| Hemophilia is a hereditary hemorrhagic disease,due to the lack of clotting factors or obstruction in productionof active clotting enzyme,and which is characterizedbyeasy bleeding and long clotting time.According to the deficiency ofclotting factors,haemophilia is divided into threetypes:Type A,F?Dis lack;type B,FIX is lack;type C,F XI is lack.According to the coagulation factors activity,hemophilia A is divided into four types:subclinical type,light type,intermediate typeand heavy type.The classification of the hemophilia A can be used for reference to type B.Hemophilic pseudotumor(HP)is one of its complication presented assubacute or chronic hematomain muscles,bones or other positions.HP is a rare complication a incidence of 1-2%in hemophilia,and was firstly described by Starker in 1918.It occurscommonly in limbs and pelvis,and also occasionally in other locations,such as in the kidney,gastrointestinal tract,abdominal cavity,lungs,chest cavity and brain.According to location of HP,Brant EE and Sim KB divided HP into three types:typeI,lesions in soft tissue;type ?,in the subperiosteum;type ?,lesions from the bone.Although HP is a benign lesion,bleeding is not easyto control.Misdiagnosisor improper treatment would make trouble for patients and clinical practice.It often resulted in musculoskeletal damage and dysfunction of the lower limbs,even disability and amputation.In clinical practice,the disease is rare.The radiological and clinical misdiagnosis often occursbecause of lack of recognition.Therefore imaging diagnosis of HP and clinical treatment has been medical difficulty and hot spot.In 1991,Guanhui Xiao et al analyzed normal X-ray finding of HP mainly located in limbs(10 cases)with an emphasis on the distensible bone destruction and periosteal hyperplasia;In 2012,Shudong Hu et aldiscussed spiral CT signs of HP derived from bone and soft tissue(16 cases)and summarized the CT features of distensible bone destruction,calcification and formation of soft tissue mass;In 2016,Xiaohui Huanget alinvestigated the imaging features of haemophilia A-HP in different image examinations.They analyzed preliminarily its MRI manifestations of musculoskeletal injury(5 cases).In 1972,Brandt and Jordan studied the basic X-ray signs of bone HP(6 cases);In 1980,Guilford reported CT findings of bone destruction in pelvic HP(2 cases);In 1997 and 2004,Jaovisidha and Park JS respectively discussed the MRI manifestations of several HP casesandsuggested that MRI was sensitive to HPlesions.In general,the MRI studies of HP found in the domestic and foreign literatures were relatively few.This could be contributed to that HP is rare and samples are limited.It is necessary to further explore the MRI manifestation of HP;there were few reports on imaging studies in joint destruction and celiac HP.This studywill preliminarily explore those aspects.Soft tissue HP will not only cause muscle damage,but also affect the adjacent bone,leading to periosteal edema or hyperplasia,compressive bone destruction or marrow cavity stenosis and bone marrow edema.The analysis of MRI appearances of soft tissue HP,such as size,growth pattern and location and discussion of their correlation with early bone changes may avoid bone destruction and severe decline of bone quality.Further understanding about HP may also be obtained.GE Signal 1.5T MRI and SEMENS MAGNETOM Skyra 3.0T intelligent MR imaging has high sensibility for HP.MRI sequences,especially T2 weighted imaging fat suppression(T2WI FS)sequence and proton density weighted imaging(PDWI)FS sequence have superiority insuppressing fat signal from soft tissue and bone marrow cavity,avoiding artifact caused by fat,clearly displaying of musculoskeletal structure,HP features and neighborhood tissue changes.GE discovery CT750HD FREEDOM CT scanner and GE discovery XR650 digital radiography(DR)machine can be used incombination for musculoskeletal examination and have advantages in effectivelyreducing the radiation dose and aiding patient's comfort.Research purposes1.To investigate imaging findings of the HP in musculoskeletal locations,pelvis and abdomen on X-ray,CT and 3TMRI forimprovingmore comprehensive understandingof their imaging findings andreducing misdiagnosis and avoiding delayed or error treatment.2.To analyze the impacts of soft tissue HP parameters on periosteum and marrow using 3TMRI T2WI FS and PDWI