Extra Medullary Fixation in Unstable Proximal Femoral Fractures by PF-LCP.

Purpose: Perception that extra-medullary fixation of these fractures are fraught with difficulties and deficiencies is becoming inappropriate. This device provides angular stable fixation retaining fracture biology with minimum interference to osseous and soft-tissue vascularity and it does not require reaming which destroys 80% of endosteal vasculature for 6-12 weeks. PFLCP averts iatrogenic fracture in lateral trochanteric wall (LTW) which is frequent with DHS, protects LTW from secondary fracture in post-operative period. Aim is to assess outcome of unstable proximal femur fracture fixation by PFLCP. Methods: Study included 64 from 2016 to 2020, divided in two groups. (A) Unstable intertrochanteric fracture and (B) subtrochanteric fracture (Seinsheimer types II-V). All fractures fixed by MIPO with PFLCP. Loss of reduction, infection, cut-out, cut-through, backing of screws, bending or breaking of plate and screw, malunion, non-union and revision were evaluated. Fracture healing and functional recovery assessed by Reborne Score and Parker Mobility Score (PMS) respectively. Results: Out of 64, 24 achieved pre-injury PMS, 32 declined by 1 point, 6 declined by 2 points and 1 by 3 points, one required revision. Using various parameters 37.5% patients had excellent results and 50% had good results, 9.38% had average and 3.12% had poor result. None reported non-union or breakage of plate. Conclusions: PFLCP provides angular stable fixation, torsional stability with high biomechanical strength to resist deforming stresses. MIPO avoids soft-tissue stripping reducing blood-loss, retains periosteal blood supply to inter-fragmentary bone fragments, enhancing fracture healing, reducing complications, such as delayed healing, nonunion, infection and implant failure.

Opalski syndrome: A comprehensive systematic review of cases.

BACKGROUND: Opalski syndrome, a subtype of lateral medullary syndrome (LMS), poses challenges due to its diverse clinical presentations and potential atypical symptoms. Understanding its epidemiology, clinical manifestations, and outcomes is crucial for optimizing patient care. METHODS: A systematic review, following PRISMA 2020 guidelines, was conducted to comprehensively analyze Opalski syndrome. Data from PubMed, Scopus, Web of Science, and Embase were included, with the search conducted in May 2023. Eligible studies spanned from included case reports, case series, and editorial letters. RESULTS: The review encompassed 78 studies from 1984 to 2024, involving 94 patients with Opalski syndrome. The analysis revealed a male predominance (76.60 %) with a male-to-female ratio of 3.1:1. Common risk factors included hypertension (63.54 %), diabetes mellitus (32.29 %), smoking (32.39 %), and alcohol consumption (22.91 %). Opalski syndrome cases were reported in 22 countries across 5 continents, with Asia being the most prevalent region (77.08 %). Initial presentations commonly included ataxia or positive finger-to-nose and knee-to-heel tests, dizziness or vertigo, hemiparesis, nystagmus, Horner's sign, and 5th or 7th cranial nerve palsy, all occurring in more than 50 % of cases. Neuroimaging techniques such as magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) were crucial for diagnosis. Despite a mortality rate of 4.16 %, no deaths have been reported since 2014, indicating advancements in clinical management. CONCLUSION: Targeted risk factor management, early recognition of symptoms, and utilization of advanced neuroimaging techniques are essential for optimizing patient outcomes. Clinicians must remain informed about Opalski syndrome to enhance diagnostic accuracy and tailor treatment strategies.

Intramedullary spinal schistosomiasis: A case report and review of the literature.

