Case report: Hereditary sensory autonomic neuropathy presenting as bifid deformity to the tongue

Hereditary sensory autonomic neuropathy (HSAN) is a group of rare genetic disorders in which affected patients have a diminished capacity to feel pain. Patients with HSAN may present with a wide range of factitial injuries, where injury to the oral cavity may be an early presenting sign. While exist...

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Main Authors: Kelsey O’Hagan-Wong, Dana Smith, Hernan Gonorazky, Michael Casas
Format: Article
Language:English
Published: Frontiers Media S.A. 2023-10-01
Series:Frontiers in Dental Medicine
Subjects:
Online Access:https://www.frontiersin.org/articles/10.3389/fdmed.2023.1179795/full
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author Kelsey O’Hagan-Wong
Dana Smith
Hernan Gonorazky
Michael Casas
author_facet Kelsey O’Hagan-Wong
Dana Smith
Hernan Gonorazky
Michael Casas
author_sort Kelsey O’Hagan-Wong
collection DOAJ
description Hereditary sensory autonomic neuropathy (HSAN) is a group of rare genetic disorders in which affected patients have a diminished capacity to feel pain. Patients with HSAN may present with a wide range of factitial injuries, where injury to the oral cavity may be an early presenting sign. While existing literature on HSAN is scant, many reports highlight the long-term outcomes that may include enucleation of eyes, amputation of fingers and limbs, and disfigurement of the tongue. This case describes a five-month-old female with repetitive injury to the tongue causing it to heal with a bifid deformity. The patient was later diagnosed with HSAN type 4. This case highlights the importance of recognition of extensive oral trauma as one of the early signs of HSAN that should provoke a timely referral for neurological assessment.
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spelling doaj.art-ed4dbf765848487194674353a7a5c9792023-10-19T12:23:44ZengFrontiers Media S.A.Frontiers in Dental Medicine2673-49152023-10-01410.3389/fdmed.2023.11797951179795Case report: Hereditary sensory autonomic neuropathy presenting as bifid deformity to the tongueKelsey O’Hagan-Wong0Dana Smith1Hernan Gonorazky2Michael Casas3Department of Dentistry, University of Toronto, Toronto, CanadaDepartment of Dentistry, The Hospital for Sick Children, University of Toronto, Toronto, ON, CanadaDepartment of Dentistry, The Hospital for Sick Children, University of Toronto, Toronto, ON, CanadaDepartment of Dentistry, The Hospital for Sick Children, University of Toronto, Toronto, ON, CanadaHereditary sensory autonomic neuropathy (HSAN) is a group of rare genetic disorders in which affected patients have a diminished capacity to feel pain. Patients with HSAN may present with a wide range of factitial injuries, where injury to the oral cavity may be an early presenting sign. While existing literature on HSAN is scant, many reports highlight the long-term outcomes that may include enucleation of eyes, amputation of fingers and limbs, and disfigurement of the tongue. This case describes a five-month-old female with repetitive injury to the tongue causing it to heal with a bifid deformity. The patient was later diagnosed with HSAN type 4. This case highlights the importance of recognition of extensive oral trauma as one of the early signs of HSAN that should provoke a timely referral for neurological assessment.https://www.frontiersin.org/articles/10.3389/fdmed.2023.1179795/fullpediatric dentistrycongenital insensitivity to painhereditary sensory autonomic neuropathytongue ulcerationbifid deformity of the tongue
spellingShingle Kelsey O’Hagan-Wong
Dana Smith
Hernan Gonorazky
Michael Casas
Case report: Hereditary sensory autonomic neuropathy presenting as bifid deformity to the tongue
Frontiers in Dental Medicine
pediatric dentistry
congenital insensitivity to pain
hereditary sensory autonomic neuropathy
tongue ulceration
bifid deformity of the tongue
title Case report: Hereditary sensory autonomic neuropathy presenting as bifid deformity to the tongue
title_full Case report: Hereditary sensory autonomic neuropathy presenting as bifid deformity to the tongue
title_fullStr Case report: Hereditary sensory autonomic neuropathy presenting as bifid deformity to the tongue
title_full_unstemmed Case report: Hereditary sensory autonomic neuropathy presenting as bifid deformity to the tongue
title_short Case report: Hereditary sensory autonomic neuropathy presenting as bifid deformity to the tongue
title_sort case report hereditary sensory autonomic neuropathy presenting as bifid deformity to the tongue
topic pediatric dentistry
congenital insensitivity to pain
hereditary sensory autonomic neuropathy
tongue ulceration
bifid deformity of the tongue
url https://www.frontiersin.org/articles/10.3389/fdmed.2023.1179795/full
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