Significance of Empty Sella in Cerebrospinal Fluid Leaks

2003 ◽  
Vol 128 (1) ◽  
pp. 32-38 ◽  
Author(s):  
Rodney J. Schlosser ◽  
William E. Bolger
Keyword(s):  
Cerebrospinal Fluid ◽  
Intracranial Hypertension ◽  
Sella Turcica ◽  
Radiographic Finding ◽  
Empty Sella ◽  
Senior Author ◽  
Csf Leak ◽  
Benign Intracranial Hypertension ◽  
Cerebrospinal Fluid Leaks ◽  
Csf Leaks

OBJECTIVE: The role of elevated cerebrospinal fluid (CSF) pressures in the pathophysiology of various CSF leaks is not clear. Empty sella syndrome (ESS) is a radiographic finding that can be associated with elevated CSF pressures and may represent a radiographic indicator of intracranial hypertension. We present our experience with CSF leaks of various causes, the prevalence of ESS in the spontaneous and nonspontaneous categories, and the potential pathophysiology and unique management issues of the spontaneous CSF leak group. METHODS: We conducted a retrospective review of medical records, imaging studies, and surgical treatment of CSF leaks in patients treated by the senior author. RESULTS: Sixteen patients with spontaneous CSF leaks and 12 patients with nonspontaneous CSF leaks were surgically treated from 1996 through 2002. In the spontaneous group, 15 patients had complete imaging of the sella turcica. Ten had completely empty sellae and 5 had partially empty sellae, for a total of 100% (15 of 15). In the nonspontaneous group, 9 patients had complete imaging of the sella. Only 11% (1 of 9) had a partially empty sella and that was a congenital leak. Comparison of proportions between these 2 groups was significant ( P = 0.01). The spontaneous group consisted primarily of obese, middle-aged females (13 of 16 patients). CONCLUSION: Empty sella probably represents a sign of elevated intracranial pressure that leads to idiopathic, spontaneous CSF leaks. Spontaneous CSF leaks are strongly associated with the radiographic finding of an empty sella and are more common in obese females, similar to benign intracranial hypertension. This unique population may require more aggressive surgical and medical treatment to prevent recurrent or multiple leaks.

Spontaneous Nasal Cerebrospinal Fluid Leaks and Empty Sella Syndrome: A Clinical Association

2003 ◽  
Vol 17 (2) ◽  
pp. 91-96 ◽  
Author(s):  
Rodney J. Schlosser ◽  
William E. Bolger
Keyword(s):  
Cerebrospinal Fluid ◽  
Intracranial Hypertension ◽  
Skull Base ◽  
Sella Turcica ◽  
Empty Sella ◽  
Radiographic Appearance ◽  
Radiographic Findings ◽  
Empty Sella Syndrome ◽  
Csf Pressure ◽  
Csf Leaks

Background Spontaneous, idiopathic nasal meningoencephaloceles are herniations of arachnoid/dura and cerebrospinal fluid (CSF) through anatomically fragile sites within the skull base. Empty sella syndrome occurs when intracranial contents herniate through the sellar diaphragm filling the sella turcica with CSF and giving the radiographic appearance of an absent pituitary gland. The objective of this study was to examine the association between spontaneous encephaloceles/CSF leaks and empty sella syndrome because of their similar clinical features and potential common pathophysiology. Methods Retrospective. Results Sixteen patients were treated for spontaneous encephaloceles between 1996 and 2001. All 16 patients had associated CSF leaks. Five patients had multiple simultaneous encephaloceles. Fifteen patients with imaging of the sella turcica had empty (10 patients) or partially empty (5 patients) sellas. One patient did not have complete imaging of the sella. Three patients had lumbar punctures with measurement of CSF pressure during computed tomography cisternograms because of multiple skull base defects. Mean CSF pressure was 28.3 cm of water (range, 19–34 cm; normal, 0–15 cm). Thirteen of 16 patients (81%) were obese women (mean body mass index, 35.9 kg/m2; normal, <25 kg/m2). Mean follow-up was 14.2 months with 100% success in closure of the defects after one procedure. Conclusion Spontaneous meningoencephaloceles and CSF leaks are strongly associated with radiographic findings of an empty sella and suggest a common pathophysiology. The underlying condition probably represents a form of intracranial hypertension that exerts hydrostatic pressure at anatomically weakened sites within the skull base. Otolaryngologists should be familiar with this disease entity and the implications intracranial hypertension has on patient management.

