-
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 -
Laryngo- Rhino- Otologie Sep 2017
Topics: Aged; Diagnosis, Differential; Encephalocele; Female; Humans; Image Interpretation, Computer-Assisted; Magnetic Resonance Imaging; Meningocele; Petrous Bone; Subarachnoid Space; Tomography, Spiral Computed; Tomography, X-Ray Computed; Trigeminal Neuralgia
PubMed: 28881371
DOI: 10.1055/s-0043-112163 -
The Journal of Pediatrics Jan 2016
Topics: Encephalocele; Female; Humans; Infant, Newborn; Meningocele
PubMed: 26601906
DOI: 10.1016/j.jpeds.2015.09.081 -
PloS One 2022Notch receptors are determinants of cell fate and function, and play an important role in the regulation of bone development and skeletal remodeling. Lateral Meningocele...
Notch receptors are determinants of cell fate and function, and play an important role in the regulation of bone development and skeletal remodeling. Lateral Meningocele Syndrome (LMS) is a monogenic disorder associated with NOTCH3 pathogenic variants that result in the stabilization of NOTCH3 and a gain-of-function. LMS presents with neurological developmental abnormalities and bone loss. We created a mouse model (Notch3em1Ecan) harboring a 6691TAATGA mutation in the Notch3 locus, and heterozygous Notch3em1Ecan mice exhibit cancellous and cortical bone osteopenia. In the present work, we explored whether Notch3 antisense oligonucleotides (ASO) downregulate Notch3 and have the potential to ameliorate the osteopenia of Notch3em1Ecan mice. Notch3 ASOs decreased the expression of Notch3 wild type and Notch36691-TAATGA mutant mRNA expressed by Notch3em1Ecan mice in osteoblast cultures without evidence of cellular toxicity. The effect was specific since ASOs did not downregulate Notch1, Notch2 or Notch4. The expression of Notch3 wild type and Notch36691-TAATGA mutant transcripts also was decreased in bone marrow stromal cells and osteocytes following exposure to Notch3 ASOs. In vivo, the subcutaneous administration of Notch3 ASOs at 25 to 50 mg/Kg decreased Notch3 mRNA in the liver, heart and bone. Microcomputed tomography demonstrated that the administration of Notch3 ASOs ameliorates the cortical osteopenia of Notch3em1Ecan mice, and ASOs decreased femoral cortical porosity and increased cortical thickness and bone volume. However, the administration of Notch3 ASOs did not ameliorate the cancellous bone osteopenia of Notchem1Ecan mice. In conclusion, Notch3 ASOs downregulate Notch3 expression in skeletal cells and their systemic administration ameliorates cortical osteopenia in Notch3em1Ecan mice; as such ASOs may become useful strategies in the management of skeletal diseases affected by Notch gain-of-function.
Topics: Abnormalities, Multiple; Animals; Bone Diseases, Metabolic; Bone and Bones; Meningocele; Mice; Oligonucleotides, Antisense; RNA, Messenger; Receptor, Notch2; Receptor, Notch3; Receptors, Notch; X-Ray Microtomography
PubMed: 35536858
DOI: 10.1371/journal.pone.0268225 -
Practical Neurology Oct 2018
PubMed: 29777013
DOI: 10.1136/practneurol-2017-001769 -
Seminars in Ultrasound, CT, and MR Dec 2014Ultrasound of the spine in the neonate is widely used as the initial modality to evaluate spinal canal anatomy, anatomical variants, and congenital malformations. The... (Review)
Review
Ultrasound of the spine in the neonate is widely used as the initial modality to evaluate spinal canal anatomy, anatomical variants, and congenital malformations. The spinal canal and its contents are best visualized in the newborn and young infant owing to incomplete ossification of the posterior vertebral elements.
Topics: Humans; Infant, Newborn; Lumbosacral Region; Meningocele; Neural Tube Defects; Sacrococcygeal Region; Spinal Canal; Spine; Teratoma; Ultrasonography
PubMed: 25454057
DOI: 10.1053/j.sult.2014.08.001 -
Journal of Personalized Medicine Apr 2024Spontaneous orbital cephaloceles are a rare condition. The purpose of this study is to provide a description of a clinical case and to carry out a systematic literature... (Review)
Review
BACKGROUND
Spontaneous orbital cephaloceles are a rare condition. The purpose of this study is to provide a description of a clinical case and to carry out a systematic literature review.
