Department of Pediatric Dentistry
Lutheran Medical Center
Date: 06/12/2009
Article title: Lesch-Nyhan syndrome: a treatment planning dilemma
Author(s): Joseph L Dicks
Journal: Pediatric Dentistry
Volume (number): vol 4 num 2
Month, Year: 1982
Major topic: Lesch-Nyhan syndrome
Minor topics:
Type of Article: Case Review
Main Purpose: Review implications of Lesch-Nyhan syndrome and treatment of patient’s with this syndrome.
Overview of method of research: Case Review, Review of Literature
Findings:
Self mutilative behavior, especially associated with the mouth makes these patients a particular challenge for dentists.
Key points/Summary :
Cause is an inheritable error of purine metabolism, in males only
Large amounts of uric acid build up, leading to renal failure if untreated
Presents as mental retardation and self mutilative behavior, which does not improve with treatment
All have cerebral palsy with sever spasticity.
Usually mutilative behavior starts with the eruption of teeth and all perioral structures are soon destroyed
Often these patients are relaxed when restrained.
There is not much in the dental literature about this syndrome (at in 1982)
In some cases, offending teeth are extracted under LA and restraint
Extremely destructive cases will have all teeth extracted to limit mutilative risks
Sometimes periods of mutilative remission will fool clinicians into delaying treatment of dangerous teeth.
Although radical, usually full mouth extraction is the only choice in mutilative cases.
In mild cases, a soft splint has been used with some success
3 catagories of the syndrome
cat 1: no oral self-mutilative or biting behavior, most rare form
cat 2: some oral self-mutilative tendencies, but can be controlled with restraint
cat 3: severe, attempt to make mouth guard, if not, full mouth extraction under GA is best.
Assessment of article: I’ve never seen a patient with this syndrome, but it is very interesting and would be heart-breaking to treat.
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