Out of 330 adult Systemic Lupus Erythematosus (SLE) cases who attended the Rheumatic Care Centre, Government General Hospital, 59 children were analysed. There was no case with onset before the age of 5 years. There were 49 females and 110 males (M:F = 1:4.9). The initial manifestations were fever (67%), arthritis (61%), skin rash (59%) and lymphadenopathy (27.1%). There was no case of Raynaud's phenomenon. Only 10.1% of patients presented with thrombocytopenic purpura. In the cumulative clinical features, arthritis in 86.6%, fever in 79.8%, skin rash in 69.4%, lymphadenopathy in 61% and hepatosplenomegaly in 39.9% were observed. Renal involvement was seen in 49.1%, neuropsychiatric manifestations in 27.1%, pleuropulmonary in 22% and cardiac manifestations in 10.2%. Anaemia was seen in 50.8%, leukopenia in 18.4%, thrombocytopenia in 11.8%, ANA in 100%, anti-dsDNA in 92.3%, anti-Sm in 34.7%, anti-SSA in 38.5%, anti-SSB in 15.4%, ACL in 30.8%, low C3 in 50% and false positive VDRL in 3.3%. Death occurred in 8 children, 3 due to infection, 2 due to renal causes, 1 due to cardiac and 2 due to central nervous system involvement.
Purpose The management of cervical facet dislocation injuries remains controversial. The main purpose of this investigation was to identify whether a surgeon's geographic location or years in practice influences their preferred management of traumatic cervical facet dislocation injuries. Methods A survey was sent to 272 AO Spine members across all geographic regions and with a variety of practice experience. The survey included clinical case scenarios of cervical facet dislocation injuries and asked responders to select preferences among various diagnostic and management options. Results A total of 189 complete responses were received. Over 50% of responding surgeons in each region elected to initiate management of cervical facet dislocation injuries with an MRI, with 6 case exceptions. Overall, there was considerable agreement between American and European responders regarding management of these injuries, with only 3 cases exhibiting a significant difference. Additionally, results also exhibited considerable management agreement between those with ≤ 10 and > 10 years of practice experience, with only 2 case exceptions noted. Conclusion More than half of responders, regardless of geographical location or practice experience, identified MRI as a screening imaging modality when managing cervical facet dislocation injuries, regardless of the status of the spinal cord and prior to any additional intervention. Additionally, a majority of surgeons would elect an anterior approach for the surgical management of these injuries. The study found overall agreement in management preferences of cervical facet dislocation injuries around the globe.
Background Arachnoid cysts are benign, non-neoplastic fluid collections within the arachnoid mater layer of the meninges. The etiology and significance of arachnoid cysts are poorly understood. Although they frequently represent incidental findings on central nervous system imaging, a wide variety of conditions have been attributed to their presence. The aim of this study is to ascertain the clinical presentation, location, and clinical course of patients with arachnoid cysts in the institution. Methods The authors analyzed the clinical presentation, radiologic images, and clinical course of 16 patients presented over a period of 6 months from August 2017 to January 2018. Results Of these 16 patients, 11 were adults and 5 were pediatric patients. Of these, seven were female and the remaining nine were male. Three patients presented with seizures, seven with headache, two with developmental delay, one with hydrocephalus, one with giddiness, one with hard of hearing, and one with bulging posterior fontanelle. Of these, 6 underwent surgery and 10 were managed conservatively. Conclusion Arachnoid cysts (non-neoplastic lesions) that produce symptoms through mass effect and obstructive hydrocephalus need surgical management, whereas a large percentage of cysts that are asymptomatic can be managed conservatively. The various surgical options available are marsupialization, cystoperitoneal shunt, ventriculoperitoneal (VP) shunt, and endoscopic fenestration. Microsurgery and endoscopy both allow for rapid decompression of the cyst and restoration of cerebrospinal fluid (CSF) circulation, whereas shunting tends to produce greater reduction in cyst size. Timing of presentation also decides upon the treatment options. Patients presenting late after the establishment of neurocognitive decline may not benefit from surgery and can be managed conservatively. A detailed genetic study may aid in the evolution of newer prevention and management options. Currently, most pediatric neurosurgeons prefer to avoid shunting, if possible. However, treatment paradigms are operator dependent and produce comparable outcomes.
Introduction The Peripheral Primitive Neuroectodermal Tumour (pPNET)/Ewing's sarcoma family tumor (ESFT) group is a recently redefined term which includes all small round cell tumors of bone, soft tissue, and nerve with a common neuroectodermal origin. These highly aggressive pediatric soft tissue tumors occur less frequently in the craniospinal axis. Primary cranial lesion associated with spinal and pelvic metastasis is a rare presentation. Clinical Presentation A 9-year old girl was hospitalized with features of progressive increase in intracranial pressure with quadriparesis. The patient had ⅘ quadriparesis with bilateral papilledema. Brain MRI showed a large, hetero intense, contrast enhancing right parietal bony lesion infiltrating the dura with mass effect over the brain. Brain CT showed bony expansion and multiple calvarial lytic lesions. MRI cervical spine revealed collapsed fourth cervical vertebral body with extensive paravertebral and paraspinal soft tissue components with cord compression. Metastatic workup revealed a lytic lesion in the left iliac bone and left lung. The patient underwent a surgically challenging C4 corpectomy with cage fixation followed by craniotomy and excision of parietal bony lesion with titanium mesh cranioplasty in the same sitting. Histopathology revealed pPNET and was confirmed by CD99 positivity. Conclusion This rare multiple site tumor presentation was carefully planned and effectively managed by a combined single-stage surgical approach involving total excision of primary parietal bone and metastatic cervical spine tumors with primary
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