Venous malformations of the lower limb with severe localized intravascular coagulopathy treated with radiofrequency ablation and resection.

Diffuse venous malformations (VMs) are relatively rare, especially the lesions locting special anatomical sites, and they are prone to casuse localized intravascular coagulopathy (LIC). Diffuse VMs can also cause bleeding and life-threatening disseminated intravascular coagulopathy (DIC) from trauma, surgery, and improper treatments. Thus, the treatment of diffuse VMs with LIC is quite tough. We report of a diffuse VMs with severe LIC that was treated with the combined use of minimally invasive treatment and open surgery.

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.

Rare Germline GLMN Variants Identified from Blue Rubber Bleb Nevus Syndrome Might Impact mTOR Signaling.

BACKGROUND AND OBJECTIVE: Blue rubber bleb nevus syndrome (BRBN) or Bean syndrome is a rare Venous Malformation (VM)-associated disorder, which mostly affects the skin and gastrointestinal tract in early childhood. Somatic mutations in TEK have been identified from BRBN patients; however, the etiology of TEK mutation-negative patients of BRBN need further investigation. METHODS: Two unrelated sporadic BRBNs and one sporadic VM were firstly screened for any rare nonsilent mutation in TEK by Sanger sequencing and subsequently applied to whole-exome sequencing to identify underlying disease causative variants. Overexpression assay and immunoblotting were used to evaluate the functional effect of the candidate disease causative variants. RESULTS: In the VM case, we identified the known causative somatic mutation in the TEK gene c.2740C>T (p.Leu914Phe). In the BRBN patients, we identified two rare germline variants in GLMN gene c.761C>G (p.Pro254Arg) and c.1630G>T(p.Glu544*). The GLMN-P254R-expressing and GLMN-E544X-expressing HUVECs exhibited increased phosphorylation of mTOR-Ser-2448 in comparison with GLMN-WTexpressing HUVECs in vitro. CONCLUSION: Our results demonstrated that rare germline variants in GLMN might contribute to the pathogenesis of BRBN. Moreover, abnormal mTOR signaling might be the pathogenesis mechanism underlying the dysfunction of GLMN protein.

[Chinese experts consensus on the use of oral propranolol for treatment of infantile hemangiomas].

Infantile hemangioma (IH) is one of the most common benign vascular tumors in children. A variety of treatment methods have been documented for the management of IH over the past years, including pharmacotherapy via oral administration or injection of corticosteroids, vincristine, alpha interferon and bleomycin; laser therapy, radionuclide therapy, cryotherapy and excisional surgery. The therapeutic efficacy of each treatment modality is variable, while adverse effects or complications are common and sometimes serious. Since the serendipitous discovery of propranolol, a nonselective beta-adrenergic receptor blocker, being very efficacious in treating IH in 2008, oral propranolol has earned a role as a first-line medical therapy for complicated IH. However, the appropriate drug dosage, dosing regimen, time for initiation, optimal duration, monitoring for side effects remains controversial. To standardize the use of propranolol in treating IH, avoid overtreatment or under-treatment, as well as minimize complications, a Chinese experts consensus on the use of oral propranolol for treatment of IH has been approved and written by a multidisciplinary experts group based on an up-to-date literature review and repeated discussion.

[Chinese expert consensus on the use of topical timolol maleate treatment of infantile hemangiomas].

Non-selective ß-blocker propranolol has been proved by FDA as the first-line agent for infantile hemangioma (IH) with dramatic response. To reduce the side effects caused by systemic administration of propranolol, timolol maleate treatment has been increasingly used as an alternative to systemic ß-blockers and watchful waiting for many IH patients in recent years. However, the appropriate indications, drug dosage, dosing regimen, time for initiation, optimal duration, monitoring for side effects still remains controversial. To standardize the use of topical timolol in treating IH, avoid overtreatment or under-treatment, as well as minimize complications, a Chinese expert consensus on the use of topical timolol treatment of IH has been approved and written by a multidisciplinary experts group based on an up-to-date literature review and repeated discussion, which can be used to reduce inappropriate variations in clinical practice and to promote the delivery of high quality, evidence-based health care for IH patients.

