ABSTRACT
Mucopolysaccharidosis (MPS) Type VI, or Maroteaux-Lamy syndrome, is characterized by a deficiency of the enzyme arylsulfatase B (ASB). In patients with this disorder, craniocervical compression, carpal tunnel syndrome, and communicating hydrocephalus are common. Traditionally, hydrocephalus occurring in patients with MPS VI has been treated with shunt placements. Considering obstruction of the outlets from the fourth ventricle at the craniocervical transition, the authors decided to treat a female patient with MPS VI via endoscopic third ventriculostomy. She was 12 years old and had refractory headaches. This seems to be the first reported instance of the neuroendoscopic treatment of hydrocephalus in a patient with MPS VI. The pathophysiology is briefly discussed.
Subject(s)
Endoscopy/methods , Hydrocephalus/etiology , Hydrocephalus/surgery , Mucopolysaccharidosis VI/complications , Third Ventricle/surgery , Ventriculostomy/methods , Child , Humans , Hydrocephalus/psychology , Male , Memory Disorders/etiology , Memory Disorders/psychology , Mucopolysaccharidosis VI/psychology , Neuropsychological TestsABSTRACT
Intracranial schwannoma not related to cranial nerves are unusual and rarely found in the subfrontal region. We report a case of olfactory groove schwannoma in a 27-year-old male, who presented with anosmia and headache initiated one year ago. At admission, bilateral papilledema was noted with absense of motor deficits or cranial nerves abnormalities. Cranial computed tomography (CT) revealed a bifrontal multicystic isodense enhancing mass lesion causing a frontal ventricular horn compression. Radiological features resembled that of a cystic olfactory groove meningioma. Decompressive bifrontal craniotomy was done. One month later, CT demonstrated a homogeneously contrast-enhancing mass in the olfactory groove region who extended into the left nasal cavity. Magnetic resonance imaging did not add more informations. A second surgical procedure was done through a nasoethmoidal approach with incomplete resection of the lesion. The complete tumor resection was only possible in a third surgery through another bifrontal approach. The hystopathological diagnosis of schwannoma was performed by conventional methods and confirmed by immunohistoquemical staining for S-100 protein. The rarity of this tumor and his clinical, radiological and histological aspects justify this publication.
Subject(s)
Cranial Nerve Neoplasms/diagnosis , Neurilemmoma/diagnosis , Olfactory Nerve Diseases/diagnosis , Adult , Cranial Nerve Neoplasms/pathology , Cranial Nerve Neoplasms/surgery , Craniotomy , Humans , Immunohistochemistry , Magnetic Resonance Imaging , Male , Neurilemmoma/pathology , Neurilemmoma/surgery , Olfactory Nerve Diseases/pathology , Olfactory Nerve Diseases/surgery , Olfactory Pathways , Reoperation , Tomography, X-Ray ComputedABSTRACT
Schwannomas intracranianos näo associados a nervos cranianos säo incomuns e raramente encontrados na regiäo subfrontal. Apresentamos raro caso de schwannoma da goteira olfatória, acometendo paciente de 27 anos, masculino, com quadro iniciado há 1 ano com perda da olfaçäo e cefaléia. Ao exame de admissäo, apresentava papiledema bilateral e anosmia. Tomografia computadorizada de Cranio (TC) revelou processo expansivo bifrontal hipodenso ao parênquima, com aspecto multicístico, sem captaçäo do contraste iodado, promovendo compressäo dos cornos ventriculares frontais. Os achados radiológicos sugeriam meningeoma cístico da goteira olfatória. Foi submetido a craniotomia frontal para descompressäo. Um mês após, TC de controle revelou processo expansivo da regiäo da goteira olfatória homogeneamente captante do contraste iodado, que se estendia para o interior da cavidade nasal esquerda. RM näo adicionou novas informações. Foi realizado segundo procedimento cirúrgico por via naso-etmoidal, com ressecçäo incompleta da lesäo. A ressecçäo completa foi possível através de re-operaçäo por craniotomia bifrontobasal. O diagnóstico histopatológico de schwannoma foi realizado através de microscopia óptica convencional e confirmado por técnica de imuno-histoquímica, utilizando o anticorpo para proteína S-100. A raridade deste tumor, seus aspectos clínicos, radiológicos e histológicos justificam esta publicaçäo
Subject(s)
Humans , Male , Adult , Cranial Nerve Neoplasms , Neurilemmoma , Olfactory Nerve Diseases , Cranial Nerve Neoplasms , Craniotomy , Immunohistochemistry , Magnetic Resonance Imaging , Neurilemmoma , Olfactory Nerve Diseases , Olfactory Pathways , Reoperation , Tomography, X-Ray ComputedABSTRACT
Os autores relatam um caso de menongeoma atípico da goteira olfatória, acometendo um paciente de 74 anos, do sexo masculino, apresentado-se com quadro de hipertensão intracraniana e confusão mental. Após exérese cirúrgica, hove recidiva local precoce e matástase ganglionar cervical. As metástases extracranianas de meningeomas intracarnianos já foram bem documentadas, mas são raramente encontradas. Ocorre em menos de 0,1 por cento desses tumores. Os locais mais comuns dessas metástases são os pulmöes (60 por cento), o fígado (34 por cento), os linfonodos cervicais (18 por cento), os ossos longos, a pélvis e o crânio (11 por cento).