Mesencephalo-oculo-facial-angiomatosis is seen in:
Correct Answer: Wyburn-Mason syndrome
Description: D i.e. Wyburn-Mason syndrome - Wyburn-Mason syndrome (or Bonnet-Dechaume Blanc syndrome mesencephalo-oculo-facial or mesencephalo-optico retinal angiomatosis syndrome) is characterized by neuro (mesencephalo) - optico / oculo / retinal - facial angiomatosis (vascular-malformations). - Wyburn-Mason syndrome presents with telangiectasia of skinQ (i.e cutaneous vascular nevi) on face + retinal cirsoid aneurysmQ and aerio-venous malformation (AVM) involving the visual pathways and midbrainQ (= entire optic tract = optic nerve, thalamus, geniculate bodies and calcarine coex). The lesions are typically unilateral mostly. It may be a/w AVMs of postrior fossa, neck, mandible/maxilla presenting in childhood. Rendu-Osler-Weber syndrome (hereditary hemorrhagic telangiectasia) is AD neurocutaneous syndrome that result in a variety of systemic fibrovascular dysplasia (i.e. telangiectasia, AVM, AV hemangioma/fistula, and aneurysm) affecting mucous membrane, skin, lung, brain and GI tract-Q. Telangiectasi is primarily found in the skin & mucous membranes. AVM and fistula are found manly in liver > brain > lung > spine. Aneurysm can invovle any size vessel. Frequent bleeding into mucous membrane, skin, lungs, genitourinary and gestrointestinal system is d/ t vascular weakness. Klippel-Trenaunay syndrome (KTS) is angio-osteo-hyperophy i.e. hyperophy of soft tissue & over growth of bone IR abnormalitie of finger/toes /limb and venous varicosities d/t large angiomatous nevus (AVM). Several KTS patients exhibit CNS findings of Sturg-Weber syndrome and are called Klippel-TrenaunayWeber syndrome. They exhibit cutaneous angiomata, soft tissue /bony hyperophy and leptomeningeal vascular malformation.
Category:
Radiology
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