[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.

Clinical observations in mesh suture treatment for infants of Kasabach-Merritt phenomenon.

AIM: This study aims to evaluate the efficacy and adverse effects of the mesh suture treatment for infants of Kasabach-Merritt phenomenon and to report our treatment experience. METHODS: Of the three patients, two of the cases occurred in the scalp and one occurred in the back of the chest, with platelet counts < 40 × 109 /L before the treatment, reduced fibrinogen levels and increased D-dimer levels. All the three patients underwent the mesh suture treatment of the tumour area. Post-treatment observations were made regarding the surface colour and texture of the tumours, periodic routine blood examination results, fibrinogen and D-dimer levels. RESULTS: After treatment, the degree of swelling on the tumour surface was reduced, surface tension was decreased, the tumour colour turned pale red from dark red and the skin gradually returned to normal. Two days after treatment, the platelet counts increased to 70 × 109 /L or higher; the platelet count reached a normal level after 1 week in two cases. The fibrinogen level increased in varying degrees after treatment, whereas the D-dimer level was reduced. One case showed a small amount of exudate at the suture area, 1 month after the treatment; improvements were observed 3 days after changing the local dressing. All the patients were followed up for 6 months to 1 year, during which the patients showed complete regression of the lesion and relapse, normal platelet counts and normal fibrinogen and D-dimer levels. CONCLUSIONS: We recommend the mesh suture treatment as the first treatment of choice for paediatric Kasabach-Merritt phenomenon.

[Clinical analysis for treatment of 1 080 cases of infantile hemangiomas with oral propranolol].

OBJECTIVE: To explore the indications of hemangiomas of different types by observing the clinical efficacy of oral propranolol. METHODS: For this retrospective study from October 2009 to June 2013, a total of 1 080 cases were classified into 5 types according to their clinical characteristics. There were 338 males and 742 females. Their types were telangiectasis (n = 58), papular (n = 424), plump (n = 106), deep (n = 176) and mixed (n = 306). Propranolol was orally administered at a dose of 1.0 or 1.5 mg/kg daily (1.0 mg/kg for infants aged 2.5 months or under; 1.5 mg/kg for those aged 2.5 months or above). Dynamic observations of hemangioma size, texture or color change and adverse events during treatment were performed. Drug withdrawal was usually made after dosing for 1 year or under when there was a total regression of hemandiomas. The efficacy was evaluated on a 4-level scale. RESULTS: Rank test results showed no significant differences between 5 types during the changes of lesions (χ² = 1.738, P > 0.05). Changing of lesions occurred 8.0 (3.7, 16.2) hours after dosing in telangiectasis type, whereas 6.5 (4.1, 14.3) hours in papular type, 7.0 (5.5, 12.7) hours in plump type, 7.5 (3.8, 11.3) hours in deep type and 6.5 (4.2, 13.4) hours in mixed type. After a follow-up of 6 months to 2.5 years, there were 378 grade IV case (35.00%), 574 grade III case (53.15%), 120 grade II cases (11.11%) and 8 grade I cases (0.74%). And there were significant differences between patients of different types (P < 0.05). Patients of deep type had the best grade IV curative effect rate of 59.09% (104/176) , then papular type of 51.25% (162/316) and telangiectasis type of 26.41% (112/424). CONCLUSION: As a first-line treatment for hemangioma, propranolol shows excellent efficacies for patients of deep, mixed, plaque and papular types.

Propranolol inhibits angiogenesis via down-regulating the expression of vascular endothelial growth factor in hemangioma derived stem cell.

