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Neurology India 2020
Topics: Child; Child, Preschool; Encephalocele; Failure to Thrive; Humans; Infant; Infant, Newborn; Intellectual Disability; Meningocele; Neurosurgical Procedures; Scalp; Skull
PubMed: 32129233
DOI: 10.4103/0028-3886.279713 -
European Review For Medical and... Aug 2022Encephalocele is a rare congenital neural tube defect (NTD) characterized by herniation of intracranial contents through a defect in the skull. In our study,...
OBJECTIVE
Encephalocele is a rare congenital neural tube defect (NTD) characterized by herniation of intracranial contents through a defect in the skull. In our study, encephalocele was diagnosed in our clinic and its association with hydrocephalus was evaluated. The effect of this association on the prognosis was discussed.
PATIENTS AND METHODS
Patients who underwent surgery and follow-up with the diagnosis of encephalocele in the neurosurgery clinic of our hospital in an 8-year period from 2013 to 2021 were retrospectively examined.
RESULTS
Patient records were obtained from the case notes of patients who underwent excision and repair for encephalocele. Of the 78 patients included in the study, 88.4% underwent surgery in the neonatal period. Moreover, 47% of the patients are male, and 31% are female. Encephalocele was present in 62.8% of patients and meningocele in 37.2%. Furthermore, 82.1% of encephalocele sacs were located in the occipital region. Chiari type 3 malformation was present in 57.6% of patients. Hydrocephalus developed in 56.4% of patients. There was an additional syndrome in 10.3% of the cases. The most common additional syndromes were corpus callosum dysgenesis with 39.7% and colpocephaly with 29.5%. The additional disease was present in 43.6% of patients. Preoperative and postoperative examination findings of more than half of patients were normal, but 33.3% were apathetic. Furthermore, 67.9% of patients, who underwent complete repair, survived, and 32.1% died. Hydrocephalus was present in 73.5% of patients with encephalocele (p<0.05). Hydrocephalus developed in 77.8% of patients with Chiari type 3 malformation (p<0.05). Hydrocephalus was found in 88.0% of patients with Ex (p<0.05).
CONCLUSIONS
Encephalocele, which is a subgroup of NTD, differs clinically by its location and accompanying additional anomalies. In encephaloceles, the risk of morbidity and mortality can only be reduced with the multidisciplinary approach. Hydrocephalus and Chiari type 3 malformation are common in patients with encephalocele. These associations adversely affect the prognosis of the disease. Further research should be conducted on the evaluation of risk factors of NTD and methods of prevention from NTD. In this regard, we recommend that the training be repeated at certain intervals and that people's awareness should be raised.
Topics: Encephalocele; Female; Humans; Hydrocephalus; Infant, Newborn; Male; Neural Tube Defects; Prognosis; Retrospective Studies
PubMed: 35993634
DOI: 10.26355/eurrev_202208_29407 -
European Annals of Otorhinolaryngology,... Dec 2016Sinonasal malignancies are rare and histologically heterogeneous. Treatment is complicated by tumor aggressiveness and location near critical anatomic structures... (Review)
Review
Sinonasal malignancies are rare and histologically heterogeneous. Treatment is complicated by tumor aggressiveness and location near critical anatomic structures (orbita, skull base, etc.). This low incidence and histologic diversity make prospective studies unfeasible, and thus therapeutic guidelines difficult to establish. The gold standard for surgery is a transfacial approach, with craniofacial resection in case of skull-base involvement. However, these techniques are associated with non-negligible perioperative morbidity. In the past two decades, endoscopic surgery has made major progress, extending its indications: initially developed for functional sinus surgery, it is now applied in benign skull-base pathologies (CSF leakage, meningocele, etc.) and, more recently, in sinonasal malignancy. Literature analysis shows a significant decrease in morbidity and improved quality of life associated with endoscopic endonasal surgery, with oncologic safety and efficacy in well-selected cases, although dependent on operator experience. Additional studies with longer follow-up and comparison between histologic subtypes will be needed.
