ABSTRACT
INTRODUCTION: A vein of Galen aneurysmal malformation (VGAM) is a high-flow arteriovenous malformation that typically presents in early childhood. Complications associated with this condition include heart failure and hydrodynamic disorders resulting from high blood flow. Hydrocephalus is one of the hydrodynamic disorders. It can present in a chronic form due to a defect in the absorption of cerebrospinal fluid (CSF) resulting from venous hypertension or in an acute/subacute form due to obstruction of normal circulation routes caused by the mass effect of the VGAM. Currently, endovascular closure of the lesion is considered the treatment of choice. However, in acute/subacute presentations of obstructive hydrocephalus, progression may occur despite endovascular treatment, necessitating a cerebrospinal fluid diversion procedure. Prior to the advent of endoscopic treatment, ventricular shunts were utilized, but outcomes were poor due to the pathological hydrodynamic parameters associated with the malformation. The role of endoscopic third ventriculostomy as a treatment option in these situations remains unclear, and there is limited literature available. Therefore, we present the case of a patient with a vein of Galen aneurysmal malformation and obstructive hydrocephalus, which was treated via ETV. Furthermore, a literature review was conducted. CASE DESCRIPTION: We present a 5-month-old infant who was diagnosed at birth with VGAM. At 2 months of age, a partial closure of the malformation was performed via endovascular. Subsequently, the patient presented at the emergency department with symptoms of irritability, lethargy, vomiting, feeding refusal, and "setting sun" sign of several days' duration due to obstructive hydrocephalus. The patient was successfully treated via ETV. Following this, a new arteriography was performed and revealed occlusion of the VGAM. The child clinically improved and currently exhibits mild psychomotor impairment after 1-year follow-up. CONCLUSION: In the literature, there are a limited number of reports on the treatment of obstructive hydrocephalus associated with VGAM via ETV. These reports have demonstrated good outcomes with minimal surgical complications. Our patient also exhibited good results. As such, ETV is a viable and safe option for the treatment of obstructive hydrocephalus associated with VGAM in pediatric patients. However, more research is needed to establish the effectiveness of ETV in comparison to other treatment options and to understand the long-term outcomes.
Subject(s)
Cerebral Veins , Hydrocephalus , Third Ventricle , Infant, Newborn , Humans , Infant , Child, Preschool , Child , Cerebral Veins/diagnostic imaging , Cerebral Veins/surgery , Third Ventricle/surgery , Hydrocephalus/complications , Hydrocephalus/diagnostic imaging , Ventriculostomy/methods , Cerebrospinal Fluid Shunts/methodsABSTRACT
BACKGROUND: The polymalformative syndromes and craniofacial anomalies association is a well-known phenomenon in patients with Crouzon, Pfeiffer, Apert, or Muenke disease. Recently, other less frequent pathologies, such as Alagille syndrome, have shown an association with alterations in the development of cranial sutures, resulting in serious cosmetic defects and neurologic disorders. CASE DESCRIPTION: We report an exceptional case of a 30-month-old girl, a nephroblastoma survivor diagnosed with Alagille syndrome, who was referred to our department with progressive anterior plagiocephaly and premature left coronal suture closure associated with a large compensating right bossing. Despite the patient's age, we offered aggressive surgical treatment performing a new forehead harvested from the skull vertex with orbital rim reconstruction. CONCLUSIONS: Alagille syndrome is a complex multisystem pathology with a poor craniosynostosis association and only 3 cases have been described in the literature.
