One-Year Results of a Low-Profile Endograft in Acute, Complicated Type B Aortic Dissection.

BACKGROUND: The safety and effectiveness of the RelayPro endograft (Terumo Aortic) was assessed for the treatment of acute, complicated type B aortic dissection (TBAD). METHODS: A prospective pivotal trial analyzed a primary end point of all-cause mortality at 30 days. Secondary end points included technical success, major adverse events (disabling stroke, renal failure, and paraplegia/paralysis), endoleaks, patency, rupture, device integrity, false lumen perfusion, reinterventions, aortic expansion, and migration evaluated to 5 years. RESULTS: The study involved 22 United States centers and enrolled 56 patients (mean age, 59.5 ± 11.4 years) from 2017 to 2021; of whom, 73.2% were men and 53.6% were African American. TBAD was complicated by malperfusion of the kidneys (51.8%), lower extremities (35.7%), and viscera (33.9%), and rupture (10.7%). Dissection extended proximally to zones 1/2 (14.3%) and zone 3 (78.6%) and distally to the iliac arteries (67.3%). Most procedures were percutaneous (85.5%). Technical success was 100%. Median hospitalization was 7 days (interquartile range, 5-12 days). All-cause mortality at 30 days was 1.8% (1 of 56; upper 95% CI, 8.2%; P < .0001). Seven major adverse events occurred in 6 patients (10.7%), consisting of paraplegia (n = 3), paraparesis (n = 2), disabling stroke (n = 1), and renal failure (n = 1). All paraplegia/paraparesis resolved with lumbar drainage. Kaplan-Meier analysis estimated a freedom from major adverse events of 89.1% at each interval from 30 days to 3 years. There was 1 endoleak (Type Ia), 2 retrograde dissections, and aortic diameter growth occurred in 2. There has been no rupture, fistula, component separation, patency loss, stenosis, kinking, twisting, bird beak, loss of device integrity, or fracture. CONCLUSIONS: RelayPro is safe and effective in acute, complicated TBAD. Follow-up is ongoing to evaluate longer-term outcomes and durability.

Open Versus Zone 0/1 Endovascular Aortic Repair for Arch Aneurysm: A Propensity Score-Matched Study from the National Clinical Database in Japan.

BACKGROUND: Although open surgical repair (OSR) is the gold standard for treating arch aneurysms, thoracic endovascular aortic repair (TEVAR) may be a less invasive alternative. However, it remains unclear which of the 2 methods yields better outcomes. In this study, we compared the perioperative outcomes of both procedures for arch aneurysms using a nationwide surgical database. METHODS: Data of patients who underwent elective aortic repair for true arch aneurysms were extracted from the National Clinical Database of Japan. Patients who underwent OSR and Zone 0/1 TEVAR were matched in a 1:1 ratio using propensity scores and their mortality and morbidity rates were compared. RESULTS: A total of 2,815 and 1,125 patients underwent OSR and Zone 0/1 TEVAR, respectively. After propensity score matching, 1,058 patients were included in both groups. Compared with OSR, Zone 0/1 TEVAR was associated with a significantly higher incidence of stroke (5.8 vs. 10.0%, P < 0.001) and paraplegia/paraparesis (1.6 vs. 4.4%, P < 0.001). However, there were no significant differences in the 30-day and operative mortality rates between the 2 groups (2.2 vs. 2.7% and 4.5 vs. 5.4%, respectively). In the Zone 0/1 TEVAR group, postoperative computed tomography was performed in 92.4% of patients, and types I and III endoleaks were identified in 6.4% and 1.1% of patients, respectively. CONCLUSIONS: Zone 0/1 TEVAR has higher incidences of stroke and paraplegia/paraparesis than OSR, with a risk of postoperative endoleaks. Resolving these problems is the key for expanding the application of Zone 0/1 TEVAR and in the meantime OSR remains the gold standard for surgically fit patients.

Spinal artery syndrome following kyphoplasty in the setting of a non-compressive extradural cement extravasation: a case report.

INTRODUCTION: Cement extravasation (CE) during vertebroplasty or kyphoplasty for vertebral compression fracture (VCF) is not uncommon, though neurological deficits occur rarely and when paraparesis occurs severe cord compression has been described. We report a case of progressive paraparesis in the setting of non-compressive extradural CE during kyphoplasty with evidence for spinal artery syndrome and neurological recovery after treatment. CASE PRESENTATION: A 77-year-old female with T12 VCF failed conservative treatment and underwent kyphoplasty. In the recovery room, the patient was noted to have bilateral leg weakness, left worse than right, and had urgent CT scan that showed right paracentral CE without cord compression or arterial cement embolization. The patient was transferred to a tertiary hospital and had MRI of the spine that confirmed extradural CE and no cord compression. Because the patient had progression of lower extremity deficits despite medical management, she underwent surgical decompression, cement excision, and spinal fusion with instrumentation. Post op MRI showed T2 hyperintensities in the spinal cord consistent with spinal artery syndrome. One month post op, she had almost complete recovery of her neurological function. DISCUSSION: Spinal artery syndrome may be considered in patients with neurological deficit s/p kyphoplasty even if the extravasated cement does not compress the spinal cord and even if the deficits are worse contralateral to the cement extravasation. If spinal artery syndrome is present and medical management does not improve the deficits, surgery may be indicated even if there is no cord compression.

