11/41. Cervical cord tethering due to split cord malformation at the cervico-dorsal junction presenting with self-mutilation of the fingers.An unusual case of cervical spinal cord tethering with diplomyelia is described. A 12-month old female presented with self-mutilation of the fingers due to sensory loss in the hands, absent reflexes, poor muscle tone, and reduced distal upper-limb movements. There was a deep skin dimple overlying the T1 spinous process. Imaging showed angulation of the lower cervical cord and an operation revealed a low cervical meningocele and a split cord malformation with tethering of one half of the cord; the cord was untethered. In this report the literature is reviewed.- - - - - - - - - - ranking = 1keywords = meningocele (Clic here for more details about this article) |
12/41. Multiple coexistent dysraphic pathologies.INTRODUCTION: Four distinct dysraphic anomalies were observed in a single child. While combinations of such anomalies are well recognised, quadruple dysraphic pathology, nevertheless, is extremely uncommon. To our knowledge, no previous cases have been reported in the literature. CASE REPORT: We present the management of a child with a concurrent segmental meningocele, a type-1 split cord malformation (SCM) associated with hemivertebrae, lipomyelomeningoceles in each hemicord of the SCM and a terminal myelocystocele, and we review the literature on potential mechanisms of dysmorphogenesis. DISCUSSION: Existing embryologic hypotheses for the dysraphic spectrum lack experimental evidence and studies in animal models. This case challenges the existing hypotheses and illustrates our incomplete understanding of human terminal spinal cord embryogenesis. Further studies on the morphogenetic basis for these anomalies are required.- - - - - - - - - - ranking = 2keywords = meningocele (Clic here for more details about this article) |
13/41. prenatal diagnosis of diastematomyelia.INTRODUCTION: Diastematomyelia, also termed split cord malformation, is a form of occult spinal dysraphism characterized by a cleft in the spinal cord. prenatal diagnosis of this anomaly is possible by ultrasonography (US), and fetal MRI can be used to diagnose the type of diastematomyelia precisely. Diastematomyelia can be isolated or associated with other dysraphisms, segmental anomalies of the vertebral bodies, or visceral malformations (horseshoe or ectopic kidney, utero-ovarian malformation, and anorectal malformation). We present three cases of fetal diastematomyelia investigated using a multimodal prenatal work-up (US, MRI, 3D-CT). CASES: The first case, detected at 20 weeks' gestation, had a lumbar meningocele. At 30 weeks' gestation, direct US visualization revealed the division of the spinal cord into two hemicords. This patient illustrates an isolated type II diastematomyelia with a favorable prognosis. The second case, detected at 22 weeks' gestation, presented with disorganization of bony process of the vertebral column with a midline echogenic bony spur, asymmetrical hemicords, and a foot malposition. Fetal MRI at 26 weeks' gestation and CT/3D reconstructed at 32 weeks' gestation confirmed a type I diastematomyelia with orthopedic malposition. The third case, detected at 22 weeks' gestation, presented with widening of the lumbar canal and scoliosis. Prenatal work-up (US, MRI) disclosed other visceral malformations (pelvic kidney), which led to the assumption of a complex polymalformative syndrome. The pregnancy was terminated. Fetopathologic examination disclosed even more visceral malformations (anal atresia and unicorn uterus).- - - - - - - - - - ranking = 1keywords = meningocele (Clic here for more details about this article) |
14/41. Cervical meningocele in association with spinal abnormalities.CASE REPORT: This case report presents a newborn baby girl, who was diagnosed at birth with a mid-cervical meningocele. Further radiographic workup by MRI revealed co-existing thoracic diplomyelia and bilateral tethered cords. At birth the patient was found to be neurologically intact. Surgery was performed at 4 months of age, the patient undergoing simultaneous repair of the cervical meningocele, exploration of the diplomyelia, and release of the tethered cords bilaterally. Long-term follow-up revealed an ambulating patient with no bowel or bladder incontinence, who has developed well for her chronological age so far. review OF THE literature: A review of the literature relevant to this case is also presented.- - - - - - - - - - ranking = 6keywords = meningocele (Clic here for more details about this article) |
15/41. Diastematomyelia presenting as progressive weakness in an adult after spinal fusion for adolescent idiopathic scoliosis.BACKGROUND CONTEXT: Diastematomyelia is uncommon and rarely presents in adulthood. This report draws attention to the fact that patients who underwent spinal fusion for deformity before the widespread use of computed tomography (CT) and magnetic resonance imaging (MRI) may have unrecognized spinal cord abnormalities. This should be considered if revision surgery is contemplated. PURPOSE: This case report focuses on the late presentation of lower-extremity weakness in a 44-year-old woman with a split cord malformation (diplomyelia), diastematomyelia and tethered cord syndrome. STUDY DESIGN/SETTING: methods: The patient underwent instrumented posterior spinal fusion with a Harrington rod as a child for progressive thoracolumbar scoliosis. As an adult, she developed paraparesis after a traumatic event.The patient underwent decompressive laminectomy, subtotal resection of the old fusion mass and resection of the osseous septum. Postoperatively, an anterior spinal fluid leak in the lower thoracic region required repeated fascial grafting, resection of a pseudomeningocele and reverse left latissimus dorsi flap transfer. The leak was controlled, and the patient had near complete resolution of her paraparesis 1 year after her surgery. RESULTS: The case described herein is unusual in that patients with diplomyelia and diastematomyelia rarely are symptomatic in adulthood. However, trauma may precipitate the onset of neurologic symptoms. This patient underwent spinal surgeries to address deformity, pain and progressive lower-extremity weakness. Preoperative CT and MRI studies showed a split cord malformation and diastematomyelia at L1-L2 with spinal stenosis and tethering of both hemicords. CONCLUSIONS: Progressive weakness without any previous neurologic deficit or neurocutaneous stigmas of an underlying spinal cord abnormality may develop in the adult with unrecognized diastemotomyelia. This case demonstrates that a thorough preoperative workup of patients with complex spinal deformities is imperative.- - - - - - - - - - ranking = 1keywords = meningocele (Clic here for more details about this article) |
