Management of recurrent cerebrospinal fluid rhinorrhea caused by sequential, anatomically separated skull base defects - A case-based systematic review.

OBJECTIVE: Recurrent cerebrospinal fluid (CSF) rhinorrhea caused by sequential, anatomically separated skull base defects are rarely reported in the literature. Neither management nor etiology are sufficiently investigated. We herein present an illustrative case and a systematic review of the literature regarding etiology, diagnostics, and management of this rare phenomenon. METHODS: A systematic literature search looking for articles reporting sequential CSF-leaks with multiple skull base defects was performed. Data from included articles was descriptively reported, the quality of the included studies was assessed with GRADE. RESULTS: A 71-year-old female patient with posttraumatic rhino- and left-sided otorrhea due to a left-sided longitudinal fracture of the petrous bone presented at our institution. After initial surgical repair and a ten-week symptom-free interval, CSF-rhinorrhea reoccurred. Imaging review revealed a pre-existing contralateral meningoencephalocele of the lateral sphenoid recess causing recurrent CSF-rhinorrhea most likely after initial traumatic laceration. The defect was successfully treated. Literature search identified 366 reports, six of which were included in the systematic review with a total of ten cases. Quality was deemed good in 8/10 cases. The most common location for primary and sequential CSF-leaks was along the sphenoid bone (4/10 and 5/10 patients, respectively). All publications except one reported the presence of a meningo(encephalo)cele as cause of the sequential CSF-leak. CONCLUSION: Occurrence of recurrent CSF-rhinorrhea due to an anatomically separated sequential skull base lesion remains a rare yet described phenomenon. Reassessment of imaging studies and a structured diagnostic work-up to detect sequential CSF-leaks independent of the primary lesion should therefore be considered.

Endoscopic Management of a Case of Spontaneous Cerebrospinal Fluid Leaks in Anterior Skull Base.

Spontaneous cerebrospinal fluid (sCSF) leaks are rare, and their diagnosis and treatment often present significant challenges. This paper discusses and reports cases experienced at our facility. We retrospectively reviewed three of five cases of sCSF leaks experienced at the Department of Otolaryngology and Head and Neck Surgery, Kyushu University, from December 2020 to December 2022, excluding CSF otorrhea. All three patients were female; their mean age was 56 years (44-71 years). Two of the three patients were obese (first degree), and one was average weight (according to the criteria of the Japan Society for the Study of Obesity). Two patients had hypertension, and one had sleep apnea syndrome as an underlying disease. In all cases, leakage sites, which were all the cribriform plate, can be endoscopically identified, and all could be closed by an endoscopic intranasal approach. We reviewed cases of sCSF leaks. Although some patients had difficulty identifying the leakage site in a narrow and complex nasal cavity, an endoscopic survey was useful in identifying the leakage site. All cases were closed and there were no signs of recurrence. Identifying the site of leakage and selecting the appropriate closure method depending on the extent of the leakage is essential in treating such cases.

Clinical application of transcranial neuroendoscopy combined with supraorbital keyhole approach in minimally invasive surgery of the anterior skull base.

To explore the techniques, safety, and feasibility of minimally invasive neurosurgery through the supraorbital eyebrow arch keyhole approach by neuroendoscopy. Retrospective analysis of clinical data of patients with various cranial diseases treated by transcranial neuroendoscopic supraorbital eyebrow keyhole approach in our hospital from March 2021 to October 2023. A total of 39 complete cases were collected, including 21 cases of intracranial aneurysms, 9 cases of intracranial space occupying lesions, 5 cases of brain trauma, 3 cases of cerebrospinal fluid rhinorrhea, and 1 case of cerebral hemorrhage. All patients' surgeries were successful. The good prognosis rate of intracranial aneurysms was 17/21 (81%), and the symptom improvement rate of intracranial space occupying lesions was 8/9 (88.9%). Among them, the initial symptoms of one patient with no improvement were not related to space occupying, while the total effective rate of the other three types of patients was 9/9 (100%). The average length of the craniotomy bone window of the supraorbital eyebrow arch keyhole is 3.77 ± 0.31 cm, and the average width is 2.53 ± 0.23 cm. The average postoperative hospital stay was 14.77 ± 6.59 days. The average clearance rate of hematoma by neuroendoscopy is 95.00% ± 1.51%. Our results indicate that endoscopic surgery through the supraorbital eyebrow arch keyhole approach is safe and effective for the treatment of anterior skull base lesions and cerebral hemorrhage. However, this retrospective study is a single center, small sample study, and the good surgical results do not exclude the subjective screening of suitable patients by clinical surgeons, which may have some bias. Although the clinical characteristics such as indications and contraindications of this surgical method still require further prospective and multicenter clinical research validation, our study still provides a new approach and choice for minimally invasive surgical treatment of anterior skull base lesions.

