1/14. Anterior sacral meningocele as a pelvic complication of marfan syndrome.Anterior sacral meningocele (ASM) is well recognized in marfan syndrome as a consequence of dural ectasia. Two cases presenting as nongynaecological pelvic masses are described highlighting the clinical difficulty in diagnosis and the classical radiological findings. The classification of ASM and associated anomalies of the sacrum are reviewed.- - - - - - - - - - ranking = 1keywords = meningocele (Clic here for more details about this article) |
2/14. Anterior sacral meningocele in a patient with marfan syndrome.Anterior sacral meningocele has been reported to be associated with marfan syndrome (MFS) in few cases, differently from dural ectasia appearing up to two thirds of affected patients. A new instance of this association is described in an 18-year-old man with marfan syndrome, diagnosed upon MRI morphological evaluation which showed a huge cystic mass in the pelvic space. Surgical excision even if curative was not performed in consideration of a stationary picture after one year since diagnosis.- - - - - - - - - - ranking = 1keywords = meningocele (Clic here for more details about this article) |
3/14. Herniation of mitral leaflets in the ehlers-danlos syndrome.A 47 year old patient with the ehlers-danlos syndrome (hypermobility of the joints, hyperextensibility of the skin and atrophic cutaneous scars) was evaluated because of a mitral regurgitant murmur and paroxysmal atrial tachycardia. angiocardiography and echocardiography demonstrated marked systolic herniation of the posterior leaflets of the mitral valve. An echocardiogram from the patient's daughter, who also had paroxysmal atrial tachycardia, revealed the same abnormality. Although a "floppy" valve is common in Marfan's syndrome, this report emphasizes that it may also occur in patients with other heritable disorders of connective tissue.- - - - - - - - - - ranking = 0.0057941512716157keywords = herniation (Clic here for more details about this article) |
4/14. Dural ectasia and back pain: review of the literature and case report.Dural ectasia is defined as a ballooning of the dural sac which is more common in patients with connective tissue disorders such as the marfan syndrome. Several studies have shown that dural ectasia may be associated with such conditions as back pain, headaches, radiculopathies, or incontinence. We present a case of a 52 year old woman with marfan syndrome who presented with a significantly large anterior sacral meningocele without having associated symptoms. In light of this case, we recommend that asymptomatic Marfan patients with dural ectasia should be closely observed without need for immediate surgical intervention.- - - - - - - - - - ranking = 0.2keywords = meningocele (Clic here for more details about this article) |
5/14. Diaphragmatic hernia.We describe a case of acute dyspnea in a patient with marfan syndrome secondary to bowel herniation into the thoracic cavity.- - - - - - - - - - ranking = 0.0057941512716157keywords = herniation (Clic here for more details about this article) |
6/14. prenatal diagnosis of intrathoracic stomach (gastric herniation).Intrathoracic stomach is a rare and serious congenital abnormality. The anomaly may be complicated by gastric volvulus and can lead to ischemic gastric infarction in the neonate. If diagnosed antenatally, neonatal management can be planned in advance so as to reduce morbidity. This anomaly must be differentiated from the more common congenital diaphragmatic hernia, as associated pulmonary hypoplasia is common in the latter and rare with gastric herniation. We report an infant born to a mother with Marfan's syndrome with the antenatal diagnosis of intrathoracic stomach. The ultrasound and magnetic resonance imaging features of this congenital abnormality are described. A review of the literature would indicate that this is the first case report of gastric volvulus diagnosed in utero.- - - - - - - - - - ranking = 0.028970756358079keywords = herniation (Clic here for more details about this article) |
7/14. Endoscopic cure of a giant sacral meningocele associated with Marfan's syndrome: case report.An unusual anterior sacral meningocele associated with Marfan's syndrome, with demonstrated dural anomalies related to Marfan's syndrome is reported. endoscopy enabled complete exploration of the meningocele, its morphological description, and ultimate cure. The meningocele resembled an hourglass made up of intrasacral and anterior sacral components. The communication of this bilobed malformation with the dural sac was so small that only the intrathecal injection of a colored solution allowed its localization. The closure of this communication was accomplished under endoscopic view by a single stitch. The postoperative course of the patient was characterized by immediate disappearance of the preoperative clinical signs. Computed tomographic scans demonstrated progressive complete collapse of the anterior sacral meningocele and partial resolution of the intrasacral component. Dural anomalies of Marfan's syndrome are described, and their relationship to the formation of the meningocele is discussed.- - - - - - - - - - ranking = 1.8keywords = meningocele (Clic here for more details about this article) |
8/14. Chiari-like tonsillar herniation associated with intracranial hypotension in marfan syndrome. Case report.The authors describe the case of a 12-year-old girl with marfan syndrome, sacral dural ectasia, and tonsillar herniation, who presented with headache. Initially, it was hypothesized that the headaches were secondary to the tonsillar herniation, and the patient consequently underwent surgical decompression of the foramen magnum. Postoperatively, the patient's condition did not improve, and additional magnetic resonance (MR) imaging demonstrated evidence of a cerebrospinal fluid (CSF) leak at the level of the dural ectasia. It was surmised that the girl's symptoms were due to spontaneous intracranial hypotension (SIH) and that the tonsillar herniation was caused by the leakage. The patient responded well to application of a blood patch at the level of the demonstrated leak, and her headache resolved. This appears to be the first reported case of a patient with marfan syndrome presenting with a symptomatic spontaneous CSF leak complicated by tonsillar herniation. In this rare association of SIH and connective tissue disorders, recognition of the clinical signs and typical MR imaging features of SIH may lead to more appropriate and less invasive treatment, potentially avoiding surgery.- - - - - - - - - - ranking = 0.046353210172926keywords = herniation (Clic here for more details about this article) |
9/14. Case report 582: Lumbosacral meningocele and aortic aneurysm in marfan syndrome.A patient with marfan syndrome presented with enlargement of lumbosacral spinal canal with a bulky meningocele, and a fusiform aneurysm of the ascending aorta. In marfan syndrome, the presence of a meningocele is extremely rare, but 60% of the patients develop cardio-vascular complications (a frequent cause of death). MRI is the imaging technique of choice for the diagnosis of meningocele and aortic aneurysm.- - - - - - - - - - ranking = 1.4keywords = meningocele (Clic here for more details about this article) |
10/14. Sacral abnormalities in marfan syndrome.Five women with marfan syndrome had abnormalities of the sacrum disclosed by computed tomography. Each had evidence of expansion of the central sacral spinal canal and enlargement of sacral foramina associated with extensive bony erosion. Three patients complained of low back pain, two were asymptomatic. Sacral meningoceles were demonstrated in three cases (including two of the three with symptoms). A fourth patient had a thoracic meningocele. The sacral abnormalities are attributed to defective collagen resulting in expansion of weakened dura which leads to pressure erosion of contiguous osseous structures.- - - - - - - - - - ranking = 0.4keywords = meningocele (Clic here for more details about this article) |
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