Siringomielia no secundaria a Chiari. Actualización en fisiopatología y manejo / Update on the pathophysiology and management of syringomyelia unrelated to Chiari malformation

Introducción: Son muchos los conocimientos y publicaciones existentes sobre la siringomielia relacionada con la malformación de Chiari, pero existe poca difusión de este cuadro cuando no se presenta en relación con dicha malformación. Ello es importante ya que es una entidad propia que precisa de un conocimiento y manejo específico. Presentamos esta revisión con el objetivo de dar a conocer las ideas más aceptadas y difundidas a día de hoy al respecto de la fisiopatología, manejo y otros aspectos de la siringomielia no secundaria a malformación de Chiari. Desarrollo: Se ha realizado una revisión de la literatura más relevante en torno a esta patología, centrándose en su fisiopatología, presentación clínica, estudio diagnóstico y manejo. Conclusiones: La siringomielia no relacionada con malformación de Chiari es una entidad propia que precisa de un conocimiento adecuado en su profundidad para su sospecha, seguimiento y manejo adecuado. Ante el hallazgo de este cuadro debe realizarse un estudio detallado encaminado a intentar identificar la causa, quedando el tratamiento sintomático como opción de rescate

Introduction: Much has been published on syringomyelia related to Chiari malformation. In contrast, little is known about the condition when it is not associated with this malformation, but this presentation of syringomyelia constitutes a different entity and therefore requires specific management. We conducted a literature review to summarise the most accepted and widespread ideas about the pathophysiology, management and other aspects of syringomyelia unrelated to Chiari malformation. Development: We reviewed the most relevant literature on this condition, focusing on the pathophysiology, clinical presentation, diagnosis, and treatment. Conclusions: Syringomyelia unrelated to Chiari malformation is a distinct entity that must be well understood to guarantee correct diagnosis, monitoring, and management. When the disease is suspected, a thorough study should be conducted to identify its aetiology. Treatment must aim to eliminate the cause of the disease; symptomatic treatment should remain a second-line option

Update on the pathophysiology and management of syringomyelia unrelated to Chiari malformation. / Siringomielia no secundaria a Chiari. Actualización en fisiopatología y manejo.

INTRODUCTION: Much has been published on syringomyelia related to Chiari malformation. In contrast, little is known about the condition when it is not associated with this malformation, but this presentation of syringomyelia constitutes a different entity and therefore requires specific management. We conducted a literature review to summarise the most accepted and widespread ideas about the pathophysiology, management and other aspects of syringomyelia unrelated to Chiari malformation. DEVELOPMENT: We reviewed the most relevant literature on this condition, focusing on the pathophysiology, clinical presentation, diagnosis, and treatment. CONCLUSIONS: Syringomyelia unrelated to Chiari malformation is a distinct entity that must be well understood to guarantee correct diagnosis, monitoring, and management. When the disease is suspected, a thorough study should be conducted to identify its aetiology. Treatment must aim to eliminate the cause of the disease; symptomatic treatment should remain a second-line option.

A cellular and molecular basis for the selective desmopressin-induced ACTH release in Cushing disease patients: key role of AVPR1b receptor and potential therapeutic implications.

CONTEXT: Desmopressin is a synthetic agonist of vasopressin receptors (AVPRs). The desmopressin stimulation test is used in the diagnosis and postsurgery prognosis of Cushing disease (CD). However, the cellular and molecular mechanisms underlying the desmopressin-induced ACTH increase in patients with CD are poorly understood. OBJECTIVE: The objectives of this study were to determine, for the first time, whether desmopressin acts directly and exclusively on pituitary corticotropinoma cells to stimulate ACTH expression/release and to elucidate the cellular and molecular mechanisms involved in desmopressin-induced ACTH increase in CD. DESIGN: A total of 8 normal pituitaries (NPs), 23 corticotropinomas, 14 nonfunctioning pituitary adenomas, 17 somatotropinomas, and 3 prolactinomas were analyzed for AVPR expression by quantitative real-time RT-PCR. Primary cultures derived from corticotropinomas, nonfunctioning pituitary adenomas, somatotropinomas, prolactinomas, and NPs were treated with desmopressin, and ACTH secretion/expression, [Ca(2+)]i kinetics, and AVPR expression and/or proliferative response were evaluated. The relationship between AVPR expression and plasma adrenocorticotropin/cortisol levels obtained from desmopressin tests was assessed. RESULTS: Desmopressin affects all functional parameters evaluated in corticotropinoma cells but not in NPs or other pituitary adenomas cells. These effects might be due to the dramatic elevation of AVPR1b expression levels found in corticotropinomas. In line with this notion, the use of an AVPR1b antagonist completely blocked desmopressin stimulatory effects. Remarkably, only AVPR1b expression was positively correlated with elevated plasma adrenocorticotropin levels in corticotropinomas. CONCLUSIONS: The present results provide a cellular and molecular basis to support the desmopressin stimulation test as a reliable, specific test for the diagnosis and postsurgery prognosis of CD. Furthermore, our data indicate that AVPR1b is responsible for the direct/exclusive desmopressin stimulatory pituitary effects observed in CD, thus opening the possibility of exploring AVPR1b antagonists as potential therapeutic tools for CD treatment.

