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Background: Extended endoscopic endonasal surgery (EEES) is an essential part of treatment of various pathologies of the anterior skull base. In addition to significant improvements in the quality of life of affected patients and a lower complication profile compared to open skull base surgery, the therapeutic results are comparable if the indications are correct., Materials and Methods: Data of all endoscopic endonasal skull base procedures performed at the University Skull Base Center Hamburg under the direction of the Department of Otorhinolaryngology between June 2018 and November 2022 were retrospectively collected., Results: A total of 50 cases were identified. Of these, 56% (28/50) were malignant tumors, 24% (12/50) were benign pathologies with direct skull base involvement, and 20% (10/50) were anterior skull base defects with rhinoliquorrhea. In 96% (48/50) of cases, the preoperatively set goal of surgery (representative biopsy, complete resection, closure of the skull base defect) could be achieved. Complications grade III or higher according to Clavien-Dindo occurred in 4/50 cases. During the observation period, n = 5 olfactory neuroblastomas were diagnosed, all of which were exclusively and successfully operated on endoscopically., Conclusion: In recent years, the spectrum of endoscopically resectable pathologies of the anterior skull base has steadily expanded. In particular, midline-related tumors such as olfactory neuroblastoma or iatrogenic/idiopathic skull base defects with cerebrospinal fluid rhinorrhea are treated completely endoscopically with very good results. Nevertheless, there are also limitations to this technique. Due to high variance in the scope of frontobasal surgery, the extent, and the complex anatomy, as well as the overlapping responsibilities of the specialist disciplines, establishment of certified skull base centers and bundling of frontobasal surgery at these centers is highly relevant for quality assurance., (© 2024. The Author(s).)

Cryptococcosis is caused by Cryptococcus neoformans and is fatal in children. The fungus is known to enter respiratory tract by inhalation and localizes in lungs in immunocompetent host. Patients with immunocompromised state facilitate dissemination of disease. However, disseminated cases have been described in immunocompetent HIV-negative individuals. CSF rhinorrhoea as a predisposing cause of cryptococcal meningitis has been rarely reported. We hereby describe C. neoformans directly spreading to the meninges in 1 year child due to CSF rhinorrhoea and the fungus was detected on fluid cytology., (© 2021 Wiley Periodicals LLC.)

Background: Aspiration pneumonitis is an inflammatory disease of the lungs which is difficult to diagnose accurately. Large-volume aspiration of oropharyngeal or gastric contents is essential for the development of aspiration pneumonitis. The role of cerebrospinal fluid (CSF) rhinorrhea is often underestimated as a rare etiological factor for aspiration in the diagnosis process of aspiration pneumonitis., Case Presentation: We present a case of a patient with 4 weeks of right-sided watery rhinorrhea accompanied by intermittent postnasal drip and dry cough as the main symptoms. Combined with clinical symptoms, imaging examination of the sinuses, and laboratory examination of nasal secretions, she was initially diagnosed as spontaneous sphenoid sinus meningoencephalocele with CSF rhinorrhea, and intraoperative endoscopic findings and postoperative pathology also confirmed this diagnosis. Her chest computed tomography showed multiple flocculent ground glass density shadows in both lungs on admission. The patient underwent endoscopic resection of meningoencephalocele and repair of skull base defect after she was ruled out of viral pneumonitis. Symptoms of rhinorrhea and dry cough disappeared, and pneumonitis was improved 1 week after surgery and cured 2 months after surgery. Persistent CSF rhinorrhea caused by spontaneous sphenoid sinus meningoencephalocele was eventually found to be a major etiology for aspiration pneumonitis although the absence of typical symptoms and well-defined risk factors for aspiration, such as dysphagia, impaired cough reflex and reflux diseases., Conclusions: We report a rare case of aspiration pneumonitis caused by spontaneous sphenoid sinus meningoencephalocele with CSF rhinorrhea, which can bring more attention and understanding to the uncommon etiology for aspiration, so as to make more accurate diagnosis of the disease and early surgical treatment., (© 2021. The Author(s).)

Background: Gangliogliomas are low-grade gliomas, and patients generally present with seizures. Gangliogliomas commonly occur in the temporal lobe and predominantly occur in children. Malignant progression is rare., Case Description: A 26-year-old woman presented with a history of seizures since childhood and cerebrospinal fluid rhinorrhea for the past 6 months. Generally, patients with gangliogliomas seek early medical attention, as they present with dramatic complex partial seizures. This patient experienced seizures for 10 years and never sought medical attention during this time. She did not present for medical evaluation until she developed cerebrospinal fluid rhinorrhea., Conclusions: This case gave us an opportunity to learn the natural history of ganglioglioma, although clinical presentations vary according to tumor location. This is the first case report to our knowledge of a patient with ganglioglioma presenting with cerebrospinal fluid rhinorrhea., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: Basal meningoceles are rare herniations of the meninges that tend to present unilaterally with cerebrospinal fluid rhinorrhea. Growing evidence suggests that intracranial hypertension contributes considerably to the formation of spontaneous basal meningoceles., Case Presentation: A 50-year-old man of Middle East ethnicity presented with a 16-week history of cerebrospinal fluid rhinorrhea, short-term memory loss, and slight decline in cognitive function. We present a case of bilateral spontaneous meningoceles with bone defects in the left lateral sphenoid sinus and right anterior cribriform plate, as well as with a remodeled sella. A neuronavigation-assisted expanded endoscopic endonasal surgery was performed to resect the meningoceles. Postoperative imaging demonstrated complete resolution of the bilateral meningoceles., Conclusions: This case reports the first bilateral basal spontaneous meningoceles in the literature. Furthermore, based on this case's imaging results and the literature reviewed, elevated intracranial pressure may be a determining factor behind the development of spontaneous meningoceles.

