[Intra-nasal meningoceles and encephalo-meningoceles].
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Rudimentary meningocele: remnant of a neural tube defect?
BACKGROUND: Rudimentary meningocele, a malformation in which meningothelial elements are present in the skin and subcutaneous tissue, has been described in the past under a variety of different terms and has also been referred to as cutaneous meningioma. There has been debate as to whether rudimentary meningocele is an atretic form of meningocele or results from growth of meningeal cells displaced along cutaneous nerves OBJECTIVE: We reviewed the clinical, histological, and immunohistochemical characteristics of rudimentary meningocele in an attempt to assess the most likely pathologic mechanism for it. DESIGN: Retrospective study. SETTING: University hospitals. PATIENTS: Thirteen children with rudimentary meningocele. MAIN OUTCOME MEASURES: Medical records were reviewed and histopathologic examination as well as immunohistochemistry studies were performed for each case. A panel of immunoperoxidase reagents (EMA, CD31, CD34, CD57, S-100, and CAM 5.2) was used to assess lineage and to confirm the meningothelial nature of these lesions. RESULTS: Recent evidence indicating a multisite closure of the neural tube in humans suggests that classic meningocele and rudimentary meningocele are on a continuous spectrum. CONCLUSION: Rudimentary meningocele seems to be a remnant of a neural tube defect in which abnormal attachment of the developing neural tube to skin (comparable to that in classic meningocele) could explain the presence of ectopic meningeal tissue. In the majority of cases, no underlying bony defect or communication to the meninges could be detected. However, in light of the probable pathogenesis, imaging studies to exclude any communication to the central nervous system should precede any invasive evaluation or intervention.
Child↗
Meningoceles and neurological involvement.
The term meningocele is used in this communication to include all lesions presenting as swellings associated with spina bifida, when neural tissue is not exposed on the surface. 144 meningoceles were operated on during an 18-year period. The stalk of the meningocele was connected with the neural tissue in 113 of them. Two distinct types of lesions have been noted in these patients and have been designated "meningocele with tethered cord" (52) and "meningocele with tethered roots" (56). In both conditions about half the number of cases presented clinically before the age of 3 months, when paraplegia was evident in only 5 of the 26 infants with tethered cord and 3 of the 25 with tethered roots. In contrast, all or the majority of babies reporting after the age of one year, had paraplegia. Hence, a case is made out for an early operation in all these patients. At surgery the main lesion was a fatty mass in 29 babies with tethered cord, and a cystic mass in 30 with tethered roots. While hydrocephalus was virtually absent in these babies, there were 5 others who manifested obstructive hydrocephalus with meningocele. "Meningomyelocele", the commonest lesion associated with spina bifida, presents with an exposed spinal cord at birth, is associated with communicating hydrocephalus, and can be clearly distinguished from the meningocele.
Diagnosis, Differential↗
A case with cauda equina syndrome due to bacterial meningitis of anterior sacral meningocele.
STUDY DESIGN: A case of a patient with anterior sacral meningocele that was misdiagnosed as perianal abscess is presented. After the transrectal aspiration, the patient developed meningitis and cauda equina syndrome. OBJECTIVE: To report a rare case of anterior sacral meningocele in which primary presentation was mimicking perianal abscess. SUMMARY AND BACKGROUND DATA: Anterior sacral meningocele is a rare example of spinal dysraphism. It is created by a herniation of a dural sac through a defect in the sacral wall. It is usually asymptomatic until later decades. Most of the presenting symptoms are related to the pelvic organs. In the management of anterior sacral meningocele, surgical treatment is necessary. METHODS: The reported case is that of a 35-year-old female with cauda equina syndrome due to bacterial meningitis of the anterior sacral meningocele. At the beginning, she presented signs and symptoms resembling perianal abscess. She was misdiagnosed as anorectal abscess according to the computed tomography findings. The patient then underwent transrectal aspiration. Following the aspiration, meningitis and cauda equina syndrome were developed. Meningitis was treated with the appropriate antibiotics, and the patient underwent rehabilitation for paraplegia and bladder and bowel incontinence. RESULTS: Following the rehabilitation program, the patient has recovered completely within 8 months. CONCLUSION: This case represents a rare example of anterior sacral meningocele in which the patient was misdiagnosed as perianal abscess. Meningitis either iatrogenic or spontaneous may occur during the course of anterior sacral meningocele. Once it has occurred, it may result in severe morbidity and mortality. However, our case had been treated effectively, and she had regained her health with rehabilitation program.
