Pilomyxoid astrocytomas (PMA) is a particular form of glial tumors distinct from pilocytic astrocytomas (PA). On the last 2016 WHO classification for CNS tumours, no definite grade assignment was proposed for these lesions. They may be more aggressive with a different clinical course compared to PA due to their greater propensity for local recurrence and cerebrospinal dissemination. Most cases arise from the hypothalamic region. Only few studies reported cerebellar localization of the lesion. We report 3 pediatric cases treated for pediatric PMA of the posterior fossa. Clinical, radiological, and prognostic features were reviewed. The age of our patients was between 1 and 9 years old. Signs of intracranial hypertension were found in all patients. One of them presented an increased head circumference and the 2 others had a cerebellar syndrome. Brain CT-scan and MRI displayed a large wellcircumscribed intra-axial solid and cystic posterior fossa tumor. Total surgical resection was performed for all tumors. After a 2 years follow up, no signs of recurrence were noticed. In the literature, PMA been reported with overwhelming majority in children aged between 2 months and 4 years. Despite of many pathological similarities with PAs, PMAs have some specific features in histology, leading to their identification as independent type of glioma. Radiological differential diagnosis between PMAs and Pas can be made using arterial spin labeling imaging, which shows low perfusion parameters in PAs. Clinical and radiological follow up are mandatory do to different natural history and higher rates of local recurrence of this tumor compared to PA. Prognosis is favorable when complete surgical exeresis is possible.
BackgroundSpinal tuberculosis (Pott’s disease) is the most common as well as one of the most dangerous forms of skeletal tuberculosis and accounts for 50% of all cases of skeletal tuberculosis. Pott’s disease is still common in developing countries. Although the thoracolumbar junction seems to be the most common site of the spinal column involvement, cervical localization is scarce and accounts for 2% to 5% of spinal tuberculosis. Furthermore, the incidence of neurologic complications in spinal tuberculosis varies from 10% to 43%.ObjectivesThe purpose of this study was to perform an updated review and present our experience with 5 cases of tuberculosis of cervical spine, including clinical characteristics, diagnostic modalities and management of spinal tuberculosis.MethodsA review of 5 cases of cervical Pott’s disease collected at the Department of Neurosurgery of National Institute of Neurology of Tunis over a period of 2 years, between 2011 and 2012 and an updated literature review.ResultsThe average age of our patients was 35 years old with extremes ranging from 16 to 63 years old. There is a slight male predominance. The diagnostic delay is on average 6 months. The clinical manifestations were dominated by cervical pain (4 cases) and progressive spinal cord compression syndrome (3 cases). The biological inflammatory syndrome is found in only one patient. The intra-dermal reaction to tuberculin is positive in 4 patients. X-ray of the cervical spine, CT scan and magnetic resonance imaging were performed in all patients. All patients underwent a surgical resection. The medical treatment was administered to all our patients. The evolution was favourable, clinically and biologically, under anti tubercular treatment.Tuberculous spondylodiscitis remains a major global public health problem in endemic countries that affects mostly young adults in their most productive years. Thoracolumbar junction seems to be the most common site of the spinal column involvement in spinal tuberculosis (95%) and cervical spine is concerned in only 5% of cases. The delayed diagnosis, between 3 and 20 months, explains the frequency of neurological deficits which are found in proportions of 20% to 40%. For the diagnosis of spinal tuberculosis, magnetic resonance imaging is more sensitive imaging technique than x-ray and more specific than computed tomography. Antituberculous treatment remains the cornerstone of treatment. Surgery may be required in selected cases. With early diagnosis and early treatment, prognosis is generally good.ConclusionsCervical Pott’s disease is a rare localization. The diagnosis is easy in front of the cervical signs. The conservative management of cervical spine immobilisation and antitubercular chemotherapy remains a sufficient attitude to healing. Surgery is reserved in case of neurological aggravation or spinal instability.References[1] Jain AK. Tuberculosis of the spine: a fresh look at an old disease. J Bone Joint Surg Br2010;92(7):905–13.[2] Jain AK, Dhammi IK. Tuberculosis of the spine: a review. Clin Ort...