Objectives/hypothesis: Spontaneous cerebrospinal fluid (CSF) leaks are associated with increased intracranial pressure (ICP) and considered a manifestation of idiopathic intracranial hypertension. Although postoperative acetazolamide and placement of CSF shunt systems are considered valuable interventions for elevated ICP, the impact on recurrence rate remains unclear. The objective of this study was to systematically review evidence from reported literature to evaluate whether postoperative ICP management reduces recurrence rates after primary endoscopic repair., Study Design: Prospective case series and systematic review., Methods: Demographics, defect location, success rates, and ICP management in spontaneous CSF leak patients were prospectively collected over 8 years. A search was also conducted in PubMed to identify studies reporting cases of spontaneous CSF rhinorrhea., Results: Fifty-six articles with nonduplicated data were identified and combined with a prospective series of 108 patients for a total of 679 patients treated for spontaneous CSF rhinorrhea. Average age was 50.4 years with 77% female. Average body mass index was 35.8 kg/m 2 . Defects were most commonly located in the sphenoid sinus (n = 334) followed by the ethmoid (n = 318) and the frontal sinus (n = 46). Successful primary repair was 92.82% in patient cohorts where ICP evaluation and intervention with acetazolamide or CSF shunt systems was performed, but was significantly decreased to 81.87% in series with no active management of elevated ICP (P < .001)., Conclusions: Evaluation and intervention for elevated ICP in spontaneous CSF leaks is associated with significantly improved success rates following primary endoscopic repair., Level of Evidence: 4. Laryngoscope, 127:2011-2016, 2017., (© 2017 The American Laryngological, Rhinological and Otological Society, Inc.)

Objective: To Summarize and analyze the clinical characteristics and treatment of patients with spontaneous cerebrospinal fluid rhinorrhea, and to explore the related factors of recurrence., Method: Retrospective- ly analyze the clinical data of 58 patients of spontaneous cerebrospinal fluid rhinorrhea from July 2002 to July 2012, combined with its clinical characteristics, and statistically analyze the related recurrence factors., Result: Fifty-eight cases were accepted the nasal endoscopic repairment of cerebrospinal fluid rhinorrheak, follow-up 3 years, 20 cases (34.5%) recurred, 1 case recurred in half a year after operation. In the first year, there were 10 cases recurred. In the second year there were 9 cases recurred, and 1 case recurred in the third year. Through multiariable analysis it was found that higher BMI, empty sella and skull base bone defect were the independent risk factors influencing the recurrence of spontaneous cerebrospinal fluid rhinorrhea (P < 0.05)., Conclusion: The recurrence rate of spontaneous cerebrospinal fluid rhinorrhea is high, needing long-term follow-up. Higher BMI, empty sella, skull base bone defect are independent risk factors of recurrence of the disease, and the treatment should be individualized.

A 72-year-old woman was admitted for elective L4/L5 laminectomy. The operative procedure was extradural, and a Jackson-Pratt (JP) drain was placed in the tissue bed and set to wall suction during skin closure. During closure, the patient developed a 15 s period of asystole. The patient was haemodynamically stable, but was comatose for 3 days postoperatively. Cardiac enzymes and EEG were unrevealing. Head CT showed traces of subarachnoid haemorrhage and signs suggestive of cerebral anoxia. JP drain at the incision produced 170-210 mL/day of fluid, positive for β-2 transferrin, indicating cerebrospinal fluid (CSF). The patient fully returned to baseline on hospital day 10. MRI on hospital day 8 normalised. The reversible coma and radiographic findings were most consistent with acute intracranial hypotension relating to acute loss of CSF. Because radiographic findings can mimic hypoxic-ischaemic injury, acute intracranial hypotension should be considered in the differential diagnosis of postoperative coma after cranial or spinal surgery.

Brain herniations into dural venous sinuses (DVS) are rare findings recently described and their etiology and clinical significance are controversial. We describe five patients with brain herniations into the DVS or calvarium identified on MRI, and discuss their imaging findings, possible causes, and relationship to the patient's symptoms. All patients were examined with MRI including high resolution pre- and post-contrast T1- and T2-weighted sequences. With respect to brain herniations we documented their locations, signal intensities in different sequences, and size. We then reviewed clinical records in an attempt to establish if any symptoms were related to the presence of these herniations. Three males and two females were examined (age range, 11-68 years). Three patients had unilateral temporal lobe herniations into transverse sinuses, one had a cerebellar herniation into the skull, and one had bilateral temporal lobe herniations into the transverse sinuses as well as a cerebellar herniation into the sigmoid sinus. In all, the herniated brain and surrounding cerebrospinal fluid (CSF) had normal signal intensity on all MRI sequences. When correlated with clinical symptoms, brain herniations were thought to be incidental and asymptomatic in three patients and two patients had histories of headaches. Brain herniations with surrounding CSF into the DVS/skull should be considered potential sources of filling defects in the DVS. We believe that they are probably incidental findings that may be more common than previously recognized and should be not confused with the more common arachnoid granulations, clots, or tumors. Two patients had headaches, but their relation to the presence of herniated brain was uncertain.

Delayed posttraumatic cerebrospinal fluid rhinorrhea (CSFr) without meningitis is considered to be relatively rare. However, even years after trauma, recurrence or delayed onset of CSFr and meningitis due to CSFr are possible. In this article, a case of delayed CSFr from the sphenoid sinus without meningitis three years after the transfacial gunshot wound is reported. Plain high-resolution computed tomography sections through the sphenoid sinus showed a bone defect at the roof with CSF-density fluid extending into the sphenoid sinus. Arachnoid membrane herniation into the sphenoid sinus was found and site of CSF fistula confirmed during the surgery. Skull base defect was reconstructed through an endoscopic approach without any complications and the patient was followed up for 12 months without recurrence. The cause, timing, clinical course and location of CSFr make this an apparently unique case. Patients with a skull base defect without CSFr should be closely followed up and may need further evaluation or management due to the possibility of CSFr development. The positive diagnosis of a CSFr raises the matter of choosing the adequate surgical approach for its repair. Endoscopic closure of CSFr is both safe and effective.

