Oral manifestations and uncommon finding in children with Moebius syndrome: report of two cases

Lucas Fernando Oliveira Tomáz Ferraresso; Kátia Kely Bragança de  Souza; Fábio Anevan Ubiski Fagundes; Mariella  Padovese; Beatriz Cruz Lopes; Tatiane Garcia; Farli Aparecida Carrilho Boer; Cássia Cilene Dezan; Mariana Emi Nagata

doi:10.47990/ahvmdz27

Authors

Lucas Fernando Oliveira Tomáz Ferraresso Department of Preventive and Restorative Dentistry, São Paulo State University (UNESP), School of Dentistry, Araçatuba, Brasil. https://orcid.org/0000-0002-2225-9262
Kátia Kely Bragança de Souza Department of Oral Medicine and Pediatric Dentistry, State University of Londrina (UEL), Londrina, Paraná, Brazil https://orcid.org/0000-0003-4965-5043
Fábio Anevan Ubiski Fagundes Department of Oral Medicine and Pediatric Dentistry, State University of Londrina (UEL), Londrina, Paraná, Brazil https://orcid.org/0000-0001-7837-4692
Mariella Padovese Department of Oral Medicine and Pediatric Dentistry, State University of Londrina (UEL), Londrina, Paraná, Brazil https://orcid.org/0000-0001-6549-293X
Beatriz Cruz Lopes Department of Oral Medicine and Pediatric Dentistry, State University of Londrina (UEL), Londrina, Paraná, Brazil https://orcid.org/0000-0003-4830-7696
Tatiane Garcia Departamento de Odontologia Preventiva e Restauradora da Universidade Estadual Paulista (UNESP), Faculdade de Odontologia, Araçatuba (SP) Brasil. https://orcid.org/0000-0002-3387-1035
Farli Aparecida Carrilho Boer Department of Oral Medicine and Pediatric Dentistry, State University of Londrina (UEL), Londrina, Paraná, Brazil https://orcid.org/0000-0003-1911-8247
Cássia Cilene Dezan Department of Oral Medicine and Pediatric Dentistry, State University of Londrina (UEL), Londrina, Paraná, Brazil https://orcid.org/0000-0002-8660-0677
Mariana Emi Nagata Department of Oral Medicine and Pediatric Dentistry, State University of Londrina (UEL), Londrina, Paraná, Brazil https://orcid.org/0000-0003-3754-4303

DOI:

https://doi.org/10.47990/ahvmdz27

Keywords:

Facial Paralysis;, Maxillofacial Abnormalities;, Mobius syndrome.

Abstract

Moebius syndrome represents a rare congenital disorder characterized by non-progressive unilateral or bilateral paralysis of the facial and abducens nerves. The aim of this study is to report the oral and dental manifestations of Moebius syndrome present in two Brazilian children. Clinical case 1: 9-year-old girl, diagnosed with Moebius-Poland syndrome, presented to the Baby Clinic of (omitted information) for preventive dental treatment. Clinical and radiographic examination revealed anterior open bite, unilateral crossbite, microstomia, lack of buccal elasticity, dry labial mucosa, dental fluorosis in upper and lower teeth associated with enamel hypoplasia, dental crowding, upper right canine ectopic eruption, palate ogival, micrognathism, lip sealing and adequate lingual tonus. Clinical case 2: 26-month-old girl, attended in the same service for preventive dental treatment. Clinical and radiographic examination revealed anterior open bite, microstomia, lack of buccal elasticity, high arched palate, hypotonic tongue, micrognathism, inadequate lip seal and dental caries. In both cases, episodes of dentoalveolar trauma were related by parents. Pediatric dentists play a fundamental role in the early diagnosis of oral and facial characteristics that may be present from birth and imply potential repercussions on the development of the stomatognathic system.

References

Mobius PJ. "Ueber angeborene doppelseitige Abducens-Facialis-Lahmung." Munch Med Wochenschr. 1888;35: 91-94.

Prieto Díaz J, Souza Dias C. Estrabismo. "Síndromes Oculomotoras Especiais." (1986): 371-7.

