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Endoscopic transnasal repair of two cases of spontaneous cerebrospinal fluid fistula in the foramen rotundum
Journal of Clinical Neuroscience xxx (2018) xxx–xxx
Contents lists available at ScienceDirect
Journal of Clinical Neuroscience journal homepage: www.elsevier.com/locate/jocn
Case report
Endoscopic transnasal repair of a spontaneous cerebrospinal fluid fistula in the foramen rotundum Benjamin Harley a,⇑, Agadha Wickremesekera a, Neil Tan b, Elgan Davies b, Simon Robinson c, Campbell Baguley c, Peter J. Wormald b a b c
Department of Neurosurgery, Wellington Hospital, Riddiford Street, Newtown, Wellington 6021, New Zealand Department of Ear Nose and Throat, Royal Adelaide Hospital, Port Road, Adelaide, SA 5000, Australia Department of Ear Nose and Throat Surgery, Wellington Hospital, Riddiford Street, Newtown, Wellington 6021, New Zealand
a r t i c l e
i n f o
Article history: Received 17 April 2018 Accepted 4 October 2018 Available online xxxx Keywords: Cerebrospinal fluid Rhinorrhoea Foramen rotundum
a b s t r a c t We report two female patients aged 16 and 33 who presented with spontaneous cerebrospinal fluid (CSF) rhinorrhoea. Beta-2 transferrin was positive in both cases. Initial high-resolution CT showed fluid in the maxillary sinus but no obvious bony defect. MR imaging revealed maxillary sinus cysts with high signal on T2 sequences. Endoscopic transnasal surgery with intrathecal fluorescein was undertaken and in both cases a leak was identified from foramen rotundum and repaired. Both patients are symptom free at 6 months. These cases highlight the rare occurrence of spontaneous CSF leak from the foramen rotundum, and how they can be effectively repaired using the endoscopic transnasal approach. Ó 2018 Elsevier Ltd. All rights reserved.
1. Introduction CSF fistulae of the anterior skull base are abnormal connections between the subarachnoid space and the nasal cavity without a clear initiating event [1]. They are infrequent, making up 6–23% of all cases of CSF rhinorrhoea [2]. The most common manifestation is unilateral rhinorrhoea and diagnosis is often delayed due to the intermittent nature of the leak or confusion with other common pathologies [1]. Diagnosis involves obtaining a sample of the nasal discharge and measuring beta-2 transferrin – a polypeptide with high specificity and sensitivity for CSF [3]. CT imaging may show skull base defects and MRI, especially T2 sequences, can show sites of CSF leak [1]. Due to complications of CSF leak such as meningitis (up to 10% risk per year [4,5]), intracranial abscess and pneumocephalus, surgical repair is recommended [6,7]. While spontaneous CSF leak from the skull base has been extensively reported [8], we could find no reports of CSF leak from the foramen rotundum. 2. Case reports
gested a bony defect in the region of the right posterior ethmoids, but subsequent MRI showed only a mucus cyst in the right maxillary sinus. Initial transnasal surgery failed to identify a leak, however the mucus cyst was resected. Patient had ongoing rhinorrhoea. Repeat MRI 6 months later showed a recurrent maxillary cyst (Fig. 1A). The decision was made to repeat the surgery with pre-operative intrathecal fluorescein. An endoscopic prelacrimal approach to the right maxillary sinus and pterygopalatine fossa (PPF) taken, revealing the cyst (Fig. 2A) with fluorescein (Fig. 2B) within. An aspirate was taken, and was positive for beta-2 transferrin. The mucosa and cyst wall were incised, bone removed and the maxillary nerve identified, which was followed back to the foramen rotundum. In repairing the defect two layers of Duramatrix [Stryker, USA] were cut in a keyhole fashion to create a seal around the maxillary nerve at the foramen rotundum (Fig. 2C), over-sprayed with Duraseal [Integra, United Kingdom] and packed with paraffin soaked ribbon gauze. Saline irrigation and topical steroid were used in the recovery phase. The patient remains free of symptoms at 6 months with no recurrence of maxillary sinus cyst (Fig. 1B).
2.1. Case 1 2.2. Case 2 A 16-year-old female patient presented with episodic clear right rhinorrhoea. Beta-2 transferrin testing was positive. CT sug⇑ Corresponding author. E-mail address: [email protected] (B. Harley).
