Effect of a cervical collar on optic nerve sheath diameter in trauma patients

BACKGROUND:As advocated in advanced trauma life support and prehospital trauma life support protocols,cervical immobilization is applied until cervical spine injury is excluded.This study aimed to show the difference in optic nerve sheath diameter(ONSD)between patients with and without a cervical collar using computed tomography(CT).METHODS:This was a single-center,retrospective study examining trauma patients who presented to the emergency department between January 1,2021,and December 31,2021.The ONSD on brain CT of the trauma patients was measured and analyzed to determine whether there was a difference between the ONSD with and without the cervical collar.RESULTS:The study population consisted of 169 patients.On CT imaging of patients with(n=66)and without(n=103)cervical collars,the mean ONSD in the axial plane were 5.43±0.50 mm and 5.04±0.46 mm respectively for the right eye and 5.50±0.52 mm and 5.11±0.46 mm respectively for the left eye.The results revealed an association between the presence of a cervical collar and the mean ONSD,which was statistically significant(P<0.001)for both the right and left eyes.CONCLUSION:A cervical collar may be associated with increased ONSD.The effect of this increase in the ONSD on clinical outcomes needs to be investigated,and the actual need for cervical collar in the emergency department should be evaluated on a case-by-case basis.

Epithelioid malignant peripheral nerve sheath tumor of the bladder and concomitant urothelial carcinoma: A case report

BACKGROUND Epithelioid malignant peripheral nerve sheath tumor(EMPNST)of the bladder is a rare entity with devastating features.These tumors are thought to originate from malignant transformation of pre-existing schwannomas of pelvic autonomic nerve plexuses,and unlike the conventional malignant peripheral nerve sheath tumor(MPNST),are not associated with neurofibromatosis.The tumor has dis-tinctive morphological,immunohistochemical and molecular features.Addi-tionally,it tends to be more aggressive and have a higher mortality.This is the first case that presents with a synchronous urothelial carcinoma of the bladder and the epithelioid variant of MPNST in the literature.It’s also the second re-ported case of EMPNST originating from the bladder wall.CASE SUMMARY In this case report,we present the detailed clinical course of a 71-year-old patient with EMPNST of the bladder alongside a literature review.CONCLUSION During the management of EMPNST cases,offering aggressive treatment moda-lities to the patient,such as radical cystectomy,is appropriate for the best chance to contain the disease,regardless of the tumor stage and the extent of local disease at initial diagnosis.

Optic nerve sheath diameters in nontraumatic brain injury:A scoping review and role in the intensive care unit

BACKGROUND Neuromonitoring in medical intensive care units is challenging as most patients are unfit for invasive intracranial pressure(ICP)modalities or unstable to transport for imaging.Ultrasonography-based optic nerve sheath diameter(ONSD)is an attractive option as it is reliable,repeatable and easily performed at the bedside.It has been sufficiently validated in traumatic brain injury(TBI)to be incorporated into the guidelines.However,currently the data for non-TBI patients is inconsistent for a scientific recommendation to be made.AIM To compile the existing evidence for understanding the scope of ONSD in measuring ICP in adult non-traumatic neuro-critical patients.METHODS PubMed,Google Scholar and research citation analysis databases were searched for studies in adult patients with non-traumatic causes of raised ICP.Studies from 2010 to 2024 in English languages were included.RESULTS We found 37 articles relevant to our search.The cutoff for ONSD in predicting ICP varied from 4.1 to 6.3 mm.Most of the articles used cerebrospinal fluid opening pressure followed by raised ICP on computed tomography/magnetic resonance imaging as the comparator parameter.ONSD was also found to be a reliable outcome measure in cases of acute ischaemic stroke,intracerebral bleeding and intracranial infection.However,ONSD is of doubtful utility in septic metabolic encephalopathy,dysnatremias and aneurysmal subarachnoid haemorrhage.CONCLUSION ONSD is a useful tool for the diagnosis of raised ICP in non-traumatic neuro-critically ill patients and may also have a role in the prognostication of a subset of patients.