Subject(s)
Alagille Syndrome/diagnostic imaging , Alagille Syndrome/surgery , Craniosynostoses/diagnostic imaging , Craniosynostoses/surgery , Plagiocephaly/diagnostic imaging , Plagiocephaly/surgery , Alagille Syndrome/complications , Child, Preschool , Craniosynostoses/complications , Female , Humans , Plagiocephaly/complications , Printing, Three-Dimensional/statistics & numerical dataABSTRACT
BACKGROUND: Parasagittal meningiomas arise from the arachnoid cells of the angle formed between the superior sagittal sinus (SSS) and the brain convexity. In this retrospective study, we focused on factors that predict early recurrence and recurrence times. METHODS: We reviewed 125 patients with parasagittal meningiomas operated from 1985 to 2014. We studied the following variables: age, sex, location, laterality, histology, surgeons, invasion of the SSS, Simpson removal grade, follow-up time, angiography, embolization, radiotherapy, recurrence and recurrence time, reoperation, neurologic deficit, degree of dependency, and patient status at the end of follow-up. RESULTS: Patients ranged in age from 26 to 81 years (mean 57.86 years; median 60 years). There were 44 men (35.2%) and 81 women (64.8%). There were 57 patients with neurologic deficits (45.2%). The most common presenting symptom was motor deficit. World Health Organization grade I tumors were identified in 104 patients (84.6%), and the majority were the meningothelial type. Recurrence was detected in 34 cases. Time of recurrence was 9 to 336 months (mean: 84.4 months; median: 79.5 months). Male sex was identified as an independent risk for recurrence with relative risk 2.7 (95% confidence interval 1.21-6.15), P = 0.014. Kaplan-Meier curves for recurrence had statistically significant differences depending on sex, age, histologic type, and World Health Organization histologic grade. A binary logistic regression was made with the Hosmer-Lemeshow test with P > 0.05; sex, tumor size, and histologic type were used in this model. CONCLUSIONS: Male sex is an independent risk factor for recurrence that, associated with other factors such tumor size and histologic type, explains 74.5% of all cases in a binary regression model.
Subject(s)
Meningeal Neoplasms/surgery , Meningioma/surgery , Neoplasm Recurrence, Local/diagnosis , Adult , Age Factors , Aged , Aged, 80 and over , Female , Humans , Kaplan-Meier Estimate , Logistic Models , Male , Meningeal Neoplasms/diagnosis , Meningeal Neoplasms/epidemiology , Meningeal Neoplasms/pathology , Meningioma/diagnosis , Meningioma/epidemiology , Meningioma/pathology , Middle Aged , Neoplasm Recurrence, Local/epidemiology , Neoplasm Recurrence, Local/pathology , Prognosis , Risk Assessment , Risk Factors , Sex Factors , Tumor BurdenABSTRACT
El condroma intracraneal es un tumor cartilaginoso benigno inusual con una incidencia inferior al 1% de todos los tumores primarios intracraneales. El origen de los condromas intracraneales no se ha establecido definitivamente. Rara vez se detectan en el ángulo pontocerebeloso. Describimos un nuevo caso de condroma del ángulo pontocerebeloso que clínica y radiológicamente se diagnosticó como schwannoma. Aunque el estudio histopatológico sigue siendo el «gold standard» para el diagnóstico definitivo, es importante que neurólogos, radiólogos y neurocirujanos conozcan esta entidad para realizar el diagnóstico diferencial y tratamiento apropiados (AU)
Intracranial chondroma is a rare, benign, cartilaginous tumour accounting for less than 1% of all primary intracranial tumours and with an uncertain origin. Very few cases have been reported involving the cerebellopontine angle. We describe a new case of cerebellopontine angle chondroma which was clinically and radiologically diagnosed as schwannoma. Although histopathological examination is the gold standard for definitive diagnosis, it is important that neurologists, radiologists and neurosurgeons are aware of this entity for correct differential diagnosis and treatment (AU)
Subject(s)
Humans , Male , Adult , Chondroma/diagnosis , Chondroma/pathology , Brain Neoplasms/pathology , Brain Neoplasms , Cerebellopontine Angle/pathology , Cerebellopontine Angle , Chondroma , Nerve Sheath Neoplasms/pathology , Diagnosis, Differential , Neuroimaging/instrumentation , Neuroimaging/methods , NeuroimagingABSTRACT
Los condromas craneales son tumores derivados de remanentes de células embrionarias condrocíticas que habitualmente aparecen en la sincondrosis de la base del cráneo. A diferencia del resto del organismo,donde los tumores condroides constituyen el tumor óseo primario más frecuente solo por detrás de los de estirpe hematopoyética, a nivel craneal constituyen una entidad poco frecuente con una incidencia de menos del 1% de los tumores intracraneales. Presentamos el caso de un varón de 42años remitido a nuestra consulta por el hallazgo de una lesión extraaxial situada en la región del cavum de Meckel y extensión a la fosa posterior con compresión del troncoencéfalo tras clínica de paraparesia de 6meses de evolución. Bajo el diagnóstico de un neurinoma del V par craneal se realiza una exéresis subtotal del tumor mediante un abordaje combinado supra-infratentorial presigmoideo. El resultado anatomopatológico postoperatorio confirma el diagnóstico de condroma craneal
