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Semi-Fixed Lip Bumper in Lesch-Nyhan Syndrome: An Interim Treatment Modality

  • Journal of the Korean academy of pediatric dentistry
  • Abbr : J Korean Acad Pediatr Dent
  • 2020, 47(1), pp.93-98
  • DOI : 10.5933/JKAPD.2020.47.1.93
  • Publisher : The Korean Academy Of Pediatric Dentistry
  • Research Area : Medicine and Pharmacy > Dentistry
  • Received : September 16, 2019
  • Accepted : November 4, 2019
  • Published : February 29, 2020

Junhee Lee 1 Eungyung Lee 1 Shin, Jonghyun ORD ID 2 Shin Kim ORD ID 2 JEONG,TAE-SUNG ORD ID 2

1부산대학교 치의학전문대학원 소아치과학교실
2부산대학교

Accredited

ABSTRACT

Lesch-Nyhan syndrome is a rare X-linked, recessively inherited disorder of purine metabolism, caused by complete absence of the enzyme hypoxanthine-guanine phosphoribosyl transferase. This syndrome is characterized by 3 major features: neurological dysfunction, hyperuricemia, and cognitive and behavioral disturbances (e.g., self-mutilation, which begins at 2 to 3 years of age). Uncontrollable self-mutilation begins with biting of the perioral tissues and extends into patterns such as finger biting and head hitting. This report describes the case of a 31-month-old boy who was diagnosed with Lesch-Nyhan syndrome with severe lip injuries caused by self-mutilative behaviors. The behaviors were blocked with a semi-fixed lip bumper for a short period. The device was applied to the patient on the day of the visit without the requirement for an oral impression. It was easy to manage oral hygiene and adjust the device because it was detachable by clinicians and guardians. Therefore, a semi-fixed lip bumper may be useful as an interim appliance to block selfmutilative behaviors in children with Lesch-Nyhan syndrome.

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