Kasabach-Merritt phenomenon (KMP) is characterized by thrombocytopenia with enlarging vascular tumour,KMP usually develops in infancy and is associated with significant morbidity and mortality,the mortality rate is reported as high as 30％.It commonly reported sites of tumor include extremities,trunk,retroperitoneum and neck.There is no consensus in treatment and various regimens have been used by different authors.This report is aim to learn the pathophysiology of the KMP and its diagnosis and treatment.

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 To evaluate the role of magnetic resonance angiography ( MRA ) in diagnosing peripheral hemangioma andvascular malformation . Methods 61 cases of hemangioma and vascular malformations in peripheral soft tissue were undergone MRAexamination.Results Of 13 patients with hemangioma,the arteries within hemangioma were increased and gradually fine from proximal to distal in 7 cases and in company with arteriovenous fistulae in 2 cases,there were no arteries within hemangioma in 6 cases.Vascular malformations were found in 48 patients,arteries and veins of vascular malformation were showed in 35 cases,but arteries of vascular malformations were only showed in one case.Arteries of vascular malformation were showed in 5 cases and the arteries were pressed on arterial angioyraphy in 23 cases.On MR venography(MRV),the shallow malformed veina were showed in 25 cases and in company with deep malformed veina in 13 cases,only the shallow and deep veina increased and thickness be showed in 2 cases.Arterioveinous fistulae could be seen in 8 cases on MR aterio-venography.There were no vessel be showed in 12 cases within the losions.Conclusion MRA is of significant value in diagnosis and differential diagnosis of peripheral hemangioma and vascular malformations.

Background Venous malformation damages the local tissue severely because of the progressive development and often presents with invasive biological behavior.Galectin-3 (Gal-3) is proved to be closely associated with local invasion of malignant tumor.Studying the role of Gal-3 on tissue invasion in venous malformation of ocular region is of important clinical significance.Objective This study was to explore the role of Gal-3 protein and mRNA expression in venous malformation of ocular region.Methods One hundred and eighteen pathological sections were collected from ocular venous malformation patients who received surgery in Department of Hemangioma Surgery,People's Hospital of Henan Province and Henan Eye Institute from June 2009 to June 2014.The specimens were further diagnosed by histopathological examination.Then the expressions of Gal-3 protein and mRNA in venous malformation of ocular region were detected by using immunohistochemistry and in situ hybridization and compared with 20 pieces of distal cutting edge specimens which were evidently normal.The associations of Gal-3 positive expressions with invasion and configuration of lesions were analyzed.Results Pathological examination showed that venous malformations tissues contain many big blood vessels lacuna, lined with fiat endothelial cells.Immunochemistry and in situ hybridization exhibited that Gal-3 protein and mRNA were expressed in the cytoplasm and nuclei.The positive expression rates of Gal-3 protein and mRNA in the venous malformation tissues were 55.93％ (66/118) and 59.32％ (70/118) , but those in the normal tissue were 15.00％ (3/20) and 20.00％ (4/20) ,showing significant differences between them (x2 =11.461, 10.633, both at P＜0.05).No significant differences were seen in the positive expression rates of Gal-3 protein and mRNA between the patients aged ≤ 12 years and ＞12 years or different genders (age: x2 =0.334,0.128;both at P＞0.05.gender:x2 =0.606,1.155;both at P ＞0.05).The incidence rate of invading ocular deep tissues was significantly higher in the Gal-3-positive groups than that in the Gal-3-negative groups of protein and mRNA (protein :x2 =32.688, P＜0.05;mRNA : x2 =23.695, P＜0.05).In the Gal-3-negative groups,96.15％ (Gal-3 protein negative group) and 97.92％ (Gal-3 mRNA negative group) lesions showed the spherical shape with clear boundaries.The lesions texture with the fuzzy boundaries and the incidences of vague structure in lesions were significantly higher in the Gal-3-positive groups than that in the Gal-3-negative groups of protein and mRNA (protein :x2 =28.255, P＜0.05;mRNA : 28.186, P＜0.05).Conclusions Gal-3 expression rate is raised in the deep tissue-invaded and texture disorder ocular venous malformation.These results suggest that invasion and damage of ocular venous malformation are associated with the up-regulation of Gal-3.

Objective To study MRI differential diagnosis of peripheral hemangioma and vascular malformations.Methods MRIfindings of peripheral hemangiomas and vascular malformations proved by operation in 61 cases were retrospectively analyzed in comparison with pathological results.Results In 29 cases of peripheral hemangiomas,20 cases showed equal signal intensity (equal to muscle) and 9 cases showed heterogeneous signal intensity on T1-weighted images; 24 cases showed increased signal intensity (approach fat signal) and 5 cases showed markedly increased signal intensity (increase fat signal) on T2-weighted images,25 cases had septa as low signal network on T2-weighted images,4 cases showed enhancement separated mass enhancement after intravenous contrast injection.In 32 cases peripheral vascular malformations,16 cases showed equal signal intensity and 16 cases showed heterogeneous signal intensity on T1-weighted images.3 cases showed high signal intensity and 29 cases showed markedly increased signal intensity on T2-weighted images,15 cases showed inhomogeneous enhancement after intravenous contrast injection.Conclusion MRI plays an important role in differential diagnosis of peripheral hemangiomas and vascular malformations.