Analyses on the misdiagnoses of 25 patients with unilateral optic nerve sheath meningioma

AIM：To investigate clinical features of optic nerve sheath meningioma（ONSM） that was misdiagnosed,and to find methods to reduce the misdiagnoses.METHODS：Retrospective series study.Twenty-five misdisgnosed patients with unilateral ONSM were collected from Jan.2008 to Jan.2015 and the clinical records reviewed.RESULTS：Patients were misdiagnosed with acute papillitis most frequently（P=17）,immediately followed by optic atrophy（P=8）,ischemic optic neuropathy（P=5）,acute retrobulbar optic neuritis（P=5）,optic disc vasculitis（P =3）.For each patient,the minimum frequency of misdiagnoses was once and the maximum was 4 times.As for the lasting time of being misdiagnosed,the shortest was 1.5mo and the longest was 45 mo.Twentyone cases（84%） were once treated with glucocorticoids,and its side effects was found in seventeen patients.Twenty patients（80%） complained with varying degree of vision loss.When a definite diagnosis was made,sixteen cases（64%） showed slight exophthalmos and eighteen cases（72%） had the tubular ONSM.CONCLUSION：ONSM without loss obvious exophthalmos is easily misdiagnosed in clinic,and for most of these ONSMs are tubular.

Optic nerve sheath diameters in healthy adults measured by computer tomography

AIMTo measure optic nerve sheath diameters (ONSD) in different locations by computer tomography (CT) and to recommend the best location for cases when ONSD is used for intracranial pressure monitoring.METHODSIn a prospective cohort study, CT data of 300 healthy adults were analyzed (600 eyes). In all cases, the CT investigation was performed at the Emergency Department because of the various conditions that proved not to be connected with ophthalmological or neurological pathology. The ONSD were measured at 3 mm and 8 mm distance from the globe, and 3 mm from the anterior opening of the optic canal. The correlation analysis was performed with gender, age, and ethnic background.RESULTSThe right/left ONSD are 4.94&#x000b1;1.51/5.17&#x000b1;1.34 mm at 3 mm, 4.35&#x000b1;0.76/4.45&#x000b1;0.62 mm at 8 mm from the globe, and 3.55&#x000b1;0.82/3.65&#x000b1;0.7 mm at 3 mm from the optic canal. No significant differences correlated with gender of the patients, their age, and ethnic background were found.CONCLUSIONIn healthy persons, the ONSD varies from 5.17&#x000b1;1.34 mm to 3.55&#x000b1;0.82 mm in different locations within the intraorbital space. The most stable results with lesser standard deviation can be obtained if it is measured 8-10 mm from the globe.

Prognostic values of optic nerve sheath diameter for comatose patients with acute stroke:An observational study

BACKGROUND Optic nerve sheath diameter(ONSD)measurement is one of the non-invasive methods recommended for increased intracranial pressure(ICP)monitoring.AIM This study aimed to evaluate the roles of optic nerve sheath diameter(ONSD)and ONSD/eyeball transverse diameter(ETD)ratio in predicting prognosis of death in comatose patients with acute stroke during their hospitalization.METHODS A total of 67 comatose patients with acute stroke were retrospectively recruited.The ONSD and ETD were measured by cranial computed tomography(CT)scan.All patients underwent cranial CT scan within 24 h after coma onset.Patients were divided into death group and survival group according to their survival status at discharge.The differences of the ONSD and ONSD/ETD ratio between the two groups and their prognostic values were compared.RESULTS The ONSD and ONSD/ETD ratio were 6.07±0.72 mm and 0.27±0.03 in the comatose patients,respectively.The ONSD was significantly greater in the death group than that in the survival group(6.32±0.67 mm vs 5.65±0.62 mm,t=4.078,P<0.0001).The ONSD/ETD ratio was significantly higher in the death group than that in the survival group(0.28±0.03 vs 0.25±0.02,t=4.625,P<0.0001).The area under the receiver operating characteristic curve was 0.760(95%CI:0.637-0.882,P<0.0001)for the ONSD and 0.808(95%CI:0.696-0.920,P<0.0001)for the ONSD/ETD ratio.CONCLUSION The mortality increased in comatose patients with acute stroke when the ONSD was>5.7 mm or the ONSD/ETD ratio was>0.25.Both indexes could be used as prognostic tools for comatose patients with acute stroke.The ONSD/ETD ratio was more stable than the ONSD alone,which would be preferred in clinical practice.