Spontaneous Cerebrospinal Fluid Rhinorrhea With Meningoencephalocele Recurrence After Placement of a Lumboperitoneal Shunt: A Case Report.

Cerebrospinal fluid rhinorrhea associated with meningoencephalocele is usually treated surgically. During the perioperative period, cerebrospinal fluid diversion may be employed to control intracranial pressure, but there are few indications for this method. A 51-year-old female presented with cerebrospinal fluid rhinorrhea associated with meningoencephalocele and underwent surgical repair followed by the placement of a lumboperitoneal shunt. However, cerebrospinal fluid leakage recurred, requiring a second surgery. Lumbar drainage effectively controls intracranial pressure, but it does not cure bone defects. The use of these devices should be carefully considered based on the patient's condition.

Comparative analysis of transnasal endoscopic reconstruction techniques for managing cerebrospinal fluid rhinorrhea in skull base defects.

The nasal skull base is located into the deep position of nasal cavity and closely related to important nerves and vessels. The complete removal of tumors in this area poses a complex surgical challenge.In order to investigate the clinical efficacy of utilizing free middle turbinate mucosa (FMT), fascia lata, and pedicled nasal septum flap (known as the Hadad-Bassagasteguy flap, HBF) for the treatment of cerebrospinal fluid (CSF) rhinorrhea, a retrospective analysis was conducted on clinical data from 65 patients who underwent skull base reconstruction following endoscopic resection of nasal-skull base tumors. The selection of the repair material was based on the size and location of the defect. For defects less than 1.5 cm (n = 24), FMT was chosen, while for defects greater than or equal to 1.5 cm (n = 16), HBF was preferred. In cases where HBF was not available or not suitable (specifically, when the defect was located on the posterior wall of the frontal sinus), fascia lata was selected (n = 25). The repair outcomes of all 65 patients were summarized, and subsequently, a comparison was made between the use of fascia lata and HBF. The overall success rate for one-time repairs was 93.8 %. Specifically, the success rates for repairs using FMT, fascia lata, and HBF were 91.7 %, 96.0 %, and 93.8 %, respectively. Throughout the follow-up period, there were 2 cases of postoperative CSF leakage out of 24 patients who underwent FMT reconstruction, 1 case out of 25 patients who underwent fascia lata reconstruction, and 1 case out of 16 patients who underwent HBF reconstruction. The occurrence of postoperative complications, such as intracranial infection, lung infection, and epistaxis, was observed in both the fascia lata group and the HBF group. However, there were no statistically significant differences between the two groups. The transnasal endoscopic reconstruction of skull base defect using HBF, fascia lata, and FMT demonstrated satisfactory repair effects in managing CSF rhinorrhea. Generally, FMT has been found to be a dependable repair material for small defects measuring less than 1.5 cm, while in the case of larger defects equal to or exceeding 1.5 cm, both HBF and fascia lata can be utilized with comparable repair outcomes. The selection of fascia lata becomes a viable option when HBF is unavailable or not suitable.

Diagnostic accuracy of intrathecal fluorescein versus other radiological modalities in evaluating non-congenital skull base defects: a systematic review and meta-analysis.