Topics: Endoscopy; Hemostasis, Surgical; Humans; Magnetic Resonance Imaging, Interventional; Nose Neoplasms; Paranasal Sinus Neoplasms; Patient Selection; Radiography, Interventional
PubMed: 27386803
DOI: 10.1016/j.anorl.2016.06.001 -
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 -
Head and Neck Pathology Sep 2018Neural, sclerosing, and myofibroblastic lesions of the ear and temporal bone present diagnostic challenges for both clinicians and pathologists due to significant... (Review)
Review
Neural, sclerosing, and myofibroblastic lesions of the ear and temporal bone present diagnostic challenges for both clinicians and pathologists due to significant overlap in their clinical presentations, histologic appearances, and immunohistochemical profiles. While some of these lesions, such as schwannomas, are relatively common, others are rendered even more difficult because they are encountered very rarely in routine surgical pathology practice. This review is intended to provide an update on the pathology of some of the most commonly encountered primary diagnostic entities for the ear and temporal bone, and includes the following neural lesions: schwannoma, meningioma, and encephalocele/meningocele. Sclerosing lesions that will be discussed include spindle cell and sclerosing rhabdomyosarcoma, sclerosing epithelioid fibrosarcoma, and sclerosing paraganglioma. Finally, myofibroblastic lesions that will be reviewed are nodular fasciitis, IgG4-related disease, and solitary fibrous tumor. For each of these lesions, the differential diagnosis and useful ancillary tests will be discussed in the context of a broad range of additional primary and secondary lesions.
Topics: Bone Diseases; Ear Diseases; Humans; Temporal Bone
PubMed: 30069839
DOI: 10.1007/s12105-018-0891-9 -
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 -
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 -
Neurology India 2022The culprit of trigeminal neuralgia (TGN) may occur at any point between the nerve's root entry zone (REZ) and Meckel's cave. Meckel's cave meningoencephaloceles are... (Review)
Review
BACKGROUND
The culprit of trigeminal neuralgia (TGN) may occur at any point between the nerve's root entry zone (REZ) and Meckel's cave. Meckel's cave meningoencephaloceles are rare middle cranial fossa defects that usually remain asymptomatic but may contain prolapsed trigeminal nerve rootlets and result in TGN. Their management and surgical outcomes remain poorly understood.
OBJECTIVES
To perform a systematic review of clinical presentation and surgical outcomes of middle fossa defects presenting with trigeminal nerve-related symptoms.
MATERIALS AND METHODS
A systematic review was conducted in accordance with the PRISMA guidelines for all reports of middle cranial fossa defects causing trigeminal nerve-related symptoms. The pathophysiology, presentation, surgical management, and outcomes are discussed and illustrated with a case.
RESULTS
Initial search from inception to March 2021 identified 33 articles for screening. After applying inclusion and exclusion criteria, 6 articles were included representing a total of 8 cases in addition to our case (n = 9). All 9 patients were females and 33.3% (n = 3) presented with classic trigeminal neuralgia. "Empty sella" syndrome and radiologic signs of intracranial hypertension were present in 40%-62%. No patient presented with cerebrospinal fluid leak. The preferred treatment modality was surgical with subtemporal extradural repairs using combinations of autologous fat and muscle grafts and synthetic dura. Postoperative outcomes were only available in 55.5% (n = 5) of the cases, and nearly all reported complete symptom resolution, except for one case in which the meningoencephalocele wall was incised, along with trigeminal rootlets adhered to it. Our patient had immediate and durable symptom relief after a 4-year follow-up.
CONCLUSIONS
MEC containing prolapsed trigeminal nerve rootlets can cause typical trigeminal neuralgia from chronic pulsatile stress. This supports the hypothesis that the compressive or demyelinating culprit can locate more ventrally on the course of the trigeminal nerve. Subtemporal extradural surgical repairs can be safe, effective, and durable. Incising the MEC wall should be avoided as it may have trigeminal rootlets adhered to it.
Topics: Cranial Fossa, Middle; Dura Mater; Encephalocele; Female; Humans; Male; Meningocele; Trigeminal Nerve; Trigeminal Neuralgia
PubMed: 35864609
DOI: 10.4103/0028-3886.349629