BACKGROUND: Oral propranolol (PRN) has recently been shown to be highly effective for infantile hemangiomas (IHs), and is currently recommended as the first-line treatment of complicated IHs. However, the therapeutic mechanism(s) still remain unclear. METHODS: In this study, we tested hemangioma-derived stem cells for expression of vascular endothelial growth factor (VEGF) in vitro and studied the inhibition of VEGF expression. We used PCR, Elisa, Western blotting and immunohistochemistry in vivo and in vitro trial. RESULTS: The study demonstrated that application of PRN at a "normal" concentration equivalent to plasma concentration did not inhibit proliferation or promote apoptosis of hemangioma derived stem cells (HemSCs) isolated from IH patients. PRN suppressed expression of vascular endothelial growth factor (VEGF) and basic Fibroblast Growth Factor (bFGF) in HemSCs in vitro. Morphological, histological and immunohistological improvement were observed in vivo using murine IH model in which HemSCs pre-treated with PRN were implanted into BALB/c-nu mice. In the pre-treated HemSC grafts, mean micro-vessel density (MVD) significantly decreased and protein levels of VEGF markedly decreased, while bFGF was still detectable. CONCLUSIONS: The results suggested PRN inhibited angiogenesis via down-regulating the expression of vascular endothelial growth factor in hemangioma derived stem cell. These findings provide critical insight into the potential mechanisms of PRN action on IH.

Preliminary study on plasma RPN concentration of patients with infantile hemangioma treated with propranolol.

Propranolol (PRN) has recently been recommended as the first-line medicine for complicated infantile hemangiomas (IHs), because of the significant effect. However, no pharmacokinetic parameters have ever been reported for infants who receive PRN treatment for IH. In this study, we show that plasma PRN concentration is affected by the frequency of administration of PRN. A single daily administration of PRN (1 mg/kg/d) resulted in an early elevation of plasma PRN compared to a twice a day administration of the same dose. In contrast, the twice a day application resulted in a more prolonged expression at a later time-point. Our findings provide pharmacokinetic parameters of PRN action in IH for clinic.

CD133 selected stem cells from proliferating infantile hemangioma and establishment of an in vivo mice model of hemangioma.

BACKGROUND: Infantile hemangioma (IH) is the most common benign tumor in children with prevalence in the face and neck. Various treatment options including oral propranolol have been described for IH, but the mechanism of drugs remains enigmatic. The aim of this study was to investigate the pathogenesis and establish a reliable in vivo model of IH which can provide platform for drug exploration. METHODS: Stem cells from the proliferating hemangiomas (HemSCs) were isolated by CD133-tagged immunomagnetic beads. Their phenotype and angiogenic property were investigated by flow cytometry, culturing on Matrigel, real-time polymerase chain reaction (PCR), immunofluorescent staining and injection into BALB/c-nu mice. RESULTS: HemSCs had robust ability of proliferating and cloning. The time of cells doubling in proliferative phase was 16 hours. Flow cytometry showed that HemSCs expressed mesenchymal markers CD29, CD44, but not endothelial/hematopoietic marker of CD34 and hematopoietic marker CD45. The expression of CD105 was much lower than that of the reported hemangioma derived or normal mesenchymal stem cell (MSC). Real-time PCR showed that the mRNA levels of vascular endothelial growth factor (VEGF), basic fibroblast growth factor (bFGF) and matrix metalloproteinase-1 (MMP-1) of HemSCs were higher than that of neonatal human dermal fibroblasts (NHDFs) and human umbilical vein endothelial cells (HUVECs). After HemSCs were cultured on Matrigel in vitro, they formed tube-like structure in a short time (16 hours) and differentiated into endothelial cells in 7 days. After 1 - 2 weeks of implantation into immunodeficient mice, HemSCs generated glucose transporter 1 positive blood vessels. When co-injected with HUVECs, the vascularization of HemSCs was greatly enhanced. However, the single implantation of HUVECs hardly formed blood vessels in BALB/c-nu mice (P < 0.05). CONCLUSIONS: HemSCs may be some kinds of primitive mesoderm derived stem cells with powerful angiogenic ability, which can recapitulate human hemangioma by co-injecting into immunodeficient mice with HUVECs.

Detection of RASA1 mutations in patients with sporadic Sturge-Weber syndrome.

