Diagnóstico fetal de quiste filar de la médula espinal. Comunicación de 5 casos

El quiste filar es un hallazgo poco frecuente en las comunicaciones prenatales. Descrito como una imagen con líquido anecogénico bien definida en el centro del canal raquídeo por debajo del cono medular, normalmente tiene un buen pronóstico a largo plazo. Hasta el momento se han publicado 13 casos de diagnóstico prenatal en la literatura. Comunicamos 5 nuevos casos y se revisa la literatura.

The filar cyst is a rare finding in prenatal reports. Described as an image with well-defined anechogenic fluid in the center of the spinal canal below the conus medullaris, it usually has a good long-term prognosis. So far 13 cases of prenatal diagnosis have been published in the literature, we report 5 new cases and review the literature.

Apéndice cutáneo sacro asociado a lipoma del filum terminal y médula anclada: un caso de diagnóstico prenatal

Los disrafismos espinales cerrados tienen una prevalencia aún desconocida e involucran una gran variedad de formas. El lipoma del filum terminal es considerado dentro de los lipomas espinales y suele asociarse a médula anclada. Los estigmas cutáneos lumbosacros no siempre son indicadores de disrafismo espinal cerrado. Reportamos un caso de diagnóstico prenatal de apéndice cutáneo sacro con sospecha de médula anclada, confirmado al nacer como lipoma del filum terminal con médula anclada.

Closed spinal dysraphisms have a still unknown prevalence and involve a wide variety of forms. Lipoma of the filum terminale is considered within spinal lipomas and is usually associated with tethered medulla. Lumbosacral cutaneous stigmata are not always indicative of closed spinal dysraphism. We report a case of prenatal diagnosis of sacral cutaneous appendage with suspected tethered cord, confirmed at birth as lipoma of the filum terminale with tethered cord.

Cystic paraganglioma of the filum terminale / Paraganglioma quístico del filum terminal

Spinal Paragangliomas are rare tumors, most frequently involving the cauda equina and the filum terminale. We report the case of a 62-year old woman presenting with a one month's lower back pain, left sciatica and leg weakness. Spinal magnetic resonance imaging showed a extramedullary lesion at L3-L4 level, measuring 37 x 52 x 21 mm with contrast enhancement. It presented an unenhancing intradural cystic lesion in the upper pole. The patient underwent lumbar laminectomy, and with neurofisiologic monitorization the tumor was completely resected. Microscopic examination of the tumor revealed a paraganglioma. In a review of the literature, we found only five previous reported cases in which the tumor is accompanied by a cyst.

Los paragangliomas son tumores poco frecuentes que se localizan en la región de la cauda equina y filum terminal. Presentamos el caso de una mujer de 62 años con una historia de 1 mes de evolución de lumbalgia, ciática y debilidad en el miembro inferior izquierdo. Una resonancia magnética lumbar mostró una tumoración extramedular en el nivel L3-L4 que medía 37 x 52 x 21 mm que se realzaba con contraste. Presentaba así mismo una lesión quística en el polo superior del tumor. Se realizó una laminectomía lumbar y bajo control neurofisiológico se realizó una extirpación completa el tumor. El estudio microscópico revelo que se trataba de un paraganglioma. Realizamos una revisión de la literatura encontrando únicamente otros cinco casos de paraganglioma espinal con lesión quística acompañante

Gangliocytic Paraganglioma of the Filum Terminale. A Rare Entity

Paragangliomas are rare, benign and slow-growing neuroendocrine tumors that can arise from the adrenal medulla (85­90%) or from the extra-adrenal paraganglia. In the central nervous system (CNS), they can be found at several sites, but more often at the cauda equina and filum terminale region, where they account for between 2.5 and 3.8% of total tumor cases of that region. There are only 8 cases described in the literature that mention the presence of the gangliocytic variant of this entity at the filum terminale. We present the case of a 41-year-old man with chronic lumbar pain refractory to medical treatment, without any associated neurological deficits. Magnetic resonance imaging (MRI) revealed an intradural, extramedullar oval lesion with regular contours and homogeneous caption of contrast at L1 level. He was submitted to surgical treatment, with complete resection of the lesion. The histological analysis revealed a gangliocytic paraganglioma of the filum terminale. At 5 years of follow-up, he remains asymptomatic and without any signs of relapse. These are lesions with an overall good prognosis with gross total resection. Although the recurrence rate is extremely low, prolonged observation is recommended due to the slow-growing nature of the tumor, being estimated that between 1 and 4% can recur even after gross total removal.

Management of Chiari I Deformity in Children and Adolescents: A Report from the Consensus Taskforce of the Brazilian Society of Pediatric Neurosurgery

Much controversy remains on the current management of Chiari I deformity (CID) in children, with many clinical, surgical and ethic-legal implications. The Brazilian Society of Pediatric Neurosurgery (SBNPed, in the Portuguese acronym) has put together a panel of experts to analyze updated published data on the medical literature about this matter and come up with several recommendations for pediatric neurosurgeons and allied health professionals when dealing with CID. Their conclusions are reported herein, along with the respective scientific background.

