J Dent Anesth Pain Med.  2016 Jun;16(2):137-140. 10.17245/jdapm.2016.16.2.137.

Local anesthesia of the temporomandibular joint to reduce pain during mouth opening for dental treatment in a patient with spinal muscular atrophy

• Chi SI
• Kim HJ
• Seo KS
• Lee JH
• Chang J

• KMID: 2328857
• DOI: http://doi.org/10.17245/jdapm.2016.16.2.137

Abstract

Spinal muscular atrophy (SMA) is an autosomal recessive, severe neuromuscular disorder in which degeneration of alpha motor neurons in the spine progressively weakens and ultimately paralyzes the proximal muscles. It occurs in one per 6,000-10,000 infants, and is a genetic disorder with the second-highest mortality rate worldwide. An 18-year-old male patient with SMA was referred for general anesthesia for difficulty in performing dental treatment due to limited mouth opening caused by temporomandibular joint (TMJ) pain. However, the patient had a high risk of general anesthesia complications, so TMJ pain during mouth opening was reduced through local anesthesia of the TMJ. Fortunately, the anesthesia was successful in reducing pain during mouth opening, enabling the patient to receive dental treatment with an adequate mouth opening.

Keyword

Dental care for disabled; Spinal muscular atrophy; Temporomandibular joint disorder

MeSH Terms

Adolescent
Anesthesia
Anesthesia, General
Anesthesia, Local*
Dental Care for Disabled
Humans
Infant
Male
Mortality
Motor Neurons
Mouth*
Muscles
Muscular Atrophy, Spinal*
Spine
Temporomandibular Joint Disorders
Temporomandibular Joint*

Figure

• 

  Fig. 1 One milliliter of 0.5% levobupivacaine each was injected into the right and left TMJ.

Reference

1. Prior TW, Snyder PJ, Rink BD, Pearl DK, Pyatt RE, Mihal DC, et al. Newborn and carrier screening for spinal muscular atrophy. Am J Med Genet A. 2010; 152A:1608–1616.

2. Bach JR, Saltstein K, Sinquee D, Weaver B, Komaroff E. Long-term survival in werdnig-hoffmann disease. Am J Phys Med Rehabil. 2007; 86:339–345.

3. Dubowitz V. Very severe spinal muscular atrophy (sma type 0): An expanding clinical phenotype. Eur J Paediatr Neurol. 1999; 3:49–51.

4. Zerres K, Rudnik-Schoneborn S. Natural history in proximal spinal muscular atrophy. Clinical analysis of 445 patients and suggestions for a modification of existing classifications. Arch Neurol. 1995; 52:518–523.

5. Azar I. The response of patients with neuromuscular disorders to muscle relaxants: A review. Anesthesiology. 1984; 61:173–187.

6. Harris SJ, Moaz K. Caesarean section conducted under subarachnoid block in two sisters with spinal muscular atrophy. Int J Obstet Anesth. 2002; 11:125–127.

7. D'Amico A, Mercuri E, Tiziano FD, Bertini E. Spinal muscular atrophy. Orphanet J Rare Dis. 2011; 6:71.

8. Bach JR, Baird JS, Plosky D, Navado J, Weaver B. Spinal muscular atrophy type 1: Management and outcomes. Pediatr Pulmonol. 2002; 34:16–22.

9. Granger MW, Buschang PH, Throckmorton GS, Iannaccone ST. Masticatory muscle function in patients with spinal muscular atrophy. Am J Orthod Dentofacial Orthop. 1999; 115:697–702.

10. Okeson JP. Management of temporomandibular disorders and occlusion. Elsevier Health Sci;2014.

11. Nascimento MM, Vasconcelos BC, Porto GG, Ferdinanda G, Nogueira CM, Raimundo RD. Physical therapy and anesthetic blockage for treating temporomandibular disorders: A clinical trial. Med Oral Patol Oral Cir Bucal. 2013; 18:E81–E85.

12. Merlini L, Granata C, Dubowitz V. Current concepts in childhood spinal muscular atrophy. Springer Wien;1989.

13. Harding A. Inherited neuronal atrophy and degeneration predominantly of lower motor neurons. Peripher Neuropathy. 1993; 2:1537–1556.

14. Bailey RO, Radin AB. Static spinal muscular-atrophy causing temporomandibular-joint dysfunction. J Oral Maxillofac Surg. 1987; 45:182–184.

15. Cha TH, Oh DW, Shim JH. Noninvasive treatment strategy for swallowing problems related to prolonged nonoral feeding in spinal muscular atrophy type ii. Dysphagia. 2010; 25:261–264.

16. Neumann MM, Davio M, Macknet M, Applegate R. Dexmedetomidine for awake fiberoptic intubation in a parturient with spinal muscular atrophy type iii for cesarean delivery. Int J Obstet Anesth. 2009; 18:403–407.