PURPOSE: The intraoperative detection of cerebrospinal fluid (CSF) leaks during endoscopic skull base surgery is critical to ensure watertight sealed defects. Intrathecal fluorescein (ITF) is a valuable adjunct to intraoperative investigation. Hence, our aim is to summarize the evidence of the efficacy of ITF as an accurate diagnostic modality and reconstruction guide for non-congenital skull base defects. METHODS: Using the Cochrane Central, MEDLINE, and Embase databases, we identified studies involving the use of ITF in non-congenital CSF leaks which were published until November 2023. The STATA 18 software was used for meta-analysis. RESULTS: Fourteen studies met the inclusion criteria, in which seven studies were included in the meta-analysis. ITF was used in 1898 (90.3%) of patients, with a detection rate of 88.1%. The overall detection rate of non-congenital CSF leaks among ITF concentrations of 5% and 10% had a statistically significant pooled effect size of 2.6 (95% CI = 2.25, 2.95), while when comparing the ITF to other alternative radiological tests, it was not statistically significant with a mean difference of 0.88 (95% CI = - 0.4, 2.16). Moreover, the pooled prevalence was statistically significant in regards of the complications associated with ITF with an effect size of 0.6 (95% CI = 0.39, 0.82), indicating that 60% of patients who underwent ITF would experience at least one of the measured complications. CONCLUSION: ITF is considered as an efficient tool in localizing skull base defects. However, there was no significant results when comparing the ITF to other alternative radiological tests. Accordingly, if the ITF intervention is indicated, patients should be carefully selected based on their clinical need.

Management of cerebrospinal fluid rhinorrhea as a rare complication of antiphospholipid syndrome.

Background: Antiphospholipid syndrome is a complex autoimmune condition associated with the formation of recurrent thrombosis in any vascular bed throughout the body. Jugular vein thrombosis is very rare with only a 0.9% occurrence and is not typically associated with cerebrospinal rhinorrhea as a result of raised intracranial pressure. Case Description: A 54-year-old patient presented with a 9-month history of cerebrospinal fluid (CSF) rhinorrhea and headache on a background of antiphospholipid syndrome. Investigations showed a superior vena cava (SVC) and right internal jugular vein (IJV) obstruction with moderately elevated intracranial venous pressures. Her magnetic resonance imaging (MRI) brain was consistent with a CSF leak. The patient underwent successful endovascular stenting of her obstructed SVC and right IJV followed by surgical repair of a herniating meningocele in the posterior left ethmoid air cells. Conclusion: CSF rhinorrhea is uncommon and never previously reported associated with SVC thrombosis induced by antiphospholipid syndrome. A combination of endovascular techniques and surgical repair is recommended for this challenging presentation.

Management of frontoethmoidal osteoma causing pneumocephalus and cerebrospinal fluid leakage with minimally invasive techniques: illustrative cases.

BACKGROUND: Osteoid osteoma is a common benign bone tumor frequently seen in the frontoethmoid region. However, involvement of the skull base is rare, with few cases previously reported. OBSERVATIONS: The authors report two cases of spontaneous, symptomatic frontoethmoidal osteoma: one presented with neurological deficit secondary to tension pneumocephalus and the other with cerebrospinal fluid leakage. The first case was managed with a transfrontal sinus craniotomy and pneumocephalus decompression with osteoma resection and skull base reconstruction. The second case was managed with a uninaral endoscopic endonasal approach to the anterior skull base with osteoma resection and reconstruction. LESSONS: Given the paucity of cases with associated tension pneumocephalus described in the literature, it was relevant to describe the authors' experience with surgical decision-making and the expected outcomes among patients with this pathology when using minimally invasive techniques.

Spontaneous rhinorrhea and idiopathic intracranial hypertension: a complex and challenging association.

