AIMS:Fibroadipose vascular anomaly (FAVA) is a complex vascular malformation that is likely to be under-recognised. In this study we aimed to report the pathological features and somatic PIK3CA mutations associated with the most common clinicopathological features. METHODS AND RESULTS:Cases were identified by reviewing the lesions resected from patients with FAVA registered at our Haemangioma Surgery Centre and unusual intramuscular vascular anomalies in our pathology database. There were 23 males and 52 females, who ranged in age from 1 to 51 years. Most cases occurred in the lower extremities (n = 62). The majority of the lesions were intramuscular, with a few disrupting the overlying fascia and involving subcutaneous fat (19 of 75), and a minority of the cases had cutaneous vascular stains (13 of 75). Histopathologically, the lesion was composed of anomalous vascular components that were intertwined with mature adipocytic and dense fibrous tissues and vascular components with: (a) clusters of thin-walled channels, some with blood-filled nodules and others with thin walls resembling pulmonary alveoli; (b) numerous small vessels (arteries, veins and indeterminate channels) - proliferative small blood vessels were often mixed with adipose tissue; (c) larger abnormal venous channels usually irregularly and sometimes excessively muscularised; (d) lymphoid aggregates or lymphoplasmacytic aggregates were usually observed; and (e) lymphatic malformations were sometimes seen as minor elements. All patients had their lessons subjected to PCR, and 53 patients had somatic PIK3CA mutations (53 of 75). CONCLUSIONS:FAVA is a slow-flow vascular malformation with specific clinicopathological and molecular characteristics. Its recognition is fundamental for its clinical/prognostic implications and for targeted therapy.

ObjectiveTo investigate the value of ultrasonography as a diagnostic aid in differentiating intramuscular capillary-type hemangioma (ICTH) from fibro-adipose vascular anomaly (FAVA).MethodsA retrospective analysis was conducted of the clinical and ultrasound imaging data of 20 patients with ICTH and 45 patients with FAVA who were admitted to and pathologically confirmed in hospital between January 2013 and April 2023. The clinical and ultrasonographic appearances of the lesions in the two groups were compared and analyzed. A stepwise regression analysis was performed, and a joint diagnostic equation was constructed using the final variables selected. The receiver operating characteristic (ROC) curve and indicators, including sensitivity and specificity, were used to evaluate the efficacy of the joint diagnostic model.ResultsThe two groups of patients suffering from ICTH and FAVA presented a statistically significant difference (P< 0.05) in terms of ‘age’, ‘lesion size’, ‘fascial tail sign’, ‘presence of a fatty-tissue-like hyperecho around the lesion’, ‘blood flow’ and ‘presence of straight blood capillaries within the lesion’. Finally, the variables ‘fascial tail sign’ and ‘presence of straight blood capillaries within the lesion’ were selected to construct the model. The constructed joint diagnostic model had a sensitivity value of 70.0% (95% CI: 59.00–81.00), a specificity value of 98.0% (95% CI: 94.70–100.00) and a ROC curve value of 0.908, indicating the high efficacy of the combined diagnosis method.ConclusionsUltrasonography can be utilized to differentiate ICTH from FAVA, and the combined diagnosis method can further improve the technique’s diagnostic efficacy.

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.

See Original Article here. Dear Editor, With great interest, we read the article by Lin et al.1 published in 2020 in the Br J Clin Pharmacol. The authors performed a meta-analysis and concluded that “topical β-receptor blocker (propranolol and timolol), especially timolol, may replace oral propranolol as a first-line treatment for superficial infantile hemangiomas.” At the outset, we would like to congratulate the authors for writing an informative article with novelty. Nevertheless, we have several suggestions and queries that we would like to communicate with the authors. First and foremost, the authors mentioned that a variety of databases were used in this study, but they were not enough. It would make the outcomes more convincing if the authors included other databases, like clinicaltrials.gov, Web of Science, NLM Gateway, and BIOSIS previews, to obtain more literature. Besides, the manual search protocols should also be included in this meta-analysis. Essential literature will be ignored if the manual search protocol is incomplete, and unpublished data such as grey literature should be included. Another concern of us is about the high heterogeneities in the results. High inter-study heterogeneity could not result in a definite conclusion according to Cochrane Handbook for Systematic Reviews. With such large heterogeneities found in this some outcomes, subgroup analysis and sensitivity analysis had not been conducted to explain the source of heterogeneity. Therefore, we suggest that the authors should mention the possible sources of heterogeneities by using adequate subgroup analyses. The possible sources of heterogeneities include study design, age differences, duration of treatment, diseased region, and drug dosage. As far as we know, a more robust model, named Inverse Variance Heterogeneity (IVhet) model, has been introduced in the study of Doi et al.2 Doi et al. examined an improved alternative to the random effects (RE) model for meta-analysis of heterogeneous studies. It was shown that the known issues of underestimation of the statistical error and spuriously overconfident estimates with the RE model could be resolved by the use of an estimator under the fixed effect model assumption with a quasi-likelihood based variance structure—the IVhet model. Thus, we suggest that the IVhet model be used to re-analyse the outcomes in this study. Finally, many included studies were not randomized controlled trials, and there were significant language biases in this paper, which should be pointed out in the discussion. Overall, Lin et al. analysed a valuable issue, but the results of this meta-analysis should be interpreted with caution due to the limitations mentioned above. There are no competing interests to declare.