OBJECTIVE: The aim of this study was to identify RASA1 mutation in Chinese population with sporadic Sturge-Weber syndrome (SWS). METHODS: Genomic DNA was obtained from peripheral blood of nine patients with sporadic SWS. The 25 exons, promoter regions (-1,000 bp) as well as intron-exon boundaries of RASA1 were amplified by polymerase chain reaction, and products were sequenced directly. RESULTS: A novel synonymous mutation (c.1229 G > A [p.K420K]) of RASA1 was identified in the present series. CONCLUSION: It implied that RASA1 may be not a virulence gene, but further study is needed to know RASA1 gene mutation in SWS patients.

[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.

[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.

[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.

[Treatment of infantile hemangiomas with low-dose propranolol: evaluation of short-term efficacy and safety].

OBJECTIVE: To evaluate the short-term efficacy and safety of propranolol in the treatment of infantile hemangiomas. METHODS: Between October 2008 and May 2009, oral propranolol was applied to 58 infants with hemangiomas at a dose of 1.0 - 1.5 mg per kilogram of body weight per day in Linyi, Shandong and Shanghai. There were 19 males and 39 females 1 to 12 months old with a mean age of 4 months. The primary tumor size was 1.5 cm x 1.0 cm to 18.0 cm x 5.0 cm. Twenty-seven were superficial, nine deep-seated and 22 mixed. The tumors were located in head and neck (n = 41), trunk and extremities (n = 12), labium vulvae (n = 2), perianal region (n = 1), perineum (n = 1) and scrotum (n = 1). The patients were hospitalized for 7 to 10 days, continued medication at home and revisited every 2 weeks. The changes of tumor size, texture and color 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-point scale system. RESULTS: At 24 hours post-medication, all the tumors decreased in density, color and size. The changes became conspicuous within 5 to 7 days. Seven patients had medication for 2 months, 22 for 3 months, 21 for 4 months and 8 for 5 months. The follow-up period was 5 to 9 months. The overall response was scale I (poor) in 1 patient (1.7%), scale II (moderate) in 12 patients (20.7%), scale III (good) in 35 patients (60.4%) and scale IV (excellent) in 10 patients (17.2%). Statistical analysis showed that the treatment response for deep-seated hemangiomas was significantly better than that for superficial hemangiomas (P < 0.05), but no significant difference was found among different primary sites (P > 0.05). The main adverse effects were bradycardia (100%), diarrhea (63.8%) and sleep change (30.2%), which resolved after expectant treatment without any significant sequel. No serious adverse effect was observed. CONCLUSIONS: Oral propranolol treatment at a low dose is a safe and effective regimen for infantile proliferating hemangiomas. And it can be used as the first-line therapeutic modality. The short-term efficacy is excellent while the side effects are minimal.

Photodynamic therapy of port wine stains-a report of 238 cases.

OBJECTIVE: Retrospective analysis of clinical effects of vascular acting photodynamic therapy (PDT) for the treatment of port wine stains (PWS). METHODS: Between September 1997 and June 2003, a total of 238 PWS cases (2-56 years old) were treated with Photocarcinorin-mediated PDT using a copper vapour laser. Among them, 20 cases were pink lesions (Type I), 44 cases red lesions (Type II), 99 cases dark red lesions (Type III), 51 cases purple lesions (Type IV), and 24 cases nodular or thickened lesions (Type V), respectively. Patient received a slow intravenous injection of Photocarcinorin (4-5mg/kg b.w.) and light was delivered during the drug injection at dose levels of 160-260J/cm(2) at fluence rates of 70-100mW/cm(2). The same procedure was repeated 2-4 times for some patients. All patients were followed up for 6 months to 4 years. RESULTS: Sixty-eight cases (28.6%) showed excellent response, 76 cases (31.9%) good response, 87 cases (36.6%) fair response and 7 cases (2.9%) poor or no response. Secondary scar formation was reported in three cases. Highest good to excellent response rates were seen in patients of 5-20 years old. PDT-induced transitional hyperpigmentation was reported in some patients but disappeared without the need of treatment within 3-6 months. CONCLUSION: Copper vapour laser PDT can selectively destroy PWS vessels without damage to the normal skin. If the technique is applied properly, it can cure superficial lesions and greatly improve thick lesions.