INTRODUCTION: Spontaneous CSF leak is a known complication of idiopathic intracranial hypertension (IIH). Patients with CSF rhinorrhea present a unique challenge within the IIH population, as the occurrence of a leak can mask the typical IIH symptoms and signs, complicating the diagnosis. Treatment of leaks in this population can also be challenging, with the risk of rhinorrhea recurrence if intracranial hypertension is not adequately treated. OBJECTIVE: The aim of this narrative review was to examine current literature on the association between spontaneous CSF rhinorrhea leaks and IIH, focusing on key clinical features, diagnostic approaches, management strategies, and outcomes. MATERIAL AND METHODS: A literature search was executed using the PubMed and Scopus databases. The search was confined to articles published between January 1985 and August 2023; extracted data was then analysed to form the foundation of the narrative review. RESULTS: This search yielded 26 articles, comprising 943 patients. Average age was 46.8 ± 6.5 years, and average body mass index was 35.8 ± 4.8. Most of the patients were female (74.33%). Presenting symptoms were rhinorrhea, headaches and meningitis. The most common imaging findings were empty sella and encephalocele. The standard treatment approach was endoscopic endonasal approach for correction of CSF rhinorrhea leak, and shunt placement was also performed in 128 (13%) patients. Recurrences were observed in 10% of cases. CONCLUSIONS: The complex relationship between spontaneous CSF leaks and IIH is a challenge that benefits from multidisciplinary evaluation and management for successful treatment. Treatments such as endoscopic repair, acetazolamide, and VP/ /LP shunts reduce complications and recurrence. Personalised plans addressing elevated intracranial pressure are crucial for successful outcomes.

Non-Epstein-Barr Virus Sinonasal Undifferentiated Carcinoma Presenting as Diplopia and Rhinorrhea.

Sinonasal undifferentiated carcinoma (SNUC) is an extremely rare and highly aggressive malignant neoplasm of the nasal cavity and/or paranasal sinuses. SNUC is clinicopathologically distinctive from other tumors but is difficult to study due to its low incidence. There is also very little consensus about the etiology of SNUC, including its association with Epstein-Barr virus (EBV). Treatment modalities include surgery, chemotherapy, and radiation depending on the stage and grading. Herein, we discuss a patient who presented to the emergency department with chronic rhinorrhea and various ophthalmologic symptoms such as flashes, floaters, and diplopia. The patient was later diagnosed with SNUC in the setting of negative serological testing for EBV in addition to his previously concomitant history of bladder cancer. The purpose of this case report is to contribute to the broader literature of SNUC and the specifics surrounding the diagnostic modalities utilized, management, and outcome of non-EBV sinonasal undifferentiated carcinoma in a patient with atypical symptomatology.

A Safe Technique for Excising the Perpendicular Plate of the Ethmoid Bone in Patients with Crooked Nose: A Finite Element Analysis.

BACKGROUND: Correction of the crooked nose, especially the perpendicular plate of the ethmoid bone, has the potential to cause skull base injury. At present, the safe and effective method for perpendicular plate resection has not been clearly defined through biomechanics. METHOD: CT scan data of 48 patients with crooked nose and deviated nasal septum were divided into C-type, angular deformity-type, and S-type based on the morphology of the 3D model. Different types of finite element models of the nasal bony septum and skull base were established. The osteotomy depth, angle, and force mode of the PPE resection were simulated by assembling different working conditions for the models. The von Mises stress of the anterior cranial fossa was observed. RESULTS: When the osteotomy line length was 0.5 cm, the angle was at 30° to the Frankfurt plane, and 50 N·mm torque was applied, the von Mises stress of the skull base was minimal in the four models, showing 0.049 MPa (C-type), 0.082 MPa (S-type), 0.128 MPa (angular deformity-type), and 0.021 MPa (control model). The maximum von Mises stress values were found at the skull base when the osteotomy line was 1.5 cm, the angle was 50°, and the force was 10 N along the X-axis, showing 0.349 MPa (C-type), 0.698 MPa (S-type), 0.451 MPa (angular deformity-type), and 0.149 MPa (control model). CONCLUSION: The use of smaller resection angle with the Frankfurt plane, conservative resection depth, and torsion force can better reduce the stress value at the skull base and reduce the risk of basicranial fracture. It is a safe and effective technique for perpendicular plate resection of the ethmoid bone in the correction of crooked nose. LEVEL OF EVIDENCE IV: This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .

Reliability of High-resolution Gadolinium-enhanced MR Cisternography and Gasket-seal Technique for Management of Anterior Skull Base Defects.