A 33 year old female presented with a five month history of rhinorrhoea. Endoscopy of the nasal cavity showed no abnormalities, but beta-2 transferrin was positive. High resolution CT did not show any abnormality in the cribriform plate, fovea ethmoidalis
https://doi.org/10.1016/j.jocn.2018.10.020 0967-5868/Ó 2018 Elsevier Ltd. All rights reserved.
Please cite this article in press as: Harley B et al. Endoscopic transnasal repair of a spontaneous cerebrospinal fluid fistula in the foramen rotundum. J Clin Neurosci (2018), https://doi.org/10.1016/j.jocn.2018.10.020
2
Case report / Journal of Clinical Neuroscience xxx (2018) xxx–xxx
Fig. 1. A) Pre-operative axial T1 and sagittal T2 MRI of right maxillary sinus mucus cyst; B) Postop axial T1 and sagittal T2 MRI 6 months after skull base repair.
Fig. 2. A) Intraoperative image of the right maxillary mucus cyst with the middle turbinate being pushed medially by a Freer elevator; B) Intraoperative image showing fluorescein in the maxillary sinus; C) Intraoperative images showing repair of CSF leak, arrow indicates maxillary division of the trigeminal nerve.
Please cite this article in press as: Harley B et al. Endoscopic transnasal repair of a spontaneous cerebrospinal fluid fistula in the foramen rotundum. J Clin Neurosci (2018), https://doi.org/10.1016/j.jocn.2018.10.020
Case report / Journal of Clinical Neuroscience xxx (2018) xxx–xxx
3
or sphenoid, but fluid was noted filling the left maxillary sinus. MRI revealed T2 hyperintense fluid within the maxillary sinus and PPF, thus a diagnosis of dural cyst within the region of the foramen rotundum with extension into the PPF was made. The patient subsequently underwent endoscopic transnasal repair using intrathecal fluorescein administered one hour prior to anaesthetic. A medial maxillectomy was performed to expose the cyst and posterior maxillary wall. After resection of the cyst and exposure of the PPF and infratemporal fossa, the maxillary nerve was identified and followed back to the foramen rotundum. A patch of Duramatrix [Stryker, USA] was cut in a keyhole fashion and wrapped around the nerve, tucked into the PPF and mucosa of the maxillary sinus laid over the repair. The maxillary sinus was packed with paraffin soaked ribbon gauze for a duration of two weeks. After 6 months patient has had no recurrence of symptoms.
less than 10% [1]. The vast majority of surgeries are now carried out by endoscopic approach due to its minimally invasive nature and high success rates [7]. Intrathecal fluorescein may help identify the leak and has become more common. A recent review noted no major reported complications when used within recommended dose ranges [7]. These cases are the first to our knowledge of spontaneous CSF rhinorrhea from the foramen rotundum. They highlight the importance of considering this unusual site of CSF rhinorrhoea in the workup of such patients. Both patients were treated successfully with endoscopic surgery utilising intrathecal fluorescein.
3. Discussion
Conflicts of interest
There is a clear association between spontaneous CSF leak and female gender (72%) [8]. This has been suggested to be due to generally thinner skull base in females but other associations including raised intracranial pressure (40%), obstructive sleep apnoea OSA (43%) and most significantly obesity (80%) have been noted [8]. Neither of our patients were obese nor had confirmed raised intracranial pressure or OSA. Within the sphenoid, spontaneous leaks generally occur in the perisellar region and the lateral recess of the sphenoid sinus. In the lateral recess adjacent to the foramen rotundum, both acquired and congenital factors have been suggested. Thinning of bone caused by on-going pneumatisation of the sphenoid lateral to the foramen rotundum is reported to occur in up to 27% of adults and may contribute to a spontaneous leak, particularly in individuals with anatomical flattening of this region of the skull base (1). It has also been postulated that a persisting lateral craniopharyngeal canal (failure of fusion between the basisphenoid and greater wing of sphenoid, medial to the foramen rotundum) may contribute to the development of a leak [1]. Prominent anomalous arachnoid granulations have also been correlated with areas of bone thinning in the skull base, and impaired CSF absorption raising intracranial pressure may also contribute [1,9]. Spontaneous CSF leak typically does not self-resolve and requires surgical repair [7]. Surgical repair has recurrence rates of
Sources of support None.
