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European Annals of Otorhinolaryngology,... Jun 2016Nasal obstruction is a very frequent symptom in children, with numerous etiologies. Clinical diagnosis is straightforward, but general impact and rare etiologies should... (Review)
Review
Nasal obstruction is a very frequent symptom in children, with numerous etiologies. Clinical diagnosis is straightforward, but general impact and rare etiologies should be explored for. Complementary examinations are guided by diagnostic orientation. Although not usually a severe condition, nasal obstruction may be life-threatening in neonates and infants. An exhaustive list of etiologies is impossible and would not be useful, but it is worth distinguishing infantile nasal obstruction and nasal obstruction in older children, as causes differ greatly. This is the topic of the present update.
Topics: Adenoids; Child; Craniofacial Abnormalities; Foreign Bodies; Humans; Hypertrophy; Meningocele; Nasal Obstruction; Nasal Polyps; Nasal Septum; Nose Neoplasms; Rhinitis; Teratoma
PubMed: 26471039
DOI: 10.1016/j.anorl.2015.09.011 -
Global Pediatric Health 2023Meningocele is a rare congenital malformation of the central nervous system resulting from a herniation of the meninges containing cerebrospinal fluid through a bony...
Meningocele is a rare congenital malformation of the central nervous system resulting from a herniation of the meninges containing cerebrospinal fluid through a bony defect in the skull. It is part of neural tube closure anomalies. The overall incidence of meningocele is 0.8 to 3 per 10 000 live births; it varies based on geographical location and race, with a predominance of occipital localization. Among diagnostic methods, computed tomography (CT) and magnetic resonance imaging (MRI) remain crucial examinations. Prenatal diagnosis of this malformation primarily relies on ultrasound and screening through maternal serum alpha-fetoprotein (AFP) levels. They typically manifest very early at birth with a highly suggestive radioclinical presentation. Surgical treatment usually allows for a definitive cure. Superinfection and rupture of the meningocele are exceptional, resulting from delayed diagnosis and management. In several developed countries, prevention through genetic counseling and the intake of folic acid during the periconceptional period, along with accurate prenatal diagnosis and the legalization of therapeutic abortion, has led to a decrease in the prevalence of meningoceles. In less developed countries, where there is a delay in diagnosis in exceptional cases, superinfection and rupture may occur. We present the case of an 8-month-old infant with a complicated occipital meningocele with superinfection and rupture.
PubMed: 37846398
DOI: 10.1177/2333794X231204498 -
Current Opinion in Neurology Feb 2023Idiopathic intracranial hypertension (IIH) is a disorder of raised intracranial pressure (ICP). Although the majority of patients with IIH present classically with... (Review)
Review
PURPOSE OF REVIEW
Idiopathic intracranial hypertension (IIH) is a disorder of raised intracranial pressure (ICP). Although the majority of patients with IIH present classically with headache and papilledema, some patients may have unusual presentations or manifestations. Recent advancements in neuroimaging have facilitated the identification of other presentations associated with IIH. This review provides an overview of the expanding clinical spectrum of IIH.
RECENT FINDINGS
Presentations of IIH that are considered unusual include highly asymmetric or unilateral papilledema, IIH without papilledema, and IIH associated with cranial nerve involvement. These presentations likely reflect differences in the way cerebrospinal fluid (CSF) pressure is transmitted intracranially. Radiological signs of intracranial hypertension are increasingly recognized in patients with IIH and provide further insights into the effects of raised ICP on intracranial structures. Osseous changes in the skull base leading to formation of meningoceles and encephaloceles have been identified in patients with IIH, spontaneous skull base CSF leak, and drug-resistant temporal lobe epilepsy, suggesting a possible association.
SUMMARY
Clinicians should be familiar with the expanding clinical spectrum of IIH and the implications for the management of these presentations.
Topics: Humans; Pseudotumor Cerebri; Papilledema; Intracranial Hypertension; Cerebrospinal Fluid Leak; Neuroimaging
PubMed: 36444979
DOI: 10.1097/WCO.0000000000001131 -
BMJ Case Reports May 2017
Topics: Humans; Magnetic Resonance Imaging; Male; Meningocele; Middle Aged; Sciatica; Spinal Dysraphism
PubMed: 28478392
DOI: 10.1136/bcr-2017-220213 -
Surgical Neurology International 2022Intrasacral occult sacral meningoceles (OSM) are uncommon congenital lesions that rarely become symptomatic, even over a patient's life time.
BACKGROUND
Intrasacral occult sacral meningoceles (OSM) are uncommon congenital lesions that rarely become symptomatic, even over a patient's life time.
METHODS
We operated on six patients with symptomatic OSM diagnosed on MR studies (all six) and/or CT examinations (four cases).
RESULTS
All six patients had uneventful postoperative recoveries. Nevertheless, despite the resolution of low back pain and radiculopathy, preoperative bladder dysfunction improved postoperatively in only one patient.
CONCLUSION
Few cases of patients undergoing surgery for symptomatic OSM are reported in the literature. Here, we recount our experience with six patients with symptomatic OSM who demonstrated significant postoperative neurological recoveries except for rare improvement in bladder function.
PubMed: 35399876
DOI: 10.25259/SNI_1258_2021 -
Neurology India 2018
Topics: Adult; Encephalocele; Female; Fetus; Gestational Age; Humans; Magnetic Resonance Imaging; Meningocele; Spinal Canal; Ultrasonography, Prenatal
PubMed: 29766970
DOI: 10.4103/0028-3886.232299 -
Cureus Feb 2023The sphenoethmoidal meningocele is a herniation of the meninges through a communication of the skull base with an aeric cavity. It means the presence of an...
The sphenoethmoidal meningocele is a herniation of the meninges through a communication of the skull base with an aeric cavity. It means the presence of an osteomeningeal breach, which is manifested by cerebrospinal rhinorrhea and nasal obstruction. iIs diagnosis is based on a very specific radiological assessment and biology allows the dosage of certain substances to confirm the nature of the cerebrospinal fluid, such as beta-2-transferrin, Once the breach has been found, the endoscopic route exclusively allows the pathology to be treated and the defect to be reconstructed using different materials before the occurrence of serious complications such as meningitis.
PubMed: 36938298
DOI: 10.7759/cureus.35022 -
International Medical Case Reports... 2018Optic nerve sheath meningocele, also called dural ectasia of the optic nerve, is a benign dilation of the optic nerve sheath. We report two interesting cases of primary...
Optic nerve sheath meningocele, also called dural ectasia of the optic nerve, is a benign dilation of the optic nerve sheath. We report two interesting cases of primary optic nerve sheath meningocele. Etiology, clinical features, and management options are discussed.
PubMed: 30237744
DOI: 10.2147/IMCRJ.S166655 -
Proceedings of the Royal Society of... Sep 1939
PubMed: 19992089
DOI: No ID Found -
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