INTRODUCTION: Spinal cord schistosomiasis is an extremely rare entity presenting with a wide range of neurological symptoms. The early diagnosis and treatment can improve neurological outcome. Histopathological examination is the gold standard for establishing the diagnosis of spinal schistosomiasis, revealing schistosoma eggs. CASE REPORT: We report a case of a 13-year-old male, from Mauritania, with a history of drinking unsafe water, presenting with an acute urinary retention and gait disturbances evolving for 1 month. His clinical examination found an incomplete conus medullary syndrome made up of urinary retention, lively patellar reflexes on the right, ataxia when walking on the same side and indifferent cutaneous planter reflex. The magnetic resonance imaging (MRI) on dorsal spine revealed an enhancing mass involving the conus medullaris in the L1-L2 region suggestive of an arteriovenous malformation or a cavernoma. The resection tissue specimens for diagnosis were fixed with 10 % buffered formalin. The slides were stained with haematoxylin-eosin staining for light microscopy. The diagnosis of schistosomiasis spinal cord was retained. The child has been treated with oral praziquantel 25 mg/kg. DISCUSSION: Diagnosis of schistosomiasis is based on a combination of clinical evaluation, imaging studies, and laboratory tests. However, definitive diagnosis typically requires histopathological examination of spinal cord lesions obtained through biopsy. Differential diagnosis is broad, including an acute vascular event and/or tumor, especially in children from endemic areas for schistosomiasis. CONCLUSION: Schistosomiasis infection should be suspected when encountering medullary lesion associated to peripheral hypereosinophilia. Surgical excision combined with praziquantel may help improve neurological deficits.

Radiological Features of Extramedullary Hematopoiesis in a Young Male With Beta-Thalassemia: A Case Report.

The formation of the blood elements and their maturation is called hematopoiesis. In adults, this typically takes place in the bone marrow of vertebrae, ribs, and long bones. In contrast, during fetal development, the primary sites of hematopoiesis are the spleen, liver, and the yolk sac. This process of hematopoiesis, when it occurs in sites other than the bone marrow, is called the extramedullary hematopoiesis (EMH). Extramedullary hematopoiesis usually happens in patients with blood disorders like sickle cell disease and thalassemia, where there is failure of hematopoiesis in the primary sites. Here, we present a young male with beta-thalassemia who presented with shortness of breath and palpitations for one month. This manuscript discusses the imaging findings of the EMH in our patient.

High success rate of first proficiency testing for RET fusions and RET mutations in lung and thyroid cancer samples by various methods.

This study describes the external quality assessment (EQA) scheme for molecular testing of RET alterations in non-small cell lung cancer (NSCLC), medullary thyroid carcinomas (MTC), and non-MTC. The lead panel institute and Quality Assurance Initiative in Pathology (Qualitätssicherungs-Initiative Pathologie [QuIP] GmbH) selected formalin-fixed paraffin-embedded (FFPE) tissue from MTC for RET mutation testing by next-generation sequencing (NGS) methods and FFPE tissue from NSCLC and non-MTC for RET gene fusion testing using either in situ hybridisation (ISH) or NGS methods, forming 3 sub-schemes of the EQA scheme. Tissue material underwent an internal validation phase followed by an external testing phase. The internal validation phase served as a cross-validation step conducted by panel institutes. In the external testing phase, the number of participating institutes in the RET point mutation sub-scheme, RET fusion (ISH) sub-scheme, and RET fusion (NGS) sub-scheme was 32, 24, and 38, respectively. The reported success rates for external testing were 96.0%, 89.5%, and 93.5% for the RET point mutation, the ISH RET fusion, and the NGS RET fusion EQA sub-schemes, respectively. These findings confirm the reliability of the NGS method in detecting RET alterations and align with current screening recommendations. Overall, 31 institutes were certified for RET point mutation testing by NGS methods, 22 institutes were certified for RET fusion testing by ISH, and 36 institutes were certified for RET fusion testing by NGS methods. Results can be employed to inform real-world diagnostic decisions in Germany, Austria, and Switzerland.

Expression of integrin αvß3 in medullary thyroid carcinoma.