Spontaneous Skull Base Cerebrospinal Fluid Leaks and Their Relationship to Idiopathic Intracranial Hypertension

2020 ◽  
Vol 35 (1) ◽  
pp. 36-43 ◽  
Author(s):  
Samuel Bidot ◽  
Joshua M. Levy ◽  
Amit M. Saindane ◽  
Kannan M. Narayana ◽  
Michael Dattilo ◽  
...  
Keyword(s):  
Cerebrospinal Fluid ◽  
Optic Nerve ◽  
Intracranial Hypertension ◽  
Skull Base ◽  
Optic Nerve Head ◽  
Idiopathic Intracranial Hypertension ◽  
Signs And Symptoms ◽  
Csf Leak ◽  
Imaging Evaluation ◽  
Cerebrospinal Fluid Leaks

Background The association between spontaneous skull base cerebrospinal fluid (CSF) leaks and idiopathic intracranial hypertension (IIH) has been suggested, but its significance remains unclear. Objective To estimate the prevalence of IIH in spontaneous skull base CSF leak patients. Methods Systematic collection of demographics, neuro-ophthalmic and magnetic resonance imaging evaluation of spontaneous skull base CSF leak patients seen pre- and post-leak repair in one neuro-ophthalmology service. Patients with preexisting IIH were diagnosed with definite IIH if adequate documentation was provided; otherwise, they were categorized with presumed IIH. Classic radiographic signs of intracranial hypertension and bilateral transverse venous sinus stenosis were recorded. Results Thirty six patients were included (age [interquartile range]: 50 [45;54] years; 94% women; body mass index: 36.8 [30.5;39.9] kg/m2), among whom six (16.7%, [95% confidence interval, CI]: [6.4;32.8]) had a preexisting diagnosis of definite or presumed IIH. Of the remaining 30 patients, four (13.3%, 95%CI: [3.8;30.7]) had optic nerve head changes suggesting previously undiagnosed IIH, while one was newly diagnosed with definite IIH at initial consultation. One out of 29 patients with normal findings of the optic nerve head at presentation developed new onset papilledema following surgery (3.4%, 95%CI: [0.1;17.8]) and was ultimately diagnosed with definite IIH. Overall, the prevalence of definite IIH was 19.4% (95%CI: [8.2;36.0]). Conclusion Striking demographic overlap exists between IIH patients and those with spontaneous CSF leak. Definite IIH was present in approximately 20% of our patients. However, its true prevalence is likely higher than identified by using classic criteria. We therefore hypothesize that an active CSF leak serves as an auto-diversion for CSF, thereby “treating” the intracranial hypertension and eliminating characteristic signs and symptoms at initial presentation.

A Unique Subset: Idiopathic Intracranial Hypertension Presenting as Spontaneous CSF Leak of the Anterior Skull Base

2021 ◽  
Author(s):  
Christopher S. Hong ◽  
Adam J. Kundishora ◽  
Aladine A. Elsamadicy ◽  
Eugenia M. Vining ◽  
R. Peter Manes ◽  
...  
Keyword(s):  
Literature Review ◽  
Intracranial Hypertension ◽  
Skull Base ◽  
Idiopathic Intracranial Hypertension ◽  
Empty Sella ◽  
Csf Leak ◽  
Presenting Symptoms ◽  
History Of ◽  
Sinus Stenosis ◽  
Csf Leaks