As a complex vascular malformation, fibro-adipose vascular anomaly was first proposed in 2014. Its overlap with other vascular malformations regarding imaging and clinical features often leads to misdiagnosis and improper management. To construct a radiomics-based machine learning model to help radiologists differentiate fibro-adipose vascular anomaly from common venous malformations. We retrospectively analyzed 178 children, adolescents and young adults with vascular malformations (41 fibro-adipose vascular anomaly and 137 common vascular malformation cases) who underwent MRI before surgery between May 2012 to January 2021. We extracted radiomics features from T1-weighted images and fat-saturated (FS) T2-weighted images and further selected features through least absolute shrinkage and selection operator (LASSO) and Boruta methods. We established eight weighted logistic regression classification models based on various combinations of feature-selection strategies (LASSO or Boruta) and sequence types (single- or multi-sequence). Finally, we evaluated the performance of each model by the mean area under the receiver operating characteristics curve (ROC-AUC), sensitivity and specificity in 10 runs of repeated k-fold (k = 10) cross-validation. Two multi-sequence models based on axial FS T2-W, coronal FS T2-W and axial T1-W images showed promising performance. The LASSO-based multi-sequence model achieved an AUC of 97

Objective:To investigate the differential performance of ultrasound between fibro-adipose vascular anomaly (FAVA) and venous malformations(VMs).Methods:From January 2015 to December 2020, the patients diagnosed with lower extremity FAVA by pathology in Henan Provincial People’s Hospital were enrolled as FAVA group. The patients diagnosed with lower extremity VMs by pathology were enrolled as the control group. The clinical and ultrasound imaging data were retrospectively analyzed. Through the single factor analysis of the two groups’data, the ultrasonic imaging indicators which may be valuable for distinguishing FAVA from VMs were screened. Further, the indicators valuable for differential diagnosis were determined by multi-factor Logistic regression analysis, and a multi-factor joint diagnosis model was constructed. The diagnostic efficiency of the joint diagnosis model was evaluated by the receiver operator characteristic curve (ROC curve), sensitivity, and specificity of the subjects.Results:A total of 20 patients with FAVA were involved, including 11 males and 9 females. The mean age was (18.1±12.2) years. Forty-six patients with VMs were involved, including 20 males and 26 females. The mean age was (19.9±13.6) years. Results of the single-factor analysis were differences in the lesion echo, fascial tail, blood flow, extrusion test, and posterior echo enhance characteristics between groups ( P<0.05). Multivariate analysis showed significant differences between groups in three aspects: fascial tail, extrusion test, and posterior echo enhancement ( P=0.001, 0.008, 0.007). The sensitivity and specificity of the multi-factor combined diagnosis model were 90.0% (95% CI: 68.3%-98.8%) and 93.5%(95% CI: 82.1%-98.6%), indicating high diagnostic efficiency. The ROC(AUC) area was 0.964(95% CI: 0.886-0.994), indicating high diagnostic efficiency. Conclusions:The ultrasonic imaging features of FAVA and VMs were different. The combined diagnosis of the fascial tail, compression test, and posterior echo enhancement has a higher auxiliary diagnostic value.