Thoracoabdominal aortic aneurysm repair based on pre- and postoperative evaluation of the artery of Adamkiewicz.

OBJECTIVES: The aim of this study was to evaluate our experience with thoraco-abdominal aortic aneurysm repair based on the pre- and postoperative evaluation of the artery of Adamkiewicz (AKA). METHODS: Between April 2017 and May 2021, 32 patients who underwent thoracoabdominal aortic repair underwent pre- and postoperative multidetector row computed tomography (MDCT) for AKA evaluation. Based on the identification of the AKA on preoperative MDCT, only 1 critical segmental artery was reattached to the AKA (CSA-AKA). Postoperative MDCT was used to evaluate the patency of the reattached CSA-AKA. RESULTS: Pre- and postoperative MDCT helped identify and visualize the AKA in all patients (100%). In 8 patients, alternative continuity to the AKA developed through collateral circulation. The total number of CSA-AKA with collateral circulation was 48 among the 32 cases; the number of reattached segmental arteries per case was 1.3 ± 0.9 (range, 0-4). The overall rate of patency of the reattached CSA-AKA was 53% (23/43). Three patients exhibited spinal cord injuries (paraplegia, 2; paraparesis, 1). In the 2 paraplegia cases exhibiting partial or complete occlusion of the reattached CSA-AKA, the development of collateral circulation was not visualized via postoperative MDCT. In the paraparesis case, postoperative MDCT helped visualize the development of collateral circulation to the CSA-AKA, resulting in full recovery at discharge. CONCLUSIONS: The study findings suggest that spinal cord injuries do not occur in the presence of a patent preoperatively identified CSA-AKA or the development of collateral circulation. Pre- and postoperative identification of collateral pathways to the AKA may help reveal paraplegia risk factors.

Identifying risk factors for early neurological outcomes following thoracic endovascular aortic repair using the SUMMIT database.

OBJECTIVES: The aim of this study was to assess risk factors for early neurological complications following thoracic endovascular aortic repair (TEVAR) for multiple thoracic aortic diseases using an aggregated dataset. METHODS: The Study to Assess Outcomes After Endovascular Repair for Multiple Throacic Aortic Disease dataset included data from 6 studies evaluating Zenith thoracic endografts. Post hoc analysis identified early (30-day) neurological complications by TEVAR indication and corresponding risk factors. RESULTS: The study included 594 TEVAR patients (67% male; mean age 66 ± 15 years) with thoracic aortic aneurysm (n = 329), ulcer (n = 56), acute (n = 126) or non-acute (n = 33) type B aortic dissection (TBAD) or blunt injury (n = 50). Overall early stroke rate was 3.5% (n = 21). Overall early paraplegia and paraparesis rates were 1.3% (n = 8) and 2.5% (n = 15), respectively. Multivariable analysis identified acute TBAD [versus others, odds ratio (OR) = 3.47, 95% confidence internal (CI): 1.41-8.52) and longer procedural time (OR = 1.33, CI: 1.02-1.73) as early stroke risk factors. Risk factors for paraplegia or paraparesis included more endografts deployed (OR = 2.43, CI: 1.30-4.55), older age (OR = 1.05, CI: 1.01-1.10) and higher preoperative serum creatinine (OR = 1.31, CI: 1.05-1.64). Endografts landing proximal to the left subclavian artery (LSA) increased stroke rate (versus distal to the LSA; 6.8% vs 2.3%, P = 0.014). Intraoperative LSA revascularization was performed in 20.9% of patients with endografts proximal to the LSA; revascularization did not significantly alter stroke rate (8.1% with revascularization vs 6.4% without, P = 0.72). CONCLUSIONS: Acute TBAD and prolonged procedure time increased early stroke risk, while more endografts placed, age and preoperative renal impairment increased early paraplegia or paraparesis risk. For acute TBAD, endograft placement proximal to the LSA, but not LSA patency, increased stroke risk.

Clinical Reasoning: A 57-Year-Old Man With Stepwise Progressive Paraparesis, Sensory Loss, Urinary Retention, and Constipation.

