Displasia fibrosa craneofacial y quiste óseo aneurismático en una paciente con síndrome de McCune-Albright. Presentación de un caso y revisión de la literatura / Craniofacial fibrous dysplasia and aneurismal bone cyst in a patient with McCune-Albright syndrome. A case report and review of the literature

El síndrome de McCune-Albright (SMA) es un trastorno genético heterogéneo que se caracteriza por la tríada de displasia fibrosa (DF) poliostótica, manchas café con leche y múltiples endocrinopatías hiperfuncionales. En general, se diagnostica clínicamente. De la tríada, 2 de los hallazgos son suficientes para hacer el diagnóstico. La DF craneofacial es un término que se usa para describir la displasia fibrosa, que se localizaba en el esqueleto craneofacial y es común en pacientes con SMA. El quiste óseo aneurismático (QOA) es una lesión ósea no neoplásica infrecuente que afecta principalmente a los huesos largos y las vértebras, y puede ocurrir muy raramente en los huesos craneofaciales. Los QOA pueden ocurrir como enfermedades óseas secundarias en asociación con varios tumores óseos benignos y malignos y con displasia fibrosa. El QOA secundario que ocurre en la DF craneofacial es excepcional. Presentamos el caso de una paciente de 21 años tratada en nuestro centro de un quiste óseo aneurismático orbitario derecho asociado a SMA y realizamos una revisión de la literatura relevante (AU)

McCune-Albright syndrome (MAS) is a rare heterogeneous genetic disorder that is characterized by a triad of polyostotic fibrous dysplasia (FD), café au lait spots (CAL), and multiple hyperfunctional endocrinopathies. In general, it is diagnosed clinically. From the triads, 2of the findings are enough to make the diagnosis. Craniofacial fibrous dysplasia is a term that is used to describe the fibrous dysplasia, which was localized at the craniofacial skeleton and is common in MAS patients. Aneurysmal bone cyst (ABC) is a rare non-neoplastic bone lesion that involves mostly the long bones and vertebrae and may occur very rarely in the craniofacial bones. ABCs may occur as secondary bony pathologies in association with various benign and malignant bone tumors and with fibrous dysplasia. Secondary ABC occurring in craniofacial FD is extremely rare. We present the case of a 21-year-old patient treated at our center for a right orbital aneurysmal bone cyst associated with MAS and provide a review of the relevant literature (AU)

Craniofacial fibrous dysplasia and aneurismal bone cyst in a patient with McCune-Albright syndrome. A case report and review of the literature.

McCune-Albright syndrome (MAS) is a rare heterogeneous genetic disorder that is characterized by a triad of polyostotic fibrous dysplasia (FD), café au lait spots (CAL), and multiple hyperfunctional endocrinopathies. In general, it is diagnosed clinically. From the triads, 2 of the findings are enough to make the diagnosis. Craniofacial fibrous dysplasia is a term that is used to describe the fibrous dysplasia, which was localized at the craniofacial skeleton and is common in MAS patients. Aneurysmal bone cyst (ABC) is a rare non-neoplastic bone lesion that involves mostly the long bones and vertebrae and may occur very rarely in the craniofacial bones. ABCs may occur as secondary bony pathologies in association with various benign and malignant bone tumors and with fibrous dysplasia. Secondary ABC occurring in craniofacial FD is extremely rare. We present the case of a 21-year-old patient treated at our center for a right orbital aneurysmal bone cyst associated with MAS and provide a review of the relevant literature.

Pneumosinus dilatans frontal en niña de 9 años. Presentación de un caso y revisión de la literatura / Pneumosinus dilatans frontalis in 9-year-old child. Case report and review of the literature

El pneumosinus dilatans (PD) representa una enfermedad infrecuente en adultos, y verdaderamente excepcional en niños. Existen aproximadamente 100 casos descritos en adultos en la literatura, de los cuales menos de 10 han sido descritos en chicos con edades comprendidas entre los 12-16 años, y ninguno en niños. Se describe como una aireación anómala de un seno paranasal más allá del límite normal del hueso con mucosa normal. Puede producir alteraciones cosméticas o funcionales. Su etiología permanece siendo desconocida. Afecta fundamentalmente al seno frontal. Presentamos el caso de una niña de 9 años con diagnóstico de PD frontal desde los 3 años, intervenida quirúrgicamente por alteración cosmética relevante. La intervención quirúrgica fue practicada mediante abordaje endocraneal a través de incisión coronal con resultados cosméticos excelentes (AU)

