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      Spinal dural arteriovenous fistula rupture after Rathke’s cleft cyst endoscopic resection: Case report and literature review

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          Abstract

          Background:

          Spinal dural arteriovenous fistula (SDAVF) is the most frequent vascular malformation of the spine and accounts for approximately 70% of all vascular spinal malformations. In rare cases, SDAVF rupture and subsequent subarachnoid hemorrhage or intramedullary hematoma may occur. The aim of this article is to present a fatal case of SDAVF rupture after a Rathke’s cleft cyst (RCC) endoscopic resection.

          Case Description:

          An 80-year-old female was referred to our hospital with a clinical presentation of bilateral reduction in visual acuity, bitemporal hemianopsia, and sellar magnetic resonance imaging (MRI) highly suggestive of RCC. After the first endonasal endoscopic surgery, the cyst was partially removed and vision improved. No signs of cerebrospinal fluid (CSF) leak were observed. After 1 year, the patient returned because of RCC recurrence and decreased visual acuity. In the second procedure, the lesion was totally resected and CSF leak was observed. A nasoseptal flap was rotated to cover the skull base defect. The patient developed subtle paraparesis followed by paraplegia on the 4 th postoperative day. The dorsal spine MRI revealed a T3-T4 intramedullary hematoma. A dorsal laminectomy was performed and a SDAVF was observed. During microsurgery, at the right T3 nerve root level, an arteriovenous shunting point was identified, coagulated, and divided. The intramedullary hematoma was evacuated. The patient developed neurogenic and septic shock and died.

          Conclusion:

          Venous hypertension, venous wall fragility, and venous thrombosis seem to be the main factors involved in SDAVF rupture. In this particular case, reduction of the extravascular pressure and sudden variation in the pressure gradient caused by sustained CSF leak, also appeared to play an important role in SDAVF rupture. It may represent one more complication related to radical resection of RCC.

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          Most cited references24

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          Spinal dural arteriovenous fistulas.

          Spinal dural arteriovenous (AV) fistulas are the most commonly encountered vascular malformation of the spinal cord and a treatable cause for progressive para- or tetraplegia. They most commonly affect elderly men and are classically found in the thoracolumbar region. The AV shunt is located inside the dura mater close to the spinal nerve root where the arterial blood from a radiculomeningeal artery enters a radicular vein. The increase in spinal venous pressure leads to decreased drainage of normal spinal veins, venous congestion, and the clinical findings of progressive myelopathy. On MR imaging, the combination of cord edema, perimedullary dilated vessels, and cord enhancement is characteristic. Therapy has to be aimed at occluding the shunting zone, either by superselective embolization with a liquid embolic agent or by a neurosurgical approach. Following occlusion of the fistula, the progression of the disease can be stopped and improvement of symptoms is typically observed.
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            Endoscopic reconstruction of the cranial base using a pedicled nasoseptal flap.

