No abstract available.
Encephalocele*

No abstract available.
Encephalocele*

“Enlarged parietal foramina” is a congenital malformation with autosomal dominant inheritance. The condition is usually self-limiting and doesn’t require any treatment. However, it may also be associated with encephalocele, vascular anomalies or may be a part of syndrome. We present a case of enlarged parietal foramina in a child and discuss its imaging findings and the associated intracranial vascular malformations.
Encephalocele

Atretic cephalocele is a rare congenital cranial anomaly. We have experienced 3 cases of atretic cephalocele at the Department of Neurosurgery, Kyungpook University Hospital between 1990 and 1993. Clinical features, operative considerations, and outcome are discussed.
Encephalocele* ; Gyeongsangbuk-do ; Neurosurgery

This 24-year-old woman presented to ENT outpatients with an enlarging swelling in the right external auditory canal. A radical mastoidectomy for chronic suppurative otitis media with cholesteatoma had previously been undertaken at another institution. On clinical examination there was an otologic mass that was tender on probing. High resolution imaging of the temporal bones and a subsequent MRI brain confirmed the mass was a temporal lobe encephalocele. A temporal lobe encephalocele is where a segment of the temporal lobe invaginates through a defect in the tegmen tympani. The brain is separated from the middle ear and mastoid process by an exceptionally thin layer of bone – the tegmen tympani. Damage to the tegmen compromises the barrier with the brain and may occur for a number of reasons. This includes congenital, traumatic, post-infectious, malignant invasion, post-radiation therapy and post-surgical causes.1 When this occurs the brain may extrude through the defect resulting in a temporal lobe encephalocele. A bony defect alone, whatever the cause, is insufficient to always result in an encephalocele. Even with dehiscence of the tegmen the dura is capable of supporting the brain issue without herniation. Only when the integrity of the dura is compromised does an encephalocele occur.2 This may be due to the underlying disease process (such as cholesteatoma causing an intracranial abscess) or both purposeful (opening dura to drain an adjacent intracranial abscess) /non-purposeful surgical intervention. Mainstream microsurgical techniques however have lowered the incidence of dural violation.3 Historically, infection was a major cause, but with the ready availability of antibiotics and prompt management, the key contemporary cause is iatrogenic, following mastoid surgery. However, the overall incidence is uncommon following otologic surgery. In a review of 25 years of middle ear/mastoid encephalocele cases 77% were identified to be iatrogenic in origin.4 This patient presented with the finding of a mass observed in the external auditory canal. Less common findings at attendance include tympanic perforation, cholesteatoma, otorrhoea and meningitis.4 The key to diagnosis hinges on cross-sectional imaging: combined imaging with CT to assess the osseous structures and MRI for soft tissue review. The high-resolution CT (HRCT) of the temporal bones illustrates a large defect in the right tegmen tympani with a large soft tissue lesion occupying the post-surgical mastoid cavity abutting the tympanic membrane. (Figures 1A, B) The defect of 15mm in the tegmen was more than double the average of 7.2mm reported elsewhere.4 The MRI confirms the defect in the tegmen with the protrusion of a knuckle of the right temporal lobe and its overlying meninges through the defect into the mastoid cavity. The dumb-bell appearance is typical with the narrower neck at the site of the tegmental dehiscence. The extruded brain occupies the post-operative middle ear cavity. (Figures 2 A, B and C) The defect size and volume of herniated brain can be accurately assessed, both of which may be key determinates of the type of surgical procedure. Revision mastoidectomy with repair of the tegmen defect and dural integrity using a combined intracranial-mastoid approach is planned as a joint case with neurosurgical colleagues.
Encephalocele ; Jaw Abnormalities ; Facial Bones

Median cleft face syndrome is diagnosed by two or more of the following anomalies : ocular hypertelorism, cranium bifidum occultum, median cleft nose, median cleft lip and median cleft premaxilla. The most consistent and prominent ocular finding associated with this syndrome is hypertelorism. We experienced a case of median cleft face syndrome which had orbital hypertelorism, cranium bifidum occultum, bifid cleft nose, V-shaped frontal hairline, and polysyndactly. We report this case with a brief review of the associated literature.
Cleft Lip ; Encephalocele ; Hypertelorism ; Nose ; Orbit

Encephaloceles are known as rare craniofacial developmental anomalies which are herniation of cranial contents through a skull defect. Sphenoidal meningoencephalocele represents an important cause of reVersible visual loss. We report a rare case of pituitary adenoma accompanying sphenoidal meningoencephalocele with clival extension. Although the definitive diagnosis of cystic lesions in the sellar region before surgery is difficult, accurate diagnosis of these lesions is important to determine the type of treatment and predict prognostic outcome.
Craniofacial Abnormalities ; Diagnosis ; Encephalocele ; Pituitary Neoplasms* ; Skull

Encephaloceles are known as rare craniofacial developmental anomalies which are herniation of cranial contents through a skull defect. Sphenoidal meningoencephalocele represents an important cause of reVersible visual loss. We report a rare case of pituitary adenoma accompanying sphenoidal meningoencephalocele with clival extension. Although the definitive diagnosis of cystic lesions in the sellar region before surgery is difficult, accurate diagnosis of these lesions is important to determine the type of treatment and predict prognostic outcome.
Craniofacial Abnormalities ; Diagnosis ; Encephalocele ; Pituitary Neoplasms* ; Skull

Congenital Abnormalities ; Encephalocele ; Humans ; Nose Diseases ; congenital

Chiari type I malformation is a tonsillar herniation more than 3 mm from the level of foramen magnum, with or without concurrent syringomyelia. Different surgical treatments have been developed for syringomyelia secondary to Chiari's malformations: craniovertebral decompression with or without plugging of the obex, syringo-subarachnoid, syringo-peritoneal, and theco-peritoneal shunt placement. Shunt placement procedures are useful for neurologically symptomatic large-sized syrinx. In this paper, authors define the first successful treatment of a patient with syringomyelia due to Chiari type I malformation using a pre-defined new technique of syringo-subarachnoid-peritoneal shunt with T-tube system.
Decompression ; Encephalocele ; Foramen Magnum ; Humans ; Syringomyelia*