Clinical Analysis of 73 Cases of Intraspinal Nerve Sheath Tumor

Seventy-three patients with spinal nerve sheath tumor who were surgically treated in our hospital during the years 2004-2010 were retrospectively reviewed with respect to recovery of neurological function, recurrence of the tumor and occurrence of kyphotic deformities. Preoperative clinical manifestations, imaging data, surgical records and follow-up results were comprehensively analyzed. The follow-up duration was 12-60 months with an average of 32.0 months. Out of the 73 cases enrolled, 69 had gradual recovery of sensation, motor and sphincter functions 1 week to 3 months after operation. Forty-six cases had incomplete paralysis, whose American Spinal Injury Association （ASIA） grades, however, were gradually increased during the follow-up period, 4 cases had no significant improvement of the clinical symptoms and no change in ASIA grades during the follow-up period. Two cases had postoperative recurrence of the tumor. There were no deaths, no spinal instability, and no kyphotic malformations fotmd in any cases. Our study indicated that complete removal of the tumor is important for good recovery, and an ideal surgical method may reduce the recurrence of the tumor or the occurrence of complications.

The clinical value of dynamic ultrasound measurement of optic nerve sheath diameter in the treatment of patients with moderate and severe craniocerebral injury

Objective:To explore the clinical value of dynamic ultrasound monitoring of optic nerve sheath diameter(ONSD)in the treatment of patients with moderate and severe head injury(CI).Methods:A total of 160 patients with moderate and severe CI admitted to the First Affiliated Hospital of Hainan Medical University from January 2018 to January 2020 were selected and divided into observation group(80 cases)and control group(80 cases)a Januaryccording to the random number table.Patients in control group and observation group were dehydrated to reduce intracranial pressure(ICP)according to clinical symptoms/brain CT and ONSD monitoring guidance.National Institutes of Health Stroke Scale(NIHSS),Acute Physiology and Chronic Health EvaluationⅡ(APACHEⅡ),Glasgow Coma Scale(GCS),complications,prognosis,ICU stay time and mechanical ventilation time were compared between the two groups.Results:NIHSS score[control group:(19.58±3.19)points vs(37.98±5.75)points,observation group:(10.33±2.42)points vs(38.05±5.83)points]and APACHE II score[control group:(14.55±2.17)points vs(19.87±3.50)points,observation group:(8.71±2.03)points vs(20.12±3.56)points]of the two groups at 1 month after injury were significantly lower than those at admission(P<0.05),GCS score[control group:(10.78±1.66)points vs(8.03±1.34)points,observation group:(13.10±1.72)points vs(7.99±1.32)points]were significantly higher than that at admission(P<0.05).At 1 month after injury,NIHSS score[(10.33±2.42)points vs(19.58±3.19)points],APACHE II score[(8.71±2.03)points vs(14.55±2.17)points]in the observation group were significantly lower than those in the control group(P<0.05),and GCS score[(13.10±1.72)points vs(10.78±1.66)points]in the observation group was significantly higher than that in the control group(P<0.05).The proportion of hydrocephalus(2.50%vs 12.50%),total complication rate(5.00%vs 21.25%),proportion of severe disability(5.00%vs 17.50%),proportion of survival in plant man(3.75%vs 15.00%),mortality rate(2.50%vs 12.50%),ICU stay time[(5.01±1.25)d vs(8.38±2.29)D],mechanical ventilation time[(2.18±0.75)D]in observation group were lower than those in the control group,and the good rate(56.25%vs 32.50%)and the total effective rate(93.75%vs 72.50%)in the control group were significantly higher than those in the control group(P<0.05).Conclusion:Dynamic ultrasound monitoring ONSD is effective in guiding dehydration treatment of patients with moderate and severe CI,it can significantly reduce ICP and complications,improve prognosis,which is worthy of promotion and application.