Objective To compare the curative effect of non-surgical treatment,early operation and advanced operation on the Kaposiform hemagioendothelioma (KHE).To explore the optimal treatment for KHE.Methods The 106 cases of KHE patients admitted by our hospital from January 2004 to December 2016 were selected as the study subjects.The age is the minimum 1d and the maximum 43 years,median age 94.5 d.According to different treatment methods,they were divided into non-surgical treatment and surgical treatment:according to the onset of the disease and the day of the operation,the early operation was performed less than three months since the onset of the disease,and the advanced operation more than 3 months.Efficacy evaluation criteria:The recovery meant that the tumor disappeared or mostly disappeared,the platelets returned to normal,and there was no recurrence more than half a year after treatment." Effective" indicated that tumors were shrunken significantly and the platelet counting rose significantly." Ineffective" indicated that the tumor was not shrunken or continued to increase and platelet counting did not rise or continued to decrease.The three groups of patients were followed up,and the efficacy of the three groups after treatment and the changes of platelet counting before and after treatment on day 1,7 and 14 were compared.The two groups of early and late treatment were compared for operative and postoperative conditions.Results All patients were followed up for 6-23 months,24 cases were effective,12 cases were ineffective,and 5 cases died.After 7 days of treatment,the platelet counting of the three groups were 68 (31,157) × 109/L in the non-surgical treatment group,(294.75± 150.59) × 109/L in the early operation group,(221.48± 148.71)× 109/L in the late operation group,with statistical significance (P ＜ 0.05).After 14 days of treatment,the platelet counting of the three groups were 78 (25.51,54.5) in the non-surgical treatment group,281 (234,356) × 109/L in the early operation group,and in the late operation group (219.67±140.95)× 109/L,with statistical significance (P ＜ 0.05).After at least half a year of follow-up,the total effective rate of clinical efficacy in the three groups was the non-surgical treatment group 62.07％,the early operation group 96.34％ and the late operation group 81.82％;the difference was statistically significant between two groups (P ＜ 0.05);There was significant difference between the early operation group and the late operation group in the amount of bleeding and operation time,the time of platelet counting and the total hospitalization time (P＜ 0.05).Conclusions Early surgical treatment of Kaposiform hemangioendothelioma is superior to late-operative and non-surgical treatment.Surgical treatment will be the preferred option for treatment of Kaposiform hemangioendothelioma,and examinations should be operated to potential patients for early diagnosis.

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.

AIMS:Infantile hemangioma (IH) is the most common vascular neoplasm in infant and young children. Long non-coding RNAs (lncRNAs) are known to be associated with IH. This study aims to investigate the role and underlying mechanism of lncRNA-MALAT1 in IH.MAIN METHODS:qRT-PCR was used to quantify the expressions of MALAT1, miR-424, and MEKK3 in IH tissues. The cell proliferation, apoptosis, migration, invasion, and tube formation ability were assessed by MTT assay, colony formation assay, flow cytometric analysis, transwell assay and tube formation assay, respectively. The interaction among MALAT1, miR-424 and MEKK3 was evaluated by luciferase reporter assay. Immunohistochemistry (IHC) and Western blotting were utilized to evaluate the expression levels of MEKK3, Ki-67 and NF-κB pathway-related proteins both in vitro and in vivo.KEY FINDINGS:In IH tissues, MALAT1 and MEKK3 were overexpressed while miR-424 was down-regulated. Silencing MALAT1 or overexpression of miR-424 significantly inhibited the IH cell proliferation, migration and tube formation, but promoted the cell apoptosis. Knockdown of MALAT1 suppressed the expression of MEKK3 and inactivated the IKK/NF-κB pathway by sponging miR-424. Overexpression of MEKK3 in HemEcs reversed the impact of knockdown of MALAT1 and overexpression of miR-424 on the cell proliferation, apoptosis, migration, invasion and tube formation rate. The tumor xenografts experiments demonstrated that silencing MALAT1 significantly inhibited the tumor growth in vivo and Ki-67 in the tumor tissues was also significantly suppressed.SIGNIFICANCE:MALAT1 promoted the IH progression through inhibiting miR-424 to activate MEKK3-mediated IKK/NF-κB pathway, suggesting that MALAT1, miR-424 and MEKK3 could be used as potential targets to improve IH treatment efficiency.

Objective To observe the effects of different concentrations and doses of urea on the proliferation and apoptosis of human hemangioma endothelial cells,in order to provide evidence for the further mechanism study of urea in the treatment of hemangioma.Methods Human hemangioma endothelial cells (HemECs) and normal endothelial cells (VE) were cultured in vitro.Cell viability was detected by CCK-8 after invention with different concentrations(40％,50％,60％,70％) and doses(3,6,9 pl/ml) of urea.The apoptosis of HemECs was detected by flow cytometry dual-dye and propidium lodide single dye.Results The viability of HemECs was significantly lower than that of VE under different concentrations and doses of urea (P＜0.05).The inhibition rate of 40％ urea on HemECs increased with the increase of urea dose (P＜0.05),and the inhibition effect was most obvious at 4 h and 12 h.The apoptosis of HemECs increased in a time and dosage dependent manner with the treatment of 40％ urea.High dose(9 μl/ml) of 40％ urea significantly promoted the apoptosis of HemECs(P＜0.05).Conclusions Low dose of 40％ urea significantly inhibited the proliferation of HemECs,and had no significant effect on VE.However,high doses of urea promoted apoptosis of HemECs.