Objective: To describe the effect on postoperative cerebrospinal fluid (CSF) leak after anterior skull base (ASB) surgery and complications associated with the addition of the vascularized nasoseptal flap (NSF) to an existing reconstruction protocol., Methods: A prospective database of all patients undergoing endoscopic ASB approaches was reviewed. Patients were divided into three groups based on the date the use of the NSF was adopted. Group A included patients with high-volume CSF leak closed using the NSF in addition to a multilayer closure. Group B included patients operated on during the same time period with no high-volume leak and no NSF. Group C included patients operated on before the adoption of the NSF with all types of CSF leak. Rates of intraoperative and postoperative CSF leak were analyzed for statistical significance., Results: Of 415 consecutive patients undergoing endoscopic ASB surgery, there were 96 in group A, 114 in group B, and 205 in group C. CSF leak rates in group A (3.1%) and group B (2.6%) were significantly lower than in group C (5.9%; P < 0.05). Lumbar drains and the gasket seal closure were performed more frequently in group A (75% and 32%) compared with group B (21% and 12%) and group C (28% and 11%). NSF carried a 2% risk of postoperative mucocele., Conclusions: The addition of NSF to an algorithm for multilayer closure can decrease the rate of postoperative CSF leak., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Background: This study was designed to determine if differences in overall skull base thickness exist in patients with spontaneous cerebrospinal fluid (SCSF) leaks and to compare our institution's 10-year experience with the endoscopic repair of these leaks to the existing literature. A retrospective cohort study was performed in a tertiary rhinologic practice., Methods: A retrospective study by two blinded independent reviewers compared the skull base thickness on computerized tomography (CT) imaging in our SCSF leak patients to nonleaking controls and patients with traumatic CSF leaks. Surgical outcomes were compared with a pooled analysis of the published literature., Results: SCSF leaks were seen most commonly in obese, middle-aged women of African American descent. SCSF leak patients were shown to have thinner skull bases in the region of the ethmoid roof, lateral lamella, and anterior face of the sella (p < 0.05) compared with patients with traumatic leaks and nonleaking controls. No racial difference in skull base thickness was observed when patients were matched according to leak type. The success rate of primary endoscopic intervention in our patients was high at 94% and comparable with the published literature., Conclusion: Differences in skull base thickness exist in SCSF patients, but whether this predisposes to such leaks or is a consequence of the underlying pathophysiology remains unknown. The endoscopic endonasal approach provides a highly effective means of repairing such leaks with success rates comparable with the endoscopic repair of other leak types. Adjuvant measures including weight reduction, lumbar drain, and acetazolamide use may increase success rates and should be considered in their management.

Background: Patients with concomitant injuries to the thorax and thecal sac requiring chest tube drainage are at risk for cerebral herniation caused by overdrainage of cerebrospinal fluid (CSF)., Case Description: A 40-year-old man presented to the trauma service awake, alert, and oriented with an isolated gunshot wound to the midaxillary line through the tenth intercostal space and a complete spinal cord injury at T12. The patient was stabilized and intubated. A chest tube was placed, and he was transferred to the neurologic intensive care unit. The patient was found to be comatose with complete absence of brainstem reflexes 3 hours after admission., Results: Magnetic resonance imaging (MRI) and autopsy revealed diffuse cerebral edema, occlusion of the bilateral posterior cerebral arteries, and tonsillar herniation extending several centimeters below the foramen magnum, with petechial hemorrhages and absence of gliosis., Conclusions: To the authors' knowledge, this case represents the first report of cerebral herniation secondary to chest tube drainage of a CSF leak caused by traumatic injury to the thecal sac., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Cerebrospinal fluid (CSF) rhinorrhoea results from a direct communication between the CSF-containing subarachnoid space and the mucosa-lined space of the nasal cavity and paranasal sinuses. We present a case of 40-year-old woman, presenting with clear, watery discharge through the right nostril spontaneously. The CT cisternography confirmed the diagnosis of sphenoid sinus CSF rhinorrhoea, with no intracranial pathology. The patient was managed by transnasal endoscopic procedure, wherein bath plug technique was followed using temporalis fascia and overlay grafting with fascia lata and fibrin glue. The patient has been symptom free for the last year.

Spontaneous cerebrospinal fluid rhinorrhoea is a rare clinical entity. The accurate localisation of the leakage site is essential for surgical planning. Imaging techniques such as high-resolution computed tomography (CT), CT cisternography and magnetic resonance cisternography in variable combinations are performed for this purpose. This pictorial essay aims to present the spectrum of imaging findings in cases of spontaneous cerebrospinal fluid rhinorrhoea, which may be useful for radiologists in the determination of the site of cerebrospinal fluid leakage.

Expanded polytetrafluoroethylene (ePTFE) porous material (GORE(®) PRECLUDE(®) Dura Substitute) does not degenerate or deteriorate in vivo, and is currently used as artificial dura mater. This material does not adhere well to the surrounding tissues, but cerebrospinal fluid leakage along the suture line has been observed in several cases. We describe a case of craniotomy for tumor resection performed 14 years after dural repair with ePTFE sheet. Histological examination of the ePTFE sheet revealed that the sheet was structurally intact, with no evidence of tissue adhesion or cellular infiltration. However, collagen deposition was observed around the suture thread. When the suture thread was removed the collagen was also removed, and the original needle hole appeared again. No significant changes were observed in the features of the ePTFE sheet even 14 years postoperatively. The formation of fibrous tissue around the needle hole was important in preventing cerebrospinal fluid leakage.

