Next Article in Journal
Bacillus cereus Induced Necrotizing Fasciitis Mimicking Gastroenteritis: A Case Report
Previous Article in Journal
Klebsiella pneumoniae-Induced Multiple Infections in a Diabetes Mellitus Patient: Pneumonia, Liver Abscess, Endogenous Endophthalmitis, Urinary Tract Infection
Article Menu

Export Article

Open AccessCase Report

Management and Prevention of Oral Self-Injuries in Lesch–Nyhan Syndrome

CIR Dental School, Section of Paediatric Dentistry, University of Turin, 10124 Torino, Italy
Author to whom correspondence should be addressed.
Received: 19 March 2018 / Revised: 28 March 2018 / Accepted: 6 April 2018 / Published: 9 April 2018
Full-Text   |   PDF [869 KB, uploaded 3 May 2018]   |  


Lesch–Nyhan syndrome (LNS) is a rare X-linked recessive disorder with an incidence of 1/100,000–380,000 live births. It is characterized by neurological manifestations, including symptoms of compulsive self-mutilation, which result in the destruction of oral and perioral tissues. This report describes a case of a four-year-old boy diagnosed with LNS, who was referred for evaluation and treatment of self-injury behaviour (SIB). The parents requested the prevention of self-mutilation of the lower lip and tongue by the child’s own teeth. After a thorough discussion with the parents, it was agreed that a conservative approach, avoiding extraction, should be followed initially. A removable dental appliance was fabricated. The parents were instructed and trained about insertion, removal, and cleaning of the appliance. The child was re-examined after one week: biting of the lips and tongue improved immediately after the insertion of the appliances. Initial healing of the lesion was observed. After two and four weeks, positive results were seen. The lesion had resolved completely. In conclusion, appropriate preventive methods have to be developed for each individual patient on the basis of the observation of each single case. Oral appliances represent a conservative solution for SIB and an alternative to more invasive approaches. They can be the initial solution for the management of oral self-injury in LNS patients. View Full-Text
Keywords: Lesh–Nyhan syndrome; oral self-injury; prevention; appliance Lesh–Nyhan syndrome; oral self-injury; prevention; appliance

Figure 1

This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited (CC BY 4.0).

Share & Cite This Article

MDPI and ACS Style

Caserta, G.; Defabianis, P. Management and Prevention of Oral Self-Injuries in Lesch–Nyhan Syndrome. Reports 2018, 1, 8.

Show more citation formats Show less citations formats

Note that from the first issue of 2016, MDPI journals use article numbers instead of page numbers. See further details here.

Article Metrics

Article Access Statistics



[Return to top]
Reports EISSN 2571-841X Published by MDPI AG, Basel, Switzerland RSS E-Mail Table of Contents Alert
Back to Top