Bedside ultrasonography of optic nerve sheath diameter for detection of raised intracranial pressure in nontraumatic neurocritically ill patients

BACKGROUND Delay in treatment of raised intracranial pressure(ICP)leads to poor clinical outcomes.Optic nerve sheath diameter(ONSD)by ultrasonography(US-ONSD)has shown good accuracy in traumatic brain injury and neurosurgical patients to diagnose raised ICP.However,there is a dearth of data in neuro-medical intensive care unit(ICU)where the spectrum of disease is different.AIM To validate the diagnostic accuracy of ONSD in non-traumatic neuro-critically ill patients.METHODS We prospectively enrolled 114 patients who had clinically suspected raised ICP due to non-traumatic causes admitted in neuro-medical ICU.US-ONSD was performed according to ALARA principles.A cut-off more than 5.7 mm was taken as significantly raised.Raised ONSD was corelated with raised ICP on radiological imaging.Clinical history,general and systemic examination findings,SOFA and APACHE 2 score and patient outcomes were recorded.RESULTS There was significant association between raised ONSD and raised ICP on imaging(P<0.001).The sensitivity,specificity,positive and negative predictive value at this cut-off was 77.55%,89.06%,84.44% and 83.82% respectively.The positive and negative likelihood ratio was 7.09 and 0.25.The area under the receiver operating characteristic curves was 0.844.Using Youden’s index the best cut off value for ONSD was 5.75 mm.Raised ONSD was associated with lower age(P=0.007),poorer Glasgow Coma Scale(P=0.009)and greater need for surgical intervention(P=0.006)whereas no statistically significant association was found between raised ONSD and SOFA score,APACHE II score or ICU mortality.Our limitations were that it was a single centre study and we did not perform serial measurements or ONSD pre-and post-treatment or procedures for raised ICP.CONCLUSION ONSD can be used as a screening a test to detect raised ICP in a medical ICU and as a trigger to initiate further management of raised ICP.ONSD can be beneficial in ruling out a diagnosis in a low-prevalence population and rule in a diagnosis in a high-prevalence population.

Expressions of matrix metalloproteinase-9 and tissue inhibitor of metalloproteinase-1 in malignant peripheral nerve sheath tumor

