Keywords :
Congenital insensitivity to pain and anhydrosis, Pain insensitivity, Marvan syndrome, Painless whitlows
Citation Information :
Ravichandra K, Kandregula CR, Koya S, Lakhotia D. Congenital Insensitivity to Pain and Anhydrosis: Diagnostic and Therapeutic Dilemmas Revisited. Int J Clin Pediatr Dent 2015; 8 (1):75-81.
First described in 1932 by Dearborn as ‘congenital pure analgesia’, congenital insensitivity to pain and anhydrosis (CIPA) or hereditary sensory and autonomic neuropathy (HSAN) type IV is an extremely rare autosomal recessive disorder. A 7-year-old female child who is an established case of congenital insensitivity to pain and anhydrosis visited the department of pediatric medicine with osteoarthritic neuropathy. A multidisciplinary team approach was utilized to treat the child under general anesthesia. This article also discusses the diagnostic and therapeutic dilemmas involved in treating this type of children.
How to cite this article: Ravichandra KS, Kandregula CR, Koya S, Lakhotia D. Congenital Insensitivity to Pain and Anhydrosis: Diagnostic and Therapeutic Dilemmas revisited. Int J Clin Pediatr Dent 2015;8(1):75-81.
BAR-On E, Weigl D, Parvari R, Katz K, Weitz R, Steinberg T. Congenital insensitivity to pain orthopaedic manifestations. J Bone Joint Surg Br 2002;84-B(2):252-257.
Sezgin B, Bolgul N, Hamamci N, Açkiran EA, Çelenk S, Ayna B. Congenital insensitivity to pain: a case report with dental implications. HKJ Paediat (new series) 2010;15:234-237.
Swanson AG. Congenital insensitivity to pain with anhidrosis: a unique syndrome in two male siblings. Arch Neurol 1963;8(3):299-306.
Dave N, Sonawane A, Chanolkar S. Hereditary sensory autonomic neuropathy and anaesthesia—a case report. Ind J Anaesth 2007;51(6):528-530.
Bonkowsky JL, Johnson J, Carey JC, Smith AG, Swoboda KJ. An infant with primary tooth loss and palmar hyperkeratosis: a novel mutation in the NTRK1 gene causing congenital insensitivity to pain with anhidrosis. Pediatrics 2003 Sep;112(3 Pt 1):e237-241.
Shatzky S, Moses S, Levy J, et al. Congenital insensitivity to pain with anhidrosis (CIPA) in Israeli-Bedouins: genetic heterogenicity, novel mutations in the TRKA/NGF receptor gene, clinical findings, and results of nerve conduction studies. Am J Med Genet 2000;92(5):353-360.
Shalimar A, Sharaf I, Wahida IF, Ruszymah BH. Congenital insensitivity to pain with anhydrosis in a Malaysian family: a genetic analysis. J Orthopaedic Surg 2007;15(3):357-360.
Jewesbury ECO. Congenital indifference to pain. In Vincken PW, Bruyn GW, editors. Handbook of clinical neurology, Amsterdam: Elsevier; 1970. p. 187-204.
Nagasako EM, Oaklander AL, Dworkin RH. Congenital insensitivity to pain: an update. Pain 2003;101(3):213-219.
McMurray GA. Experimental study of a case of insensitivity to pain. Arch Neurol Psych 1950;64(5):650-667.
Treede RD, Kenshalo DR, Gracely RH, Jones AKP. The cortical representation of pain. Pain 1999;79(2-3):105-111.
Ogden TE, Robert F, Carmichael EA. Some sensory syndromes in children: indifference to pain and sensory neuropathy. J Neurol Neurosurg Psychiatry 1959;22:267-276.
Pinsky L, Di George AM. Congenital familial sensory neuropathy with anhidrosis. J Pediatr 1966;68(1):1-13.
Dyck PJ, Ohta M. Neural atrophy and degeneration predominantly affecting peripheral sensory neurons, in Peripheral Neuropathy, Dyck PJ, Thomas PK, Lambert EH, editors. Toronto: WB Saunders Co, 1975;2:791-812.
Karimi M, Fallah R. A Case Report of Congenital Insensitivity to Pain and Anhidrosis (CIPA). Iran J Child Neurol 2012;6(3): 45-48.
Kouvelas N, Terzoglou DPC. Congenital insensitivity to pain with anhidrosis: case report. Pediat Dent 1989;11(1): 47-51.
Neves BG, Roza RT, Castro GF. Traumatic lesions from congenital insensitivity to pain with anhidrosis in a pediatric patient: dental management. Dent Traumatol 2009;25(5): 545-549.
Rahalkar MD, Rahalkar AM, Joshi SK. Congenital insensitivity to pain and anhidrosis. Ind J Radiol Imaging 2008;18(2): 132-134.
Paduano S, Iodice G, Farella M, Silva R, Michelotti A. Orthodontic treatment and management of limited mouth opening and oral lesions in a patient with congenital insensitivity to pain: case report. J Oral Rehabil 2009;36(1):71-78.
Zamuri Z, Goh KL, Aminuddin CA, Mohamed Azril MA, Shukrimi A. Congenital insensitivity to pain with recurrent septic arthritis of the left knee. Int Med J Malaysia 2011; 10(1):51-52.
Inouye D. Congenital insensitivity to pain with anhydrosis. Accessed google search engine on: 04/01/2015. Available at: http://hilo.hawaii.edu/academics/hohonu/documents/Vol06x04CongenitalInsensitivitytoPainwithAnhidrosis.pdf.