Mild Narrowing Research Articles

Lumbar Dorsal Root Ganglion Pulsed Radiofrequency for Spinal Stenosis with an Atypical Fluoroscopic Neurogram: A Case Study

Background: We present a case of an atypical fluoroscopic neurogram pattern for complex chronic axial low back pain during a lumbar dorsal root ganglia (DRG) pulsed radiofrequency stimulation. Case Report: A 70 year old female with a history of complex chronic axial low back pain with radicular leg pain. Initially her pain was described as intermittent, burning, and sharp with an intensity of 9/10 on the Numeric Rating Scale (NRS). This pain worsens with ambulation, lumbar extension, turning, weight bearing and spinal loading activities. MRI imaging noted lumbar anterolisthesis, moderate to severe central spinal stenosis, and mild bilateral neural foraminal space narrowing. From these findings, her pain is likely secondary to components of sacroiliac (SI) joint dysfunction, lumbar facetogenic pain, lumbar radiculitis, and spinal stenosis with neurogenic claudication. A multimodal and multidisciplinary rehabilitation approach involving minimally invasive interventional procedures and medication management was implemented with varying pain relief. She responded to L5 transforaminal epidural nerve root injections, SI joint prolotherapy, and sacral lateral branch radiofrequency ablation. She completed bilateral L5 DRG pulsed radiofrequency stimulation, which did not provide radicular pain relief. We performed a unilateral L4 DRG pulsed radiofrequency stimulation, which revealed an atypical L4 fluoroscopic neurogram pattern. At the 3 month follow up she reported the pain severity to have a NRS between 7-8/10. At 4 months, the pain severity was reported to have a NRS between 4-6/10 with subjective improvements with her daily activities. Conclusion: In a normal transforaminal epidural fluoroscopic neurogram, the anteroposterior view should demonstrate contrast spread medially around the pedicle and laterally following the exiting nerve root. In the case of this patient the combined anatomical presentation of anterolisthesis, central spinal canal stenosis, and circumferential disc bulging are all potential reasons for insufficient contrast passage resulting in the atypical neurogram.

Abstract 160: Recurrent Multi‐territorial Ischemic Stroke as an Initial Presentation of Thrombotic Thrombocytopenic Purpura

Introduction Thrombotic thrombocytopenic purpura (TTP) is a rare hypercoagulable disorder characterized by acute hemolytic anemia, thrombocytopenia, neurologic deficits, and renal failure. Here, we describe a young patient with recurrent multi‐territory stroke, with the development of thrombocytopenia later in her course. Her prior diagnosis of etiologies for her ischemic stroke was confounded by the presence of grade 3 PFO and intrinsic arteriopathy attributed to accelerated atherosclerosis due to smoking tobacco with marijuana. Methods A 42‐year‐old woman, active tobacco and marijuana smoker, and recent cryptogenic ischemic and hemorrhagic stroke involving multiple vascular territories with hypercoagulable workup pending, presented with sudden left hemiplegia. Vessel imaging demonstrated irregularity and mild narrowing of the right posterior M2/M3 MCA branches without evidence of large vessel occlusion. MRI showed evidence of evolving left frontal encephalomalacia, prior ischemic stroke on the right inferior MCA and corpus callosum, and prior hemorrhagic stroke on the right parietal and left PCA. In addition, it showed T2 hyperintensities in bilateral anterior and posterior watershed areas. Work‐up was significant for grade 3 PFO/intrapulmonary shunt. Doppler ultrasound was negative for DVT. Basic labs showed LDL 56, A1c 4.8, and TSH 1.8. Hypercoagulable work‐up returned negative. Her platelet count during the admission was initially 169, and 208 on discharge to rehab on single agent antiplatelet (81mg aspirin) and high‐dose statin. After 11 days in rehab, she returned with sudden‐onset slurred speech and right‐sided hemiplegia. Vessel imaging was again negative for LVO and showed significant multi‐vessel arteriopathy. A repeat brain MRI showed a new stroke on the left precentral gyrus, left high frontal lobe, left medial parietal lobe, and left cerebellar hemisphere. The suspected mechanism of stroke was cardioembolic although the patient’s normal left atrial size was presumed to make this mechanism less likely. A few days into admission, her platelets began to decrease. With new‐onset thrombocytopenia (lowest platelet count was 38), ADAMTS screen was ordered and returned positive. The ADAMTS inhibitor Bethesda titer was notably high, 1.4 (nl <0.4). Uncharacteristically for TTP, the patient did not have a fever, anemia (Hb 12), or renal failure (good urine output and Cr 0.7). Nonetheless, given the absence of a clear alternative attributable stroke mechanism, thrombotic thrombocytopenia was suspected as the etiology of her ischemic strokes. For acute therapy, 3‐day 1 mg/kg methylprednisolone was started and hematology service was consulted for co‐management. The patient completed four rounds of plasmapheresis while receiving 90 mg of prednisone daily. She was then started on a regimen to complete four doses of weekly rituximab. The patient improved clinically during the course of her stay and with noted improvements in platelet count and ADAMTS as well. Results N/A Conclusion A low threshold to consider atypical presentations of cryptogenic stroke such as TTP in this case should be in mind when evaluating young adults with recurrent multi‐territory ischemic stroke. Thrombocytopenia may not be present until later in the disease course representing atypical presentations of TTP. As in our case, patients may present without fever, hemolytic anemia, and renal failure.

