Anterior dural ectasia is an extremely rare finding in ankylosing spondylitis (AS). The authors describe a unique case of AS in which the patient presented with cauda equina syndrome as well as an unusual imaging finding of erosion of the posterior aspect of the L-1 (predominantly) and L-2 vertebral bodies due to anterior dural ectasia. Symptomatic patients with long-standing AS should be monitored for the presence of dural ectasia, which can be anterior in location, as is demonstrated in the present case.

Noninfectious cerebral aneurysms are rare in patients with congenital cyanotic heart disease. We present a patient with DiGeorge/velocardiofacial syndrome with a complex congenital cyanotic heart disease with a ruptured anterior communicating artery aneurysm. The 10-year-old child was managed by surgical clipping of the aneurysm. Surgical challenges included prominent veins in the Sylvian fissure, difficulty in differentiating arterial and venous bleed, and anesthetic risks. The patient recovered without any neurological deficits. This is the first report of a patient with 22q11.2 deletion syndrome, with a noninfectious cerebral aneurysm.

OBJECTIVE Moyamoya angiopathy (MMA) affects the distal internal carotid artery and is designated as moyamoya disease (MMD) when predisposing conditions are absent, or moyamoya syndrome (MMS) when it occurs secondary to other causes. The authors aimed to investigate the reason for this anatomical site predilection of MMA. There is compelling evidence to suggest that MMA is a phenomenon that occurs due to stereotyped mechanobiological processes. Literature regarding MMD and MMS was systematically reviewed to decipher a common pattern relating to the development of MMA. METHODS A systematic review was conducted to understand the pathogenesis of MMA in accordance with PRISMA guidelines. PubMed MEDLINE and Scopus were searched using “moyamoya” and “pathogenesis” as common keywords and specific keywords related to six identified key factors. Additionally, a literature search was performed for MMS using “moyamoya” and “pathogenesis” combined with reported associations. A progressive search of the literature was also performed using the keywords “matrix metalloprotease,” “tissue inhibitor of matrix metalloprotease,” “endothelial cell,” “smooth muscle cell,” “cytokines,” “endothelin,” and “transforming growth factor” to infer the missing links in molecular pathogenesis of MMA. Studies conforming to the inclusion criteria were reviewed. RESULTS The literature search yielded 44 published articles on MMD by using keywords classified under the six key factors, namely arterial tortuosity, vascular angles, wall shear stress, molecular factors, blood rheology/viscosity, and blood vessel wall strength, and 477 published articles on MMS associations. Information obtained from 51 articles that matched the inclusion criteria and additional information derived from the progressive search mentioned above were used to connect the key factors to derive a network pattern of pathogenesis. CONCLUSIONS Based on the available literature, the authors have proposed a unifying theory for the pathogenesis of MMA. The moyamoya phenomenon appears to be the culmination of an interplay of vascular anatomy, hemodynamics, rheology, blood vessel wall strength, and a plethora of intricately linked mechanobiological molecular mediators that ultimately results in the mechanical process of occlusion of the blood vessel, stimulating angiogenesis and collateral blood supply in an attempt to perfuse the compromised brain.

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Orbital cavernous hemangiomas (OCHs) are well encapsulated vascular lesions. They are amenable to dissection off the surroundings because they do not infiltrate the surrounding tissues. They are the third most common intraorbital lesion in the adult following lymphoid tumors and idiopathic orbital inflammation. 1-3 A spread pattern of contrast enhancement on dynamic CT or MRI favours OCHs. 4 An elderly lady presented with a history of protrusion of the right eye ball since 2 to 3 months. She also complained of increased tearing since 2 to 3 weeks. There was no history of diplopia or visual deterioration. Imaging revealed an intraconal lesion in the right eye which was inferomedial to the optic nerve. In view of the location, an endonasal transethmoidal approach was deemed most suitable. 5 We present a 2 dimensional video of the same. Informed consent was secured from the patient. The participants and any identifiable individuals consented to publication of his/her image. Histopathological examination revealed a cavernous hemangioma. Recovery was uneventful with complete resolution of proptosis.