Conclusions: We found no differences in clinicopathologic factors and post-treatment effects between patients who did and did not receive lumbar drains (LDs) in the management of cerebrospinal fluid (CSF) rhinorrhea. Care should be taken in using LDs, after considering their effectiveness and complications, although routine use of an LD is not indicated in the management of traumatic CSF rhinorrhea., Objectives: We assessed the role and effectiveness of LDs in patients managed conservatively and surgically., Methods: Of 117 patients treated for CSF rhinorrhea, 61 were managed conservatively and 56 were initially managed surgically. We compared the clinicopathologic features and factors related to treatment outcome between patients who did and did not receive LDs., Results: Of 61 conservatively managed patients, 32 were managed without and 29 with LDs. There were no between-group differences in any clinicopathologic factors, recurrence rate, or incidence of meningitis during/after treatment, but duration of hospitalization differed significantly (15.3 vs 23.2 days, p = 0.03). Of 56 surgically managed patients, 24 were managed without and 32 with postoperative LDs. Except for age, there were no between-group differences in clinicopathologic factors. In terms of treatment outcome-related factors, there was no significant difference in recurrence rate, incidence of meningitis, or duration of hospitalization.

Cerebrospinal fluid leakage causing a pseudomeningocele is a well-recognized complication after spine surgery. The repair of pseudomeningocele in a symptomatic patient is usually accomplished with direct open surgical repair of the durotomy, prolonged lumbar drainage, and/or placement of an epidural blood patch. The authors highlight a unique method of pseudomeningocele repair by presenting 2 cases of adolescent girls with symptomatic lumbar pseudomeningoceles. In both cases ultrasound was used to guide the aspiration of CSF from each pseudomeningocele and to apply the epidural blood patch. Both patients had complete and immediate resolution of symptoms. The authors found ultrasound to be a useful tool to assess the extent of the CSF leakage, to determine the degree of aspiration of the extradural CSF, and to confirm the injection of the blood into the epidural space and the space created by the pseudomeningocele.

Background: This study aimed to assess the efficacy of MR images with 3D T2-weighted turbo spin-echo (3D T2-TSE) sequences for early identification of postoperative cerebrospinal fluid (CSF) leaks., Methods: We analyzed the medical records and radiological reports for 72 consecutive patients who underwent an endoscopic endonasal approach for sellar and parasellar lesions between April 2009 and December 2010. Patients were 38 men and 34 women with a mean age of 46.4 years. All underwent MR scanning within 2 postoperative days, which included 3D T2-TSE sequences as well as a conventional T2-weighted (T2W) protocol. Sequence accuracy in predicting postoperative CSF leaks was assessed for sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV)., Results: A postoperative CSF leak was confirmed in 6 of 72 patients (8.3 %). On immediate postoperative MR images, 39 patients were suspicious for CSF leaks on conventional T2W sequences, while 18 patients on 3D T2-TSE. The 3D T2-TSE imaging had superior specificity and PPV (50 % vs. 81.8 %, 15.4 % vs. 33.3 %), while there was no difference in sensitivity and NPV compared with conventional T2W sequences., Conclusion: Compared to the conventional T2W protocol, MR imaging with the 3D T2-TSE protocol provides differential images around the sellar area with improved specificity and PPV for the detection of postoperative CSF leaks.

Purpose: To present an illustrative case study of a patient with spontaneous intracranial hypotension (SIH) and to increase awareness of this condition among nurse practitioners (NPs)., Data Sources: A literature search was conducted, and deidentified patient information forms the basis of this presentation. The authors' experience and appropriate images enhance the presentation of the case study., Conclusions: SIH is a condition that typically occurs without a traumatic event, although it can be associated with minor trauma. It occurs when cerebrospinal fluid (CSF) leaks through a focal weakness in the dural sac or meningeal diverticula, resulting in CSF hypovolemia. Patients usually present with an orthostatic headache. The most common brain magnetic resonance imaging findings are diffuse pachymeningeal enhancement, descent of the cerebellar tonsils, and subdural fluid collections. Treatment options range from management of symptoms to surgical repair of the leak., Implications for Practice: As NPs continue to provide care in a variety of settings, including emergency departments and urgent care areas, they must be familiar with the progression of symptoms that might indicate SIH and be prepared to make appropriate referrals to prevent iatrogenic morbidity., (©2012 The Author(s) Journal compilation ©2012 American Academy of Nurse Practitioners.)

Background: Reconstruction of the skull base is essential to prevent postoperative leakage of cerebrospinal fluid (CSF). However, a reliable method of reconstructing the middle cranial fossa via a subtemporal keyhole is not available., Objective: To determine whether less invasive reconstruction of the middle cranial fossa under endoscopic guidance with a pedicled deep temporal fascia approach via a subtemporal keyhole is feasible and useful., Methods: The middle cranial fossa in 4 fresh cadaver heads was reconstructed with a 4-mm 0° rigid endoscope., Results: A subtemporal skin incision (subtemporal incision) was followed by 2 small skin incisions (temporal line incisions) made on the superior temporal line. The endoscope was inserted through the temporal line incisions, and then the deep temporal fascia was separated from the superficial temporal fascia and temporal muscle under endoscopic view. A pedicled flap was harvested from the subtemporal incision and applied to the middle cranial fossa after subtemporal keyhole craniotomy. The pedicled deep temporal fascial flap was flexible, long, and large enough to overlay skull base defects., Conclusion: This purely endoscopic technique using a pedicled deep temporal fascial flap provided reliable reconstruction of the middle cranial fossa through a subtemporal keyhole. This technique would also be applicable in preventing CSF leakage or treating traumatic, acquired nontraumatic, or congenital encephalocele in the middle cranial fossa.