Broussard AB, Borazjani JG. The faces of Moebius syndrome: recognition and anticipatory guidance. MCN Am J Matern Child Nurs. 2008 Sep-Oct;33(5):272-8; quiz 279-80. doi: 10.1097/01.NMC.0000334892.45979.d5. PMID: 18758328.

Ruge-Peña NO, Valencia C, Cabrera D, Aguirre DC, Lopera F. Moebius syndrome: Craniofacial clinical manifestations and their association with prenatal exposure to misoprostol. Laryngoscope Investig Otolaryngol. 2020 Aug 3;5(4):727-733. doi: 10.1002/lio2.377. PMID: 32864445; PMCID: PMC7444781.

Herrmann J, Pallister PD, Gilbert EF, Vieseskul C, Bersu E, Pettersen JC, et al. Studies of malformation syndromes of man XXXXI B: nosologic studies in the Hanhart and the Möbius syndrome. Eur J Pediatr. 1976 Apr 6;122(1):19-55. doi: 10.1007/BF00445030. PMID: 1261566.

Verzijl HT, van der Zwaag B, Cruysberg JR, Padberg GW. Möbius syndrome redefined: a syndrome of rhombencephalic maldevelopment. Neurology. 2003 Aug 12;61(3):327-33. doi: 10.1212/01.wnl.0000076484.91275.cd. PMID: 12913192.

Carvalho G, Dias da Silva F, Celestino de Almeida H, Boas M, Gontijo Álvares M. Möbius syndrome: clinical and surgical findings in 7 patients. Arq Bras Oftalmol. 2001;64:211‐215.

Puvabanditsin S, Garrow E, Augustin G, Titapiwatanakul R, Kuniyoshi KM. Poland-Möbius syndrome and cocaine abuse: a relook at vascular etiology. Pediatr Neurol. 2005 Apr;32(4):285-7. doi: 10.1016/j.pediatrneurol.2004.11.011. PMID: 15797189.

Pastuszak AL, Schüler L, Speck-Martins CE, Coelho KE, Cordello SM, Vargas F, et al. Use of misoprostol during pregnancy and Möbius' syndrome in infants. N Engl J Med. 1998 Jun 25;338(26):1881-5. doi: 10.1056/NEJM199806253382604. PMID: 9637807.

Dubrey SW, Patel MC, Malik O. Moebius-Poland syndrome and drug associations. BMJ Case Rep. 2009;2009:bcr09.2008.0953. doi: 10.1136/bcr.09.2008.0953. Epub 2009 Apr 7. PMID: 21686544; PMCID: PMC3029089.

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 Apr 7;16(1):162. doi: 10.1186/s13023-021-01808-2. PMID: 33827605; PMCID: PMC8028757.

Picciolini O, Porro M, Cattaneo E, Castelletti S, Masera G, Mosca F, et al. Moebius syndrome: clinical features, diagnosis, management and early intervention. Ital J Pediatr. 2016 Jun 3;42(1):56. doi: 10.1186/s13052-016-0256-5. PMID: 27260152; PMCID: PMC4893276.

Rasmussen LK, Rian O, Korshoej AR, Christensen S. Fatal complications during anaesthesia in Moebius syndrome: a case report and brief discussion of relevant precautions and preoperative assessments. Int J Anesthesiol Res. 2015;3(6),116-118.

Orphanet Report Series Prevalence of rare diseases: http://www.orpha.net/orphacom/cahiers/docs/GB/Prevalence_of_rare_diseases_by_alphabetical_list.pdf; 2019. Accessed Jun 2024.

Scarpelli AC, Vertchenko TB, Resende VL, Castilho LS, Paiva SM, Pordeus IA. Möbius syndrome: a case with oral involvement. Cleft Palate Craniofac J. 2008 May;45(3):319-24. doi: 10.1597/07-084.

Pradhan A, Gryst M. Atraumatic restorative technique: case report on dental management of a patient with Moebius syndrome. Aust Dent J. 2015 Jun;60(2):255-9. doi: 10.1111/adj.12319.

Cudzilo D, Matthews-Brzozowska T. Moebius syndrome: The challenge of dental management. Eur J Paediatr Dent. 2019 Jun;20(2):143-146. doi: 10.23804/ejpd.2019.20.02.12.