Aim: Tumor markers often remain elevated after intended curative resection of medullary thyroid carcinoma (MTC). The aim of this study was to determine the expression of αvß3, a promising theranostics target, in MTC and its metastases. Materials & methods: Avß3 expression was analyzed in 104 patients using a tissue microarray and correlated with clinicopathological variables and survival. Results: Cytoplasmic αvß3 positivity was seen in 70 patients and was associated with lymph node metastases at time of initial surgery. Membranous positivity was considered positive in 30 patients and was associated with sporadic MTC. Conclusion: Avß3 was expressed in the cytoplasm of 67% of MTC patients. Membranous expression, which is presumably most relevant for the theranostic use of αvß3, was seen in 29%.

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Atypical Presentation of Acute Suppurative Otitis Media with Facial Palsy: Extra Medullary Manifestation of AML in Temporal Bone.

Acute Myeloid Leukemia (AML) is the most common leukemia observed in the adult population, accounting for 80% of all leukemia cases. Extramedullary involvement in AML, where leukemic cells are found in organs or tissues outside the blood or bone marrow, is a rare occurrence [1]. The most frequent sites of extramedullary disease include the skin, central nervous system (CNS), and lymph nodes [2, 3]. In this case report, we present an instance of extramedullary AML in the temporal bone, which initially presented with symptoms such as earache, discharge, and facial asymmetry, mimicking acute suppurative otitis media with facial palsy. The patient underwent mastoid exploration and facial nerve decompression. A post-operative bone marrow biopsy confirmed the diagnosis of AML, leading to the initiation of chemotherapy. The patient is currently under follow-up care.

A Rare Case of Multiple Bone Infarctions and Abnormal Pulmonary Function Tests in a Patient With Compound Heterozygous Hemoglobin S and Type 2 Hereditary Persistence of Fetal Hemoglobin.

Sickle cell disease (SCD) is a group of inherited blood disorders characterized by abnormal hemoglobin production, affecting individuals worldwide with varying prevalence across different populations. Manifestations vary, ranging from severe to mild. SCD is characterized by the presence of hemoglobin S (HbS), which distorts erythrocytes upon deoxygenation, leading to sickling. This results in hemolytic anemia, painful vaso-occlusive crises (VOC), and multiple organ damage, including bones, due to microinfarcts. Sickle cell trait (SCT), or carrier status, is not considered an SCD and often runs a benign course. We report a 44-year-old man of African descent presenting with a one-month history of pain in his ankles and feet. He had a prior diagnosis of sickle cell "trait" without previous VOC. Hematological indices were normal. Hemoglobin electrophoresis showed absent HbA, elevated HbS, elevated HbF, and normal HbA2. X-rays and MRI revealed bilateral bone infarction in diaphyses of right proximal and bilateral distal tibias. Molecular analysis of [Formula: see text]-globin revealed compound heterozygous hemoglobin S and type 2 deletion of persistence of fetal hemoglobin (HPFH). Pulmonary function tests revealed restrictive lung disease. A literature review from 1946 to May 2024 via PubMed, EMBASE, and Medline was performed, revealing two cases of HbS-HPFH with avascular necrosis affecting the femoral neck were briefly reported more than 60 years ago. Although pulmonary function tests in SCD typically show a mild restrictive pattern with decreased diffusion capacity and rarely an obstructive pattern, no cases of HbS-HPFH were identified. In conclusion, multiple bone infarctions are extremely rare in HbS-HPFH. Lung and bone diseases might be unrecognized in this unique disorder.

A practical nomogram for preoperatively predicting lateral cervical lymph node metastasis in medullary thyroid carcinoma: a dual-center retrospective study.

