Cardiac involvement is common in primary systemic vasculitides and may be due to direct effect of the disease on the heart or due to therapy. We shall review involvement of the heart in the various forms of primary systemic vasculitis. Among anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV), eosinophilic granulomatosis with polyangiitis most commonly involves the heart. Involvement of the heart confers poorer prognosis in AAV, which is also complicated by increased risk of cardiovascular events. Kawasaki's disease (KD) is the most common form of medium-vessel vasculitis to affect the heart, with coronary artery aneurysms being the most common manifestation. These predispose patients with KD to develop premature ischemic heart disease. Takayasu's arteritis is the most common large-vessel vasculitis to involve the heart and can result in aortic incompetence, myocarditis, or coronary heart disease. Involvement of the heart in Behcet's disease is usually in the form of intracardiac mass lesions, thrombosis, or endomyocardial fibrosis. Drugs used in the treatment of systemic vasculitis influence the risk of developing cardiovascular events. Corticosteroid therapy has been shown to increase the risk of myocardial infarction, whereas methotrexate, azathioprine, mycophenolate mofetil, rituximab, and anti-tumor necrosis alpha agents favorably modulate the risk of cardiovascular events, predominantly by dampening systemic inflammation. Awareness of cardiac involvement in vasculitis and accelerated cardiovascular risk in these patients should help clinicians to maximize the modulation of modifiable risk factors for heart disease in these individuals.

Symptomatic canal stenosis at the level of atlas (C1) without atlantoaxial dislocation is thought to be very rare in children. Though common, anomalies of the arch of atlas are generally incidental findings in X-rays. High cord compression due to a narrow canal from a bifid posterior arch, or an absent posterior arch, is a very rare condition. We report 5 children with high cord compression from stenosis of Cl arch.

We report a case of cervical intramedullary cystic schwannoma associated with segmental syrinx in a young adult without evidence of neurofibromatosis. The relevant literature is reviewed.

Neurenteric cysts are rare congenital lesions of the spine that are lined with entodermal epithelium. We describe 4 unusual cases of neurenteric cysts in patients aged between 3 and 16 years. The cyst was situated anteriorly in 3 patients and posteriorly in 1. All the patients had varied clinical and radiological features. The child with dorsal neurenteric cyst had stigmata of spinal dysraphism along with other vertebral anomalies. Another child had an associated syringomyelia along with evidence of severe arachnoiditis. All 4 patients underwent laminectomy and total microsurgical excision of the cysts through a posterior approach. The patient with diastematomyelia developed early recurrence of the cyst and underwent reoperation. Three patients showed an excellent recovery after surgery. The child with arachnoiditis failed to recover neurologically.