Bannayan-Zonana syndrome

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Bannayan-Zonana syndrome (Bannayan-Riley-Ruvalcaba syndrome) is a rare hamartomatous disorder with occurrence of multiple subcutaneous lipomas, macrocephaly and hemangiomas.[1][2] The disease is inherited in an autosomal dominant form, but sporadic cases have been reported. The disease belongs to a family of hamartomatous polyposis syndromes, which also includes Peutz-Jeghers syndrome, juvenile polyposis and Cowden syndrome.

Most lesions of BZS are slowly growing and easily resectable. Visceral as well as intracranial involvement may occur in rare cases, and can cause bleeding and symptomatic mechanical compression, especially of the spinal cord or spinal nerve roots. This may require surgical resection.

The macroencephaly of BZS is symmetrical, and does not cause widening of the ventricles or raised ICP (intracerebral pressure).

[edit] References

  1. ^ Buisson P, Leclair MD, Jacquemont S, et al (September 2006). "Cutaneous lipoma in children: 5 cases with Bannayan-Riley-Ruvalcaba syndrome". J. Pediatr. Surg. 41 (9): 1601–3. doi:10.1016/j.jpedsurg.2006.05.013. PMID 16952599. 
  2. ^ Gujrati M, Thomas C, Zelby A, Jensen E, Lee JM (August 1998). "Bannayan-Zonana syndrome: a rare autosomal dominant syndrome with multiple lipomas and hemangiomas: a case report and review of literature". Surg Neurol 50 (2): 164–8. PMID 9701122.