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Spontaneous delayed CSF rhinorrhea associated with encephalocele following surgical resection of a giant frontoparietal meningioma, an extremely rare entity and overlook of English literature
Abstract
Introduction and importance
Spontaneous cerebrospinal fluid (CSF) rhinorrhea without a history of head trauma is rare. The authors describe a scarce case of spontaneous, nontraumatic, delayed CSF rhinorrhea due to ethmoidal encephalocele associated with frontoparietal giant meningioma.
Case presentation
A 49-year-old male complained of a slowly growing skull mass lasting 10 years since a blunt head trauma and progressive hemiparesis 3 months ago and was referred to our center. There was a heterogeneous extra-axial mass (77×77×70 mm) with calcified components at his right frontal lobe, demonstrating notable adjacent parenchymal edema and a midline shift to the left. There was a sizeable extra-axial mass (113×95×80 mm) in his right front-temporoparietal lobe associated with adjacent invasion to calvarium leading to the lytic and sclerotic appearance of the calvarial bone showing intense heterogeneous contrast enhancement and an adjacent enhancing dural tail. A considerable mass effect on the adjacent frontotemporal cortex is evident, with mild parenchymal edema leading to a notable midline shift to the left, right uncal herniation, and left ventriculomegaly. On postoperation day 5, the patient developed a recurrent CSF leak exacerbated by hydrocephalus. He was rescheduled to undergo endoscopic endonasal surgery and dural repair on postoperative day 7. The postoperative course was uneventful.
Clinical discussion
Frontoparietal lobe encephaloceles represent the least common cause of spontaneous CSF rhinorrhea. Early diagnosis and surgical management remain crucial to minimize the subsequent complications.
Conclusion
The ventricular anatomy and CSF fluid dynamics alteration following tumor surgical resection seem to contribute to an environment by which a herniated ethmoidal encephalocele developed a delayed nontraumatic CSF leak.
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Introduction and importance
Meningiomas without dural attachments are quite a novelty, with such meningiomas in the posterior fossa being even more far and few between. The authors describe an extremely rare cerebellopontine angle (CPA) meningioma lacking a dural attachment arising from the choroid plexus of the foramen of Luschka (CPFOL).
Case presentation
A 35-year-old male presented to our center complaining of a generalized and progressive headache for 10 months. A 3 cm × 4 cm well-defined lesion in the left CPA, hypointense in T1 and hyperintense in T2-weighted magnetic resonance imaging (MRI), was noted with no evident dural base or dural attachment. CPFOL was appreciated right at the point where the tumoral base was detected, which implied that the tumor originated from CPFOL. A gross total resection (GTR) was achieved, with the postoperative period remaining uneventful. The histopathologic investigation confirmed a transitional meningioma World Health Organization (WHO) grade I with no atypical features.
Clinical discussion
Meningioma without dural attachment remains a rare phenomenon, with few available in English literature, and such pathology in the posterior fossa is even more unusual. Discriminating between CPFOL and another kind of CPA meningioma is mandatory when dealing with intracranial meningioma surgeries.
Conclusion
While managing a space-occupying lesion around CPA, although extremely rare, a meningioma originating from CPFOL is suggested to be included in the differentials with necessary imaging analysis and advised to be imperatively pursued before proceeding for surgical intervention.
Introduction and importance:
Primary central nervous system (CNS) lymphomas (PCNSLs) comprise a heterogeneous subset of intracranial disorders, predominantly of the intraparenchymal high-grade non-Hodgkin's lymphoma. Intracranial pseudolymphoma represents an exceedingly rare entity; as few as 3 reports in the English literature. We describe the first multiple large intracranial pseudolymphomata leading to increased intracranial pressure, visual loss, and recurrence during a short while. It also represents the first report of intracranial pseudolymphoma presented as a skull base tumor.
Case presentation:
We describe a 67-year-old female suffering from left-sided loss of visual acuity, headache, nausea, vomiting, and improper balance. Axial brain computed tomography (CT) scan revealed an isodense anterior interhemispheric mass lesion with surrounding edema in both frontal lobes. T1 and T2 weighted magnetic resonance imaging (MRI) and T1 weighted with gadolinium injection revealed two extra-axial isointense dural-based mass lesions with homogenous enhancement compressing both frontal lobes. The morphologic findings favored B cell pseudolymphoma and meningeal B cell lymphoid hyperplasia. One year later, she developed headaches, disorientation, and progressive meaningless speech lasting 2 months. Subsequent MRI demonstrated the rapid growth of the lesion of the lesser sphenoid wing and recurrence of the lesion at the same site of surgery, thereby undergoing revision surgery in which both tumors were maximally resected using a pterional approach.
Clinical discussion:
Intracranial pseudolymphoma remains exceedingly rare, and despite its benign cellular nature, it may proliferate and recur quickly.
Conclusion:
Intracranial pseudolymphoma should always be considered a rare but potentially differential diagnosis leading to the intraventricular lesion.
Intracranial hypotension (IH) represents a rare complication, mainly following cerebrospinal fluid (CSF) leakage at the thoracic or cervicothoracic junction level. Iatrogenic IH may be expected secondary to the previous surgery or other procedures invading the patient's dura. Magnetic resonance imaging (MRI), computerized tomography (CT) scan images, CT cisternography, and magnetic resonance cerebrospinal fluid flow (MR CSF) remains the modality of choice to establish the diagnosis. The patient is in her late sixth decade, reflecting a history of progressive headaches, nausea, and vomiting. Once a diagnosis of foramen magnum meningioma was established using MRI, total microscopic resection was applied. Brain sagging and subdural fluid collection were identified on postoperative day three, suggesting intracranial hypotension due to cerebrospinal fluid leakage. Diagnosing IH following the CSF leak during the postoperative phase remains challenging. Although rare, early clinical suspicion must be considered to establish the diagnosis.
