Diagnosis is vital for subsequent surveillance for complications such as basal cell carcinomas (BCCs) and jaw cysts, and for giving genetic information.

Families should be offered regular screening, ideally with one clinician or genetic department monitoring and co-ordinating the care.

  • Most babies with Gorlin syndrome have larger than average size heads and so may need assistance in delivery either by forceps or Caesarean section; an ultrasould may help in predicting this.
  • Monitoring of head circumference throughout childhood is recommended for signs of any rapid enlargement due to increased risk of hydrocephalus. Awareness of the risk of medulloblastoma in the early years of life is important and for this reason development and physical examination is justified.
  • Dental screening should commence from 8 years; an orthopantogram examination is recommended every 12 - 18 months for the detection and early treatment of jaw cysts.
  • Annual surveillance of the skin by a Dermatologist or Plastic Surgeon is recommended. In some individuals there may be a need for more frequent surveillance.

Ref: Neviod Basal Cell Carcinoma Syndrome. Synonyms: Basal Cell Nevus syndrome (BCNS), Gorlin syndrome, NBCCS. D Gareth Evans, MD, FRCP and Peter A Farndon, MD, FRCP Gene Reviews 20301330 Mar 2013

To see full article see Gene Reviews

Next Review January 2017

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 Originally produced by Professor P. A. Farndon, Clinical Geneticist, Jim Costello (deceased) and Margaret Costello.  We are reliant on a team of medical advisors for the clinical content of the website. We are grateful for their continuing support. 
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