06/11/2010 A Preventative Approach to Oral Self-mutilation in Lesch-Nyhan Syndrome: A Case Report

Resident: Hencler
Date: 06/11/2010

Article title: A Preventative Approach to Oral Self-mutilation in Lesch-Nyhan Syndrome- A Case Report

Author(s): Jeong et al.
Journal: Pediatric Dentistry-28:4 2006

Major topic: Oral Self-mutilation in Lesch-Nyhan Syndrome
Type of Article: Case Report

Main Purpose:
To present and discuss a Lesch-Nyhan syndrome patient who was treated successfully w/ noninvasive approaches, including a soft mouthguard and psychiatric pharmacologic therapy to prevent further damage of perioral soft tissues.

Background:
Lesch-Nyhan syndrome is a disorder of purine metabolism causing accumulation of sodium urate crystal in the joints, kidneys, CNS, and other tissues. Clinical symptoms include impaired kidney function, joint pain, and a progressive neurological disorder, resulting in retardation of mental and motor development. The most distressing aspect of the syndrome is the compulsive urge for self mutilation.

Case Description:
4 yo male w/ Lesch-Nyhan Syndrome and cerebral palsy receiving 2 mg diazepam daily to relieve biting action with limited success. EOE: Self-mutilated wounds noted on the lower lip and finger caused by primary central incisors. IOE: Revealed caries free intact primary dentition. A maxillary impression taken under sedation and a soft mouthguard was fabricated. Simultaneously, a psychiatric analysis was conducted. Child was biting lower lip since 12 months old whenever frustrated to control anxiety and express aggression. Sertraline and risperidone was prescribed to control the anxiety and self-mutilating behavior. After doubling dosages self-injurious behavior was markedly reduced at day 15. Continued pharmacological therapy at 1 month resulted in 1 attempts to self-mutilate per wk and at 4 months self-mutilating behavior ceased. At recall visits the lower lip was healed, mom was highly satisfied, and the patient appeared to be comfortable and less agitated.

Discussion:
Self-mutilation can result in destruction of the lower lip. EXT of primary and permanent incisors are frequently advocated as a solution, however, canines and perhaps premolars may also be involved. Oral devices to prevent self-mutilation include soft mouthguard, bite block, various types of shields that guard the tongue and lips, and lip bumpers. These methods have an advantage of preserving the teeth, but patient compliance is poor. Also, devices covering the skin may result in dermatitis. In this case, after 6 months the soft mouthguard was ill fitting due to wear and needed replacing. It is important to maintain POEs to reassess integrity of any oral appliance.

Summary of conclusions:
There are no standard methods for the prevention of self-mutilation. Appropriate preventative methods need to be developed on an individual patient basis. As an alternative treatment to EXT, a therapy combining psychiatric, pharmacologic, and dental soft mouth guard treatment may be a ideal option in treating transient and acute episodes of self-injurious behavior involving the lower lip.

Assessment of article:
Interesting case. If the medication can reduce oral self-mutilation, then combination with a soft mouthguard would be a great alternative to EXTs, just as long as the compliance is there. It would be important to preserve the models for appliance fabrication for as long as possible because to get an accurate impression on this patient population would indicate impressing under sedation of some kind. Captain obvious signing off.