Objective To analyze the surgical treatment of hemangioma of limbs complicated by Kasabach-Mcrritt phenomenon in children.Methods A retrospective study was carried out on 31 children with hemangioma of limbs complicated by Kasabach-Merritt phenomenon.Lower limbs were affected in 19 patients,and upper limbs in 12 patients.Twenty-six patients showed decreased platelet count ((2.6-60) × 109/L) and fibrinogen level (＜ 1 g/L),and 21 prolonged partial prothrombin time (＞ 50 seconds).All the patients who had responded poorly to medications including glucocorticoids received surgical treatment.Results Of these patients,30 were cured,and one died of massive blood loss.After surgical treatment,the platelet count returned to normal in 1-3 days in 25 patients,3-7 days in 4 patients,and more than 10 days in one patient.Conclusions For pediatric hemangiomas of limbs complicated by Kasabach-Merritt phenomenon,surgical treatment should be given as early as possible so as to reduce mortality in patients.

Objective: This study aims (1) to evaluate the surgical outcome of intramuscular venous malformations, and associated nerve and tendon encapsulated; (2) to summarize the surgical procedure, with the purpose of providing individualized surgical treatment. Methods: This is a retrospective study of 114 patients of intramuscular venous malformations with associated nerve and tendon encapsulated. All the patients were admitted in the Henan Provincial People′s Hospital from January 2012 to December 2014. The surgeries were produced, according to the patient′s condition. The surgical outcome was evaluated after 1-3 years follow up, by the overall evaluation of radiological imaging, symptoms and functions of limbs. The evaluation criteria: (1) Invalid: clinical symptoms or radiological imaging showed no improvement or even worsen; (2) Improvement: clinical symptoms or radiological imaging was significantly improved; (3) Cure: clinical symptoms were gone or the radiological imaging was normal. Results: All the 114 patients were followed up for 1-3 years, with the average of 1.8 years. Three cases were evaluated as invalid, 26 cases were improved, and 85 cases were cured. The effective rate was 97%, and the cure rate was 75% on 1 year after surgery. Conclusions It is difficult to treat intramuscular venous malformations as it often involves nerve and tendon. Universal treatment is few. Therefore, an individualized surgical plan is recommendable.

Objective: To analyze the causes of equinus deformity caused by intramuscular venous malformation onset posterior muscles of leg, and discuss the corresponding treatment methods. Methods: A retrospective study was conducted on 69 cases of intramuscular venous malformations with equinus deformity from January 2012 to December 2017. Based on patient's main complain, physical examination and imaging data, the causes were divided into two categories: pain disorder and contracture disorder. Classification was on the basis of definite diagnosis of MRI. When the main complaint of medical history and physical examination indicated pain relief or passivity of the affected limb, and when the back extension of ankle joint was greater than 75 degrees, it was a pain disorder; when the medical history and physical examination indicated pain relief or passivity of the affected limb, the back extension of ankle joint was less than 15 degrees, it was a contracture disorder. Therapeutic methods included drug conservative treatment and surgical treatment. For the patients with pain disorder, the first choice was drug conservative treatment, and for the patients with contracture disorder, the first choice was surgery. Operative methods include simple venous malformation resection, venous malformation resection and Z-type Achilles tendon anastomosis lengthening. After operation patients received systematic functional rehabilitation exercise and calculated the satisfaction rate. Results: 13 cases of painful disorders were firstly treated by conservative medicine, but 4 cases were treated by operation after series of conservative treatments, satisfaction rate was 69.2%(9/13). 56 contracture cases were treated by operation, including 11 cases of simple venous malformation resection, 45 cases resection and Z-type anastomosis lengthening of Achilles tendon. All the patients were followed up for 6 months to 2 years after operation. 53 patients recovered to normal walking after operation, and 3 patients had mild limp, satisfaction rate was 94.6%(53/56). Patient satisfaction was 100%. Conclusions The equinus deformity caused by intramuscular venous malformation onset posterior muscles of leg affect the quality of life. Muscle/tendon contracture was the main cause. Correct surgical treatment combined with early rehabilitation exercise post operation can restore normal walking posture.

