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1.
Chinese Journal of Radiology ; (12): 868-872, 2022.
Article in Chinese | WPRIM | ID: wpr-956743

ABSTRACT

Objective:To investigate the MRI and pathological features of intramuscular fibro-adipose vascular anomaly (FAVA).Methods:The clinical and imaging data of 44 patients with intramuscular FAVA confirmed by pathology from December 2012 to March 2021 in Henan Province People′s Hospital were retrospectively analyzed. Twenty-five females and 19 males were included, with the age of (15±6), from 5 to 29 years old. The clinical and MRI features including the type, location, boundary, signal intensity, enhancement mode and degree, and the vascular flow voids in the lesion were summarized and compared with pathological results.Results:The thigh and calf muscles were involved in 1 patient simultaneously, and 1 site was involved in 43 patients, including 20 calf muscles, 15 thigh muscles, 5 forearm muscles, 1 upper arm muscle, 1 gluteal muscle, and 1 shoulder muscle. The gastrocnemius muscle of lower leg was most commonly involved (13/44), followed by soleus muscle (10/44) and quadriceps femoris muscle (9/44). All the lesions were solid on MRI, including 24 cases of focal mass type, 15 cases of diffuse infiltration type and 5 cases of local infiltration type. The long axis of all the lesions were consistent with the long axis of the muscles. All lesions showed inhomogeneously moderate hyperintensity on T 1WI and T 2WI, and significantly hyperintensity on fat suppression T 2WI. All lesions showed tortuous and dilated abnormal vessels, of which 18 cases showed vascular flow voids. Thrombosis was found in 10 cases. On contrast-enhanced imaging, the lesions showed moderate to obvious inhomogeneous enhancement. Pathologically, the diseased skeletal muscle was infiltrated by fibrous tissue, fat components, irregular abnormal veins and vessels, which led to inhomogeneous MRI signals. Among the 7 patients who underwent human PIK3CA gene mutation detection, and 6 were mutant. Conclusions:Intramuscular FAVA has certain characteristics in clinic, MRI imaging and histopathology, and its MRI signal characteristics can reflect its complex pathological components.

2.
Chinese Journal of Plastic Surgery ; (6): 29-34, 2020.
Article in Chinese | WPRIM | ID: wpr-798818

ABSTRACT

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.

3.
Chinese Journal of Dermatology ; (12): 557-560, 2020.
Article in Chinese | WPRIM | ID: wpr-870317

ABSTRACT

Objective:To analyze efficacy of comprehensive surgical treatment for Kasabach-Merritt phenomenon (KMP) .Methods:Clinical data were collected from 18 patients with KMP who underwent surgical treatment in Department of Hemangioma, Henan Provincial People′s Hospital from November 2017 to March 2019, and analyzed retrospectively. Preoperative treatment included preoperative use of glucocorticoids and immunoglobulins to increase platelet counts in patients. For patients irresponsive to glucocorticoids, a single large-dose of platelets was infused 12-24 hours before surgery. The platelet dose for infusion should be 0.3 and 0.2 therapeutic dose (TD) /kg respectively for patients with platelet counts lower than 30 × 10 9/L and those with platelet counts higher than 30 × 10 9/L, and the total infusion dose should be no more than 1 TD (containing about 2.5 × 10 11 platelets in 200-250 ml of infusion solution) . During the operation, tumor tissues were removed as much as possible; if there were difficulties in suturing in patients with large tumors, in situ autologous skin grafting would be performed; for children with potential postoperative functional dysfunction, a clinical consultation with rehabilitation specialists was given, and individualized functional exercises were prescribed to promote functional restoration. Results:Eighteen patients were enrolled, including 9 males and 9 females, with an average age of 73 days (range, 7-354 days) . Skin lesions were located on the extremities in 3 cases, on the trunk in 11, and on the head, face and neck in 4. Surgeries were successful in 17 patients, but 1 died. Among the 17 patients with successful operation, platelet counts and coagulation function returned to normal within 1 week after the surgery in 16, and did not returned to normal until after regular oral administration of sirolimus in 1. Favorable movement ability was maintained in all the children.Conclusion:Comprehensive surgical treatment shows rapid efficacy with less adverse reactions in the therapy of KMP.

