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Laurence-Moon-Bardet-Biedl syndrome


ICD-10: Q87.8

ORPHA: 110

General information

Estimated occurrence
Uncommon diagnosis. Thirteen known cases in Sweden (2004).
Unknown. Genetically determined. Changes have been found in six chromosomes with different mutations in different families. Laurence-Moon-Bardet-Biedl syndrome (LMBB) is an autosomal recessive disorder.
General symptoms
Symptoms may vary but the core symptoms are retinal changes, extra fingers and/or toes, obesity, underdevelopment of the male reproductive organs (hypogenitalism) and low concentrations of sexual hormones (hypogonadism), renal changes, intellectual disability and coordination difficulties.
Bardet-Biedl syndrome, Laurence-Moon syndrome

Orofacial/odontological symptoms

Tooth anomalies may occur, such as lack of tooth buds for one or more teeth (hypodontia), small/thin teeth and short tooth roots. Snoring is common. Impaired oral motor function occurs and may cause speech and eating difficulties, as well as drooling.

Advice on follow-up and treatment

  • Early contact with dental services for intensified prophylactic care and oral hygiene information is essential.
  • Regular check-ups of dental and jaw development. Orthodontist should be consulted when needed.
  • Extra care is recommended in orthodontic treatment of patients with short tooth roots.
  • Snoring problems should be investigated by a physician in cases of suspected sleep apnea (frequent breathing pauses during sleep).
  • Oral motor training may be required in cases of eating difficulties, speech impairment and drooling.


National Board of Health and Welfare

Updated: 2019-11-12 14:53