Concomitant spinal dural arteriovenous fistula and nodular fasciitis in an adolescent: case report

Background Spinal dural arteriovenous fistula (SDAVF) usually occurs during the 4th to 6th decades of life, and adolescent SDAVF is rarely reported. SDAVF arising around a tumor is also rare, and reported tumors are mostly schwannoma and lipoma. Case presentation We reported a 16-year-old male presented with progressive weakness and numbness of lower limbs for 3 months. A SDAVF was found, which was fed by right radicular arteries from segmental artery at L2 level and drained retrogradely into perimedullary veins. A concomitant spinal extradural nodular fasciitis at right L1/L2 intervertebral foramen was also noted. The SDAVF was completely obliterated by endovascular treatment and the tumor was debulked. The patient recovered well after the procedures. Conclusions Our case report suggests SDAVF can occur in adolescent. The concomitant presence with a nodular fasciitis indicates that although it usually arises in subcutaneous tissue but can rarely form on the dura of spine.

Radiation Dose and Fluoroscopy Time of Diagnostic Angiography in Patients with Spinal Dural Arteriovenous Fistula

Purpose Spinal dural arteriovenous fistulas (SDAVFs) represent the most common indication for a spinal angiography. The diagnostic reference level (DRL) for this specific endovascular procedure is still to be determined. This single-center study provides detailed dosimetrics of diagnostic spinal angiography performed in patients with SDAVFs. Methods Retrospective analysis of all diagnostic spinal angiographies between December 2011 and January 2021. Only patients with an SDAVF who had baseline magnetic resonance angiography (MRA), diagnostic digital subtraction angiography (DSA), treatment and follow-up at this institution were included. Dose area product (DAP, Gy cm2) and fluoroscopy time were compared between preoperative and postoperative angiographies, according to SDAVF locations (common versus uncommon), MRA results at baseline (positive versus negative) and DSA protocols (low-dose, mixed-dose, normal-dose). The 75th percentile of the DAP distribution was used to define the local DRL. Results A total of 62 spinal angiographies were performed in 25 patients with SDAVF. Preoperative angiographies (30/62, 48%) yielded a significantly higher DAP and longer fluoroscopy time when compared to postoperative angiographies (32/62, 53%) (p < 0.01). The local DRL was 329.41 Gy cm2 for a nonspecific (n = 62), 395.59 Gy cm2 for a preoperative and 138.6 Gy cm2 for a postoperative spinal angiography. Preoperative angiography of uncommonly located SDAVFs yielded a significantly longer fluoroscopy time (p = 0.02). The MRA-based fistula detection had no significant impact on dosimetrics (p > 0.05). A low-dose protocol yielded a 61% reduction of DAP. Conclusion The results of the present study suggest novel DRLs for spinal angiography in patients with SDAVF. Dedicated low-dose protocols enable radiation dose optimization in these procedures.

Mimicking Amyotrophic Lateral Sclerosis: A Case of a Spinal Dural Arteriovenous Fistula

A number of conditions can mimic amyotrophic lateral sclerosis (ALS), which are in general excluded by neurophysiological and neuroimaging investigation. We present a novel mimicking disorder. A 58-year-old male, without relevant past medical history, presented with a 7-year history of progressive paraparesis. On examination, he had bilateral thigh atrophy, fasciculations, and asymmetric paraparesis (severe on the left side). Upper motor neuron signs were present in the lower limbs, with normal sensory examination. Needle EMG disclosed mild chronic neurogenic changes in the lower limbs. Brain and spinal cord neuroimaging was normal, namely, in the dorso-lumbar segment. Lumbar puncture showed mild hyperproteinorachia. Diagnosis of slowly progressive (possible) ALS was established. One year later, he required a bilateral support to walk, and neurological examination revealed weak tendon reflexes, abnormal pinprick, and proprioceptive sensation in the legs. Repeated lumbar MRI showed an extensive spinal cord oedema from T7 to the conus with multiple perimedullary vessel flow voids suggestive of a vascular malformation. Conventional angiography revealed a spinal dural arteriovenous fistula in L2–L3 with the left L4 lumbar branch as the afferent artery. Dural arteriovenous fistula is the most common vascular malformation of the spinal cord, despite being rare. It leads to arterialization of spinal veins, causing venous hypertension, spinal cord oedema, and ischaemia. The clinical picture includes a stepwise, sometimes fluctuant, myeloradiculopathy. In this case, EMG changes did not meet Awaji criteria. This case reinforces the need to critically follow atypical cases to ascertain clinical progression in patients with suspected ALS.

Intraoperative Spinal Angiography during Microsurgical Occlusion of Spinal Dural Arteriovenous Fistula within the Hybrid Operation Room

Background Spinal dural arteriovenous fistula (SDAVF) is a rare cause of progressive myelopathy in predominantly middle-aged men. Treatment modalities include surgical obliteration and endovascular embolization. In surgically treated cases, failure of obliteration is reported in up to 5%. The aim of this technical note is to present a safe procedure with complete SDAVF occlusion, verified by intraoperative digital subtraction angiography (DSA). Methods We describe four patients with progressive leg weakness who underwent surgical obliteration of SDAVF with spinal intraoperative DSA in the prone position after cannulation of the popliteal artery. All surgeries took place in our hybrid operating room (OR) and were accompanied by electrophysiologic monitoring. Surgeries and cannulation of the popliteal artery were performed in the prone position. Ultrasound was used to guide the popliteal artery puncture. A 5-Fr sheath was inserted and the fistula was displayed using a 5-Fr spinal catheter. Spinal intraoperative DSA was performed prior to and after temporary clipping of the fistula point as well after the final SDAVF occlusion. Results The main feeder of the SDAVF fistula in the first patient arose from the right T11 segmental artery, which also supplied the artery of Adamkiewicz. The second patient initially underwent endovascular treatment and deteriorated 5 months later due to recanalization of the SDAVF via a small branch of the T12 segmental artery. The third and fourth cases were primarily scheduled for surgical occlusion. Access through the popliteal artery for spinal intraoperative DSA proved to be beneficial and safe in the hybrid OR setting, allowing the sheath to be left in place during the procedure. During exposure and after temporary and permanent occlusion of the fistulous point, intraoperative indocyanine green (ICG) video angiography was also performed. In one case, the addition of intraoperative DSA showed failure of fistula occlusion, which was not visible with ICG angiography, leading to repositioning of the clip. Complete fistula occlusion was documented in all cases. Conclusion Spinal intraoperative DSA in the prone position is a feasible and safe intervention for rapid localization and confirmation of surgical SDAVF occlusion.