Abscess↗
[A case of neurofibromatosis with intrathoracic meningocele presenting respiratory failure caused by pleural effusion].
We reported a 58-year-old man with neurofibromatosis and an intrathoracic meningocele. He was admitted to our hospital because of left-sided chest pain and dyspnea on exertion. He presented with severe kyphoscoliosis and showed a round, well circumscribed mass lesion in the paravertebral region of the left upper lung on a chest roentgenogram. Just before admission, pleural effusion accumulated in the left thoracic cavity, which had caused the respiratory symptoms. The mass was diagnosed as an intrathoracic meningocele by MRI and iotrolan CT myelography. The pleural effusion was transudate fluid and no leakage from the meningocele to pleural cavity was demonstrated. Posterolateral extradural approach with laminectomy was done and dural plasty to close the connection between the meningocele and the subarachnoidal space was carried out. After the operation, both the intrathoracic meningocele and the pleural effusion disappeared with remarkable improvement in the respiratory function. Intrathoracic meningocele is known to be seen in association with neurofibromatosis and scoliosis, but it is very rare to see an intrathoracic meningocele which causes respiratory failure due to massive pleural effusion like this report.
Humans↗
Management of occult intrasacral meningocele associated with lumbar disc prolapse.
Occult intrasacral meningocele is an uncommon condition that may produce chronic low back pain, sciatica, and bladder dysfunction. We describe a patient suffering from low back pain and radicular symptoms, in whom multimodality radiological examinations showed an intrasacral meningocele and a lumbar disc prolapse. The protruded disc was considered to be the cause of the clinical symptoms in this patient rather than the meningocele. Surgical removal of the protruded disc was performed without interference with the meningocele. Postoperatively there was recovery of the preoperative deficits. Clinical and radiographic findings in occult intrasacral meningocele are discussed and criteria for differential diagnosis with disc prolapse are stressed.
Humans↗
Thoracic and lumbar meningocele in neurofibromatosis type 1. Report of two cases and review of the literature.
We report two cases of ventrolateral thoracic and lumbar meningoceles associated with neurofibromatosis type 1, and present a review of the literature. Thoracic and lumbar meningoceles are rare lesions which are associated with neurofibromatosis in 60% to 85% of cases. Large meningoceles may cause pain, neurological signs, or respiratory complaints when located intrathoracically. Surgery is indicated in such cases, as well as when small meningoceles enlarge with time. The etiology of meningoceles in neurofibromatosis remains controversial. The authors postulate that ventrolateral lesions of the thoracic and lumbar spine are secondary to congenital mesodermal dysplasia and hypoplastic bone changes. MRI is the diagnostic method of choice, since it most adequately visualizes the pathological anatomy of the spinal cord, meninges, and adjacent structures in every sectional plane. CT scans are particularly useful in assessing the spinal bony changes.
Adult↗
Is meningocele really an isolated lesion?
OBJECT: We designed this study to elucidate the associated occult spinal lesions in patients with simple dorsal meningocele. METHODS: The study population was comprised of two groups. Group I comprised newly diagnosed patients with dorsal spinal meningocele, and group II comprised patients who had had surgery for meningocele and presented with progressive neurological deficits. Magnetic resonance imaging (MRI) scans of the whole spinal column were done. The associated spinal cord malformations were also treated at the same operation. There were 14 boys and 8 girls, with an age range from birth to 4 years (mean 3.9 months), in group I. Of 20 patients (90%) with associated spinal lesions, 6 had more than one lesion, excluding hydromyelia. Group II was made up of 6 patients who had been previously operated on for a meningocele and who presented with tethered cord syndrome. These were 4 boys and 2 girls, who ranged in age from 4 to 10 years (mean 6 years). RESULTS: The level of the conus terminalis was lower than L3 in all patients. The other findings on MRI, besides low conus, were as follows: tight filum, split cord malformation, epidermoid, dorsal lipoma and hydromyelia. CONCLUSIONS: Meningocele frequently camouflages a second, occult, spinal lesion. MRI of the whole spinal column should be performed. An intradural exploration performed with a microneurosurgical technique is needed to detect the fibrous bands that may lead to spinal cord tethering and to release the entrapped nerve roots. The other associated spinal anomalies should be operated on during the same operation.
