1/31. Anaesthetic management of a woman who became paraplegic at 22 weeks' gestation after a spontaneous spinal cord haemorrhage secondary to a presumed arteriovenous malformation.A 19-yr-old woman developed a paraplegia with a T10 sensory level at 22 weeks' gestation. The spinal injury was caused by spontaneous bleed of a presumed arteriovenous malformation in the spinal cord. She presented for Caesarean section at term because of the breech position of her fetus. The successful use of a combined spinal epidural-regional anaesthetic is described and the risks of general and regional anaesthesia are discussed.- - - - - - - - - - ranking = 1keywords = arteriovenous malformation, malformation (Clic here for more details about this article) |
2/31. Our surgical approach towards the treatment of urethrocele and urethral fistula.A new technique, described in the text, has been elaborated inspired by that of Monseur (1968) for urethral techniques. It has been performed with success in three paraplegics and in one incomplete tetraparesis. The plastic reconstruction of the diseased part of the urethra after excision of the stenosis and a fistula or diverticula by rotation and fixation to the subcavernal groove creates, in fact, an enlarged neo-urethra rendering the recurrence of the primary lesion practically impossible. This technique, first applied to spinal cord injuries, has been extended with permanent success to other lesions, such as tuberculosis stenosis and malformations.- - - - - - - - - - ranking = 0.0054346852181686keywords = malformation (Clic here for more details about this article) |
3/31. Acute flank pain: an unusual presentation of a spinal AVM.The authors report the case of a 6-year-old boy with a spinal cord arteriovenous malformation (AVM) who presented with acute flank pain and a delayed onset of paraplegia. An early diagnosis of a spinal cord AVM was made difficult by the absence of neurological findings on initial evaluation. Included is a description of his clinical course, and the presentation of spinal AVMs to the emergency physician is discussed.- - - - - - - - - - ranking = 0.0054346852181686keywords = malformation (Clic here for more details about this article) |
4/31. Spinal dural arteriovenous malformation: a cause of myelopathy.Spinal vascular malformation is an uncommon but potentially treatable cause of myelopathy. We describe two cases of angiographically proven spinal vascular malformation in malaysia. The first case is a 47-year-old man who had a progressive myelopathy and the second a 60-year-old man with intermittent attacks of transient paraparesis leading to paraplegia. As the clinical presentation of spinal vascular malformation is variable, it should be considered as a cause of patients with myelopathy.- - - - - - - - - - ranking = 0.81630405565451keywords = arteriovenous malformation, malformation (Clic here for more details about this article) |
5/31. Intramedullary neurenteric cyst presenting as infantile paraplegia: a case and review.The authors report a case of a 3-month-old male child with paraplegia in whom magnetic resonance imaging (MRI) revealed a nonenhancing intramedullary cystic lesion extending from the level of D1 to D7 without any other associated anomaly. Intraoperatively, these findings were confirmed and the spinal cord was found to have splayed circumferentially into a papery thin rim. The patient underwent marsupialization of the cyst with subtotal excision of the cyst wall. Histopathological examination revealed ciliated pseudostratified columnar epithelium consistent with the diagnosis of a neurenteric cyst. Intramedullary neurenteric cysts are rare developmental malformations, and out of the 13 previously reported cases, only 3 were evaluated by MRI. This is the first case report in the literature of an intramedullary neurenteric cyst presenting as infantile paraplegia. In the present report, the embryology, etiopathogenesis, radiological imaging and management of this rare clinical entity are discussed and a detailed literature review is presented.- - - - - - - - - - ranking = 0.0054346852181686keywords = malformation (Clic here for more details about this article) |
6/31. Intrauterine west nile virus: ocular and systemic findings.PURPOSE: To report the first documented case of intrauterine transmission of west nile virus (WNV) with resulting congenital chorioretinal scarring and central nervous system malformation in a newborn. DESIGN: Case report. methods: Ophthalmic findings and laboratory data in an otherwise presumed healthy 2-day-old female are presented. The infant's mother developed paraplegia due to WNV during the second trimester of her pregnancy. The newborn's external and general physical examination were unremarkable. RESULTS: Ophthalmic examination disclosed marked chorioretinal changes, and magnetic resonance imaging of the brain demonstrated severe abnormalities. serology for WNV was positive. Other causes of congenital chorioretinal changes were ruled out with the appropriate serology. CONCLUSIONS: Intrauterine transmission of WNV may result in significant ocular and neurologic morbidity. Titers for this important and emerging viral pathogen should be obtained when standard serologies are negative in an infant with congenital chorioretinal scarring.- - - - - - - - - - ranking = 0.0054346852181686keywords = malformation (Clic here for more details about this article) |
