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Anterior sacral meningocele and tuberculous spondylitis of the sacrum in a patient with neurofibromatosis. Case report and review of the literature.

We report a case of an adult male with neurofibromatosis and chronic low back pain. Evaluation revealed an anterior sacral meningocele, pulmonary tuberculosis, and later in the course of his illness, an osteolytic tuberculous mass in the sacrum. The patient was treated medically with a good outcome. The nature of anterior sacral meningoceles and tuberculosis spondylitis, the differential diagnoses, and relevant treatment options are discussed.

Adult↗

[Meningitis as a complication of anterior-sacral meningocele].

A 19-year-old young man was admitted with a serious clinical picture of an infected anterior sacral meningocele. Escherichia coli was identified as the pathogenic organism in the spinal putrid fluid. A catheter percutaneously introduced in the hernial sac served as an external drainage of the fluid. At the same time a systemic antibiotic treatment was carried out. After the sanation, the opening of the meningocele was surgically closed in the interval transdurally from the dorsal direction. For this fibrinous adhesive was used in addition to the suture. After six months the hernial sac closure could be demonstrated in the check-up myelo- and computer tomogram.

Adult↗

[Meningocele in the cervical segment of the spine associated with vertebral fusion].

The authors describe a patient aged 22 years in whom a developmental anomaly was found with fusion of the vertebral bodies C4 through C6, posterior spina bifida from C5 through Th1 and meningocele at the level C4 through C7. The meningocele was removed surgically. The case illustrates an integral connection between this set of anomalies and the malformations from the group of dysraphism.

Abnormalities, Multiple↗

Anterior sacral meningocele in pregnancy.

Anterior sacral meningocele is a rare abnormality. Only 14 cases of it have been reported in association with pregnancy. Of those 14 patients, three died as a result of rupture of the meningocele during labor and postpartum. A new case is presented; the clinical presentation, the diagnosis, and treatment are discussed. The obstetric management of the case is discussed and a plan for management is suggested.

Adult↗

Congenital meningocele-encephalocele in an experimental swine herd.

Fourteen instances of meningocele-encephalocele in an experimental herd were recorded over a 5-year period. Thirteen of these affected animals were subjected to extensive pedigree analyses. Familial associations were strong, and clearly the meningocele-encephalocele in this herd was under genetic influence. However, additional studies are needed to clarify the mode of inheritance and, perhaps, to identify other factors that might be involved.

Animals↗

Sacral meningocele with conotruncal heart defects: a possible autosomal recessive trait.

Three of four siblings had sacral meningocele with subsequent development of hydrocephaly; two died during the neonatal period due to conotruncal heart defects (transposition of the great vessels and truncus arteriosus type I, respectively). An in utero diagnosis of open neural tube defect was made on the third sibling; persistent slightly elevated alpha-fetoprotein levels in amniotic fluid and increased number of rapidly adhering cells in short term amniotic cell culture were found. The unique combination of sacral meningocele and conotruncal malformations in this sibship suggests a new autosomal recessive condition. It also emphasizes the heterogeneity of both the open neural tube defects and congenital heart defects. Awareness of this variant is necessary in regard to the 25% recurrence risk instead of the 3% to 5% recurrence risk given for both congenital heart defects and open neural tube defects as isolated anomalies. The difficult prenatal diagnosis for the small neural tube defect should be appreciated.

Female↗

Experimentally induced cranial meningocele and cerebral microgyria.

Teratological study on microgyra is rare, and its morphogenesis is still on dispute. Microgyra associated with cranial meningocele and progressive hydrocephalus were induced in rat siblings by administration of 10 mg/kg.bw of n-methyl n-nitrosourea (MNU) to rat dams on day 9 of gestation. Microgyra were closely similar to human cases. They were formed during progressive stage of hydrocephalus, when the expansion of cranial meningocele was proceeding. This stage was in accord with migratory and postmigratory phase of neuroblasts. The results of this study suggest that the formation of microgyra may be related to the cortical laminar destruction during this critical period of differentiating brain.

Abnormalities, Drug-Induced↗

[A case of intrathoracic meningocele associated with neurofibromatosis].

A rare case of intrathoracic meningocele associated with neurofibromatosis was reported. A 47-year-old female was admitted to our institute because of an abnormal shadow on chest X-ray. The chest X-ray film showed homogenous opacity with a well defined margin in the right apex. Chest CT showed an enlarged intervertebral foramen and defect of the vertebral arch around the tumor. There was no scoliosis. Though preoperative diagnosis was dumbbell type neurinoma, the tumor was found to be a protrusion of dura mater with spinal fluid out of the spinal canal, covered by thickening tumorous tissue. Part of the wall was excised and the residual opening was carefully repaired and wrapped by the pedicled parietal pleura. Retrospectively, MRI showed characteristic findings of intrathoracic meningocele. The tumorous part of it was diagnosed as neurofibroma on histological examination.

Female↗

Bilateral intrathoracic meningoceles.

Bilateral intrathoracic meningoceles are reported in a 22-year-old man with a family history of neurofibromatosis. Clinical evaluation and conventional radiography are inadequate to differentiate intrathoracic meningocele from other posterior mediastinal masses, especially dumbbell neurofibroma. Myelography is the diagnostic procedure of choice.

Diagnosis, Differential↗

Anterior sacral meningocele and Marfan syndrome: a review.

