Giant Limb Imaging Pathology And Surgical Treatment Methods Of Proteus Syndrome

ObjectiveTo investigate the clinical Proteus syndrome patients with20cases, were threecontents pathological, imaging and surgical operation treatment of Proteus syndrome thatclinical complex cases, characteristics and the pathological and radiological contrastgrowth mutually embedded and white fat and muscle in pathological conditions furtherimprove the standard of diagnosis, to provide theoretical basis for reasonable selection ofoperation methods, for solving the deformation syndrome this medical problem step.MethodsStudy on diagnosed clinically Proteus syndrome20cases as the object of study, male11cases, female9cases, age9-45years old, in the20patients with postoperativeexamination before the medical photography, imaging (CT plain scan, limb CT3D imaging,vascular lesions of CT three-dimensional imaging), check again pathological anatomy(operation anatomy photography, isolated limb anatomy observation, tissue pathologicalobservation), pathological tissue paraffin sections for HE staining, silver staining, oil red Ostaining. Routine preoperative laboratory tests, electrocardiogram, research on hugemisshapen limbs operation shaping method.Results1Imaging: lipoma disorders (some with fat invasive growth is given priority to, causemuscle, fat and blood vessel distribution, unbalanced distribution in pathological changesof the limb; Some growth with subcutaneous fat homogeneity, pathological changes ofvascular enlargement, growth, twisted, associated with varicose veins; Onto hyperplasia,occur in a variety of bone; Fat distribution is a kind of pathological changes of thedistribution of fat and muscle is chimeric type growth; Is a kind of muscle fiber hadobvious sign a zebra crossing, fat is a kind of muscle mass in fat distribution.2Pathology： Gross anatomy shows that: the muscle and fat in the form of three types ofdistribution:(1) lesions in homogeneous hyperplasia of fat;(2) normal fat ball in muscletissue growth is outstanding;(3) muscle fat in the form of Mosaic exist: one is pure fatpiece of form, is a kind of fat and muscle sandwich sample distribution is a distribution offat in the muscles again.In pathological morphology showed that HE staining and silver staining slices ofmuscle and fat distribution, a chimeric type growth, a grow fat between the muscle fiberand fat cells, volume increase; Epidermal nevus epithelial change obviously, epithelial cellgrowth is active; Oil red O staining on the volume of fat cells, large quantity, irregularform obvious gap increases, the fat cells, cell active.3Operation treatment results: Surgery to try to keep body adipose tissue localexcision, hybrid lipoma selectively remove, subcutaneous adipose tissue blocks to removeskin and implant, giant foot bone cutting orthopaedic achieved a result:(1) mast bodysignificantly narrowed, improve the patient’s ability to walk;(2) in patients with limbdeformity correction, can wear normal shoes. For lesions can’t keep serious life-threateningbody amputation stump tissue volume reduction, stub restore good prosthesis for functionalexercise after installation.Conclusion1This study found that the distortion of the syndrome of imaging and pathologicalchimerism change is one of the key basis of deformation syndrome diagnosis, surgicaltreatment of the important basis and design.2Through imaging examination were performed three dimension imaging, is theimportant basis of design operation scheme.3We puts forward the improved deformation syndrome diagnostic criteria through thestudy:(1) limbs, head and neck, or the trunk has asymmetric disproportionate overgrowthof congenital malformations;(2) the affected limb has a hand (or foot) macrodactyly (toe)deformity;(3) imaging and histopathological examination organized chimeric lesions, theskin or muscle lipoma, radioactive sandwiches), radioactive zebra crossing betweensyndrome diagnosis of deformation. Above three have two and exclusion hyperostosis-varices syndrome, k-t syndrome, neurofibromatosis, syndrome can be diagnosed withdeformation.4Plastic surgery is the only effective treatment, personalized staging surgery can geteffective treatment effect, can make the limb deformity correction, can wear normal shoesand walk normally, improve patients with limb function, make the skin beautiful.