Background:
The Surgical CAse REport (SCARE) guidelines were first published in 2016 as a tool for surgeons to document and report their surgical cases in a standardised and comprehensive manner. However, with advances in technology and changes in the healthcare landscape, it is important to revise and update these guidelines to ensure they remain relevant and valuable for surgeons. This paper presents an update to these guidelines.
Materials and Methods:
The updated guidelines were produced through a Delphi consensus exercise. Members of the SCARE 2020 guidelines Delphi group, editorial board members, and peer reviewers were invited to participate. Potential contributors were contacted by email. An online survey was completed to indicate their agreement with the proposed changes to the guideline items.
Results:
A total of 54 participants were invited to participate and 45 (83.3%) completed the survey. There was a high degree of agreement among reviewers, with 36 items (83.7%) meeting the threshold for inclusion within the updated guidelines.
Conclusion:
Through a completed Delphi consensus exercise we present the SCARE 2023 guidelines. This will provide surgeons with a comprehensive and up-to-date tool for documenting and reporting their surgical cases while highlighting the importance of patient-centered care.
Encephalocele is a protrusion of the intracranial contents through a cranium defect. Encephalocele is divided into primary (congenital) and secondary (acquired) classes. From an epidemiological point of view, primary encephalocele is much more prevalent than secondary cases. Furthermore, among the secondary causes of this condition, iatrogenic encephalocele is recognized as a rare phenomenon. In this case report, we introduce a case of secondary encephalocele in a 30-year-old pregnant female who came to our emergency center at 5 weeks of gestation with a vague headache in her forehead and a runny nose. she reported a history of nasal polypectomy 9 months ago and a 10-day hospitalization for meningitis 5 months prior to admission. MRI of the patient's brain showed evidence of cerebral parenchymal herniation to the right nasal cavity, which was suggestive of encephalocele. She was scheduled for endoscopic transnasal reconstruction, and during the operation, a significant right-sided posterior ethmoidal roof defect with CSF leak and encephalocele was revealed. Eventually, the skull defect was successfully repaired with a vascularized flap, and the patient was discharged in good general condition.
Meningiomas are the most common primary intracranial tumors. The majority of meningiomas are benign, but they can present different grades of dedifferentiation from grade I to grade III (anaplastic/malignant) that are associated with different outcomes. Radiological surveillance is a valid option for low-grade asymptomatic meningiomas. In other cases, the treatment is usually surgical, aimed at achieving a complete resection. The use of adjuvant radiotherapy is the gold standard for grade III, is debated for grade II and is not generally indicated for radically resected grade I meningiomas. The use of systemic treatments is not standardized. Here we report a review of the literature on the clinical, radiological and molecular characteristics of meningiomas, available treatment strategies and ongoing clinical trials.
Diagnosis and treatment of neurosurgical pathology present unique challenges in underserved areas, and many conditions may go undiagnosed, misdiagnosed, or untreated for prolonged periods. The development of an unusual complication, seemingly unrelated to an area of neurosurgical intervention, may be particularly perplexing to non-neurosurgical providers, particularly in areas where neurosurgical procedures have not historically been available. A 44-year-old male presented with a giant meningioma which was successfully resected. A nasal encephalocele was noted preoperatively but was not addressed due to lack of associated symptoms and distance from the tumor. The patient lived on a remote island and was lost to follow-up. He developed delayed cerebral spinal fluid (CSF) rhinorrhea three months after surgery, which was diagnosed and treated by local providers as allergic rhinitis for 11 months until he presented with new-onset seizure. Imaging demonstrated descent of the lateral ventricle into the encephalocele. The encephalocele was amputated and the skull base defect was repaired successfully. The alteration of ventricular anatomy and CSF fluid dynamics following tumor resection appears to have created an environment where a non-traumatic CSF leak could develop where it had previously shown no signs of developing. It may be prudent to treat skull base defects prophylactically to prevent this type of complication, particularly in patients of remote regions where regular follow-up is difficult.
Meningoencephaloceles of the skull base most commonly occur as a sequela of head trauma or they can more rarely be congenital malformations. Several types of encephalocele exist depending on anatomic features and localisation. Clinical presentation and symptoms can vary. Different methods of management and repair of the concurring skull base defects have been described and ranging vary from endoscopic to open surgical approaches. We report the case of a 56-year-old Caucasian woman with the diagnosis of right sided spontaneous transethmoidal meninoencephalocele. Clinical presentation of this rare pathology, methods of diagnostic and management and its outcome are presented. Spontaneous skull base meningoencephaloceles are rare entities, without clear underlying etiologies. Multidisciplinary management is recommended. The transnasal endoscopic approach provides a wide skull base exposure with more advantages and less morbidity in comparison with the conventional open approach.
Background
Underserved women (rural, minority or poor) are disproportionally diagnosed with late-stage cervical cancer, indicative of inadequate access to, and use of, preventative healthcare. The Institute of Medicine (IOM) has proposed that nurse practitioners (NP) can address provider shortages among underserved populations, but to reduce shortages, scope-of-practice laws that restrict the delivery of care, must be revised. We examined the IOM recommendation of NP expanded scope-of-practice laws on reducing the disparity of underserved women diagnosed with late-stage cervical cancer.
Methods
We examined the cohort of 10 673 women diagnosed with cervical cancer between 2010 and 2014 and reported to the Surveillance, Epidemiology and End Results cancer registry. We linked state-level laws regarding NP scope-of-practice to patients with cancer by their state of residence, diagnosis date and law enactment date. Hierarchical regression was used to explore NP full scope-of-practice law’s impact on late-stage cancer diagnoses considering the moderating effect of women living in medically underserved areas. We adjusted for known confounders available in this population-based data set.
Results
Medically underserved women living in states with laws that restrict NP full scope-of-practice are twofold more likely to be diagnosed with late-stage cancer; adjusted OR and 95% CI (OR 2.08, 95% CI 1.4 to 3.1). These disparities were not observed among underserved women living in areas with NP full scope-of-practice laws (OR 0.95, 95% CI 0.7 to 1.3).
