiration: what do we know? Dysphagia. 2005;20:218–25.CrossRef Ramsey D, Smithard D, Kalra L. Silent aspiration: what do we know? Dysphagia. 2005;20:218–25.CrossRef
19.
go back to reference Teramoto S, Ishii T, Yamamoto H, Yamaguchi Y, Ouchi Y. Nasogastric tube feeding is a cause of aspiration pneumonia in ventilated patients. Eur Respir J. 2006;27:436–7 author reply 437; author reply:436–7; author reply 437.CrossRef Teramoto S, Ishii T, Yamamoto H, Yamaguchi Y, Ouchi Y. Nasogastric tube feeding is a cause of aspiration pneumonia in ventilated patients. Eur Respir J. 2006;27:436–7 author reply 437; author reply:436–7; author reply 437.CrossRef
20.
go back to reference Sugimachi K, Ueo H, Natsuda Y, Kai H, Inokuchi K, Zaitsu A. Cough dynamics in oesophageal cancer: prevention of postoperative pulmonary complications. Br J Surg. 1982;69:734–6.CrossRef Sugimachi K, Ueo H, Natsuda Y, Kai H, Inokuchi K, Zaitsu A. Cough dynamics in oesophageal cancer: prevention of postoperative pulmonary complications. Br J Surg. 1982;69:734–6.CrossRef
21.
go back to reference Rimaniol JM, D’Honneur G, Duvaldestin P. Recovery of the swallowing reflex after propofol anesthesia. Anesth Analg. 1994;79:856–9.CrossRef Rimaniol JM, D’Honneur G, Duvaldestin P. Recovery of the swallowing reflex after propofol anesthesia. Anesth Analg. 1994;79:856–9.CrossRef
22.
go back to reference Fujita I, Koyanagi T, Kukita J, Yamashita H, Minami T, Nakano H, et al. Moebius syndrome with central hypoventilation and brainstem calcification: a case report. Eur J Pediatr. 1991;150:582–3.CrossRef Fujita I, Koyanagi T, Kukita J, Yamashita H, Minami T, Nakano H, et al. Moebius syndrome with central hypoventilation and brainstem calcification: a case report. Eur J Pediatr. 1991;150:582–3.CrossRef
23.
go back to reference Hamaguchi H, Hashimoto T, Mori K, Tayama M, Fukuda K, Endo S, et al. Moebius syndrome: continuous tachypnea verified by a polygraphic study. Neuropediatrics. 1993;24:319–23.CrossRef Hamaguchi H, Hashimoto T, Mori K, Tayama M, Fukuda K, Endo S, et al. Moebius syndrome: continuous tachypnea verified by a polygraphic study. Neuropediatrics. 1993;24:319–23.CrossRef
24.
go back to reference Skoretz SA, Flowers HL, Martino R. The incidence of dysphagia following endotracheal intubation: a systematic review. Chest. 2010;137:665–73.CrossRef Skoretz SA, Flowers HL, Martino R. The incidence of dysphagia following endotracheal intubation: a systematic review. Chest. 2010;137:665–73.CrossRef
25.
go back to reference Soutome S, Hasegawa T, Yamguchi T, Aoki K, Kanamura N, Mukai T, et al. Prevention of postoperative pneumonia by perioperative oral care in patients with esophageal cancer undergoing surgery: a multicenter retrospective study of 775 patients. Support Care Cancer. 2020;28:4155–62.CrossRef Soutome S, Hasegawa T, Yamguchi T, Aoki K, Kanamura N, Mukai T, et al. Prevention of postoperative pneumonia by perioperative oral care in patients with esophageal cancer undergoing surgery: a multicenter retrospective study of 775 patients. Support Care Cancer. 2020;28:4155–62.CrossRef
26.
go back to reference Ishimaru M, Matsui H, Ono S, Hagiwara Y, Morita K, Yasunaga H. Preoperative oral care and effect on postoperative complications after major cancer surgery. Br J Surg. 2018;105:1688–96.CrossRef Ishimaru M, Matsui H, Ono S, Hagiwara Y, Morita K, Yasunaga H. Preoperative oral care and effect on postoperative complications after major cancer surgery. Br J Surg. 2018;105:1688–96.CrossRef
27.
go back to reference Berchier CE, Slot DE, Van der Weijden GA. The efficacy of 0.12% chlorhexidine mouthrinse compared with 0.2% on plaque accumulation and periodontal parameters: a systematic review. J Clin Periodontol. 2010;37:829–39.CrossRef Berchier CE, Slot DE, Van der Weijden GA. The efficacy of 0.12% chlorhexidine mouthrinse compared with 0.2% on plaque accumulation and periodontal parameters: a systematic review. J Clin Periodontol. 2010;37:829–39.CrossRef
28.
go back to reference Spreadborough P, Lort S, Pasquali S, Popplewell M, Owen A, Kreis I, et al. A systematic review and meta-analysis of perioperative oral decontamination in patients undergoing major elective surgery. Perioper Med (Lond). 2016;5:6.CrossRef Spreadborough P, Lort S, Pasquali S, Popplewell M, Owen A, Kreis I, et al. A systematic review and meta-analysis of perioperative oral decontamination in patients undergoing major elective surgery. Perioper Med (Lond). 2016;5:6.CrossRef
29.
go back to reference Fernandes CR, Pinto Filho WA, Cezar LC, Alves Gomes JM, Florencio da Cunha GK. Fatal recrudescence of malignant hyperthermia in an infant with Moebius syndrome. Braz J Anesthesiol. 2013;63:296–300.CrossRef Fernandes CR, Pinto Filho WA, Cezar LC, Alves Gomes JM, Florencio da Cunha GK. Fatal recrudescence of malignant hyperthermia in an infant with Moebius syndrome. Braz J Anesthesiol. 2013;63:296–300.CrossRef
Metadata
Title
Difficult intubation and postoperative aspiration pneumonia associated with Moebius syndrome: a case report
Authors
Aya Oda
Kana Oue
Yuki Oda
Shima Taguchi
Tamayo Takahashi
Akari Mukai
Mitsuru Doi
Yoshitaka Shimizu
Masahiro Irifune
Mitsuhiro Yoshida
Publication date
01-12-2022
Publisher
BioMed Central
Published in
BMC Anesthesiology / Issue 1/2022
Electronic ISSN: 1471-2253
DOI
https://doi.org/10.1186/s12871-022-01859-3

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