Objective: To determine the etiology and site of leak of cerebrospinal fluid (CSF) rhinorrhea and the surgical efficacy of Functional Endoscopic Sinus Surgery (FESS), in closing the defect of CSF leak by different graft materials., Study Design: Case series., Place and Duration of Study: Department of ENT-Head and Neck Surgery, Dow University of Health Sciences, Karachi, from September 2005 to December 2008., Methodology: Clinical and pathological records of 23 patients who were diagnosed to have cerebrospinal fluid leak and managed by endoscopic approach were studied. Different graft materials alone or in combination were used endoscopically to seal the CSF leak. Results were obtained in terms of successful closure of the CSF leak, as evidenced by cessation of CSF rhinorrhea., Results: Iatrogenic trauma, due to previous surgery, was the commonest cause in (n=9, 39%) patients. Lateral lamella of cribriform plate (fovea ethmoidalis) was the commonest site of leak (n=10, 43.5%). The leak was closed solely by fat in 15 (65.2%) cases, combination of fat and middle turbinate mucosa (two layered) in 7 (30.4%) patients and temporalis fascia only in one patient (4.3%). Successful closure of the CSF leak in the first attempt was achieved in 22 (95.6%) patients., Conclusion: Success rate achieved by endoscopic approach in this study was high. However, more data is required to prove the superiority of FESS over intracranial approach for management of CSF rhinorrhea.

Objective: The objective of this study was to examine the anatomical, audiological, and surgical factors that can affect the hearing outcome in Pendred syndrome cochlear implant recipients. Patients with Pendred syndrome represent challenging cochlear implant candidates, combining goiter, severe-to-profound hearing loss, and inner-ear dysplasias. The hearing outcome and further relevant parameters in such patients have not been adequately evaluated yet., Material and Methods: Retrospective study in a tertiary referral cochlear implant centre. Data of patients with Pendred syndrome, who had undergone cochlear implantation in the 10-year period 1999-2008, were evaluated., Results: Five cochlear implant recipients with Pendred syndrome were identified, four unilateral and one bilateral. The average postoperative follow-up duration for these patients was 4.8 years. Enlarged vestibular aqueduct and Mondini dysplasia were the most common anatomical findings. Although these anatomical variances did not affect the electrode insertion, they prolonged the duration of the surgery. Cerebrospinal fluid gusher was the most common intraoperative complication. All patients enjoyed satisfactory hearing rehabilitation., Discussion: Cochlear implantation is the proper method for optimal hearing rehabilitation in patients with Pendred syndrome. Although the precise pathogenetic mechanism and the genetic background of deafness in Pendred syndrome have not been completely understood, the preexisting hearing experience represents a positive factor for satisfying hearing outcome. The inner-ear malformations can cause mild surgical difficulties and extended surgery duration. The cochlear implant surgeon should be aware of these difficulties to avoid complications.

Object: The authors aim to identify the characteristics of primary spontaneous CSF rhinorrhea and propose a hypothesis for its pathogenesis., Methods: Between 2003 and 2009, 21 patients diagnosed with primary spontaneous CSF rhinorrhea underwent surgery in the authors' hospital. The clinical aspects were retrospectively reviewed, and their characteristics were analyzed., Results: There were 18 women and 3 men, whose ages ranged from 37 to 74 years (mean 53 years). Body mass index (BMI) ranged from 22 to 58.8 kg/m(2) (mean 31.2 kg/m(2)). Eighteen patients (85.7%) were overweight, and 18 (85.7%) suffered from headache or tinnitus before rhinorrhea. Radiological images revealed fully or partially empty sellae in 14 patients (66.7%). The preoperative intracranial pressure (ICP) ranged from 11 to 28 cm H(2)O (mean 17.6 cm H(2)O), while the postoperative ICP ranged from 21 to 32 cm H(2)O (mean 25.5 cm H(2)O, p < 0.01). An endoscope-assisted transnasal approach was chosen for the repair. Postoperatively, in 95.2% of patients a cure was achieved. Rhinorrhea recurred in only 1 patient, and a leakage from a new defect occurred in another patient 4 years after the operation. Both patients underwent additional surgery, which was successful. The follow-up period varied from 5 to 75 months with a mean of 34 months., Conclusions: All patients had direct or indirect evidence of elevated ICP, most patients presented with symptoms of idiopathic intracranial hypertension (IIH), and most patients were women and obese. Primary spontaneous CSF rhinorrhea may be due to IIH, and it is a rare symptom of IIH. When treating or monitoring these patients during follow-up, ICP should be controlled, and other symptoms of IIH should be noted.

We describe the case of a 26-year-old man with orthostatic headache. Cerebral angiography revealed thrombosis in the sagittal sinus. Spine MRI showed cerebrospinal fluid collection at the C1-2 level. We performed blood patch and the symptoms disappeared. We report a rare case of intracranial hypotension caused by CSF leak and describe our hypothesis that SIH can change the velocity of cerebral blood flow and cause thrombosis.

Cervical intradural disc herniation (IDH) is a rare condition and only 25 cases of cervical have been reported. We report a 45-year-old male who presented with sudden onset right lower limb weakness after lifting heavy weight. Magnetic resonance imaging of the cervical spine showed C5/6 disc prolapse with intradural extension. The patient underwent C5/6 discectomy through anterior cervical approach. Postoperatively, the patient improved in stiffness but developed cerebrospinal fluid leak and the leak resolved with multiple lumbar punctures.

