Venous malformations with severe localized intravascular coagulopathy treated with microwave ablation.

OBJECTIVES: To evaluate the safety and feasibility of microwave ablation for treating venous malformations (VMs) with severe localized intravascular coagulopathy (LIC). PATIENTS AND METHODS: Data for patients with the diagnosis of VMs coupled with severe LIC who underwent color Doppler-guided microwave dynamic ablation between January 2017 and June 2019 were retrospectively reviewed and analyzed. All patients had previously received sclerotherapy or other treatments with poor outcomes and gradual aggravation of coagulation abnormalities. Microwave treatment with "dynamic ablation" was performed with real-time color Doppler monitoring and was repeated if necessary after 3 months. Low-molecular-weight heparin (LMWH) was used to control consumptive coagulopathy. The therapeutic efficacy including coagulation function and lesion size was evaluated using the four-level scale developed by Achauer. RESULTS: Among 15 patients with extensive diffuse or multiple VMs, 10 patients presented with lesions in a single lower extremity, one in both lower extremities and the perineum, one in both upper extremities and the trunk, and three with multiple lesions. The patients underwent a total of 74 microwave ablation sessions, with an average of 4.9 sessions per person. Coagulation abnormalities were temporarily aggravated in 59 sessions within the first seven days post-ablation but improved to grade II (fair) a week later. From six months to three years after the ablation, the lesions improved to grade IV (excellent) in one patient, grade III (good) in six patients, and grade II (fair) in eight patients. Moreover, the coagulation function improved to grade IV in four patients, grade III in eight patients, and grade II in three patients, resulting in an efficiency rate of 80% (12/15). Post-ablation complications included fever, hemoglobinuria, and elevations in aspartate aminotransferase, lactate dehydrogenase, and alanine aminotransferase. The patients with fever and hemoglobinuria recovered after specific therapeutic measures, but elevations in aspartate aminotransferase, lactate dehydrogenase, and alanine aminotransferase recovered spontaneously without further interventions. CONCLUSIONS: Ablation coupled with anticoagulation can effectively treat VMs in patients with severe LIC and improve the long-term coagulation function.

Analysis of 18 complex diffuse arteriovenous malformation cases treated with percutaneous radiofrequency ablation.

BACKGROUND: The aim of the present study is to evaluate the short-term efficacy and feasibility of radiofrequency ablation in the treatment of complex diffuse arteriovenous (AV) malformations. METHODS: The data of 18 patients (8 male and 10 female) with complex AV malformations treated between December 2014 and June 2019 were analyzed retrospectively. The lesion area was 10 × 7 cm ~ 28 × 30 cm. Under duplex ultrasound guidance, the site with the most abundant blood flow signals in the lesion was percutaneously punctured with the radiofrequency ablation needle (electrode). The impedance automatic adjustment mode was adopted, and ablation was monitored usingduplex ultrasoundduring the entire process. RESULTS: Of the included patients, 1 had a high fever after two rounds of treatment, 2 had transient hemoglobinuria, and 1 had tissue necrosis in the original ruptured tumor area as well as a penetrating defect in the cheek, which was repaired with a pedicled trapezius myocutaneous flap. In 9 patients who experienced bleeding, the bleeding stopped after one round of treatment. During the follow-up period of 1-5 years, there were 0 grade I (poor) cases, 0 grade II (medium) cases, 7 grade III (good) cases, and 11 grade IV (excellent) cases. CONCLUSION: The "high power and continuous" radiofrequency ablation technique conducted under real-time duplex ultrasoundmonitoring can completely destroy the deep core lesions of AV malformations and effectively control life-threatening massive hemorrhage; it is an effective alternative treatment method for complex diffuse AV malformations in which interventional embolization, sclerotherapy, and surgery are ineffective.

Standards of care for Kasabach-Merritt phenomenon in China.

Kasabach-Merritt phenomenon (KMP) is a rare disease that is characterized by severe thrombocytopenia and consumptive coagulation dysfunction caused by kaposiform hemangioendothelioma or tufted hemangioma. This condition primarily occurs in infants and young children, usually with acute onset and rapid progression. This review article introduced standardized recommendations for the pathogenesis, clinical manifestation, diagnostic methods and treatment process of KMP in China, which can be used as a reference for clinical practice.

[Clinical analysis of 17 cases of pneumatic compression therapy in infants with Kasabach-Merritt phenomenon].

OBJECTIVE: To summarize the efficacy and safety of employing pneumatic compression therapy in infants with Kasabach-Merritt phenomenon (KMP). METHODS: Seventeen patients with KMP (11 males, 6 females) were treated with pneumatic compression therapy from October 1997 to May 2008. And their clinical characteristics, course of treatment and clinical and laboratory data were retrospectively analyzed. Among 17 patients, 8 cases were located in trunk, 5 in lower extremities and 4 in upper extremities. The diameters of lesions exceeded 8 cm in all patients. The platelet count was all < 100 x 10(9)/L while hemoglobin < 110 g/L and fibrinogen < 2.0 g/L. The self-designed device for pneumatic compression hemangioma therapy was employed (Patent No: ZL97232266. 3). Biopsy and exairesis were performed from the local lesions with KMP in order to determine the pathological features. RESULTS: Two patients were cured after pneumatic compression therapy for 4 and 6 months respectively, and their lesions disappeared, blood parameters became normal and remained relapse-free after a 5/11-year follow-up. Eleven patients were effective after pneumatic compression therapy for 4 - 6 months, and improvement was demonstrated after 6 - 24 months follow-up without any treatment. Two patients showed improvement after pneumatic compression therapy for 6 months after a follow-up for 5 months or 2 years without progression. After a 6-month pneumatic compression therapy, 2 ineffective patients underwent surgical resection. There were 14 cases of kaposiform hemangioendothelioma (KHE) and 3 cases of tufted hemangioma (TA). CONCLUSION: Pneumatic compression therapy has definite curative effects for KMP lesions in extremities and trunk and its side effects are fewer.