METHODS
A systematic review of the English literature published on the Pubmed, Scopus, and Web of Science databases was conducted, according to the PRISMA recommendations.
RESULTS
A 6-year-old patient was admitted for right otomastoiditis and thrombosis of the sigmoid and transverse sinuses, as well as the proximal portion of the internal jugular vein. Radiological examinations revealed a left orbital mass (22 × 14 mm) compatible with asymptomatic orbital meningocele (MC) herniated from the superior orbital fissure (SOF). The child underwent a right mastoidectomy. After the development of symptoms and signs of intracranial hypertension (ICH), endovascular thrombectomy and transverse sinus stenting were performed, with improvement of the clinical conditions and reduction of the orbital MC. The systematic literature review encompassed 29 publications on 43 patients with spontaneous orbital MC. In the majority of cases, surgery was the preferred treatment.
CONCLUSIONS
The present case report and systematic review highlight the importance of ICH investigation and a pathophysiological-oriented treatment approach. The experiences described in the literature are limited, making the collection of additional data paramount.
PubMed: 38793047
DOI: 10.3390/jpm14050465 -
NeoReviews Dec 2019Spinal dysraphism, which includes conditions such as myelomeningocele and sacral agenesis, is one of the most common causes of congenital lower urinary tract... (Review)
Review
Spinal dysraphism, which includes conditions such as myelomeningocele and sacral agenesis, is one of the most common causes of congenital lower urinary tract dysfunction. Early evaluation of the neurogenic bladder serves to minimize renal damage, and the main goals of management include preserving renal function, achieving acceptable continence, and optimizing quality of life. The survival of patients with such conditions has improved to greater than 80% reaching adulthood, owing to advances in diagnostic and therapeutic modalities. The result is a real, and unfortunately often unmet, need for successful transitional care in this complex patient population. Clinicians must be able to identify the unique challenges encountered by patients with neurogenic bladder as they shift through different stages of their life.
Topics: Abnormalities, Multiple; Acetylcholine Release Inhibitors; Botulinum Toxins, Type A; Cholinergic Antagonists; Fetal Therapies; Humans; Hydronephrosis; Meningocele; Meningomyelocele; Sacrococcygeal Region; Spina Bifida Occulta; Spinal Dysraphism; Transition to Adult Care; Ultrasonography, Prenatal; Urinary Bladder; Urinary Bladder, Neurogenic; Urinary Catheterization; Urinary Tract Infections; Urodynamics; Urology; Vesico-Ureteral Reflux; Watchful Waiting
PubMed: 31792158
DOI: 10.1542/neo.20-12-e711 -
Acta Neurochirurgica Oct 2021Symptomatic midline sacral meningeal cysts (MSMC) are rare, and, as a consequence, so are reports on the surgical techniques to address these lesions. Here we provide a...
BACKGROUND
Symptomatic midline sacral meningeal cysts (MSMC) are rare, and, as a consequence, so are reports on the surgical techniques to address these lesions. Here we provide a description of the senior author's (ATC) technique.
METHOD
A sacral laminectomy is performed. The cyst's relation with the dural sac and sacral nerves is inspected; it is then opened and drained. Its lumen is explored for its point of communication with the dural sac, and this ostium is closed off with non-penetrating clips. A lumbar drain is inserted in select cases.
CONCLUSION
Cyst wall resection is unnecessary and closing the ostium is sufficient to treat MSMC.
Topics: Central Nervous System Cysts; Cysts; Decompression; Humans; Laminectomy; Magnetic Resonance Imaging; Meningocele; Sacrum
PubMed: 34417877
DOI: 10.1007/s00701-021-04948-3 -
The Pan African Medical Journal 2022
Topics: Humans; Spinal Dysraphism; Meningomyelocele
PubMed: 36338552
DOI: 10.11604/pamj.2022.42.258.35894