Purpose: Lateral lymph node metastasis (LLNM) is very common in medullary thyroid carcinoma (MTC), but there is still controversy about how to manage cervical lateral lymph nodes, especially for clinically negative MTC. The aim of this study is to develop and validate a nomogram for predicting LLNM risk in MTC. Materials and methods: A total of 234 patients from two hospitals were retrospectively enrolled in this study and divided into LLNM positive group and LLNM negative group based on the pathology. The correlation between LLNM and preoperative clinical and ultrasound variables were evaluated by univariable and multivariable logistic regression analysis. A nomogram was generated to predict the risk of the LLNM of MTC patients, validated by external dataset, and evaluated in terms of discrimination, calibration, and clinical usefulness. Results: The training, internal, and external validation datasets included 152, 51, and 31 MTC patients, respectively. According to the multivariable logistic regression analysis, gender (male), relationship to thyroid capsule and serum calcitonin were independently associated with LLNM in the training dataset. The predictive nomogram model developed with the aforementioned variables showed favorable performance in estimating risk of LLNM, with the area under the ROC curve (AUC) of 0.826 in the training dataset, 0.816 in the internal validation dataset, and 0.846 in the external validation dataset. Conclusion: We developed and validated a model named MTC nomogram, utilizing available preoperative variables to predict the probability of LLNM in patients with MTC. This nomogram will be of great value for guiding the clinical diagnosis and treatment process of MTC patients.

Clinical Presentation and Treatment Outcomes of Renal Medullary Angiitis in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis: A Single-Center Case Series.

Introduction: Antineutrophil cytoplasmic antibody-associated vasculitis (AAV) with renal involvement primarily affects the renal cortex and presents with key histopathologic findings of a pauci-immune necrotizing and crescentic glomerulonephritis. Infrequently reported and poorly characterized is renal medullary angiitis (RMA), a pathologic variant of AAV primarily involving the renal medulla. This study seeks to describe the presentation and treatment outcomes of RMA. Methods: In this single-center cohort, renal pathology samples classified as AAV with renal involvement underwent secondary review to determine if they met histopathologic criteria for RMA. Demographic, clinical, and laboratory data were obtained via electronic medical record review. Descriptive statistical analysis was performed on key variables. Results: Of the 136 kidney biopsy samples classified as AAV with renal involvement, histopathologic features of RMA were present in 13 cases. The mean (SD) age at the time of RMA diagnosis was 65 (19) years, and 54% were female. Most cases presented with extrarenal manifestations of disease. Initial median (IQR) estimated glomerular filtration rate and proteinuria on presentation were 16 (10-19) mL/min/1.73 m2 and 1,100 (687-2,437) mg, respectively. The primary histologic features were high degrees of interstitial inflammation comprised leukocytes, neutrophils, plasma cells, and eosinophils along with either interstitial hemorrhage or necrosis. All patients were treated with glucocorticoids in combination with either cyclophosphamide, rituximab, or mycophenolate. All patients achieved disease remission. During a median (IQR) follow-up of 42 (14-68) months, 1 patient reached ESKD and 1 patient died. Conclusions: In this single-center case series, we identified the presence of RMA in 9.5% of AAV samples that underwent secondary review. RMA presented with severe impairment in renal function and multisystem disease. Standard of care immunosuppression for AAV was effective for remission induction in RMA. It remains unclear whether standard prognostication tools are useful in this population.

Cortical compensation mechanism for swallowing recovery in patients with medullary infarction-induced dysphagia.

Introduction: This study aims to examine brain activity during different swallowing actions in patients with dysphagia caused by medullary infarction (MI) before and after treatment using blood oxygen level-dependent (BOLD) functional magnetic resonance imaging. Methods: Fifteen patients were enrolled in this study. Brain activation during saliva swallowing and effortful saliva swallowing was observed using BOLD imaging in the acute phase of stroke and after 4 weeks of rehabilitation training. Differences in the activation of brain regions during saliva swallowing before and after treatment, during effortful saliva swallowing before and after treatment, and between the two swallowing actions before and after treatment were compared. Results: In the acute phase of stroke, only the bilateral precentral and left lingual gyrus were partially activated during saliva swallowing, and there was no obvious activation in the insula. Effortful saliva swallowing activated more brain regions than saliva swallowing before treatment, including the bilateral supplementary motor area (SMA), postcentral gyrus, and right insular cortex. The number of brain regions activated during saliva swallowing increased after treatment, including the bilateral precentral gyrus, postcentral gyrus, insula, thalamus, and SMA. Discussion: Cortical activation increases after recovery from dysphagia, and the increased activation of the postcentral gyrus might play a functional compensatory role. Effortful saliva swallowing is a more effective rehabilitation training method for patients with dysphagia caused by MI.