Analysis of surgical effect of adult lipomatous tethered cord syndrome / 中国医师进修杂志

Objective To discuss the surgical effect of adult lipomatous tethered cord syndrome (TCS). Methods The clinical data of 29 patients with adult lipomatous TCS were systematically analyzed. Eleven of male and 18 of female,who ranged from 20 to 59 years. All patients were performed filum terminale release and lipoma excision, the clinical outcome after operation was analyzed according to Hoffman grading criteria and visual analog scale (VAS). Results According to Hoffman grading criteria, 9 cases were obviously improved in leg muscle weakness, including 1 case with urination disturbance improved significantly. Also the lower back pain of patients were improved obviously: (2.63 ± 1.30) scores vs. (8.67±0.30) scores, there was significant difference (P<0.05). Conclusions Filum terminale release and lipoma excision can effectively improve the symptom of lower back pain of adult lipomatous TCS patients, meanwhile the symptom of paraparesis can improve appropriate postoperatively. But urinary deficits do not show a significant change.

Arteriovenous Fistula of the Filum Terminale Misdiagnosed and Previously Operated as Lower Lumbar Degenerative Disease

Filum terminale arteriovenous fistula (FTAVF) presenting as a cause of failed back surgery syndrome is a rare entity. We report a 48-year-old male patient who presented with clinical features of a conus medullaris/cauda equina lesion. He had upper and lower motor neuron signs in both the lower limbs with autonomic dysfunction. The patient was misdiagnosed and was operated twice earlier for lumbar canal stenosis and disc prolapse. After reviewing his clinical and radiological findings a diagnosis of FTAVF was made. He underwent surgery and there was a significant improvement in his neurological functions. We discuss the case and review the literature on FTAVF's.

Cauda Equina Syndrome Associated with Dural Ectasia in Chronic Anlylosing Spondylitis

Cauda equina syndrome (CES) associated with dural ectasia is a rare neurologic complication in patients with longstanding ankylosing spondylitis (AS). We report a 68-year-old male with a 30-year history of AS who presented a typical symptom and signs of progressive CES, urinary incontinence and neuropathic pain of the lumbosacral radiculopathy. Computed tomography (CT) and magnetic resonance imaging (MRI) findings showed the unique appearances of dural ectasia, multiple dural diverticula, erosion of posterior element of the lumbar spine, tethering of the conus medullaris and adhesion of the lumbosacral nerve roots to the posterior aspect of the dural ectasia. Considering the progressive worsening of the clinical signs, detethering of the conus medullaris through resection of the filum terminale was performed through a limited laminectomy. However, the urinary incontinence did not improve and there was a partial relief of the neuropathic leg pain only. The possible pathogenetic mechanism of CES-AS and the dural ectasia in this patient with longstanding AS are discussed with a literature review.

Solitary Hemangioblastoma at the Filum Terminale: A Case Report and Review of Literature

Spinal hemangioblastomas at the filum terminale are rare. Only a few reports present immunohistopathological features of spinal hemangioblastomas. A 55-year-old woman presented with sustained low back pain and intermittent numbness in both lower extremities. She already had a decompressive surgery for a left L5-S1 extraforaminal lesion 7 months ago. Her incidental mass lesion had been slightly enlarged for 7 months and concordant symptoms had also been prolonged. Her tumor was removed en bloc with a unilateral hemilaminotomy and bilateral decompression technique. The patient's symptoms improved immediately after the surgery. In immunohistochemical stains, the stromal cells were positive for inhibin-alpha, neuron specific enolase (NSE), and the endothelial cells were positive for CD31, CD34, and factor VIII related antigen in confirming the diagnosis of hemangioblastoma. We report a rare case of intradural extramedullary spinal hemangioblastoma at the filum terminale. Complete en bloc resection was achieved with no morbidity. The immunohistopathological staining include inhibin-alpha confirmed an accurate diagnosis of the hemangioblastoma distinguishing from other vascular tumors.

Primary filum terminale ependymoma: a series of 16 cases / Ependimoma primário de filum terminale: análise de uma série de 16 casos

Filum terminale ependymomas are slow growing tumors of the cauda equina with a high incidence in young adults. Although a complete microsurgical resection can lead to a cure, recurrence is not uncommon. Sixteen cases of filum terminale ependymomas treated at the Instituto de Neurologia de Curitiba were analyzed. Eleven patients were females and 5 males, their age ranging from 7 to 84 years. Symptoms and signs included lumbar pain (31.25 percent), radicular pain (56.25 percent) and neurological deficits (12.5 percent). In three cases, patients had previously undergone surgery in other hospitals. All were tested through MRI and were operated on. Two underwent a laminoplasty and 14 a laminectomy. The last 8 patients of this series had neuro-physiological monitoring during surgery. In all patients a total microsurgical resection was achieved. Histologically, 2 cases were cellular ependymomas and 14 cases myxopapillary ependymomas. There was no recurrence during a 2 to 84 month follow-up period.