BACKGROUND： Matrix metalloproteinase-9 （MMP-9） can degrade collagen IV （the main structural ingredient of basilar membrane）, and it also plays an important role in tumor vascularization, tumor cell progression, formation of metastatic focus, etc. Tissue inhibitor of metalloproteinase-1 （T1MP-1） can bind with MMP-9 to form 1 ： 1 compound and inhibit its activity, and can negatively regulate the tumor progression and metastasis. OBJECTIVE： To analyze the relationship of MMP-9 and T1MP-1 expressions with the pathological grade, metastasis and prognosis of malignant peripheral nerve sheath tumor （MPNST）. DESIGN： An observational comparative experiment. SETTING： Heze Medical College. PARTICIPANTS： Fifty-eight surgical pathological samples, which were clearly diagnosed to be MPNST, were collected from the pathological laboratory archives in the Department of Pathology, Heze Municipal Hospital from January 1988 to December 2003. The MPNST pathological types were common tumor in 53 cases, malignant triton tumor in 2 cases, epithelial MPNST in 2 cases and MPNST with gland differentiation in 1 case. The pathological grade was grade 1 in 11 cases, grade 2 in 24 cases and grade 3 in 23 cases. Besides, the resected tumor samples of 20 patients with benign peripheral nerve tumor （10 cases of nerve sheath tumor and 10 cases of neurofibromatosis） and the normal peripheral nerves （by-products of some surgeries） of 5 patients were also collected. The samples were used with the approval of the patients. Rat-anti-human MMP-9, TIMP-1 monoclonal antibody and S-P kit were purchased from Fuzhou Maixin Biotechnology, Co.,Ltd. METHODS： The documented paraffin blocks were again prepared to sections of 5 lJ m. The expressions of MMP-9 and TIMP-1 in the samples were detected with mmunohistochemical S-P method. The relationships of the MPNST severity, recurrence, metastasis and survival rate with the expressions of MMP-9 and TIMP-1 were analyzed. MAIN OUTCOME MEASURES： Relationships of MMP-9 and TIMP-1 expressions with the MPNST severity and prognosis. RESULTS： ①Expressions of MMP-9 and TIMP-1 in three tissues： MMP-9 and TIMP-1 stainings were mainly observed in cytoplasm. Among the 58 MPNST patients, the MMP-9 expression was significantly higher than those in normal peripheral nerve and benign tumor （P 〈 0.05）, while the TIMP-1 expression in MPNST was lower than those in normal peripheral nerve and benign tumor （P 〈 0.05）. ②Relationship of MMP-9 and TIMP-1 expressions with the severity and prognosis of MPNST： The expressions of both proteins were observed in the four subtypes. The positive expression of MMP-9 in the MPNST patients of grades 2 - 3 was significantly higher than that in the MPNST patients of grade 1 （P 〈 0.05）, while the expression of MMP-9 was significantly lower than that in the MPNST patients of grade 1 （P 〈 0.05）. The metastatic rate was positively correlated with MMP-9 expression （r =1.696, P 〈 0.05）, but negatively correlated with TIMP-1 expression （r = - 2.125, P 〈 0.05）. CONCLUSION： MMP-9 and TIMP-1 are associated with MPNST pathological grades and metastasis, and can be used as the indicators for judging the severity and orognosis of MPNST.

Malignant melanotic nerve sheath tumors in the spinal canal of psammomatous and non-psammomatous type: Two case reports

BACKGROUND A malignant melanotic nerve sheath tumor(MMNST),previously known as a melanotic schwannoma,is a rare variant of a peripheral nerve sheath tumor composed of Schwann cells with melanotic differentiation.Only a few reports of spinal MMNST have been reported.CASE SUMMARY In the first case,a 58-year-old woman presented with a history of low back pain and paresthesia.Magnetic resonance imaging(MRI)and computed tomography(CT)of the lumbar spine revealed an intradural extramedullary mass lesion with amorphous linear calcification.Complete tumor resection was performed and histological examination revealed a psammomatous melanotic schwannoma.In the second case,a 72-year-old man presented with low back pain and paresthesia.MRI of the thoracolumbar spine revealed an intramedullary mass lesion at the T11 vertebral body level.The mass lesion was hypointense on T2WI and hyperintense on T1WI.Tumor resection was performed and the histologic result was melanotic schwannoma.CONCLUSION MMNST should be considered in the differential diagnosis when calcification or melanin is seen in an intradural spinal tumor.

Imaging characteristics of orbital peripheral nerve sheath tumors:Analysis of 34 cases