Acute neurological deterioration after surgical interruption of spinal dural arteriovenous fistulas: clinical characteristics, possible predictors, and treatment. Patient series

BACKGROUND Acute neurological deterioration develops paradoxically in some patients after obliteration of a spinal dural arteriovenous fistula (SDAVF), with thrombosis of the spinal cord veins as its primary cause. The authors aimed to clarify the clinical and radiological characteristics of acute deterioration to identify high-risk patients. They also discussed the optimal treatment for this complication. OBSERVATIONS Ten patients with SDAVF presenting with congestive myelopathy who received microsurgical interruption were retrospectively reviewed. Severe myelopathy developed in three patients on postoperative days 1 to 3. Anticoagulation therapy was effective; however, discontinuing anticoagulants under residual spinal cord congestion caused redeterioration. These patients were characterized by significantly extended transit time on angiography and significant prolongation of spinal cord congestion. Acute deterioration exhibited a strong correlation with transit time (coefficient, 0.825; p = 0.006) and a strong correlation with spinal cord edema before surgery (coefficient, 0.656; p = 0.040). LESSONS Acute deterioration after SDAVF treatment is likely to develop in patients with severe venous outflow impairment. Its pathology is prolonged spinal cord congestion caused by postoperative venous thrombosis and preexistent severe venous outflow impairment. Anticoagulation treatment should be continued for patients with acute deterioration until the resolution of spinal cord congestion is confirmed with magnetic resonance imaging.

The Bilateral Sacral Origin of a Spinal Dural Arteriovenous Fistula: A Case Report

: Spinal dural arteriovenous fistulas (SDAVFs) are characterized by an abnormal connection between a spinal radicular artery and a perimedullary vein, mainly fed by a radicular artery at the nerve root sleeve. Here, we describe the case of a 40-year-old woman, presenting with progressive weakness of the lower extremities and the sphincter. Thoracic magnetic resonance imaging (MRI) showed spinal cord edema and signal voids on the dorsal surface of the cord. Spinal angiography demonstrated a SDAVF with a nidus at the sacral level; the feeder of the arteriovenous fistula was a lateral sacral artery, as a branch of the internal iliac artery. The lateral sacral artery was subselectively catheterized, and SDAVF was embolized with 25% n-butyl cyanoacrylate (NBCA) glue (glue: lipiodol ratio, 1:3). After embolization, no definite residual connection was visualized between the arterial and venous systems.

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) five years prior, 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 post-lumbar puncture, with longitudinally extensive mid-thoracic 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 twelve 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 (eg, 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 neurological 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 three weeks postoperatively.

A Septuagenarian With Progressive Lower Extremity Weakness and Pain

A 75-year-old man was referred for evaluation of treatment-resistant transverse myelitis. His medical history included hypertension, coronary artery disease, benign prostatic hyperplasia, and chronic kidney disease. Eight years earlier, the patient noted development of radiating pain down the left lower extremity during long drives, lower extremity weakness and pain, on the left greater than right. He received epidural lumbar corticosteroid injections. Nine months before the current evaluation, his symptoms became refractory, and he underwent surgical decompression with laminectomy at L3-L5. This provided substantial relief for the lower extremity pain. Review of outside magnetic resonance imaging indicated multilevel lumbar stenosis before his surgery and possible, faint, T2-hyperintense cord signal extending into the conus. At the time his symptoms worsened, magnetic resonance imaging of the thoracic spine showed longitudinally extensive T2 hyperintensity extending from the thoracic cord into the conus without contrast enhancement. Evaluation in our department included cerebrospinal fluid analysis, which showed an increased protein concentration of 92 mg/dL, 1 total nucleated cell/µL, normal immunoglobulin G index, and no supernumerary oligoclonal bands. Magnetic resonance angiography of the spinal canal showed mild prominence of vascularity at T10-T12 but no clear spinal dural arteriovenous fistula. However, given the strong suspicion for spinal dural arteriovenous fistula in an older man with progressive myelopathy worsening with corticosteroids, longitudinally extensive lesion extending into the conus, and no evidence of inflammation, spinal digital subtraction angiography was performed. The spinal digital subtraction angiography confirmed the diagnosis of left spinal dural arteriovenous fistula at T11. A T11-12 laminectomy and ligation of the spinal dural arteriovenous fistula was successfully performed without complication. The patient followed up with his local providers for rehabilitation. Spinal dural arteriovenous fistula is the most common spinal arteriovenous malformation, arising from an acquired abnormal connection between a radicular artery and radiculomedullary vein. Progressive congestion and cord edema lead to neurologic deficits over time. Cases are commonly seen in older men with a history of back surgery or trauma. A delay in diagnosis of 1 to 3 years is common.