Assessment of Complex Flow Patterns in Patients With Carotid Webs, Patients With Carotid Atherosclerosis, and Healthy Subjects Using 4D Flow MRI.

Carotid webs (CaWs) are fibromuscular projections in the internal carotid artery (ICA) that cause mild luminal narrowing (<50%), but may be causative in up to one-third of seemingly cryptogenic strokes. Understanding hemodynamic alterations caused by CaWs is imperative to assessing stroke risk. Time-Average Wall Shear Stress (TAWSS) and Oscillatory Shear Index (OSI) are hemodynamic parameters linked to vascular dysfunction and thrombosis. To test the hypothesis: "CaWs are associated with lower TAWSS and higher OSI than mild atherosclerosis or healthy carotid bifurcation." Prospective study. A total of 35 subjects (N = 14 bifurcations with CaW, 11F, age: 49 ± 10, 10 mild atherosclerosis 6F, age: 72 ± 9, 11 healthy 9F, age: 42 ± 13). 4D flow/STAR-MATCH/3D TOF/3T MRI, CTA. 4D Flow velocity data were analyzed in two ways: 1) 3D ROI in the ICA bulbar segment (complex flow patterns are expected) was used to quantify the regions with low TAWSS and high OSI. 2) 2D planes were placed perpendicular to the centerline of the carotid bifurcation for detailed analysis of TAWSS and OSI. Independent-samples Kruskal-Wallis-H test with 0.05 used for statistical significance. The percent surface area where low TAWSS was present in the ICA bulb was 12.3 ± 8.0% (95% CI: 7.6-16.9) in CaW subjects, 1.6 ± 1.9% (95% CI: 0.2-2.9) in atherosclerosis, and 8.5 ± 7.7% (95% CI: 3.6-13.4) in healthy subjects, all differences were statistically significant (ƞ2 = 0.3 [95% CI: 0.05-0.5], P-value CaW vs. healthy = 0.2). OSI had similar values in the CCA between groups (ƞ2 = 0.07 [95% CI: 0.0-0.2], P-value = 0.5), but OSI was significantly higher downstream of the bifurcation in CaW subjects compared to atherosclerosis and normal subjects. OSI returned to similar values between groups 1.5 diameters distal to the bifurcation (ƞ2 = 0.03 [95% CI: 0.0-0.2], P-value = 0.7). Lower TAWSS and higher OSI are present in the ICA bulb in patients with CaW when compared to patients with atherosclerotic or healthy subjects. 2 TECHNICAL EFFICACY: Stage 2.

RETRACTED ARTICLE: Clinical characterization of familial 1p36.3 microduplication

Unlike the 1p36 microdeletion syndrome, which has been extensively described, 1p36.3 microduplications have rarely been reported. We report the two siblings of familial 1p36.3 microduplication, presenting with a severe global developmental delay, epilepsy, and a few dysmorphic features. They were referred to moderate-to-severe developmental delay (DD) and intellectual disability (ID). Both were considered eyelid myoclonus with absence of epilepsy (Jeavons syndrome). The EEG is characterized by widespread 2.5–3.5 Hz spikes and spike slow complex wave, eye closure sensitivity, and photosensitivity. The children has same dysmorphic features, including mild bitemporal narrowing and sloping forehead, sparse eyebrows, hypertelorism, ptosis, strabismus, infraorbital creases, wide nasal bridge with bulbous nasal tip, dystaxia, hallux valgus, and flat feet. Family exome sequencing revealed a maternally inherited 3.2-Mb microduplication of chromosomal band 1p36.3p36.2. However, DNA purified from blood samples of either parent did not find evidence for a microduplication of 1p36 in somatic tissue, indicating that such a mutation might be carried in the germline of the parents as gonadal mosaicism. No other family members of the affected siblings’ parents were reported to be affected by the symptoms found.

Abstract #1400018: Genetic Alterations in NRAS, TERT, PIK3CA and SPEN Genes Leading to an Aggressive Metastatic RAI Refractory Follicular Thyroid Cancer