Experience with coflex interspinous implant.

A first generation of Coflex implant for non-rigid stabilization of lumbar spine was presented by Samani (Study of a semi-rigid interspinous U fixation system. Spinal Surgery, Child Orthopaedics: 1707, 2000).We started to treat patients with this Coflex device in 2004 and since then more than 600 patients have been operated in our Neurosurgical Department. We are reporting 156 patients treated between December 2004 and 2006 with complete follow-up. The clinical trials of this and other implants provide evidence that this interspinous non-rigid stabilization is useful against low-back pain due to degenerative instability and without serious complications.

[Solitary fibrous tumors of the meninges: report of three cases and literature review]. / Tumor fibroso solitario meníngeo: descripción de tres casos y revisión de la literatura.

We report 3 patients with fibrous solitary tumor of meningeal location where we described the histological study, as well as evolution after the surgical treatment. The described patients presented ages of 37, 52 and 65 years, after the resection has not appeared an objective sign of recurrence in any case after 4, 6 and 7 years of follow-up respectively. Checking the literature the tumor is indistinguishable clinical and radiolocally of the typical meningioma, doing necessary the use of inmunohistochemistry to do the differential diagnosis, where positiveness for CD34 and the negativeness for EMA define the fibrous solitary tumor. It is about a benign tumor, where total removing is the principal factor in prognosis, nevertheless there are cases of local recurrences and long-distance metastasis. We can find all these characteristics in the showed cases of the present article, having the uncertainty of its local or systemic relapse ability in the future.

Tumor fibroso solitario meníngeo: descripción de tres casos y revisión de la literatura / Solitary fibrous tumors of the meninges: report of three cases and literature review

Presentamos 3 pacientes con tumor fibroso solitario de localización meníngea donde describimos el estudio histológico, así como la evolución después del tratamiento quirúrgico. Los pacientes descritos tenían edades de 37, 52 y 65 años y tras la resección total no se ha objetivado signo de recidiva en ningún caso después de 4, 6 y 7 años de seguimiento respectivamente. Revisando la literatura se trata de un tumor indistinguible clínica y radiológicamente del meningioma típico, haciendo necesario el uso de pruebas inmunohistoquímicas para realizar el diagnóstico diferencial, donde la positividad para el CD34 y la negatividad para el EMA definen al tumor fibroso solitario. Se trata de un tumor benigno, en el que la resección total es el principal factor pronóstico; no obstante, se han descrito casos de recidivas locales y metástasis a distancia. Todas estas características las encontramos en los casos presentados en el presente trabajo, quedando la incertidumbre de su capacidad de recidiva local o sistémica en el futuro

We report 3 patients with fibrous solitary tumor of meningeal location where we described the histological study, as well as evolution after the surgical treatment.The described patients presented ages of 37, 52 and 65years, after the resection has not appeared an objective sign of recurrence in any case after 4, 6 and 7 years of follow-up respectively. Checking the literature the tumor is indistinguishable clinical and radiolocally of the typical meningioma, doing necessary the use of inmunohistochemistry todo the differential diagnosis, where positiveness forCD34 and the negativeness for EMA define the fibrous solitary tumor. It is about a benign tumor, where total removing is the principal factor in prognosis, nevertheless there are cases of local recurrences and long-distance metastasis. We can find all these characteristics in the showed cases of the present article, having the uncertainty of its local or systemic relapse ability in the future

[Protective effects of the growth hormone (GH) on the irradiated spinal cord in rats]. / Radioprotección de la médula espinal mediante la hormona de crecimiento (GH). Estudio experimental en ratas.