Abstract Background Spontaneous cerebrospinal fluid (CSF) leaks represent a unique clinical presentation of idiopathic intracranial hypertension (IIH), lacking classical features of IIH, including severe headaches, papilledema, and markedly elevated opening pressures. Methods Following a single-institution retrospective review of patients undergoing spontaneous CSF leak repair, we performed a literature review of spontaneous CSF leak in patients previously undiagnosed with IIH, querying PubMed. Results Our literature review yielded 26 studies, comprising 716 patients. Average age was 51 years with 80.8% female predominance, and average body mass index was 35.5. Presenting symptoms included headaches (32.5%), visual disturbances (4.2%), and a history of meningitis (15.3%). Papilledema occurred in 14.1%. An empty sella was present in 77.7%. Slit ventricles and venous sinus stenosis comprised 7.7 and 31.8%, respectively. CSF leak most commonly originated from the sphenoid sinus (41.1%), cribriform plate (25.4%), and ethmoid skull base (20.4%). Preoperative opening pressures were normal at 22.4 cm H2O and elevated postoperatively to 30.8 cm H2O. 19.1% of patients underwent shunt placement. CSF leak recurred after repair in 10.5% of patients, 78.6% involving the initial site. A total of 85.7% of these patients were managed with repeat surgical intervention, and 23.2% underwent a shunting procedure. Conclusion Spontaneous CSF leaks represent a distinct variant of IIH, distinguished by decreased prevalence of headaches, lack of visual deficits, and normal opening pressures. Delayed measurement of opening pressure after leak repair may be helpful to diagnose IIH. Permanent CSF diversion may be indicated in patients exhibiting significantly elevated opening pressures postoperatively, refractory symptoms of IIH, or recurrent CSF leak.

scholarly journals Case Report: An Intracranial Complication of COVID-19 Nasopharyngeal Swab

2021 ◽  
Vol 5 (3) ◽  
pp. 341-344
Author(s):  
Alexandria Holmes ◽  
Bryant Allen
Keyword(s):  
Cerebrospinal Fluid ◽  
Case Report ◽  
Acute Onset ◽  
Csf Leak ◽  
Nasopharyngeal Swab ◽  
Unique Case ◽  
Cerebrospinal Fluid Leaks ◽  
Streptococcal Meningitis ◽  
Persistent Headache ◽  
Csf Leaks

Introduction: Cerebrospinal fluid (CSF) leaks are often the result of trauma or recent surgical procedures; however, a subset can develop from non-traumatic etiologies. Cerebrospinal fluid leaks from congenital and spontaneous encephaloceles can be clinically occult and have devastating consequences if undetected for prolonged periods of time. This report highlights a unique case of meningitis after CSF leak caused by ruptured congenital meningocele during a routine nasopharyngeal swab.  Case Report: A 54-year-old female with diagnosed CSF leak presented to the emergency department (ED) with acute onset of severe headache, and neck and back pain. Prior to this presentation, the patient had experienced two months of persistent headache and rhinorrhea since her coronavirus disease 2019 (COVID-19) nasopharyngeal swab. As part of her outpatient workup, an otolaryngology consultation with subsequent beta-2 transferrin testing and magnetic resonance imaging was performed and she was diagnosed with a CSF leak from ruptured congenital meningocele. On ED presentation, she was afebrile, but with mild tachycardia, leukocytosis, and meningismus. Lumbar puncture revealed acute streptococcal meningitis. This patient’s meningitis developed due to prolonged occult CSF leak after her COVID-19 nasopharyngeal swab ruptured a pre-existing congenital meningocele.  Conclusion: Nasopharyngeal swabs are being performed much more frequently due to the COVID-19 pandemic. All front-line providers should be aware of the potential presence and rupture of congenital meningoceles in patients who have undergone recent nasopharyngeal swab when risk-stratifying for potential CSF leak and meningitis.

scholarly journals Current insights in CSF leaks: a literature review of mechanisms, pathophysiology and treatment options

2017 ◽  
Vol 7 (27) ◽  
pp. 143-151
Author(s):  
Elena Patrascu ◽  
Claudiu Manea ◽  
Codrut Sarafoleanu
Keyword(s):  
Cerebrospinal Fluid ◽  
Treatment Options ◽  
Bed Rest ◽  
Future Trend ◽  
Csf Leak ◽  
High Resolution Computed Tomography ◽  
Intracranial Complications ◽  
Cerebrospinal Fluid Leaks ◽  
Dural Lesion ◽  
Csf Leaks

Abstract A cerebrospinal fluid (CSF) leak occurs when there appears a fistula between the dura and the skull base and it is usually characterized by discharge of cerebrospinal fluid from the nose. Cerebrospinal fluid leaks may have many etiologies, the most common being trauma. The most common site of dural lesion is the cribriform plate of the ethmoid. Diagnosis can be achieved by a multitude of techniques, high-resolution computed tomography being the modality of choice and it may be completed with magnetic resonance imaging or cisternography. Treatment may be either conservative, either surgical, related to the cause, the site and the duration of CSF leak. Conservative treatment usually includes strict bed rest, elevated bed head and no straining, nose blowing or stretching, with resolution of the majority of traumatic CSF leaks in seven days. Surgical treatment consists of a variety of approaches (intracranial/extracranial, open/endoscopic). The future trend is represented by minimally invasive endoscopic approaches, with a success rate of almost 90%; however, open transcranial or extracranial interventions still have indications in the surgical management. CSF leaks must be correctly diagnosed and treated, because the risk of intracranial complications increases 10-fold when the leakage persists.

