[Multidisciplinary approach to the endolymphatic sac tumour]. / Abordaje multidisciplinar al tumor de saco endolinfático.

INTRODUCTION: Papilar adenocarcinoma of endolymphatic sac is related with Von Hippel Lindau disease at 15% of cases, has a slow growing with a high local aggressiveness, and doesn't metastasize. It causes symptoms of Meniere's syndrome due to the compression that produces at endolymphatic duct. When it presents with hearing loss is usually sudden and irreversible manner. The diagnostic is made with image tests and analysis of its structure with immunohistochemical tests. The elective treatment is surgical remove, and its main complication the perioperative bleeding it can be avoided with preoperative embolization or stereotactic radiation. CASE REPORT: A case of endolymphatic sac tumour is presented, in a 17-years-old male with unilateral deafness and crisis of rotate vertigo, with family history of Von Hippel-Lindau disease. Perceptive deafness and right vestibular arreflexia are detected at technical exploration. In a petrous bone computer tomography appears a mass at vestibular aqueduct. We performed a petrosectomy with presigmoidal approach and saving of inner ear. Pathological analysis revealed an endolymphatic sac tumour. DISCUSSION: In patients with a family history of Von Hippel Lindau disease and clinical symptoms of vertigo and normal hearing or with slight hearing loss we should suspect the presence of endolymphatic sac tumor. The clinical presentation of hearing loss can be sudden and irreversible even with negative or inconclusive images. Therefore, a quick action is important for the preservation of this function.

Abordaje multidisciplinar al tumor de saco endolinfático / Multidisciplinary approach to the endolymphatic sac tumour

Introducción. El adenocarcinoma papilar de saco endolinfático se asocia a la enfermedad de Von Hippel Lindau en el 15% de los casos, tiene un crecimiento lento pero elevada agresividad local, y no metastatiza. Clínicamente produce un Síndrome de Menière derivado de la compresión que provoca en el conducto endolinfático. Cuando debuta con pérdida de audición suele ser de forma brusca e irreversible. Se diagnostica con técnicas de imagen y el análisis de su estructura con inmunohistoquímica. Su tratamiento electivo es la exéresis quirúrgica, y su principal complicación el sangrado perioperatorio, que se puede evitar con embolización o radiación estereotáctica preoperatorio.Caso clínico. Presentamos un caso de un tumor de saco endolinfático en un paciente de 17 años aquejado de sordera unilateral y crisis de vértigo rotatorio, con antecedentes familiares de enfermedad de Von Hippel Lindau. Las pruebas complementarias mostraron una hipoacusia neurosensorial y una arreflexia vestibular derechas. En tomografía computarizada de peñascos se apreciaba una lesión en el acueducto vestibular. Se sometió al paciente a una petrosectomía con abordaje presigmoideo y preservación de laberinto, realizándose una exéresis total de la lesión. Se diagnosticó de tumor del saco endolinfático en el análisis anatomopatológico.Discusión. Ante un paciente con antecedentes familiares de enfermedad de Von Hippel Lindau y un cuadro clí-nico de vértigo incluso sin hipoacusia, o siendo esta leve, habría que pensar en la presencia de un tumor del saco endolinfático. La presentación clínica de sordera puede ser brusca e irreversible, incluso con imágenes negativas o poco concluyentes, por lo que una rápida actuación es importante para la preservación de esta función (AU)

Introduction. Papilar adenocarcinoma of endolymphatic sac is related with Von Hippel Lindau disease at 15% of cases, has a slow growing with a high local aggressiveness, and doesn’t metastasize. It causes symptoms of Meniere’s syndrome due to the compression that produces at endolymphatic duct. When it presents with hearing loss is usually sudden and irreversible manner. The diagnostic is made with image tests and analysis of its structure with immunohistochemical tests. The elective treatment is surgical remove, and its main complication the perioperative bleeding it can be avoided with preoperative embolization or stereotactic radiation.Case report. A case of endolymphatic sac tumour is presented, in a 17-years-old male with unilateral deafness and crisis of rotate vertigo, with family history of Von Hippel-Lindau disease. Perceptive deafness and right vestibular arreflexia are detected at technical exploration. In a petrous bone computer tomography appears a mass at vestibular aqueduct. We performed a petrosectomy with presigmoidal approach and saving of inner ear. Pathological analysis revealed an endolymphatic sac tumour.Discussion. In patients with a family history of Von Hippel Lindau disease and clinical symptoms of vertigo and normal hearing or with slight hearing loss we should suspect the presence of endolymphatic sac tumor. The clinical presentation of hearing loss can be sudden and irreversible even with negative or inconclusive images. Therefore, a quick action is important for the preservation of this function (AU)

Delayed extradural hematoma after mild head injury: report of three cases.

BACKGROUND: Extradural hematoma has been classically considered to be an acute complication of head injury whose maximum development takes place in the minutes following trauma. Delayed extradural hematoma (DEH) is defined on the basis of an exclusively radiologic criterion: epidural hematoma that is not present in the first neuroradiologic examination made after trauma but that appears in sequential neuroradiologic examinations during patient evolution. This is an infrequent complication that usually appears in hypotensive multiple trauma patients or is related to severe head injury with other intracranial lesions. CASE DESCRIPTION: We present three cases of DEH after mild head injury (GCS > 12) without associated intracranial or traumatic systemic lesions. Therefore, those usually considered to be "protective mechanisms" responsible for delayed development of an extradural hematoma were absent in our three patients. Diagnosis was attained by means of repetition of cranial computed tomography (CT) scan after neurologic impairment was noted. Surgical evacuation of DEH was immediately performed after diagnosis. Postoperative outcome was favorable in two patients who suffered DEH in the supratentorial compartment. One patient who presented a posterior fossa DEH died 3 days after surgery. CONCLUSIONS: Early diagnosis and immediate treatment have proved to be essential for improving the prognosis of patients affected by DEH. Hospital admission under neurologic observation is recommended for patients who have sustained mild head injury associated with those factors that are related to the development of DEH, including GCS score under 15 or the detection of a skull fracture. Normality of a CT scan does not rule out subsequent appearance of delayed traumatic lesions.

Intradiploic epidermoid cysts.

We studied 37 intradiploic epidermoid cysts, reviewing typical and atypical radiological features and the differential diagnosis. The most common clinical feature was a long standing lump in the scalp, occurring in 25 patients (67.7%). Plain films were the most cost-effective radiological technique in diagnosis. The typical finding was a well-defined lytic lesion with sclerotic border, seen in 29 cases (78%). Atypical lesions were those larger than 5 cm and/or with an ill-defined edge, being observed in 8 cases (22%). CT and MRI were the best methods for assessing atypical ones. In all cases with typical radiological findings a preoperative diagnosis of intradiploic epidermoid cyst was suggested.

Idiopathic chronic hypertrophic craniocervical pachymeningitis: case report.

A 55-year-old woman with a unique form of chronic hypertrophic pachymeningitis involving the posterior fossa and upper cervical spine is reported. Unlike other cases previously described, the clinical picture was dominated by signs of increased intracranial pressure, lower cranial nerve disorders, and a progressive cervical radiculomyelopathy. The diagnosis was made by means of a contrast-enhanced magnetic resonance imaging scan and confirmed by histological examination of the excised dura. Surgical treatment with removal of the hypertrophic dura provided temporary relief, although the natural history of the disease was not modified. Exhaustive bacteriological and histopathological studies failed to identify a specific cause for this diffuse hypertrophy of the cranial and cervical dura. The literature is reviewed, and other histologically documented cases are discussed.