Pneumosinus dilatans (PD) is a rare condition in adults and truly exceptional in children. There are approximately 100 reported cases in adults, with fewer than 10 in adolescents aged 12-16, and none in children. It is described as a benign airfilled expansion of a paranasal sinus beyond the normal osseous boundaries. It could result in cosmetic and functional complications. The precise etiology of the condition remains unknown. The frontal sinus is the most commonly affected. It is reported the case of a 9-year-old female diagnosed with PD frontalis at 3 years of age. She underwent surgery for significant cosmetic alterations. The surgical plan included an intracranial approach through coronal incision with excellent cosmetic results (AU)

Neurocitoma atípico de cerebelo que simula un hemangioblastoma. Presentación de un caso / Atypical cerebellar neurocytoma resembling a hemangioblastoma. A case report

Hasta agosto 2013 se han descrito alrededor de 105 casos de neurocitomas extraventriculares intracraneales, de los cuales el 6% se localizan en el cerebelo y el 22% son neurocitomas extraventriculares atípicos. El neurocitoma extraventricular atípico es una variante infrecuente, con solo 24 casos descritos y con un pronóstico más sombrío que el neurocitoma central típico. Se presenta un neurocitoma extraventricular atípico de cerebelo, nunca publicado hasta la fecha; de ahí el interés de este trabajo. Destaca la singularidad de ser un tumor quístico con nódulo mural, una presentación poco frecuente. Los neurocitomas extraventriculares son tumores con baja incidencia que deben considerarse en el diagnóstico diferencial inicial de lesiones cerebelosas quísticas con nódulo mural. Dado que el pronóstico depende del grado de atipia y de la resección quirúrgica, en casos de neurocitomas extraventriculares atípicos el seguimiento debe ser más estrecho, por el mayor riesgo de recidivas que presentan

Through August 2013, 105 cases of intracranial extraventricular neurocytoma (EVN) had been described; 6% were located in cerebellum and 22% were atypical EVN.A rare morphologic form of neurocytoma, atypical EVN has had only 24 cases reported to date. Its prognosis is poorer than the typical central neurocytoma. This case report describes an atypical cerebellar EVN, a form that has not been reported yet, hence the interest of this article. We emphasise its cystic nature and mural nodule, in an infrequent presentation.EVN are low-incidence tumours that we need to take into consideration when making the differential diagnosis of cystic cerebellar lesions with mural nodule. Given that the prognosis of atypical EVNs depends on the atypical nature and on the grade of resection, medical follow up has to be more constant, due to the greater degree of recurrence

[Atypical cerebellar neurocytoma resembling a hemangioblastoma. A case report]. / Neurocitoma atípico de cerebelo que simula un hemangioblastoma. Presentación de un caso.

Through August 2013, 105 cases of intracranial extraventricular neurocytoma (EVN) had been described; 6% were located in cerebellum and 22% were atypical EVN. A rare morphologic form of neurocytoma, atypical EVN has had only 24 cases reported to date. Its prognosis is poorer than the typical central neurocytoma. This case report describes an atypical cerebellar EVN, a form that has not been reported yet, hence the interest of this article. We emphasise its cystic nature and mural nodule, in an infrequent presentation. EVN are low-incidence tumours that we need to take into consideration when making the differential diagnosis of cystic cerebellar lesions with mural nodule. Given that the prognosis of atypical EVNs depends on the atypical nature and on the grade of resection, medical follow up has to be more constant, due to the greater degree of recurrence.

Fijación lumbar posterior empleando el sistema de imagen quirúrgica O-arm: experiencia inicial / Posterior lumbar fusion using the O-arm surgical imaging system: initial experience

Objetivo: La fijación con tornillos transpediculares en la columna lumbar es una técnica ampliamente aceptada para el tratamiento de patología traumática y degenerativa. Las complicaciones de la instrumentación espinal pueden ser graves. Sobrepasar caudal o medialmente la cortical del pedículo puede provocar lesiones nerviosas o durotomías incidentales. La cirugía guiada por imagen computarizada ha demostrado tasas más altas de precisión en la colocación de tornillos transpediculares comparada con la fluoroscopia convencional.El O-arm tiene la capacidad de obtener imágenes similares a la tomografía computarizada (TC) y realizar reconstrucciones multiplanares. En este estudio evaluamos una cohorte de pacientes en los que se realizó fijación lumbar posterior con tornillos transpediculares empleando el sistema de imagen quirúrgica O-arm. Métodos Se realizó un estudio retrospectivo de 40 casos consecutivos de fijación lumbar posterior empleando el O-arm. La población a estudio incluía 14 hombres y 26 mujeres. El rango de edad iba de 39 a 85 años, con una media de edad de 63,78 años. Veintiún pacientes presentaban estenosis del canal degenerativa (52,5%) y 19 espondilolistesis (47,5%). Se obtuvieron imágenes con TC intraoperatoria. Se recoge el (..) (AU)

