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Child's Nervous System : ChNS :... Mar 2022Lateral meningocele syndrome is characterized by multiple lateral meningoceles with a distinctive craniofacial appearance, hyperextensibility of the skin, and...
Lateral meningocele syndrome is characterized by multiple lateral meningoceles with a distinctive craniofacial appearance, hyperextensibility of the skin, and hypermobility of the joints. The syndrome is caused by heterozygous truncating variants in the last exon, exon 33, of the NOTCH3 gene. Here, we present a 2-year-old girl for whom an early genomic analysis allowed us to recognize the presence of lateral meningoceles and to begin early monitoring of her condition for possible neurological complications. She had a characteristic facial appearance, hyperextensibility of the skin and mobility of the joints, and developmental delays. Given that lateral meningocele syndrome is a rare syndrome, the existence of lateral meningoceles is suspected only when the causative gene is detected by genetic testing. MRI scans are unlikely to be performed in infancy in the absence of neurological symptoms suggestive of meningoceles. No formal guidelines have been established for the neurosurgical indications for lateral meningocele syndrome. Given the features of hyperextensibility of the skin and hypermobility of the joints, lateral meningocele syndrome can be categorized as a connective tissue disease and may be progressive, as with the dural ectasia in Marfan syndrome and Loeys-Dietz syndrome. Watchful monitoring of dural ectasia may be warranted in patients with lateral meningocele syndrome.
Topics: Abnormalities, Multiple; Child, Preschool; Early Diagnosis; Female; Genomics; Humans; Infant; Meningocele
PubMed: 34121137
DOI: 10.1007/s00381-021-05232-6 -
Neuro-Chirurgie 1988The authors report 11 cases of anterior sacral meningocele, cystic mass connected with lumbar sac through a sacral bony defect: 2 children and 9 adults. Clinic... (Review)
Review
The authors report 11 cases of anterior sacral meningocele, cystic mass connected with lumbar sac through a sacral bony defect: 2 children and 9 adults. Clinic presentations are analysed. Examination including, X ray, ultrasonography, scanner is discussed. The authors believe that these lesions must be treated surgically because a risk of rupture in the rectum with important meningitis exists. There is also a septic risk if an error of diagnosis ends at a puncture. The transacral approach is easy and it gives the best results.
Topics: Adult; Aged; Female; Humans; Lumbosacral Region; Male; Meningocele; Middle Aged; Sacrum
PubMed: 3043248
DOI: No ID Found -
Journal of Spinal Disorders & Techniques Oct 2006Simple meningoceles are infrequent forms of dysraphism and are often benign. These lesions are usually associated with other congenital spinal anomalies, and are... (Review)
Review
Simple meningoceles are infrequent forms of dysraphism and are often benign. These lesions are usually associated with other congenital spinal anomalies, and are typically diagnosed in childhood. Most become symptomatic in childhood because of progressive spinal cord or nerve root tethering. This article describes the case of a 47-year-old man who presented with a sac in his posterior cervical region that had been leaking colorless fluid for 3 months. He was also having difficulty walking and complained of stiffness in his lower extremities. Cervical magnetic resonance imaging revealed a cystic mass in the region of the C4 to C5 laminae. Partial laminectomies were performed (lower portion C4 lamina, upper portion C5 lamina), the sac was totally excised, and tissue tethering the spinal cord were cut. There was no recurrence of symptoms in 12 months of follow-up. Although in cases where a cervical meningocele-myelomeningocele is detected at any time, early treatment is essential to eliminate the high risk of future neurological impairment. This is the first report of an untreated cervical meningocele manifesting symptoms in adulthood.
Topics: Cervical Vertebrae; Gait Disorders, Neurologic; Humans; Male; Meningocele; Middle Aged
PubMed: 17021419
DOI: 10.1097/01.bsd.0000211223.51436.0b -
Journal of Neurosurgical Anesthesiology Jan 2022
Topics: Drainage; Humans; Infant; Meningocele
PubMed: 32868519
DOI: 10.1097/ANA.0000000000000724 -
Arquivos de Neuro-psiquiatria Sep 2001Intrasacral meningoceles are rare conditions that can cause symptoms of nerve root compression. We report a case of a 18-year-old female, who presented with urinary... (Review)
Review
Intrasacral meningoceles are rare conditions that can cause symptoms of nerve root compression. We report a case of a 18-year-old female, who presented with urinary incontinence. The neurological and urological evaluation revealed lack of detrusor muscle reflex. This finding supported the hypothesis of spinal disraphism. Magnetic resonance image revealed an intrasacral meningocele. The patient was submitted to surgical excision of the lesion without postoperative complications. In the present paper, the pathogenesis, clinical picture, neuroradiological findings and surgical treatment are discussed.
Topics: Adolescent; Female; Humans; Magnetic Resonance Imaging; Meningocele; Radiography; Sacrum
PubMed: 11593292
DOI: No ID Found -
Neurology India 2022
Topics: Child; Humans; Meningocele; Developmental Disabilities; Sacrum; Spinal Diseases; Meningomyelocele; Magnetic Resonance Imaging
PubMed: 36352675
DOI: 10.4103/0028-3886.359169 -
A&A Practice May 2021A 6-year-old male undergoing bilateral hydrocelectomy was to receive caudal analgesia after induction of general anesthesia. After insertion of the caudal needle,...
