Dermoid cyst with cerebellar meningoencephalocele at different locations accompanied by posterior fossa abnormalities: case report

ABSTRACT CONTEXT: Dermoid cysts are well-defined cysts containing sebaceous glands and dermal structures. In the literature, dermoid cysts and associated closure defects have been described in the same locations. CASE REPORT: In this case, a dermoid cyst was found at the base of the mouth with a coexisting closure defect in the occipital calvarium. Additional abnormalities were also observed, including posterior myeloschisis, right cerebellar dysgenesis, vermian hypogenesis and posterior fusion of the second and third vertebrae. The finding of a dermoid cyst located at the base of the mouth is discussed here, with additional imaging findings. CONCLUSION: Dermoid cysts in the head and neck region may be accompanied by posterior fossa abnormalities.

Single stage repair and reconstruction of trigonocephaly and fronto orbito ethmoidal encephalocele

A nine- year-old Iranian girl presented with trigonocephaly and frontonasal deformity secondary to encephalocele of fronto orbito ethmoidal region. She had undergone an attempt to correct the deformity at the age of three. The case was managed by a single stage repair and reconstruction of both fronto orbito nasal deformity and trigonocephaly along with plication of the herniating encephalocele. Fronto nasal deformity, interorbital hypertelorism nasal deformity and associated trigonocephaly are the main aesthetic concerns in the management of craniofacial disfigurement associated with fronto ethmoidal encephalaceles. A single stage repair and reconstruction of these cases is always preferred

Basal encephalocele associated with morning glory syndrome: case report

The basal encephaloceles refer to rare entities and they correspond to herniation of brain tissue through defects of skull along the cribiform plate or the sphenoid bone. A rare morning glory syndrome, with characteristic retinal defect has been reported in association with basal encephaloceles. Hypophysis hormonal deficiencies may occur. We accounted for a pituitary dwarfism with delayed diagnosed transsphenoidal encephalocele associated with morning glory syndrome, showing the alterations found in retinography, computed tomography and magnetic resonance imaging.

As encefaloceles basais são entidades raras e correspondem a herniações do tecido cerebral através de um defeito do crânio, ao longo da lâmina crivosa etmoidal ou do osso esfenoidal. A rara síndrome morning glory, com alterações de fundo de olho características pode apresentar-se associada à encefalocele basal. Deficiências hormonais hipofisárias podem ocorrer. Relatamos caso de nanismo hipofisário com encefalocele transesfenoidal de diagnóstico tardio associada à síndrome de morning glory, mostrando as alterações na retinografia, tomografia computadorizada e ressonância magnética.

CSF Rhinorrhea and recurrent meningitis caused by transethmoidal meningoencephaloceles.

We report two children presenting with intermittent CSF rhinorrhea and recurrent meningitis. CT scan showed transethmoidal meningoencephalocele.

Meckel Gruber syndrome--a single gene cause of recurrent neural tube defects.

Meckel Gruber syndrome (MGS), an autosomal recessive disorder characterised by posterior encephalocoele, multicystic kidneys and post-axial polydactyly should be recognised by obstetricians and paediatricians to counsel parents regarding the 25% recurrence risk. We report a consanguineous family with MGS affecting three infants.

Concomitancia de meningoencefalocele y colesteatomas en el oído medio: comentarios sobre 1 caso / Concomitance of meningoencephalocele and cholesteatomas in the middle ear: comments on 1 case

Se presenta el caso de una niña de 9 años, con un meningoencefalocele mastoideo, adosado a un colesteatoma residual, secundarios a un colesteatoma primario petromastoideo, operado 2 años antes, que ocupaban toda la cavidad mastoidea hasta el meato auditivo externo. Conjuntamente se extirparon 3 perlas de colesteatoma de la caja timpánica que destruyeron parte de la cadena osicular, y fue necesario realizarle una columelización con los restos del martillo. Actualmente, 15 años después, conserva buena evolución clínica y audiométrica

Exoftalmos por encefalocele intraorbitario / Exophtalmos due to intraorbital encephalocele

Se estudia a una paciente de 8 años de edad que asiste a consulta por exoftalmia unilateral. Se realiza una descripción bibliográfica acerca de esta enfermedad y sus diferentes causas. Se destaca el valor del examen clínico y los diferentes métodos diagnósticos. Sobresalen como detalles de interés la baja incidencia del encefalocele basal como causa de exoftalmos y la importancia de conocer los signos producidos por defectos de la línea media y que pueden asociarse a esta entidad

Rinoliquorréia: origem múltipla? / Cerebrospinal rhinorrhea: multiple origin?

É apresentado um caso de rinoliquorréia, que surgiu 12 anos após traumatismo craniano. Tendo o paciente apresentado nos últimos anos, duas meningites. A perda líquida nasal, cessou apenas após a correçäo cirúrgica de três lesöes encontradas: a nível das células da mastóide, a nível da lâmina crivosa do etmóide e por correçäo de meningoencefalocele frontal. Trata-se de um caso peculiar, para o qual näo encontramos semelhante na literatura consultada