PURPOSE: Precise preoperative localization of anterior skull base defects is important to plan surgical access, increase the success rate and reduce complications. A stable closure of the defect is vital to prevent recurrence of cerebrospinal fluid (CSF) rhinorrhea. The purpose of this retrospective case series was to evaluate the reliability of a new high-resolution gadolinium-enhanced compressed-sensing SPACE technique (CS T1 SPACE) for magnetic resonance (MR) cisternography to detect cerebrospinal fluid leaks of the anterior skull base and to assess the long-term success rate of the gasket-seal technique for closure of skull base defects. METHOD: All patients with spontaneous or postoperative cerebrospinal fluid rhinorrhea and defects of the anterior skull base presenting to the Departments of Otorhinolaryngology and Neurosurgery between 2019 and 2020, receiving a computed tomography (CT) cisternography and MR cisternography (on a 3T whole-body MR scanner using a 64-channel head and neck coil) with CS T1 SPACE sequence and closure of the defect with the gasket-seal technique, were enrolled in the study. For the cisternography, iodinated contrast agent (15 ml Solutrast 250 M®), saline (4 mL) mixed with a 0.5 mL of gadoteridol was injected into the lumbar subarachnoid space. RESULTS: A total of four patients were included in the study and MR cisternography with CS T1 SPACE sequence was able to precisely localize CSF leaks in all patients. The imaging results correlated with intraoperative findings. All defects could be successfully closed with the gasket-seal technique. The mean follow-up was 35.25 months (range 33-37 months). CONCLUSION: MR cisternography with CS T1 SPACE sequence could be a promising technique for precise localization of CSF leaks and the gasket-seal technique resulted in good closure of the CSF fistula in this case series.

Frontal Meningoencephalocele With Cerebrospinal Fluid Rhinorrhea: A Case Report.

Traumatic brain injuries are a significant public health concern often associated with immediate consequences. However, delayed complications can manifest, including rare congenital neural tube defects such as encephaloceles. We present a case of a 45-year-old male with a history of traumatic brain injuries who developed a posttraumatic frontal meningoencephalocele associated with cerebrospinal fluid rhinorrhea. This case emphasizes the need for vigilance in assessing patients with a history of head trauma for delayed complications, even years after the initial injury. Early diagnosis and intervention can significantly impact outcomes.

How I do it? Endoscopic endonasal transpterygoid repair of sphenoid lateral recess cerebrospinal fluid leak after previous unsuccessful transcranial surgery.

BACKGROUND: Direct access to the sphenoid lateral recess offers the best chance of sealing spontaneous cerebrospinal fluid (CSF) rhinorrhea caused by lateral sphenoid encephaloceles of the Sternberg canal defect. METHOD: We present a case of spontaneous left-sided sphenoid lateral recess CSF leak after previous unsuccessful transcranial surgery managed with an endoscopic endonasal transpterygoid approach (EETA). An anatomical-based step-by-step illustration of the EETA was presented in the surgical video. CONCLUSION: This case demonstrates the value of endoscopic endonasal transpterygoid corridor in the exposure and manipulation of the sphenoid lateral recess.

The Etiology, Diagnosis, and Management of Cerebrospinal Fluid Rhinorrhea: A Tertiary Center Experience.

Introduction The aim of the present study was to describe our institution's nine years of experience in the endoscopic endonasal management of cerebrospinal fluid (CSF) rhinorrhea and to discuss the causes, sites, and outcomes.  Methodology The medical records of patients diagnosed with CSF rhinorrhea in King Abdulaziz Medical City-Jeddah (KAMC-J) between 2014 and 2023 were retrospectively reviewed, and all relevant information including body mass index, medical and surgical history, and postoperative outcomes were obtained. Results A total of 20 cases were included in the present study, sixteen (80%) of which were females and four (20%) were males. The mean age of participants was 42.59±13.9 years. Nine cases (45%) were spontaneous CSF rhinorrhea and 11 (55%) were traumatic; within the traumatic group, six cases (54%) were iatrogenic either following previous neurosurgery or functional endoscopic sinus surgery, while the remaining five cases were related to motor vehicle accidents. The mean body mass index for the spontaneous CSF leak was 32 Kg/m2, and 33 Kg/m2 for the traumatic leaks, no statistically significant difference was noted. The cribriform plate was the most common site of leakage (65%). A multilayer surgical technique using facia lata graft with nasoseptal flap was the most common choice for reconstruction with a first-attempt success rate approximating 90%. A recurrence was observed in two patients only. No major complications were reported. The average length of stay was nine days. Conclusion The endoscopic endonasal repair of CSF leak is a safe and reliable procedure and is associated with high success rates and low risk of complications. Therefore, it should be preferred as a first-line treatment for CSF rhinorrhea.