Follicular thyroid cancer (FTC) is a well-differentiated thyroid tumor and the second most common type after papillary thyroid cancer (PTC) with higher incidence of distant metastases, more aggressive behavior, and poorer outcome than PTC Patient is 43-year-old female with 6.2 cm TR4 left thyroid nodule on Neck US. On Exam, Cricoid is deviated to the right with left thyroid firm nodule. FNA showed Follicular neoplasm. TSH level was 1.59 (0.45-4.70 mIU/L) and Free T4 level: 1.19 (0.78-2.20 ng/dl) CT scan of neck with contrast and MRI thoracic spine: Large, solid heterogenous mass in left thyroid lobe causing lateral displacement and mild narrowing of the trachea with retrosternal extension, Osteolytic lesion of T4 vertebral body and right sided pleura-based mass at the level of T7-T8 with No enlarged cervical lymph nodes. IR biopsy of vertebra: thyroid Origin Metastatic Carcinoma, Patient underwent total thyroidectomy with central neck dissection. Follicular thyroid cancer (STAGE II pT3A,pN1B,pM1) Mutational Testing: Negative BRAF and KRAS with Positive NRAS, PIK3CA, SPEN and TERT. Patient started on TSH suppression therapy. 1 month Later: Thyroglobulin: 2443.4 ng/ml (1.3-31.8), Thyroglobulin Ab< 0.9 (0.0-4.0 IU/ml), TSH 0.02 with Free T4 2.53 FDG PET: Multifocal osseous metastatic disease, predominantly in the axial skeleton Thyrogen stimulated WBS I-123 showed No evidence of residual or metastatic disease. Patient recieved 214 mCi of iodine-131. Post treatment MN scan showed accumulation of iodine in the metastatic bone lesions with small focal activity in the thyroidectomy bed. Post RAI treatment: TG trended to 1102.7 then 1307 Patient Completed 10 fractions of external beam radiation on T4 spine Ultrasound thyroid: No residual thyroid tissue or suspicious lymph nodes Oncology started Bone directed therapy with zoledronate with consideration of PIK3CA mutation targeted therapy with Everolimus or Temsirolimus FTC is more aggressive with poorer prognosis than PTC that is more likely to hematogenously disseminate, with mortality rate of 50%. The neoplastic proliferation of thyroid follicular cells develops by altering multiple molecular pathways such as the PI3K/AKT pathway involving the RAS, RAF, RET, and NTRK1 genes. RAS is a dual activator of the mitogen activated protein kinase and PI3K/AKT pathways TERT is a catalytic subunit of the telomerase complex. Reported in 11–17% of FTC. TERT promoter mutations associated with tumor aggressiveness and mortality PIK3CA encodes p110-alpha, is a catalytic subunit of phosphatidylinositol 3-kinase (PI3K) involves in thyroid cancer tumorigenesis and progression, reported in 7.7% of FTC with sensitivity to therapies targeting PI3K49-56, AKT57-58, or mTOR59-66. In our patient, pretreatment MN scan failed to show RAI avid lesions but PET showed increase FDG uptake and since receiving RAI treatment helped in lowering TG that indicate a possibility of mixture of RAI avid and non-RAI avid lesions existing at same time so RAI treatment is still recommended despite negative Diagnostic Scan for persistent elevated TG.

Anomalous Aortic Origin of Coronary Arteries in Children: Postoperative High-risk Anatomic Features

BackgroundThis study aimed to assess postoperative presumed high-risk anatomic features (HRAFs) by using computed tomographic angiography (CTA) in patients with anomalous aortic origin of a coronary artery (AAOCA) after surgical unroofing vs transection and reimplantation (TAR) if unroofing was thought to provide unsatisfactory results. MethodsThe study included 62 children with postoperative CTA performed at a median of 3 months (interquartile range, 3-4 months) after unroofing (n = 45) and TAR (n = 17). HRAFs included slitlike ostium, intramural course, acute angle takeoff (<45o), interarterial course, proximal stenosis >50%, or course through a thickened intercoronary pillar. ResultsMedian age at surgery was 13.8 years (interquartile range, 10.5-15.8 years). None of the patients had a slitlike ostium or an intramural course on postoperative CTA. Acute takeoff was seen in 100% after unroofing and in 2 of 17 (12%) after TAR (P < .001). After unroofing, the interarterial course improved to 35 of 45 (78%) from 43 of 45 (96%) (P = .003), and a thickened intercoronary pillar improved to 10 of 45 (22%) from 22 of 45 (49%) (P = .0001), compared with none seen after TAR. Preoperative intramural length <5 mm was associated with a postoperative thickened intercoronary pillar in right AAOCA after unroofing (P = .0004). Severe coronary stenosis occurred in 2 of 17 (12%) after TAR, and both patients needed urgent revision procedures. All patients except 2 (97%) returned to exercise activities at a median follow-up of 4.9 years (range, 0.6-9.2 years). ConclusionsThe slitlike ostium and intramural course resolved in all patients. Residual acute angle takeoff, an interarterial course, and mild coronary narrowing related to a thickened intercoronary pillar were common after unroofing. TAR allows resolution of all HRAFs, although severe narrowing requiring surgical revision happened only in TAR. Long-term studies are needed to understand the clinical significance of these residual presumed HRAFs.

Degenerative Lumber Spondylosis: An Educational Radiographs and Expert Therapeutic Recommendation

Background: Spondylosis or degenerative spondylosis which is also called spinal osteoarthritis osteoarthrosis is a disorder characterized by degeneration of the joints of the spine. Clinical the condition presents with low back pain of variable severity and the diagnosis is confirmed by X-ray. Patients And Methods: A man who was born in 1967 presented with acute lumbago, lower back pain that was associated with restriction of movement because of pain. Results: At about the age of 55 years, a diabetic and hypertensive presented with acute lumbago, lower back pain that was associated with restriction of movement because of pain. The pain occurred suddenly while standing and was not associated with lifting a weight. Physical examination revealed no neurologic deficit. Because of the sudden onset of the condition, and the association with painful mobility radiographs of the lower spine was performed and showed loss of the normal lordosis, marginal osteophytosis, subchondral sclerosis, and mild narrowing of the L4-L5 space. A radiologic diagnosis of degenerative spondylosis was made. Conclusion: In the case of degenerative lumbar spondylosis presenting with acute lumbago, the current expert opinion recommends the addition of low dose baclofen to the analgesic-anti-inflammatory medication, based on the evidence provided by Migliorini et al and Dapas et al. Supplementation with collagen and hyaluronic acid is recommended for few months with aim of improving the degenerative process, based on the evidence provided by Giacomo Farì and his research group.

