Eur Arch Otorhinolaryngol DOI 10.1007/s00405-014-3049-8
Lateral sphenoid sinus recess cerebrospinal fluid leak: a case series Nelson Almeida d’Ávila Melo · Bruno Barros Pinto Borges · Pedro Augusto Magliarelli Filho · Maria Dantas Costa Lima Godoy · Larissa Vilela Pereira · Fabio de Rezende Pinna · Richard Louis Voegels
Received: 9 February 2014 / Accepted: 3 April 2014 © Springer-Verlag Berlin Heidelberg 2014
Abstract The lateral recess of the sphenoid sinus is one of the most common sites of meningocele and spontaneous cerebrospinal fluid (CSF) leak. Despite the availability of several techniques for closure of skull base defects occurring in this location, recurrence still poses a major challenge. This report reviews the experience of surgical repair of lateral sphenoid sinus recess CSF leak at a tertiary referral center and provides a brief discussion of this rare lesion. Nine surgeries were performed for six cases of spontaneous lateral sphenoid sinus recess CSF leak (two revisions and one repair of a new defect). Two patients presented with intracranial hypertension (ICH) and four with meningocele or meningoencephalocele. The transpterygoid approach was used in two procedures. A multilayer graft was used in seven cases and a nasoseptal flap in two. Three patients received lumbar or ventricular shunts, and one received acetazolamide for ICH management. Two minor complications were recorded, and the overall surgical success rate was 78 %. We conclude that nasoseptal flaps are a valid option for repair of recurrent CSF leaks, particularly in the lateral sphenoid sinus recess. Furthermore, identification and correction of ICH plays an essential role in the success of treatment in this patient population.
N. A. d. Melo (*) · B. B. P. Borges · P. A. M. Filho · M. D. C. L. Godoy · L. V. Pereira · F. de Rezende Pinna · R. L. Voegels Hospital Das Clínicas Da Universidade de São Paulo, São Paulo, Brazil e-mail: [email protected]
M. D. C. L. Godoy Hospital Das Clínicas Da Universidade de São Paulo, Hospital Do Servidor Publico Estadual de São Paulo, São Paulo, Brazil
Keywords Idiopathic intracranial hypertension · Cerebrospinal fluid rhinorrhea · Meningocele · Sphenoid sinus · Natural orifice endoscopic surgery
Introduction The lateral recess of the sphenoid sinus and the cribriform plate of the ethmoid bone are the most common sites of meningocele and spontaneous cerebrospinal fluid (CSF) leak. Those occurring in the sphenoid sinus originate from a bony dehiscence in the lateral wall of the sphenoid, known as Sternberg’s canal or the lateral craniopharyngeal canal . Several materials and techniques have been employed for closure of minor skull base defects . Nevertheless, recurrences and large defects still pose a major challenge. Furthermore, most of these patients display clinical and radiological signs of intracranial hypertension (ICH) . Use of neurovascular-pedicled nasoseptal flaps for repair of these defects, as described by Hadad  in 2006, has become a promising alternative. This report describes the profile of a series of patients with lateral sphenoid sinus recess CSF leaks, with particular focus on surgical approaches, technical challenges, and leak recurrence.
Methods This case series recruited adult patients who underwent endoscopic endonasal repair of CSF leak at the department of otorhinolaryngology of a large tertiary teaching hospital from 2002 to 2013. Hospital records were searched for all patients with a diagnosis of lateral sphenoid sinus recess CSF leak.
Clinical and radiological data, endoscopy findings, and information on intraoperative and postoperative outcomes were obtained by means of a chart review and patient interviews as necessary.