We present the case of a 57-year-old man with protein S deficiency and left leg deep vein thrombosis (DVT) 5 years earlier, who developed stepwise progressive bilateral lower limb weakness, numbness/paresthesia, gait imbalance, hesitancy of micturition, and constipation in the setting of recurrent left common femoral DVT treated with apixaban. Symptoms amplified with Valsalva, corticosteroids, and postlumbar puncture, with longitudinally extensive midthoracic T2-hyperintense lesion extending to the conus associated with hazy holocord enhancement on magnetic resonance imaging (MRI), raising suspicion for spinal dural arteriovenous fistula (sDAVF). Initial digital subtraction angiography (DSA) was negative for sDAVF. However, cerebral spinal fluid (CSF) was herpes simplex virus (HSV)-2 positive, and he was treated with antiviral therapy. Unfortunately, he continued to worsen despite treatment. Repeat neuroimaging 12 months after initial presentation demonstrated persistent lower thoracic/conus lesion in addition to cauda equina enhancement and subtle dorsal T2-hypointense flow voids. We raised red flags (e.g., lack of clinical prodrome, no herpetic rash, no CSF pleocytosis, and rostral extent of the lesion) that suggested the HSV2 nucleic acid detection was perhaps unrelated to the neurologic syndrome. Given the high index of suspicion for sDAVF, we repeated spinal vascular imaging. Spinal MRA demonstrated dilated right dorsal perimedullary veins from T10 to T11. Repeat DSA revealed a right T10 sDAVF. Microsurgical treatment rather than embolization of the fistula was successful without complication, with significant improvement in motor, sphincter, and to a lesser extent sensory function, with residual gait imbalance after inpatient rehabilitation 3 weeks postoperatively.

Spinal Epidural Fistulas-A Separate Entity to Dural Fistulas with Different Angioarchitecture and Treatment Approach.

OBJECTIVE: Spinal epidural arteriovenous fistulas (SEAVFs) are the rarest variety of spinal vascular malformation and are often misdiagnosed as type 1 spinal dural fistula. This retrospective study highlights the salient anatomic differentiating points of these entities and also highlights the importance of a planned endovascular treatment approach using different routes of access. Efficacy of the endovascular treatment at 3 months follow-up was also studied. METHODS: We retrospectively reviewed 11 treated patients with SEAVF. Existence of epidural arteriovenous fistula in all these patients was confirmed by spinal angiography. The Aminoff-Logue Scale score was assigned both before and after the procedure. The statistical results were expressed as percentages, and the preprocedure scale was compared with the postprocedure scale at 3 months by using a nonparametric Wilcoxon signed-rank test. RESULTS: The patients ranged in age from 7 to 53 years, with male predominance. Paraparesis was the commonest symptom, and 1 patient had congestive cardiac failure caused by a large fistula. Location was mostly dorsolumbar with intradural venous reflux into the perimedullary venous system (Castilla type A) noted in 3/11(27%) patients, and the remaining 73% patients had Castilla type B1 with an enlarged epidural venous sac. Therapeutic embolization was performed from arterial, venous, or combined routes. The 3-month postprocedure clinical assessment showed statistically significant (P < 0.004) improvement in the Aminoff-Logue Scale score. CONCLUSIONS: The differentiating points between SEAVF compared with type 1 spinal dural fistula are emphasized. The study also highlights the importance of a good angiographic assessment to best access the fistula by arterial, venous, percutaneous, or combined routes. Endovascular treatment resulted in statistically significant clinical improvement at 3 months follow-up.

Paraparesis and Disseminated Osteolytic Lesions Revealing Cholangiocarcinoma: A Case Report.

Bone metastases in cholangiocarcinoma are uncommon. We report the case of a patient with disseminated osteolytic lesions who was admitted to the Neurology Department for progressive paraparesis. On the computed tomography examination, specific features for cholangiocarcinoma were described, confirmed later by the histopathological aspect of the bone lesions.

Spinal cordectomy for the management of thoracic malignant intraspinal tumors in paraplegic or irreversibly, severely paraparetic patients: A technical remark.