Single-cell sequencing has revealed a more complex array of thymic epithelial cells.

Thymic epithelial cells participate in the maturation and selection of T lymphocytes. This review explores recent insights from single-cell sequencing regarding classifying thymic epithelial cells in both normal and neoplastic thymus. Cortical thymic epithelial cells facilitate thymocyte differentiation and contribute to positive selection. Medullary epithelial cells are distinguished by their expression of AIRE. Cells progress from a pre-AIRE state, containing precursors with cortical and medullary characteristics, termed junctional cells. Mature medullary epithelial cells exhibit promiscuous gene expression and after that downregulate AIRE mRNA. Post-AIRE cells can adopt a Hassall corpuscle-like phenotype or exhibit distinctive differentiation characteristics including tuft cells, ionocytes, neuroendocrine cells, and myoid cells.

Medullary Thyroid Carcinoma in Patients with Graves' Disease-A Case Series and Literature Review.

INTRODUCTION: Graves' disease (GD) is an autoimmune disorder affecting the thyroid gland, leading to systemic manifestations such as hyperthyroidism, Graves' orbitopathy, and pretibial myxedema. Contrary to previous beliefs that hyperthyroidism protects against thyroid cancer, recent studies reveal an increased incidence of thyroid malignancies in GD patients, particularly differentiated thyroid carcinomas and, in rare cases, medullary thyroid carcinoma (MTC). CASE SERIES: This case series presents three female GD patients diagnosed with MTC, highlighting the complexities of diagnosis and management. All patients exhibited thyroid nodules with suspicious ultrasonographic features, elevated plasma calcitonin levels, and required total thyroidectomy. Histological examination confirmed MTC. DISCUSSION: These cases underscore the importance of routine calcitonin screening in GD patients with thyroid nodules to facilitate early detection and improve prognosis. Our findings suggest that while the coexistence of GD and MTC is likely incidental, vigilant monitoring and comprehensive evaluation are crucial for timely intervention. CONCLUSIONS: This study advocates for integrating calcitonin testing into the standard diagnostic protocol for GD patients presenting with thyroid abnormalities.

Opalski syndrome: a rare variant of Wallenberg syndrome: a case report and literature review.

Background: Opalski syndrome is a rare variant of Wallenberg syndrome (lateral medullary syndrome) that demonstrates concomitant ipsilateral hemiplegia due to infarctions within the lateral medulla and the cervical spinal cord, which also extend to the post-pyramidal decussation to affect the corticospinal tract. Case presentation: A 56-year-old man initially presented with a unilateral headache with right cervical pain. Consequently, he developed symptoms that indicate Wallenberg syndrome: vertigo, dysphonia, dysarthria, right limb ataxia with a tendency to fall, and ptosis, in addition to ipsilateral hemiparesis. The diffusion-weighted imaging MRI (DWI MRI) performed initially demonstrated an acute infarct in the dorsolateral medulla. Accordingly, an axial T2-weighted MRI showed a hyperintense focal region in the same area, even as magnetic resonance angiography (MRA) revealed complete stenosis of the corresponding vertebral artery. Discussion: Opalski syndrome can result from different etiologies such as vascular occlusion or dissection. Although symptoms may vary, patients commonly present with sudden weakness, vertigo, and gait instability. Diagnosis often relies on MRI or DW-MRI. Treatment is tailored depending on individual factors and may involve medications and monitoring. Complications like respiratory failure can occur but are not directly linked to Opalski syndrome. Proper management includes addressing ischemic risk factors and ensuring appropriate nutrition. Conclusion: This case highlights the importance of including Opalski syndrome early in the differential diagnosis of patients with clinically suspected ipsilateral lateral medullary infarction and hyperacute ipsilateral hemiparesis.

