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Sequestrated meningocele of the scalp.

A case of occipital sequestrated (rudimentary) meningocele in a 2-year-old girl is presented. The swelling was noticed at birth and did not grow over time. The skull radiograph showed no bone defect and ultrasound and computed tomography examinations demonstrated cystic mass with no connection to dura. The aim of this report is to draw the attention of radiology literature readers to this entity and to elaborate on the role of imaging in the preoperative assessment of such cases. The relation of sequestrated meningocele to the other conditions with ectopic meningeal tissue in the scalp is addressed.

Child, Preschool↗

Posterior sagittal approach for Currarino syndrome with anterior sacral meningocele: a case report.

The authors report a case of Currarino syndrome with anterior sacral meningocele, tethered cord, and anorectal stenosis that was treated by posterior sagittal approach. Initially, a diverting colostomy was performed. Two months later, excision of the meningocele, untethering of spinal cord, and anorectoplasty were performed simultaneously without complication. Posterior sagittal approach seems to be very beneficial for excision of the presacral mass and reconstruction of the anus.

Abnormalities, Multiple↗

Mirror movements associated with cervical meningocele: case report.

A patient with mirror movements associated with cervical meningocele has been presented in this report. The MRI showed normal cerebrum and cervical meningocele, and an anomaly at the posterior to the cervical spinal cord-medulla junction. Unilateral transcranial magnetic stimulation evoked bilateral responses at similar latencies on the thenar muscles which are quite different from those observed in normal subjects. This case adds another etiological cause to the mirror movements.

Adult↗

A shunting procedure employing cannulation of the lumbar theca through the meningocele sac.

An alternative thecoperitoneal shunting procedure is described to counter the problem of cerebrospinal fluid (CSF) fistula following surgery for meningocele and related conditions. The lumbar end of the shunt traverses the meningocele sac. The procedure was effectively employed to deal with postoperative CSF fistula in two cases operated on earlier for lipomeningomyelocele.

Catheterization↗

Intrathoracic meningocele associated with neurofibromatosis: case report.

The authors present a case of intrathoracic meningocele associated with neurofibromatosis. Computed tomography with metrizamide myelography proved valuable in locating the lesion, thus facilitating surgical intervention. With the preoperative diagnosis established and the severity of the meningocele known, the surgeons safely employed a subpleural approach at thoracotomy.

Adult↗

Metrizamide examination of traumatic lumbar nerve root meningocele.

Demonstration of traumatic lumbosacral root avulsion is a rare clinical entity because the bony pelvis offers support and protection to the nerve roots against stretching and rupture. Major vehicular trauma producing unstable pelvic fractures allows lumbosacral root avulsion to occur, and the 18th well-documented case is reported. Delay in diagnosis of several months is common. Demonstration of traumatic meningoceles is important, and metrizamide offers better visualization of the root than does iodophendylate. Absence of the root in the meningocele is equated with avulsion.

Adult↗

Hereditary occurrence of anterior sacral meningocele: report of ten cases.

Anterior sacral meningocele is a rare and often occult disorder that is important to diagnose. It usually occurs sporadically, and familial case reports are rare. This report describes ten documented cases of anterior sacral meningocele in one family. Spanning five generations, it is the largest such series ever reported. In most cases, computerized tomography (CT) was employed to make the diagnosis. There was a high incidence of associated anomalies. The mode of transmission appears to be autosomal dominant with variable penetrance, similar to several other reported series.

Adolescent↗

Intestinal mucosa on top of a rudimentary occipital meningocele in amniotic rupture sequence: disorganization-like syndrome, homeotic transformation, abnormal surface encounter or endoectodermal adhesion?

We present a case of a peculiar rudimentary occipital meningocele that was surgically removed when the patient (a boy) was 5 months of age. The patient also had distal transverse defects of both hands. The association of congenital scalp defects and distal congenital hand anomalies is relatively rare and one form is known as the Adams-Oliver syndrome. To our surprise, microscopic examination revealed intestinal mucosa on top of the skin tag containing the rudimentary meningocele. No comparable cases were found in the literature. We discuss the following possible explanations for this peculiar situation: 1) disorganization-like syndrome; 2) homeotic transformation; 3) abnormal surface encounter between the epidermis and remnants of the yolk sac or omphalo-enteric duct; and 4) endoectodermal adhesion in the presomite embryo.

Abnormalities, Multiple↗

Rhabdomyomatous mesenchymal hamartoma associated with nasofrontal meningocele and dermoid cyst.

A 1-year-6-month-old girl presented with a subcutaneous tumor of the forehead, which had developed since birth. The preoperative examinations showed nasofrontal bone defect with meningocele and subcutaneous tumor with cyst. The patient underwent excision of the tumor and reconstruction of the bone defect. Histologically, the skin tumor exhibited disordered arrangement of striated muscle fibers among normal dermal components, and the cyst was lined by cornified epithelium with a few hair adnexa and contained lamellated keratin. These findings were consistent with rhabdomyomatous mesenchymal hamartoma (RMH) and dermoid cyst. This is an interesting case of RMH co-existing with nasofrontal meningocele and dermoid cyst in the same area. We suggest embryologic errors as a possible etiology, which is incomplete dysjunction of the neural ectoderm from the cutaneous ectoderm. Failure of insertion of mesoderm between the ectoderms caused the bone defect and the disordered proliferation and differentiation of mesoderm-derived tissue, leading to formation of hamartoma.

