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Preoperative Diagnosis and Management
Published in Alaaeldin (Alaa) Azmi Ahmad, Aakash Agarwal, Early-Onset Scoliosis, 2021
Computerised tomography (CT) scans are usually used as a preoperative radiological investigation to assess the bony anatomy. It is indicated in congenital scoliosis to assess vertebral failure of formation or segmentation and pedicle shape. Dural ectasia can be diagnosed in Marfan syndrome through CT by showing anterior meningoceles, wide interpediculate distance, vertebral scalloping, and increased sagittal diameter [3,15]. Dural ectasia was reported in 63% of Marfan syndrome patients [8].
Bones and joints
Published in Brian J Pollard, Gareth Kitchen, Handbook of Clinical Anaesthesia, 2017
Brian J Pollard, Gareth Kitchen
Significant scoliosis is common and spine deformity occurs early. Dural ectasia is common. Approximately 63% of patients were reported to have scoliosis and >50% kyphosis; 12% developed progressive scoliosis requiring surgery. Further complications may include instrumentation fixation failure, pseudarthrosis and curve decompensation.
Test Paper 1
Published in Teck Yew Chin, Susan Cheng Shelmerdine, Akash Ganguly, Chinedum Anosike, Get Through, 2017
Teck Yew Chin, Susan Cheng Shelmerdine, Akash Ganguly, Chinedum Anosike
A common cause of localised posterior vertebral scalloping is increased intraspinal pressure secondary to an expanding mass. Widening of the interpediculate distance and alteration of the configuration of the pedicles are associated signs. Relatively large, slow-growing lesions that originate during a period of active skeletal growth (such as ependymomas) are most likely to give rise to posterior vertebral scalloping. Dural ectasia is thought to cause posterior vertebral scalloping due to loss of the normal protection provided to the vertebral body by a strong, intact dura. Dural ectasia classically occurs in association with inherited connective-tissue disorders such as Marfan syndrome (classical) and Ehlers–Danlos syndrome. Posterior vertebral scalloping is also commonly seen in patients with neurofibromatosis, most likely due to dural ectasia but also secondary to neurofibromas or a thoracic meningocoele. It has also been reported in patients with AS; in these cases, the development of associated arachnoid cysts may give rise to cauda equina syndrome. Acromegaly has been described as a further cause of diffuse posterior vertebral scalloping, probably because of a combination of soft-tissue hypertrophy in the spinal canal and increased bone resorption.
Anesthetic management for cesarean section and tubal ligation in a patient with Marfan syndrome, multiple sclerosis, and multiple postdural puncture headaches
Published in Baylor University Medical Center Proceedings, 2018
Michael P. Hofkamp, Jacqueline M. Galvan
Patients with Marfan syndrome who have an aortic diameter >4 cm are at higher risk for aortic dissection.5 Our patient had an aortic diameter of 4.1 cm, was managed with beta-blockers, and was advised by her cardiologist not to become pregnant again. Epidural anesthesia has been recommended for patients with Marfan syndrome undergoing cesarean delivery due to its propensity to avoid large hemodynamic shifts.6 Patients with Marfan syndrome also have a predisposition for dural ectasia, which increases the risk of unintentional dural puncture.7
The association of spinal deformity with dural ectasia in neurofibromatosis type 1
Published in British Journal of Neurosurgery, 2019
A chi-squared test was used to determine the statistical significance of gender as a determinant of dural ectasia (M:F, 16:22). The association between dural ectasia and spinal deformity was tested. This was further analysed using MRI scans of all the patients with dural ectasia to observe the specific radiological characteristics of the dilated dural sac that resulted in spinal deformities.