Medullary Compression Research Articles

Ectatic vertebral artery associated medullary compression treated with microvascular decompression: a case report and review of literature

BackgroundVertebral artery compression syndrome is a rare condition defined as the clinical presentation of dizziness, vertigo, ataxia, dysarthria, dysphagia, progressive or acute paralysis, hemisensory loss, and cervical myelopathy secondary to compression of the medulla and upper spinal cord by the vertebral artery.Case descriptionHere we describe a 57-year-old Mexican–American male with bulbar symptoms, positional vertigo, and diplopia. The patient’s symptoms were managed through microsurgical decompression of the medulla utilizing a far lateral transcondylar approach. The patient reported clinical improvement on follow-up.ConclusionThis case underscores the clinical complexity and heterogeneity of this rare neurovascular pathology and the importance of elucidating the pathophysiological mechanisms underlying the symptomatic presentation of vertebral artery compression syndrome.

Intracranial pulse pressure amplitude as an indicator of intracranial compliance: observations in symptomatic children with Chiari malformation type I.

Reduced intracranial compliance (ICC) may be an important factor in the pathophysiology of Chiari malformation type I (CM-I). However, direct measurement of ICC is controversial because of its invasiveness, particularly in children. Instead, ICC may be estimated from continuous measurements of intracranial pressure (ICP), where the metric mean wave amplitude (MWA) has been found to be more useful as a surrogate marker of ICC than mean ICP. This observational study investigated the distribution of MWA and mean ICP in symptomatic children with CM-I, as well as their association with clinical and radiological findings. From a consecutive series of children treated for CM-I at a single institution between 2006 and 2023, the authors analyzed ICP scores in those who underwent an overnight preoperative ICP recording in which MWA was calculated. Clinical and radiological data were retrieved from the patient records. Thirty-seven children (mean age 12.4 ± 3.6 years) with symptomatic CM-I were identified. From the overnight ICP measurements, the average MWA was 5.2 ± 1.3 mm Hg: 56% of children had an abnormal MWA (> 5 mm Hg) and 33% had a borderline MWA (4-5 mm Hg). In contrast, the average mean ICP was 9.7 ± 4.1 mm Hg: 8% of children had an abnormal mean ICP (> 15 mm Hg) and 41% had a borderline mean ICP (10-15 mm Hg). Thus, more children were found to have an abnormal MWA than an abnormal mean ICP (p < 0.001). MWA was significantly higher in the subgroup of children with medullary compression in the foramen magnum, as seen on MRI, than in those without (5.6 ± 1.0 mm Hg vs 4.7 ± 1.4 mm Hg, p = 0.03), whereas a similar difference was not observed for mean ICP (9.9 ± 4.6 mm Hg vs 9.7 ± 3.7 mm Hg, p = 0.889). In this cohort of symptomatic children with CM-I, MWA was more frequently abnormal than mean ICP, with a clinically significant discrepancy in half of the patients. Moreover, MWA was significantly higher in patients with medullary compression. Based on these findings, the authors' interpretation is that in children with CM-I, the ICC may be reduced, as indicated by increased MWA, even though the mean ICP is within normal thresholds.

Ventrolateral medullary compression by vascular contact in primary hemifacial spasm: a radiological analysis.

The neurovascular conflict (NVC) causing hemifacial spasm (HFS) can also cause compression of ventrolateral medulla (VLM) which contains the central sympathetic neurons. VLM compression has been associated with hypertension. Whether the VLM compression in HFS patients is associated with hypertension is not clear. To determine the frequency, severity of VLM compression and its association with hypertension in HFS patients. A cross-sectional, hospital-based, case control study and recruited 120 study subjects (50 cases of primary HFS, 30 hypertensive and 40 normotensive age-, sex- matched controls). The VLM compression was assessed in magnetic resonance imaging Constructive Interference in Steady State (CISS) 3D sequences. Hypertension was present in 30 cases (60%). Six patients with HFS (20%) were detected to be hypertensive after the onset of HFS. VLM compression was seen in 24 cases (48%), 7 hypertensive controls (23.3%) and 5 normotensive controls (10%) (p = 0.03). Twenty-four patients with hypertension had VLM compression and remaining 6 patients with hypertension did not have VLM compression (80% vs 20%; p = 0.02). Normotensive patients did not have VLM compression. Vertebral artery was the most common artery causing VLM compression (22 patients; 7 hypertensive and 5 normotensive controls). VLM compression is more common in HFS patients as compared to hypertensive and normotensive controls. It is more common in hypertensive HFS patients in comparison with normotensive HFS patients. Microvascular decompression is an option in hypertensive HFS patients with VLM compression if the hypertension is medically refractory.