Radiotherapy or irradiation of SNC AVM's or tumors also presents a high risk for provoking lesions in adjacent surrounding tissue. The objective of our study is to demonstrate radiotherapy induced alterations in a rat spinal cord model and evaluate the protective effect of Growth Hormone (GH) on rats exposed to high radiotherapy doses. The experimental study employed two groups of Wistar rats: Group A (control group):10 rats, which received 30 Gy at the spinal cord . Group B: 10 rats, these animals received 30 Gy and dose of 2mg/kg/day GH. Growth hormone administration was begun three days before radiotherapy and continued until two days after radiotherapy for a total of six days. At 14 days postradiotherapy, all the rats were sacrificed and the spinal cord extracted immediately. Hematoxyline-eosine histologic studies showed that control animals only exposed to radiotherapy had severe alterations with hemorrhage and vacuolisation of the entire irradiated segment while these alterations were much less severe in the GH-treated group. In conclusion, 30 Gy irradiation produced morphological changes including vascular endothelial oedema, necrosis, hemorrhage, and inflammatory exudates. A 2 mg/kg/day dose of GH protected the rat spinal cord against the noxious effects of the radiotherapy, decreasing the clinical, macro and microscopic damage in the treated animals.

Radioprotección de la médula espinal mediante la hormona de crecimiento (GH). Estudio experimental en ratas / Protective effects of the growth hormone (GH) on the irradiated spinal cord in rats

La radioterapia empleada en el tratamiento de los tumores o malformaciones vasculares del SNC presenta riesgo de causar lesiones en los tejidos adyacentes. El objetivo de nuestro estudio es demostrar las alteraciones provocadas por la radioterapia en la medula espinal de ratas a altas dosis y el valorar el efecto protector de la hormona de crecimiento (GH). El estudio experimental fue realizado con dos grupos de ratas Wistar. Grupo A (grupo control): Constó de 10 ratas que se irradiaron con 30 Gy en la medula espinal. Grupo B: Fue un grupo de 10 ratas irradiadas con 30Gy a las que se administró 2mg/kg/día de GH tres días previos a la radioterapia, el día de la radioterapia, y dos días después. A los 14 días de la radioterapia fueron perfundidas mostrando el estudio histológico de la médula espinal de las ratas tratadas sólo con radioterapia de forma intensa, hemorragias y trombosis de capilares. Al grupo a las que se administró GH mostró una importante disminución de las lesiones. En resumen la radioterapia administrada en dosis de30 Gy causa cambios morfológicos como edema lesión del endotelio vascular, necrosis, hemorragias, exudado inflamatorio. La dosis de 2mg/kg/día ejerce un efecto protector en la medula espinal de ratas tras la administración de radioterapia disminuyendo el daño macro y microscópico en las ratas estudiadas

Radiotherapy or irradiation of SNC AVM's or tumors also presents a high risk for provoking lesions in adjacent surrounding tissue. The objective of our study is to demonstrate radiotherapy induced alterations in a rat spinal cord model and evaluate the protective effect of Growth Hormone(GH) on rats exposed to high radiotherapy doses. The experimental study employed two groups of Wistar rats: Group A (control group):10 rats, which received 30 Gy at the spinal cord . Group B: 10 rats, these animals received 30 Gy and dose of 2mg/kg/day GH. Growth hormone administration was begun three days before radiotherapy and continued until two days after radiotherapy for a total of six days. At 14 days postradio therapy, all the rats were sacrificed and the spinal cord extracted immediately. Hematoxy line-eosine histologic studies showed that control animals only exposed to radiotherapy had severe alterations with hemorrhage and vacuolisation of the entire irradiated segment while these alterations were much less severe in the GH-treated group. In conclusion, 30 Gy irradiation produced morphological changes including vascular endothelial oedema, necrosis, hemorrhage, and inflamantory exudates. A2 mg/kg/day dose of GH protected the rat spinal cord against the noxious effects of the radiotherapy, decreasing the clinical, macro and microscopic damage in the treated animals

[Causes of reoperations in patients with lumbar spinal stenosis treated with instrumentations]. / Causas de reintervención quirúrgica en pacientes con fijación lumbar intervenidospor estenosis de canal.

OBJECTIVE: In present study we analyze the causes leading to reoperation patients treated for lumbar spinal stenosis with laminectomy and lumbar instrumentation. MATERIAL AND METHODS: 74 patients operated between January of 1996 and December of 2000 with a minimum 4 year follow-up were seleted for the study. RESULTS: 16 patients (21.1%) underwent a new surgical intervention. The main cause for reoperation was stenosis of the adjacent level superiorly to the instrumentation (in 50%). Other causes were radicular fibrosis, persistence of stenosis after the surgery, neuropatic pain, failure of the instrumentation system, transpedicular screws misplacement and break of screws. CONCLUSIONS: Complications related with the initial surgical procedure are low, as half of the reoperations were due to stenosis of the segment superior to the fixation.