A 38-year-old woman presented with a case of post-traumatic ethmoidal cerebrospinal fluid (CSF) leak that was repaired using a purely endoscopic endonasal approach. Six weeks after a mild domestic maxillofacial trauma, she started complaining of clear, watery discharge from the left nostril and headache. Neuroimaging investigations disclosed a linear fracture of the left anterior ethmoidal roof without evidence of large bony breaches or any brain tissue damage. After conservative medical treatment with carbonic anhydrase inhibitors failed, she was referred to our hospital for surgical repair of the osteodural defect. An endoscopic endonasal transethmoidal approach was successfully performed, and an overlay technique was used to reconstruct the defect. The patient was discharged without clinical evidence of CSF rhinorrhea and no leaks were apparent at the 3-month follow-up clinical and radiological examinations. Post-traumatic CSF rhinorrhea occurs in cases of dural tears associated with small bone breaks, most frequently involving the anterior skull base. Recovery is often spontaneous following only bed rest, or with medical treatments such as inhibitors of carbonic anhydrase diuretics, steroids, or eventually stool softeners to help reduce CSF pressure. Surgical repair is required when first-line conservative treatment proves ineffective. The present case shows that the endoscopic endonasal technique for the management of CSF leaks provides a less invasive surgical route to achieve valid dural repair.

Objective: Repairing bone defects after drilling in skull base surgery is difficult in deeper locations, the posterior wall of the internal auditory canal, and the petrous apex. These defects traditionally have been repaired by soft tissue, such as fascia, muscle, or adipose tissue, coupled with lumbar drainage for several days postoperatively., Methods: To repair cerebrospinal fluid (CSF) leakage through air cells in the petrous bone, we applied a multilayer technique using hydroxyapatite cement (HAC) in three patients with postoperative CSF leakage. The opening of air cells was drilled widely to visualize the whole site of the opening and then packed with HAC covered with dura mater, fat, and fibrin glue., Results: In all patients, CSF leak did not recur after repair with HAC, and there was no postoperative would infection, local inflammation, or cranial nerve damage., Conclusion: HAC is an effective biomaterial for the repair of refractory CSF leakage due to the opening of air cells in deeper surgical fields such as the petrous bone., (Copyright © 2010 Elsevier Inc. All rights reserved.)

Object: The object of this study was to assess the efficacy and complications of endoscopic management of anterior skull base defects., Method: The authors reviewed the medical records of 28 children (20 boys and 8 girls) undergoing endoscopic repair of anterior skull base defects in their tertiary referral center between 2001 and 2008; 18 cases were congenital and 10 cases posttraumatic. During the endoscopic procedure, rigid telescopes--2.7 or 4 mm in diameter, with 0° or 30° lenses--were used. In 23 patients the anterior skull base defect was sealed with fragments of middle turbinate (bone and mucosa). In the remaining 5 patients it was sealed with cartilage harvested from the nasal septum (3 cases) or from the auricle (2 cases), fibrin glue, and oxidized cellulose. A combined external and endoscopic approach was required in 3 cases because of the size and extensions of the encephalocele. Outcome was primarily assessed by means of clinical examination, nasal fibroscopy, and imaging., Results: The mean duration of follow-up was 26.7 months (range 9-57 months). One patient treated by a combined approach died of meningitis 2 years after surgery. In the remaining 27 patients, there was no recurrence of CSF leak, meningitis, or encephalocele. An iatrogenic frontal or ethmoidal mucocele was observed in 4 cases., Conclusions: The endoscopic approach is a minimally invasive, safe, and efficient technique for removing nasal encephaloceles in children.

Objectives/hypothesis: Spontaneous nasal cerebrospinal fluid (CSF) fistula represents a rare clinical entity. The possible etiology and the localization of the rhinorrhea remain an ongoing clinical challenge. The purpose of this study was to evaluate the localization of spontaneous CSF fistula and to correlate it with anatomical studies., Study Design: Retrospective clinical study, prospective anatomical study., Methods: Twenty-nine patients with spontaneous CSF rhinorrhea were retrospectively studied, 10 males and 19 females. Ages ranged from 10 to 92 years (mean, 50 years). In addition, 48 human skulls from newborns to adults were examined for the postnatal development of the anterior and middle cranial fossa., Results: In our study isolated cribriform plate defects were found in four patients. The lateral lamina of the ethmoid bone showed defects in three patients. In nine patients the bony defect could be found in the region of the fovea ethmoidalis. The bony defect between the extra- and intracranial space was found in the lateral recess of the sphenoid sinus in eight patients. Five patients had special sites (e.g., supraorbital recess and frontal recess)., Conclusions: This study supports the theory that bony dehiscence in the lateral lamina of the ethmoid bone can be congenital and can also be spontaneously acquired later. The bony dehiscence in the lateral wall of the sphenoid sinus can only develop during pneumatization.

Objective: Cerebrospinal fluid (CSF) rhinorrhea is leakage of CSF from the nasal cavity caused by cranial base or meningeal defects. Surgical treatment of CSF rhinorrhea is still problematic. We evaluated the clinical outcomes of 132 consecutive cases of CSF rhinorrhea treated via transcranial or transnasal endoscopic approaches according to the patient's condition. The indications for the approaches are discussed., Methods: Of 132 patients with CSF rhinorrhea, a transnasal endoscopic approach was used in 98 to repair cranial base defects in the ethmoid and sphenoid sinuses. A transcranial intradural approach was used in the remaining 34 patients for frontal sinus defects, multiple fractures of the cranial base, or combination nerve injury., Results: CSF rhinorrhea resolved after initial surgery in 124 of 132 patients, giving a success rate of 94%. Of the 8 failures or recurrent cases, 4 were successfully repaired by repeat endoscopic surgery, 2 were cured by transcranial revision surgery, and 2 refused additional surgery (the condition subsequently resolved without treatment in these patients). Postoperative complications included intracranial infection (8 patients) and anosmia (1 patient). No neurological deficits were apparent over the 10-month mean follow-up period., Conclusion: Transnasal endoscopic repair is a reliable method for CSF rhinorrhea patients whose fistulae are located in the ethmoid and sphenoid sinuses. The transcranial procedure should be the treatment of choice for patients with frontal sinus fracture, multiple or complex anterior cranial base fractures, or nerve injury. A satisfactory surgical outcome depends on exact diagnosis, proper operative approach, and the surgeon's skill and experience.