The authors declare no conflicts of interest. References [1] Alonso RC, de la Pena MJ, Caicoya AG, Rodriguez MR, Moreno EA, de Vega Fernandez VM. Spontaneous skull base meningoencephaloceles and cerebrospinal fluid fistulas. Radiographics 2013;33:553–70. [2] Schuknecht B, Simmen D, Briner HR, Holzmann D. Nontraumatic skull base defects with spontaneous CSF rhinorrhea and arachnoid herniation: imaging findings and correlation with endoscopic sinus surgery in 27 patients. AJNR Am J Neuroradiol 2008;29:542–9. [3] Gacek RR, Gacek MR, Tart R. Adult spontaneous cerebrospinal fluid otorrhea: diagnosis and management. Am J Otol 1999;20:770–6. [4] La Fata V, McLean N, Wise SK, DelGaudio JM, Hudgins PA. CSF leaks: correlation of high-resolution CT and multiplanar reformations with intraoperative endoscopic findings. AJNR Am J Neuroradiol 2008;29:536–41. [5] Pelosi S, Bederson JB, Smouha EE. Cerebrospinal fluid leaks of temporal bone origin: selection of surgical approach. Skull Base 2010;20:253–9. [6] Zweig JL, Carrau RL, Celin SE, Snyderman CH, Kassam A, Hegazy H. Endoscopic repair of acquired encephaloceles, meningoceles, and meningo-encephaloceles: predictors of success. Skull Base 2002;12:133–9. [7] Nyquist GG, Anand VK, Mehra S, Kacker A, Schwartz TH. Endoscopic endonasal repair of anterior skull base non-traumatic cerebrospinal fluid leaks, meningoceles, and encephaloceles. J Neurosurg 2010;113:961–6. [8] Lobo BC, Baumanis MM, Nelson RF. Surgical repair of sponaneous cerebrospinal fluid leaks: a systematic review. Laryngoscope Invest Otolaryngol 2017;2 (5):215–24. [9] Schlosser RJ, Woodworth BA, Wilensky EM, Grady MS, Bolger WE. Spontaneous cerebrospinal fluid leaks: a variant of benign intracranial hypertension. Ann Otol Rhinol Laryngol 2006;115:495–500.
Please cite this article in press as: Harley B et al. Endoscopic transnasal repair of a spontaneous cerebrospinal fluid fistula in the foramen rotundum. J Clin Neurosci (2018), https://doi.org/10.1016/j.jocn.2018.10.020
Contents lists available at ScienceDirect
Journal of Clinical Neuroscience journal homepage: www.elsevier.com/locate/jocn
Case report
Endoscopic transnasal repair of a spontaneous cerebrospinal fluid fistula in the foramen rotundum Benjamin Harley a,⇑, Agadha Wickremesekera a, Neil Tan b, Elgan Davies b, Simon Robinson c, Campbell Baguley c, Peter J. Wormald b a b c
Department of Neurosurgery, Wellington Hospital, Riddiford Street, Newtown, Wellington 6021, New Zealand Department of Ear Nose and Throat, Royal Adelaide Hospital, Port Road, Adelaide, SA 5000, Australia Department of Ear Nose and Throat Surgery, Wellington Hospital, Riddiford Street, Newtown, Wellington 6021, New Zealand
a r t i c l e
i n f o
Article history: Received 17 April 2018 Accepted 4 October 2018 Available online xxxx Keywords: Cerebrospinal fluid Rhinorrhoea Foramen rotundum
a b s t r a c t We report two female patients aged 16 and 33 who presented with spontaneous cerebrospinal fluid (CSF) rhinorrhoea. Beta-2 transferrin was positive in both cases. Initial high-resolution CT showed fluid in the maxillary sinus but no obvious bony defect. MR imaging revealed maxillary sinus cysts with high signal on T2 sequences. Endoscopic transnasal surgery with intrathecal fluorescein was undertaken and in both cases a leak was identified from foramen rotundum and repaired. Both patients are symptom free at 6 months. These cases highlight the rare occurrence of spontaneous CSF leak from the foramen rotundum, and how they can be effectively repaired using the endoscopic transnasal approach. Ó 2018 Elsevier Ltd. All rights reserved.