Association of medullary reticular formation ventral part with spasticity in mice suffering from photothrombotic stroke.

Strokes cause spasticity via stretch reflex hyperexcitability in the spinal cord, and spastic paralysis due to involuntary muscle contraction in the hands and fingers can severely restrict skilled hand movements. However, the underlying neurological mechanisms remain unknown. Using a mouse model of spasticity after stroke, we demonstrate changes in neuronal activity with and without electrostimulation of the afferent nerve to induce the stretch reflex, measured using quantitative activation-induced manganese-enhanced magnetic resonance imaging. Neuronal activity increased within the ventral medullary reticular formation (MdV) in the contralesional brainstem during the acute post-stroke phase, and this increase was characterised by activation of circuits involved in spasticity. Interestingly, ascending electrostimulation inhibited the MdV activity on the stimulation side in normal conditions. Moreover, immunohistochemical staining showed that, in the acute phase, the density of GluA1, one of the α-amino-3 hydroxy-5 methyl -4 isoxazolepropionic acid receptor (AMPAR) subunits, at the synapses of MdV neurons was significantly increased. In addition, the GluA1/GluA2 ratio in these receptors was altered at 2 weeks post-stroke, confirming homeostatic plasticity as the underlying mechanisms of spasticity. These results provide new insights into the relationship between impaired skilled movements and spasticity at the acute post-stroke phase.

LncRNA ZFAS1 promotes invasion of medullary thyroid carcinoma by enhancing EPAS1 expression via miR-214-3p/UCHL1 axis.

lncRNA ZFAS1 was identified to facilitate thyroid cancer, but its role in medullary thyroid carcinoma (MTC) remains unknown. This study aimed to unravel the potential function of this lncRNA in MTC by investigating the involvement of the lncRNA ZFAS1 in a ceRNA network that regulates MTC invasion. Proliferation, invasion, and migration of cells were evaluated using EdU staining and Transwell assays. Immunoprecipitation (IP) assays, dual-fluorescence reporter, and RNA IP assays were employed to examine the binding interaction among genes. Nude mice were used to explore the role of lncRNA ZFAS1 in MTC in vivo. ZFAS1 and EPAS1 were upregulated in MTC. Silencing ZFAS1 inhibited MTC cell proliferation and invasion under hypoxic conditions, which reduced EPAS1 protein levels. UCHL1 knockdown increased EPAS1 ubiquitination. ZFAS1 positively regulated UCHL1 expression by binding to miR-214-3p. Finally, silencing ZFAS1 significantly repressed tumor formation and metastasis in MTC. LncRNA ZFAS1 promotes invasion of MTC by upregulating EPAS1 expression via the miR-214-3p/UCHL1 axis.

Lateral medullary syndrome resulting from atrial fibrillation due to rheumatic heart disease: A case report and literature review.

Lateral medullary syndrome, resulting from cerebellar/brainstem infarction, can occur due to cardioembolic stroke from atrial fibrillation caused by rheumatic heart disease. This rare association highlights the importance of strict arrhythmia management, prophylactic anticoagulation, and timely diagnosis to prevent debilitating neurological outcomes.

Well-defined survival outcome disparity across age cutoffs at 45 and 60 for medullary thyroid carcinoma: a long-term retrospective cohort study of 3601 patients.