Object: The use of transpedicular screw fixation has been widely accepted for the treatment of degenerative and traumatic pathology of the lumbar spine. Complications of spinal instrumentationcanbeserious.Screwmisplacementcanresultinunintendeddurotomy,nerveroot and/or cauda equina injury. In comparison to fluoroscopy-assisted screw placement, computer-assisted image guidance has been shown to achieve overall higher rates of accuracy. The O-arm is able to obtain computed tomography (CT)-type images with multiplanar reconstruction. In this study we evaluated a cohort of patients who underwent posterior lumbar fusion with pedicle screws utilizing the O-arm imaging system. Methods: A retrospective review of 40 consecutive patients who underwent posterior lumbar fusion surgery with O-arm utilization, was performed. The study population included (..) (AU)

Aneurisma de la arteria cerebral anterior que se presenta como masa del tercer ventrículo e hidrocefalia. A propósito de un caso / No disponible

Los aneurismas que se presentan como masas del tercer ventrículo son poco comunes, y en la mayor parte de los casos surgen del ápex de la arteria basilar. Presentamos el caso de un paciente varón de 67 años de edad que fue ingresado en el hospital con un cuadro de 4 semanas de evolución de inestabilidad de la marcha, incontinencia urinaria y pérdida progresiva de agudeza visual. La tomografía computarizada craneal mostraba una masa hiperdensa en el tercer ventrículo con dilatación triventricular. La resonancia magnética cerebral, la angiografía por resonancia magnética y la angiografía convencional identificaron esta lesión como un aneurisma parcialmente trombosado de la arteria cerebral anterior. Según nuestro conocimiento, este es el primer caso descrito de aneurisma de arteria cerebral anterior con estas características clínicas y radiológicas (AU)

[Anterior cerebral artery aneurism presenting as a third ventricular mass and hydrocephalus. Case report]. / Aneurisma de la arteria cerebral anterior que se presenta como masa del tercer ventrículo e hidrocefalia. A propósito de un caso.

Aneurysms which appear as third ventricular masses are uncommon; most are giant aneurysms arising from the basilar apex. We present the case of a 67-year-old male who was admitted to hospital with a 4-week history of gait instability, urinary incontinence and progressive visual loss. A cranial computed tomography scan revealed a hyperdense mass in the third ventricle with triventricular dilatation. Cerebral magnetic resonance imaging, magnetic resonance-angiography and conventional angiography identified this lesion as a partially thrombosed aneurysm of the anterior cerebral artery. To our knowledge, this is the first report of an anterior cerebral artery aneurysm with these clinical and radiological features.

[Posterior lumbar fusion using the O-arm surgical imaging system: initial experience]. / Fijación lumbar posterior empleando el sistema de imagen quirúrgica O-arm: experiencia inicial.

OBJECTIVES: The use of transpedicular screw fixation has been widely accepted for the treatment of degenerative and traumatic pathology of the lumbar spine. Complications of spinal instrumentation can be serious. Screw misplacement can result in unintended durotomy, nerve root and/or cauda equina injury. In comparison to fluoroscopy-assisted screw placement, computer-assisted image guidance has been shown to achieve overall higher rates of accuracy. The O-arm is able to obtain computed tomography (CT)-type images with multiplanar reconstruction. In this study we evaluated a cohort of patients who underwent posterior lumbar fusion with pedicle screws utilizing the O-arm imaging system. METHODS: A retrospective review of 40 consecutive patients who underwent posterior lumbar fusion surgery with O-arm utilization, was performed. The study population included 14 males and 26 females. Age range was 39-85 years with an average of 63.8 years. Twenty one patients had degenerative lumbar stenosis (52.5%) and 19 had spondylolisthesis (47.5%). Intraoperative CT-images were obtained. The mean time for surgery and screw placement was assessed. RESULTS: A total of 252 pedicle screws were sited using O-arm navigation system, with a mean of 6.3 screws per patient (range 4-10). On the basis of intraoperative CT, 3 screws were redirected, representing a 98.81% accuracy rate. The mean duration of surgery was 157.2 (90-240) minutes and the mean time for screw placement was 7.13 (3.08-15) minutes per screw. Three patients (7.5%) developed superficial wound infections which were treated conservatively. No patients required a return to the operating room because of screw malposition. CONCLUSION: The use of intraoperative O-arm imaging system with computer-assisted navigation significantly increases the surgical accuracy and safety of pedicle screw placement in lumbar fusion surgery.