Incidence of basilar invagination in patients with tonsillar herniation ? a case control craniometrical study / Incidência da invaginação basilar com heriação tonsilar ? estudo da craniometria do tipo caso-controle

A retrospective case-control study based on craniometrical evaluation was performed to evaluate the incidence of basilar invagination (BI). Patients with symptomatic tonsillar herniation treated surgically had craniometrical parameters evaluated based on CT scan reconstructions before surgery. BI was diagnosed when the tip of the odontoid trespassed the Chamberlain’s line in three different thresholds found in the literature: 2, 5 or 6.6 mm. In the surgical group (SU), the mean distance of the tip of the odontoid process above the Chamberlain’s line was 12 mm versus 1.2 mm in the control (CO) group (p<0.0001). The number of patients with BI according to the threshold used (2, 5 or 6.6 mm) in the SU group was respectively 19 (95%), 16 (80%) and 15 (75%) and in the CO group it was 15 (37%), 4 (10%) and 2 (5%).

Realizamos estudo retrospectivo tipo caso-controle baseado na avaliação craniométrica para avaliar a incidência da Invaginação Basilar (IB). Pacientes com herniação tonsilar sintomática tratada cirurgicamente foram avaliados quanto a parâmetros craniométricos obtidos em reconstrução de TC antes da cirurgia. IB foi diagnosticada quando a ponta do odontóide passava acima da linha de Chamberlain em 2, 5 ou 6,6 mm. No grupo cirúrgico (CI), a distância média da ponta do odontóide acima da linha de Chamberlain foi de 12 mm versus 1,2 mm no grupo controle (CO) (p<0.0001). O número de pacientes com IB conforme o critério diagnóstico usado (2, 5 ou 6,6 mm) foi de 19 (95%), 16 (80%) e 15 (75%) no grupo CI, respectivamente, contra 15 (37%), 4 (10%) e 2 (5%) no grupo CO. Pacientes com herniação tonsilar tinham maior incidência de IB comparados ao grupo controle.

Tectocerebellar dysraphia and occipital encephalocele: an unusual association with abdominal situs inversus and congenital heart disease.

Tectocerebellar dysraphia is a rare constellation of malformations comprising of occipital encephalocele, aplasia of the cerebellar vermis and deformity of the tectum. We describe a 7 month old infant who presented with tectocerebellar dysraphia associated with double outlet right ventricle, pulmonary stenosis and abdominal situs inversus. This association has not been reported in the literature, to the best of our knowledge.

Adult atretic parietal cephalocele.

Atretic parietal cephalocele is a congenital anomaly of childhood with poor prognostic value. The authors report an adult with associated brain malformations (interhemispheric cyst, partial corpus callosum agenesis) - but, interestingly, without any clinical symptoms related to the entity.

Peso del encéfalo normal del venezolano adulto según sexo y edad / Normal brain weight in venezuelan adults related to sex and age

Se revisa el peso de los encéfalos de sujetos con edades comprendidas entre 19 y 100 años, obtenidos de 3967 necropsias suscesivas realizadas durante un período de diez años en el Hospital General del Sur de Maracaibo. El análisis del peso de 1104 cerebros adultos normales arroja un promedio de 1215 g para sujetos sel sexo masculino y de 1122 g para el sexo femenino. Se obseva que el peso encefálico disminuye a medida que se incrementa la edad de los sujetos, disminución que es más acentuada después de los 65 años en los hombres y de 75 años en las mujeres

Atretic encephalocele/myelocele: case reports with emphasis on pathogenesis

Atretic encephaloceles or myelomeningoceles are frequently solid due to hamartomatous proliferation of fibrous tissue and blood vessels. Because of the fibrous nature of the tumor with no cystic cavity and unusual location with no connection to CNS, they are frequently regarded as insignificant hamartomas. Apart from this terminology, they are also described as cutaneous meningiomas or hamartomas with ectopic meningothelial elements by the presence of meningothelial cells. We report a case of atretic encephalocele in the parietal scalp of an 8 year-old boy and a case of myelomeningocele in the posterior mediastinum of a 31 year-old woman. The terms atretic encephalocele and myelomeningocele are more appropriate for these cases because they include their pathogenesis and the non-neoplastic nature of the lesion.

Encefalocele basal y Síndrome de Morning Glory / Basal encephalocele and Morning Glory Syndrome

Se presenta un caso de síndrome de "Morning Glory" asociado con encefalocele nasal (anteriormente operado) destacándose la importancia de esta asociación con los defectos de línea media

Meckel-Gruber syndrome: a lethal combination of abnormalities

We herein describe two cases of Meckel-Gruber Syndrome identified in stilborn infants. Both had all three elements of the classical triad, namely, occipital encephalocele, renal cystic dysplasia and post-axial polydactyly. In addition, many of the other well-known accompanying abnormalities were present. Awareness of this entity in this region is important because of its high risk of recurrence in subsequent pregnancies

Diagnóstico prenatal del síndrome de Meckel-Gruber / Prenatal diagnostic of Meckel-Gruber syndrome

Se diagnosticaron por ecosonografía dos casos con el síndrome de Meckel-Gruber, caracterizados por microcefalia con encefalocele occipital, riñones poliquísticos y polidactilia. Se comenta su frecuencia, asociación con otras malformaciones. Se discute el valor de la alfa-fetoproteína en el suero y el líquido amniótico. Enfatizamos la necesidad del consejo genético y de la valoración ecográfica, debido a la racurrencia de un 25% de este síndrome