Abstract 10115: Unique Imaging Morphology and Rare Location of Papillary Fibroelastoma Resulting in a Cardioembolic Stroke

Clinical Presentation: Afro-Caribbean female, age 59, with a comorbidity of hypertension presented with acute painless right-sided vision loss. Brain MRI revealed numerous scattered T2/FLAIR hyperintense foci throughout the periventricular, deep, and subcortical white matter, which were favored to represent chronic microvascular ischemic changes. CTA of the head and neck demonstrated mild atherosclerotic calcification of the cavernous segment of the left internal carotid artery without significant stenosis, mild noncalcified atherosclerotic plaque at the right carotid bifurcation, and mild irregularity and narrowing of the mid basilar artery. Cardiac Imaging: Transthoracic Echocardiography (TTE) demonstrated severe concentric left ventricular hypertrophy, left ventricle ejection fraction 60-65%, and a mobile, intracavitary mass measuring 1.60cm x 1.59cm. Repeat TTE estimated the mass to be 1.8cm x 1.2cm in the region of the posteromedial papillary muscle. Transesophageal Echocardiography (TEE) redemonstrated a mobile left ventricular mass measuring 2.0cm x 1.8cm along the inferior wall of the interventricular septum. Left heart catheterization demonstrated nonobstructive coronary artery disease with a 30% stenosis of the proximal Right Coronary and Distal Circumflex. Patient underwent surgical removal of the cardiac mass, biopsy-proven to be a Papillary Fibroelastoma (PFE). Discussion: PFEs account for 7.9% of all primary benign cardiac tumors and are the most common valvular tumor. More than 80% of these tumors are attached on the aortic or mitral valve, unlike in this patient where it was attached to the left ventricular trabeculae without involvement of the mitral valve or sub-valvular apparatus. The classic appearance of PFEs on TTE is pedunculated and often compared to a sea anemone but appeared homogenous in nature with central vacuolization in this case. This made characterizing the mass as a thrombus or cardiac tumor challenging.

69 Large vessel vasculitis occurring after COVID-19 infection: 2 Takayasu cases

BackgroundTakayasu arteritis (TA) is a rare, chronic, granulomatous large vessel vasculitis that affects the aorta, its main branches, and the pulmonary artery. Although the aetiology of TA is not known entirely, it is thought to be multifactorial. To date, cases have been reported after Mycobacterium tuberculosis, human immunodeficiency virus (HIV), and influenza infection. Cases of postinfectious vasculitis have been reported in the literature during the COVID-19 pandemic. Here, we present two patients diagnosed with TA after COVID-19 infection.Case 1A thirteen-year-old female patient was admitted to the pediatric emergency department of our hospital with chest pain and syncope. His uncle had a COVID-19 infection 6 months ago and they stayed in the same house during the infection.On physical examination, respiratory sounds were less audible in the left lung baseline. The blood pressure was measured 128/71 mmHg in the right arm, 122/72 mmHg in the left arm, 106/67 mmHg in the right leg, and 107/74 mmHg in the left leg. Peripheral pulses were weaker in the lower extremities than in the upper extremities.High acute phase reactants and anaemia were detected in the laboratory. (Table 1) Anti-ds DNA was positive, and anti-neutrophil cytoplasmic antibody (cANCA) was negative. Infection screening was negative.In thorax and abdomen computed tomography (CT), 20 mm pericardium and 10 mm pleural effusion and mural thickening along the aorta of ∼20–25 cm from the descending thoracic aorta to the suprarenal level in the proximal 2 cm of the left subclavian artery and mild narrowing of the celiac and superior mesenteric artery orifices were detected. The patient was diagnosed with TA. She was treated with intravenous 30 mg/kg/day methylprednisolone for 3 days. The patient was discharged with oral prednisolone, methotrexate, and anti-TNF therapy.Case 2A 17-year-old female patient was admitted to our center with complaints of back pain for 2 months, dry cough, weight loss (3 kg in 2 months), and fever for 2 days. It was learned that the patient had a COVID-19 infection 5 months ago.On physical examination, blood pressure was measured 94/59 mmHg in the left arm, 103/60 mmHg in the right arm, 125/77 mmHg in the left leg, and 132/80 mmHg in the right leg. Peripheral pulses were weaker in the lower extremities compared with the upper ones. There was a 1/6 systolic murmur in the aortic focus. Other system examination was normal.Laboratory tests revealed elevated acute phase reactants, thrombocytosis, and anaemia. (Table 1) Positron emission tomography-CT (PET-CT) revealed increased FDG uptake in both common carotid, aortic arch, ascending and descending aortic walls. In MR angiography, localized enlargement and stenosis of the aortic arch, wide ascending aorta, stenosis in the left subclavian artery, and the origin level of the left renal artery, and then a slight enlargement was observed. Based on the present findings, the patient was diagnosed with TA. Infection screening was negative. The patient was given 30 mg/kg/day intravenous methylprednisolone for 3 days and continued with oral prednisolone, methotrexate, and etanercept.DiscussionTimely diagnosis and treatment in TA is very important in preventing irreversible vascular damage resulting in ischaemia of vital organs. It should be kept in mind that TA and other vasculitides may occur after COVID-19 infection.

