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Cerebrospinal fluid otorrhea at myringotomy. A meningocele through a defect in the tegmen.

Spontaneous cerebrospinal fistulae can simulate secretory otitis media or be discovered at myringotomy, but the diagnosis is frequently made after one or more episodes of meningitis. Congenital perilabyrinthine fistulae are extremely rare, and unlike translabyrinthine fistulae there is usually no sensorineural hearing loss. This is the first reported case of a congenital meningocele through the tegmen in a child. An 11-year-old boy presented with signs and symptoms which mimicked serous otitis media. A cerebrospinal fistula was produced at myringotomy and this was closed surgically via an endaural approach. A CT scan defined a defect in the tegmen tympani anterior to the superior semicircular canal.

Cerebrospinal Fluid Otorrhea↗

Congenital transsphenoidal meningocele: case report and review of the literature.

Intrasphenoidal meningoencephalocele is a rare clinical entity. Its origin can be congenital, traumatic, tumoral, or spontaneous. Presenting as cerebrospinal fluid fistula with rhinorrhea, the diagnosis and treatment of this uncommon disease is a real challenge for the otorhinolaryngologist. We report a case of sphenoidal meningocele treated using an endoscopic procedure and review the literature regarding its congenital origin.

Aged↗

Meningocele and ankyloblepharon following in utero exposure to olanzapine.

Although atypical antipsychotics are widely used during pregnancy, their safety is not well established. This case highlights the possible teratogenic effect of olanzapine, in which the baby was born with meningocele and ankyloblepharon. It is suggested that olanzapine may interfere with embryonic development at different stages of pregnancy.

Adult↗

Segmental spinal cord hypoplasia and meningocele with preservation of medullary function: case report.

BACKGROUND: In very rare dysraphic cases, it is not clear whether the primary abnormalities are in the neural elements, or in the bony elements. CASE DESCRIPTION: We describe a case of segmental hypoplasia of the spinal cord, with absent nerve roots in the afflicted segments, and associated meningocele and vertebral abnormalities. We illustrate the arguments for and against the classification of this lesion either as an atypical case of myelomeningocele (MMC) or as a mild case of segmental spinal dysgenesis (SSD). CONCLUSIONS: Possibly, in this exceptional case, the primary defect is in the neural tissue like in more usual cases of MMC and not in the spine, like in segmental spinal dysgenesis.

Child↗

Polymicrobial meningitis revealing an anterior sacral meningocele in a 23-year-old woman.

Polymicrobial meningitis has become increasingly rare during recent decades. Historically, it has mainly been reported as being associated with disorders of the ENT-sphere. The treatment of these infections being optimized, polymicrobial meningitis nowadays is essentially a complication of gastrointestinal or gynaecological disorders and trauma. We present a case of polymicrobial meningitis following puncture of a unrecognized pre-sacral meningocele in a patient with Currarino syndrome and review of the relevant literature.

Adult↗

The clinical spectrum of rudimentary meningocele.

Rudimentary meningocele is rare, only 50 cases having been reported. Clinically it may simulate dermoid cyst, lipoma, or nevus sebaceous. We present two cases of this uncommon condition, each with its own unique clinical features.

Child↗

The occult intrasacral meningocele.

Three cases of occult intrasacral meningocele, one of them with an intrasacral and presacral neurofibroma, are reported. The literature is briefly reviewed. Pathogenesis, clinical picture, radiological findings and surgical treatment are discussed.

Bone Neoplasms↗

MR imaging of anterior sacral meningocele.

The magnetic resonance appearance of a case of an anterior sacral meningocele is described and the particular advantages of this technique, compared with other radiological studies, are considered.

Adult↗

Multiple lateral meningoceles, distinctive facies and skeletal anomalies: a new case of Lehman syndrome.

We describe a 19-year-old boy who presented with facial dysmorphism, multiple lateral meningoceles, skeletal abnormalities and normal intelligence. Neurofibromatosis and Marfan syndrome were excluded. Electron microscopy of the skin showed non-specific abnormalities suggesting a connective tissue disorder. The features of this boy closely resemble those in a mother and daughter with Lehman syndrome.

Abnormalities, Multiple↗

Rudimentary meningocele of the scalp.