A 6-year-old male undergoing bilateral hydrocelectomy was to receive caudal analgesia after induction of general anesthesia. After insertion of the caudal needle, cerebrospinal fluid was unexpectedly aspirated and the caudal was abandoned. The surgeon performed bilateral ilioinguinal nerve blocks just before incision. Surgery was uneventful. The patient had difficulty ambulating postoperatively, and a detailed neurologic examination revealed quadriceps weakness. A lumbosacral magnetic resonance imaging (MRI) revealed a sacral meningocele. By the next morning, quadriceps function had returned, and he was ambulating normally. The ilioinguinal block was most likely deep to the internal oblique muscle and produced femoral nerve dysfunction.
Topics: Analgesia; Child; Humans; Magnetic Resonance Imaging; Male; Meningocele; Pain Management
PubMed: 33973901
DOI: 10.1213/XAA.0000000000001457 -
Journal of Neurosurgery. Pediatrics Jun 2013Intrasacral meningoceles are rare cystic lesions that can cause focal compression within the bony sacral canal. Their mechanisms are poorly understood, but most...
OBJECT
Intrasacral meningoceles are rare cystic lesions that can cause focal compression within the bony sacral canal. Their mechanisms are poorly understood, but most intrasacral meningoceles appear to be intrasacral extradural cysts caused by arachnoid herniating through a small dural defect in the caudal end of the thecal sac. As opposed to perineural cysts, they are not associated with an exiting nerve root. When symptomatic, they can cause sacral pain or sacral nerve root dysfunction due to local compression.
METHODS
This is a retrospective series from Boston Children's Hospital. All patients in whom symptomatic intrasacral meningocele that required surgical treatment was diagnosed between May 1994 and March 2011 were included in the study. Spine MRI was the diagnostic modality of choice. All patients underwent sacral exploration, with ligation and obliteration of the cyst. Resected cyst wall was subjected to pathological examination.
RESULTS
There were 13 patients (11 boys and 2 girls) who underwent operation for intrasacral meningocele. The median age was 8 years (range 5 months-16 years). The most common presenting symptom was back pain (in 5) often described as deep tail bone pain, followed by urinary incontinence (3) and constipation (2). Three patients had evidence of associated tethered cord on MRI studies. Four patients were asymptomatic and their diagnosis was made following imaging for other reasons; they were surgically treated because of the increasing size of the lesion or association with other congenital lesions. Most patients had symptomatic improvement after surgery.
CONCLUSIONS
Intrasacral meningoceles are rare lesions that may result from a congenital dural weakness and a resultant arachnoid diverticulum. They present in childhood either incidentally or with symptoms secondary to nerve root compression. Identification of the point of herniation through the dura mater and ligation of the lesion provides cyst cure and resolution of symptoms in most patients.
Topics: Adolescent; Arachnoid; Back Pain; Boston; Child; Child, Preschool; Constipation; Female; Humans; Imaging, Three-Dimensional; Incidental Findings; Infant; Magnetic Resonance Imaging; Male; Meningocele; Myelography; Neural Tube Defects; Neurosurgical Procedures; Retrospective Studies; Sacrum; Spina Bifida Occulta; Tarlov Cysts; Tomography, X-Ray Computed; Treatment Outcome; Urinary Incontinence
PubMed: 23601014
DOI: 10.3171/2013.3.PEDS12519 -
Pediatric Neurosurgery 2009Atretic meningoceles are infrequent congenital malformations resulting from the herniation of meninges through a skull defect. All prior reported cases have had a...
Atretic meningoceles are infrequent congenital malformations resulting from the herniation of meninges through a skull defect. All prior reported cases have had a subcutaneous component with or without an underlying osseous defect. We report the unusual case of a completely intraosseous atretic meningocele.
Topics: Bone Marrow; Child; Female; Humans; Meningocele; Skull
PubMed: 19690448
DOI: 10.1159/000235606 -
Child's Nervous System : ChNS :... Jun 2014Anterior sacral meningoceles are lesions that are uncommonly reported and can be associated with other pathology including presacral masses, tethered spinal cord, and... (Review)
Review
BACKGROUND AND IMPORTANCE
Anterior sacral meningoceles are lesions that are uncommonly reported and can be associated with other pathology including presacral masses, tethered spinal cord, and syringomyelia. Tethered spinal cord and syringomyelia can result in neurologic deficits, while large meningoceles and presacral masses can have gastroenterologic, urologic, reproductive, and oncologic consequences.
CLINICAL PRESENTATION
The authors report a case of a 14-year-old girl with an anterior sacral meningocele, tailgut cyst, and tethered cord with holocord syringomyelia who presented with a tethered cord syndrome, manifested by constipation, urinary retention, bilateral lower extremity weakness, and sensory deficits. After extensive radiographic and urodynamic workups were performed, the patient was treated by the neurosurgery and pediatric surgery teams with a posterior sagittal approach for cord detethering, resection of an intradural cystic mass, resection of the anterior sacral meningocele, and resection of the adjacent presacral mass. After surgical treatment, motor weakness and sensory deficits were resolved, though urinary symptoms persisted. The syrinx resolved after detethering alone. Pathology of the intradural cystic mass and the presacral mass inferior to the anterior sacral meningocele were consistent with tailgut cyst.
CONCLUSION
The patient's clinical and surgical management are discussed, and a literature review related to anterior sacral meningoceles and their related pathologies is presented. An interdisciplinary approach is required for the best treatment of this constellation of findings.
Topics: Adolescent; Female; Follow-Up Studies; Hamartoma Syndrome, Multiple; Humans; Magnetic Resonance Imaging; Meningocele; Neural Tube Defects; Neurosurgical Procedures; Syringomyelia
PubMed: 24562417
DOI: 10.1007/s00381-014-2379-6