Lateral medullary vascular compression manifesting as paroxysmal hypertension

Neurovascular compression of the rostral ventrolateral medulla (RVLM) has been described as a possible cause of refractory essential hypertension. We present the case of a patient affected by episodes of severe paroxysmal hypertension, some episodes associated with vago-glossopharyngeal neuralgia. Classical secondary forms of hypertension were excluded. Imaging revealed a neurovascular conflict between the posterior inferior cerebellar artery (PICA) and the ventrolateral medulla at the level of the root entry zone of the ninth and tenth cranial nerves (CN IX-X REZ). A MVD of a conflict between the PICA and the RVLM and adjacent CN IX-X REZ was performed, resulting in reduction of the frequency and severity of the episodes. Brain MRI should be performed in cases of paroxysmal hypertension. MVD can be considered in selected patients.

Contribution of Myélo-Computed Tomography in the assessment of slow spinal cord compressions: Retrospective study of 33 cases in Niamey

Purpose: To describe the contribution of Myélo-computed tomography during the assessment of slow spinal cord compressions and to determine the etiological profile. Patients and methods: This is a retrospective, cross-sectional, analytical and descriptive study of 33 cases collected in the radiology and imaging department of the National Hospital of Niamey (HNN) over a period of 14 months (from 31/12/2015 to 10/02/2017). Were included in the study all patients with slow spinal cord compression occurred in a non-traumatic setting, admitted to the service for a Myélo-computed tomography. Results: The mean age of the patients was 47.03 years with extremes ranging from 27 to 75 years. The 30-35 age group was the most affected with 23.2% of cases. In our study, Myélo-computed tomography detected medullary compression in 33 patients (33%). The extra-dural compartment was the most common site of slow medullary compression with 97% and intra dural extra marrow with 3%. The dorsal spine was the most affected stage by slow spinal compression with 52% followed by the cervical (27%) and lumbar (22%) stage. Infectious pathology was the most common cause of spinal cord compression with a frequency of 63.6%, followed by degenerative (27.3%) and tumoral (9.1%) pathology. Conclusion: Myélo-computed tomography plays an important role in topographic and etiologic diagnosis of slow spinal cord compression in the absence of MRI.

Retro-Odontoid Pseudotumor due to Rheumatoid Arthritis Pannus and its Craniocervical Instability

Retro-Odontoid Pseudotumor due to Rheumatoid Arthritis Pannus and its Craniocervical Instability

Navigating the complexities of encephalocraniocutaneous lipomatosis: a case series and review

IntroductionEncephalocraniocutaneous lipomatosis (ECCL) is a rare congenital syndrome with complex skin, eye, and central nervous system (CNS) symptoms. Diagnosis and treatment are challenging due to its rarity and diverse manifestations. It often involves issues like porencephalic cysts, cortical atrophy, and low-grade gliomas in the CNS, resulting in developmental delays. The spinal cord is frequently affected, leading to problems like medullary compression and radiculopathy, causing back pain and sensory/motor deficits. Surgical interventions are reserved for symptomatic cases to address hydrocephalus or alleviate spinal lipomas. This article reviews a case series to assess surgical risks and neurological outcomes.Case seriesWe present a case series ECCL, focusing on the diffuse lipomatosis of the spinal cord and the intricate surgical procedures involved. A multi-stage surgical approach was adopted, with continuous neuromonitoring employed to safeguard motor pathways. We discuss clinical characteristics, imaging studies, and indications for neurosurgical interventions.DiscussionECCL is a complex syndrome. Diagnosis is challenging and includes clinical evaluation, neuroimaging, and genetic testing. Treatment targets specific symptoms, often requiring surgery for issues like lipomas or cerebral cysts. Surgery involves laminectomies, spinal fusion, and motor pathway monitoring. Thorough follow-up is crucial due to potential CNS complications like low-grade gliomas. Hydrocephalus occurs in some cases, with endoscopic third ventriculostomy (ETV) preferred over ventriculoperitoneal shunt placement.ConclusionNeurosurgery for ECCL is for symptomatic cases. ETV is preferred for hydrocephalus, while the treatment for lipoma is based on the presence of symptoms; the follow-up should assess growth and prevent deformities.