Report of Cases Six cases of spontaneous lateral sphenoid sinus recess CSF leak were treated surgically at the study facility during the period of interest. Nine surgeries were performed, including two revisions (cases 4 and 5) and repair of a new skull base defect that emerged during follow-up (case 4). Case 1 A 45-year-old woman presented with a history of low-grade, left-sided rhinorrhea. There was no history of trauma and no comorbidities. A computed tomography (CT) of the face showed opacification of the left sphenoid sinus with a bony defect in the lateral recess. Endoscopic surgery was performed in January 2006. Intrathecal fluorescein was administered preoperatively. No fluorescein leak was seen intraoperatively, but a bony defect in the sphenoid sinus and a meningocele in the region of the lateral recess were observed. The meningocele was partially resected and the bony defect repaired with Surgicel® (Ethicon, Fig. 1 Illustration shows the posterior aspect of both nasal septum incisions to confection the nasoseptal flap in a right nasal fossa
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Somerville, NJ), Gelfoam® (Baxter, Hayward, CA), and fibrin glue (Baxter, Vienna, Austria). Postoperatively, the patient was prescribed a high-residue diet, bed rest (strict for 3 days and modified for 1 day), and ceftriaxone and instructed to avoid heavy lifting. As of February 2006, she was free of recurrence. Case 2 A 53-year-old woman with diabetes, hypertension, and dyslipidemia presented with severe headache and high-flow rhinorrhea from the right naris. She denied any trauma. CT of the paranasal sinuses showed opacification and a bony defect in the right sphenoid sinus. Endoscopic surgery was performed in April 2006. Intraoperative CSF leak was observed after intrathecal fluorescein administration, and a bony defect was seen in the lateral sphenoid sinus recess on the right, with no meningocele. The defect was repaired with Surgicel®, fibrin glue, a bovine pericardial patch (Labcor, Belo Horizonte, Brazil), and a middle turbinate mucosa graft. The sphenoid sinus was obliterated with Surgicel®. A lumbar shunt was placed and kept for 48 h postoperatively. As of December 2006, the leak had not recurred. Case 3 A 51-year-old woman presented with a 2-year history of pulsatile frontal headache and spontaneous clear, colorless rhinorrhea. There was no history of trauma, but
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the patient had experienced three episodes of meningitis in the past. Comorbidities included heart failure, hypertension, and obesity. Imaging showed two bony defects (3 and 2 mm) in the superior wall of the lateral sphenoid sinus recess on the left. In March 2011, endoscopic sphenoidotomy was performed, during which a meningocele was detected and partially resected. The bony defects were obliterated with fascia lata grafts placed in an underlay/overlay fashion with fibrin glue between the layers. The lateral recess was filled with fat, Surgicel®, and glue. The postoperative regimen was as in case 1. As of June 2012, the graft remained secure and there was no sign of recurrence. Case 4 A 39-year-old woman with hypertension, obesity, depression, and hypothyroidism presented with spontaneous, unilateral rhinorrhea from the right naris. There was no history of trauma. In 2005, the patient underwent a surgical procedure for repair of meningoencephalocele and CSF leak in the lateral recess of the right sphenoid sinus, followed by revision surgery 18 months later for repair of another leak in the same site. In both procedures, repair was performed with a combination of bovine pericardium, inferior turbinate mucosa, and Surgicel® with fibrin glue between layers. In April 2011, the patient presented with a new complaint of left-sided rhinorrhea. A meningoencephalocele was found in the left sphenoid sinus. After cauterization and complete resection, the bony defect was repaired
with a vascular-pedicled nasoseptal flap based on the posterior septal artery, that is, the posterior branch of the sphenopalatine artery. To confection the flap initially, the nasal septum was infiltrated with 2 % lidocaine with adrenalin 1/200.000. Two parallel incisions were performed in the septal nasal mucosa through right nasal fossa (Fig. 1). The first incision (inferior) was parallel to the posterior edge of the vomer toward the floor of the nasal fossa. Then, it was extended anteriorly between the anterior septum and nasal floor until it reaches the lowest point of the caudal edge of the septum. The second incision (top) was taken starting from the junction between the anterior wall of the sphenoid bone and the nasal septum at a point just below the ostium of the sphenoid. The superior incision was extended anteriorly, parallel to the skull base, respecting 1.0 cm away from the roof of the nasal cavity. This incision was extended up to the level where the anterior end of the vertical portion of the middle turbinate is inserted at the skull base. From this point, the incision was extended in the superior direction toward the nasal dorsum at an angle of approximately 80° (Fig. 2). Upon reaching the nasal dorsum, the incision was extended anteriorly to the upper limit of the caudal edge of the septum. Finally, these two incisions were joined at the front by an incision at the caudal edge of the septum in proximity to the columella. The flap was elevated in subperichondrial and subperiosteal plane to expose the anterior wall of right sphenoid.