Spontaneous Cerebrospinal Fluid Leaks: A Variant of Benign Intracranial Hypertension

2006 ◽  
Vol 115 (7) ◽  
pp. 495-500 ◽  
Author(s):  
Rodney J. Schlosser ◽  
Bradford A. Woodworth ◽  
Eileen Maloney Wilensky ◽  
M. Sean Grady ◽  
William E. Bolger
Keyword(s):  
Cerebrospinal Fluid ◽  
Intracranial Hypertension ◽  
Benign Intracranial Hypertension ◽  
Cerebrospinal Fluid Leaks

Primary empty sella syndrome in a series of 142 patients

2005 ◽  
Vol 103 (5) ◽  
pp. 831-836 ◽  
Author(s):  
Giulio Maira ◽  
Carmelo Anile ◽  
Annunziato Mangiola
Keyword(s):  
Cerebrospinal Fluid ◽  
Intracranial Hypertension ◽  
Empty Sella ◽  
Csf Leak ◽  
Csf Shunt ◽  
Csf Dynamics ◽  
Empty Sella Syndrome ◽  
Content Type ◽  
Fine Print ◽  
Primary Empty Sella

Object. The primary empty sella syndrome (ESS) represents a heterogeneous clinical picture characterized by endocrine disturbances and signs of intracranial hypertension. An increase in intracranial pressure (ICP) is proposed to be one of the involved pathogenetic factors. Methods. The series included 142 patients who were observed during a period of 20 years. All patients underwent an ICP and cerebrospinal fluid (CSF) dynamics evaluation through the use of a lumbar constant-rate infusion test. Impairment of ICP and CSF dynamics was observed in 109 patients (76.8%). In 35 of the 36 patients affected by severe intracranial hypertension without rhinorrhea, improvement in adverse neurological symptoms was achieved after implanting a CSF shunt. Visual function, already seriously compromised before surgery, remained severely altered in one patient. In the group of 34 patients affected by rhinorrhea, CSF leakage was controlled using different surgical treatments: CSF shunt placement in 16 cases, surgical repair of the sellar floor in three, and both procedures in the remaining 13. Two patients refused any surgical treatment. Conclusions. The role of increased ICP in the pathogenesis and perpetuation of primary ESS has been confirmed. Adverse neurological signs and a CSF leak are correlated with an actual increase in ICP and are relieved after CSF shunt insertion. Cerebrospinal fluid rhinorrhea is more common than generally thought. Its resolution can be achieved using a careful diagnostic protocol and sometimes may require different surgical procedures.

Spontaneous Congenital Perilabyrinthine Cerebrospinal Fluid Fistulas

2021 ◽  
pp. 000348942110072
Author(s):  
Benjamin D. Lovin ◽  
Eric N. Appelbaum ◽  
Latifah Makoshi ◽  
William E. Whitehead ◽  
Alex D. Sweeney
Keyword(s):  
Systematic Review ◽  
Cerebrospinal Fluid ◽  
Intracranial Hypertension ◽  
Middle Ear ◽  
Jugular Bulb ◽  
Csf Leak ◽  
Review Of The Literature ◽  
Multiple Sources ◽  
Lateral Skull Base ◽  
Csf Leaks

