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Hunter disease/mucopolysaccharidosis type II/iduronate sulfatase deficiency
Published in William L. Nyhan, Georg F. Hoffmann, Aida I. Al-Aqeel, Bruce A. Barshop, Atlas of Inherited Metabolic Diseases, 2020
Hearing loss is regularly observed. Retinal dysfunction may be documented by electroretinography [35]. Chronic papilledema has been reported in the absence of increased intracranial pressure [36, 37]. Hydrocephalus appears to be rare in the mild forms of Hunter disease [28]. Arachnoid cysts have been observed [38]. Spinal stenosis, especially cervical may cause cord compression [39].
Intracranial Cysts
Published in Amar Bhide, Asma Khalil, Aris T Papageorghiou, Susana Pereira, Shanthi Sairam, Basky Thilaganathan, Problem-Based Obstetric Ultrasound, 2019
Amar Bhide, Asma Khalil, Aris T Papageorghiou, Susana Pereira, Shanthi Sairam, Basky Thilaganathan
Arachnoid cysts are rare cysts arising from the arachnoid and contain cerebrospinal fluid. They do not communicate with the ventricular system. They are most often isolated, regular, and non-midline. The size can be very variable. Midline shift of the brain may be seen due to pressure effects. If they are isolated, the outlook is usually good, unless the size is very large.
Arachnoid cyst
Published in Alisa McQueen, S. Margaret Paik, Pediatric Emergency Medicine: Illustrated Clinical Cases, 2018
This patient has an arachnoid cyst. Arachnoid cysts are fluid-filled sacs around the spinal cord or (more commonly) inside the head. These cysts are often present at birth but can also be a secondary result of head trauma. Arachnoid cysts are diagnosed with a CT or an MRI.
Management of quadrigeminal arachnoid cyst associated with obstructive hydrocephalus: report on stereotactic ventricular - cystic stenting
Published in British Journal of Neurosurgery, 2019
Yosef Laviv, Sergio Neto, Ekkehard M. Kasper
Arachnoid cysts [AC] are developmental abnormalities presenting as fluid filled cystic structures embedded in the arachnoid mater. While it is generally accepted that asymptomatic cases do not require any intervention, controversy still remains regarding the preferred treatment for symptomatic lesions. Management of ACs is further complicated by the presence of hydrocephalus. The prevalence of hydrocephalus associated with ACs is varied, ranging from 9%–66%. However, this association is location dependent, as the prevalence can be as high as 100% in cases of suprasellar ACs (foramen of Monroe obstruction), quadrigeminal ACs (QAC; aqueduct of Sylvius obstruction) and infratentorial ACs (4th ventricle obstruction).1–3
Pathogenesis, management strategies, and outcome of non-communicating extradural spinal arachnoid cyst (NEAC): a systematic review
Published in British Journal of Neurosurgery, 2023
Mohammad Shahidul Islam Khan, Nazmin Ahmed, Kanak Kanti Barua, Bipin Chaurasia, Atul Vats, Atul Goel
For asymptomatic patients, conservative treatment with observation and follow up is recommended.8,44,47 Complete surgical excision with primary dural closure or duraplasty is the treatment of choice for symptomatic lesion.36,49 Simple cyst drainage possesses a high recurrence rate with temporary relief only. If the cyst does not communicate with the subarachnoid space, complete excision can be performed without subsequent repair of the dural defect (Figure 5). In presence of communicating pedicle, onlay dural grafting by muscle patch, augmented by fibrin glue should be considered before taking decision regarding cysto peritoneal shunt. On the other hand, when dural defect is large and not amenable to repair then cysto peritoneal shunt is a good option.41 Tanaka et al. reported an extradural arachnoid cyst in the lumbosacral region for which they managed the case by ligating the pedicle and marsupialize the cyst.47 Sometimes, cyst wall tightly adhere with nerve root, in that case cyst has to be fenestrated or marsupialize to relieve the compression. Now a days, minimally invasive surgery gains popularity for treating arachnoid cysts. Ying et al. reported a case of communicating variety of spinal arachnoid cyst where they injected contrast medium into subarachnoid space under digital subtraction angiography (DSA) and disclosed the fistula.49 Confirming the location of fistula enabled them to perform minimally invasive surgery to ligate the fistula. Besides this, often large cyst required extensive laminectomy/laminoplasty (Figure 6).50
Spinal intradural arachnoid cyst associated with diastematomyelia in an adult: a case report and review of literature
Published in British Journal of Neurosurgery, 2023
This case was a diagnostic dilemma. There were two possibilities to be considered. The cystic lesion could be a cystic degeneration in the spinal cord due to ischaemic necrosis, which occurs in untreated tethered cord. The second possibility was of an extramedullary lesion compressing the cord. The clinical picture did not favour an intramedullary lesion as there were no disturbance of bladder and bowel function and the sacral sensation and anal tone were normal. On MRI, the axial section showed two hemi-cords above the level of the lesion. However, at the level of the lesion the cyst the left hemi-cord was barely discernible. The diagnosis of an arachnoid cyst was only confirmed intra-operatively.