Fig. 2 Illustration shows the nasoseptal flap partially elevated from the septum and its limits in a right nasal fossa
Fig. 3 Illustration shows the nasoseptal flap attached to its pedicle, completely free from septum and settled in the nasopharynx in a right nasal fossa
Fig. 4 Illustration shows how the right nasoseptal flap was positioned through the opening in intersphenoid septum reaching the left sphenoid lateral recess defect. Note that the nasal septum was didactically removed to enable viewing of the right nasal cavity
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Fig. 5 Nasal endoscopy showing a meningocele in the right nasal fossa, lateral to the middle turbinate
Carefully, the pedicle of the flap was laterally dissected from the anterior wall of the sphenoid bone to facilitate flap’s rotation (Fig. 1) and the flap positioned in the nasopharynx (Fig. 3). The intersphenoidal septa were removed, and the sphenoid sinuses joined to allow flap positioning. Nasoseptal flap was positioned above the bare floor of the sphenoid sinus floor until reach the contralateral defect (Fig. 4). Thereafter, both sphenoid sinuses were obliterated with fat graft in an overlay fashion. The graft was made to cover the entire sphenoid cavity with the aid of biological adhesive and Surgicel®. The nasal cavity was dressed bilaterally with Merocel® packs (Medtronic, Minneapolis, MN), which were kept in place for 2 days. A lumbar shunt was placed, but was unsuccessful at controlling ICH and was ultimately removed after two failed attempts at repositioning. As a complication, the patient developed meningitis, which resolved after a course of vancomycin and ceftriaxone. As of March 2012, the patient continued to complain of rhinorrhea, but fiberoptic nasal endoscopy showed no evidence of CSF leak. The patient is currently on acetazolamide for the control of intracranial pressure and continues to be followed at the neurology clinic.
Case 5 A 35-year-old woman with hypertension and class I obesity presented with an 18-month history of continuous, right-sided clear liquid rhinorrhea. There was no history of trauma, surgery, or meningitis. Nasal endoscopy revealed a pulsating mass in the right naris (Fig. 5). CT and magnetic resonance imaging (MRI) showed a welldelineated mass lesion with a fluid-filled component and no contrast enhancement occupying the right sphenoid sinus and sphenoethmoidal recess, communicating with the ipsilateral middle cranial fossa (Figs. 6, 7). There were no indirect signs of ICH. Surgery was performed via the endonasal endoscopic transpterygoid approach (Fig. 8). A meningocele with high-flow CSF leak was identified and resected, and a small bony defect in the lateral recess of the right sphenoid sinus was repaired. A multilayer closure was performed with a combined fascia lata, abdominal fat, and middle turbinate mucosa graft with fibrin glue between the layers. On the third postoperative day, the patient developed recurrent CSF leak. Revision surgery was performed, and the defect closed with a posterior nasoseptal flap as described in case 4 based on Hadad technique . At the time of writing, the patient was free of recurrence (Fig. 9). Case 6 A 60-year-old woman presented with a 2-year history of left-sided rhinorrhea and frontal headache. There was no history of trauma, meningitis, alcoholism, or smoking. Comorbidities included