Causas de reintervención quirúrgica en pacientes con fijación lumbar intervenidos por estenosis de canal / Causes of reoperations in patients with lumbar spinal stenosis treated with instrumentations

Objetivo. En el presente trabajo analizamos las causas por las que se ha debido reintervenir a pacientes diagnosticados de estenosis de canal que habían sido tratados mediante descompresión quirúrgica y fijación lumbar. Material y métodos. Seleccionamos 74 pacientes entre enero de 1996 y diciembre de 2000 con un seguimiento mínimo de 4 años. Resultados. 16 (21,1%) fueron sometidos a una nueva intervención quirúrgica. La principal causa fue la estenosis de niveles adyacentes superiores a la instrumentación (en el 50%). Otras causas fueron la fibrosis perirradicular, la persistencia de estenosis tras la cirugía, el dolor neuropático, la desconexión del sistema de fijación, la compresión radicular por tornillos transpediculares y la rotura de tornillos. Conclusiones. Las complicaciones relacionadas con la técnica quirúrgica son bajas ya que la mitad de las reintervenciones en pacientes con estenosis del canal lumbar está producida por la estenosis del segmento superior a la fijación

Objective. In present study we analyzes the causes leading to reoperation patients treated for lumbar spinal stenosis with laminectomy and lumbar instrumentation. Material and methods. 74 patients operated between January of 1996 and December of 2000 with a minimum4 year follow-up were seleted for the study. Results. 16 patients (21 1%) underwent a new surgicall intervention. The main cause for reoperation was stenosis of the adjacent level superiorly to the instrumentation (in 50%). Other causes were radicular fibrosis, persistence of stenosis after the surgery, neuropatic pain, failure of the instrumentation system, transpedicular screws misplacement and break of screws. Conclusions. Complications related with the initial surgical procedure are low, as half of the reoperations were due to stenosis of the segment superior to the fixation

[Intramedullary spinal cord metastasis as the first manifestation of a renal carcinoma]. / Mestástasis intramedular como primera manifestación de un carcinoma renal

We present the case of a 69 year-old male without known antecedente who presented a clinical loss of distal force in his lower limbs during the last two months. Magnetic resonance imaging (MRI) showed an intramedullary spinal cord mass at the level of the medullaris cone. The patient was operated on; the histological diagnosis was clear cells carcinoma suggestive of metastasis from an unsuspected renal tumor that was later confirmed with an abdominal CT. When the patient's state is good, surgery can correct the neurological deficit produced by an intramedullary spinal cord lesion. The neurological state of our patient improved after the intervention, and 14 months after surgery, he has no neurological deficit in the lower limbs.

Metástasis intramedular como primera manifestación de un carcinoma renal / Intramedullary spinal cord metastasis as the first manifestation of a renal carcinoma

Presentamos el caso de un varón de 69 años sin patología previa de interés que presentó clínica de pérdida de fuerza distal en miembros inferiores de dos meses de evolución. La resonancia magnética (RM) mostró la presencia de una masa intramedular a nivel del cono. El paciente fue intervenido practicándose resección de la tumoración, mostrando el estudio histológico la presencia de un carcinoma de células claras correspondiente a una sospecha de metástasis de un tumor renal, que posteriormente se confirmó con una tomografía axial computarizada (TAC) abdominal. Cuando el estado del paciente es bueno, la cirugía puede subsanar el déficit neurológico producido por la lesión intramedular. Nuestro paciente, tras la intervención, mejoró su estado neurológico; y actualmente, tras 14 meses, se mantiene sin déficit neurológico en miembros inferiores

We present the case of a 69 year-old male without known antecedente who presented a clinical loss of distal force in his lower limbs during the last two months. Magnetic resonance imaging (MRI) showed an intramedullary spinal cord mass at the level of the medullaris cone. The patient was operated on; the histological diagnosis was clear cells carcinoma suggestive of metastasis from an unsuspected renal tumor that was later confirmed with an abdominal CT. When the patient's state is good, surgery can correct the neurological deficit produced by an intramedullary spinal cord lesion. The neurological state of our patient improved after the intervention, and 14 months after surgery, he has no neurological deficit in the lower limbs

Early amniocentesis with the filtration technique: neonatal outcome in 123 singleton pregnancies.