1. Introduction CSF fistulae of the anterior skull base are abnormal connections between the subarachnoid space and the nasal cavity without a clear initiating event [1]. They are infrequent, making up 6–23% of all cases of CSF rhinorrhoea [2]. The most common manifestation is unilateral rhinorrhoea and diagnosis is often delayed due to the intermittent nature of the leak or confusion with other common pathologies [1]. Diagnosis involves obtaining a sample of the nasal discharge and measuring beta-2 transferrin – a polypeptide with high specificity and sensitivity for CSF [3]. CT imaging may show skull base defects and MRI, especially T2 sequences, can show sites of CSF leak [1]. Due to complications of CSF leak such as meningitis (up to 10% risk per year [4,5]), intracranial abscess and pneumocephalus, surgical repair is recommended [6,7]. While spontaneous CSF leak from the skull base has been extensively reported [8], we could find no reports of CSF leak from the foramen rotundum. 2. Case reports
gested a bony defect in the region of the right posterior ethmoids, but subsequent MRI showed only a mucus cyst in the right maxillary sinus. Initial transnasal surgery failed to identify a leak, however the mucus cyst was resected. Patient had ongoing rhinorrhoea. Repeat MRI 6 months later showed a recurrent maxillary cyst (Fig. 1A). The decision was made to repeat the surgery with pre-operative intrathecal fluorescein. An endoscopic prelacrimal approach to the right maxillary sinus and pterygopalatine fossa (PPF) taken, revealing the cyst (Fig. 2A) with fluorescein (Fig. 2B) within. An aspirate was taken, and was positive for beta-2 transferrin. The mucosa and cyst wall were incised, bone removed and the maxillary nerve identified, which was followed back to the foramen rotundum. In repairing the defect two layers of Duramatrix [Stryker, USA] were cut in a keyhole fashion to create a seal around the maxillary nerve at the foramen rotundum (Fig. 2C), over-sprayed with Duraseal [Integra, United Kingdom] and packed with paraffin soaked ribbon gauze. Saline irrigation and topical steroid were used in the recovery phase. The patient remains free of symptoms at 6 months with no recurrence of maxillary sinus cyst (Fig. 1B).
2.1. Case 1 2.2. Case 2 A 16-year-old female patient presented with episodic clear right rhinorrhoea. Beta-2 transferrin testing was positive. CT sug⇑ Corresponding author. E-mail address: [email protected] (B. Harley).
A 33 year old female presented with a five month history of rhinorrhoea. Endoscopy of the nasal cavity showed no abnormalities, but beta-2 transferrin was positive. High resolution CT did not show any abnormality in the cribriform plate, fovea ethmoidalis
https://doi.org/10.1016/j.jocn.2018.10.020 0967-5868/Ó 2018 Elsevier Ltd. All rights reserved.
Please cite this article in press as: Harley B et al. Endoscopic transnasal repair of a spontaneous cerebrospinal fluid fistula in the foramen rotundum. J Clin Neurosci (2018), https://doi.org/10.1016/j.jocn.2018.10.020
2
Case report / Journal of Clinical Neuroscience xxx (2018) xxx–xxx
Fig. 1. A) Pre-operative axial T1 and sagittal T2 MRI of right maxillary sinus mucus cyst; B) Postop axial T1 and sagittal T2 MRI 6 months after skull base repair.
Fig. 2. A) Intraoperative image of the right maxillary mucus cyst with the middle turbinate being pushed medially by a Freer elevator; B) Intraoperative image showing fluorescein in the maxillary sinus; C) Intraoperative images showing repair of CSF leak, arrow indicates maxillary division of the trigeminal nerve.
Please cite this article in press as: Harley B et al. Endoscopic transnasal repair of a spontaneous cerebrospinal fluid fistula in the foramen rotundum. J Clin Neurosci (2018), https://doi.org/10.1016/j.jocn.2018.10.020
Case report / Journal of Clinical Neuroscience xxx (2018) xxx–xxx
3
or sphenoid, but fluid was noted filling the left maxillary sinus. MRI revealed T2 hyperintense fluid within the maxillary sinus and PPF, thus a diagnosis of dural cyst within the region of the foramen rotundum with extension into the PPF was made. The patient subsequently underwent endoscopic transnasal repair using intrathecal fluorescein administered one hour prior to anaesthetic. A medial maxillectomy was performed to expose the cyst and posterior maxillary wall. After resection of the cyst and exposure of the PPF and infratemporal fossa, the maxillary nerve was identified and followed back to the foramen rotundum. A patch of Duramatrix [Stryker, USA] was cut in a keyhole fashion and wrapped around the nerve, tucked into the PPF and mucosa of the maxillary sinus laid over the repair. The maxillary sinus was packed with paraffin soaked ribbon gauze for a duration of two weeks. After 6 months patient has had no recurrence of symptoms.
less than 10% [1]. The vast majority of surgeries are now carried out by endoscopic approach due to its minimally invasive nature and high success rates [7]. Intrathecal fluorescein may help identify the leak and has become more common. A recent review noted no major reported complications when used within recommended dose ranges [7]. These cases are the first to our knowledge of spontaneous CSF rhinorrhea from the foramen rotundum. They highlight the importance of considering this unusual site of CSF rhinorrhoea in the workup of such patients. Both patients were treated successfully with endoscopic surgery utilising intrathecal fluorescein.