Background: Medullary thyroid cancer (MTC) is a challenging malignancy. The survival outcome of MTC based on AJCC staging system does not render a discriminant classifier among early stages. Methods: 3601 MTC patients from 2000 to 2018 were identified from the Surveillance, Epidemiology, and End Results (SEER) database. Smooth curve fitting, Cox proportional hazard regression and competing risk analysis were applied. Results: A linear correlation between age and log RR (relative risk of overall death) was detected. Overlaps were observed between K-M curves representing patients aged 45-50, 50-55, and 55-60. The study cohort was divided into 3 subgroups with 2 age cutoffs set at 45 and 60. Each further advanced age cutoff population resulted in a roughly "5%" increase in MTC-specific death risks and an approximately "3 times" increase in non-MTC-specific death risks. Conclusions: The survival outcome disparity across age cutoffs at 45 and 60 for MTC has been well defined.

Effects of sodium-glucose transporter-2 inhibition on systemic hemodynamics, renal function, and intra-renal oxygenation in sepsis-associated acute kidney injury.

BACKGROUND: People with type 2 diabetes mellitus treated with sodium-glucose transporter-2 inhibitors (SGLT2i) have lower rates of acute kidney injury (AKI). Sepsis is responsible for the majority of AKI in critically ill patients. This study investigated whether SGLT2i is renoprotective in an ovine model of Gram-negative septic AKI. METHODS: Sixteen healthy merino ewes were surgically instrumented to enable measurement of mean arterial pressure, cardiac output, renal blood flow, renal cortical and medullary perfusion, and oxygenation. After a 5-day recovery period, sepsis was induced via slow and continuous intravenous infusion of live Escherichia coli. Twenty-three hours later, sheep were randomized to receive an intravenous bolus of 0.2 mg/kg empagliflozin (n = 8) or a fluid-matched vehicle (n = 8). RESULTS: Empagliflozin treatment did not significantly reduce renal medullary hypoperfusion or hypoxia, improve kidney function, or induce histological changes. Renal cortical oxygenation during the intervention period was 47.6 ± 5.9 mmHg in the empagliflozin group compared with 40.6 ± 8.2 mmHg in the placebo group (P = 0.16). Renal medullary oxygenation was 28.0 ± 18.5 mmHg in the empagliflozin compared with 25.7 ± 16.3 mmHg (P = 0.82). Empagliflozin treatment did not result in significant between-group differences in renal blood flow, kidney function, or renal histopathological changes. CONCLUSION: In a large mammalian model of septic AKI, a single dose of empagliflozin did not improve renal microcirculatory perfusion, oxygenation, kidney function, or histopathology.

Single hole, high-flow, spinal cord peri-medullary arteriovenous fistula (PMAVF) in a child, treated with transarterial embolization: A case report.

Peri-medullary arteriovenous fistula (PMAVF) is a rare spinal vascular malformation that manifests as progressive neurologic deficits or hemorrhage in the spinal canal. We report a case of high-flow PMAVF in a child, with a single feeder artery and a large venous pouch, which was successfully treated with transarterial endovascular intervention. A 2-year-old boy was referred with a progressive 2-year history of myelopathy. The MRI revealed a large venous pouch at the midthoracic spinal cord with segmental surrounding edema. A spinal angiogram confirmed high-flow PMAVF with a single feeder artery from the anterior radiculomedullary artery, draining into the peri-medullary vein. The patient underwent transarterial embolization at the distal feeder artery, resulting in gradual motor strength improvement. PMAVF is classified as type IV spinal vascular malformation, usually presenting as a large, high-flow fistula with multiple feeders, although there was only one in this case. PMAVFs are intradural and may cause severe neurologic deficits due to mass effect, venous congestion, or hemorrhage, hence requiring prompt treatment. Treatment options for PMAVF include microsurgery, endovascular intervention, or a combination of the 2. Endovascular intervention with coil or liquid embolic material is considered first-line treatment for IVc PMAVF, and effective in type IVb with good clinical outcome. PMAVF is a rare spinal vascular malformation commonly manifesting as severe neurologic deficits but has the potential of favorable outcomes with endovascular therapy. This case demonstrates a unique angioarchitecture of high-flow PMAVF with a single feeder artery and large venous pouch, treated successfully with endovascular therapy.