S3487 Superior Mesentery Artery Syndrome Presenting in an Otherwise Healthy Female

Introduction: Superior Mesentery Artery (SMA) Syndrome is a rare gastrovascular disorder due to narrowing of the space between the aorta and superior mesenteric artery, also known as aortomesenteric angle, which can compress the third part of the duodenum. Clinically, SMA syndrome in adults is associated with conditions, such a malignancy, trauma, extreme weight loss, or corrective spinal surgeries. We report a case of SMA syndrome in a patient without comorbidities to alert clinicians of the non-specific manifestations of this rare phenomenon. Case Description/Methods: A 41-year-old female presented with a 30-year-history of post-prandial epigastric pain and acid reflux. She also reported early satiety, frequent belching, nausea, and constipation. She denied vomiting, diarrhea, hematochezia, and weight loss. Physical examination revealed epigastric tenderness to palpation. Laboratory studies were unremarkable. Upper endoscopy showed patchy erythema and few erosions in the antrum consistent with gastritis. Colonoscopy, gastric emptying study, and abdominal ultrasound were unremarkable. CT abdomen and pelvis showed nondilated bowels without thickening. Magnetic resonance angiography abdomen showed narrowing of the aortomesenteric distance at 5 mm, decreased aortomesenteric angle at 23 degrees, and mild narrowing of the left renal vein as it passes between the aorta and superior mesenteric artery (Figure). SMA syndrome was diagnosed. She was started on metoclopramide 5mg twice a day with subsequent improvement of her acid reflux by 90 percent and resolution of her epigastric pain. Discussion: SMA Syndrome may present with insidious non-specific signs and symptoms, which can lead to delayed diagnosis. When diagnosis is delayed, it can result in significant morbidity and mortality. Diagnosis is challenging. Gastric emptying studies may be falsely negative when the duodenum obstruction is relieved by positional changes. CT or magnetic resonance angiography allows visualization of the vascular compression. In mild cases without small bowel obstruction, prokinetic pharmacotherapy may help decrease symptoms. Surgical correction may be needed. Clinicians should consider SMA syndrome in select patients who present with symptoms suggestive of small bowel obstruction when gastrointestinal studies are unrevealing.Figure 1.: Decreased aortomesenteric angle of 23 degrees. A. Sagittal view. B. 3-dimensional reconstruction view.

The (extended) achondroplasia foramen magnum score has good observer reliability

BackgroundAchondroplasia is the most common skeletal dysplasia. A significant complication is foramen magnum stenosis. When severe, compression of the spinal cord may result in sleep apnea, sudden respiratory arrest and death. To avoid complications, surgical decompression of the craniocervical junction is offered in at-risk cases. However, practice varies among centres. To standardize magnetic resonance (MR) reporting, the achondroplasia foramen magnum score was recently developed. The reliability of the score has not been assessed.ObjectiveTo assess the interobserver reliability of the achondroplasia foramen magnum score.Materials and methodsBase of skull imaging of children with achondroplasia under the care of Sheffield Children’s Hospital was retrospectively and independently reviewed by four observers using the achondroplasia foramen magnum score. Two-way random-effects intraclass coefficient (ICC) was used to assess inter- and intra-observer reliability.ResultsForty-nine eligible cases and five controls were included. Of these, 10 were scored normal, 17 had a median score of 1 (mild narrowing), 11 had a median score of 2 (effacement of cerebral spinal fluid), 10 had a score of 3 (compression of cord) and 6 had a median score of 4 (cord myelopathic change). Interobserver ICC was 0.72 (95% confidence interval = 0.62–0.81). Intra-observer ICC ranged from 0.60 to 0.86. Reasons for reader disagreement included flow void artefact, subtle T2 cord signal and myelopathic T2 cord change disproportionate to canal narrowing.ConclusionThe achondroplasia foramen magnum score has good interobserver reliability. Imaging features leading to interobserver disagreement have been identified. Further research is required to prospectively validate the score against clinical outcomes.

A Rush of Blood to the Head: Tacrolimus Related Reversible Cerebral Vasoconstriction Syndrome After Pediatric Heart Transplantation