7/31. Spinal cord arteriovenous malformation in a person with congenital lymphatic abnormalities.Spinal cord arteriovenous malformations have been described in association with a variety of congenital diseases affecting the vasculature, including klippel-trenaunay-weber syndrome, Rendu-Osler-Weber syndrome and others, but rarely in association with lymphatic abnormalities. We report the case of a young man with congenital lymphedema and arteriovenous malformations of one lower extremity and a spinal cord arteriovenous malformation. awareness of the possible presence of a central nervous system arteriovenous malformation in individuals with pre-existing arteriovenous and lymphatic abnormalities may be helpful in their diagnosis and management.- - - - - - - - - - ranking = 1.6keywords = arteriovenous malformation, malformation (Clic here for more details about this article) |
8/31. Allan-Herndon-Dudley syndrome and the monocarboxylate transporter 8 (MCT8) gene.Allan-Herndon-Dudley syndrome was among the first of the X-linked mental retardation syndromes to be described (in 1944) and among the first to be regionally mapped on the x chromosome (in 1990). Six large families with the syndrome have been identified, and linkage studies have placed the gene locus in Xq13.2. Mutations in the monocarboxylate transporter 8 gene (MCT8) have been found in each of the six families. One essential function of the protein encoded by this gene appears to be the transport of triiodothyronine into neurons. Abnormal transporter function is reflected in elevated free triiodothyronine and lowered free thyroxine levels in the blood. Infancy and childhood in the Allan-Herndon-Dudley syndrome are marked by hypotonia, weakness, reduced muscle mass, and delay of developmental milestones. Facial manifestations are not distinctive, but the face tends to be elongated with bifrontal narrowing, and the ears are often simply formed or cupped. Some patients have myopathic facies. Generalized weakness is manifested by excessive drooling, forward positioning of the head and neck, failure to ambulate independently, or ataxia in those who do ambulate. speech is dysarthric or absent altogether. Hypotonia gives way in adult life to spasticity. The hands exhibit dystonic and athetoid posturing and fisting. Cognitive development is severely impaired. No major malformations occur, intrauterine growth is not impaired, and head circumference and genital development are usually normal. behavior tends to be passive, with little evidence of aggressive or disruptive behavior. Although clinical signs of thyroid dysfunction are usually absent in affected males, the disturbances in blood levels of thyroid hormones suggest the possibility of systematic detection through screening of high-risk populations.- - - - - - - - - - ranking = 0.0054346852181686keywords = malformation (Clic here for more details about this article) |
9/31. [Foix-Alajouanine syndrome: case report.]In a 52-year-old woman, spinal arteriovenous malformation (AVM) has been associated with what has been known as Foix-Alajouanine syndrome. The pathophysiology of the AV fistula is probably related to increased venous pressure from the AVM plus thrombotic process. The most common initial symptoms are sensory disturbance, pain and leg weakness. Definitive diagnosis of spinal AVMs requires radiographic demonstration of the vascular anomaly. Nevertheless, in this case, suggestive defects of malformations could not be seen, in contrast to the MRI findings and macroscopical and anatomical-pathological lesion. These findings rise our attention, about the need to keep in mind the clinical suspicion of AVM in cases of back pain and motor deficit, and an early surgical conduct in this situation.- - - - - - - - - - ranking = 0.20543468521817keywords = arteriovenous malformation, malformation (Clic here for more details about this article) |
10/31. paraplegia after tethered cord surgery: an uncommon combined anomaly of spinal arteriovenous fistula and sacral lipoma--case report.OBJECTIVE AND IMPORTANCE: The coexistence of spinal arteriovenous malformation (AVM) with congenital abnormalities is relatively common. However, the association of a spinal AVM and lipoma is rare. We present an adult patient with this combined anomaly and discuss the clinical relevance of this case. CLINICAL PRESENTATION: A 42-year-old Caucasian man with progressive paraparesis initially underwent surgery for a tethered spinal cord. Postoperatively, he became paraplegic. He improved gradually over an interval of 8 months and, at that point, worsened again. Subsequent angiographic study revealed a spinal dural arteriovenous fistula located at S1-S2. Additionally, an occipital dural AVM was discovered near the transverse sinus. INTERVENTION: The spinal arteriovenous fistula was excised along with the sacral lipoma. The occipital arteriovenous fistula was embolized successfully at a later time. CONCLUSION: The patient had immediate improvement in sensory symptoms after surgery. At a 9 month follow-up examination, he had regained the ability to walk with crutches, but his bladder dysfunction persisted. Recognition of co-existing vascular anomalies, such as spinal AVMs, is important in patients with tethered cords. The mechanisms involved in this patient's worsening neurological condition after release of the tethered cord are discussed.- - - - - - - - - - ranking = 0.2keywords = arteriovenous malformation, malformation (Clic here for more details about this article) |
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