Iranian Journal of Pediatrics
Tehran University of Medical Sciences Press
Vol. 22, No. 4, 2012, pp. 559-563
Bioline Code: pe12104
Full paper language: English
Document type: Case Report
Document available free of charge
Iranian Journal of Pediatrics, Vol. 22, No. 4, 2012, pp. 559-563
© Copyright 2012 - Iranian Journal of Pediatrics
Congenital Insensitivity to Pain with Anhidrosis (HSAN Type IV), Extremely Rare Syndrome that Can Be Easily Missed by Bone and Joint Surgeons: A Case Report|
Ali, Nadeem; Sharma, Sudesh; Sharma, Sonali; Kamal, Younis & Sharma, Sushil
Background: Congenital insensitivity to pain with anhidrosis is an extremely rare disorder in which injuries
can often be missed by patient, parents and even by orthopedic surgeon. Pain and tenderness, on which a
trauma team so much depends to make a clinical diagnosis and to decide whether to go for radiological
evaluation can be misleading in this rare syndrome. So complete clinical examination still forms the corner
stone to avoid misdiagnosis and pick up the rare disorders.
Case Presentation:We present a 5 year old girl child, who was brought to us as a case of one and a half month
old neglected trauma left leg and was diagnosed to be suffering from congenital insensitivity to pain with
anhidrosis (HSAN Type IV).
Conclusion: Congenital insensitivity to pain with anhidrosis is extremely rare entity, in which patients are
subjected to repeated injuries which are often neglected. There is no specific treatment but patient training
and parent education are key to avoid further neglect and damage.
Congenital Insensitivity to Pain with Anhidrosis; Hereditary sensory autonomic neuropathy; Anhidrosis; Osteoacrolysis; Hyperpyrexia
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