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.

Hemangioma (HA) is one of the commonest benign vascular neoplasms of infancy. Inhibitor of differentiation 1 (ID-1) has been reported to be an oncogene in multiple cancers. However, the role of ID-1 and its molecular mechanism in HA progression have not been elucidated. In the present study, we found that ID-1 expression at mRNA and protein levels was up-regulated in HA-derived endothelial cells (HDECs). Knockdown of ID-1 inhibited proliferation, facilitated apoptosis, and enhanced propranolol cytotoxicity in HDECs. Knockdown of ID-1 decreased the protein levels of phospholyrated protein kinase-B (Akt) and phospholyrated mammalian target of rapamycin (mTOR). Inhibition of PI3K/Akt/mTOR pathway by LY294002 abrogated ID-1-mediated pro-proliferation and anti-apoptosis effects in HDECs. In conclusion, knockdown of ID-1 suppressed proliferation and promoted apoptosis by inactivating phosphatidylinositol 3-kinase (PI3K)/Akt/mTOR signaling in HDECs, shedding light on the function of ID-1 in HA progression and highlighting the therapeutic value of ID-1 for HA.

Kasabach-Merritt phenomenon (KMP) is a rare potentially life-threatening consumptive coagulopathy characterized by thrombocytopenia and hypofibrinogenemia occurring associated with the vascular tumors kaposiform hemangioendothelioma (KHE) and tufted angioma (TA). KHE in the specific sites will also cause some non-hemorrhagic complications. We report a two-month old female infant with KHE in her pelvic cavity, bladder and ureter who responded well to surgical excision.

To investigate the relationship between glucocorticoid receptor (GRα,GRβ) expression and hormone sensitivity in kaposiform hemangioendothelioma (KHE) patients complicated by Kasabach-Merritt phenomenon (KMP).Methods In this study,25 cases of KHE with KMP (mean age 2.4 ± 1.5 months),including 9 males and 16 females at Henan Provincial People's Hospital between May 2013 and May 2016 were included.All patients underwent surgical resection after being treated with hormone for one week,and were divided into sensitive group (10 cases) and resistance group (15 cases) according to the efficacy evaluation criteria.Normal tissues collected from 15 patients received surgical excision of lipoma was performed as a control group.Immunohistrochemical SP method was adopted in detecting GRα and GRβ expression in all groups.The expression intensity and positive rate were analyzed.Statistical significance was determined using the Wilcoxon rank sum test for the group samples and the Kruskal-Wallis test for multiple samples.Values of P ＜ 0.05 were considered statistically significant.Results ①There was no significant difference among the control group,the resistance group and the sensitive group in expression intensity of GRα (control group 4.20 ± 1.01,resistance group 4.10 ± 0.99,sensitive group 3.53 ± 0.52,P =0.632).The number of GRα positive cells in the sensitive group (46.20 ± 2.57)was higher than that in the resistance group (40.93 ± 5.18,P =0.032).②The expression intensity of GRβ in the resistance group(5.40 ±0.51)was significantly higher than that in the sensitive group(2.60 ± 0.52)and the control group(2.87 ±0.64,P =0.000,0.002);there was significant difference among the sensitive group(29.70 ± 2.50),the control group (36.07 ± 3.47) and the resistance group (47.27 ± 5.59) in the number of GRβ positive cells (P =0.000).③The ratio of GRα/Grβ expression intensity was significantly lower in the resistance group than in the sensitive group and the control group;The ratio of GRα/GRβ positive cells was gradually decreased in the sensitive group,control group and resistance group,and the difference was statistically significant (P =0.000).Conclusions In the tumor tissue of KHE children with KMP,the increase of GRβ expression negatively regulats GRα,leading to imbalance of the expression ratio of the two,which may be an important cause for GC resistance.

The aim of this study was to investigate the manner of urea-modulated UT-B urea transporter (UT) internalization in infantile hemangioma-derived vascular endothelial cells (HemECs). The immunohistochemistry assay was performed to identify infancy hemangioma-derived endothelial cell line (XPTS-1) cells. Cell toxicity was detected with the 3-[4,5-dimethylthiazol-2-yl]-2,5-diphenyl tetrazolium bromide (MTT) assay. Quantitative real-time polymerase chain reaction and Western blot analysis were measured to analyze the expression of UT-B. UT-B internalization was observed by confocal microscopy. The clathrin inhibitor chlorpromazine (CPZ) and caveolin endocytic disrupter methyl-β-cyclodextrin (MβCD) were used in XPTS-1 cells transfected with UT-B-GFP to repress endocytosis. Urea-promoted UT-B expression in a concentration-dependent manner in an infantile XPTS-1 cell line. CPZ and MβCD significantly inhibited UT-B protein internalization. The pretreatment of UT-B-GFP cells with adaptor protein2 (AP2)-μ2-siRNA and caveolin-siRNA significantly inhibited UT-B protein internalization. Our findings suggested that urea-mediated UT-B UT internalization is clathrin and caveolae dependent in infantile HemECs.