Child↗
Multiple anterolateral cervical meningoceles associated with neurofibromatosis.
In neurosurgical practice, the term 'spinal meningocele' is generally used to describe a congenital spinal malformation including protrusion of the spinal meninx from the congenitally dysraphic vertebrae. Although non-dysraphic meningocele is very rare, it is usually associated with neurofibromatosis or Marfan's syndrome in the literature. Thoracic and/or lumbar spinal levels are the most common localization. Anterolateral localization of meningocele is very rare in the cervical region. Operative treatment is indicated if the lesion is symptomatic. Detailed radiological assessment of the meningocele is necessary since it may be associated with neuroma in the sac. If the neuroma is found in the meningocele, an anterior surgical approach should be considered for the treatment of both of the lesions.
Adult↗
Paraparesis after excision of intrathoracic meningoceles in a patient with neurofibromatosis.
Intrathoracic meningocele associated with neurofibromatosis is a rarity. We treated a 16-year-old boy with neurofibromatosis, marked kyphoscoliosis, and two right-sided intrathoracic meningoceles. Because his chief complaints of cough and chest pain were thought to be caused by the meningoceles, resection of these lesions was performed prior to correction of the spinal deformity. On the day after the resection, complete paraplegia developed, followed by recovery to paraparesis. Decompressive lumbar puncture was performed, but intraspinal pressure was normal. Postoperative spinal cord damage and consequent paresis may have resulted from a loss of pressure buffering by the meningocele, which rendered the cord vulnerable to injury. The possibility of a similar unusual complication should be borne in mind when treating patients with intrathoracic meningocele associated with neurofibromatosis.
Adolescent↗
Lateral thoracic meningocele.
A case of intrathoracic meningocele is reported, and 95 cases from the literature are reviewed. Thoracic meningoceles are usually associated with neurofibromatosis; nevertheless their etiology remains controversial. The authors postulate the role of spinal trauma in some patients and the origin of the meningocele from an abnormal prolongation of a nerve sleeve in nontraumatic cases. Thoracic meningoceles often are asymptomatic or produce radicular intercostal pain. Spinal computed tomography is the most useful investigation, particularly in the exploration of large meningoceles. Surgery is required in symptomatic cases and usually results in remission of the pain.
Accidents, Traffic↗
Evaluation of endoscopy in the treatment of rare meningoceles: preliminary results.
BACKGROUND: Endoscopy is used on different occasions-for instance, to open the floor of the third ventricule in triventricular hydrocephalus, to open a cyst into the cerebrospinal fluid circulation, for biopsy or for partial resection of some tumors, or to insert a shunt in hydrocephalus or syringohydromyelia. However, the use of endoscopes for evaluating and treating meningoceles remains to be assessed. METHODS: Five different kinds of rare meningoceles are presented. In each, neuroendoscopy was used as the main tool for exploration and treatment. RESULTS: Two sacral meningoceles and one oral cephalocele were cured through a keyhole opening under endoscopic control. One posterior sacral meningocele was explored and no communication with normal subarachnoid spaces was observed, allowing a simple suture of the posterior to the anterior walls. And, last, a complex case with three intrasacral meningocles was explored and partially treated. CONCLUSIONS: Meningocles with very small communication within the normal subarachnoid spaces appeared the most suitable to be cured by an endoscopic procedure. In case of a larger communication, the meningocele could be treated, or at least the morphology can be better understood, by using a keyhole procedure under endoscopic control. In all cases the surgery was of short duration (less than 1 hour) and very well tolerated.
Adolescent↗
Dandy-Walker cyst associated with occipital meningocele.
Dandy-Walker cyst associated with occipital meningocele is very rare. Only 12 patients have been reported. We report a female infant with Dandy-Walker cyst and occipital meningocele whose diagnosis was suspected antenatally by in utero ultrasonography. At birth, head circumference was normal for 37 weeks gestation. She underwent surgical repair of the occipital meningocele immediately after birth. The post-operative course was uneventful until the sixth day of life when progressive enlargement of the head with progressive ventricular dilatation was recognized. Communication between the posterior fossa cyst and the occipital meningocele was confirmed neuroradiologically; the occipital meningocele may have compensated for the increased intracranial pressure in fetal life.
Dandy-Walker Syndrome↗
Intrathoracic meningocele.
