1
Brandon Isaacson MD, FACS UT Southwestern Medical Center Email: [email protected] Objective: To determine the prevalence of elevated intracranial hypertension in patients with spontaneous cerebrospinal fluid otorrhea (SCSFO). Study design: Case series with chart review Setting: Tertiary care referral center Subjects and Methods: Patients included have undergone operative repair of spontaneous CSF otorrhea between January 2007 and May 2012. Lumbar puncture opening pressure was used to measure intracranial pressure (ICP) 6 weeks after surgery. Preoperative magnetic resonance imaging was also reviewed for evidence of elevated ICP. Results: : Thirty-eight patients underwent operative repair of spontaneous cerebrospinal fluid otorrhea. Of these, 22 underwent postoperative lumbar puncture with measurement of the opening pressure. The opening pressure was elevated (>20 cm/H 2 0) in 8 patients (36.4%). Preoperative magnetic resonance imaging was available for review by a neuroradiologist in 27 patients. Radiographic evidence of elevated intracranial pressure was present in 48.1% of patients. Conclusion: Elevated intracranial pressure is common in patients with spontaneous cerebrospinal fluid otorrhea. However, when directly measured by lumbar puncture opening pressure, a minority of patients has elevated intracranial pressure. Elevated Intracranial Pressure in Patients with Spontaneous Cerebrospinal Fluid Otorrhea Kyle P Allen MD, MPH 1 , Carlos L. Perez MD 2 , J. Walter Kutz, Jr., MD 1 , Peter S. Roland, MD 1 , Deniz Gerecci 3 , Brandon Isaacson, MD 1 1 Department Of Otolaryngology, UT Southwestern Medical Center (UTSW), 2 Department of Radiology, UTSW, 3 UTSW Medical School After institutional review board approval, patients undergoing repair SCSFO at a single, tertiary care referral center between January 2007 and May 2012 were identified. Patients with CSF fistula due an etiology of trauma, cholesteatoma, or neoplasm were excluded from the analysis. Operative repair was performed via a middle fossa or transmastoid approach. When available, preoperative magnetic resonance imaging (MRI) was reviewed for evidence of elevated ICP (empty sella, optic nerve dilation, reverse cupping of the optic disc, tortuous/elongated optic nerve, flattened sclera, or Meckel’s cave diverticula). Patient underwent lumbar puncture opening pressure (LPOP) measurement 6 weeks after operative repair to measure intracranial pressure. While elevated ICP is a common finding in patients with SCSFO, only a minority of patients have elevated pressure by LPOP or MRI findings. This may indicated that while elevated ICP may contribute to the development of some cases of SCSFO, it is not a universal finding. Other etiologies, such as a congenitally thin skull base or aberrant arachnoid granulations may be a more common etiology of SCSFO. INTRODUCTION CONCLUSIONS METHODS REFERENCES ABSTRACT RESULTS Spontaneous cerebrospinal fluid otorrhea (SCSFO) occurs when cerebrospinal fluid (CSF) fills the aerated confines of the temporal bone in the absence of trauma, chronic infection, neoplasm, or iatrogenic cause. The exact etiology is unclear, but may be due to erosion of the tegmen by aberrant arachnoid granulations or constant pulsation of the temporal lobe against a congenitally thin tegmen resulting in a bony and dural defect. 1 More recently, the role of increased intracranial pressure (ICP) and its correlation with idiopathic intracranial hypertension (IIH) in the pathogenesis SCSFO has come to the forefront. The upper limit of normal for lumbar puncture opening pressure (LPOP) is 20 cm/H 2 0, however, some consider pressures up to 25-28 cm/H 2 0 to be normal. 