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:This study aims to discuss the clinical effect of surgical treatment of vascular anomalies in the sciatic nerve region.Methods:Retrospective analysis of clinical data was performed on patients with pain and dysfunction in the sciatic nerve region in the Department of Hemangioma, Henan Provincial People’s Hospital, between July 2013 and December 2018. The operation time, intraoperative blood loss, hospitalization time, postoperative complications, and wound healing were recorded. Postoperative follow-up included physical and imaging examination. Visual analogue scale (VAS) was used to evaluate the pain intensity before operation and at discharge. Oswestry dysfunction index (ODI) was used to evaluate the pain and limb function before operation and during follow-up.Results:This study enrolled 76 cases (32 males, aged from 2 to 55 years ), including 48 cases of venous malformation (VM), 9 cases of arteriovenous malformation (AVM), 6 cases of lymphatic malformation (LM), and 13 cases of fibro-adipose vascular anomaly (FAVA). Thirteen patients had been treated with local sclerotherapy, and four patients had been treated with interventional embolization for AVM without improvement or exacerbation. The operative time, intraoperative blood loss, and hospitalization time of FAVA patients were (113.33±30.11) min, (81.67±22.29) ml, and (3.83±0.98) d respectively, which were less than those of the other three vascular anomalies with the lest surgical damage and quickest recovery. The operative time, intraoperative blood loss, and hospitalization time of AVM patients were (288.33±33.71) min, (981.67±164.85) ml, and (11.17±1.47) d respectively, which were more than those of the other three vascular anomalies. The operation time of VM and LM cases was close, but the amount of blood loss [(396.67±85.71) ml] in VM cases varies greatly, with a minimum of 150 ml and a maximum of 1 100 ml. The vital signs of all patients were stable during and after the operation, without injury of main vessels and nerves. Seventy-one patients had grade A wound healing, four patients had grade B wound healing, and one patient had grade C wound healing. The postoperative follow-up was 6-36 months (mean 13 months), and the pain was completely relieved in 73 cases and partially relieved in 3 cases at the last follow-up. Seventy-two cases returned to normal, and 4 cases improved with lower limb dysfunction. Imaging examination showed that lesions of 70 cases disappeared and of 6 cases were residual. Preoperative VAS scores of four groups were 2.00 (1.00, 3.75), 2.00 (1.00, 3.50), 4.00 (0.75, 6.25), 4.00 (1.50, 6.00) respectively, and the postoperative VAS scores were all 0.00 (0.00, 0.00). The preoperative ODI scores of four groups were 17.78% (15.56%, 22.22%), 17.78% (13.33%, 35.56%), 50.00% (31.67%, 84.44%), 42.22% (31.11%, 56.67%). Apart from LM [4.44% (0.00%, 22.22%)], the postoperative ODI of the others was all 0.00% (0.00%, 0.00%). VAS and ODI were statistically significant differences before and after surgical treatment among all four groups ( P<0.05). Conclusions:Surgical treatment is safe and effective for vascular anomalies in the sciatic nerve region, which can significantly improve pain and joint mobility disorders.

Objective:This study aims to discuss the clinical effect of surgical treatment of vascular anomalies in the sciatic nerve region.Methods:Retrospective analysis of clinical data was performed on patients with pain and dysfunction in the sciatic nerve region in the Department of Hemangioma, Henan Provincial People’s Hospital, between July 2013 and December 2018. The operation time, intraoperative blood loss, hospitalization time, postoperative complications, and wound healing were recorded. Postoperative follow-up included physical and imaging examination. Visual analogue scale (VAS) was used to evaluate the pain intensity before operation and at discharge. Oswestry dysfunction index (ODI) was used to evaluate the pain and limb function before operation and during follow-up.Results:This study enrolled 76 cases (32 males, aged from 2 to 55 years ), including 48 cases of venous malformation (VM), 9 cases of arteriovenous malformation (AVM), 6 cases of lymphatic malformation (LM), and 13 cases of fibro-adipose vascular anomaly (FAVA). Thirteen patients had been treated with local sclerotherapy, and four patients had been treated with interventional embolization for AVM without improvement or exacerbation. The operative time, intraoperative blood loss, and hospitalization time of FAVA patients were (113.33±30.11) min, (81.67±22.29) ml, and (3.83±0.98) d respectively, which were less than those of the other three vascular anomalies with the lest surgical damage and quickest recovery. The operative time, intraoperative blood loss, and hospitalization time of AVM patients were (288.33±33.71) min, (981.67±164.85) ml, and (11.17±1.47) d respectively, which were more than those of the other three vascular anomalies. The operation time of VM and LM cases was close, but the amount of blood loss [(396.67±85.71) ml] in VM cases varies greatly, with a minimum of 150 ml and a maximum of 1 100 ml. The vital signs of all patients were stable during and after the operation, without injury of main vessels and nerves. Seventy-one patients had grade A wound healing, four patients had grade B wound healing, and one patient had grade C wound healing. The postoperative follow-up was 6-36 months (mean 13 months), and the pain was completely relieved in 73 cases and partially relieved in 3 cases at the last follow-up. Seventy-two cases returned to normal, and 4 cases improved with lower limb dysfunction. Imaging examination showed that lesions of 70 cases disappeared and of 6 cases were residual. Preoperative VAS scores of four groups were 2.00 (1.00, 3.75), 2.00 (1.00, 3.50), 4.00 (0.75, 6.25), 4.00 (1.50, 6.00) respectively, and the postoperative VAS scores were all 0.00 (0.00, 0.00). The preoperative ODI scores of four groups were 17.78% (15.56%, 22.22%), 17.78% (13.33%, 35.56%), 50.00% (31.67%, 84.44%), 42.22% (31.11%, 56.67%). Apart from LM [4.44% (0.00%, 22.22%)], the postoperative ODI of the others was all 0.00% (0.00%, 0.00%). VAS and ODI were statistically significant differences before and after surgical treatment among all four groups ( P<0.05). Conclusions:Surgical treatment is safe and effective for vascular anomalies in the sciatic nerve region, which can significantly improve pain and joint mobility disorders.