4.
Chinese Journal of Plastic Surgery ; (6): 1102-1106, 2019.
Article in Chinese | WPRIM | ID: wpr-801083

ABSTRACT

Objective@#To explore the feasibility of application of multi-point puncture immunohistochemical to determine the invasion extent of the epidermis in Kaposiform hemangioendothelioma before surgery and choose the surgical method.@*Methods@#From July 2013 to April 2016, 17 patients with Kaposiform hemangioendothelioma were admitted to the People′s Hospital of Zhengzhou University, including 7 males and 10 females. The first consultation age was 75.60±31.55 days. Preoperative multi-point pathological puncture D2-40, CD31, CD34 immunohistochemical staining was to test the invasion extent of the epidermis in Kaposiform hemangioendothelioma and to determine whether undergo autologous skin grafting. Skin graft survival areas were recorded postoperatively. Platelet values were examined after 1 week, 4 weeks, 12 weeks and 24 weeks.@*Results@#In these 17 cases, 15 cases with epidermis and dermis unaffected was performed in situ grafting. 2 cases with the tumor invasion, superficial skin grafting were repaired. In these 15 cases of in situ grafting, platelet values returned to normal at 1 week after the operations, and platelet values were higher than 100×109/L at 4 weeks, 12 weeks and 24 weeks. Among them, 12 cases of epidermal survival area were greater than 90%, 2 cases were 70%-90%, and 1 cases was about 50%.@*Conclusions@#During the complete resection of Kaposiform hemangioendothelioma, the multi-puncture D2-40, CD31 and CD34 staining are used to determine invasion extent of the tumor to the skin, which can maximize the retention of normal tissue surface, reduce postoperative complications. This is a new multi-disciplinary surgical approach.

5.
Chinese Journal of Plastic Surgery ; (6): 371-375, 2019.
Article in Chinese | WPRIM | ID: wpr-804984

ABSTRACT

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.

6.
Chinese Journal of Plastic Surgery ; (6): 189-194, 2019.
Article in Chinese | WPRIM | ID: wpr-804744

ABSTRACT

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 μl/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.

7.
Chinese Journal of Plastic Surgery ; (6): 384-390, 2018.
Article in Chinese | WPRIM | ID: wpr-806538

ABSTRACT

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.

8.
Chinese Journal of Plastic Surgery ; (6): 356-359, 2018.
Article in Chinese | WPRIM | ID: wpr-806531

ABSTRACT

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.

9.
Chinese Journal of Applied Clinical Pediatrics ; (24): 874-877, 2018.
Article in Chinese | WPRIM | ID: wpr-696520

ABSTRACT

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.

10.
Chinese Journal of Stomatology ; (12): 700-703, 2017.
Article in Chinese | WPRIM | ID: wpr-809508

ABSTRACT

PHACE syndrome is a syndrome of multiple organ and multisystem abnormalities associated with infantile segmental hemangioma, characterized by abnormal posterior fossa development, infant hemangioma, aortic abnormalities, aortic coarctation and heart defects, eye anomalies and other symptoms. The incidence of the disease is low, but there exist life-threatening factors. Once clinically diagnosed, it should be highly valued and multidisciplinary consultation must be conducted. This article reviews the diagnostic criteria of PHACE syndrome and its associated facial segmental hemangioma, as well as the treatment and prognosis of brain abnormalities.

11.
Chinese Journal of Plastic Surgery ; (6): 433-437, 2017.
Article in Chinese | WPRIM | ID: wpr-808853

ABSTRACT

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.

12.
Chinese Journal of Plastic Surgery ; (6): 25-29, 2017.
Article in Chinese | WPRIM | ID: wpr-808003

ABSTRACT

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.

13.
Chinese Journal of Applied Clinical Pediatrics ; (24): 1432-1434, 2016.
Article in Chinese | WPRIM | ID: wpr-502159

ABSTRACT

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.

14.
Chinese Journal of Experimental Ophthalmology ; (12): 919-923, 2015.
Article in Chinese | WPRIM | ID: wpr-637624

ABSTRACT

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.

15.
Chinese Journal of Applied Clinical Pediatrics ; (24): 1583-1586, 2014.
Article in Chinese | WPRIM | ID: wpr-466646

ABSTRACT

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.

16.
Chinese Journal of Applied Clinical Pediatrics ; (24): 789-791, 2014.
Article in Chinese | WPRIM | ID: wpr-453389

ABSTRACT

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.

17.
Chinese Journal of Dermatology ; (12): 822-823, 2013.
Article in Chinese | WPRIM | ID: wpr-442121

ABSTRACT

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.

18.
Journal of Practical Radiology ; (12)2001.
Article in Chinese | WPRIM | ID: wpr-543830

ABSTRACT

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.

19.
Journal of Practical Radiology ; (12)1991.
Article in Chinese | WPRIM | ID: wpr-542712

ABSTRACT

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.

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