Background: Craniopharyngioma has two subtypes: adamantinomatous and squamous-papillary. Squamous-papillary craniopharyngioma may develop from remnants of the craniopharyngeal duct, anterior pituitary cells with squamous metaplasia, suprasellar epidermoid cyst, or Rathke cleft cyst., Aim: While ciliated craniopharyngioma is considered to represent a transitional stage between Rathke cleft cyst and squamous-papillary craniopharyngioma, ciliated craniopharyngioma following Rathke cleft cyst at the same site has not previously been described., Results: We report a case of ciliated craniopharyngioma developing from Rathke cleft cyst., Conclusion: The clinical course for this case is discussed together with a review of the pathological literature for ciliated craniopharyngioma.

Background: The frequency of spontaneous CSF rhinorrhea in macroprolactinoma patients is poorly documented and was previously thought to be a very rare occurrence., Methods: Thirty patients with macroprolactinomas (>1.0 cm diameter) identified from the Swansea neuroendocrine database were studied retrospectively., Results: At presentation, the median serum prolactin was 28,354 (range, 1844 to >6,000,000) mU/L; radiologically, 4 adenomas were invading the cavernous sinus, one the sphenoid sinus and 5 both the cavernous and sphenoid sinus. After commencement of medical therapy, 4 subjects developed CSF rhinorrhea requiring surgical correction; all 4 had tumors invading both the cavernous and sphenoid sinus and an initial serum prolactin more than 75,000 mU/L. One subject developed an acute psychosis, and a man who presented with delayed puberty committed a serious sexual offense., Conclusions: Serious adverse effects are not uncommon in medically treated macroprolactinoma patients. Subjects with adenomas invading the sphenoid sinus have a high risk of developing CSF rhinorrhea that requires neurosurgical intervention.

Background: The CSF rhinorrhea is a kind of common clinical disease. The preoperative diagnosis and intraoperative localization of CSF fistulas are critical to treatment of CSF rhinorrhea. At present, intrathecal fluorescein regarding endoscopic transnasal cerebrospinal leakage repair is a common method for localization of the fistula; however, it has some disadvantages because it needs a specific endoscope, and the trauma from lumbar puncture as well as the potential complications of intrathecal fluorescein, as a result, widely limited its clinical use. Topical intranasal fluorescein can avoid the above-mentioned shortcoming. The aim of this work was to describe the use of topical fluorescein in the intraoperative localization of CSF fistulas and to screen its use in preoperative diagnosis of CSF rhinorrhea., Methods: Fifteen patients with CSF rhinorrhea were treated with an endoscopic endonasal technique. Topical intranasal 5% fluorescein for preoperative diagnosis and intraoperative localization of the site of the leak was placed in middle turbinate meatus, the roof of the ethmoid plate, and sphenoethmoidal recesses. A change in the color of the fluorescein from brown to green fluorescence denoted the presence of CSF, and the site of the leak could be traced. The accuracy rate of diagnosis and leak site identification was made by comparison with glucose analysis, intraoperative findings, and follow-up., Results: The cause of the leak was accidental trauma in 5 patients and spontaneous in 10 patients. The preoperative use of fluorescein-soaked cotton was 100% accurate in diagnosing CSF rhinorrhea. The intraoperative use of topical intranasal fluorescein was also 100% accurate in locating the site of the CSF fistula when compared with the surgical findings. It had achieved 100% success rate in sealing the CSF fistulas in our 15 patients with no recurrence detected during the follow-up period (mean, 2-24 months). No complications have been reported., Conclusion: In the presence of a clinically diagnosed CSF leakage and location of the leakage fistula, topical fluorescein is a very easy, sensitive, safe, and highly accurate tool in the intraoperative localization of the site and extent of CSF fistulas. It should be considered as a viable noninvasive alternative to intrathecal fluorescein.

Purpose: A comprehensive classification based on high resolution computed tomography (CT) of the whole craniofacial region was correlated with clinical findings of combined skull base and maxillofacial fractures., Material and Methods: In a study of two clinical centres, 70 patients with such injuries were admitted at the Universities of Basel (n=29) and Uppsala (n=41). Clinical signs (rhinorrhoea, periorbital haematoma and pneumencephalus) and surgical versus conservative treatment were correlated with a cranio-maxillofacial injury severity score (CMF-ISS) calculated from the classification system. Fracture classifications were decided in consensus on the basis of CT and semiautomatic classification software. The classification system defined 3 fracture types (A, B, C), 3 groups (A1, A2, A3), and 3 subgroups (A1.1, A1.2, A1.3) with increasing severity from A1.1 (lowest) to C3.3 (highest)., Results: Of 70 patients, 43 were operated upon and 27 conservatively treated. The operated patients had significantly higher severity scores than non-operated. Patients with or without periorbital haematoma do not differ significantly in the severity score. The severity of the CMF-ISS score was significantly associated (two sample T-test P<0.01) with the occurrence of pneumencephalus, rhinorrhoea and treatment approach., Conclusion: Based on our present results, this system seems to be clinical useful for operative decisions and interventions.

Background: Various forms of intracranial air have been described in the literature., Aim: This report aims to present clinical, radiological and intraoperative findings of a rare intracranial air entrapment case after endoscopic sinus surgery.

