Bannayan-Zonana syndrome: Difference between revisions

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{{SI}}
#REDIRECT [[Bannayan-Riley-Ruvalcaba syndrome]]
{{CMG}}
 
{{SK}} Bannayan-Riley-Ruvalcaba syndrome
==Overview==
Bannayan-Zonana syndrome is a rare [[hamartomatous]] disorder with occurrence of multiple subcutaneous [[lipoma]]s, [[macrocephaly]] and [[hemangioma]]s.
 
==Pathophysiology==
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]].
 
==Natural History, Complications, and Prognosis==
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.
 
==Diagnosis==
===Physical Examination===
The [[macroencephaly]] of BZS is symmetrical, and does not cause widening of the ventricles or raised [[ICP]] ([[intracerebral pressure]]).
 
[[Category:Genetic disorders]]
[[Category:Disease]]
 
[[fr:Syndrome de Bannayan-Riley-Ruvalcaba]]
[[pl:Zespół Bannayana, Rileya i Ruvalcaby]]
 
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Latest revision as of 14:11, 28 January 2019