BACKGROUND Peripheral nerve sheath tumors(PNSTs),a rare group of neoplasms in the orbit,comprise only 4%of all orbital tumors.At present,there are very few studies detailing the features of these tumors identified using imaging technology.AIM To compare the differences in location,morphology,magnetic resonance imaging(MRI)signal intensity/computed tomography(CT)value,and enhancement degree of tumors of different pathological PNSTs types.METHODS Clinical,pathological,CT,and MRI data were analyzed retrospectively in 34 patients with periorbital sheath tumors diagnosed using histopathology from January 2013 to August 2021.RESULTS Among 34 cases of orbital peripheral nerve sheath tumors,21 were schwannomas,12 were neurofibromas,and 1 was a plexiform neurofibroma.Common clinical symptoms presented by patients with these types of tumors include eyelid swelling,exophthalmos,and limited eye movement.Schwannomas mostly occur in the intramuscular space with small tumor volume and rare bone involvement.Neurofibromas develop in the extrapyramidal space with larger tumor volume and more bone involvement.Radiologically,schwannomas and neurofibromas are characterized by regular morphology and uneven density and signal.One case of plexiform neurofibroma showed tortuous and diffuse growth along the nerve,with a worm-like appearance on imaging.CONCLUSION Different pathological types of orbital peripheral nerve sheath tumors have unique imaging characteristics.Comprehensive consideration of the patient's clinical and imaging manifestations is of great value in the diagnosis of orbital peripheral nerve sheath tumors.

Malignant peripheral nerve sheath tumor in an elderly patient with superficial spreading melanoma:A case report

BACKGROUND Malignant peripheral nerve sheath tumor(MPNST)is a type of spindle cell sarcoma originating from the peripheral nerve,which usually results in the corresponding nerve sign on magnetic resonance imaging(MRI).Patients with MPNST may also have neurofibromatosis type 1.CASE SUMMARY A 78-year-old male was admitted to the hospital due to a tumor in his left knee.He had a previous history of superficial spreading melanoma on the left thigh.Color Doppler ultrasonography showed a hypoechoic mass in the subcutaneous soft tissues of the medial left knee with an abundant rich blood flow.Computed tomography scanning did not show obvious signs of bone destruction,but the skin adjacent to the tumor was slightly thickened.MRI examination revealed that the hypervascular lesion was well-circumscribed,lobulated,invaded the surrounding soft tissues and demonstrated heterogeneous enhancement but lacked an entering and exiting nerve sign.The MRI result indicated the invasiveness of the tumor.The patient underwent a left knee joint mass expanded resection and the first histopathological examination showed a MPNST with positive surgical margins.Therefore,the second extended resection was performed,and the patient had a good outcome in the short term.CONCLUSION MRI is a useful technique for revealing the biological characteristics of MPNST and provides clinical support for evaluation of the surgical area before operation.

Reconstruction of the chest wall after resection of malignant peripheral nerve sheath tumor:A case report

BACKGROUND Malignant peripheral nerve sheath tumors(MPNSTs)are a group of rare and aggressive sarcomas that often arise from major peripheral nerves and represent a notable challenge to efficacious treatment.MPNSTs can occur in any body surface and visceral organs with nerve fiber distribution.The treatment options for MPNSTs include surgery,chemotherapy,and adjuvant radiotherapy.CASE SUMMARY A 26-year-old female cellist presented with chest pain on her left side when she squatted to lift the cello.One week later,a chest X-ray was performed and revealed fracture of the fourth rib on the left side.Three months later,the patient inadvertently touched a mass on the left side of the chest wall.Chest computed tomography(CT)three-dimensional reconstruction of the ribs revealed bone destruction of the fourth rib on the left side with a soft tissue mass shadow measuring 5.7 cm×3.7 cm.CT-guided puncture biopsy of the tumor showed that heterotypic cells(spindle cells)tended to be nonepithelial tumor lesions.PET-CT demonstrated bone destruction and a soft tissue mass with avid 18F-fluorodeoxyglucose activity(SUVmax7.5)in the left fourth rib.The tumor of the left chest wall was resected under general anesthesia,and reconstruction of the chest wall was performed.The postoperative pathological report exhibited an MPNST.CONCLUSION MPNSTs are relatively chemo-insensitive tumors.The mainstay of treatment for MPNSTs remains resection with tumor-free margins.