3. Discussion
Conflicts of interest
There is a clear association between spontaneous CSF leak and female gender (72%) [8]. This has been suggested to be due to generally thinner skull base in females but other associations including raised intracranial pressure (40%), obstructive sleep apnoea OSA (43%) and most significantly obesity (80%) have been noted [8]. Neither of our patients were obese nor had confirmed raised intracranial pressure or OSA. Within the sphenoid, spontaneous leaks generally occur in the perisellar region and the lateral recess of the sphenoid sinus. In the lateral recess adjacent to the foramen rotundum, both acquired and congenital factors have been suggested. Thinning of bone caused by on-going pneumatisation of the sphenoid lateral to the foramen rotundum is reported to occur in up to 27% of adults and may contribute to a spontaneous leak, particularly in individuals with anatomical flattening of this region of the skull base (1). It has also been postulated that a persisting lateral craniopharyngeal canal (failure of fusion between the basisphenoid and greater wing of sphenoid, medial to the foramen rotundum) may contribute to the development of a leak [1]. Prominent anomalous arachnoid granulations have also been correlated with areas of bone thinning in the skull base, and impaired CSF absorption raising intracranial pressure may also contribute [1,9]. Spontaneous CSF leak typically does not self-resolve and requires surgical repair [7]. Surgical repair has recurrence rates of
Sources of support None.
The authors declare no conflicts of interest. References [1] Alonso RC, de la Pena MJ, Caicoya AG, Rodriguez MR, Moreno EA, de Vega Fernandez VM. Spontaneous skull base meningoencephaloceles and cerebrospinal fluid fistulas. Radiographics 2013;33:553–70. [2] Schuknecht B, Simmen D, Briner HR, Holzmann D. Nontraumatic skull base defects with spontaneous CSF rhinorrhea and arachnoid herniation: imaging findings and correlation with endoscopic sinus surgery in 27 patients. AJNR Am J Neuroradiol 2008;29:542–9. [3] Gacek RR, Gacek MR, Tart R. Adult spontaneous cerebrospinal fluid otorrhea: diagnosis and management. Am J Otol 1999;20:770–6. [4] La Fata V, McLean N, Wise SK, DelGaudio JM, Hudgins PA. CSF leaks: correlation of high-resolution CT and multiplanar reformations with intraoperative endoscopic findings. AJNR Am J Neuroradiol 2008;29:536–41. [5] Pelosi S, Bederson JB, Smouha EE. Cerebrospinal fluid leaks of temporal bone origin: selection of surgical approach. Skull Base 2010;20:253–9. [6] Zweig JL, Carrau RL, Celin SE, Snyderman CH, Kassam A, Hegazy H. Endoscopic repair of acquired encephaloceles, meningoceles, and meningo-encephaloceles: predictors of success. Skull Base 2002;12:133–9. [7] Nyquist GG, Anand VK, Mehra S, Kacker A, Schwartz TH. Endoscopic endonasal repair of anterior skull base non-traumatic cerebrospinal fluid leaks, meningoceles, and encephaloceles. J Neurosurg 2010;113:961–6. [8] Lobo BC, Baumanis MM, Nelson RF. Surgical repair of sponaneous cerebrospinal fluid leaks: a systematic review. Laryngoscope Invest Otolaryngol 2017;2 (5):215–24. [9] Schlosser RJ, Woodworth BA, Wilensky EM, Grady MS, Bolger WE. Spontaneous cerebrospinal fluid leaks: a variant of benign intracranial hypertension. Ann Otol Rhinol Laryngol 2006;115:495–500.
Please cite this article in press as: Harley B et al. Endoscopic transnasal repair of a spontaneous cerebrospinal fluid fistula in the foramen rotundum. J Clin Neurosci (2018), https://doi.org/10.1016/j.jocn.2018.10.020