Anterior Unilateral Plagiocephaly in Patient with Alagille Syndrome: Case Report.

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.

Risk of Recurrence in Operated Parasagittal Meningiomas: A Logistic Binary Regression Model.

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.

Condroma adyacente al cavum de Meckel simulando un neurinoma del quinto par craneal. A propósito de un caso / Chondroma adjacent to Meckel's cave mimicking a fifth cranial nerve neurinoma. A case report

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

[Chondroma adjacent to Meckel's cave mimicking a fifth cranial nerve neurinoma. A case report]. / Condroma adyacente al cavum de Meckel simulando un neurinoma del quinto par craneal. A propósito de un caso.

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.