Dermoid Cyst↗

Rudimentary meningocele: a variant of "primary cutaneous meningioma".

We studied 5 primary cutaneous meningiomas. All were congenital. Four were nodules or plaques on the scalp, and one was a lumbar polyp. Two were alopecic. A skull defect was present deep to one lesion, and the lumbar polyp was attached to dura. The tumors were concentrated in the subcutis, where strands of meningocytes were embedded in dense collageous tissue. Meningocytes wrapped around collagenous fibers, producing "collagen bodies". These formed the nidus for calcification that included psammoma bodies. Meningocytes also dissected between collagenous fibers, creating anastomosing spaces that mimicked a vascular tumor. Meningothelial-lined clefts, several milimeters in length, were present in 4 cases. Two lesions extended through dermal defects into the superficial dermis, where adnexa were reduced or absent. The meningocytes contained vimentin and epithelial membrane antigen. They lacked cytokeratin, S100 protein, and endothelial markers. The meningothelial lesions described herein lack the nodular and sheet-like growth patterns that typify meningiomas of the central nervous system and most primary ectopic meningiomas, including some that develop within the skin. They appear closely related to meningoceles and should be viewed as developmental abnormalities rather than neoplasms. The term "rudimentary meningocele" seems appropriate for these lesions.

Adolescent↗

Unilateral naso-orbital meningocele and bilateral congenital fistulae of the lacrimal passages.

Orbital meningocele is the protrusion of a sac containing cerebrospinal fluid into the orbit, through a defect called cranium bifidum. Although the occipital and frontal basis of the cranial cavity constitute the two most frequent localizations, this pathology may rarely be located in the naso-orbital region. Other developmental anomalies of the eyes may accompany the anomalies of the bony orbit. The case described in the present paper had a right naso-orbital meningocele associated with bilateral fistulae of the lacrimal passages which represents a very rare condition.

Adult↗

Surgical repair of a cranial meningocele in a calf.

A meningocele at the anterior fontanelle was diagnosed in a two-and-a-half month old Meuse-Rhine-Yssel calf. After radiographic and ultrasonographic evaluation, the meningocele was successfully repaired surgically.

Animals↗

Cervical meningoceles and myelocystoceles: a unifying hypothesis.

The classification and embryogenesis of cystic cervical dysraphic lesions are discussed in the light of the authors' experience and review of the literature. It is felt that these lesions are best described as meningoceles or myelocystoceles, and the use of the term 'myelomeningocele' may be more confusing than clarifying. The authors hypothesize that the cervical meningocele and the myelocystocele are part of a spectrum of the same underlying developmental abnormality, namely limited dorsal myeloschisis, with the eventual abnormality depending on the presence or absence of associated hydromyelia.

Cervical Vertebrae↗

Presacral meningocele associated with hereditary sacral agenesis and treated surgically: evaluation in three members of the same family.

OBJECTIVE AND IMPORTANCE: Presacral meningocele in hereditary sacral agenesis is a complex and unusual spinal dysgenetic syndrome. Recognition of the syndromic triad, its natural history, and familial presentation has important practical applications for the management of this disease as well as its complications. CLINICAL PRESENTATION: This report concerns three patients in one family with Currarino syndrome. We detail its clinical presentation, operative management, and outcome and suggest management procedures based on reports in the literature and the results of our surgical techniques, which focus on cases with an "incomplete" triad. INTERVENTION: Three members of the same family, one adult and two children, underwent surgery through the posterior sacral approach tying off the communication between dural sac and anterior meningocele. The adult underwent a second surgical procedure in which a custom-designed surgical technique was used to resolve postoperative cerebrospinal fluid leakage. One of the children underwent an additional posterior sagittal anorectoplasty to remove a presacral teratoma. CONCLUSION: We report a rare occurrence of three familial cases of sacral agenesis accompanied by a presacral mass with various degrees of phenotypic expression and with male dominant transmission. Because of its rarity, the best surgical technique and timing remain an open question especially in cases with incomplete triad syndrome.

Abnormalities, Multiple↗

Retroperitoneal approach for lumbar lateral meningocele--case report.

A 29-year-old female with neurofibromatosis presented with a right lumbar lateral meningocele. Abdominal computed tomography (CT) showed a huge right retroperitoneal cyst expanding anterolaterally and displacing the right kidney. CT following myelography disclosed the cyst expanding through a wide defect of the right pedicles of the T-12 and L-1 vertebrae. The cyst was resected through a retroperitoneal approach with right flank oblique incision. Postoperatively, cerebrospinal fluid leakage occurred, which improved after lumboperitoneal shunt. Careful screening for lateral meningocele, including the lumbar region, should be undertaken in a patient with neurofibromatosis who presents with vertebral anomalies.

Adult↗

Dandy-Walker syndrome associated with occipital meningocele and spinal lipoma--case report.

A neonate presented with Dandy-Walker syndrome associated with occipital meningocele and spinal lipoma, manifesting as soft masses on the skull and lumbosacral regions. Magnetic resonance imaging demonstrated a large posterior fossa cyst between the fourth ventricle and occipital meningocele, but the aqueduct was patent and there was no sign of hydrocephalus. A cyst-peritoneal shunt was emplaced at the age of 8 days followed by partial removal of the spinal lipoma and untethering of the cord at the 3 months. Follow-up examination of age 3 years found almost normal development, although the cyst still persisted.

Adult↗