Bonnet-Dechaume-Blanc syndrome: Difference between revisions

Jump to navigation Jump to search
Line 14: Line 14:
== Diagnosis ==
== Diagnosis ==
== Treatment ==
== Treatment ==
The treatment for Bonnet–Dechaume–Blanc syndrome is controversial due to a lack of consensus on the different therapeutic procedures for treating arteriovenous malformations.<ref name=":7">{{cite journal|last1=Schmidt|first1=D|last2=Pache|first2=M|last3=Schumacher|first3=M|title=The congenital unilateral retinocephalic vascular malformation syndrome (bonnet-dechaume-blanc syndrome or wyburn-mason syndrome): review of the literature.|journal=Survey of ophthalmology|date=2008|volume=53|issue=3|pages=227–49|pmid=18501269}}</ref>  The first successful treatment was performed by Morgan et al.<ref name=":8">{{cite journal|last1=Bhattacharya|first1=JJ|last2=Luo|first2=CB|last3=Suh|first3=DC|last4=Alvarez|first4=H|last5=Rodesch|first5=G|last6=Lasjaunias|first6=P|title=Wyburn-Mason or Bonnet-Dechaume-Blanc as Cerebrofacial Arteriovenous Metameric Syndromes (CAMS). A New Concept and a New Classification.|journal=Interventional neuroradiology : journal of peritherapeutic neuroradiology, surgical procedures and related neurosciences|date=30 March 2001|volume=7|issue=1|pages=5–17|pmid=20663326}}</ref> They combined intracranial resection, ligation of ophthalmic artery, and selective arterial ligature of the [[external carotid artery]], but the patient did not have retinal vascular malformations.<ref name=":6">{{cite journal|last1=Lester|first1=Jacobo|last2=Ruano-Calderon|first2=Luis Angel|last3=Gonzalez-Olhovich|first3=Irene|title=Wyburn-Mason Syndrome|journal=Journal of Neuroimaging|date=July 2005|volume=15|issue=3|pages=284–285|doi=10.1111/j.1552-6569.2005.tb00324.x}}</ref>


If lesions are present, they are watched closely for changes in size. Prognosis is best when lesions are less than 3&nbsp;cm in length. Most complications occur when the lesions are greater than 6&nbsp;cm in size.<ref name=":0">{{cite journal|last1=SINGH|first1=A|last2=RUNDLE|first2=P|last3=RENNIE|first3=I|title=Retinal Vascular Tumors|journal=Ophthalmology Clinics of North America|date=March 2005|volume=18|issue=1|pages=167–176|doi=10.1016/j.ohc.2004.07.005|url=http://www.ophthalmology.theclinics.com/article/S0896-1549(04)00088-4/fulltext}}</ref> Surgical intervention for intracranial lesions has been done successfully. Nonsurgical treatments include [[embolization]], radiation therapy, and continued observation.<ref name=":3">{{cite journal|last1=Dayani|first1=P. N.|last2=Sadun|first2=A. A.|title=A case report of Wyburn-Mason syndrome and review of the literature|journal=Neuroradiology|date=18 January 2007|volume=49|issue=5|pages=445–456|doi=10.1007/s00234-006-0205-x}}</ref> Arterial vascular malformations may be treated with the [[cyberknife]] treatment. Possible treatment for cerebral arterial vascular malformations include stereotactic radiosurgery, endovascular embolization, and microsurgical resection.<ref name=":0" />
If lesions are present, they are watched closely for changes in size. Prognosis is best when lesions are less than 3&nbsp;cm in length. Most complications occur when the lesions are greater than 6&nbsp;cm in size.<ref name=":0">{{cite journal|last1=SINGH|first1=A|last2=RUNDLE|first2=P|last3=RENNIE|first3=I|title=Retinal Vascular Tumors|journal=Ophthalmology Clinics of North America|date=March 2005|volume=18|issue=1|pages=167–176|doi=10.1016/j.ohc.2004.07.005|url=http://www.ophthalmology.theclinics.com/article/S0896-1549(04)00088-4/fulltext}}</ref> Surgical intervention for intracranial lesions has been done successfully. Nonsurgical treatments include [[embolization]], radiation therapy, and continued observation.<ref name=":3">{{cite journal|last1=Dayani|first1=P. N.|last2=Sadun|first2=A. A.|title=A case report of Wyburn-Mason syndrome and review of the literature|journal=Neuroradiology|date=18 January 2007|volume=49|issue=5|pages=445–456|doi=10.1007/s00234-006-0205-x}}</ref> Arterial vascular malformations may be treated with the [[cyberknife]] treatment. Possible treatment for cerebral arterial vascular malformations include stereotactic radiosurgery, endovascular embolization, and microsurgical resection.<ref name=":0" />


When pursuing treatment, it is important to consider the size of the malformations, their locations, and the neurological involvement.<ref name=":6" /> Because it is a congenital disorder, there are not preventative steps to take aside from regular follow ups with a doctor to keep an eye on the symptoms so that future complications are avoided.
When pursuing treatment, it is important to consider the size of the malformations, their locations, and the neurological involvement.<ref name=":6">{{cite journal|last1=Lester|first1=Jacobo|last2=Ruano-Calderon|first2=Luis Angel|last3=Gonzalez-Olhovich|first3=Irene|title=Wyburn-Mason Syndrome|journal=Journal of Neuroimaging|date=July 2005|volume=15|issue=3|pages=284–285|doi=10.1111/j.1552-6569.2005.tb00324.x}}</ref> Because it is a congenital disorder, there are not preventative steps to take aside from regular follow ups with a doctor to keep an eye on the symptoms so that future complications are avoided.


== References ==
== References ==

Revision as of 17:14, 4 June 2019


Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Jyostna Chouturi, M.B.B.S [2]

Synonyms and keywords: Wyburn mason's syndrome; Retinoencephalofacial angiomatosis

CT scan showing intracranial hemorrhage

Overview

.

Signs and symptoms

Causes

Mechanism

Epidemiology

Diagnosis

Treatment

If lesions are present, they are watched closely for changes in size. Prognosis is best when lesions are less than 3 cm in length. Most complications occur when the lesions are greater than 6 cm in size.[1] Surgical intervention for intracranial lesions has been done successfully. Nonsurgical treatments include embolization, radiation therapy, and continued observation.[2] Arterial vascular malformations may be treated with the cyberknife treatment. Possible treatment for cerebral arterial vascular malformations include stereotactic radiosurgery, endovascular embolization, and microsurgical resection.[1]

When pursuing treatment, it is important to consider the size of the malformations, their locations, and the neurological involvement.[3] Because it is a congenital disorder, there are not preventative steps to take aside from regular follow ups with a doctor to keep an eye on the symptoms so that future complications are avoided.

References

  1. 1.0 1.1 SINGH, A; RUNDLE, P; RENNIE, I (March 2005). "Retinal Vascular Tumors". Ophthalmology Clinics of North America. 18 (1): 167–176. doi:10.1016/j.ohc.2004.07.005.
  2. Dayani, P. N.; Sadun, A. A. (18 January 2007). "A case report of Wyburn-Mason syndrome and review of the literature". Neuroradiology. 49 (5): 445–456. doi:10.1007/s00234-006-0205-x.
  3. Lester, Jacobo; Ruano-Calderon, Luis Angel; Gonzalez-Olhovich, Irene (July 2005). "Wyburn-Mason Syndrome". Journal of Neuroimaging. 15 (3): 284–285. doi:10.1111/j.1552-6569.2005.tb00324.x.