Cranial chondromas are tumours arising from chondrocyte embryonic remnants cells that usually appear in the skull base synchondrosis. In contrast to the rest of the organism, where chondroid tumours are the most common primary bone tumour just behind the haematopoietic lineage ones, they are a rarity at cranial level, with an incidence of less than 1% of intracranial tumours. The case is reported on a 42 year-old male referred to our clinic due to the finding of an extra-axial lesion located close to the Meckel's cave region, with extension to the posterior fossa and brainstem compression after progressive paraparesis of 6 months onset. With the diagnosis of trigeminal schwannoma, a subtotal tumour resection was performed using a combined supra-infratentorial pre-sigmoidal approach. The postoperative histopathology report confirmed the diagnosis of cranial condroma
Subject(s)
Humans , Male , Adult , Chondroma/surgery , Brain Neoplasms/surgery , Skull Base Neoplasms/surgery , Dura Mater/pathology , Cartilage/pathologyABSTRACT
Cranial chondromas are tumours arising from chondrocyte embryonic remnants cells that usually appear in the skull base synchondrosis. In contrast to the rest of the organism, where chondroid tumours are the most common primary bone tumour just behind the haematopoietic lineage ones, they are a rarity at cranial level, with an incidence of less than 1% of intracranial tumours. The case is reported on a 42 year-old male referred to our clinic due to the finding of an extra-axial lesion located close to the Meckel's cave region, with extension to the posterior fossa and brainstem compression after progressive paraparesis of 6 months onset. With the diagnosis of trigeminal schwannoma, a subtotal tumour resection was performed using a combined supra-infratentorial pre-sigmoidal approach. The postoperative histopathology report confirmed the diagnosis of cranial chondroma.
Subject(s)
Chondroma/diagnosis , Cranial Nerve Neoplasms/diagnosis , Neurilemmoma/diagnosis , Skull Base , Skull Neoplasms/diagnosis , Trigeminal Nerve Diseases/diagnosis , Adult , Diagnosis, Differential , Humans , MaleABSTRACT
El creciente auge actual del uso de la craniectomía descompresiva conlleva implícita la aparición de complicaciones derivadas de la alteración en la presión barométrica intracraneal y del equilibrio tanto hidrostático como hemodinámico. La herniación paradójica transtentorial («paradoxycal transtentorial herniation») representa una rara manifestación, englobada dentro del síndrome del craniectomizado, de extrema gravedad y de tratamiento relativamente simple. Presentamos el caso de una mujer de 56 años, sin antecedentes de interés, que tras ser intervenida de un meningioma del surco olfatorio presenta un hematoma en el lecho quirúrgico con abundante edema, requiriendo una segunda intervención de urgencia sobre este sin posibilidad de realizar la reposición ósea. Durante el posoperatorio se observa un gran deterioro neurológico de la paciente con el ortostatismo, que se resolvía espontáneamente con el decúbito y que se solventa con la reposición ósea posteriormente. Se analizan los posibles factores predisponentes y las posibles etiologías del cuadro
The current increasing use of decompressive craniectomy carries the implicit appearance of complications due to alterations in both intracranial pressure and in the hydrostatic-hemodynamic equilibrium. Paradoxical transtentorial herniation represents a rare manifestation, included in trephine syndrome, extremely critical but with relatively simple treatment. We present the case of a 56-year-old woman with no interesting medical history, who, after an olfactory groove meningioma surgery, presented a haemorrhage located in the surgical area with an important oedema. The patient required a second emergency surgery without any chance of conserving the cranial vault. During the post-operational period, great neurological deterioration in orthostatic position was noticed, which resolved spontaneously in decubitus. This deficit was resolved with bone replacement afterwards. We discuss possible predisposing factors and aetiologies of this pathology
Subject(s)
Humans , Female , Middle Aged , Encephalocele/physiopathology , Decompressive Craniectomy/adverse effects , Intracranial Hypotension/physiopathology , Encephalocele/etiology , Brain Edema/etiology , Risk FactorsABSTRACT
The current increasing use of decompressive craniectomy carries the implicit appearance of complications due to alterations in both intracranial pressure and in the hydrostatic-hemodynamic equilibrium. Paradoxical transtentorial herniation represents a rare manifestation, included in "trephine syndrome", extremely critical but with relatively simple treatment. We present the case of a 56-year-old woman with no interesting medical history, who, after an olfactory groove meningioma surgery, presented a haemorrhage located in the surgical area with an important oedema. The patient required a second emergency surgery without any chance of conserving the cranial vault. During the post-operational period, great neurological deterioration in orthostatic position was noticed, which resolved spontaneously in decubitus. This deficit was resolved with bone replacement afterwards. We discuss possible predisposing factors and aetiologies of this pathology.
