We present a case study of a 10-year-old child with severe burns that were misinterpreted as inflicted burns. Because of multiple injuries since early life, the family was under suspicion of child abuse and therefore under supervision of the Child Care Board for 2 years before the boy was burned. Because the boy incurred the burns without feeling pain, we conducted a thorough medical examination and laboratory testing, evaluated detection and pain thresholds, and used MRI to study brain morphology and brain activation patterns during pain between this patient and 3 healthy age- and gender-matched controls. We found elevated detection and pain thresholds and lower brain activation during pain in the patient compared with the healthy controls and reference values. The patient received the diagnosis of hereditary sensory and autonomic neuropathy type IV on the basis of clinical findings and the laboratory testing, complemented with the altered pain and detection thresholds and MRI findings. Hereditary sensory and autonomic neuropathy IV is a very rare congenital pain insensitivity syndrome characterized by the absence of pain and temperature sensation combined with oral mutilation due to unawareness, fractures, and anhidrosis caused by abnormalities in the peripheral nerves. Health care workers should be aware of the potential presence of this disease to prevent false accusations of child abuse.
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Case Report|
May 01 2014
Pain Insensitivity Syndrome Misinterpreted as Inflicted Burns
Gerbrich E. van den Bosch, MD;
aIntensive Care and
Departments of bPediatric Surgery,
Address correspondence to Gerbrich E. van den Bosch, MD, Erasmus MC–Sophia Children’s Hospital, Room KP-2881, PO Box 2060, 3000 CB Rotterdam, Netherlands. E-mail: g.vandenbosch@erasmusmc.nl
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Martin G. A. Baartmans, MD;
Martin G. A. Baartmans, MD
cDepartment of Pediatrics and
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Paul Vos, MD;
Paul Vos, MD
dDepartment of Pediatrics, Medical Center Haaglanden, Den Haag, Netherlands
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Jan Dokter, MD;
Jan Dokter, MD
eBurn Center, Maasstad Hospital, Rotterdam, Netherlands; and
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Tonya White, MD;
Tonya White, MD
fChild and Adolescent Psychiatry/Psychology, and
gRadiology, Erasmus MC–Sophia Children’s Hospital, Rotterdam, Netherlands;
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Dick Tibboel, MD
Dick Tibboel, MD
aIntensive Care and
Departments of bPediatric Surgery,
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Address correspondence to Gerbrich E. van den Bosch, MD, Erasmus MC–Sophia Children’s Hospital, Room KP-2881, PO Box 2060, 3000 CB Rotterdam, Netherlands. E-mail: g.vandenbosch@erasmusmc.nl
FINANCIAL DISCLOSURE: The authors have indicated they have no financial relationships relevant to this article to disclose.
Pediatrics (2014) 133 (5): e1381–e1387.
Article history
Accepted:
November 05 2013
Citation
Gerbrich E. van den Bosch, Martin G. A. Baartmans, Paul Vos, Jan Dokter, Tonya White, Dick Tibboel; Pain Insensitivity Syndrome Misinterpreted as Inflicted Burns. Pediatrics May 2014; 133 (5): e1381–e1387. 10.1542/peds.2013-2015
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