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.

自1982年Mulliken和Glowacki[1]提出先天性血管病变的病理学分类以来，对于先天性血管病变的认识和治疗水平有了飞速的发展。依据病灶内血管内皮细胞的特点将先天性血管病变分为两大类：血管瘤和血管畸形。血管瘤以血管内皮细胞的数量及层数增多为特点，典型的临床表现为出生后1年内快速生长期及之后的缓慢自发性消退期；而血管畸形的血管内皮细胞层数正常，终生缓慢发展，不会消退。静脉畸形是最为常见的一种血管畸形，占所有血管畸形的2/3[2]，可发生于任何有正常血管生长的部位，导致毁容及功能丧失，严重者影响凝血功能甚至危及生命[3-4]。位于女性外阴部的海绵状血管瘤即为静脉畸形，由于其初期临床表现不典型，易被误诊为静脉曲张、巴氏腺囊肿、疝及其他软组织肿瘤。对于该病的治疗，国内、外尚未制定统一的标准及指南，治疗方式多样，效果不一，预后差别较大。单纯的手术切除，存在术中出血多、视野不清晰、瘤组织残留的问题。瘤体内注射尿素用于治疗血管畸形已有近30年历史[5]，静脉畸形病灶内尿素注射可使病灶边界清晰，病灶内血栓形成[6-7]，弥补单纯手术治疗的不足。本研究对2009年1月至2014年3月河南省人民医院血管瘤科收治的采用瘤内尿素注射联合手术治疗的32例女性外阴部海绵状血管瘤患者的临床资料进行回顾性分析，现报道如下。

OBJECTIVE:To summarize the characters of Kasabach-Merritt syndrome (KMS) and to evaluate the therapeutic effect of drug therapy combined with surgery.METHODS:From 2004 to 2010, 59 cases with KMS, who underwent drug therapy and surgery, were retrospectively studied. The average age of the patients, including 33 male and 26 female (male/female, 1.269/1), was 2.9 months (range, 7 days-2.5 years). 28 cases with maxillofacial lesions were treated with the ligation of external carotid artery and injection of carbonyldiamide and methylprednisolone. 31 cases with lesions at trunks and extremities were treated by excision of lesions. All the patients were followed up for 2.8 years (range, 6.5 months -7.3 years). Therapeutic outcomes were assessed by evaluating platelet counts,size of lesion, function of trunk and limb.RESULTS:58 cases were cured except for one dead case. Emergency operation was given in 4 cases, and selective operation was performed in other cases (55 cases). The thrombocyte count, hemoglobin and blood coagulation function returned to normal within 1-2 weeks. The mental condition, appetite, body weight,sleeping were greatly improved one week after treatment. The size of the lesions decreased gradually after the management of ligation of external carotid artery including 18 cases within 6-12 months and 10 cases within 13-24 months. Long term follow-up studies indicated that there was no recurrent case, and the weight, height, immunity of the patients with good function activities were in keeping with the normal counterparts.CONCLUSIONS:The drug combined with surgery therapy is a very reliable management with high curative rate, short disease period and minimum side-effect.

目的观察普萘洛尔联合尿素治疗婴儿眼部血管瘤的效果及安全性。方法眼部血管瘤患儿95例,依据治疗方法分为A组30例,B组30例,C组35例,A组局部注射尿素,B组口服普萘洛尔,C组联合应用尿素和普萘洛尔。观察3组不同分型血管瘤患者治疗效果,并进行比较。结果 A组治愈23例(76.7%),B组治愈19例(63.3%),C组治愈34例(97.1%),C组治愈率高于A,B组(P<0.05);C组表皮型、软组织肿块型、眶内混合型血管瘤治愈率均高于A,B组(P<0.05),A组表皮型、软组织肿块型血管瘤治愈率高于B组(P<0.05),眶内混合型血管瘤治愈率低于B组(P<0.05)。结论普萘洛尔联合尿素治疗表皮型、软组织肿块型、眶内混合型血管瘤均可获得良好效果,局部注射尿素适用于表皮型、软组织肿块型血管瘤,口服普萘洛尔适用于治疗眶内混合型血管瘤。