Chen B, Li LX, Zhou LL. Dental management of a patient with Moebius syndrome: A case report. World J Clin Cases. 2021 Aug 26;9(24):7269-7278. doi: 10.12998/wjcc.v9.i24.7269.

Domingos AC, Lopes SL, Almeida SM, Boscolo FN, Whaites EJ. Poland-Moebius syndrome: a case with oral anomalies. Oral Dis. 2004 Nov;10(6):404-7. doi: 10.1111/j.1601-0825.2004.01045.x.

Zaidi SMH, Syed IN, Tahir U, Noor T, Choudhry MS. Moebius Syndrome: What We Know So Far. Cureus. 2023 Feb 19;15(2):e35187. doi: 10.7759/cureus.35187.

Chowdhury S, Sarkar S, Guha D, Dasgupta MK. Moebius Syndrome: A Rare Entity or a Missed Diagnosis? J Pediatr Neurosci. 2020 Apr-Jun;15(2):128-131. doi: 10.4103/jpn.JPN_72_19.

Glass GE, Mohammedali S, Sivakumar B, Stotland MA, Abdulkader F, Prosser DO, et al. Poland-Möbius syndrome: a case report implicating a novel mutation of the PLXND1 gene and literature review. BMC Pediatr. 2022 Dec 30;22(1):745. doi: 10.1186/s12887-022-03803-3.

Bavinck JN, Weaver DD. Subclavian artery supply disruption sequence: hypothesis of a vascular etiology for Poland, Klippel-Feil, and Möbius anomalies. Am J Med Genet. 1986 Apr;23(4):903-18. doi: 10.1002/ajmg.1320230405.

Hashim EAA, Quek BH, Chandran S. A narrative review of Poland's syndrome: theories of its genesis, evolution and its diagnosis and treatment. Transl Pediatr. 2021 Apr;10(4):1008-1019. doi: 10.21037/tp-20-320. PMID: 34012849; PMCID: PMC8107865.

De Serpa Pinto MV, De Magalhães MH, Nunes FD. Moebius syndrome with oral involvement. Int J Paediatr Dent. 2002 Nov;12(6):446-9. doi: 10.1046/j.1365-263x.2002.00402.x.

Renault F, Flores-Guevara R, Baudon JJ, Sergent B, Charpillet V, Denoyelle F, et al. Orofacial motor dysfunction in Moebius syndrome. Dev Med Child Neurol. 2020 Apr;62(4):521-527. doi: 10.1111/dmcn.14379.

Gillberg C, Steffenburg S. Autistic behaviour in Moebius syndrome. Acta Paediatr Scand. 1989 Mar;78(2):314-6. doi: 10.1111/j.1651-2227.1989.tb11076.x.

Kalina A, Rożniatowski P, Regulski P, Turska-Szybka A. The occurrence and intensity of taurodontism among patients in the Hospital of the Infant Jesus: Biometric analysis of panoramic radiographs. Dent Med Probl. 2015;52(4):455–461. doi:10.17219/dmp/59227.

Chetty M, Roomaney IA, Beighton P. Taurodontism in dental genetics. BDJ Open. 2021 Jul 9;7(1):25. doi: 10.1038/s41405-021-00081-6.

Castro T, Ortega AO, Mussi MC, Braga MM, Gallottini M. Caries Experience in Individuals with Moebius Syndrome. Pediatr Dent. 2016 Jan-Feb;38(1):68-71. PMID: 26892218.

Martins Mussi MC, Moffa E, Castro T, Lira Ortega A, Freitas G, Braga M, et al. Salivary parameters and oral health in the Moebius syndrome. Spec Care Dentist. 2016 Sep;36(5):265-70. doi: 10.1111/scd.12175.

Corrêa-Faria P, Martins CC, Bönecker M, Paiva SM, Ramos-Jorge ML, Pordeus IA. Clinical factors and socio-demographic characteristics associated with dental trauma in children: a systematic review and meta-analysis. Dent Traumatol. 2016 Oct;32(5):367-78. doi: 10.1111/edt.12268.

Magno MB, Nadelman P, Leite KLF, Ferreira DM, Pithon MM, Maia LC. Associations and risk factors for dental trauma: A systematic review of systematic reviews. Community Dent Oral Epidemiol. 2020 Dec;48(6):447-463. doi: 10.1111/cdoe.12574.