Construction and clinical validation of risk model for predicting bone cement leakage after the surgical management of spinal metastases.

This study aimed to comprehensively analyze the risk factors associated with bone cement leakage (LCK) during the surgical management of spinal metastases, construct a joint risk model for predictive assessment, and validate the clinical applicability of the risk model in an independent patient cohort. A retrospective analysis was conducted on patients who underwent surgery for spinal metastases between February 2022 and June 2023. Patients were divided into a non-LCK group (n=134) and an LCK group (n=86) based on the presence or absence of bone cement leakage after surgery. Additionally, a validation group was established, consisting of 21 patients with LCK and 65 patients without. Analysis focused on patient demographics, intraoperative parameters, LCK location, complications, pain management, and improvements in activities of daily living (ADL). Logistic regression, calibration curve, clinical impact curve (CIC) analysis, decision curve analysis (DCA) and receiver operating characteristic (ROC) analysis were used to assess the risk factors and construct a joint risk model. There were significant differences between the two groups in pathologic fracture, Tomita classification, posterior wall destruction, injected laterality, injected bone cement volume, radicular pain, pulmonary embolism, and medullary compression. Pathologic fracture, radicular pain, pulmonary embolism, and medullary compression were positively correlated with the occurrence of LCK, while Tomita classification, posterior wall destruction, injection laterality, and injected bone cement volume were negatively correlated with the occurrence of LCK. Pathological fracture, Tomita classification, posterior wall destruction, injected laterality, injected bone cement volume, and specific postoperative complications were identified as significant risk factors associated with LCK. The constructed joint risk model, incorporating these risk factors, demonstrated substantial predictive value, with an Area Under the Curve (AUC) of 0.885. Clinical validation in an independent patient cohort further confirmed the predictive power of the joint risk model, with an AUC of 0.846. This study underscores the multifactorial nature of LCK in surgical management of spinal metastases, providing valuable insights for risk assessment and management.

Compressive Myelopathy Caused by Arachnoid Cyst and Extramedullary Hematopoietic Tissue in a Patient with Thalassemia Major

AbstractThalassemias, inherited diseases of hemoglobin synthesis, are characterized by the presence of deficient hemoglobin chains that deposit in red blood cells, inducing hemolytic anemia. Extramedullary hematopoiesis represents a compensatory picture that usually affects the liver, the spleen, and lymph nodes. The involvement of the epidural space with spinal cord compression is extremely rare. Our objective was to describe the case of RMS, 31 years old, male, β-thalassemia major carrier, admitted with 2-month progressive paraparesis and urinary retention due to medullary compression by extramedullary hematopoietic tissue and thoracic arachnoid cyst, and to discuss therapeutic options. Magnetic resonance imaging (MRI) showed an extensive intraspinal and extramedullary lesion with homogeneous contrast enhancement of T3-T11 in addition to a T1-T3 cystic lesion isointense to cerebrospinal fluid (CSF). After the presumed diagnosis of spinal cord compression by proliferative hematopoietic tissue, a 10-session fractional radiotherapy treatment was immediately performed. After the radiotherapy treatment, the neurological deficits of the patient persisted despite the excellent image response with almost complete disappearance of the intraspinal mass. However, the MRI showed a persistent T1-T3 cystic lesion with significant mass effect on the spinal cord. The patient was submitted to microsurgery for total resection of this cystic lesion. In the postoperative period, the patient improved his sphincter control and motor deficits. Medullary compression by extramedullary epidural hematopoiesis is a rare complication in thalassemic patients and may be treated with surgery and/or radiotherapy. There are successful cases with the exclusive use of radiotherapy, especially in extensive lesions.