2 The modified Dandy criteria for the diagnosis of IIH require an opening pressure greater than 25 cm/H 2 0, among other criteria. 3 It has been proposed that spontaneous CSF leaks of sinonasal and temporal bone origin may be a result of IIH. 4,5 In one study of spontaneous CSF leaks of sinonasal origin, 72% of patients (8 of 11) were noted to have a LPOP greater than 20 cm/H 2 0, and 54.5% (6 of 11) had a pressure greater than 25 cm/H 2 0. 4 Empty sella syndrome (ESS), a radiographic correlate of elevated ICP, has an increased prevalence in patients with spontaneous CSF of sinonasal and temporal bone origin. 4,6 This retrospective review was undertaken to examine the prevalence of elevated intracranial pressure in a series of patients who have undergone repair of SCSFO. Patient characteristics Operative repair of SCSFO was performed for 40 ears in 38 consecutive patients. Demographic for the patients is included in Table 1. The majority of patients were female (76.3%), and the left ear was more commonly involved in 65% of affected ears (26/40). Two patients had bilateral SCSFO and underwent staged bilateral repairs. By body mass index (BMI) criteria, 30 patients (78.9%) were obese (BMI >30 kg/m 2 ). Preoperative Magnetic Resonance Imaging Preoperative magnetic resonance imaging was available for review by a neuroradiologist in 27 patients. Radiographic evidence of elevated intracranial pressure was present in 13 (48.1%) patients. Radiographic findings are listed in Table 2. RESULTS Figure 1: Coronal T2 MRI and CT of the temporal bone demonstrating a left meningoencephalocele (arrows) in a 59 year-old male patient. 1. Gacek RR, Gacek MR, Tart R. Adult spontaneous cerebrospinal fluid otorrhea: diagnosis and management. Am J Otol. 1999; 20: 770-76. 2. Whiteley W, Al-Shahi R, Warlow CP, Zeidler M, Lueck CJ. CSF opening pressure: Reference interval and the effect of body mass index. Neurology. 2006; 67: 1690-1691. 3. Smith J.L.: Whence pseudotumor cerebri?. J Clin Neuroophthalmol. 1985; 5: 55-56. 4. Schlosser RJ, Woodworth BA, Wilensky EM, Grady MS, Bolger WE. Spontaneous cerebrospinal fluid leaks: a variant of benign intracranial hypertension. Ann Otol, Rhin & Laryng. 2006; 115: 495-500. 5. LeVay AJ, Kveton JF. Relationship between obesity, obstructive sleep apnea, and spontaneous cerebrospinal fluid otorrhea. Laryngoscope. 2008; 118: 275-8. 6. Prichard CH, Isaacson B, Oghalai JS, Coker NJ, Vrabec JF. Adult spontaneous CSF otorrhea: correlation with radiographic empty sella. J Oto HNS. 2006; 134: 767-71. Patients 38 Female 29 (76.3% Male 9 (23.7%) CSF Otorrhea Unilateral 29 (94.7%) Bilateral 2 (5.3%) Age Range (years) 40-75 Mean (years) 60.3 BMI Range (kg/m 2 ) 24.2-57.8 Mean (kg/m 2 ) 36.8 Obese (BMI >30 kg/m 2 ) 30 (78.9%) Table 1 Lumbar Opening Pressure Measurements Twenty-two patients underwent postoperative LPOP measurement . Opening pressures ranged from 10-55 cm/H 2 0 with a mean of 19.4 cm/H 2 0. The opening pressure was elevated (>20 cm/H 2 0) in 8 patients (36.4%). Two patients (9.1%) met the modified Dandy LPOP criteria for IIH (>25 cm/H 2 0). All patients with elevated LPOP were female. There were no significant differences in age or BMI in patients with or without elevated LPOP. MRI Findings # % Total Patients 27 Empty Sella 10 37.0 Meckel's Cave Diverticula 9 33.3 Optic Nerve Dilation 4 14.8 Tortuous/Elongated Optic Nerve 3 11.1 Flattened Sclera 1 3.7 Reverse Cupping of the Optic Disc 0 0 Evidence of Elevated ICP 13 48.1 Table 2 Intracranial pressure measurement by lumbar puncture opening pressure Figure 2: Example of an empty sella (arrow) demonstrated on a coronal T2 MRI in a 56 year-old female patient with SCSFO and elevated LPOP. Figure 3: Bilateral Meckel’s cave diverticula (arrows) in a 68 year-old male patient with SCSFO.

