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A Rare Case Report of Primary Spontaneous Transclival CSF Rhinorrhoea

Year: 2024
Type: article
Study design: Case Report
Abstract: Sir, The causes of cerebrospinal fluid (CSF) rhinorrhoea are divided into primary and secondary categories. Primary CSF rhinorrhoea can be spontaneous or congenital. Secondary CSF rhinorrhoea occurs because of trauma, neoplasm, or iatrogenic reasons. The most serious complication of CSF rhinorrhoea is meningitis which can be lethal and hence early surgical repair is advised.[1] Primary spontaneous CSF rhinorrhoea occurs only in 3–4% of cases. Even rarer are the cases with primary clival defect and to the best of our knowledge, only 10 cases have been reported in the literature till now. The clivus is present near the center of the skull base in close proximity to various vital structures like brainstem, cranial nerves, vessels, etc. Surgical treatment of this region is challenging, particularly given the possibility of neurovascular injury. A rare case of an adult with a history of CSF rhinorrhoea due to clival defect is presented that was successfully managed by the endoscopic transsphenoidal approach. A 59-year-old male patient presented with clear liquid discharge from both the nostrils (left > right) for a month. The discharge used to increase in sitting position and decreases on lying down position. There was no recent history of fever, trauma, headache, vomiting, or past surgery. The patient had a past history of CSF rhinorrhoea from left nostril 2 years back complicated by meningitis that was managed conservatively with antibiotics at a local hospital. The patient did not have any neurologic deficit. CSF rhinorrhoea was confirmed using the beta-2 transferrin test. MRI of the brain and CT Cisternography were done in 2017 that had revealed skull base defect however no surgical intervention was carried out. Current CT and MRI imaging revealed a bony defect in the clivus [Figure 1]. The patient was counseled and advised for endoscopic transsphenoidal CSF leak repair.Figure 1: (a) CT brain reveals a bony defect in the posterior wall of the sphenoid sinus along the clivus, (b) MRI brain cisternography [CISS sequence] shows the presence of CSF in the sphenoid sinus. A black star sign is placed just above the communication site between the prepontine cistern and the sphenoid sinusThe patient underwent endoscopic transsphenoidal CSF leak repair. Clival defect was identified during the operation and confirmed using neuronavigation [Figure 2].Figure 2: Clival bony defect showing pre-pontine cisternThe margins of the defect were smoothened and repaired with a 3 layered graft; initially fat used as a bath plug to plug the defect and the second layer of an on lay tensor fascia latae/fat (fat and fascia harvested from the thigh) reinforced with fibrin glue. This was in turn covered with Hadad-Bassagasteguy nasoseptal flap. Foley’s Catheter with an inflated balloon was kept in over the nasoseptal flap and removed after 5 days [Figure 3].Figure 3: Surgical steps: A 3-layered graft plugged into the defect followed by Hadad flap, Gel foam, and Foley’s CatheterCSF rhinorrhoea can occur due to disruption in the arachnoid and dura mater, associated with an osseous defect. If untreated, CSF rhinorrhoea can lead to life-threatening meningitis and other complications. CSF rhinorrhoea has been subdivided into “high pressure,” usually due to the tumors and hydrocephalous and “low pressure” caused by congenital abnormalities or osteomyelitis as per Ommaya et al.[2] Head injury is the most common cause of CSF rhinorrhoea (approximately 80%) while iatrogenic causes stand second with 16% cases. Only 3–4% of cases of CSF rhinorrhoea are spontaneous.[3] For primary spontaneous CSF fistulas, there is a marked predominance for the sphenoid sinus (60%).[4] The localization of leak at clivus is extremely rare in a case of primary spontaneous CSF rhinorrhoea and to the best of our best knowledge, only 10 cases have been reported in the literature till now. Coiteiro et al. had published two reports of CSF rhinorrhoea due to clival defect in 1995, that were managed by transsphenoidal surgery.[5] As per Ramos et al., a clival defect was seen in one case of Marfan syndrome.[6] Oleś published a case of recurrent meningitis due to the posterior clivus framework in 2015.[7] Codina Aroca et al. published two cases of spontaneous transclival CSF rhinorrhoea.[8] Asad et al. reported a case of clival defect with CSF leak into the sphenoid sinus.[9] In 2017, Tandon et al. reported a case of a mid-clival defect that was treated by sublabial rhinoseptal transsphenoidal repair.[10] Ahmad et al. reported a case of a mid-clival bony defect in a 50-year-old male patient, that was corrected by endonasal surgery.[11] Recently in 2019, Ahmet Naci Emecen et al. reported a case of clival defect which was managed conservatively with appropriate antibiotics.[12] Meningitis is seen in all the cases mentioned in the literature. Primary spontaneous CSF transclival rhinorrhoea can occur because of various anatomical and functional factors. Anatomically, excessive pneumatization of the sphenoid bone can leads to the weakening of the bone posteriorly and disruption of the dura and arachnoid. As a functional factor, a pressure gradient produced by continuous CSF pressure may lead to clival fenestration.[13] In this case, a 3.8 × 2.6 mm size focal bony defect was noted in the posterior wall of sphenoid sinus along the clivus, communicating with the prepontine cistern. It was confirmed intraoperatively. In our case, it is likely that the arachnoid and dura would have bulged and later ruptured due to the continuous pressure gradient. The high success rate of a CSF leak repair surgery depends on the accurate localization of defects preoperatively and intraoperatively. Preoperative MRI and CT cisternography helped in localizing the defect and neuronavigation helped us in localization during the surgery. Appropriate usage of graft materials during surgery is also important. The layering and tucking of the defect with Fat-Fascia latae-Fat helped in the plugging of the defect smoothly. The plugging of graft helps to hold the graft in place. Additional reinforcements with Hadad-Bassagasteguy nasoseptal pedicle flap and Gelfoam helps in avoiding any future recurrences. A Foley’s catheter to stabilize the whole system in the immediate postoperative period increases the successful repair. A timely surgery helped us in avoiding the complication of meningitis. There was no further leak at a 2-month follow-up. Primary spontaneous CSF rhinorrhoea from a clival defect is a very rare condition. Accurate and timely diagnosis as well as early multilayer repair by endoscopic transsphenoidal approach resulted in a successful outcome. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.
DOI: 10.4103/ni.ni_673_20
Language: English
Subfield: Surgery
Field: Medicine
Open Access status: hybrid
OpenAlex ID: W4409106103
FWCI:
Cites: 12
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Related to: 10