Objectives: To determine the clinical presentation and treatment outcome of pediatric intracranial cavernous malformation (CM) in a single-centered institution. Methods: Clinical data review of 30 patients under 18 years-old who had undergone surgery for cavernous malformation from January 1993 to December 2011. Results: The Study Group included 18 males and 12 females (mean age: 8.7 years-old). Symptoms at presentation were seizures (16/30, 53.3%), headache (15/30, 50.0%), and focal neurological deficits (11/30, 36.6%). Multiple cavernous malformations were found in 5/30 (16.6%). According to location, patients were classified in groups: (G1) brain-steam in 5/30 (16.6%), (G2) cerebellum in 2/30 (6.6%), (G3) supratentorial associated with seizures in 16/30 (53.3%), and (G4) supratentorial without seizures in 7/30 (23.3%). Surgical resection was performed in 26 out of 30 (86.6%) patients. The mean follow-up period was 4.1 years. Of 15 children followed-up with preoperative seizures, all were rendered seizure-free after surgery. Conclusions: For symptomatic solitary cavernous malformation, the treatment of choice is complete microsurgical excision preceded by careful anatomical and functional evaluation. For multiple cavernous malformation or asymptomatic patients, the treatment modalities must be cautiously considered.
The management of a large complex skull defect in children is still controversial. Each case should be extensively debated in craniofacial units. In our case, bioceramics proved to be an effective and good alternative for final skull reconstruction.
The interest of this case lies in the rarity of the causative organism and the atypical features of clinical and neurological images in a term neonate.
RESUMO -Schwannomas intracranianos não associados a nervos cranianos são incomuns e raramente encontrados na região subfrontal. Apresentamos raro caso de schwannoma da goteira olfatória, acometendo paciente de 27 anos, masculino, com quadro iniciado há 1 ano com perda da olfação e cefaléia. Ao exame de admissão, apresentava papiledema bilateral e anosmia. Tomografia computadorizada de Cranio (TC) revelou processo expansivo bifrontal hipodenso ao parênquima, com aspecto multicístico, sem captação do contraste iodado, promovendo compressão dos cornos ventriculares frontais. Os achados radiológicos sugeriam meningeoma cístico da goteira olfatória. Foi submetido a craniotomia frontal para descompressão. Um mês após, TC de controle revelou processo expansivo da região da goteira olfatória homogeneamente captante do contraste iodado, que se estendia para o interior da cavidade nasal esquerda. RM não adicionou novas informações. Foi realizado segundo procedimento cirúrgico por via naso-etmoidal, com ressecção incompleta da lesão. A ressecção completa foi possível através de re-operação 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 publicação. PALAVRAS-CHAVE: anosmia, schwannoma; nervo olfatório, imuno-histoquímica.Olfactory groove schwannoma: case report Olfactory groove schwannoma: case report Olfactory groove schwannoma: case report Olfactory groove schwannoma: case report Olfactory groove schwannoma: case report ABSTRACT -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.
RESUMO PALAVRAS-CHAVE Meningeoma. Metástase de meningeoma. ABSTRACT Metastatic meningioma to cervical lymph nodes
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