Objective: To define a new subtype of vascular anomaly, named fibro-adipose vascular anomaly(FAVA)and to discuss the methods of diagnosis and treatment in order to decrease the misdiagnosis rate and improve the recovery rate. Methods: From Jan. 2011 to Dec. 2015, 34 cases with FAVA on the lower extremities received surgical treatment in our center. The clinical data were collected to summarize the imaging and pathological characteristics for diagnosis. Results: The misdiagnosis rate was 76.5%(26/34) in all these 34 patients. The cure rate was 100% after operation. The patients were followed-up for 1-3 years(average, 19 months) with normal function and no recurrence. Conclusions FAVA is usually misdiagnosed as hemangioma or vascular malformation. The disease should be well defined to help the diagnosis and treatment. Surgical excision is one of the optional treatments.

Objective: To assess the safety and effectiveness of sufficient, short-term platelet (PLT) transfusion for the surgery preparation of the infantile patients with Kasabach-Merritt phenomenon, who were insensitive to glucocorticoids. Methods: The infantile cases were retrospectively analyzed during May 2011 to December 2016, who were clinically diagnosed as KMP and insensitive to glucocorticoids, received PLT transfusion and surgical resection. PLT transfusion in patients whose PLTC was less than 30×10⁹/L, was 0.3 therapeutic dose(TD)/kg, and 0.2 TD/kg in PLTC≥30×10⁹/L group. The maximum was 1 TD.Criteria of the PLT transrusion: 1 hour after the transfusion, the PLT count (PLTC) were tested and the corrected count increment of platelet (CCI) and practical platelet recovery (PPR) was calculated. PLTC ≥100×10⁹/L, CCI>7.5×10⁹/L and PPR>30% were defined as effective; while PLTC=(50-99)×10⁹/L, CCI>7.5×10⁹/L and PPR>30% as partial effective; PLTC<50×10⁹/L, or CCI≤7.5×10⁹/L, or PPR ≤30% were defined as ineffective. By reviewing the method and response of their PLT infusions, to figure out the most effective way in rising PLT, as a part of pre-operation treatment. Results: There were 46 cases in the research. Based on the PLTC, CCI and PPR 1 hour after PLT transfusion, there were 44 effective transfusion, 2 patients with partial effectiveness, and no ineffective case. There was no allergic or heart failure happened in any cases. No critical potential complications of PLT transfusion occurred, including fluid and iron overload, alloimmunization to human leukocyte antigen and/or PLT antigen. Conclusions Pre-operative sufficient and short-term PLT infusions are more effective than low dose and long-term ones. They can create a more optimistic opportunity for surgical resections.

Objective: To analyze the pain caused by intramuscular venous malformation, so as to avoid misdiagnosis. Methods: We retrospectively analyzed 173 patients who received surgical treatments in our department between Jan.2012 to Dec.2014, with the main complaint of local pain and were diagnosed as intramuscular venous malformation. The mechanisms of the local pain, based on the image data, intra-operative findings, pathology reports and the comparison of the Visual Analogue Scale(VAS) data before and after operation were summarized. The surgical interventions included simple excision or excision + adhesiolysis or excision + adhesiolysis + nerve decompression. Results: The reasons of local pain can be divided into 4 categories: ①lesion located in the tendon insertions; ②lesion involving the local nerve, inducing the thickening and tensing of its epineurium and the increasing of its diameter; ③lesion infiltrated to the periosteum; ④phlebolith in it. All the 173 patients received surgical treatments and got varying degrees of relieving from local pain. 63 patients got a decrease of the VAS by 5 or more, and 95 cases′ VAS number decreased by 3-4, the rest 15 patients′ VAS cut down by 1-2. Conclusions Intramuscular venous malformation is an important reason for local pain and should not be neglected. Surgical treatment can be an effective method to remove the lesion and relieve local pain.