            Reconstruction of the cranial base using vascularized tissue promotes rapid and complete healing, thus avoiding complications caused by persistent communication between the cranial cavity and the sinonasal tract. The Hadad-Bassagasteguy flap (HBF), a neurovascular pedicled flap of the nasal septum mucoperiosteum and mucoperichondrium based on the nasoseptal artery, seems to be advantageous for the reconstruction of the cranial base after endonasal cranial base surgery. We performed a retrospective review of patients who underwent endonasal cranial base surgery at the University of Pittsburgh Medical Center from January 30, 2006 to January 30, 2007, identifying patients who experienced reconstruction with a vascularized septal mucosal flap (HBF). We analyzed the demographic data, pathological characteristics, site and extent of resection, use of cerebrospinal fluid (CSF) diversion techniques, and outcome. Seventy-five patients who underwent endonasal cranial base endoscopic surgery received repair with the HBF. In this population, we encountered eight postoperative CSF leaks (10.66%), all in patients who required intra-arachnoidal dissection. When we correct the statistical analysis to include only patients with intra- arachnoidal lesions, the postoperative CSF leak rate is 14.5% (eight of 55 patients). It is notable that six CSF (33%) leaks occurred in our first 25 repairs, whereas we encountered only two postoperative leaks (4%) in the last 50 patients. The corrected CSF leak rate, considering only intra-arachnoidal lesions, was two (5.4%) of 37 patients. This improvement in the CSF leak rate reflects our growing experience and comfort with this reconstructive technique. All of our failures could be matched to a specific technical mistake. In addition, we modified the flap-harvesting technique to allow for staged procedures and the removal of caudal lesions. These special circumstances require storage of the flap in the antrum during the removal of caudal lesions, and suturing of the flap in its original position for staged procedures. One patient experienced a posterior nose bleed from the posterior nasal artery. This was controlled with bipolar electrocautery, thereby preserving the flap blood supply. We encountered no infectious or wound complications in this series of patients. The donor site accumulates crusting, which requires debridement until mucosalization is complete; this usually occurs 6 to 12 weeks after surgery. The HBF is a versatile and reliable reconstructive technique for repairing defects of the anterior, middle, clival, and parasellar cranial base. Its use has resulted in a significant decrease in our incidence of CSF leaks after endonasal cranial base surgery. Attention to technical details is of paramount importance to achieve the best outcomes.
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              Modified classification of spinal cord vascular lesions.

              The literature on spinal vascular malformations contains a great deal of confusing terminology. Some of the nomenclature is inconsistent with the lesions described. Based on the experience of the senior author (R.F.S.) in the treatment of more than 130 spinal cord vascular lesions and based on a thorough review of the relevant literature, the authors propose a modified classification system for spinal cord vascular lesions. Lesions are divided into three primary or broad categories: neoplasms, aneurysms, and arteriovenous lesions. Neoplastic vascular lesions include hemangioblastomas and cavernous malformations, both of which occur sporadically and familially. The second category consists of spinal aneurysms, which are rare. The third category, spinal cord arteriovenous lesions, is divided into arteriovenous fistulas and arteriovenous malformations (AVMs). Arteriovenous fistulas are subdivided into those that are extradural and those that are intradural, with intradural lesions categorized as either dorsal or ventral. Arteriovenous malformations are subdivided into extradural-intradural and intradural malformations. Intradural lesions are further divided into intramedullary, intramedullary-extramedullary, and conus medullaris, a new category of AVM. This modified classification system for vascular lesions of the spinal cord, based on pathophysiology, neuroimaging features, intraoperative observations, and neuroanatomy, offers several advantages. First, it includes all surgical vascular lesions that affect the spinal cord. Second, it guides treatment by classifying lesions based on location and pathophysiology. Finally, it eliminates the confusion produced by the multitude of unrelated nomenclatural terms found in the literature.
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                Author and article information

                Contributors
                Journal
                Surg Neurol Int
                Surg Neurol Int
                Surgical Neurology International
                Scientific Scholar (USA )
                2229-5097
                2152-7806
                2021
                06 September 2021
                : 12
                : 455
                Affiliations
                [1 ]Department of Neurosurgery Pedro Ernesto University Hospital, Rio de Janeiro State University, Rio de Janeiro, Brazil.
                [2 ]Department of Endocrine, Pedro Ernesto University Hospital, Rio de Janeiro State University, Rio de Janeiro, Brazil.
                Author notes
                [* ] Corresponding author: Jefferson Trivino-Sanchez, Department of Neurosurgery, Pedro Ernesto University Hospital, Rio de Janeiro State University, Rio de Janeiro, Brazil. jeff_sts2@ 123456hotmail.com
                Article
                10.25259/SNI_654_2021
                10.25259/SNI_654_2021
                8492439
                34621570
                aa704742-80b2-4ee7-93e8-caa11bd75f58
                Copyright: © 2021 Surgical Neurology International

                This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-Share Alike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.

                History
                : 30 June 2021
                : 11 August 2021
                Categories
                Case Report

                Surgery
                cerebrospinal fluid fistula,rathke’s cleft cyst,rupture,spinal dural arteriovenous fistula,transsphenoidal resection

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