Cerebrospinal fluid rhinorrhea with meningoencephalocele related to Sternberg's canal: A report of two cases.

Background: Cerebrospinal fluid (CSF) rhinorrhea with meningoencephalocele (MEC) associated with Sternberg's canal is rare. We treated two such cases. Case Description: A 41-year-old man and a 35-year-old woman presented with CSF rhinorrhea and mild headache worsening with standing posture. Head computed tomography showed a defect close to the foramen rotundum in the lateral wall of the left sphenoid sinus in both cases. Head magnetic resonance (MR) imaging and MR cisternography revealed that brain parenchyma had herniated into the lateral sphenoid sinus through the defect of the middle cranial fossa. The intradural and extradural spaces and bone defect were sealed with fascia and fat through both intradural and extradural approaches. The MEC was cut away to prevent infection. CSF rhinorrhea completely stopped after the surgery. Conclusion: Our cases were characterized by empty sella, thinning of the dorsum sellae, and large arteriovenous malformations that suggest chronic intracranial hypertension. The possibility of Sternberg's canal in patients with CSF rhinorrhea with chronic intracranial hypertension should be considered. The cranial approach has the advantages of lower infection risk and the ability to close the defect with multilayer plasty under direct vision. The transcranial approach is still safe if performed by a skillful neurosurgeon.

Anterior Skull Base Reconstruction.

Anterior skull base reconstruction requires careful preoperative planning to use the most effective technique for the expected defect. Adherence to the principles of skull base reconstruction is imperative to minimize complications and improve patient outcomes.

Correlation between Pneumatization Variants of Paranasal Sinuses and Skull Base and Spontaneous Cerebrospinal Fluid Rhinorrhea: A Case-Control Study.

INTRODUCTION: Spontaneous cerebrospinal fluid rhinorrhea (SCSFR) is the most common type of cerebrospinal fluid leakage and may cause serious cerebral complications. The aim of this research was to investigate the relationship between the degree of pneumatization variants of the paranasal sinus and skull base and the incidence of SCSFR. METHODS: In total, 131 patients with SCSFR were analyzed, and 50 patients suffering from the nasal septal deviation were selected as controls. The pneumatization of the paranasal sinus and skull base was observed by CT scan. RESULTS: Among the 137 fistulas, 55 (40.15%) were found in the ethmoid sinus. The incidences of Onodi cells (27.27 vs. 8%) and type 3 lateral recess of the sphenoid sinus (LRSS, 70.37 vs. 22%) in the SCSFR subgroups were significantly higher than those in the control group (p < 0.05). Moreover, the occurrence of SCSFR was linearly correlated with the classification of Onodi cells and LRSS (p < 0.05). There was no significant difference in the incidence of frontal cells, anterior clinoid process pneumatization, and posterior clinoid process pneumatization between the SCSFR patients and the controls. CONCLUSION: The most common site of SCSFR is the ethmoid sinus. The excessive pneumatization of the Onodi cell and LRSS increases the risk for the occurrence of SCSFR in the ethmoid sinus and sphenoid sinus, respectively. The possible association between the paranasal sinus ontogeny and SCSFR pathophysiology needs further studies.

A 58-year-Old non-smoking woman with intractable cough and rhinorrhea.

Our case highlights an uncommon cause of CSF rhinorrhea. The patient was diagnosed with bacterial meningitis and appropriately treated after which, she developed unilateral rhinorrhea followed by non-productive cough. These symptoms were not responsive to multiple treatment regimens, and eventually imaging revealed a dehiscence in the ethmoid air sinus which was surgically repaired. We also performed a literature review on CSF rhinorrhea and provide insights into its evaluation.

Pneumocephalus following sneeze suppression.

Pneumocephalus is defined as air in the intracranial space. It is commonly caused by traumatic skull fractures and is diagnosed by head plain computer tomography. Treatment involves initial stabilization of vital signs and surgical intervention if symptoms do not resolve. We report a unique case of pneumocephalus following sneeze suppression.