Conclusions
NP full scope-of-practice laws could provide a pragmatic and cost-effective solution to healthcare provider shortages associated with late stage of cervical cancer diagnoses among underserved women.
Spontaneous cerebrospinal fluid (CSF) rhinorrhea in an adult without prior history of head trauma is a rare occurrence. Neuroimaging is done to ascertain the site of bony defects in the cranium and breach in the continuity of dura to localize the site of CSF leakage. Encephaloceles into the sphenoid sinus occurring through congenital bony defects in the walls of the sinus are an extremely rare cause for CSF rhinorrhea. We present a case of spontaneous, nontraumatic CSF rhinorrhea due to an anteromedial encephalocele where the temporal lobe was seen herniating into the right sphenoid sinus through a congenital bony defect in the wall of the sinus. A review of the present literature is also presented.
Encephalocele is a rare congenital abnormality characterized by abnormal protrusion of brain and meninges through an opening in the skull. We report an 8-year-old girl who presented with a swelling in the right lower lid for the last 6 years. In her infancy, she had undergone surgery for a very small swelling located in the right nasolacrimal area. On further clinicoradiological evaluation, anterior encephalocele was diagnosed. This case highlights the uncommon site of anterior encephalocele; misdiagnosis and mismanagement of which could result in dreaded complications such as meningitis and cerebrospinal fluid leaking fistula formation.
Introduction:
Although encephalocele is a rare congenital abnormality, secondary encephalocele is extremely rare and can cause fatal complications. Here, we report a case of secondary encephalocele caused by frontal sinus wall defect due to chronic sinusitis, which was completely removed by cranialization with autologous bone graft. A 50-year-old man with a 10-year history of chronic sinusitis visited our hospital due to suddenly altered mentality characterized by stupor. Computerized tomography scanning and magnetic resonance imaging revealed an enlarged left frontal sinus with sinusitis. The frontal sinus cavity was calcified, and the left frontal lobe had herniated into the cavity accompanied by yellow pus. A large dural defect was also found around the frontal sinus area. After removal of the abscess and some of the frontal lobe, frontal skull base repair by cranialization was performed using autologous bone graft. Streptococcus pneumoniae was cultured from the cerebrospinal fluid (CSF), necessitating treatment with antibiotics. After the operation, the mental status of the patient improved and no CSF leakage was observed.
Discussion:
In addition to correct diagnosis and early treatment including antibiotics, the surgical repair of defects is needed in patients with secondary encephalocele to prevent further episodes of meningitis. Surgical correction of frontal sinus encephalocele can be achieved through bifrontal craniotomy or endoscopic transnasal repair. If a patient has CSF leakage, open craniotomy may facilitate repair of the dural defect and allow for cranialization of the sinus.
Conclusions:
Removal of dysplastic herniated brain tissue and cranialization of the frontal sinus may be a good option for treating secondary encephalocele and its associated complications, including meningitis, abscess formation, and infarction of the herniated brain parenchyma.
Basal encephalocele is rare in adults. Congenital and acquired cases have been reported with regard to the developmental mechanism, and the pathology has not been elucidated in detail.
We encountered an adult with basal encephalocele strongly suggesting congenital development because of the presence of minor anomalies: strabismus and ocular hypertelorism. The disease manifested as persistent spontaneous cerebrospinal fluid rhinorrhea and repeated meningitis in a 66-year-old Japanese man. On computed tomography, brain tissue protruded through a part of the ethmoid bone of his right anterior skull base, and it was diagnosed as transethmoidal-type basal encephalocele. Regarding his facial form, the distance between his bilateral eyeballs was large compared to his facial width, and his canthal index (defined as inner to outer inter canthal ratio x 100) was calculated as 38.5, based on which it was judged as ocular hypertelorism. In addition, his right eyeball showed strabismus. A right frontotemporal craniotomy was performed for spontaneous cerebrospinal fluid rhinorrhea, and the defective dura mater region was patched with temporal fascia.
Mild minor anomalies that require no treatment are overlooked in adults, but the presence of several anomalies increases the possibility of congenital disease. Therefore, it may be necessary to examine minor anomalies in cases of adult basal encephalocele when considering the possibility that the disease may be congenital.
The sphenoid sinus is rarely implicated as a site of spontaneous CSF fistula. We undertook this study to evaluate the potential etiopathogenesis of spontaneous CSF fistula involving the sphenoid sinus and to review the imaging findings.
We retrospectively reviewed the imaging findings of 145 cases of CSF fistula from our departmental archives (August 1995 through August 1998). Fifteen (10%) patients had CSF fistulas involving the sphenoid sinus. Eleven (7%) patients had spontaneous CSF fistulas, whereas in four patients, the CSF fistulas in the sphenoid sinus were related to trauma. Of the 11 patients, nine underwent only plain high-resolution CT and MR cisternography. One patient additionally underwent contrast-enhanced CT cisternography, and one other patient underwent MR cisternography only. For each patient, the CSF fistula site was surgically confirmed. The MR imaging technique included T1-weighted and fast spin-echo T2-weighted 3-mm-thick coronal sequences obtained with the patient in the supine position. The plain high-resolution CT study included 3-mm-thick, and sometimes 1- to 1.5-mm-thick, coronal sections obtained with the patient in the prone position. Similar sections were obtained after injecting nonionic contrast material intrathecally via lumbar puncture for the CT cisternographic study. We evaluated each of the 11 patients for the exact site of CSF leak in the sphenoid sinus. We also determined the presence of pneumatization of lateral recess of the sphenoid sinus, orientation of the lateral wall of the sphenoid sinus, presence of arachnoid pits, presence of brain tissue herniation, and presence of empty sella in each of these patients.