<h3>Introduction</h3> Posterior reversible encephalopathy syndrome (PRES) is a known complication associated with calcineurin inhibitor (CNI) therapy after solid organ transplant. A pathophysiologically related but clinically distinct diagnosis, reversible cerebral vasoconstriction syndrome (RCVS), is rarely reported in pediatric patients. RCVS is characterized by segmental constrictions of the cerebral arteries versus edema seen in PRES. Here we present RCVS related to tacrolimus in two adolescents after heart transplantation. <h3>Case Report</h3> A 10-year-old female with a history of tricuspid atresia status post Hemi-Fontan palliation and progressive ventricular dysfunction underwent heart transplantation. Her post-transplant course was complicated by graft diastolic dysfunction and early donor specific antibody production, treated with intravenous immunoglobulin and rituximab. Her immunosuppression was prednisone, tacrolimus, and mycophenolate mofetil. Seven weeks after transplantation she developed a thunderclap headache, acute severe hypertension and mental status changes and was found to have a multifocal subarachnoid hemorrhage (SAH) without identifiable aneurysm, most consistent with RCVS. Her tacrolimus trough was therapeutic. She was transitioned from tacrolimus to cyclosporine and symptoms resolved after 2 days. She successfully transitioned back to tacrolimus at one year post transplant without RCVS recurrence. A 14-year-old-male with a history of dilated cardiomyopathy status post left ventricular assist device underwent heart transplantation. His immunosuppression was prednisone, tacrolimus, and mycophenolate mofetil. His post-transplant course was uncomplicated until one week after transplant he developed a thunderclap headache and acute severe hypertension, and was found to have a SAH, with mild multisegmental narrowing of the circle of Willis, consistent with RCVS. His tacrolimus trough was subtherapeutic. He was transitioned from tacrolimus to cyclosporine with no recurrence of RCVS symptoms. <h3>Summary</h3> This is the first reported case series of tacrolimus related RCVS in pediatric heart transplant patients without elevated tacrolimus levels. CNI related RCVS should be considered if acute SAH occurs and may be managed with switching CNI to another immunosuppression agent.

Staged left ventricular recruitment facilitated by Sano conduit upsizing

Staged left ventricular recruitment facilitated by Sano conduit upsizing

Acute and Delayed Cerebrovascular Injury From Gunshot to the Head in a 12-Year-Old Child During the COVID-19 Pandemic

Acute and Delayed Cerebrovascular Injury From Gunshot to the Head in a 12-Year-Old Child During the COVID-19 Pandemic

P186 Can transabdominal bowel ultrasound accurately detect both small and large bowel Crohn Disease in pediatric patients when compared to MRE?

Abstract Background Magnetic resonance enterography (MRE) has been the modality of choice to assess small bowel inflammation in patients with Crohn disease (CD). It can also assess large bowel when not feasible to assess endoscopically. Transabdominal bowel ultrasound (TABUS) is an attractive non-invasive tool to assess CD activity. Studies to date have focused on its correlation to large bowel endoscopic findings in adult CD. This study aimed to compare TABUS and MRE in identifying small and large bowel CD in pediatric patients. Methods Pediatric patients (≤18 years old) seen for suspected CD were prospectively enrolled and underwent baseline TABUS, endoscopy, MRE, blood work and stool studies. The Pediatric Crohn’s Disease Activity Index (PCDAI) and Simple Endoscopic Score for Crohn’s Disease (SES-CD) were used to assess disease activity. Bowel wall thickness (BWT) was assessed by TABUS and MRE. TABUS measurements were scored with the Simple Ultrasound Activity Score for Crohn Disease (SUS-CD) (0=≤3mm, 1=3–4.9mm, 2=5–7.9mm, 3=≥8mm). Pearson Chi-square was used to compare BWT between TABUS and MRE. Results 21 patients (71% male) were included, with a median age of 13 years (range 6–16). Median PCDAI was 40 (25–49) and SES-CD 15 (6.5–22). There was a high level of agreement between MRE and TABUS (Figure 1) with some disagreement in the jejunum (n=9/21, 43%). For terminal ileal (TI) disease where there was disagreement, endoscopy of TI was in agreement with TABUS in two patients and MRE in five patients. Endoscopic TI disease that was documented as normal on TABUS was mild (SES-CD 3). In patients with abnormal MRE, BWT was not significantly different between the two modalities (Table 1). In patients with normal MRE, TABUS SUS-CD scores were accurately 0 in almost all segments (score 1 in TI) (Table 2). TABUS correctly identified no fistulae and 2 small bowel strictures (identified at endoscopy), but did not detect 1 intra-abdominal abscess and 4 large bowel strictures (only 1 stricture seen on endoscopy). Excessive lymph nodes (&amp;gt;4) were successfully seen on TABUS in 10/16 patients. TABUS correctly identified 75% of patients with significant mesenteric fibrofatty proliferation. Hyperemia on TABUS corresponded to an abnormal MRE for that segment in 92–100% of small bowel and 50–86% of large bowel. Conclusion TABUS and MRE are comparable modalities in the assessment of small and large bowel disease in newly diagnosed pediatric CD patients. Assessment of proximal small bowel is complex, and MRE may over-call disease. MRE still plays an important role in assessing abscesses and mild large bowel narrowing. Hyperemia on TABUS is an important indicator of active bowel inflammation, and is particularly useful for endoscopically inaccessible small bowel.

