Bannayan-Zonana syndrome: Difference between revisions
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{{SI}} | {{SI}} | ||
{{CMG}} | {{CMG}}; {{AE}} | ||
{{SK}} Bannayan-Riley-Ruvalcaba syndrome | {{SK}} Bannayan-Riley-Ruvalcaba syndrome | ||
==Overview== | ==Overview== | ||
Bannayan-Zonana syndrome is a rare [[hamartomatous]] disorder with occurrence of multiple subcutaneous [[lipoma]]s, [[macrocephaly]] and [[hemangioma]]s. | Bannayan-Zonana syndrome is a rare [[hamartomatous]] disorder with occurrence of multiple subcutaneous [[lipoma]]s, [[macrocephaly]] and [[hemangioma]]s. | ||
==Historical Perspective== | |||
==Classification== | |||
==Pathophysiology== | ==Pathophysiology== | ||
The disease is inherited in an [[autosomal dominant]] form, but sporadic cases have been reported. | 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]]. | The disease belongs to a family of hamartomatous polyposis syndromes, which also includes [[Peutz-Jeghers syndrome]], [[juvenile polyposis]] and [[Cowden syndrome]]. | ||
==Causes== | |||
==Differentiating {{PAGENAME}} from Other Diseases== | |||
==Epidemiology and Demographics== | |||
==Risk Factors== | |||
==Screening== | |||
==Natural History, Complications, and Prognosis== | ==Natural History, Complications, and Prognosis== | ||
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==Diagnosis== | ==Diagnosis== | ||
===Diagnostic Criteria=== | |||
==History and Symptoms== | |||
===Physical Examination=== | ===Physical Examination=== | ||
The [[macroencephaly]] of BZS is symmetrical, and does not cause widening of the ventricles or raised [[ICP]] ([[intracerebral pressure]]). | The [[macroencephaly]] of BZS is symmetrical, and does not cause widening of the ventricles or raised [[ICP]] ([[intracerebral pressure]]). | ||
[[Category: | ==Laboratory Findings== | ||
==Imaging Findings== | |||
==Other Diagnostic Studies== | |||
==Treatment== | |||
===Medical Therapy=== | |||
===Surgery=== | |||
===Prevention=== | |||
[[Category:Endocrinology]] | |||
[[Category:Disease]] | [[Category:Disease]] | ||
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[[pl:Zespół Bannayana, Rileya i Ruvalcaby]] | [[pl:Zespół Bannayana, Rileya i Ruvalcaby]] | ||
{{ | {{WS}} | ||
{{ | {{WH}} | ||
Revision as of 16:52, 22 July 2016
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:
Synonyms and keywords: Bannayan-Riley-Ruvalcaba syndrome
Overview
Bannayan-Zonana syndrome is a rare hamartomatous disorder with occurrence of multiple subcutaneous lipomas, macrocephaly and hemangiomas.
Historical Perspective
Classification
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.
Causes
Differentiating Bannayan-Zonana syndrome from Other Diseases
Epidemiology and Demographics
Risk Factors
Screening
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
Diagnostic Criteria
History and Symptoms
Physical Examination
The macroencephaly of BZS is symmetrical, and does not cause widening of the ventricles or raised ICP (intracerebral pressure).