Acute cervical spinal cord injury after head and neck surgery: A CARE case report

ObjectiveTo describe and analyze a case of acute spinal cord injury after head and neck surgery. DescriptionOne hour after left lobo-isthmectomy under laryngeal neuromonitoring for a 3-cm EU-TIRADS 4 – Bethesda 4 thyroid nodule in a 48-year-old euthyroid male without any known comorbidity, left hemiparesis occurred. Cervical spine MRI showed an anteromedial herniated C6-7 disk with medullary compression. The disk was resected, compression was released and C6-7 fusion was performed via an anterior cervical approach on postoperative day 1. Postoperative course was unremarkable, with complete recovery of motion within 2 days. One month later, neurological clinical examination was normal and interview revealed left cervicalgia with onset a few days prior to lobo-isthmectomy. One year later, at the time of writing, the patient was doing fine. ConclusionOtorhinolaryngologists and head and neck surgeons must be aware of the risk of acute cervical spinal injury after cervical mobilization in head and neck surgery, and should take all measures to avoid this exceptional but dramatic complication.

Vascular Compression of Medulla Oblongata by Non-Dolichoectatic Vertebral Artery

AbstractNeurovascular conflicts are common in the posterior fossa and basal cisterns due to coexistence of important neural and vascular structures. Neurovascular conflict arising from compression of the cranial nerves by pulsatile flow in the adjacent atherosclerotic arteries is well known and is associated with conditions like trigeminal neuralgia, hemifacial spasm, and glossopharyngeal neuralgia. The medulla is known to be affected by dilated tortuous (dolichoectatic) vertebrobasilar arteries in the elderly or hypertensive. The vertebral artery causing the compression can be dolichoectatic or normal dominant vertebral artery or an elongated tortuous artery. Very few cases of medullary compression by non-dolichoectatic elongated tortuous or dominant vertebral artery were reported in the literature. In this article, we report three cases of medullary compression by the dominant and angulated vertebral artery. Magnetic resonance (MR) imaging with MR angiography showed indentation of the anterolateral aspect of the inferior medulla by the vertebral artery. The patients are managed conservatively and on regular follow-up.

Cervical disc arthroplasty with systematic total bilateral uncuscectomy - Adapted technique particularly in severe spondylosis: A prospective study

IntroductionCervical disc arthroplasty (CDA) is mainly used in young patients with soft herniated discs and seems to have several advantages over anterior cervical discectomy and fusion (ACDF). Severe spondylosis is common and represents a contraindication for performing CDA. Research questionIs it possible to expand the indications for the implantation of cervical prostheses by adapting the surgical technique, particularly for severe spondylosis, to benefit from the advantages of prostheses over ACDF ? Materials and methodsWe propose a prospective two-center study to compare the possible clinical benefit of the placement of a cervical prosthesis with systematic total bilateral uncuscectomy (or uncinectomy) compared to the classical technique of ACDF, particularly for severe spondylosis. Visual analog scales for brachialgia, cervicalgia, and neck disability index were measured before and one year after surgery. Odom’s criteria were assessed one year after surgery. ResultsWe compared 81 patients treated with CDA and systematic total bilateral uncuscectomy versus 42 patients treated with ACDF for symptomatic radicular or medullary compression. Patients treated with CDA and uncuscectomy showed greater improvements in VASb, VASc, NDI, and Odom’s criteria than those treated with ACDF, with statistically significant results. Moreover, no difference was found between the severe spondylosis subgroup and the non-severe spondylosis subgroup treated with CDA and uncuscectomy. Discussion and conclusionThis study assessed the value of systematic total bilateral uncuscectomy for cervical arthroplasty. Our prospective clinical results suggest a surgical technique to reduce cervical pain and improve function one year after surgery, even in cases of severe spondylosis.

