ORAL MANIFESTATIONS AND PROSTHETIC REHABILITATION IN HEREDITARY SENSORY AND AUTONOMIC NEUROPATHY (HSAN) TYPE IV: A CASE REPORT

Abstract

Hereditary sensory and autonomic neuropathies (HSAN) are rare genetic syndromes of unknown etiology. They are seen in early childhood and categorized in six different types by their symptoms. HSAN type 4 has an autosomal recessive transmission, with such major characteristics as loss of sense of pain, self-mutilation, anhydrosis and mental retardation. Sympathetic innervations are deficient despite the existence of sweat glands. Sufferers are hypotonic without any tendon reflexes, and neuro-motor development is retarded. In some cases tactile sensation and vibration may be intact. Biting injuries due to lack of pain sensation cause laceration, ulceration and scarring of the tongue, lips and other parts of oral mucosa. Tooth luxation and severe dental attrition have been observed. This case report presents oral and dental findings, surgical treatments and prosthetic rehabilitation of an 11- year-old boy with HSAN type 4.

Keywords

• Hereditary sensory and autonomic neuropathy,type IV,Dental needs; Removable partial prosthesis

References

1. Dearborn G. A case of congenital general pure analgesia. J Nerv Ment Dis 1932;75(6):612-615.
2. Gardner RJM SG. Syndromes affecting the central nervous system. Oxford University Press, New York:1990, p.587-620.
3. PJ. D. Neuronal atrophy and degeneration predominantly affecting peripheral sensory and autonomic neurons. Philadelphia: WB Saunders 1993, p.1065-1093.
4. Nolano M, Crisci C, Santoro L, Barbieri F, Casale R, Kennedy WR, Wendelschafer-Crabb G, Provitera V, Di Lorenzo N, Caruso G. Absent innervation of skin and sweat glands in congenital insensitivity to pain with anhidrosis. Clin Neurophysiol 2000;111(9):1596-1601.
5. Guidera KJ, Multhopp H, Ganey T, Ogden JA. Orthopaedic manifestations in congenitally insensate patients. J Pediatr Orthop 1990;10(4):514-521.
6. Gwathmey FW, House JH. Clinical manifestations of congenital insensitivity of the hand and classification of syndromes. J Hand Surg Am 1984;9(6):863-869.
7. Ozbarlas N, Sarikayalar F, Kale G. Congenital insensitivity to pain with anhidrosis. Cutis 1993;51(5):373-374.
8. Schulman H, Tsodikow V, Einhorn M, Levy Y, Shorer Z, Hertzanu Y. Congenital insensitivity to pain with anhidrosis (cipa): The spectrum of radiological findings. Pediatr Radiol 2001;31(10):701-705.

9. Yagev R, Levy J, Shorer Z, Lifshitz T. Congenital insensitivity to pain with anhidrosis: Ocular and systemic manifestations. Am J Ophthalmol 1999;127(3):322-326.
10. Bodner L, Woldenberg Y, Pinsk V, Levy J. Orofacial manifestations of congenital insensitivity to pain with anhidrosis: A report of 24 cases. ASDC J Dent Child 2002;69(3):293-296, 235.
11. Glazer M, Joshua BZ, Woldenberg Y, Bodner L. Mandibular fractures in children: Analysis of 61 cases and review of the literature. Int J Pediatr Otorhinolaryngol 2011;75(1):62-64.
12. Axelrod FB, Pearson J. Congenital sensory neuropathies. Diagnostic distinction from familial dysautonomia. Am J Dis Child 1984;138(10):947-954.
13. Thompson CC, Park RI, Prescott GH. Oral manifestations of the congenital insensitivity-to-pain syndrome. Oral Surg Oral Med Oral Pathol 1980;50(3):220-225.
14. Amano A, Akiyama S, Ikeda M, Morisaki I. Oral manifestations of hereditary sensory and autonomic neuropathy type iv. Congenital insensitivity to pain with anhidrosis. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1998;86(4):425-431.
15. Diab M. Self-inflicted orodental injury in a child with leigh disease. Int J Paediatr Dent 2004;14(1):73-77.
16. Machtei A, Levy J, Friger M, Bodner L. Osteomyelitis of the mandible in a group of 33 pediatric patients with congenital insensitivity to pain with anhidrosis. Int J Pediatr Otorhinolaryngol 2011;75(4):523-526.
17. Pedersen J, Stensgaard K, Melsen B. Prevalence of malocclusion in relation to premature loss of primary teeth. Community Dent Oral Epidemiol 1978;6(4):204-209.