FS sequence to help physicians and clinicians to obtain a new understanding of HP and provide a reference to clinicians for pretreatment.The parameters include largest longitudinal diameter(LLD),largest transverse diameter(LLD),distance between pseudotumor and bone(D)and scope of wrapped bone(SOB W).Materials and methodsStudy population The study subjects were patients with HP who stayed in our hospital between February 2001 and December 2016.There were 42 cases diagnosed by clinical examinations,radiography or surgical results.All hemophilia cases were type A or type B.HP occurred in soft tissues and(or)bone.Exclusion criteria are as follows:history of surgery;the diseased areais combined with other diseases history of other basic diseases related to muscle bone.General clinical materalsAll patients were male,ages were from 10 to 58 years old,the mean age was 32.4 ±12.0 years.There were 34 cases with hemophilia A(34/42,80.9%)and 8 cases with hemophilia B(8/42,19.1%).Light degree was found in 2 patients(2/42,4.8%),medium degree in 31 cases(31/42,73.8%)and heavy degree in 9 cases(9/42,21.4%).Medical history was from 3 months to 36 years and themean time was 23±9.4 years.Twenty-three cases were discovered and diagnosed in childhood.Research methods1Of the 42 cases,31 cases performed with X-ray,CT and 3.0 TMRI examinations,while 11 patients,due to physical cause or radiation,performed only two types of the three examinations.According to the locations,HPs were divided into four groups:soft tissue group,bone group,joint involvedgroup and abdominal-pelvis group.The imaging features of HPwasdiscussed in each group,respectively.2 Investigationof the impact of soft tissue HP on bone.Thirty-two patients with soft tissue HP in their limbs were selected as subjects.There were totally 39 lesions located in 39 limbs.25patients occurred in unilateral limb,and 7 cases in bilateral limbs.On T2WI FS or PD FS sequence,the longest transverse diameter(LTD)of HP,scope of bone wrapped(SOB W)by HP,distance(D)between HP and bone and longest longitudinal diameter(LLD)were measured.Periosteal edema or hyperplasia,cortical thinning or disappear,bone marrow edema,compression or damage of marrow cavitywere observed.Patients with periosteal changes were chosen as the periosteum-change group,cases with no periosteal changes were the no periosteal-changes group;cases with bone marrow edemaas the bone-marrow edema group,cases with no marrow change asthe no marrow-change group.The imaging findings of all HPs and the four parameters of soft tissue HPin each groupwere measured by two senior radiologists,respectively.The measurements of LTD and LLD included diameter of pseudotumor itself and width of pseudocapsule.Multiple lesions in the same area were regarded as a whole.The dataobtained by two doctors were averaged.Statistical methodsSPSS 22 statistical software was used.Data were shown as mean ± standard deviation(X±SD).Statistical analysis of LTD,LLD,D and SOBW between observation group 1 and contrast group 1,between observation group 2 and contrast group 2 were performed.Kolmogorov Smirnov test was used to test the normal distribution of the data.Independent sample t-test was used when data were distributed normally.If not.Mann-Whitney U test was used.P<0.05 was assumed statistically significant.Study results1 Imaging(1)bone HP(III type),17 patients,a total of 27 lesions,pure osteolytic destruction was 8 lesions,cyst-like bone destruction was 6,expansile bone destruction was 131esions.Thinning of the bone cortex or discontinuous changes were 14 lesions,involved adjacent joints was 51esions,mottled and small calcification were seen in 16 lesions,soft tissue mass were formed in 11 lesions,uneven density was in 19 lesions.T1WI,T2WI FS and PDWI FS images,mixed high and low signal was shown in 18 lesions,bone sclerosis of rims or formation of psuedocapsule was revealed in 23 lesions.(2)The intra-articular HP was 3 cases,destruction of joint surface and extremities were shown,joint deformity was seen.(3)Subperiosteal HP(? type),5 patients were found,and 8 lesions were seen,5 lesions grew along the longitudinal axis,bone cortex(or medullary cavity)were oppressed in 8 lesions.Periosteal hyperplasia was seen in 6 lesions and triangular periosteum was shown in 2 lesions.