Intramedullary arachnoid cyst: A rare cause of medullary compression

This clinical case of intramedullary arachnoid cyst is the second reported case in Senegal. It concerns a 50-yearold woman, with no particular history, admitted for back pain associated with intermittent spinal claudication. The physical examination showed a dorsal spinal syndrome, spastic paraparesis, hypoesthesis under T9 sensitive territory with bilateral Babinski sign. Cutaneous-abdominal reflexes were abolished at T8. Magnetic resonance imaging (MRI) revealed an intramedullary cyst formation. The patient was operated on the day after her admission by a posterior approach with scopic tracking. She underwent a laminectomy from T7 to T9. Thereafter the cyst has been removed, then discharged three days after the surgery. The clinical follow-up showed a full recovery.

Patients with degenerative cervical myelopathy exhibit neurophysiological improvement upon extension and flexion: a retrospective cohort study with a minimum 1-year follow-up

BackgroundCervical extension and flexion are presumably harmful to patients with degenerative cervical myelopathy (DCM) because they worsen medullary compression visible on dynamic magnetic resonance imaging (MRI). Dynamic somatosensory evoked potentials (SSEPs) are an objective tool to measure the electrophysiological function of the spinal cord at different neck positions. In contrast to previous hypotheses, a considerable proportion of patients with DCM present improved SSEPs upon extension and flexion compared to a neutral position.MethodsPatients with DCM who underwent preoperative dynamic SSEP examinations and subsequent decompression surgeries between 2015 and 2019 were retrospectively evaluated. We compared extension and flexion SSEPs with neutral SSEPs in each patient and classified them into extension-improved (EI) or extension-nonimproved (EN) and flexion-improved (FI) or flexion-nonimproved (FN) groups. Preoperative clinical evaluations, decompression surgical methods and one-year follow-up clinical data were recorded. Cervical spondylolisthesis and cervical alignment types were evaluated on plain cervical lateral radiographs. The number of stenotic segments, Mühle stenosis grade and disc degeneration stage of the most severe segment, and presence of ligamentum flavum hypertrophy and intramedullary T2 weighted imaging (T2WI) hyperintensity were evaluated on lateral and axial MRI. Data were compared between the EN and EN groups or FI and FN groups with T-tests, chi-square tests or Kruskal-Wallis tests. Prediction criteria were determined with logistic regression analyses.ResultsForty-nine patients were included, and 9 (18.4%) and 11 (22.4%) showed improved extension and flexion SSEPs compared to their own neutral SSEPs, respectively. Interestingly, EI or FI patients had significantly better one-year postoperative mJOA recoveries than EN or FN patients (T-test, P < 0.001). Moreover, the disease duration (T-test, P = 0.024), involved segment number (Kruskal-Wallis test, P < 0.001), and cervical alignment type (chi-square test, P = 0.005) varied significantly between the EI and EN groups. The FI group presented a significantly higher Mühle stenosis grade than the FN group (Kruskal-Wallis test, P = 0.038). Furthermore, ≤ 2 involved segments and straight or sigmoid cervical alignment were significant criteria predicting improved extension SSEPs (probability: 85.7%), while Mühle stenosis Grade 3 and disease duration ≤6 months were significant criteria predicting improved flexion SSEPs (probability: 85.7%).ConclusionsOur findings provide evidence for neurophysiological improvement in patients with DCM at extension and flexion and its significance in predicting prognoses. Moreover, certain clinical and radiographic criteria may help predict neurophysiological improvement upon extension or flexion.Trial registration“[2020]151”. Retrospectively registered on April 30, 2020.

Successful rehabilitation program after AIS/Frankel C paraplegia through a recently operated lumbar disc hernia

