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Background Spinal arteriovenous malformations (AVM) consist of a heterogeneous group of pathological vascular entities that affect the spinal cord parenchyma either directly or indirectly. Case presentation We present an unusual case of spinal arteriovenous malformation (type 2) in an 18-month-old girl who presented with weakness of both lower limbs and urinary incontinence. She was diagnosed with a spinal AVM with large intramedullary nidus and paraspinal extension which was managed with endovascular embolization. Conclusion Spinal cord AVM in children can be debilitating. If presented early, patient can be taken up for embolization, which is a relatively safe procedure with better neurological outcome. Our case illustrates the successful role of interventional radiology in the treatment of this rare condition.

Objectives: Paediatric neuroradiology is one of the most challenging areas in the wide gamut of disciplines that modern radiology encompasses. There is a paucity of literature on the epidemiology of paediatric neuroimaging and contextual reporting in this field. The objectives of this study were to study the epidemiology of the paediatric neurological disorders and to study the role of contextual reporting in this field. Materials and methods: This study was conducted at a tertiary care centre in Southwestern India over 1 year. It was a retrospective epidemiological study. The authors studied 112 patients referred as in- or outpatients for a brain MRI for a wide range of indications. The authors analysed the reports issued by their radiologists and reformatted them into a newly proposed contextual reporting template for the paediatric brain. Then, the authors conducted an epidemiological analysis of the compiled data. Results: The authors found that the most common indication for paediatric neuroimaging was seizures or seizure-like episodes, followed by developmental delay. The most common abnormality on imaging was sequelae to hypoxic or hypoglycaemic insult followed by brain atrophy. The authors found a wide range of other abnormalities illustrating the wide spectrum of paediatric neuroradiology. Conclusion: The authors’ study fills a gap in current literature regarding the epidemiology of conditions encountered in paediatric neuroradiology. The authors also propose a novel reporting format for contextual reporting in this field, which may help in reducing errors in reporting and reduce reporting time.

IgG4-related disease (IgG4-RD) is an emerging disease concept that was first recognized in the 21st century. It has since then been attracting substantial attention in many fields of medicine as it tends to involve either synchronously or metachronously, various organs, including the pancreas, bile duct, lacrimal gland, salivary gland, and many others. This umbrella diagnosis now successfully explains a substantial number of disorders, which were previously regarded as “idiopathic.” Reports of the manifestations of IgG4-RD in the head and neck are extremely rare. Otologic manifestations have been reported, but only a handful of cases are available in literature that have been confirmed by immunohistopathology. We, herein, present a case of IgG4-RD of the middle ear, which manifested as a growth in the left mastoid and middle ear cavity, and became symptomatic with symptoms of chronic otitis media in the left ear, and was subsequently diagnosed to have IgG4-RD.

Kimura disease (KD) is a rare chronic inflammatory disorder of unknown cause, primarily seen in young Asian males. The disease is characterized by painless subcutaneous swelling in head and neck region, accompanied by regional lymphadenopathy and frequent salivary gland enlargement. Blood and tissue eosinophilia, and elevated immunoglobulin E (IgE) levels, are common associations. Characteristic histopathological findings of biopsy specimens obtained from the subcutaneous swellings or lymph nodes include eosinophilic infiltrates, follicular hyperplasia, and proliferation of postcapillary venules. The course is usually waxing and waning, albeit benign. Early diagnosis may spare the patient from unnecessary invasive procedures. We herein, describe a case of KD in an 18 years old male, who presented with subcutaneous swelling in the both cheek (right more than left) in 2014, underwent multiple surgical interventions for the same, before being reassessed and finally diagnosed as KD in 2020, based upon peripheral blood eosinophilia, raised serum IgE levels and histopathological findings. He subsequently made a good recovery on oral steroids.

Anti-phospholipid syndrome is an autoimmune disorder characterized by episodes of arterial and/or venous thrombosis and/or pregnancy morbidity in the presence of anti-phospholipid antibodies. Catastrophic anti-phospholipid syndrome is an accelerated form of the disease with rapid involvement of multiple organ systems often posing a diagnostic challenge. There is a paucity of literature on the myriad presentations of catastrophic anti-phospholipid syndrome owing to the orphan nature of the disease. We present three cases of catastrophic anti-phospholipid syndrome in patients with systemic lupus erythematosus that presented with episodes of thrombosis involving both arterial and venous systems and multisystem organ failure. Timely diagnoses were made based on a high index of suspicion and were managed with a combination of systemic glucocorticoids, cyclophosphamide, plasmapheresis, intravenous immunoglobulin and other supportive measures. However, despite providing the standard of care, we encountered a poor outcome in two of these patients, highlighting the high mortality associated with catastrophic anti-phospholipid syndrome.