ACUTE MULTIPLE TERRITORIES STROKE IN AN ANTICOAGULATED COVID-19 PATIENT

TOPIC: Chest Infections TYPE: Medical Student/Resident Case Reports INTRODUCTION: The complications of coronavirus disease 2019 (COVID-19) involve the nervous system, including acute stroke, which is currently linked to the hypercoagulable state associated with COVID-19 [1]. CASE PRESENTATION: Our patient is a 70-year-old man with past medical history of hypertension and diabetes mellitus who presented to the emergency department with shortness of breath and fatigue for five days duration. Vitaly was stable, except for saturating 88% on the room air. He was alert and oriented on physical exam, lung exam was remarkable for bibasilar rales. He had no focal neurological deficits. Lab Work showed elevated COVID-19 markers. Chest x-ray showed left-sided infiltrates. He was admitted to the respiratory care unit and was started on dexamethasone, remdesevir, ceftriaxone, azithromycin, and enoxaparin prophylactic dose. His oxygen requirement continued to increase, and on the ninth day, he was placed on a nasal high-flow oxygen device with 60 liters FiO2 of 90%. On the eleventh day, the patient developed right facial droop, aphasia and right-sided weakness. The National Institutes of Health Stroke Scale was 16. Code stroke was called, and a brain computed tomography scan without contrast revealed cortical infarct in the high left parietal cerebral hemisphere without hemorrhage. Computed tomography angiography (CTA) of the head and neck showed mild narrowing within the middle cerebral artery. Magnetic resonance imaging, later on, showed left medial frontoparietal infarct in ACA territory. Cardiology workup confirmed no cardiac pathology. The neurology team decided not to start the tissue plasminogen activator (tPA) because he was out of the tPA window, and as no large vessel occlusion was found, a thrombectomy was not performed. DISCUSSION: COVID-19 infection is considered a highly coagulable status, especially the severe form of the disease. The pathophysiology behind this is not well understood yet. However, Severe COVID-19 is associated with cytokine activation, which activates endothelial cells and mononuclear cells with expression of tissue factor leading to coagulation cascade activation [2]. It has also been observed that COVID-19 can induce Antiphospholipid antibodies, which might play a role in thrombosis in those patients [3]. Our case confirms that COVID-19 induced acute stroke can occur in multiple territories despite the patient being well anticoagulated. CONCLUSIONS: Acute stroke should be suspected in COVID-19 patients with acute neurological changes even if the patient is on prophylactic anticoagulation. Frequent screening neurological examination can lead to early detection and treatment of acute stroke. REFERENCE #1: Verstrepen, K., Baisier, L. & De Cauwer, H. Neurological manifestations of COVID-19, SARS and MERS. Acta Neurol Belg 120, 1051–1060 (2020). https://doi.org/10.1007/s13760-020-01412-4 REFERENCE #2: Beyrouti R, Adams ME, Benjamin L, et alCharacteristics of ischaemic stroke associated with COVID-19Journal of Neurology, Neurosurgery & Psychiatry 2020;91:889-891. REFERENCE #3: Zhang Y, Xiao M, Zhang S, Xia P, Cao W, Jiang W, Chen H, Ding X, Zhao H, Zhang H, Wang C, Zhao J, Sun X, Tian R, Wu W, Wu D, Ma J, Chen Y, Zhang D, Xie J, Yan X, Zhou X, Liu Z, Wang J, Du B, Qin Y, Gao P, Qin X, Xu Y, Zhang W, Li T, Zhang F, Zhao Y, Li Y, Zhang S. Coagulopathy and Antiphospholipid Antibodies in Patients with Covid-19. N Engl J Med. 2020 Apr 23;382(17):e38. doi: 10.1056/NEJMc2007575. Epub 2020 Apr 8. PMID: 32268022;PMCID: PMC7161262. DISCLOSURES: No relevant relationships by Abdul Rahman Al Armashi, source=Web Response No relevant relationships by Ameed Bawwab, source=Web Response No relevant relationships by Arthur Boyd, source=Web Response No relevant relationships by Kanchi Patell, source=Web Response No relevant relationships by Keyvan Ravakhah, source=Web Response No relevant relationships by Francisco Somoza-Cano, source=Web Response

Reversible Cerebral Vasoconstriction Syndrome Following Exposure to Oleoresin Capsicum “Pepper Spray”

ObjectivesTo report a case associating the use of Oleoresin Capsicum Pepper Spray (OCPS) during law enforcement training with development of Reversible Cerebral Vasoconstriction Syndrome (RCVS). Materials and MethodsRCVS is radiographically characterized by multifocal smooth narrowing of cerebral arteries heralded by clinical manifestations of recurrent thunderclap headaches. 70% of cases with RCVS have a clear precipitating factor and agents commonly implicated were cannabis, selective serotonin reuptake inhibitors, nasal decongestants, cocaine, postpartum state, eclampsia and strenuous physical/sexual activity.1 Results24-year-old female police officer with no past medical history who presented with thunderclap headaches after exposure to pepper spray to her face during work training. Neurological examination was unremarkable. CT angiogram (CTA) of the head and neck and subsequent conventional angiogram revealed multifocal mild arterial narrowing of bilateral middle cerebral arteries (MCA), bilateral posterior cerebral arteries (PCA) and left anterior cerebral artery (ACA) concerning for RCVS. Eight weeks later, she had a repeat MRA head and neck demonstrating complete resolution of the previously noted narrowing of her cerebral arteries. ConclusionsOCPS is widely used in law enforcement training as well as by general population as a self- defense tool. It is generally assumed to be safe, although the consequences of its use can never be predicted with certainty.2 As our case highlights, use of OCPS may be associated with development of RCVS and awareness needs to be raised regarding this rare but serious complication.

Role of Extradural Clinoidectomy and Optic Unroofing in Resection of an Anterior Clinoidal Meningioma with Encasement of the Internal Carotid Artery and Its Branches.