Os ependimomas do filum teminale são tumores da cauda eqüina de crescimento lento com maior incidência em adultos jovens. A ressecção microcirúrgica total possibilita a cura da doença, recidivas, entretanto, apresentam sérias dificuldades no tratamento. Com o objetivo de estudar os aspectos clínicos, anatomopatológicos e do tratamento, analisaram-se 16 casos de ependimomas do filum teminale tratados no Instituto de Neurologia de Curitiba, 11 do sexo feminino e 5 do sexo masculino, com idade entre 7 e 84 anos, que apresentavam dor lombar (31,25 por cento), radiculopatia (56,25 por cento) e déficits neurológicos (12,5 por cento). Em 3 casos, os pacientes tinham sido operados em outro serviço anteriormente. Em todos os casos o diagnóstico foi confirmado pela ressonância magnética. Em 2 pacientes realizou-se laminoplastia e em 14 laminectomia. Nos últimos 8 pacientes empregou-se monitorização neurofisiológica. Em todos os casos a ressecção microcirúrgica foi total. Do ponto de vista histológico, demonstraram-se 2 casos de ependimoma celular e 14 casos mixopapilares. Não houve recidiva do tumor em um seguimento entre 2 e 84 meses.

Co-existence of Lipoma and Myxopapillary Ependymoma in a Filum Terminale Tumor

A 65-year-old woman presented with a history of severe lower back pain on forward-flexion for 2 months duration. Magnetic resonance imaging revealed a high signal mass with a tail on T1-weighted images at the L3 level. A total surgical resection was performed via a posterior approach with the aid of a microscope. Histopathological examination of the tumor revealed two pathological components: lipoma and myxopapillary ependymoma. The presence of dual histological components in one spinal cord tumor is rare. There are no prior reports of both types of cells (adipose and ependymal) grown simultaneously in a single tumor of the filum terminale in the medical literature. We report a unique case of the co-existence of lipoma and myxopapillary ependymoma within the same tumor located at the filum terminale and review related literature.

Isolated Hemangioblastoma of the Filum Terminale

The filum terminale is an exceptional location for isolated hemangioblastoma, and most commonly hemangioblastomas are present in patients with von Hippel-Lindau(VHL) syndrome. We describe here a case of hemangioblastoma of filum terminale not associated with VHL, presenting with the history of progressive back pain, particularly severe in recumbent posture, and recurrent bilateral sciatica. MRI and spinal angiography revealed a well-vasculized mass lesion in filum terminale. The tumor was resected surgically. Histological examination confirmed the hemangioblastoma diagnosis. We recommended that, although rare, hemangiblastoma of the filum terminale be included in the differential diagnosis of a patient with low back pain.

Morphological Organization of Cauda Equina

OBJECTIVE: Topographic arrangement and morphometric pattern of cauda equina are studied. METHODS: In 10 adult formalin fixed cadavers, the entire dural sac of lumbosacral spine including extradural nerve roots was extracted. After paraffin fixation, serial cross section from sacral nerve roots to conus medullaris was performed. We investigated following structures at each disc level using a slide scanner after haematoxylin and eosin and Masson's trichrome stains: 1) location of filum terminale(FT), 2) arrangement of motor and sensory bundles, 3) morphometric characteristics of sensory bundles to motor bundle of each spinal nerve root. RESULTS: The FT was located in the dorsal half of midline at L2-3 disc level and in the most dorsal portion of midline from the L4-5 disc level and downward. All bundles of each nerve root showed an oblique arrangement. Motor bundle of each spinal nerve root was always located in the anterior or anteromedial portion of the corresponding sensory bundles. At L4-5 and L5-S1 disc levels, S1 to S4 nerve roots were arranged inverted V-shape centered FT. The sensory root was composed of several bundles whereas the motor root was always composed of a single bundle. The areas occupied by sensory bundles were larger than 2 to 4 times in S1-3 sacral nerve roots and 1.9 to 2.4 times in L3-5 lumbar nerve roots compared with those occupied by the corresponding motor roots. CONCLUSION: This study will be helpful to prevent the nerve root injury during the operation of cauda equina and predict postoperative complications.

Paraganglioma of the Cauda Equina

Paraganglioma of the cauda equina or filum terminale is rare tumor and was first described in 1970. The authors present a case of paraganglioma of the cauda equina in a 55 year-old-man with nonspecific back pain. To our knowledge, there has been only a few report of MRI of a paraganglioma of the cauda equina. Diagnosis can be made with immunohistochemical study and electron microscopy after surgical removal. Because of frequent recurrence, total excision is mandatory.