[Subsequent therapy for infantile hemangiomas after discontinuation of oral propranolol].

PURPOSE: To summarize the subsequent therapy experiences for infantile hemangiomas after discontinuation of oral propranolol treatment, and explore the relationships between clinical interventions and types of infantile hemangioma. METHODS: In this retrospective study from January 2010 to May 2014, a total of 137 infants with hemangiomas undergoing sequential therapy after oral propranolol treatment. There were 41 males and 96 females. The median age was 16 months, ranging from 14 to 25 months. After oral propranolol treatment, the outcomes were evaluated to be grade III in 74 cases, grade IIin 62 cases and grade Ⅰ in 1 case. The types were papula (n=31), telangiectasis (n=11), plump (n=74), deep (n=12) and compound (n=9). The primary sites were 3 cases in scalp, forty-nine in face, thirty-three in trunk, thirty-eight in extremities. Cutis laxa presented in 45 cases, and parenchyma hypertrophy presented in 80 cases. Sequential therapy were performed including laser therapy for 38 cases, intralesional Pingyangmycin injection for 63 cases, and plastic surgery for 16 cases. The efficacy was re-evaluated on a 4-level scale, combined with evaluations of scar, cutis laxa or pigment alteration. SPSS18.0 software package was used for statistical analysis. RESULTS: Chi-square test showed significant differences between 5 types in occurrence of cutis laxa and parenchyma hypertrophy (x(2)=28.458,68.276, P<0.01). After a follow-up of 6 months to 4 years, the outcomes were evaluated to be grade IV in 122 cases, grade III in 15 cases, without grade IIor gradeⅠ case. There were significant differences in 5 types of infantile hemangiomas before and after sequential therapy( H=53.445, 9.941, 120.324, 17.000, 18.899, P<0.01). Postoperative scar was presented in 2 cases around to joints, and mild pigment alteration was noticed in 2 cases after intralesional Pingyangmycin injection. CONCLUSIONS: Cutis laxa and parenchyma hypertrophy may be more likely present in infantile hemangiomas after oral propranolol treatment. Laser therapy is recommended for patients with papula or telangiectasis, when necessary, intralesional Pingyangmycin injection should be combined. Intralesional Pingyangmycin injection should be the first choice for plump or compound type，whereas surgery should be executed in patients with deep type or other plump type complicated by severe hyperplasia, after which combined intralesional Pingyangmycin injection may be required for postoperative superficial residues.

[Treatment of infantile vascular malformations associated with airway obstruction].

OBJECTIVE: To summarize the clinical features of vascular malformations complicated with airway obstruction and to evaluate the therapeutic methods of these disease. METHODS: Forty-seven children with airway obstruction and dyspnea (25 males, 22 females) were treated from Jun 1985 to Dec 2007, and their clinical data were retrospectively analyzed. Among 47 patients, there were 27 cases of venous malformations, 17 cases of macrocystic lymphatic malformations, and 3 cases of microcystic lymphatic malformations. Injection with absolute alcohol were performed in 20 patients with venous malformations, whereas both surgery and injection were performed in 7 patients with extensive or multiple lesions. Seventeen patients with macrocystic lymphatic malformations were treated with pingyangmycin injection. While surgery combined with pingyangmycin injection were used in other 3 patients with microcystic lymphatic malformations. According to the degree of airway obstruction and therapeutic conditions, tracheal intubation was performed in 27 patients, urgent preoperative tracheotomy was performed in 3 patients, prophylactic tracheotomy was performed in 2 patients, and postoperative tracheotomy was performed in 1 patient. RESULTS: Tracheal intubation was remained for 24 to 48 hours in 30 patients, whose intubation was removed successfully in 29 patients except 1 patient who occurred dyspnea after removal of tracheal intubation resulting in tracheotomy. Tracheal cannula was successfully removed in all 6 patients 3 weeks to 4 months after the tracheotomy. There were 9 patients treated once, whereas injections were repeated 2 to 5 times in 38 patients. Necrosis of mucosa occurred in 2 cases after the injection with absolute alcohol, while temporary hemoglobinuria one occurred in 1. There were 5 cases of light or mediate fever after the pingyangmycin injection who recovered well after the symptomatic treatment. Follow-up lasted 1 to 23 years, 38 patients cured, 9 patients valid, and no patient invalid. CONCLUSIONS: It is suggested that sclerotherapy should be the first choice in the treatment of vascular malformations complicated with airway obstruction, in which absolute alcohol should be used in venous malformations compared to pingyangmycin in lymphatic malformations. Combined therapy should be carried out in patients with extensive lesions in order to shorten the course of treatment and to get good therapeutic result.

[Treatment of infantile parotid hemangioma with propranolol].

OBJECTIVE: To evaluate the short-term results and safety of propranolol for the treatment of infantile parotid hemangioma. METHODS: Oral propranolol was administered to 17 infants with parotid hemangioma at a dose of 1.0-1.5 mg per kilogram of body weight per day. The patients were revisited once a week. The changes of the tumor size, texture and colour were monitored and recorded at a regular interval. The adverse effects after medication were observed and managed accordingly. The short-term results were evaluated using a 4 scales system. RESULTS: Among the 17 patients treated, the follow-up time was 5 to 10 months. The overall response was scale I in 0 patient, scale II in 0 patients, scale III in 5 patients, and scale IV in 12 patients. No serious adverse effects were encountered. CONCLUSIONS: Oral propranolol at a lower dose is a safe and effective method for the treatment of infantile parotid hemangioma. The short-term results were excellent and the side effects minimal.