Anterior sacral meningocele is a not rare entity. Treatment is usually surgical and consists in a transacral posterior approach with patching of the communication between the malformation and the dural sac. But surgery can be difficult and require multiple procedures. We report the first case of a giant anterior sacral meeningocele rapidly cured by a simple endoscopic procedure. We believe that more attention should be used in the diagnosis of such cases as they can be so easily cured. We have reviewed the literature about anterior sacral meningocele and particularly its treatment and its association either with Marfan syndrome or a familial occurrence.

Adult↗

Trigonocephaly associated with anterior basal meningocele. Case report.

Pathogenesis of craniosynostosis is poorly understood. Several different factors (primary fusion of a cranial vault suture, abnormal tensile forces acting on the cranial sutures, genetic control, fetal constraint) may be responsible for the malformation. Trigonocephaly is a relatively rare form of cranial malformation ranging from 5 to 20% of all the craniosynostotic syndromes. This form of craniosynostosis may be simple not associated with other cerebral defects or complex, associated with arhinencephaly and hypoplasia of frontal lobes; three gradations of malformation (marked, mild, incomplete) may be considered. A case of trigonocephaly associated with anterior basal meningocele is reported. The operative treatment and radiological findings are described. The pathogenetic role of the basal meningocele to determine the premature sutural fusion through tensile forces transmitted by abnormal dural attachments is evaluated.

Craniosynostoses↗

[Two cases of craniolacunia associated with meningocele and meningoencephalocele (author's transl)-a1].

Craniolacunia (lacunar skull, Luckenschadel) is characterized by multiple, round or oval, radiolucent defects, sharply separated by dense strip of bone (honey comb like configuration) which tend to cluster in the cranial vault on plain skull film. Craniolacunia is present at birth and frequently associated with myelomeningocele, encephalocele or other congenital abnormalities of the central nervous system. Patients with carniolacunia have high mortality due to these associated lesions, and to the secondary effects of these neurological lesions. Recently, it is interested that the presence of carniolacunia can be used as an early indicator of intellectual capacity or recommendation of early indicator of intellectual capacity or recommendation of early surgery for associated lesions. Two cases of craniolacunia with meningocele in the lumbar region and encephalocele in the frontal region are presented and the etiology, clinical significance, prognosis of craniolacunia are discussed. Case 1 (Fig. 1, 2, 3), who had a soft tumor in the lumbar region since birth, was admitted to Saiseikai Yahata Hospital under the diagnosis of meningocele on October 26, 1973. The circumference of the head was 32.5 cm, and the lumbar tumor was infant fist growth, oval, brownish and soft in appearance. The patient had no neurological positive signs or other abnormalities including chest, abdomen and extremities. Plain skull film showed typical craniolacunia in the parietal, frontal and occipital region of the vault. Three days after admission, the patient had opisthotonus like posture at times and convulsive seizure of extremities. Suspected of meningitis, ventricle tap was performed. From the findings of obtained cloud xanthchromic cerebrospinal fluid which was revealed pleocytosis and many Klebsiella or other Gram (-) bacilli on bacterial culture, the diagnosis of ventriculitis was made...

Encephalocele↗

Anterior sacral meningocele and the scimitar sign. Report of a case.

Anterior sacral meningocele is an unusual lesion that usually presents as a presacral mass. This is a case presentation of a young woman who presented with amenorrhea. On physical examination, a large presacral mass was found. Ultrasonography revealed a large cystic structure. Radiography of the pelvis demonstrated a sacral deformity or "scimitar sign" that is pathognomonic for anterior sacral meningocele. The diagnosis was confirmed by computed tomography with myelographic enhancement.

Adult↗

[Presacral tumor with sacral meningocele].

Due to rectal atresia a 10-year-old boy had been operated on using Rehbein's procedure in babyhood in another hospital. Rectal incontinence remained and we planned to improve it by a gracilis plastic operation. During preoperative diagnosis we found a so-called "Currarino triad", because the boy did not only have an anorectal malformation but also an anterior sacral meningocele and a solid presacral tumour. Initially, two surgical interventions were done to remove the tumour and meningocele and three months later a gracilis plastic operation was performed successfully.

Child↗

Transoral repair of a rare basioccipital meningocele in a neonate: case report.

An extremely rare case of a basioccipital meningocele causing obstruction of the upper airway in a neonate is described. A transoral approach was used to remove the meningocele and repair the dural fistula. Bilateral posterior lateral pharyngeal releasing incisions were made to allow mucosal and muscle coverage over the dural repair. This report demonstrates and emphasizes the use of lateral pharyngeal releasing incisions to cover midline full thickness defects in the posterior oropharyngeal wall that might be encountered during transoral transclival operations.

Airway Obstruction↗

Rhomboid perforator flap for a large skin defect due to lumbosacral meningocele: a simple and reliable modification.

To date, very few studies have reported the use of perforator flaps in newborn infants with an immature vascular system. Therefore, it is not clear whether perforator flaps can be used in newborns, as in adults. In this study, we applied the perforator flap procedure to a newborn infant, who had a large skin defect due to lumbosacral meningocele. We used the rhomboid perforator flap, which was a combination of using a rhomboid flap reported by Ohtsuka et al and preserving paraspinal perforator vessels according to Thomas. Although perforator vessels were so thin as to necessitate careful dissection and flap design, a good result was obtained by this procedure. We consider that the rhomboid perforator flap is a simple and reliable procedure for the treatment of lumbosacral meningocele.

Humans↗