epilepsy, obesity, hypertension, dyslipidemia, hyperthyroidism, depression, and cardiac arrhythmia. CT scan of the facial bones showed a small defect in the posterosuperior aspect of the left lateral recess; the ipsilateral sphenoid sinus was completely occupied by hypodense material. MRI revealed signs of ICH, including a partially empty sella and CSF in the trigeminal cave and optic nerve sheaths bilaterally. There was no evidence of meningocele or encephalocele. The patient underwent simultaneous ventriculoperitoneal shunt placement and endoscopic endonasal leak repair via the transpterygoid approach. After intrathecal fluorescein injection and transpterygoid access, the defect was
Fig. 6 Computed tomography scan (CT, obtained with soft tissue window), axial (a) and coronal (b) slices, showing material isodense with soft tissues occupying both sphenoid sinuses and communicating with the middle turbinate through a bone defect
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Fig. 7 Magnetic resonance imaging (MRI) showing a large sphenoidal mass in contact with the middle turbinate and nasal cavity, hypointense on T1-weighted images (a, sagittal) and hyperintense on T2-weighted images (b, axial), consistent with meningocele
Table 1 Clinical conditions of patients at baseline (n = 6) Conditions
Meningocele Meningoencephalocele Intracranial hypertension Meningitis
3 (50) 1 (16.6) 2 (33.3) 1 (16.6)
Cerebrospinal fluid leak
Table 2 Procedures performed in each surgical repair (n = 9)
Fig. 8 Endoscopic transpterygoid approach provides safe and effective access to the lateral sphenoid sinus recess
Fig. 9 Nasoseptal flap covering a bone defect for CSF leak repair
closed with a multilayer graft of temporalis fascia, contralateral inferior turbinate mucosa, Surgicel®, and fibrin glue. Left-sided epistaxis occurred at the end of the procedure and was managed with unilateral placement of a glove finger anterior nasal pack.
Intrathecal fluorescein Transpterygoid approach Fat graft Bovine pericardium Fascia lata graft Temporalis fascia graft Middle turbinate mucosa graft Inferior turbinate mucosa graft Surgicel Nasoseptal flap Fibrin glue Gelfoam
3 (33.3) 2 (22.2) 3 (33.3) 3 (33.3) 2 (22.2) 1 (11.1) 2 (22.2) 3 (33.3) 6 (66.6) 2 (22.2) 9 (100) 1 (11.1)
Anterior nasal packing
Postoperatively, the patient continued to exhibit persistent, low-volume CSF output, which resolved spontaneously after 15 days. The sole complication was synechia, which was divided successfully on an outpatient basis. At 5-month follow-up, there were no signs of recurrence and intracranial pressure was adequately controlled. Two patients presented with signs of intracranial hypertension (ICH) and four with meningocele
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or meningoencephalocele (Table 1). A transpterygoid approach was used in two procedures. Repair was accomplished with a multilayer graft in seven procedures, while a nasoseptal flap was used in two (Table 2). Two patients received lumbar shunt, while another two received ventriculoperitoneal shunt or acetazolamide for control of ICH. Two minor procedure-related complications were recorded; synechia was present in one patient and epistaxis in other. Meningitis in case 4 was probably related to manipulation during attempted repositioning of the lumbar shunt. The overall surgical success rate in this series was 78 % (seven of nine procedures).