BACKGROUND: Malignant Intramedullary Spinal Cord Tumor are a relatively uncommon entity affecting patients whose prognosis is quickly and relentlessly dismal. Since the '50s Spinal Cordectomy' has been advocated for the surgical management of these conditions, but to date, no standard operative protocol has been reported yet. OBJECTIVE: Although apparently "easy", burdened by virtually no further risk for the neurological function in paraplegic or severely paraparetic patients, SCt conceals notable pitfalls and surgical problems that are to date not yet completely discussed. The objective of the present paper is therefore to report a detailed stepwise description of the surgical technique. METHODS AND RESULTS: SCt addresses the problem of reaching a surgical radicality in patients whom neurological preoperative conditions have already irreversibly declined to a deep nonfunctional motor impairment and whose preoperative Brain MRI scan rules out intracranial seeding. The dural sac along with the radicular pouches must be considered as possible seeding and recurrence locations therefore such structure should be "en-bloc" removed. The cranial medullary end of the resection should be identified on the ground of the preoperative MRI and intraoperatively confirmed with fresh histological examinations ruling out the presence of tumor cells above the cordectomy. Due to the topographic and functional medullary arterial anatomy, no SCt should be performed above T3. The risk of postoperative sagittal imbalance is high and therefore a concurrent posterior vertebral stabilization is required. CONCLUSION: Spinal Cordectomy is a safe and feasible "last chance" treatment to prolong survival in paraplegic or severely paraparetic patients.

Triptan-induced Reversible Cerebral Vasoconstriction Syndrome Presenting With Thunderclap Headache and Paraparesis.

Reversible cerebral vasoconstriction syndrome is a rare clinical syndrome characterized by sudden thunderclap headache often an under diagnosed neurological emergency. It is often provoked by postpartum state or exposure to provocative drugs. We report a rare case of Rizatriptan-induced reversible cerebral vasoconstriction syndrome presenting with thunderclap headache and paraparesis with complete recovery of neurological and imaging findings.

An Unusual Cause of Myelopathy: Ochronotic Spondyloarthropathy With Positive HLA B27.

Ochronosis is a late developing complication of alkaptonuria, a black brownish pigment in the fibrous and cartilaginous tissues. Although most previous studies reported alkaptonuria and back pain due to ochronosis, thoracic myelopathy is an extremely rare complication. In this report, a paraparetic patient who has ochronotic spondiloarthropathy with the presence of HLA B27 antigen is described. He had low back and leg pain and morning stiffness for 5 yrs. Last year, these were followed by tingling, numbness, and weakness the in lower extremities and he was operated on with preliminary diagnosis of prolapsed disc herniation and cord compression. Surgery is suggested for disc herniations related to ochronotic spondyloarthropathy if it is necessary or neurologic symptoms are present. However, his pain and weakness have partially recovered after the operation. After medical and physical treatment, he showed clinically significant improvements. This case report demonstrates that the management of ochronosis needs a multidisciplinary approach with physiologic, neurologic, and psychologic effects and proper treatment may significantly improve functional outcomes in these patients.

Spinal Stenosis with Paraparesis in a Korean Boy with Albright's Hereditary Osteodystrophy: Identification of a Novel Nonsense Mutation in the GNAS.

Children with Albright's hereditary osteodystrophy (AHO) suffering from spinal cord compression are rarely reported worldwide. The association of compressive myelopathy with AHO is not still well known. AHO is a rare heterogeneous group of inherited disorders and results from the GNAS mutation. AHO manifests in two different phenotypes, pseudohypoparathyroidism type Ia (PHP-Ia) and pseudopsedohypoparthyroidim (PPHP), which may happen in the same family members. We present the case of a 15-year-old boy with AHO features, who was later diagnosed with PHP-Ia. He suffered from cervical myelopathy with paraparesis due to spinal stenosis. His mother with AHO phenotype was diagnosed with PPHP without spinal stenosis. Genetic analysis revealed a novel heterozygous nonsense mutation within exon 1 of GNAS(c.49A>T; p.Lys17*) in both of them. This is the first clinically, biochemically, and genetically identified child case of spinal stenosis and paraparesis associated with PHP-Ia, having a novel GNAS mutation in Korea.

Protection of the remaining spinal cord function with intraoperative neurophysiological monitoring during paraparetic scoliosis surgery: a case report.

OBJECTIVE: To demonstrate the usefulness of rectus femoris muscle MEPs monitoring in a paraparetic neuromuscular scoliosis case. METHODS: Multiple monitoring modalities including SEPs, MEPs and EMG were performed for an anterior and posterior correction surgery for a neuromuscular scoliosis patient with no motor and sensory function below the knees. RESULTS: Bilateral tibial nerve SEPs were absent, and no MEPs were recordable from anterior tibialis and abductor hallucis muscles bilaterally at baseline. Robust MEPs were recorded on abductor pollicis brevis and rectus femoris muscles bilaterally. Spinal cord monitoring mainly relied on MEPs from bilateral rectus femoris muscles (RF-MEPs). Twice RF-MEPs were absent following deformity correction and returned after removal of both rods. The patient's remaining spinal cord function was preserved. CONCLUSIONS: Intraoperative neurophysiological monitoring should be used for neuromuscular scoliosis cases with paraparesis if proximal function, such as the rectus femoris muscle, exists.