16/41. Thoracic meningocele, meningomyelocele or myelocystocele? Diagnostic difficulties, consequent implications and treatment.spina bifida cystica is a closing disorder of the neural tube which infrequently occurs in the thoracic region. A rare lesion called myelocystocele is a variant of spina bifida cystica and is associated with syringomyelia, Chiari type 2 malformation and hydrocephalus. Usually the patient has no neurological deficit, but future deterioration can occur due to posterior tethering of the spinal cord by adhesions. The prenatal diagnosis by ultrasound study can be misleading and in order to attain the correct diagnosis, especially if abortion is considered, a prenatal MRI scan should be done before the parents are counselled, and should be repeated prior to operative treatment. Surgical correction of myelocystocele is not only for cosmetic reasons, but also to untether the spinal cord prophylactically to prevent future neurological deterioration. In this case report, we present a child born with a thoracic myelocystocele, the diagnostic difficulties, consequent implications and surgical treatment.- - - - - - - - - - ranking = 4keywords = meningocele (Clic here for more details about this article) |
17/41. Triple neural tube defect--cranium bifidum with rostral and caudal spina bifida--live evidence of multi-site closure of the neural tube in humans.OBJECTIVE: The coexistence of three neural tube defects (NTDs) in a single child is an exceptional event. A review of the literature revealed nine published "double" NTD cases, but no cases of "triple" NTDs have been reported to date. CASE REPORT: The rare case of a two-year-old boy with three distinct NTDs is presented. The boy had a 17x15x15-cm(3) parieto-occipital encephalocele, a small cervical myelomeningocele, and a 11x11x8-cm(3) thoracolumbar myelomeningocele. hydrocephalus and Chiari II malformation accompanied the NTDs. All three lesions were surgically treated with good cosmetic results and satisfactory neurologic outcome. CONCLUSIONS: Current neural tube closure theories and models are reviewed in an attempt to better understand this extremely unusual coexistence. The multi-site closure model is clearly more useful in our understanding of NTDs.- - - - - - - - - - ranking = 2keywords = meningocele (Clic here for more details about this article) |
18/41. Subacute formation of syrinx post-untethering of spinal cord.Tethered spinal cord syndrome is characterized by a pathological fixation of the cord resulting in a low situated conus medullaris below the L1-2 interspace. With growth and development, this results in excessive cord stretching causing neuronal dysfunction. Untethering of the spinal cord aims to prevent the development of neurological or orthopedic sequelae which may arise if left untreated. Various complications of this operation have been described, including pseudomeningocele, CSF fistula, and meningitis. The authors present a case of a patient who developed a symptomatic syrinx, several days following untethering of the cord. Significant neurological improvement was achieved following revision surgery. This is the first case report of a syrinx developing several days post-untethering of the cord.- - - - - - - - - - ranking = 1keywords = meningocele (Clic here for more details about this article) |
19/41. Intradural mesh: an unusual cause of spinal cord tethering: case report.A 13-year-old boy with a myelomeningocele experienced progressive foot deformity and lower-extremity pain while walking. magnetic resonance imaging demonstrated a low-lying spinal cord with scarring near the site of a previous repair. During surgery, the terminal nerve roots were found to have scarred and adhered to a piece of metal mesh lying in the intradural space. The mesh had originally been placed to bridge a sacral ossification defect that was present at the initial closure of the child's myelomeningocele.- - - - - - - - - - ranking = 2keywords = meningocele (Clic here for more details about this article) |
20/41. Presacral meningocele associated with hereditary sacral agenesis and treated surgically: evaluation in three members of the same family.OBJECTIVE AND IMPORTANCE: Presacral meningocele in hereditary sacral agenesis is a complex and unusual spinal dysgenetic syndrome. Recognition of the syndromic triad, its natural history, and familial presentation has important practical applications for the management of this disease as well as its complications. CLINICAL PRESENTATION: This report concerns three patients in one family with Currarino syndrome. We detail its clinical presentation, operative management, and outcome and suggest management procedures based on reports in the literature and the results of our surgical techniques, which focus on cases with an "incomplete" triad. INTERVENTION: Three members of the same family, one adult and two children, underwent surgery through the posterior sacral approach tying off the communication between dural sac and anterior meningocele. The adult underwent a second surgical procedure in which a custom-designed surgical technique was used to resolve postoperative cerebrospinal fluid leakage. One of the children underwent an additional posterior sagittal anorectoplasty to remove a presacral teratoma. CONCLUSION: We report a rare occurrence of three familial cases of sacral agenesis accompanied by a presacral mass with various degrees of phenotypic expression and with male dominant transmission. Because of its rarity, the best surgical technique and timing remain an open question especially in cases with incomplete triad syndrome.- - - - - - - - - - ranking = 6keywords = meningocele (Clic here for more details about this article) |
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