Comparative analysis of CT and pathological findings of peripheral nerve sheath tumors

Objective: To improve the qualitative diagnosis of peripheral nerve sheath tumors by computed tomography (CT). Methods: CT findings of 64 cases of pathologically confirmed nerve sheath tumors were compared with the pathological findings of the tumors. Results: Low density of the tumors shown in plain CT images was related to dominating reticular structure in the tumor as found pathologically. Tumors with intact capsule found by pathological findings were shown with smooth margin in CT images. Inhomogeneous density and enhancement of the tumors in CT images was related to tumor necrosis, liquefaction and cystic degeneration, and inhomogeneous enhancement also involved the reticular structure. Conclusion: Nerve sheath tumors are characterized by distribution along the nerves, lower density than that of muscles in plain CT images, and inhomogeneous enhancement in enhanced CT, which can help differentiate nerve sheath tumors from other soft tissue tumors. When nerve sheath tumors lack distinctive CT features, the diagnoses have to depend on their pathological findings.

Clinicopathological study of malignant peripheral nerve sheath tumors in the head and neck:Case reports and review of literature

BACKGROUND Malignant peripheral nerve sheath tumor(MPNST)is a rare and aggressive soft tissue sarcoma that poses a major diagnostic and therapeutic challenge.CASE SUMMARY We retrospectively reviewed patients with head and neck MPNSTs treated in our hospital from 2000 to 2021.The clinical features,pathological manifestations,treatments,and prognoses were summarized.We also reviewed the literature,focusing on MPNST in the mandible and maxilla.The study population consisted of five women and five men aged 22–75 years(mean age,49 years).Of the 10 patients,7 were initial cases and 3 were recurrent cases.All lesions were sporadic.The most common site was the mandible.The most frequently encountered symptoms were a progressive mass and local swelling.Complete or partial loss of trimethylation at lysine 27 of histone H3(H3K27me3)was evident on staining in four of nine cases(one case was excluded due to lack of tissue for evaluation of loss of H3K27me3).The 2-and 5-year disease-specific survival rates were 86%a nd 43%,respectively.The average survival time was 64 mo.CONCLUSION MPNST is a highly malignant tumor with a poor prognosis,prone to a high risk of recurrence and distant metastasis.Complete surgical resection is the main treatment.

Malignant peripheral nerve sheath tumor with hemophilic syndrome and bone marrow fibrosis:A rare case report

BACKGROUND Malignant schwannoma is a rare tumor in the peripheral nervous system,accounting for approximately 5%to 10%of systemic soft tissue sarcomas.Especially,malignant schwannoma occurring in the broad ligament of the uterus with hemophilic syndrome and bone marrow fibrosis is extremely rare in clinical practice.Here,we report the first case of an patient diagnosed with malignant peripheral nerve sheath tumor(MPNST)of the broad ligament of the uterus with hemophilic syndrome and bone marrow fibrosis,and share our reference clinical diagnosis and treatment experience.CASE SUMMARY A patient was diagnosed with MPNST of the uterus harboring hemophilic syndrome and bone marrow fibrosis.She received combination,and repeated imaging revealed further encountered rare complications(hemophilia syndrome and bone marrow fibrosis)after two cycles of chemotherapy.Thereafter,combined treatment with pazopanib,gemcitabine,and dacarbazine was initiated.Unfortunately,the patient succumbed to death at hospital after two weeks.CONCLUSION This report firstly provided reference clinical practice for a patient with MPNST of the uterus harboring hemophilic syndrome and bone marrow fibrosis.Our case raises a reminder about the tolerance and safety of combination therapy,especially in young women.