Discussion Defects of the lateral recess of the sphenoid sinus pose a major surgical challenge to the ear, nose, and throat specialist, due to their high rate of recurrence (especially when associated with ICH) and difficult anatomy. CSF leaks and meningoceles originating in the lateral sphenoid sinus recess are rare; only 22 cases have been reported in the literature. Obesity, middle age, high intracranial pressure, highly pneumatized sphenoid sinus, and lower success rates after surgical correction are all associated with this condition [2, 5–8]. Sternberg’s canal, a point of incomplete bony fusion during formation of the sphenoid bone located at the posterior portion of the lateral sphenoid wall, laterally and inferiorly to the maxillary nerve, is the structure most commonly associated with this type of meningocele. After craniofacial development, Sternberg’s canal is closed by connective tissue alone and thus constitutes a place of least resistance in the skull base [5, 6]. In some patients, successful CSF leak repair requires correction of ICH (defined as an intracranial pressure of >15–20 cm H2O) . Normal intracranial pressure, which may vary depending on time of day, level of physical activity, and the cardiorespiratory cycle , usually ranges 5–15 cm H2O at the lumbar cistern with the body in supine position. In case 3 of our series, the CSF leak was only corrected after improvement of high CSF pressures. There is no consensus in the literature as to the optimal timing of shunt placement. In case 4, a lumbar shunt was placed in an attempt to control ICH. This condition may have been associated with the development and persistence of two sequentially occurring defects at different sites in the sphenoid sinus. In case 6, ICH was controlled successfully by means of a permanent ventriculoperitoneal shunt. Surgical access to the lateral recess can be obtained by several approaches: conventional neurosurgical approaches, transseptal sphenoidotomy, Le Fort I osteotomy, the
transethmoidal-sphenoidal approach (TESA), and the transethmoidal-sphenoidal-pterygoidal approach (TESPA or TEPSA). Neurosurgical approaches and Le Fort I osteotomy are associated with increased morbidity, prolonged hospital stay, and high cost. Transseptal sphenoidotomy does not provide adequate visualization of the area. The TESA consists of removal of the pterygoid process medial to the vidian nerve and is not useful for repair of lesions lateral to this structure [9–11]. The lateral recess can also be accessed by means of a sphenoidotomy extending laterally through the pterygopalatine space (transpterygopalatine approach), as described by Al-Nashar et al. . Although faster, this technique appears to provide less control of neurovascular structures. Nevertheless, it may be effective in the absence of ICH, as in cases 1 and 2 in this series. Patient 5 in this report had a large meningocele protruding through a small defect in the lateral sphenoid sinus recess, extending intranasally, and a high-flow CSF leak. In this case, the defect was repaired via the TEPSA approach, which consists of the following steps: (a) wide ethmoidectomy and sphenoidectomy, (b) wide antrostomy, (c) localization and cauterization of the sphenopalatine artery at its point of emergence, (d) anterolateral dissection to the neurovascular bundle of the vertical plate of the palatine bone and posterior wall of the maxillary sinus, (e) careful dissection of the fascia, fat, and neurovascular structures in the pterygopalatine fossa, (f) incision into the posterior fascia of the pterygopalatine fossa, (g) partial removal of the pterygoid process, and (h) access to the lateral sphenoid sinus recess [13, 14]. Risks associated with this procedure include injury of the sphenopalatine, vidian, nasoseptal, and pharyngeal arteries and of the nasopalatine, vidian, and pharyngeal nerves. In the present series, epistaxis indicative of arterial injury occurred in only one patient (case 6). No patients developed any signs or symptoms of nerve injury, such as dry eye. Nasoseptal flaps have been used for defect closure at revision surgery in an attempt to ensure better outcomes. Use of the Hadad–Bassagasteguy flap , a pedicled neurovascular flap that consists of septal mucoperiosteal and mucoperichondrial tissue supplied by the posterior septal artery, has been associated with excellent outcomes in reconstruction of large skull base defects after expanded endonasal access [14, 15]. Several graft types have been used for multilayer repair of small bony defects. In his review of the transpterygoid approach, Bolger describes a technique for CSF leak closure with a bone and temporalis fascia graft raised by means of a retroauricular approach . In this respect, nasoseptal grafts are advantageous because they are located entirely within the operative field, thus obviating the need for secondary incisions.
The recurrence rates of lateral recess CSF leaks are high even in the best case series [2, 12, 14]. In a recent study, Tomazic and Stammberger reported a success rate of 60 % for surgical correction of lateral recess leaks versus 95 % success when data for all leak sites were pooled . In the case series reported herein, the success rate thus far was 78 % higher than that reported in the literature, bearing in mind that some patients are still being actively followed.