International Journal of Case Reports and Images (IJCRI) is an international, peer reviewed, monthly, open access, online journal, publishing high-quality, articles in all areas of basic medical sciences and clinical specialties.Aim of IJCRI is to encourage the publication of new information by providing a platform for reporting of unique, unusual and rare cases which enhance understanding of disease process, its diagnosis, management and clinico-pathologic correlations. Von Hippel-Lindau (VHL) disease is a complex and systemic entity that can be discovered by neurological complications. Its association with other serious malformative disease like nutcracker syndrome (NCS) was not described, and its complex presentation must not make some other serious problem easily missed or neglected. Case Report: A 27-year-old male patient with 3 intramedullary hemangioblastoma of six months of insidious evolution complicated by medullary compression. The lesions were completely removed with excellent neurological postoperatory outcome. Thinking of VHL disease, our investigations were expanded and revealed multiple vermian and cerebellar hemangioblastomas, renal masses, and multiple pancreatic cysts. In addition, an incidentally found of posterior NCS during the abdominal computed tomography (CT) done for VHL disease investigations. Our attitude to both neurosurgical and urological problems (the serious presentation of VHL disease with multiple locations of hemangioblastoma and right renal carcinomas) and for the missed symptomatic posterior nutcracker syndrome were discussed. IJCRI publishes Conclusion:The complex presentation of the VHL disease must not makesome other serious problems easily missed or neglected like renal carcinoma or nutcracker syndrome as seen in our case.
BackgroundAneurysmal bone cysts (ABC) are rare, benign, highly vascular pseudotumors of unknown cause. It most often affects individuals during their second decade of life.ObjectivesOur goal was to document the clinical characteristics, diagnostic modalities and treatment results of ABCs.MethodsWe reviewed our institution’s database over a period of 15 years to identify patients diagnosed with aneurysmal bone cysts of the spine. Four patients underwent surgery in our department (2 men and 2 women). For those four patients, we tabulated the clinical characteristics, location, diagnostic modalities and treatment.ResultsThe clinical manifestations were gait disturbance in 2 patients and legpain in the other two. The tumour occurred in the dorsal spine in 2 cases and in the lumbar spine in the other two. All patients underwent surgical resection with total removal in only 2 patients. Postoperatively, clinical signs improved in all patients. Only one case presented tumour recurrence requiring second intervention and instrumentation.ABCs constitute approximately 1.5% of spinal bone tumours. They usually affect the posterior elements of the spine. MRI is the most useful modality for preoperative planning. It also helps to evaluate the fluidfluid level, which is characteristic for ABC on MRI. The differential diagnosis is mainly with giant cell tumours and osteoblastomas. The primary option for treatment is surgery. Instrumentation is sometimes necessary because of the increased risk of postoperative instability, especially in cervical spine surgery. The recurrence rate is 20% to 30% in case of incomplete resection.ConclusionsABCs are benign lesions. Surgical resection en bloc has the lowest recurrence rate. Instrumentation is sometimes necessary because of the increased risk of postoperative instability.References[1] Boriani S, De Iure F, Campanacci L, et al. (2001) Aneurysmal bone cyst of the mobile spine: report on 41 cases. Spine. 26:27–35[2] Pennekamp W, Peters S, Schinkel C, Kuhnen C, Nicolas V, Muhr G, Frangen TM (2008) Aneurysmal bone cyst of the cervical spine. Eur Radiol18:2356–2360[3] Sundaram M, McDonald DJ, Steigman CK, Bocchini T (1997) Metachronous multiple aneurysmal bone cysts. Skeletal Radiol26(9):564–567[4] Keenan S, Bui-Mansfield LT (2006) Musculoskeletal lesions with fluid–fluid level: a pictorial essay. J Comput Assist Tomogr30:517–524[5] Saccomanni R (2008) Aneurysmal bone cyst of spine: a review of literature. Arch Orthop Trauma Surg128:1145– 1147Disclosure of InterestNone declared
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