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.

Objective:To investigate the clinicopathological features, classification, and genetic characteristics of common lymphatic malformation (CLM) in superficial soft tissue.Methods:A retrospective study of 110 patients with the diagnosis of CLM at the Henan Province People′s Hospital, China from August 2019 to August 2022 was performed. The clinicopathological features, relevant immunohistochemical (IHC) staining results, and fluorescence quantitative PCR of PIK3CA mutation were analyzed, and patients were followed up.Results:Among the 110 CLM patients, there were 53 males and 57 females; 65 cases (65/110, 59.1%) were first detected when the patients were≤2 years old. The most common location was the head and neck in 41 cases (41/110, 37.3%). Clinically, 102 cases (102/110, 92.7%) were solitary, 83 cases (83/110, 75.5%) were skin-colored, 69 cases (69/110, 62.7%) had indistinct borders, and 10 cases (10/110, 9.1%) had diffuse and severe macroscopic manifestations. There were 52 macrocystic type (52/110, 47.3%), 23 microcystic type (23/110, 20.9%), and 35 combined type (35/110, 31.8%). The macrocystic CLM presented as soft, translucent masses with large cystic cavities on the cut surface, and histologically they were composed of large, irregularly dilated channels that were thicker with irregular smooth muscle and lymphocytic infiltration. Microcystic CLM showed wartlike projections or translucent blisters on the skin, with small honeycomb structures on the cut surface, and histologically consisted of round or angular dilated small lymphatic vessels with little or no smooth muscle. The combined CLM had both macrocystic and microcystic morphologies. IHC staining showed that the lymphatic endothelial cells were positive for LYVE-1, D2-40, PROX1, CD31, and VEGFR3 but negative for CD34; in the macrocystic and combined CLM vessel walls were positive for SMA. Eight of 13 CLM had PIK3CA mutation. All patients were followed up, and 24 (24/110, 21.8%) had relapses, which more frequently occurred in combined type, followed by microcystic type.Conclusions:CLM is a congenital vascular malformation composed of dilated, abnormal lymphatic channels, with PIK3CA mutation. There are significant differences in clinicopathological characteristics among the different types. Since microcystic and combined CLM are prone to recurrence, accurate pathological subtyping is necessary to guide treatment and to predict prognosis.

Objective:To strengthen the consciousness of postoperative rehabilitation exercise for focal intramuscular venous malformations and to explore the necessity of postoperative rehabilitation exercise for patients with focal intramuscular venous malformations.Methods:A retrospective analysis of patients with focal intramuscular venous malformations. From June 2017 to April 2018, 19 patients with focal intramuscular venous malformation were treated in the Hemangioma & Vascular Malformation Treatment Center of Henan Provincial People’s Hospital. All patients complained of pain and discomfort. According to the location of the lesion and the anatomical relationship between the lesion and the surrounding tissues, the operation method was selected. The rehabilitation physicians and surgeons worked out the rehabilitation plan together to carry out rehabilitation exercise treatment. The VAS scores before and after operation were compared to evaluate the effect of symptom relief and postoperative functional recovery.Results:A total of 19 patients were enrolled, including 11 females and 8 males, with an average age of 17.7 years (range, 3-44 years). The patients were followed up for 6 months to 1 year. The comparison of postoperative MRI and preoperative MRI showed that the lesions were completely removed. The VAS score of all patients was lower than that at admission. The preoperative VAS was 4.84±2.32 and the postoperative VAS was 2.16±1.17, P<0.01, indicating that the preoperative and postoperative VAS score had changed, and the VAS score had decreased. After rehabilitation exercise, all patients’ body functions were improved, joint stability, muscle strength and walking ability were restored to normal life. The preoperative joint function score was 10.47±3.29, and the postoperative joint function score was 14.46±3.50, P<0.01, indicating that the preoperative and postoperative joint function score had changed, and all the joint function scores had improved. Patients’ satisfaction was achieved. Conclusions:Postoperative rehabilitation guidance can effectively prevent postoperative limb dysfunction and accelerate the recovery of body function.