Intervertebral disc herniation is the pathological process by which fragments of the nucleus pul-posus tear the fibers of the annulus fibrosus and come into contact with the root of the spinal nerve (1). The most significant functional damage generated by the lumbar disc herniation is paraplegia and can be ameliorated by an early and staged specific rehabilitation program. Materials and Methods: This paper presents the case of a 56-year-old patient who was admitted to the Neurosurgery Clinic (NCH) III of SCUBA for low back pain and motor deficit, AIS Frankel C paraplegia. The left lower limb was more affected than the right one. It occurred following a thoraco-lumbar medullary compression and a paramedian lumbar disc herniation L3. The pa-tient was treated surgically. Subsequently, the patient was transferred to the Neuro-Muscular Recovery Clinic of SCUBA for the specific rehabilitation treatment, with indication for mobiliza-tion. He was dynamically evaluated using the following scales: quality of life assessment (QOL), modified Ashworth scale, Functional Assessment Classification, FAC, Activities of daily living (ADL), Spinal Cord Independence Measure (SCIM), evaluation of muscle strength on Medical Re-search Council, MRC, scale, evaluation of American Spinal Injury Association Impairment Scale (AIS). Results: The patient benefited from a complex program of neuro-muscular rehabilitation, hav-ing a favorable evolution with an improving score of the evaluating scales and finally gaining his gait balance, including ascending and descending stairs (instrumentally assisted for left plan-tar dorsiflexion movement with orthosis walking). At discharge the patient’s neurological defi-cit was reclassified as AIS D paraplegia, with the neurogenic bladder and bowel having been remitted. Discussions: The disc herniation at L3 level generated a cauda equina syndrome, which initially generated a paraplegia. When the inflammation remitted it became clear that the left L4 root was affected, with a complete deficit of plantar dorsiflexion. In conclusion it was not a case of spinal cord syndrome (as the spinal cord ends at L2 level) or conus medullaris syndrome. Conclusions: The interdisciplinary therapeutic approach together with a specific, customized re-habilitation program for a patient with AIS C paraplegia after a surgically treated disc hernia is successfully improving the neuromuscular deficit and upgrading the patient's quality of life. Keywords: rehabilitation, disc hernia, low back pain, paraplegia

Vertebral artery compression of the medulla oblongata: A benign radiological finding?

Background: To the best of our knowledge, no study has documented the natural history of rostral medullary compression of the vertebral artery (RMCVA) as radiological finding. The aim of this study was to explore it.Methods: A total of 57 patients with RMCVA and not presenting symptoms of medullary compression syndrome were enrolled. These participants underwent cerebral magnetic resonance imaging with contrast, and 19 of them who were followed for 5.7 ± 1.9 years (range: 3.0–10.3 years) were analyzed in detail. For comparison, clinical courses of two other patients with vertebrobasilar dolichoectasia (VBDE) were presented.Results: RMCVA was well delineated in all 57 patients. In the 19 patients analyzed, RMCVA was found in 17 sides on the right and 15 on the left. Moreover, the ventrolateral medulla was the most frequent compression site, and it was found in 69% of cases, with 84.2% presenting as mild compression and 15.8% as considerable compression. During the follow-up period, no patients showed neurological deterioration or radiological progression. In contrast, the two VBDE patients demonstrated both neurological and radiological progressions during the follow-up period.Conclusion: Unlike VBDE, RMCVA seems to be a benign condition without progression, even when with a considerable compression. Degree of the compression in RMCVA may not be relevant to the patient’s neurological status.

Foramen Magnum Meningioma-The Attainment of the Intra-Arachnoidal Dissection: 2-Dimensional Operative Video.

Ventral foramen magnum meningiomas are a forbidding lesion. The stake is so high with a risk of devastating paralysis and respiratory failure. Careful preoperative clinical and radiological evaluation is necessary to implement the best treatment plan. Successful surgical intervention depends on paying high attention to minute details throughout the case, from intratracheal intubation to extubation. The neural head-on-neck position is critical to avoid further medullary compression at intubation and positioning.1 Extensive neurophysiological monitoring, including somatosensory, motor, brainstem evoked potential, and cranial nerves, during the positioning and throughout the case, is extremely helpful to detect early signs of dysfunction.1 To expose and access ventral tumors, partial condyle resection and vertebral artery transposition are invaluable techniques.2,3 Preservation and minute manipulation of the vital neurovascular structures at this junction that includes the medullar, anterior spinal artery, posterior inferior cerebellar artery, vertebral junction perforators, and lower cranial nerves are essential for good outcomes. This is achieved by microsurgical intra-arachnoidal dissection under high magnification and after debulking the tumor to establish that plane.1,3,4 The demonstration of this technique is the purpose of this article. We demonstrate these surgical tenets applied to the resection of a large ventral foramen magnum meningioma extending from the midclivus to the C3 vertebral body level in a 54-yr-old female presenting with swallowing difficulties. The patient consented to the surgical intervention and the publication of her images. Image at 1:38 reprinted with permission from Al-Mefty O, Operative Atlas of Meningiomas. Vol 1, © LWW, 1998.