Sonographic Measurement of the Optic Nerve Sheath Diameter (ONSD) and Eyeball Transversal Diameter (ETD) in Benin Healthy African Adults

Background: Measuring optic nerve sheath diameter (ONSD), a relatively re-cent technique, allows an indirect and non-invasive diagnosis of intracranial hypertension. The ONSD ratio to eyeball transverse diameter (ETD) increases this reliability of the technique. The objective of this study was to determine the normal ONSD and its ratio with ETD in black African adults in Benin. Methods: A descriptive cross-sectional study was conducted between May 2019 and August 2019. Ultrasound ONSD and ONSD/ETD ratio were meas-ured in 210 healthy adults received in the medical imaging department of the Borgou and Alibori University Hospital Center in Benin. The ONSD corre-sponded to the average of 12 measurements (03 horizontal and 03 vertical for each eye) taken 3 mm behind the papilla. The transverse ETD corresponded to the average of 03 measurements. Results: The mean ONSD was 4.20 ± 0.28 mm (95% CI = [4.17;4.24] mm and 95th percentile = 4.70 mm). The mean ETD was 24.25 ± 1.10 mm. The ONSD/ETD ratio was 0.17 ± 0.01 (95% CI = [0.17;0.17] and 95th percentile = 0.19). There was no difference between ver-tical and horizontal ONSD and between right and left ONSD. The ONSD and the ONSD/ETD ratio were not correlated with any of the socio-demographic and anthropometric characteristics studied (sex, age, ethnicity, weight, height, body mass index, head circumference and waist circumference). The ONSD was associated with ETD (p < 0.001 and r = 0.55). Conclusion: An ONSD and an ONSD/ETD ratio respectively greater than 4.70 mm and 0.19 should sus-pect intracranial hypertension. The ONSD/ETD ratio may be a better indicator of HICT because of its small variation.

Malignant Peripheral Nerve Sheath Tumor of the Thigh Invading the Superficial Femoral Artery, with Necrotic Lung Metastases as Presenting Symptoms

A NF1 (neurofibromatosis 1) patient developed multiple necrotic lung metastases from a sciatic malignant peripheral nerve sheath tumor (MPNST) invading the superficial femoral artery. The first diagnosis was metastases of a non-small-cell adenocarcinoma because the right calf MPNST was not clinically noticeable ant that the chest/abdomen PET/CT did not include the region of the legs. When the MPNST was diagnosed, new histological analysis on the metastases changed the diagnosis to that of epithelioid undifferentiated sarcoma. The article deals with the sometimes-delayed diagnosis in those NF1 patients with large palpable masses and chronic pain pre-existing the malignant transformation, and discusses the difficulty of the biopsy of necrotic metastases.

Shunting,optic nerve sheath fenestration and dural venous stenting for medically refractory idiopathic intracranial hypertension:systematic review and meta-analysis

Background:Cerebrospinal fluid(CSF)-diversion procedures have traditionally been the standard of treatment for patients with medically refractive idiopathic intracranial hypertension(IIH).However,dural venous sinus stent(VSS)placement has been described as a safe and effective procedure for the management of medically refractive IIH.We performed a meta-analysis comparing outcomes and complications of CSF-diversion procedures,VSS and optic nerve sheath fenestration(ONSF)for the treatment of medically refractive IIH.Methods:Electronic searches were performed using six databases from 1988 to January 2017.Data was extracted and meta-analysed from the identified studies.Results:From 55 pooled studies,there were 538 CSF-diversion cases,224 dural venous stent placements,and 872 ONSF procedures.Similar improvements were found in terms of postoperative headaches(CSF vs.VSS vs.ONSF:84%vs.78%vs.62%,P=0.223),papilledema(CSF vs.VSS vs.ONSF:71%vs.86%vs.77%,P=0.192),whilst visual acuity changes favored venous stenting(CSF vs.VSS vs.ONSF:55%vs.69%vs.44%,P=0.037).There was a significantly lower rate of subsequent procedures with venous stent placement(CSF vs.VSS vs.ONSF:37%vs.13%vs.18%,P<0.001),but other complication rates were similar(CSF vs.VSS vs.ONSF:13%vs.8%vs.14%,P=0.28).Subgroup analysis of lumbar-peritoneal vs.ventriculoperitoneal shunts found no differences in symptom improvements,complications and subsequent procedure rates.Conclusions:Our findings suggest that dural venous sinus stenting may be a viable alternative to traditional surgical interventions in patients who are refractory to medical treatment.