Objective To describe and examine the clinical characteristics and spectrum of tufted angiomas (TA) in infants so as to explore the therapeutic approaches to Kasabach-Merritt phenomenon (KMP).Methods The clinical and follow-up data of 24 patients with TA were retrospectively analyzed between Jan.2009 and Mar.2013.The median age of the patients was 7.5 months(ranged from 18 days to 2 years),including l0 male and 14 female.Surgical excision and observation were chosen according to the lesion and conditions of the patients.The mean follow-up period was 3.6 years (1.2 to 5.4 years).The changes in the patients' s condition were established by evaluating platelet counts,and the size of lesion.Results Common clinical features included dusky red or violaceous infiltrating cutaneous lesion,thrombocytopenia,pain or decreased function and hyperhidrosis or hypertrichosis.The following 3 clinical patterns of TA progress were described:spontaneous complete or partial regression (n =2,8.3％),TA without complications and persistence over the years(n =9,37.5％),and TA complicated by KMP(n =13,54.2％).The average interval of development of KMP for delayed cases was 45.2 days(ranged 0 d to 4 months).Each of the 13 patients who developed KMP subsequent to initial presentation was symptomatic at the time KMP was detected(enlarged lesion,n =8;increased lesion firmness with change in cutaneous stain,n =3 ;and respiratory distress,n =2).All of 13 patients were cured by surgery.Complete surgical resections were performed on 10 cases.The thrombocyte count was back to normal within 1-3 days post operation,and hemoglobin and blood coagulation function gradually returned to normal within 1 to 2 weeks.Other 3 cases received major resection surgery.The number of platelets in the patients were unstable,but significantly higher than that of preoperational stage.The platelet count remained above 60 × 109/L.The residue lesions in 2 cases disappeared gradually after the operation and medication were given within 3-6 months.And the other case died of multiple organ failure post-operation.Conclusions Surgical intervention can be applied to TA that severely makes damage to children's appearance or looks or may be complicated with KMP or functional abnormality.A closely monitored policy seems appropriate for the early small tumor without severe complications.And it is necessary to monitor the number of the platelet regularly and find the KMP by as early as possible.TA associated with KMP is vitol to infants.And surgical therapy after clear diagnosis should be done as early as possible.The surgical therapy is a reliable management with higher curative rate,short disease period and minimal side-effect.

Objective To explore the therapeutic effect and the efficacy of external carotid artery catheterization and drug infusion therapy for severe infant hemangiomas in oral and maxillofacial regions.Methods The clinical and follow-up data of 126 cases with severe infant oral and maxillofacial hemangiomas were retrospectively analyzed in Henan Provincial People's Hospital between June 2004 and December 2009.The patients included 45 male and 81 female cases,and the average visiting age of the patients was 3.9 months (ranged 10 days to 1 year and 4 months).Among 126 patients,24 cases occurred with Kasabach-Merritte syndrome (KMS).The patients were treated with ligation of external carotid artery,insertion of a tube inside the lesion and injection of 400 g/L Carbonyldiamide after operation.Methylprednisolone was added in infusion if the patients were combined with KMS.Results The mean follow-up period was 6.1 years (ranged 4.3 to 10.2 years).All of 126 cases were cured through the management including external carotid artery catheterization and drug infusion therapy in 94 cases,and the combination treatment of the infusion and local Carbonyldiamide injection in 32 cases.The size of hemangiomas gradually shrank and disappeared after the therapy.The hemangiomas in 105 cases disappeared completely in 6-12 months,and 21 cases disappeared in 1-2 years.Twenty-four patients were complicated with KMS.The thrombocyte count and blood coagulation function also returned to normal after the therapy.Among the 24 cases,the thrombocyte count in 13 cases was back to normal within 3-7 days,and 7 cases within 8-14 days.In the remaining 4 cases,it normalized within 15-30 days.The weight,height,immunity of the patients were in keeping with the healthy counterparts after long term follow-up.And no serious adverse effect was observed.Conclusions For severe infant hemangiomas in oral and maxillofacial regions,the external carotid artery catheterization and drug infusion therapy can obviously shorten the treatment time,and reduce the drug side effects.It is a better and more reliable treatment method.

OBJECTIVETo explore the therapeutic effect of radiofrequency ablation for tongue venous malformation( VM).

METHODSFrom July 2013 to July 2014, 30 cases with tongue VM (local or diffuse) were retrospectively analyzed. 23 cases underwent radiofrequency ablation treatment. The radiofrequency electrode tip(0. 5 mm in diameter) was inserted into the lesion 1 mm below the bottom with 25 W in power and 15-30 s of pulse. The treatment was repeated when the electrode tip was removed back every 1 mm. Multi-point treatment was performed.

RESULTS15 cases with unilateral VM were completely healed after one-stage radiofrequency ablation. 8 cases with bilateral VM received two-stage radiofrequency ablation with a 3-6 months of interval. Among the 8 cases, completely healing was achieved in 5 cases, partial VM residue happened in 3 cases due to its diffuse lesion and reservation of tongue function. 23 cases were followed up for 3 month to 1.5 years. Good cosmetic and functional results was achieved in 20 cases with no relapse. Partial VM residue was left in 3 cases.

CONCLUSIONSRadiofrequency ablation can effectively treat tongue VM with minimal morbidity and good cosmetic appearance. It also avoids the disadvantages of surgery.

Catheter Ablation ; instrumentation ; methods ; Electrodes ; Humans ; Retrospective Studies ; Tongue ; blood supply ; Treatment Outcome ; Vascular Malformations ; surgery ; Veins ; abnormalities

Kaposiform hemangioendothelioma(KHE) is a kind of organization form is similar to Kaposi's sarcoma(KS) and aggressive cancer,found in children and infants are rare and multiple in the limbs,torso,facial skin and deep soft tissue can also occur in the mediastinum,retroperitoneal etc.KHE is one of the main causes of Kasabach Meritt phenomenon(KMP) in infants and young children,the mortality rate is as high as 10％-37％,even 52％.At present,there is no uniform and effective treatment method at home and abroad.The clinical manifestations,diagnosis and treatment of KHE are reviewed.

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.