A 3-year-old girl presented with a transethmoidal meningoencephalocele manifesting as recurrent rhinorrhea. Initially, she developed meningitis, but after treatment she experienced rhinorrhea. Two months later, she again presented with rhinorrhea. Neuroimaging studies revealed a small protrusion (15 mm x 10 mm) at the roof of the ethmoidal sinus. Nasal endoscopy confirmed the diagnosis of meningoencephalocele. The operative findings revealed a small hole in the left olfactory bulb, which had descended into an enlarged foramen along with the arachnoid membrane. The left olfactory bulb was removed, and the enlarged foramina of the lamina cribrosa were covered with a frontal pericranial flap. The defect in the bone was very small, but contributed to the development of meningitis and leakage of the cerebrospinal fluid. Basal cephalocele should be considered in a patient with recurrent rhinorrhea and intracranial infections, even in the absence of any apparent anomaly.

Background: Sternberg's (lateral craniopharyngeal) canal was originally described in anatomic studies as a membranous space in the lateral wall of the sphenoid sinus. The canal has been etiologically associated with lateral sphenoid sinus "spontaneous" cerebrospinal fluid (CSF) leaks. A patent Sternberg's canal has been reported in up to 4% of adults, and persistent vestiges have been reported in up to 30%. However, no modern studies analyzing high-resolution CT scans have been published., Methods: A consecutive analysis of 1000 high-resolution CT scans of sphenoid bones was performed. Scans were analyzed for a lateral recess, bony defects, arachnoid pits, and holes possibly representing Sternberg's canal. Data were compared with a case series of lateral sphenoid CSF leaks., Results: Average patient age was 38.7 years (10-92 years). A sphenoid lateral recess was present in 35.3% (17.4% bilateral) of cases. Arachnoid pits were present on the floor of the middle cranial fossa in 23.4% of cases. Seven skull base defects were identified. Only one traveling medial to V2 resembled the description of Sternberg's canal. In contrast, a case series of 25 patients with lateral sphenoid sinus CSF leaks all had a lateral recess, defects, and arachnoid pits lateral to V2 (p < 0.00001)., Conclusion: Sternberg's canal as historically defined is not nearly as prevalent as previously reported. Furthermore, the presence of arachnoid pits in all sphenoid CSF leaks and the predominant leak location lateral to the sites of fusion of ossification centers suggests that the leaks are acquired. Contributing factors may include arachnoid pits/weaknesses in the skull base and intracranial hypertension.

Basal encephalocoeles are rare congenital abnormalities of the skull base. The authors describe a rare case of spontaneous CSF leak and meningitis secondary to an intra-sphenoidal encephalocoele. Initial endoscopic transsphenoidal repair was unsuccessful, necessitating a combined subtemporal and transsphenoidal approach to close the defect.

Acquired non-traumatic frontal sinus encephaloceles are very rare lesions that are usually caused by a tumour or hydrocephalus. We present a 31-year-old woman with a frontal sinus encephalocele who developed rhinorrhoea after a ventriculo-peritoneal shunt to treat her hydrocephalus and underwent radiotherapy for a tectum tumour.

Primary spontaneous cerebrospinal fluid (CSF) rhinorrhea is rare. Only two such cases with a defect in the clivus have been previously reported in the world literature. The purpose of this report is not to discuss the operative approach or results but the etiopathogenesis of the defect at this very rare site. One patient had a defect in the posterior wall of the sphenoid sinus, just caudal to the dorsum sellae. In the other, a psuedomeningocele (thickened arachnoid outpouching) was found protruding into the sphenoid sinus through a defect in the middle of the clivus. Both cases were successfully managed with transsphenoidal surgery. Two additional cases of primary spontaneous CSF rhinorrhea through a defect in clivus are reported. The possible mechanism of the fistulae are discussed.

Objective: Meticulous sealing of opened air cells in the petrous bone is necessary for the prevention of cerebrospinal fluid (CSF) fistulae after vestibular schwannoma surgery. We performed a retrospective analysis to determine whether muscle or fat tissue is superior for this purpose., Methods: Between January 2001 and December 2006, 420 patients underwent retrosigmoidal microsurgical removal by a standardized procedure. The opened air cells at the inner auditory canal and the mastoid bone were sealed with muscle in 283 patients and with fat tissue in 137 patients. Analysis was performed regarding the incidence of postoperative CSF fistulae and correlation with the patient's sex and tumor grade., Results: The rate of postoperative CSF leak after application of fat tissue was lower (2.2%) than after use of muscle (5.7%). Women had less postoperative CSF leakage (3.4%) than men (5.6%). There was an inverse correlation with tumor grade. Patients with smaller tumors seemed to have a higher rate of CSF leakage than those with large tumors without hydrocephalus. Only large tumors with severe dislocation of the brainstem causing hydrocephalus showed a higher incidence of CSF leaks., Conclusion: Fat implantation is superior to muscle implantation for the prevention of CSF leakage after vestibular schwannoma surgery and should, therefore, be used for the sealing of opened air cells in cranial base surgery.

Objective: Patients undergoing nasal septoplasty can face many complications. Some of these complications are rare but their results are life threatening. Being aware of this complication could prevent further problems such as enlargement of the bone and dural defect, herniation of the meninges and brain tissue through the defect by pulsation of the brain and ascending infection. With early diagnosis, a less aggressive method could be used to treat this complication., Case Description: A 50-year-old woman was admitted our hospital with the complaint of loss of consciousness. Her Glasgow coma score was 7 on admission. She had no lateralizing signs, but had nuchal rigidity. Blood pressure was 200/110mm Hg, the respiratory pattern was apneic, complete blood count revealed 12000 leucocytes/mm3 and arterial blood gases showed respiratory acidosis and other biochemical parameters were within normal limits. Computerized cranial tomography (CCT) showed diffuse brain edema without evidence of other signs. Lumbar puncture was performed revealing purulent and highly viscous cerebrospinal fluid (CSF)., Conclusion: Late diagnosis and late repair of arachnoidodural tearing could lead to life-threatening complications, and cases with meningitis and larger defects may require more extensive surgery instead of transnasal endoscopic repair.