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.

Double neurophysiological certification of the filum terminale during sectioning surgery in pediatric population.

BACKGROUND: Surgery of thickened-fibrolipoma filum terminale (FT) is performed routinely and without conflict but is not a risk-free surgical procedure. Intraoperative neurophysiological monitoring with mapping techniques can help to certify the FT before sectioning. However, a tailored surgical approach to cauda equina and a low threshold of surrounding nerve roots can confuse the final surgical decision. The aim is to demonstrate the usefulness of this double methodology for FT certification. METHODS: A prospective study collected and reviewed retrospectively, from 2015 to 2018, 40 patients undergoing an FT surgery section were included in the study. After opening the dura mater and under the microscope, the cauda equina mapping is performed and the recording of muscles of the lower limbs and the external anal sphincter. In addition, a high-intensity stimulation of constant current of an isolated FT for a short period of time and in a dry surgical field, obtaining a bilateral-polyradicular-symmetrical response of cauda equina nerve roots. RESULTS: Traditional motor mapping identified FT in 65% (26/40) of patients. Although, 35% (14/40) of the patients still have low-intensity stimuli response (<1 mA) of a muscle, especially anal sphincter. When this happens, the optimization of the dissection around FT is performed. After that, 25% (10/40) of the patients still having a muscle response in spite of seem isolated FT. Increasing the stimulation intensity up to 20 mA evoked a cauda equina response in all cases. No postoperative neurological impairment was observed in this series. CONCLUSION: This proposed methodology accurately confirms the FT so that it can be safely found and cut. The Double Neurophysiological Certification improves the gap of the traditional mapping techniques of cauda equina and can be used in a variety of more complex surgeries in this area.

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.

The effect of filum terminale sectioning for Chiari 1 malformation treatment: systematic review.

PURPOSE: Symptomatic Chiari type I malformation (CM) is treated with posterior fossa decompression with/ without duroplasty. Few authors suggested cerebellar tonsil caudal migration due to a supposed "caudal traction" of cranial nerve structures in a so-called occult tethered cord syndrome. For these authors, filum terminale (FT) sectioning may improve CM symptoms. The objective of this review is to evaluate the effect of FT sectioning on the treatment of CM. METHODS: Using the PRISMA guidelines for systematic reviews, we reviewed studies to evaluate patient's outcomes with CM who underwent FT sectioning. The MINORS instrument was used for methodological quality assessment. The included studies' levels of evidence (LOE) were classified according to the Oxford Centre of Evidence-Based Medicine. RESULTS: Two studies from the same group of authors were included. We cannot assure if the cited cases in the first study were also included in their latter published study. The described results suggest that outcomes were not collected in a standardized fashion. Outcomes are described vaguely as a percentage of improvement. Case series samples were small and included not only patients with CM but also patients with scoliosis and syringomyelia. The MINORS score reported that both studies had low methodological quality. Both included studies were classified as level 4 of evidence. CONCLUSION: There is no scientific support for filum terminale sectioning in patients with CM without evidence of tethered cord. This procedure may be considered experimental and should be validated in a strict criterion of inclusion clinical trial comparing outcomes in posterior fossa decompression.

Report of a case of giant cell ependymoma with unusual clinical and pathological presentation.

Giant cell ependymoma (GCE) is a very uncommon variant of ependymoma, known for having varying degrees of nuclear pleomorphism. There are only 34 reported cases of GCE in the English literature. We describe an additional case of a young woman who presented with a tumor located in sacral soft tissue, which was not connected to the spinal cord and did not show additional lesions in the central nervous system. Complete tumor resection was performed and no recurrences or metastasis were detected after 5 months of follow-up. Only one of all the reported GCE was located in the sacral subcutaneous region, where ependymomas are rarely found and usually have myxopapillary histology. Ours is the second report showing microscopic features of GCE in the soft-tissue region. GCE should be considered in the differential diagnosis of lumbosacral subcutaneous tumors to avoid misdiagnosing it as a malignant lesion. Since GCE could be an extraspinal extension of an intraneural ependymoma, it would be important to evaluate whether it is connected to the spinal cord.

Giant-cell ependymoma: Presentation of a case of the sacral region and literature review.

Ependymoma is a rare central nervous system neoplasm with an even rarer morphologic variant called giant-cell ependymoma (GCE). GCE has a characteristic discrepant, malignant-like morphology but indolent behavior. We present the case of a 21-year-old female with an extra-axial GCE located in the sacral region. To date, 16 cases of sacral GCE have been reported in the literature, with 4 cases in the sacral region; however, all those cases were intra-axial. We present the first case of an extra-axial sacral GCE.

Immunohistochemical features of giant cell ependymoma of the filum terminale with unusual clinical and radiological presentation.

BACKGROUND: Giant cell ependymoma of the filum terminale is a rare variant, generally manifested as a well-circunscribed intradural mass with an indolent biological behavior. CASE PRESENTATION: We describe the case of a 48-year-old Mexican female who non-relevant past medical history, that developed a GCE of the filum terminale. Magnetic resonance imaging and computed tomography revealed the presence of an intra-axial tumor extending from L3 to L5 with extra-medullary invasion. Therefore the tumor was considered unresectable and only incisional biopsy was obtained, establishing the tentative diagnosis of a poorly differentiated neoplasia. A second evaluation of the case revealed the presence of numerous non-cohesive pleomorphic giant cells with intranuclear inclusions and broad eosinophilic cytoplasm, alternating with intermediate size cells with round, hyperchromatic nuclei and forming a perivascular pseudo-rosettes pattern. The ependymal phenotype was supported by light microscopy and corroborated by immunohistochemistry analysis. The patient was subsequently treated with radiotherapy 54Gy. She is alive after a 27-month follow-up, with residual disease, difficulty ambulating and pain. CONCLUSIONS: GCE of filum terminale may have an atypical clinical and radiological presentation, albeit with invasive characteristics and anaplasia on histologic analysis. However, its biological behavior is indolent and associated to longer survival. Due to the presence of giant cells, the differential diagnosis of other primary neoplasias at that site were considered, including paraganglioma, malignant peripheral nerve sheath tumors as well as metastatic malignant melanoma, adrenal carcinoma, thyroid gland carcinoma and urothelial carcinoma, that may all harbor giant cells.

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.