The exact sites of the CSF fistulas were documented for all 11 patients by using plain high-resolution CT, MR cisternography, or CT cisternography. In nine (82%) patients, the sites of the CSF fistulas were at the junction of the anterior portion of the lateral wall of the sphenoid sinus and the floor of the middle cranial fossa. In the remaining two (18%) patients, the sites of the CSF fistulas were along the midportion of the lateral wall of the sphenoid sinus. Of these 11 patients, one had bilateral sites of the CSF fistula at the junction of the anterior portion of the lateral wall of the sphenoid sinus with the floor of the middle cranial fossa. In nine (82%) patients, the presence of brain tissue herniation was revealed, and this finding was best shown by MR cisternography. Ten (91%) patients had extensive pneumatization of the lateral recess of the sphenoid sinus, with an equal number having outward concave orientation of the inferior portion of the lateral wall of the sphenoid sinus. In seven (63%) patients, the presence of arachnoid pits, predominantly along the anteromedial aspect of the middle cranial fossa, was shown. In seven (63%) patients, empty sella was shown. For comparison, we reviewed the CT studies of the paranasal sinuses in 100 age-matched control subjects from a normal population. Twenty-three had extensive lateral pneumatization of the sphenoid sinus along with outward concavity of the inferior portion of the lateral wall. None of these 23 patients had arachnoid pits.
The sphenoid sinus, when implicated as a site of spontaneous CSF leak, yields a multitude of imaging findings. These are extensive pneumatization of the lateral recess of the sphenoid sinus, outward concave orientation of the inferior portion of the lateral wall of the sphenoid sinus, arachnoid pits, and empty sella. Considering the normative data, we speculate that this constellation of findings could play a role in the etiopathogenesis of spontaneous sphenoid sinus fistulas. Our findings also show the efficacy of noninvasive imaging techniques, such as plain high-resolution CT and MR cisternography, in the evaluation of sphenoid sinus CSF leak. Our data also suggest that spontaneous sphenoid sinus CSF leak is not an uncommon occurrenc
This report describes the sensitivity and specificity of glucose detection using Glucostix test strips and computed tomography (CT) of the skull base for confirming cerebrospinal fluid (CSF) fistulae in patients with persistent rhinorrhoea or otorrhoea, and comparing them with the beta-2 transferrin assay as the gold standard for CSF detection.
Fluid samples from the nose were collected from 18 patients with suspected CSF fistulae. The samples were assayed for beta-2 transferrin using the Western blotting and immunostaining technique. CT (5mm axial slice) of the skull base was performed for evidence of skull base fracture. The glucose levels and Glucostix results were compared.
Out of the 18 samples, 15 were positive for beta-2 transferrin adn the leaks were validated surgically in 10 patients. Give leaks healed spontaneously with conservative management. Glucostix tests produced three false positive results from blood and nasal mucus contaminated fluid. Glucostix failed to detect another three CSF leaks resulting from false negative tests because of low CSF glucose levels. The Glucostix glucose test was nonspecific and insensitive compared with the beta-2 transferrin assay. CT failed to detect three of the 15 beta-2 transferrin-positive leaks but there were no false positive results. CT produced six negative results, of which three were false negatives.
Glucose detection using Glucostix test strips is not recommended as a confirmatory test due to its lack of specificity and sensitivity. In the presence of a skull bas fracture on CT and a clinical CSF leak, there is no need for a further confirmatory test. In cases where a confirmatory test is needed, the beta-2 transferrin assay is the test of choice because of its high sensitivity and specificity.
Defects at the skull base leading to spontaneous CSF rhinorrhea are rare lesions. The purpose of our study was to correlate CT and MR findings regarding the location and content of CSF leaks in 27 patients with endoscopic sinus surgery observations.
Imaging studies in 27 patients with intermittent CSF rhinorrhea (CT in every patient including 10 examinations with intrathecal contrast, plain CT in 2 patients, and MR in 15 patients) were analyzed and were retrospectively blinded to intraoperative findings.
CT depicted a small endoscopy-confirmed osseous defect in 3 different locations: 1) within the ethmoid in 15 instances (53.6% of defects) most commonly at the level of the anterior ethmoid artery (8/15); 2) adjacent to the inferolateral recess of the sphenoid sinus in 7 patients including one patient with bilateral lesions (8/28 defects, 28.6%); 3) within the midline sphenoid sinus in 5 of 28 instances (17.9%). Lateral sphenoid defects (3.5 +/- 0.80 mm) were larger than those in ethmoid (2.7 +/- 0.77 mm, P < or = 0.029) or midsphenoid location (2.4 +/- 0.65 mm, P < or = 0.026). With endoscopy proven arachnoid herniation in 24 instances as reference, MR was correct in 14 of 15 instances (93.3%), CT cisternography in 5 of 8 instances (62.5%). Plain CT in 1 patient was negative.
In patients with a history of spontaneous CSF rhinorrhea, CT was required to detect osseous defects at specific sites of predilection. MR enabled differentiating the contents of herniated tissue and allowed identification of arachnoid tissue as a previously hardly recognized imaging finding.
A median defect in the region of the root of the nose, in between the two orbits, was discovered in the dried skull of a 44-year-old female cadaver, during routine undergraduate teaching. The two small nasal bones articulated with each other and the cribriform plate of the ethmoid. The lacrimal bones and frontal processes of the maxillary bones were also deformed. We propose that the median defect was due to abnormality at the fonticulus frontalis, the prenasal space and the interorbitonasal part of the nasal capsule, as well as defects in the ossification of the maxilla, lacrimal and frontal bones.
Background
Explosive growth of intracranial meningioma during pregnancy is rare and poses significant surgical and anesthetic challenges. The current paper aims to describe cesarean section (CS) and craniotomy in one anaesthesiologic setting and enumerate the challenges encountered in such rare entities in neurosurgery.
Case presentation
The authors report a pregnancy-associated giant intracranial meningioma, which underwent CS at 37 weeks of gestational age followed by emergent craniotomy in the same setting because of the mass effect and progressive lower limb weakness. To our knowledge, this is the second successful case in contemporary literature without any morbidity.