Elevated Intracranial Pressure in Patients with ...More recently, the role of increased intracranial pressure (ICP) and its correlation with idiopathic intracranial hypertension (IIH)

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  • Brandon Isaacson MD, FACSUT Southwestern Medical CenterEmail: [email protected]

    Objective: To determine the prevalence of elevated intracranial hypertension in patients with spontaneous cerebrospinal fluid otorrhea (SCSFO).

    Study design: Case series with chart review

    Setting: Tertiary care referral center

    Subjects and Methods: Patients included have undergone operative repair of spontaneous CSF otorrhea between January 2007 and May 2012. Lumbar puncture opening pressure was used to measure intracranial pressure (ICP) 6 weeks after surgery. Preoperative magnetic resonance imaging was also reviewed for evidence of elevated ICP.

    Results: : Thirty-eight patients underwent operative repair of spontaneous cerebrospinal fluid otorrhea. Of these, 22 underwent postoperative lumbar puncture with measurement of the opening pressure. The opening pressure was elevated (>20 cm/H20) in 8 patients (36.4%). Preoperative magnetic resonance imaging was available for review by a neuroradiologist in 27 patients. Radiographic evidence of elevated intracranial pressure was present in 48.1% of patients.

    Conclusion: Elevated intracranial pressure is common in patients with spontaneous cerebrospinal fluid otorrhea. However, when directly measured by lumbar puncture opening pressure, a minority of patients has elevated intracranial pressure.

    Elevated Intracranial Pressure in Patients with Spontaneous Cerebrospinal Fluid Otorrhea

    Kyle P Allen MD, MPH1, Carlos L. Perez MD2, J. Walter Kutz, Jr., MD1, Peter S. Roland, MD1, Deniz Gerecci3, Brandon Isaacson, MD11Department Of Otolaryngology, UT Southwestern Medical Center (UTSW), 2Department of Radiology, UTSW, 3UTSW Medical School

    After institutional review board approval, patients undergoing repair SCSFO at a single, tertiary care referral center between January 2007 and May 2012 were identified. Patients with CSF fistula due an etiology of trauma, cholesteatoma, or neoplasm were excluded from the analysis. Operative repair was performed via a middle fossa or transmastoid approach.

    When available, preoperative magnetic resonance imaging (MRI) was reviewed for evidence of elevated ICP (empty sella, optic nerve dilation, reverse cupping of the optic disc, tortuous/elongated optic nerve, flattened sclera, or Meckel’s cave diverticula).

    Patient underwent lumbar puncture opening pressure (LPOP) measurement 6 weeks after operative repair to measure intracranial pressure.

    While elevated ICP is a common finding in patients with SCSFO, only a minority of patients have elevated pressure by LPOP or MRI findings. This may indicated that while elevated ICP may contribute to the development of some cases of SCSFO, it is not a universal finding. Other etiologies, such as a congenitally thin skull base or aberrant arachnoid granulations may be a more common etiology of SCSFO.

    INTRODUCTION

    CONCLUSIONS

    METHODS

    REFERENCES

    ABSTRACT RESULTSSpontaneous cerebrospinal fluid otorrhea (SCSFO) occurs when cerebrospinal fluid (CSF) fills the aerated confines of the temporal bone in the absence of trauma, chronic infection, neoplasm, or iatrogenic cause. The exact etiology is unclear, but may be due to erosion of the tegmen by aberrant arachnoid granulations or constant pulsation of the temporal lobe against a congenitally thin tegmen resulting in a bony and dural defect.1

    More recently, the role of increased intracranial pressure (ICP) and its correlation with idiopathic intracranial hypertension (IIH) in the pathogenesis SCSFO has come to the forefront. The upper limit of normal for lumbar puncture opening pressure (LPOP) is 20 cm/H20, however, some consider pressures up to 25-28 cm/H20 to be normal.2 The modified Dandy criteria for the diagnosis of IIH require an opening pressure greater than 25 cm/H20, among other criteria.3It has been proposed that spontaneous CSF leaks of sinonasal and temporal bone origin may be a result of IIH.4,5 In one study of spontaneous CSF leaks of sinonasal origin, 72% of patients (8 of 11) were noted to have a LPOP greater than 20 cm/H20, and 54.5% (6 of 11) had a pressure greater than 25 cm/H20.4 Empty sella syndrome (ESS), a radiographic correlate of elevated ICP, has an increased prevalence in patients with spontaneous CSF of sinonasal and temporal bone origin.4,6

    This retrospective review was undertaken to examine the prevalence of elevated intracranial pressure in a series of patients who have undergone repair of SCSFO.