A rudimentary meningocele, a variant of primary cutaneous meningioma, was seen on the scalp of a 9-month-old Japanese boy. Clinically, the lesion on the left parietal area was round, about 1.6 cm in diameter, alopecic, and slightly elevated. Histologically, the lesion, located from the dermis to the subcutis, consisted of scattered foci of meningothelial cells, an anastomosing network of empty spaces with psammoma bodies and collagen bodies, and small vessels. Immunohistochemically, the meningothelial cells were positive for vimentin and desmin. Ultrastructurally, they had elongated cytoplasmic processes, intermediate filaments in the cytoplasm, and desmosomal junctions.

Humans↗

Anterior sacral meningocele as a pelvic complication of Marfan syndrome.

Anterior sacral meningocele (ASM) is well recognized in Marfan syndrome as a consequence of dural ectasia. Two cases presenting as nongynaecological pelvic masses are described highlighting the clinical difficulty in diagnosis and the classical radiological findings. The classification of ASM and associated anomalies of the sacrum are reviewed.

Adult↗

Meningoceles, meningomyeloceles, and encephaloceles: a neuro-dermatopathologic study of 132 cases.

Because there have been few comprehensive histopathologic studies of meningomyeloceles and related malformations, we undertook a systematic study of these lesions. One hundred and thirty two cases were obtained from our surgical pathology files; these included 38 meningoceles, 71 meningomyeloceles, and 23 encephaloceles. Tissue sections were stained with hematoxylin and eosin; special stains included trichrome, alcian blue, Fontana-Masson, Nissl, Holzer, and immunoperoxidase for glial fibrillary acidic protein. Epithelial changes included ulceration, atrophy, or nevoid hyperplasia of the epidermis, and loss of appendages. Mesodermal features included fibrous zones resembling dura, subarachnoid tissue or scar (99% of cases), increased numbers of blood vessels (83%), hypertrophy of arrector pili muscle (42%), lipoma formation (38%), and immature skeletal muscle fibers (5%) that rarely intermingled with neuropil-like matrix. The latter tissue was identified in 71% of cases and included neurons, astrocytes, oligodendroglia, and ependyma. Forty-eight percent of cases included peripheral nerve fibers or roots, and some fibers formed onion bulb or Pacinian corpuscle-like structures. Meningothelial cells were observed in 26% of cases and sometimes formed recognizable whorls. Choroid plexus was noted in 3 cases, one example showing an unusual dystrophic calcification that formed long parallel spicules. Pigmented dendritic cells were observed within zones of fibrous tissue in 10% of cases. These malformations involve complex arrangements of cutaneous, neuroectodermal, and mesodermal elements. Because they may be encountered by dermatopathologists, familiarity with the microscopic features of dysraphic lesions is essential.

Encephalocele↗

Lateral meningocele and defect of abdominal wall.

A case of lateral meningocele associated with deficiency of the thoraco-abdominal wall is reported. It is suggested that both of these defects are due to interference with the development of the paraxial mesoderm by a single injury.

Abdominal Muscles↗

Congenital arachnoid cyst mimicking meningocele.

The subject of this report is a rare case of a 5-year-old girl who developed an arachnoid cyst with a bony and dural defect in the parietal convexity. She had no history of head trauma or infection. Surgical exploration revealed the bulging lesion to consist of cerebrospinal fluid-containing spongy subcutaneous tissue and to extend into the bony and dural defect. The arachnoid cyst cavity was found beneath the subcutaneous lesion and was not connected to the adjacent subarachnoid space. Histologically, the subcutaneous tissue contained a complex of sinusoidal channels formed by an abundance of migrating arachnoidal cells, thus mimicking meningocele.

Arachnoid Cysts↗

Orbital meningocele presenting as periorbital cellulitis.

A case of orbital meningocele in a 15-day-old infant presenting as periorbital cellulitis, followed by meningitis, is described. Unlike other cases with the same anomaly, signs were not noted in the involved eye, neither before the cellulitis nor after recovery following antibiotic treatment. Preoperative, diagnosis was confirmed by tomography of the orbit. On operation a bony defect was found in the orbital roof, which was repaired. Recovery was uneventful.

Cellulitis↗