We present the case of a 63-year-old woman and review the literature. The incidence of intrathoracic meningocele is rare, even in known cases of Von Recklinghausen's neurofibromatosis. Cases of isolated intrathoracic meningocele or multiple meningoceles without neurofibromatosis are even more uncommon. The majority of intrathoracic meningoceles have been discovered by chance or after nonspecific symptoms. Although diagnosis by CT scan and MRI has been documented, we are convinced that myelography is still of great value. There is no agreement about the etiology of intrathoracic meningocele. Treatment is also discussed.
Ataxia↗
Endoscopic cure of a giant sacral meningocele associated with Marfan's syndrome: case report.
An unusual anterior sacral meningocele associated with Marfan's syndrome, with demonstrated dural anomalies related to Marfan's syndrome is reported. Endoscopy enabled complete exploration of the meningocele, its morphological description, and ultimate cure. The meningocele resembled an hourglass made up of intrasacral and anterior sacral components. The communication of this bilobed malformation with the dural sac was so small that only the intrathecal injection of a colored solution allowed its localization. The closure of this communication was accomplished under endoscopic view by a single stitch. The postoperative course of the patient was characterized by immediate disappearance of the preoperative clinical signs. Computed tomographic scans demonstrated progressive complete collapse of the anterior sacral meningocele and partial resolution of the intrasacral component. Dural anomalies of Marfan's syndrome are described, and their relationship to the formation of the meningocele is discussed.
Adult↗
Dandy-Walker syndrome forming a giant occipital meningocele--case report.
A boy was born with Dandy-Walker syndrome associated with a giant occipital meningocele, cleft lip, and cleft palate. The meningocele was actually a component of the giant posterior fossa cyst which communicated with the fourth ventricle. A cyst-peritoneal shunt achieved a considerable decrease in the size of the meningocele, but decubital ulcers developed due to restricted head movement caused by the occipital lesion. Cranioplasty removed a wide area of the inferior occipital bone, and the boundary between the superior occipital and parietal bones was thinned to allow free bending of the bone flap. The meningocele was removed totally in the third operation, but infection of the wound and pneumonia developed, causing death. The coexistence of Dandy-Walker syndrome and occipital meningocele, together with midline facial anomalies, may suggest a later pathogenesis of Dandy-Walker syndrome than previously believed.
Cerebrospinal Fluid↗
Meningocele with cervical dermoid sinus tract presenting with congenital mirror movement and recurrent meningitis.
Dermoid sinuses and meningoceles are seldom encountered in the cervical region. Besides, to the best of our knowledge, the coexistence of these types of congenital abnormalities with recurrent meningitis, as well as with mirror movement, has never been reported before. A 14-year-old female with the diagnosis of recurrent meningitis was referred to our clinic from the Department of Infectious Diseases. She had a cervical meningocele mass that was leaking cerebro-spinal fluid (CSF) and an associated mirror movement symptom. Spina bifida, dermoid sinus and meningocele lesions were demonstrated at the C2 level on computed tomography (CT) and magnetic resonance imaging (MRI). She underwent an operation to remove the sinus tract together with the sac, and at the same time the tethered cord between the sac base and the distal end of the spinal cord was detached. The diagnosis of dermoid sinus and meningocele was confirmed histopathologically. These kinds of congenital pathologies in the cervical region may also predispose the patient to other diseases or symptoms. Herein, a case of meningocele associated with cervical dermoid sinus tract which presented with recurrent meningitis and a rare manifestation of mirror movement is discussed. Neurosurgeons should consider the possible coexistence of mirror movement and recurrent meningitis in the treatment of these types of congenital abnormalities.
Adolescent↗
Meningoceles and the tethered cord syndrome.
This study shows that there is a relationship between simple meningocele and the tethered cord syndrome. Two groups of patients were examined: the first comprised patients presenting with neurological abnormality at some period after initial closure of a simple meningocele, and the second group (18 patients) had elective myelography after closure of a simple meningocele in the neonatal period (in 17 of the 18 cases). Of these 18 cases. 10 showed an intradural abnormality, and all underwent surgery confirming their myelographic diagnosis. The conclusion is drawn that after closure of a simple meningocele in the neonatal period, patients should have elective myelography in the first 12 months of life to determine whether or not the tethered cord syndrome is likely to develop during the period of growth.
Adolescent↗