(4)soft tissue HP(I type),32 patients were seen,single lesion was 18 patients,and multiple lesions were 14 patients.86 lesions were found.12 HP lesions were found in one patient.The lesions located in a muscle,inter-muscle and subcutaneous tissues.diameter of HP was from 1 cm to 34.39 cm.30 lesions grew along vertical axis,26 was round and oval,16 lesions were around bone,and 14 lesions were irregular shape;periosteal change was 30 lesions,pressed cortex was 151esions,marrow edema was 23lesions.Psuedocapsule was clearin 63 lesions,low signal lines of hemosiderin deposition was seen in 211esions.(5)HP of the pelvic and abdominal cavity,6 patients were found,13 lesions were seen,biggest diameter of HP was 17.3 cm.Surrounding tissue was pushed,and adhesion occurred in some lesions.Intestinal obstruction was shown in 1 case,and hydronephrosis and hydroureter were seen in 2 cases.2.Analysis of periostealand medullary changes caused by soft tissue HPOf the 39 lesions in 32cases,24 lesions were found with periosteal edema or hyperplasia(24/39,61.5%),the remaining 15 lesions were not found with it(15/39,38.5%).There are 15 lesins that caused bone marrow edema(15/39,38.5%),the other 24 lesions had not caused medullary edema.Five cases causedperiosteal changes and bone marrow edema at the same time.For the small sample,no comparison between those cases and the control group was carried out to analysis parameters of LTD,LLD D and SOBW.In group withperiosteal changes(n=24),the parameters of LTD,LLD,Dand SOBW were 8.61 ± 3.56 cm,14.34 ± 7.37 cm,0.21 ± 0.12 cm and 0.48 ± 0.22 round,respectively;while in no periosteal changes group without periosteal changes(n =15),the parameters were 6.82 ± 1.87 cm,11.32 ± 2.90cm;0.61 ± 0.48 cm and 0.29 ±0.16 round,respectively.In both groups,datas were not distributed normally,so the nonparametric U-test was used.Statistical analysis showed that the two groups were significantly different in LLD(U = 106.000,P = 0.033),D(U = 53.000,P = 0.000)and SOBW(U = 76.000,P = 0.002).But LTD was not statisticallysignificant(U =126.500,P = 0.122).In group with medullary edema(n = 15),the parameters of LTD,LLD,D and SOBW were 10.22 ± 3.83 cm,20.73 ± 6.92 cm,0.09 ± 0.43 cm and 0.55 ± 0.27round,respectively;while in groupwithout medullary edema(n = 24),they were 8.15 ± 3.05 cm,12.31 ± 3.96 cm,0.5 ± 0.16 cm and0.29 ± 0.12 round,respectively.In both groups,datas were not distributed normally.So nonparametric U test was used.Statistical analysis revealed that the two groups were significantly different in LLD(U = 40.000,P-0.000),D(U = 148.000,P = 0.343)and SOBW(U= 72.000,P =0.002).While LTD did not achieve statistical significance(U = 128.500,P = 0.137)Study conclusions1 Imaging findings of HPs were diverse on X-ray,CT and MRI examinations,but imaging characteristic of hematomawas seen in all lesions.MRI can accurately reveal signals of hematoma components,and is of great value in diagnosis and differential diagnosis.2 SOBW,LLD and D of soft tissue HP(type ?)are important factors that have impact on periosteal changes,and SOBW and LLD are important factors involved in marrow edema.Clinical significanceThe comprehensive analysis of imaging features of HP in musculoskeletal and special locations helpsradiologists and clinicians to get a clear and comprehensive understandingto HP.It is useful in diagnosis,differential diagnosis(such as giant cell tumor,osteosarcoma and metastatic tumor et al),reducing misdiagnosisand providing reference in formulating early and effective therapeutic strategies.Innovations1.The imaging features of 42 HP cases from different locations were systematically analyzed through combined use of X-ray,CT and MRI.Especially the use of 3.0TMRI imaging,to a certain extent,raised awareness about HP,and overcome the limitation and biascaused by limited cases.2.T2WI FS and PDWI FS sequences were used to investigate the four factors of soft tissue HP(SOBW,LLD,D and LTD).Statistical methods showed that SOBW,LLD and D were factors involved in bone changes.The quantitative analysis method usedto study the soft tissue factors affecting bone changes can eliminate the influence of subjective factors.Study shortage1 This study focused on imaging findings of HP and lacked lesions' s histopathological study.2 The studysample was small,thereforethere may be certain shortage in statisticsand may limit the broad applicability of the results. |
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