OBJECTIVE: To evaluate results of a prospective study of pregnancies in which early amniocentesis with the filtration technique was performed at 10-13 weeks' gestation (mean 12.3 weeks' gestation). METHODS: 123 singleton pregnancies in which early amniocentesis with the modified filtration technique was performed at 10-13 weeks' gestation (mean 12.3 weeks' gestation). The amniotic fluid was aspirated into the syringe and reinjected through the filter. RESULTS: All the procedures were performed successfully by a single needle insertion. Neither dry taps nor filtration failures occurred. The mean time of amniocentesis was 4.02 min (95% confidence interval, 3 min and 36 s to 4 min and 18 s). The karyotyping success rate was 99.2%. Temporary amniotic fluid leakage occurred in three women (2.4%). There were 110 (89.4%) live births. Two cases of stillbirth occurred at week 38 and week 40. Two unintended losses occurred within three weeks after sampling (1.62%). Another additional unintended fetal death was notified at the 20-week screening ultrasonography. The total fetal-loss rate was 10.6%. In one case, talipes equinovarus was detected at the 20-week screening ultrasound study. CONCLUSIONS: Further studies are needed to determine the risk of amniotic leakage and its relation to duration of the procedure in patients undergoing early amnifiltration.

Correlación entre el grado de anemia fetal intertransfusional y la medición doppler de la velocidad máxima del flujo en la arteria cerebral media en la anemia hemolítica por isoinmunización rh / Correlation between the degree of intertransfusional fetal anemia and peak flow velocity in the middle cerebral artery in fetuses with hemolytic anemia due to maternal red-cell alloinmunization

Objetivo: Evaluar la correlación entre la velocidad máxima sistólica de la arteria cerebral media y el grado de anemia, así como su potencial capacidad predictiva del grado de anemia fetal intertransfusional en tres pacientes con isoinmunización Rh.Material y métodos: Se describen tres casos de anemia fetal por isoinmunización Rh tratados con transfusión sanguínea intraútero. El seguimiento intertransfusional y la decisión de realizar una nueva cordocentesis se hizo según el protocolo clásico basado en la presencia de signos ecográficos de hidrops o la estimación del grado de hemólisis postransfusión. De forma paralela, se midió con eco-Doppler la velocidad máxima de la arteria cerebral media (VmáxACM) pre y postransfusión, así como entre las transfusiones.Resultados: Se realizaron un total de 11 cordocentesis con transfusión sanguínea. La medición de la VmáxACM se correlacionó con el grado de anemia, existiendo una correlación inversa entre ambos parámetros. La sensibilidad de la VmáxACM para detectar anemia moderada y grave fue del 100 por ciento. En nuestros casos la inclusión de la medición de la VmáxACM en los protocolos clínicos podría haber evitado tres cordocentesis.Conclusiones: Nuestros resultados preliminares sugieren la utilidad del estudio Doppler de la VmáxACM para el control del grado de anemia fetal entre transfusiones en los casos de anemia hemolítica por isoinmunización Rh. (AU)

[Spina bifida: management after 25 years]. / Espina bífida. Planteamiento después de 25 años.

The authors present their experience of 468 patients with spina bifida treated in a period of 25 years. They discuss the philosophy of the management of the severe neural tube defects based in the follow up of these patients. This attitude is based in the prevention that could be: 1) following Smithells theory; 2) trying a prenatal diagnostic before 20 weeks of pregnancy, and 3) doing a selection of spina bifida that could be operated.

Diagnosis of brain death: superiority of perfusion studies with 99Tcm-HMPAO over conventional radionuclide cerebral angiography.

The use of technetium-99m hexamethyl-propyleneamine oxime (99Tcm-HMPAO) in the diagnosis of brain death has been evaluated in 41 studies of 37 patients with severe brain injury, who were under the effect of drugs or when other diagnostic methods were equivocal. HMPAO studies were compared with conventional radionuclide angiography performed simultaneously by intravenous administration of HMPAO as a bolus. The ages of patients ranged from 4 months to 75 years. Dynamic flow images and 5-min static uptake images were acquired following bolus injection of 555 Mbq of 99Tcm-HMPAO. All patients showing no brain uptake were confirmed as brain-dead by standard clinical criteria, with no contradictory cases in the static study. In addition, all patients who were not brain-dead showed HMPAO uptake at least in the brainstem. Dynamic flow images were equivocal in five patients, four of whom had no uptake on static images and clinically confirmed brain death. In addition, two other cases showed "mismatched" dynamic and static images: in one case no perfusion was observed on flow images but uptake restricted to the posterior fossa was seen on static images; the other case showed perfusion on the dynamic study and static imaging revealed hemispheric uptake with no posterior fossa uptake. Static perfusion 99Tcm-HMPAO studies offer advantages over conventional brain scintigraphy, better results being due to adequate assessment of posterior fossa activity and avoiding equivocal studies.