Conclusion
Several strategies have been elucidated for intracranial tumors during pregnancy; however, no evidence-based protocol is currently available. The plausible recommendation is a CS as the first surgery and the neurosurgical intervention when the patient's neurological status and gestational age allow.
Background
Black women are more likely to be diagnosed at a later stage of breast cancer in part due to barriers to timely screening mammography, resulting in poorer mortality and survival outcomes. Patient navigation that helps to overcome barriers to the early detection of breast cancer is an effective intervention for reducing breast cancer disparity. However, the ability to recognize and seek help to overcome barriers may be affected by gendered and racialized social expectations of women.
Methods
Data from a randomized controlled trial, the Patient Navigation in Medically Underserved Areas study, were used. The likelihood of obtaining a follow‐up screening mammogram was compared between women who identified ≥1 barriers and those who did not.
Results
Of the 3754 women who received the Patient Navigation in Medically Underserved Areas navigation intervention, approximately 14% identified ≥ 1 barriers, which led to additional navigator contacts. Consequently, those women who reported barriers were more likely to obtain a subsequent screening mammogram. Black women, women living in poverty, and women with a higher level of distrust were less likely to report barriers.
Conclusions
Minority women living in poverty have always been the source of social support for others. However, gendered and racialized social expectations may affect the ways in which women seek help for their own health needs. A way to improve the effectiveness of navigation would be to recognize how minority women's gender images and expectations could shape how they seek help and support. A report of no barriers does not always translate into no problems. Proactive approaches to identify potential barriers may be beneficial. Cancer 2018;000:000–000. © 2018 American Cancer Society.
Background. Disparities have been observed in both the incidences of lung and esophageal cancers and the survival of those patients. Our goals were to determine if recw was associated with stage of cancer at diagnosis, and to identify predictors of advanced-stage lung and esophageal cancers. Methods. All cased of lung and esophageas cancer between 1991 and 1995 in the Savannah River Region Information System cancer registry were studied. Data were analyzed using logistic regression to identify independent predictors of advanced disease at the time of diagnosis. Results. Among lung cancer patients, histology and distance to nearest hospital predicted diagnosis at an advanced stage. Residence in an area with a high proportion of Medicaid recipients was a predictor of advanced stage in esophageal cancer patients. Conclusions. In this predominantly rural ares, decreased utilization of health services was evident among older, poor, black, rural cancer patients. Further investigation involving prospective data collection from cancer patients is warrented.
Objective: Temporal lobe encephaloceles are characterized by protrusion of brain parenchyma through a structural defect in the floor of the middle fossa. They have been reported to cause cerebrospinal fluid (CSF) leaks, conductive hearing loss, meningitis, and seizures. The association between temporal encephaloceles and epileptiform activity is particularly rare.
Patients: All patients who presented to a single tertiary referral center between 2011 and 2014 with intractable seizures and radiographic evidence of a middle cranial fossa encephalocele were evaluated. Five patients from this subset who underwent surgical repair of their encephalocele are presented.
Intervention(s): Middle cranial fossa approach for encephalocele repair.
Main Outcome Measure(s): Postoperative epileptiform activity.
Results: Five patients underwent a craniotomy for resection of a temporal lobe encephalocele with repair of a middle fossa floor defect. After surgery, CSF rhinorrhea resolved, when present, and all patients remained seizure-free through their last available follow-up. Range of follow-up time was 3.5 months to 4 years. Average follow-up time was 19.7 months.
Conclusion: Temporal lobe encephaloceles are an infrequent cause of seizures. Given that these lesions can be missed with standard imaging modalities, they are likely underdiagnosed upon initial medical evaluation. This diagnosis should be considered in patients with intractable seizures. If an encephalocele is found, focused resection of epileptogenic tissue associated with herniation and repair of the temporal floor defect can provide definitive treatment
NASAL TUMORS occasionally contain extracranial cerebral tissue. This neural tissue is usually fibrogliomatous, although several authors have observed neurons as a component.
These lesions almost always occur on the bridge of the nose, as a subcutaneous mass, but occasionally they present inside the nose and, in rare instances, in both sites simultaneously. Such masses of neural tissue have been variously interrireted as neoplasms, cctopias, or more commonly encephaloceles. When this tumor presents in the nose, it may be diagnosed as a nasal polyp, and a surgical attack may lead to meningitis.
Two cases of encephalocele diagnosed and treated as nasal polyps illustrate the complications which may arise and emphasize the need for awareness and proper diagnosis of this entity.
Report of Cases
Case 1. Clinical History—A 45-year-old Negro man had a 6-month history of constant dripping of clear fluid from the left nostril. Previously he had been told at another
Introduction:
Encephaloceles are considered by most to be a type of neural tube defect characterized by a herniation of the brain and meninges through structural weaknesses in the bony structures of the skull.
Discussion:
Many different types of encephaloceles have been classified according to the location of the bony defect. Basal and frontoethmoidal encephaloceles constitute a nasal subclass of encephaloceles, which are herniations from the skull base and ethmoid bone, respectively. Basal encephaloceles are usually occult and can herniate into nasal structures causing obstruction. Frontoethmoidal encephaloceles usually present as a protrusion that is visible at birth and enlarges during crying. Both of these entities are rare with an incidence ranging from 1 in 5,000 to 1 in 40,000 live births around the world with the majority of cases localized in Southeast Asia.
Conclusion:
Although cases have been recorded since the sixteenth century, there is still a scarcity of knowledge on the exact causes and factors associated with the development of the disease. Many studies have determined these to be caused by a combination of genetic and environmental factors. Most cases are non-life-threatening and the preferred method of treatment is surgical removal after diagnosis is confirmed with computerized tomography or magnetic resonance imaging. Prognosis is generally positive, especially in the subset of patients with frontoethmoidal encephaloceles.
