Bannayan-Zonana syndrome

Infobox_Disease
Name = PAGENAME


Caption =
DiseasesDB = 31337
ICD10 =
ICD9 =
ICDO =
OMIM = 153480
MedlinePlus =
eMedicineSubj =
eMedicineTopic =
MeshID = D006223

Bannayan-Zonana syndrome ("Bannayan-Riley-Ruvalcaba syndrome") is a rare hamartomatous disorder with occurrence of multiple subcutaneous lipomas, macrocephaly and hemangiomas. [cite journal |author=Buisson P, Leclair MD, Jacquemont S, "et al" |title=Cutaneous lipoma in children: 5 cases with Bannayan-Riley-Ruvalcaba syndrome |journal=J. Pediatr. Surg. |volume=41 |issue=9 |pages=1601–3 |year=2006 |month=September |pmid=16952599 |doi=10.1016/j.jpedsurg.2006.05.013] [cite journal |author=Gujrati M, Thomas C, Zelby A, Jensen E, Lee JM |title=Bannayan-Zonana syndrome: a rare autosomal dominant syndrome with multiple lipomas and hemangiomas: a case report and review of literature |journal=Surg Neurol |volume=50 |issue=2 |pages=164–8 |year=1998 |month=August |pmid=9701122 |doi= |url=http://linkinghub.elsevier.com/retrieve/pii/S0090-3019(98)00039-1] 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.

Presentation

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).

Eponym

It is named for George A. Bannayan and Jonathan Zonana. [WhoNamedIt|synd|1276] cite journal |author=Bannayan GA |title=Lipomatosis, angiomatosis, and macrencephalia. A previously undescribed congenital syndrome |journal=Arch Pathol |volume=92 |issue=1 |pages=1–5 |year=1971 |month=July |pmid=5091590 |doi= |url=] cite journal |author=Zonana J, Rimoin DL, Davis DC |title=Macrocephaly with multiple lipomas and hemangiomas |journal=J. Pediatr. |volume=89 |issue=4 |pages=600–3 |year=1976 |month=October |pmid=957004 |doi= |url=]

References