    Patient characteristics

    Operative repair of SCSFO was performed for 40 ears in 38 consecutive patients. Demographic for the patients is included in Table 1. The majority of patients were female (76.3%), and the left ear was more commonly involved in 65% of affected ears (26/40). Two patients had bilateral SCSFO and underwent staged bilateral repairs. By body mass index (BMI) criteria, 30 patients (78.9%) were obese (BMI >30 kg/m2).

    Preoperative Magnetic Resonance Imaging

    Preoperative magnetic resonance imaging was available for review by a neuroradiologist in 27 patients. Radiographic evidence of elevated intracranial pressure was present in 13 (48.1%) patients. Radiographic findings are listed in Table 2.

    RESULTS

    Figure 1: Coronal T2 MRI and CT of the temporal bone demonstrating a left meningoencephalocele (arrows) in a 59 year-old male patient.

    1. Gacek RR, Gacek MR, Tart R. Adult spontaneous cerebrospinal fluid otorrhea: diagnosis and management. Am J Otol. 1999; 20: 770-76.

    2. Whiteley W, Al-Shahi R, Warlow CP, Zeidler M, Lueck CJ. CSF opening pressure: Reference interval and the effect of body mass index. Neurology. 2006; 67: 1690-1691.

    3. Smith J.L.: Whence pseudotumor cerebri?. J Clin Neuroophthalmol. 1985; 5: 55-56.

    4. Schlosser RJ, Woodworth BA, Wilensky EM, Grady MS, Bolger WE. Spontaneous cerebrospinal fluid leaks: a variant of benign intracranial hypertension. Ann Otol, Rhin & Laryng. 2006; 115: 495-500.

    5. LeVay AJ, Kveton JF. Relationship between obesity, obstructive sleep apnea, and spontaneous cerebrospinal fluid otorrhea. Laryngoscope. 2008; 118: 275-8.

    6. Prichard CH, Isaacson B, Oghalai JS, Coker NJ, Vrabec JF. Adult spontaneous CSF otorrhea: correlation with radiographic empty sella. J Oto HNS. 2006; 134: 767-71.

    Patients 38Female 29 (76.3%

    Male 9 (23.7%)

    CSF Otorrhea

    Unilateral 29 (94.7%)

    Bilateral 2 (5.3%)

    Age

    Range (years) 40-75

    Mean (years) 60.3

    BMI

    Range (kg/m2) 24.2-57.8

    Mean (kg/m2) 36.8

    Obese (BMI >30 kg/m2) 30 (78.9%)

    Table 1

    Lumbar Opening Pressure Measurements

    Twenty-two patients underwent postoperative LPOP measurement . Opening pressures ranged from 10-55 cm/H20 with a mean of 19.4 cm/H20. The opening pressure was elevated (>20 cm/H20) in 8 patients (36.4%). Two patients (9.1%) met the modified Dandy LPOP criteria for IIH (>25 cm/H20). All patients with elevated LPOP were female. There were no significant differences in age or BMI in patients with or without elevated LPOP.

    MRIFindings # %

    Total Patients 27

    Empty Sella 10 37.0

    Meckel's Cave Diverticula 9 33.3

    Optic Nerve Dilation 4 14.8

    Tortuous/Elongated Optic Nerve 3 11.1

    Flattened Sclera 1 3.7

    Reverse Cupping of the Optic Disc 0 0

    Evidence of Elevated ICP 13 48.1Table 2

    Intracranial pressure measurement by lumbar puncture opening pressure

    Figure 2: Example of an empty sella (arrow) demonstrated on a coronal T2 MRI in a 56 year-old female patient with SCSFO and elevated LPOP.

    Figure 3: Bilateral Meckel’s cave diverticula (arrows) in a 68 year-old male patient with SCSFO.