Spontaneous cerebrospinal fluid rhinorrhea represents a distinct clinic entity that is likely a variant of idiopathic intracranial hypertension (IIH). Patients with spontaneous cerebrospinal fluid (CSF) leaks are generally middle-aged obese women with radiographic evidence of skull base defects, associated meningoencephaloceles, and empty sella syndrome, a common sign of increased intracranial pressure. Significant overlap exists in the characteristics of patients with spontaneous CSF leak and IIH. Endoscopic repair of the CSF fistula is the gold standard treatment for this condition, but emerging evidence supports the reduction of CSF pressure as an important adjuvant treatment in this patient population.
Racial disparities in American health care outcomes are well documented. We investigated racial disparities in hospital mortality and adverse discharge disposition after brain tumor craniotomies performed in the United States from 1988 to 2004. We explored potential explanations for the disparities.
The data source was the Nationwide Inpatient Sample. We used multivariate ordinal logistic regression corrected for clustering by hospital and adjusted for age, sex, primary payer for care, income in postal code of residence, geographic region, admission type and source, medical comorbidity, treatment year, hospital case volume, and disease-specific factors. Random-effects pooling was also used.
A total of 99 665 craniotomies were studied. Hospital mortality and adverse discharge disposition (any discharge other than directly home) were more likely in black patients than others for all tumor types. Pooled odds ratios (ORs) and 95% confidence intervals (CIs) for blacks were: hospital craniotomy mortality (OR, 1.64; 95% CI, 1.32-2.03; P < .001), and adverse discharge disposition (OR, 1.43; 95% CI, 1.31-1.56; P < .001). Medicaid patients had higher mortality, while private-pay patients had lower mortality. Hospital annual case volume was lower for black and Hispanic patients and for those with Medicaid as the primary payer in pooled analyses, whereas patients with private insurance received care at higher-volume hospitals. Black patients generally presented with higher disease severity, including more emergency or urgent admissions (OR, 1.71; 95% CI, 1.54-1.89; P < .001); more hemiparesis and hemiplegia for primary tumors, meningiomas, and metastases (P < .04 for all); and more hydrocephalus for acoustic neuromas (P = .1).
Black patients died more often or had an adverse discharge disposition after tumor craniotomies in the United States in the period studied (1988-2004). Blacks had more severe disease at presentation and were treated at lower-volume hospitals for surgery. Other socially defined patient groups also showed disparities in access and outcomes of care.
The endoscopic endonasal approach has become the preferred technique for CSF leak and encephalocele repair of the anterior skull base. The purpose of this study is to identify patient characteristics; review adjunctive perioperative treatments, reconstruction techniques, and outcomes; and identify risk factors for failure in patients undergoing endoscopic endonasal repair of anterior skull base CSF leaks and encephaloceles.
This is a prospective observational study of patients undergoing endoscopic endonasal repair of a CSF leak between October 2004 and May 2009. Twenty-eight consecutive patients underwent 32 procedures. Twenty-two of the patients were women, which represents a statistically significant trend toward the female sex (p < 0.05). The average body mass index (33.9) was significant for obesity. The origin of the skull base defect included the cribriform plate (in 9 cases), fovea ethmoidalis (in 7), combined fovea ethmoidalis/cribriform plate (in 2), lateral sphenoid sinus (in 6), sella (in 4), clivus (in 3), and frontal sinus (in 1).
The overall endonasal closure rate was 93.8% (30 of 32 procedures). One failure occurred due to overaggressive postoperative debridement, while the other recurred along the posterior wall of the frontal sinus, and endoscopic repair would have occluded the recess.
The endoscopic endonasal approach for the treatment of CSF leaks and encephaloceles of the anterior skull base is the preferred method of repair in the vast majority of cases. The authors' 93.8% closure rate in a variety of anatomical locations compares favorably with the transcranial approach and echoes the results of other endoscopic series.
Disparities in American health care based on socially-defined patient characteristics such as race, ethnicity, and socioeconomic position are well-documented. We review differences and disparities in incidence, pathobiology, processes and outcomes of care, and survival based on social factors for brain tumors of all histologies. In the US, black patients have lower incidences of most brain tumor types and lower-income patients have lower incidences of low grade glioma, meningioma and acoustic neuroma; ascertainment bias may contribute to these findings. Pathogenetic differences between malignant gliomas in patients of different races have been demonstrated, but their clinical significance is unclear. Patients in disadvantaged groups are less often treated by high-volume providers. Mortality and morbidity of initial treatment are higher for brain tumor patients in disadvantaged groups, and they present with markers of more severe disease. Long term survival differences between malignant glioma patients of different races have not yet been shown. Clinical trial enrollment appears to be lower among brain tumor patients from disadvantaged groups. We propose future research both to better define disparities and to alleviate them.
In four patients with bacterial meningitis a primary intranasal encephalocele was found as portal of entry. In two of the cases the malformation had been misdiagnosed as a nasal polyp and operated upon. In two patients a cerebrospinal fluid fistula developed spontaneously at the age of 54 years. None of the patients had associated symptoms indicating the presence of a cleft. Encephaloceles can be readily visualized by computed tomography particularly in coronal sections. The treatment of choice is transcranial surgical repair.
A radiological study of skull base anatomy was performed in patients presenting with primary spontaneous CSF rhinorrhoea. Radiology correctly identified the fistula site in 90 per cent of cases. Contrast CT imaging was found to be the most suitable technique for identifying the presence and site of CSF fistulae. However, pre-contrast bony dehiscences were identified in all patients leaking from the cribriform plate region. More significantly, all of these patients showed deviation of their crista galli, a radiological sign hitherto unreported. These findings support the theory that congenital bony dehiscence is the aetiological basis for this condition. The importance of radiology in the management of this condition is emphasized.
