1/39. Ocular malformations, moyamoya disease, and midline cranial defects: a distinct syndrome.PURPOSE: To report a 10-year-old girl with developmental anomalies of both optic disks, a chorioretinal coloboma, sphenopharyngeal meningoencephalocele, and moyamoya disease. methods: A full ophthalmologic examination, cranial magnetic resonance imaging and magnetic resonance angiography, and cerebral angiography were performed. RESULTS: The patient had a morning glory disk anomaly and microphthalmos of the right eye and optic nerve hypoplasia and retinochoroidal coloboma in the left eye. She had a midfacial cleft and an episode of seizures and a stroke. magnetic resonance imaging showed a sphenopharyngeal meningoencephalocele. magnetic resonance angiography and cerebral angiography demonstrated a pattern consistent with moyamoya disease. CONCLUSIONS: This patient had a distinct syndrome of optic disk, retinochoroidal, and carotid circulation anomalies with midline cranial defects. The recognition and treatment of the vascular abnormalities and cranial defects may prevent complications such as strokes that may occur during or after general anesthesia.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
2/39. Cutaneomeningospinal angiomatosis (Cobb syndrome) with tethered cord.A newborn presented with a skin-covered lumbar mass with a subcutaneous hemangioma and on a magnetic resonance image (MRI) revealed a tethered spinal cord with a local mass. The mass had signal characteristics compatible with a lipoma. An initial diagnosis of a lipomeningocele with tethered cord was made, and the patient underwent surgical exploration and subtotal resection of the mass. A follow-up MRI revealed that the cord was still tethered, but an additional mass was present. The initial mass with signal characteristics of lipomatous tissue was accompanied by a low-signal mass in the lumbosacral canal, ventral to the cord, and bilateral enlargement of the foramina at the lumbosacral level. Because of a concern for an intraspinal tumor, a second operative intervention was performed. Multiple biopsies of the mass inside the spinal cord, the nerve roots and at the level of the foramina revealed angiomas that had similar pathology in all the specimens. A partial resection of the masses and a release of the tethered cord was performed by sectioning the thickened filum terminale. The diagnosis of Cobb's syndrome was made. The unique association of a tethered cord and the Cobb syndrome is reported here.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
3/39. Detection of skin over cysts with Spina bifida may be useful not only for preventing neurological damage during labor but also for predicting fetal prognosis.Spina bifida is one of the most common open neural tube defects. There are two common types of spina bifida cystica, myelomeningocele and meningocele. Special attention to the thickness of the cystic sac (presence of intact skin and subcutaneous tissue) on magnetic resonance imaging is advantageous for determination of whether the child will profit from cesarean section in order to prevent neurological change (infection and drying of nerve tissue) and for management of spina bifida (most meningocele) during the perinatal period. Furthermore, skin detection may help to predict the prognosis of spina bifida after birth. meningocele, with intact skin over the cyst, has a better clinical course than myelomeningocele. Some myelomeningoceles with neural tube defects in a lower position, also frequently having an intact skin over the cyst, have almost the same clinical course as a meningocele. From this, we hypothesize that a baby with spina bifida who has intact skin over the cyst might have a good prognosis neurologically. In this report, we concentrate attention on the skin over cysts in 3 cases (1 meningocele and 2 myelomeningoceles).- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
4/39. Lumbosacral nerve root avulsion: report of a case and review of the literature.The 14th myelographically demonstrated case of lumbosacral nerve root avulsion is presented with a summary of the previously reported cases. In most cases lumbosacral nerve root avulsion is associated with pelvic fractures and sacroiliac dislocation, which cause a stretching force to be applied to the nerves of the lumbar and sacral plexuses, and in turn to the nerve roots intradurally. This force causes nerve root avulsion in the intradural course of the nerve root. The myelographic defect is a pseudomeningocele or diverticulum-like outpouching created by the tearing of the arachnoid covering of the nerve roots. myelography clearly indicates nerve root avulsion and surgical exploration is not indicated.- - - - - - - - - - ranking = 12keywords = nerve (Clic here for more details about this article) |
5/39. An unusual pterygopalatine meningocele associated with neurofibromatosis type 1. Case report.The authors describe an unusual meningocele of the lateral wall of the cavernous sinus and the anterior skull base in a young patient with typical stigmata of neurofibromatosis Type 1 (NF1). This lesion was discovered during evaluation for recurrent meningitis. It represented an anterior continuation of Meckel's cave into a large cerebrospinal fluid space within the lateral wall of the cavernous sinus, extending extracranially through an enlarged superior orbital fissure into the pterygopalatine fossa adjacent to the nasal cavity. It was successfully obliterated, via an intradural middle fossa approach, with fat packing and fenestration into the subarachnoid space. This meningocele most likely represents a variant of cranial nerve dural ectasia occasionally seen in individuals with NF1. It has as its basis the same mesodermal defect responsible for the more common sphenoid wing dysplasia and spinal dural ectasias identified with this condition. Involvement of the trigeminal nerve with expansion of the lateral wall of cavernous sinus has not been reported previously. The authors surmise, however, that it may be present in some cases of orbital meningocele associated with sphenoid wing dysplasia.- - - - - - - - - - ranking = 2keywords = nerve (Clic here for more details about this article) |
