Oro-Facial Manifestations of Hunter Syndrome with Generalised Primary Tooth Hypomineralisation and its Palliative Management Using Protective Stabilisation: A Case Report
Published: 2021-10-09
Page: 261-266
Issue: 2021 - Volume 4 [Issue 2]
Rishi Tyagi
University College of Medical Sciences, Delhi University, Dilshad Garden, New Delhi-110095, India.
Namita Kalra
University College of Medical Sciences, Delhi University, Dilshad Garden, New Delhi-110095, India.
Amit Khatri
University College of Medical Sciences, Delhi University, Dilshad Garden, New Delhi-110095, India.
Puja Sabherwal
University College of Medical Sciences, Delhi University, Dilshad Garden, New Delhi-110095, India.
Padma Yangdol *
University College of Medical Sciences, Delhi University, Dilshad Garden, New Delhi-110095, India.
Khadeeja Kulood
University College of Medical Sciences, Delhi University, Dilshad Garden, New Delhi-110095, India.
*Author to whom correspondence should be addressed.
Abstract
Mucopolysaccharidosis Type II (MPSII) or Hunter Syndrome is a rare metabolic inherited X-linked recessive disorder caused by deficiencies of various lysosomal enzymes required for glycosaminoglycans, linked to mutations in IDS gene. Hunter Syndrome has an incidence of 1:170000 with a male predilection. The present study elucidates a case of hunter syndrome (MPSII) in a 5-year-old boy encompassing a broad clinical spectrum of systemic manifestations such asshort stature, hirsutism, stiffness in joints, broad claw like fingersadenotonsillar hypertrophy, speech impairment and bilateral conductive hearing lossand distinctive oro-dental manifestations including hypo-mineralised teeth, reduced mouth opening, thick lips and macroglossia etc. The purpose of the report is to highlight the pivotal role of paediatric dentists in improvising early diagnosis, palliative dental care and utilizing modified behaviour management techniques such as the use of passive restraints. The multi-disciplinary dental management approach is preferred to be preventive, non-invasive and customized to meet the special needs of these children.
Keywords: Hunter syndrome, hypomineralised primary teeth, behaviour management, paediatric dentistry