GRANULOCYTIC SARCOMA OF THE CNS AS A CAUSE OF MEDULLARY COMPRESSION: A CASE REPORT

A 24-year-old man, previously healthy, presented to the hospital emergency in February 2021, complaining of pain in the back region radiating to the flanks and hips for 12 days. He had previously sought care on three occasions, without relief with anti-inflammatory drugs. On admission, his physical examination was normal, and laboratory tests showed 23900 leukocytosis and 70% blasts, no anemia or thrombocytopenia, normal renal and liver function tests, and negative serologies. Chest radiography and ultrasound of the abdomen plus urinary tract showed no particularities. Immunophenotyping was requested, which diagnosed maturation acute myeloid leukemia; the patient was hospitalized for therapy. He still complained of intermittent pain crises in the lumbar region, evolving with bladder dysfunction, weakness, and paresthesia in both lower limbs. Upon starting protocol with Cytarabine + Daunorubicin on the fourth day of hospitalization, the man evolved with proprioceptive loss and paraplegia of the lower limbs. Urgent magnetic resonance was requested, showing an expansive formation in the posterior epidural space at the level of T9-T11, suggestive of granulocytic sarcoma. Lesion biopsy was not performed. A course of dexamethasone was carried out and a response to chemotherapy was observed; the patient had a partial return of strength in both lower limbs and completed the systemic treatment. Acute myeloid leukemia (AML) is the most prevalent acute leukemia in adults, representing about 1% of all neoplasms, 10% of which are of the subtype called AML with maturation. This is the subtype most associated with granulocytic sarcoma, also called chloroma. Granulocytic sarcoma is a solid tumor of granulocyte precursor cells located in various extramedullary sites, which can cause a copious constellation of symptoms. In this case, MRI showed a highly suspicious lesion for infiltrative chloroma in the CNS, causing a medullary compression syndrome with serious clinical repercussions. In other tissues, local management of the tumor can be performed with surgical resection or radiotherapy, which were unfeasible therapeutic options for the case presented. Although rare, granulocytic sarcoma should be considered a differential diagnosis in patients with acute leukemia, who present with compressive symptoms. In some cases, it may represent a hematological urgency, even before the primary neoplasm diagnosis, and its correct management is essential in such situations.

Management of anesthesia for surgery in prone position for a patient with hereditary neuropathy with liability to pressure palsies. A case report and narrative literature review

The reported case describes a patient with hereditary neuropathy with liability to pressure palsies (HNPP) requiring a neurosurgical laminotomy procedure for an arachnoid cyst with medullary compression. HNPP is a rare pathology requiring meticulous anesthetic and surgical management. We give a detailed overview of the planning, simulation, and performance of the anesthesia procedure in order to minimize all potential risk factors for perioperative nerve injury. During the preparation of this case, where the positioning was extremely challenging, we reviewed the available literature for guidance. A few papers report the use of neuraxial anesthesia in patients previously diagnosed with HNPP. No reports could be found on the management of general anesthesia and positioning for major surgery in those patients.

Musculoskeletal anomalies in children with Mucopolysaccaridoses

Introduction The accumulation of glycosaminoglycan (GAGs) in the tissues in Mucopolysaccharidoses (MPS) can lead to skeletal anomalies (DYSOSTOSIS MULTIPLEX) and to soft tissue impairments (neural or medullar compression, joint stiffness, tenosynovitis). Here is a review of orthopedic issues frequently encountered in patients with MPS. Material and methods Surgery may be justified at different age and according to the type of MPS. Different surgical approaches and their indications are exposed in the article. Results The article exposes indications and techniques for orthopedic issues in MPS children: cervical stenosis, cervical instability, kyphosis, hip dysplasia and hip dislocation, genu valgum. Conclusion Various musculoskeletal anomalies can be found in patients with mucopolysaccharidoses. Neurological impairments are frequently seen due to cervical stenosis or instability and should be early detected with regular MRI of the cervical spine. Well-codified management should lead to favorable functional results and maintain functional and walking abilities.