6/39. Rudimentary meningocele: remnant of a neural tube defect?BACKGROUND: Rudimentary meningocele, a malformation in which meningothelial elements are present in the skin and subcutaneous tissue, has been described in the past under a variety of different terms and has also been referred to as cutaneous meningioma. There has been debate as to whether rudimentary meningocele is an atretic form of meningocele or results from growth of meningeal cells displaced along cutaneous nerves OBJECTIVE: We reviewed the clinical, histological, and immunohistochemical characteristics of rudimentary meningocele in an attempt to assess the most likely pathologic mechanism for it. DESIGN: Retrospective study. SETTING: University hospitals. patients: Thirteen children with rudimentary meningocele. MAIN OUTCOME MEASURES: medical records were reviewed and histopathologic examination as well as immunohistochemistry studies were performed for each case. A panel of immunoperoxidase reagents (EMA, CD31, CD34, CD57, S-100, and CAM 5.2) was used to assess lineage and to confirm the meningothelial nature of these lesions. RESULTS: Recent evidence indicating a multisite closure of the neural tube in humans suggests that classic meningocele and rudimentary meningocele are on a continuous spectrum. CONCLUSION: Rudimentary meningocele seems to be a remnant of a neural tube defect in which abnormal attachment of the developing neural tube to skin (comparable to that in classic meningocele) could explain the presence of ectopic meningeal tissue. In the majority of cases, no underlying bony defect or communication to the meninges could be detected. However, in light of the probable pathogenesis, imaging studies to exclude any communication to the central nervous system should precede any invasive evaluation or intervention.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
7/39. Post-traumatic lumbar nerve root avulsion.Lumbar nerve root avulsion is a rarely seen clinical entity that may complicate major trauma. The majority of previously reported cases have associated pelvic or lumbar vertebral fractures. Two cases of traumatic pseudomeningoceles at the lumbar level with associated avulsions of the lumbar nerve roots are presented. Both patients were involved in high velocity motor vehicle accidents. Case 1 had associated pelvic fractures but no spinal fractures and, interestingly, case 2 had no fractures of the spine or pelvis. The value of MRI in making the diagnosis is demonstrated.- - - - - - - - - - ranking = 6keywords = nerve (Clic here for more details about this article) |
8/39. Lumbar nerve root avulsions with secondary ipsilateral hip dysplasia in a child.We report on an 8-year-old child with avulsions of the left L3, L4 and L5 nerve roots and traumatic meningoceles that were not associated with lumbar spine or pelvic girdle fractures. The patient had a history of a road traffic accident. Plain radiographs of the pelvis revealed left hip dysplasia. The magnetic resonance imaging findings of the lumbar spine are illustrated. The pathogenesis of lumbar nerve root avulsions and their association with ipsilateral hip dysplasia are discussed.- - - - - - - - - - ranking = 6keywords = nerve (Clic here for more details about this article) |
9/39. Intrathoracic giant meningocele developing hydrothorax: a case report.A 67-year-old woman with neurofibromatosis type 1 presented with progressive dyspnea. Radiologic evaluation and magnetic resonance imaging revealed progression of a giant meningocele associated with hydrothorax. Laminoplasty with incision of the meningocele and dural plasty was performed, although nerve rootlets were killed. Microsurgical incision of the neck of the meningocele is a favorable operation even in large meningoceles such as the present case.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
10/39. Surgical strategy for anterior sacral meningocele.A 25-year-old male presented with an anterior sacral meningocele (ASM) manifesting as repeated urinary tract infections. Surgical correction was completed by simple ligation of the thecal sac next to the ostium via sacral laminectomy, and the thickened filum terminale was sectioned. A 22-year-old female presented with an ASM manifesting as transient difficulty in micturition. Subsequent to sacral laminectomy, the thickened filum terminale was sectioned. However, an aberrant nerve root over the ostium made simple ligation hazardous, so that transdural suture around the ostium was carried out. Complete obliteration was confirmed 5 months after the surgery. Magnetic resonance (MR) imaging could clearly demonstrate the involvement of neurologically important structures. Surgical strategy for ASM based on neurosurgical considerations is proposed, because of the frequent association of caudal spinal cord anomaly as well as presacral mass lesion. Intraoperative assistance systems such as endoscopy for cyst content examination and neurophysiological monitorings are recommended. Several months follow up with MR imaging is required to confirm successful surgical correction.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
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