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Child's Nervous System : ChNS :... Aug 2020Tourniquet syndrome is a rare condition where a tourniquet applied to an appendage leads to an obstructed blood flow and subsequent ischemic injury. Meningomyelocele and...
Tourniquet syndrome is a rare condition where a tourniquet applied to an appendage leads to an obstructed blood flow and subsequent ischemic injury. Meningomyelocele and meningocele are common birth defects, and involvement of meningocele in tourniquet syndrome is never mentioned in the literature. We managed a 10-day-old male child presenting with infected lumber meningocele with a tourniquet tied at its base. It is being presented with review of relevant literature.
Topics: Child; Humans; Male; Meningocele; Meningomyelocele; Neural Tube Defects; Spinal Dysraphism; Tourniquets
PubMed: 32172393
DOI: 10.1007/s00381-020-04557-y -
Child's Nervous System : ChNS :... Jul 2017The term meningocele manqué (MM) was coined in 1972 to describe a broad range of surgical findings characterized by intradural bands tethering neural structures to the... (Review)
Review
The term meningocele manqué (MM) was coined in 1972 to describe a broad range of surgical findings characterized by intradural bands tethering neural structures to the dorsal dura. Over the following decades, reports continued to lump intradural tethering bands associated with a variety of comorbidities under the umbrella term MM. In more recent years, disorders previously called MM have been identified as embryologically distinct and were reclassified. While this sectioning continues, there remains a set of intradural tethering disorders for which no better term than MM exists. Herein, we comprehensively review the literature surrounding MM, including embryological disorders, clinical, radiographic, and surgical presentation, as well as alternative classification methods and MM treatment.
Topics: Dura Mater; Humans; Magnetic Resonance Imaging; Meningocele; Neural Tube Defects
PubMed: 28620735
DOI: 10.1007/s00381-017-3472-4 -
Child's Nervous System : ChNS :... May 2022Lateral meningocele syndrome (LMS) is a rare genetic connective tissue disorder which is associated with meningocele-related neurologic dysfunction. Several patients... (Review)
Review
PURPOSE
Lateral meningocele syndrome (LMS) is a rare genetic connective tissue disorder which is associated with meningocele-related neurologic dysfunction. Several patients with LMS have been reported. But, guidelines for screening and treatment of LMS have not been established.
METHOD AND RESULTS
We review the current knowledge of LMS in the article. Then, we describe a boy for whom a genomic analysis which allowed us to make a diagnosis of LMS and to begin monitoring of his condition for possible neurological complications.
CONCLUSION
It would be difficult to make a diagnosis of LMS on the basis of clinical manifestations alone. The natural history of dural ectasia in patients with LMS needs to be better defined to establish surgical indications. Based upon the current literature, ventriculoperitoneal shunting (V-Ps) has been recommended as the first-line surgical treatment option for patients with symptomatic thoracolumbar meningoceles.
Topics: Abnormalities, Multiple; Child; Humans; Male; Meningocele; Meningomyelocele; Ventriculoperitoneal Shunt
PubMed: 35128567
DOI: 10.1007/s00381-022-05466-y -
Journal of Spinal Disorders Mar 1992We 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... (Review)
Review
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.
Topics: Ataxia; Back Pain; Female; Humans; Kyphosis; Meningocele; Middle Aged; Neurofibromatosis 1; Thorax
PubMed: 1533325
DOI: 10.1097/00002517-199203000-00017 -
Pediatric Neurosurgery 2020Lateral meningocele syndrome (LMS) is an exceedingly rare connective tissue disease with phenotypic anomalies similar to those seen in Marfan syndrome, Ehler-Danlos... (Review)
Review
BACKGROUND
Lateral meningocele syndrome (LMS) is an exceedingly rare connective tissue disease with phenotypic anomalies similar to those seen in Marfan syndrome, Ehler-Danlos syndrome, and Loeys-Dietz syndrome. However, this syndrome is invariably associated with the presence of multiple lateral thoracolumbar spinal meningoceles: a distinct point of phenotypic divergence from other connective tissue disorders. The etiopathogenesis of this syndrome has recently been linked to truncating mutations within exon 33 of NOTCH3. Despite numerous reports, neurosurgical management of multiple spinal meningoceles remains poorly defined in the literature. We conducted a literature review to provide insight into the nosology, clinical significance, and neurosurgical management strategies of this distinct connective tissue disorder.
SUMMARY
Our literature search revealed 11 articles (16 cases) of LMS, which included 9 males and 7 females, belonging to 14 different families. Half of these cases underwent genetic screening: all of which were discovered to exhibit a truncating mutation within exon 33 of NOTCH3. All patients exhibited multiple lateral thoracolumbar spinal meningoceles with craniofacial dysmorphisms. Other clinical characteristics included pathologic changes in spine morphology, Chiari I malformation, syringomyelia, hydrocephalus, and tethered cord. Operative management of multiple spinal meningoceles in LMS is complicated by the presence of such coexisting structural neurologic pathologies, which may alter cerebrospinal fluid flow dynamics and, ultimately, impact operative intervention. Key Messages: LMS is an exceedingly rare connective tissue disorder with severe spinal dural involvement. Neurosurgical management of multiple spinal meningoceles is complex, which is further complicated by the presence of coexisting neuropathology, such as pathologic transformation of spine morphology and Chiari I malformation. Patients with a connective tissue disorder phenotype found to have multiple spinal meningoceles on imaging studies may benefit from evaluation by a medical geneticist and a pediatric neurosurgeon.