Conclusion The endoscopic transpterygoid approach provides enhanced visualization of the lateral recess and is feasible even in the presence of large meningocele. Nasoseptal flaps are a valid option for repair of recurrent CSF leaks, particularly in the lateral sphenoid sinus recess. Furthermore, identification and correction of ICH plays an essential role in the success of treatment in this patient population. Clinical trials comparing techniques for surgical access and repair of these skull base defects are extremely necessary.
References 1. Wise SK, Schlosser RJ (2007) Evaluation of spontaneous nasal cerebrospinal fluid leaks. Curr Opin Otolaryngol Head Neck Surg 15(1):28–34 2. Tomazic PV, Stammberger H (2009) Spontaneous CSF-leaks and meningoencephaloceles in sphenoid sinus by persisting Sternberg’s canal. Rhinology 47(4):369–374 3. Blaivie C, Lequeux T, Kampouridis S, Louryan S, Saussez S (2006) Congenital transsphenoidal meningocele: case report and review of the literature. Am J Otolaryngol 27(6):422–424 4. Hadad G, Bassagasteguy L, Carrau RL, Mataza JC, Kassam A, Snyderman CH, Mintz A (2006) A novel reconstructive technique
Eur Arch Otorhinolaryngol after endoscopic expanded endonasal approaches: vascular pedicle nasoseptal flap. Laryngoscope 116(10):1882–1886 5. Schick B, Brors D, Prescher A (2000) Sternberg’s canal–cause of congenital sphenoidal meningocele. Eur Arch Otorhinolaryngol 257(8):430–432 6. Shetty PG, Shroff MM, Fatterpekar GM, Sahani DV, Kirtane MV (2000) A retrospective analysis of spontaneous sphenoid sinus fistula: MR and CT findings. AJNR Am J Neuroradiol 21(2):337–342 7. Bolger WE, Butzin CA, Parsons DS (1991) Paranasal sinus bony anatomic variations and mucosal abnormalities: CT analysis for endoscopic sinus surgery. Laryngoscope 101(1 Pt 1):56–64 8. Clyde BL, Stechison MT (1995) Repair of temporosphenoidal encephalocele with a vascularized split calvarial cranioplasty: technical case report. Neurosurgery 36(1):202–206 9. Reynolds JM, Tomkinson A, Grigg RG, Perry CF (1998) A Le Fort I osteotomy approach to lateral sphenoid sinus encephaloceles. J Laryngol Otol 112(8):779–781 10. Landreneau FE, Mickey B, Coimbra C (1998) Surgical treatment of cerebrospinal fluid fistulae involving lateral extension of the sphenoid sinus. Neurosurgery 42(5):1101–1104 11. Al-Nashar IS, Carrau RL, Herrera A, Snyderman CH (2004) Endoscopic transnasal transpterygopalatine fossa approach to the lateral recess of the sphenoid sinus. Laryngoscope 114(3):528–532 12. Bolger WE, Osenback R (1999) Endoscopic transpterygoid approach to the lateral sphenoid recess. Ear Nose Throat J 78:36–43 13. Bolger WE (2005) Endoscopic transpterygoid approach to the lateral sphenoid recess: surgical approach and clinical experience. Otolaryngol Head Neck Surg 133(1):20–26 14. Pinheiro-Neto CD, Prevedello DM, Carrau RL, Snyderman CH, Mintz A, Gardner P, Kassam A (2007) Improving the design of the pedicled nasoseptal flap for skull base reconstruction: a radio anatomic study. Laryngoscope 117(9):1560–1569 15. Zanation AM, Carrau RL, Snyderman CH, Germanwala AV, Gardner PA, Prevedello DM, Kassam AB (2009) Nasoseptal flap reconstruction of high flow intraoperative cerebral spinal fluid leaks during endoscopic skull base surgery. Am J Rhinol Allergy 23(5):518–521