Occult congenital temporal lobe encephalocele has rarely been reported in association with medically intractable complex partial seizures. The four previously reported cases were unsuspected preoperatively. We present the case of an 18-year-old woman with intractable complex partial seizures since age 13. Seizure onset was electrically localized to the right temporal lobe. Preoperative neuroimaging studies revealed a middle fossa defect and inferior herniation of the right temporal lobe. Pathologic examination of the resected encephalocele revealed prominent features of meningoangiomatosis. We believe this to be the first case of temporal lobe encephalocele and epilepsy to be diagnosed preoperatively, and the first case also to be associated with meningoangiomatosis. The relevant literature on meningoangiomatosis and on temporal lobe encephalocele as a cause of epilepsy is reviewed.
The case of a patient with spontaneous cerebrospinal fluid (CSF) rhinorrhea from an intranasal encephalomeningocele is presented. The case is unusual in that the patient was entirely asymptomatic until the age of 65, when copious CSF rhinorrhea developed without trauma or operation as an inciting event. The pertinent literature is reviewed, and no identical case has been found.
Three patients with medically refractory partial complex seizures were shown to have small and initially unsuspected encephaloceles of the middle fossa. All had bitemporal, independent, interictal epileptogenic abnormalities, and the side of origin of the seizures could not be determined by surface EEG alone. The encephaloceles were missed on the initial radiologic studies and were found retrospectively in two patients; the third was found at operation and was not demonstrable radiologically, even in retrospect. One was the sequel of remote mastoid surgery, another was neoplasm-related, and the third, developmental. In all, bony defects of the middle cranial fossa were associated with temporal lobe herniation. The patients were treated surgically and have had no further partial complex seizures since operation.
The authors report a 36-year-old woman with a 23-year history of simple and complex partial seizures who was treated surgically for an anteroinferior temporal encephalocele, with resolution of the seizure disorder. This patient's presentation, findings, and response to treatment are typical of those associated with anteroinferior temporal encephalocele, and different from the clinical patterns of four other types of spontaneous temporal encephalocele.
Cerebrospinal fluid (CSF) leaks involving the skull base are associated with considerable morbidity and mortality, and often present a diagnostic challenge. Current diagnostic methods are invasive and cumbersome and involve substantial radiation exposure of the patient. The authors identified seven patients with clinically suspected CSF leaks and evaluated them with a flow-sensitive magnetic resonance imaging (MRI) sequence in addition to more conventional studies. In cases with active CSF leakage, flow characteristics were documented with slow-flow and diffusion-weighted MRI. Unlike current approaches, MRI offers the advantages of rapidity, non-invasiveness, and absence of ionizing radiation. Preliminary results suggest that flow-sensitive MRI may have a role in the evaluation of CSF leaks involving the skull base and temporal bone.
Twenty-four patients with a clinical diagnosis of cerebrospinal fluid (CSF) fistulae who were assessed by MR are reported and the literature reviewed on the use of MR in the evaluation of CSF fistulae. Thirteen patients presented with rhinorrhoea, three had otorhinorrhoea and two had recurrent meningitis. Six patients had a combination of symptoms. Nine patients did not have active leakage of CSF at the time of MR scanning. The site of the CSF leak was identified in all 24 patients (100%) and was verified at operation in all patients. This study suggests that MR is a sensitive and accurate technique for detection of CSF leaks even in patients who are not actively leaking at the time of evaluation. MR imaging is a useful technique in the assessment of patients with CSF fistulae; it is non-invasive, offers excellent anatomical detail and has no radiation risk.
Disparities have been observed in both the incidences of lung and esophageal cancers and the survival of those patients. Our goals were to determine if race was associated with stage of cancer at diagnosis, and to identify predictors of advanced-stage lung and esophageal cancers.
All cases of lung and esophageal cancer between 1991 and 1995 in the Savannah River Region Information System cancer registry were studied. Data were analyzed using logistic regression to identify independent predictors of advanced disease at the time of diagnosis.
Among lung cancer patients, histology and distance to nearest hospital predicted diagnosis at an advanced stage. Residence in an area with a high proportion of Medicaid recipients was a predictor of advanced stage in esophageal cancer patients.
In this predominantly rural area, decreased utilization of health services was evident among older, poor, black, rural cancer patients. Further investigation involving prospective data collection from cancer patients is warranted.
Encephaloceles usually involve herniation of frontal lobe tissue through an anterior cranial fossa defect into the ethmoid sinus or nasal cavity. Encephaloceles can also result from temporal lobe herniation through a middle fossa defect into the sphenoid sinus. Within the sphenoid, encephaloceles are thought to occur most commonly in the central or midline aspect of the sinus. Lateral sphenoid encephaloceles, especially within the lateral aspect of the sphenoid sinus when the sphenoid sinus has pneumatized extensively into the pterygoid recess, are considered exceedingly rare. The objectives of the study were to review our experience with sphenoid encephaloceles to understand the relative frequency and the locations in which they occur within the sphenoid sinus and to report our experience in caring for patients with this condition.
Retrospective review.
Retrospective review of patient records and operative reports from 1991 to 2000. RESULTS Twelve patients were treated for intrasphenoid encephaloceles during a 10-year period. Eight patients had lesions located in the lateral recess of the sphenoid sinus. Surgical repair was undertaken in all 12 cases using endoscopic techniques. In 11 of 12 cases, the repair was successful with follow-up times of 12 to 69 months (mean follow-up, 31.9 mo).
Temporal lobe encephaloceles in the lateral sphenoid sinus have been reported rarely in the literature. Careful preoperative evaluation and localization of the sphenoid defect are critical for the selection of the optimal surgical approach for repair of the skull base defect. Our 10-year experience represents the largest group of patients treated endoscopically for intrasphenoid encephaloceles reported to date.