Topics: Abnormalities, Multiple; Adolescent; Child; Child, Preschool; Female; Humans; Infant; Magnetic Resonance Imaging; Male; Meningocele; Neurosurgeons; Receptor, Notch3; Spine
PubMed: 31838470
DOI: 10.1159/000504060 -
Journal of Radiology Case Reports Aug 2020The sphenoid sinus is an uncommon location for protrusion of a meningocele. When this does occur, it nearly always presents with leakage of cerebrospinal fluid through...
The sphenoid sinus is an uncommon location for protrusion of a meningocele. When this does occur, it nearly always presents with leakage of cerebrospinal fluid through the nasal cavity. We present a case of a 38-year-old female found to have a meningocele protruding into the left sphenoid sinus, who presented with intractable headache but no CSF rhinorrhea. The lesion was discovered on computed tomography angiography, which was performed in order to rule out intracranial pathology as the etiology of her headache. Prior imaging, including pre- and post-contrast MRI, demonstrated the fluid within the sphenoid sinus, but did not reveal the communication through a defect in the base of the skull. Thus, it was assumed to be strictly related to sinus disease in the past. Our case represents a phenomenon whereby meningoceles protruding through the basilar skull into the sphenoid sinus or any other location are potentially misdiagnosed due to poor visualization of the osseous defect and lack of awareness of this entity.
Topics: Adult; Computed Tomography Angiography; Female; Headache; Humans; Magnetic Resonance Imaging; Meningocele; Nausea; Photophobia; Sphenoid Sinus; Vision Disorders; Vomiting
PubMed: 33088417
DOI: 10.3941/jrcr.v14i8.3761 -
Repair of a Transclival Meningocele Through a Transoral Approach: Case Report and Literature Review.World Neurosurgery Mar 2019Transclival meningoceles and related primary spontaneous cerebrospinal fluid leaks at the clivus are extremely rare lesions, with only a few cases reported in the... (Review)
Review
BACKGROUND
Transclival meningoceles and related primary spontaneous cerebrospinal fluid leaks at the clivus are extremely rare lesions, with only a few cases reported in the literature.
CASE DESCRIPTION
An infant presented with nasal airway obstruction and meningitis secondary to cerebrospinal fluid leak from a transclival meningocele. The radiologic investigation and surgical repair of the lesion are reported, along with intraoperative findings. Basic anatomy, embryology, and development of the clivus are reviewed to clarify the relationship of anatomic variants with the formation of transclival meningoceles.
CONCLUSIONS
Transclival meningocele should be considered in patients with spontaneous cerebrospinal fluid rhinorrhea. The existing classification of meningoencephaloceles based on the location of the defect in the cranium should probably be reconsidered, taking into consideration the described entity.
Topics: Cranial Fossa, Posterior; Encephalocele; Female; Humans; Imaging, Three-Dimensional; Infant; Magnetic Resonance Imaging; Meningocele; Nasal Obstruction; Tomography Scanners, X-Ray Computed
PubMed: 30579031
DOI: 10.1016/j.wneu.2018.12.025 -
AJNR. American Journal of Neuroradiology 1981
Topics: Cranial Fossa, Posterior; Humans; Infant; Male; Meningitis; Meningocele; Occipital Bone; Radiography
PubMed: 6784543
DOI: No ID Found -
The Turkish Journal of Pediatrics 2000The coexistence of two distinct meningoceles of the spine is a very unusual event. We report a three-day-old boy with double meningoceles at the thoracic and lumbar... (Review)
Review
The coexistence of two distinct meningoceles of the spine is a very unusual event. We report a three-day-old boy with double meningoceles at the thoracic and lumbar levels. The connection between the stalk of the thoracic meningocele and the spinal cord, as seen on magnetic resonance imaging, showed a neurological involvement in this lesion. Our case is only the third without association of congenital anomalies or neurofibromatosis to be reported to date.
Topics: Humans; Infant, Newborn; Laminectomy; Lumbar Vertebrae; Magnetic Resonance Imaging; Male; Meningocele; Thoracic Vertebrae
PubMed: 11196754
DOI: No ID Found -
American Journal of Medical Genetics Jun 1997One female and two male patients with multiple lateral meningoceles are presented. They do not have neurofibromatosis or Marfan syndrome and share findings with the two... (Review)
Review
One female and two male patients with multiple lateral meningoceles are presented. They do not have neurofibromatosis or Marfan syndrome and share findings with the two previously described patients with multiple lateral meningoceles. The original report by Lehman et al. [1977: J Pediatr 90:49-54] was titled "familial osteosclerosis," because osteosclerosis was present in the proposita and her mother; the patient described by Philip et al. [1995: Clin Dysmorphol 4:347-351] also had increased bone density of the skull base and the sutures. Thickened calvaria were present in one of our patients; two had a prominent metopic suture. Other shared findings include multiple lateral meningoceles, Wormian bones, malar hypoplasia, downslanted palpebral fissures, a high narrow palate, and cryptorchidism in males. In addition, our patients showed ligamentous laxity, keloid formation, hypotonia, and developmental delay. A short umbilical cord was noted in two patients. One had a hypoplastic posterior arch of the atlas and an enlarged sella, as reported by Lehman et al. [1977]. Our patients appear to have the same syndrome as previously reported. We suggest it be called "lateral meningocele syndrome," because of this unique finding.
Topics: Abnormalities, Multiple; Bone and Bones; Child; Child, Preschool; Facies; Female; Humans; Infant; Infant, Newborn; Magnetic Resonance Imaging; Male; Meningocele; Radiography; Syndrome
PubMed: 9188658
DOI: No ID Found