Anterior clinoidal meningiomas (ACMs) remain a major neurosurgical challenge. The skull base techniques, including extradural clinoidectomy and optic unroofing performed at the early stage of surgery, provide advantages for improving the extent of resection, and thereby enhancing overall outcome, and particularly visual function. Additionally, when the anterior clinoidal meningiomas encase neurovascular structures, particularly the supraclinoid internal carotid artery and its branches, this further increases morbidity and decreases the extent of resection. Although it might be possible to remove the tumor from the artery wall despite complete encasement or narrowing, the decision of whether the tumor can be safely separated from the arterial wall ultimately must be made intraoperatively. The patient is a 75-year-old woman with right-sided progressive vision loss. In the neurological examination, she only had light perception in the right eye without any visual acuity or peripheral loss in the left eye. MRI showed a homogeneously enhancing right-sided anterior clinoidal mass with encasing and narrowing of the supraclinoid internal carotid artery (ICA). Computed tomography (CT) angiography showed a mild narrowing of the right supraclinoid ICA with associated a 360-degree encasement. The decision was made to proceed using a pterional approach with extradural anterior clinoidectomy and optic unroofing. The surgery and postoperative course were uneventful. MRI confirmed gross total resection ( Figs. 1 and 2 ). The histopathology was a meningothelial meningioma, World Health Organization (WHO) grade I. The patient continues to do well without any recurrence and has shown improved vision at 15-month follow-up. This video demonstrates important steps of the microsurgical skull base techniques for resection of these challenging tumors. The link to the video can be found at https://youtu.be/vt3o1c2o8Z0

Left Ventricular Assist Device Graft Obstruction Relieved by Transcutaneous Stenting in a Teenage Patient

<h3>Introduction</h3> We describe a case of LVAD outflow graft stenosis from external outflow graft compression in a teenage patient successfully treated with percutaneous stenting. <h3>Case Report</h3> 17-year-old female with a continuous flow left ventricular assist device implanted 3 years prior for heart failure secondary to cardiomyopathy due to a TTN gene mutation. She presented with 1 week of worsening shortness of breath and 3 kilogram weight gain. LVAD flows were 3 liters-per-minute (L/min). The patient was referred for transcatheter stent therapy due to concern for outflow cannula obstruction. Hemodynamics during initial catheterization showed a gradient of 30 mmHg across the conduit. A stent was deployed in the distal aspect of the outflow cannula. A second stent was placed to reinforce the area of persistent stenosis. Post-intervention hemodynamics showed an improvement in the gradient to 13 mmHg. She re-presented 3 months later due to LVAD low flow alarm, decreased flows to 0.3 L/min, and decreased wattage of 2.9 watts (Figure 1). CT angiography (CTA) demonstrated 80% narrowing of the outflow conduit, proximal to previously placed outflow graft stents (Figure 2a-b). Low density material between the outflow graft and circumferential wrap was identified by radiology. The previously placed stents were first re-dilated. Three additional stents were placed within the LVAD outflow cannula. We placed the stents from aortic end toward LVAD inflow. In total, the outflow graft was stented for a length of approximately 7.5cm (Figure 3). Angiography showed mild residual narrowing at the anastomotic site. LVAD flows improved to 3.5L/min. Repeat CTA showed no residual evidence of stenosis. <h3>Summary</h3> To our knowledge this is the first reported case of external compression of an LVAD outflow graft from an overwrap treated with successful transcatheter stent treatment in the pediatric population. By stenting the outflow graft we believe we pushed the built-up material into the mediastinum, relieving the obstruction.

Degrees of Cross-Sectional-Area Luminal Narrowing of the Four Major Epicardial Coronary Arteries in Patients With Otherwise Functionally and Anatomically Normal Hearts

Described herein are necropsy findings in the 4 major (left main, left anterior descending, left circumflex, and right) epicardial coronary arteries in 86 patients aged 10-70 years who never had symptoms of myocardial ischemia, and at autopsy had otherwise anatomically normal hearts. All 86 died of a non-cardiovascular condition. In each of these patients all 4 major epicardial coronary arteries were excised intact from the heart, divided into 5-mm segments, and each segment prepared for histologic examination. The degrees of cross-sectional area (CSA) narrowingwere determined from histologic examination of each 5-mm segment. The degree of narrowing in each 5 mm segment was divided into 4 categories: 0% to 25%, 26% to 50%, 51% to 75%, and 76% to 100%. Twelve patients (14%) had ≥1 artery narrowed >75% in CSA, a single artery in 9 patients, and 2 arteries in each of 3 patients. In contrast to the relative infrequency of narrowing >75%, narrowing 51-75% was common, and was present in 36 (42%) of the 86 patients. Of the 258 major coronary arteries (excludes the left main) studied in the 86 patients, 15 (6%) were narrowed >75%, and 70 (24%) were narrowed 51% to 75% in CSA. Even mild narrowing (26% to 50%) of the left main coronary artery (66 patients) was generally accompanied by 51% to 75% or greater narrowing of at least one of the other major coronary arteries. In conclusion, even hearts which have functioned normally and are otherwise anatomically normal, usually have some degree of atherosclerotic plaque in the major epicardial coronary arteries.