Objectives: To report a recalcitrant spontaneous cerebrospinal fluid (CSF) fistula arising from multiple, anatomically-linked lateral skull base defects, and to review the available literature to determine optimal techniques for operative repair of congenital CSF fistulae. Methods: A patient with recurrent episodes of otologic meningitis was found to have a patent tympanomeningeal fissure, also known as a Hyrtl’s fissure, and internal auditory canal (IAC) diverticulum that communicated with the jugular bulb. A systematic review of the literature characterized all reports of spontaneous congenital perilabyrinthine CSF leaks, and all cases of Hyrtl’s fissures. Results: An 11-year-old female was referred for recurrent meningitis. Imaging demonstrated a fistulous connection between the middle ear and IAC diverticulum via the jugular foramen. Specifically, a Hyrtl’s fissure was identified, as well as demineralized bone around the jugular bulb. Obliteration of the fissure was initially performed, and a fistula reformed 4 months later. Multifocal CSF egress in the hypotympanum was identified on re-exploration, and middle ear obliteration with external auditory canal (EAC) overclosure was performed. A systematic review of the literature demonstrated 19 cases of spontaneous congenital perilabyrinthine CSF leaks. In total, 6 cases had multiple sources of CSF leak and 2 had history suggestive of intracranial hypertension. All of these noted cases demonstrated leak recurrence. Middle ear obliteration with EAC overclosure was successful in 4 recalcitrant cases. Conclusions: Repair of spontaneous congenital perilabyrinthine CSF leaks in cases demonstrating multiple sources of egress or signs of intracranial hypertension should be approached with caution. Middle ear obliteration with EAC overclosure may provide the most definitive management option for these patients, particularly if initial attempt at primary repair is unsuccessful.

scholarly journals Risks, costs, and outcomes of cerebrospinal fluid leaks after pediatric skull fractures: a MarketScan analysis between 2007 and 2015

2019 ◽  
Vol 47 (5) ◽  
pp. E10
Author(s):  
Kunal Varshneya ◽  
Adrian J. Rodrigues ◽  
Zachary A. Medress ◽  
Martin N. Stienen ◽  
Gerald A. Grant ◽  
...  
Keyword(s):  
Pediatric Patients ◽  
Pediatric Population ◽  
Skull Fracture ◽  
Neurological Deficits ◽  
Csf Leak ◽  
Skull Fractures ◽  
Pediatric Head Trauma ◽  
Cerebrospinal Fluid Leaks ◽  
Neurosurgical Intervention ◽  
Csf Leaks

OBJECTIVESkull fractures are common after blunt pediatric head trauma. CSF leaks are a rare but serious complication of skull fractures; however, little evidence exists on the risk of developing a CSF leak following skull fracture in the pediatric population. In this epidemiological study, the authors investigated the risk factors of CSF leaks and their impact on pediatric skull fracture outcomes.METHODSThe authors queried the MarketScan database (2007–2015), identifying pediatric patients (age < 18 years) with a diagnosis of skull fracture and CSF leak. Skull fractures were disaggregated by location (base, vault, facial) and severity (open, closed, multiple, concomitant cerebral or vascular injury). Descriptive statistics and hypothesis testing were used to compare baseline characteristics, complications, quality metrics, and costs.RESULTSThe authors identified 13,861 pediatric patients admitted with a skull fracture, of whom 1.46% (n = 202) developed a CSF leak. Among patients with a skull fracture and a CSF leak, 118 (58.4%) presented with otorrhea and 84 (41.6%) presented with rhinorrhea. Patients who developed CSF leaks were older (10.4 years vs 8.7 years, p < 0.0001) and more commonly had skull base (n = 183) and multiple (n = 22) skull fractures (p < 0.05). These patients also more frequently underwent a neurosurgical intervention (24.8% vs 9.6%, p < 0.0001). Compared with the non–CSF leak population, patients with a CSF leak had longer average hospitalizations (9.6 days vs 3.7 days, p < 0.0001) and higher rates of neurological deficits (5.0% vs 0.7%, p < 0.0001; OR 7.0; 95% CI 3.6–13.6), meningitis (5.5% vs 0.3%, p < 0.0001; OR 22.4; 95% CI 11.2–44.9), nonroutine discharge (6.9% vs 2.5%, p < 0.0001; OR 2.9; 95% CI 1.7–5.0), and readmission (24.7% vs 8.5%, p < 0.0001; OR 3.4; 95% CI 2.5–4.7). Total costs at 90 days for patients with a CSF leak averaged $81,206, compared with $32,831 for patients without a CSF leak (p < 0.0001).CONCLUSIONSThe authors found that CSF leaks occurred in 1.46% of pediatric patients with skull fractures and that skull fractures were associated with significantly increased rates of neurosurgical intervention and risks of meningitis, hospital readmission, and neurological deficits at 90 days. Pediatric patients with skull fractures also experienced longer average hospitalizations and greater healthcare costs at presentation and at 90 days.

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