Encephaloceles are relatively rare phenomena produced by the protrusion of brain and dura through an anterior skull base defect. Although they can occur as congenital defects, encephaloceles can also present after trauma. The diagnosis is usually made with nasal endoscopy and imaging studies. This report reviews our recent experience repairing 5 encephaloceles in 4 patients. The diagnostic approach and the technical aspects of surgical management are discussed. Although encephaloceles are a rarity, this diagnosis should be considered as part of the differential diagnosis in evaluating a patient with a unilateral polypoid nasal mass, particularly in the setting of recurrent meningitis or cerebrospinal fluid rhinorrhea.
The suitability of the endoscopic approach for the treatment of an encephalocele of the lateral wall of the sphenoid is discussed. This is a retrospective review of 4 cases diagnosed with temporosphenoidal encephalocele and having a history of CSF leak who were surgically treated using an endoscopic endonasal approach between January 2001 and June 2002 at the Department of Otolaryngology of Sant'Orsola-Malpighi University Hospital and the Department of Neurosurgery at Bellaria Hospital in Bologna.
Three patients were female between 48 and 73 years of age (mean: 61 years). All patients had suffered from a CSF leak for 5 months to 18 years. None of the patients had a past medical history of head trauma. A fourth patient had undergone a previous microscopic approach for a previously misdiagnosed CSF leak wrongly ascribed to an empty sella. Three patients underwent an ethmoid-pterygo-sphenoidal endoscopic approach (EPSEA), while the patient who had undergone previous microscopic surgery, was treated using a transnasal transsphenoidal endoscopic approach.
The follow-up of the patients ranged from 10 to 26 months (mean: 18 months) and no case of a recurrent CSF leak was observed postoperatively.
In our report, the endoscopic approach was a useful tool for the treatment of encephaloceles of the lateral wall of the sphenoid sinus. In skilled hands, this technique permits both the resection of the encephalocele and the subsequent reconstruction of the defect also with a low rate of morbidity.
The aim of this study was to evaluate the results of conservative and surgical management options for traumatic cerebrospinal fluid (CSF) leakage complicating skull base fractures. The subjects were 81 patients who were treated between 1996 and 2003 for CSF leaks that had persisted for 24 h or longer after head injury. For each case the medical records were reviewed, and the data collected were as follows: demographic features, clinical and radiological findings, management options, complications and outcome scores. Analysis was done with patients grouped according to Glasgow coma scale (GCS) score at admission (<or=8 vs >8), and findings for three treatment methods (conservative, CSF drainage, surgery) were evaluated. In 32 cases (39.5%), the CSF leakage resolved spontaneously, and the mean hospital stay for these patients was 14+/-11 days. Twenty-four patients (29.6%) were treated by CSF drainage, and seven of these individuals ultimately required surgery to close the leak. Hospital stay was 17+/-7 days. Twenty-five patients (30.9%) underwent surgery as the initial treatment step, and the mean hospital stay for these individuals was 15+/-9 days. The large majority (74.2%) of patients with admission GCS scores <or=8 had poor outcomes. Compared with this group, a greater proportion of the CSF leaks in the patients with admission GCS scores >8 resolved spontaneously. The factors that had a critical influence on outcome in this series were level of consciousness on admission and presence of additional intracranial pathology associated with CSF leakage within cases of traumatic CSF fistulae due to skull base fractures. Treatment decisions should be dictated by the severity of neurological decline during the emergency period and the presence/absence of associated intracranial lesions. The timing for surgery and CSF drainage procedures must be decided with great care and with a clear strategy. The authors offer a treatment algorithm.
Meningiomas are the most frequently reported primary intracranial neoplasms, accounting for approximately 25% of all such lesions diagnosed in the United States. Few studies have examined the risk factors associated with a diagnosis of meningioma with two categories of exposure, hormones (both endogenous and exogenous) and radiation, most strongly associated with meningioma risk. Limited data are also available on long-term outcomes for meningioma patients, although it is clear that the disease is associated with significant morbidity and mortality. Recent legislation passed in the United States (The Benign Brain Tumor Cancer Registries Amendment Act [H.R. 5204]) mandates registration of benign brain tumors such as meningioma. This will increase the focus on this disease over the coming years as well as likely increase the reported prevalence of the disease. The increased emphasis on research dedicated to the study of brain tumors coupled with the advent of new tools in genetic and molecular epidemiology make the current era an ideal time to advance knowledge for intracranial meningioma. This review highlights current knowledge of meningioma epidemiology and new directions for research efforts in this field.
Pneumatization of the sphenoid sinus occasionally includes an extensive lateral recess creating an area beneath the temporal lobe that is relatively inaccessible to surgical intervention. Pathology in this anatomic location presents special surgical and therapeutic challenges. Recently, several authors have described the endoscopic transpterygopalatine fossa approach to this anatomic region. This approach is associated with minimal morbidity while providing direct endoscopic surgical access for managing a variety of disease processes in this region.
This study presents eight cases requiring this approach or a modification of this approach. Six patients presented with temporal lobe meningoencephaloceles with cerebrospinal fluid rhinorrhea. Each case was managed successfully through this surgical approach.
One patient experienced transient postoperative palatal anesthesia due to injury to the greater palatine nerve and another complained of ipsilateral dry eye postoperatively. Two other patients had neoplasms (inverting papilloma and chondrosarcoma), which were successfully addressed through this technique. There were no postoperative complications in these patients.
We have previously described the relationships of neural and vascular structures in this anatomic region. This article will review these relationships as they pertain to this surgical approach and will discuss the indications, techniques, and surgical outcomes in this series of patients. This approach is a valuable addition to the endoscopic armamentarium of the experienced endoscopic surgeon.
Neurosurgery International. The Mc, Graw-Hill Companies Entrapment neuropathies
- S Rengachary
- R H Wilkins
- S S Rengachary
Rengachary S, Wilkins RH, Rengachary SS. Neurosurgery International.
The Mc, Graw-Hill Companies Entrapment neuropathies; 1996. ed.
Encephalomeningocele presenting with spontaneous cerebrospinal fluid rhinorrhea in an elderly man: case report
- Dempsey