Efficacy Analysis Of The Operation Comprehensive Treatment Of Kasabach-Merritt Phenomenon

objectiveKasabach-Merritt phenomenon(Kasabach-Merritt Phenomenon,KMP)is a general term for a class of lesions with severe coagulation dysfunction associated with Kaposiform hemangioendothelioma hemangioendothelioma and Tufted hemangioma.The disease is dangerous and the disease progresses rapidly.It can threaten life or even cause death within a short period of time,with a mortality rate of up to 52%.KMP lesions mostly occur in newborns or infants and young children,and may involve limbs,trunk,maxillofacial and deep organs.Because the area of the lesion is often large,it is difficult to completely remove the tumor in one operation,and the probability of multiple operations is increased,the risk of surgery is increased,and the recovery time is extended.If the lesion is located on the face,surgical removal of the lesion is still "disfiguring." "Possibly,in combination with the above conditions,it was difficult for surgical operations to be the first choice of treatment for children with KMP in the past,and it is often only used for children with superficial and limited lesions.In recent years,with the increasing experience of surgical treatment of children with KMP,a series of corresponding surgical operation techniques have been developed.Here,we evaluate the overall effectiveness and safety of in situ skin grafting,external carotid artery catheterization,and surgery for KMP treatmentMethodRetrospectively analyzed the clinical data of children diagnosed with Kasa bach-Merritt phenomenon from January 2016 to March 2019 to our departmen t.64 patients met the inclusion criteria:①Platelet(PLT)<100 × 109,accom panied by coagulation dysfunction;②Surgical resection in our department and postoperative pathological results confirmed as Kaposiform hemangioendotheli oma or Tufted hemangioma;③Those without other hematological diseases and malignant tumors;④The child’s age is less than 18 years old.Preoperatively improve the relevant examinations to evaluate the child’s physical condition,routinely give methylprednisolone sodium succinate and intravenous human im munoglobulin(ph4)to improve the coagulation function of the child,and give a single large-dose infusion before surgery to those who are hormone-insensit ive Platelets;Children with small tumor size and limited KMP underwent sim ple tumor enlargement resection;if the tumor is large,removal of the tumor during surgery may cause suture difficulties or postoperative limb dysfunction,orthotopic skin grafting;located In patients with maxillofacial region,choose external carotid artery catheterization with or without partial tumor resection;Record the platelet and blood coagulation function changes,vital signs fluctu ation curve,postoperative skin graft survival status,and the presence or absen ce of limbs dysfunction,tumor recurrence,and surgical complications.ResultA total of 64 children were included in this study,including 36 males and 28 females,with an average age of 81 days.Including 46 cases of Kaposiform hemangioendothelioma,18 cases of Tufted hemangioma,including 33 cases of limbs,20 cases of trunk,11 cases of head,face and neck.One case died due to heavy bleeding and coagulation dysfunction during the operation,and died after rescue of invalid circulatory system failure during the operation,and 63 cases were successfully operated.Eight patients were located in the maxillofacial region and underwent external carotid artery catheterization.After treatment,they were treated with 40%urea solution daily(3-5ml)for 2 to 4 weeks.7 cases were effective,1 case was partially effective,a total of one patient experienced a continuous decrease in platelet values during treatment,which was alleviated by combined glucocorticoid injection.In other cases,the color of the superficial skin of the tumor was significantly changed,and the tumor body was significantly reduced.The patients who underwent external carotid artery drug infusion had an average platelet recovery of about 7 days after surgery;18 patients underwent orthotopic skin grafting.The survival area of 15 cases was>90%,and the survival area of2 cases was between 70%and 90%.The condition improved after regular dressing change,infrared irradiation and symptomatic treatment.The survival area of 1 case was<70%.The second operation was cured by skin graft repair.The remaining 37 patients underwent simple tumor resection,and 37 patients had successful operation.The platelet and coagulation function returned to normal values within 24 hours after surgery,generally not exceeding 48 hours.In this study,the surgical treatment of KMP,the overall treatment efficiency was about 98%,the average operation time was about 81 minutes,and the average recovery period after platelet and coagulation function was about 2.8 days.conclusion1.Surgical removal of the Kasabach-Merritt phenomenon lesions in children,the effect is positive,the recovery is rapid,and the side effects are small.2.The external carotid artery catheterization drug treatment is used to treat the Kasabach-Merritt phenomenon tumors in children’s face.It is safe and effective,with little side effects,short postoperative recovery time,little impact on appearance and beauty,and is easily accepted by the children’s family.3.In situ skin grafting is applied to the huge tumor of children with Kasabach-Merritt phenomenon.If the suture is difficult after complete resection or long-term limb dysfunction is possible,it can effectively solve this problem.