This study aims to reduce the misdiagnosis rate of PWSs and improve the diagnosis of CM-AVM using ultrasound. Furthermore, we seek to provide more evidence-based recommendations for the treatment of patients with probable CM-AVM syndrome, particularly those presenting with erythema.
Background: Exacerbation of erythema following photodynamic therapy (PDT) in patients with probable capillary malformation-arteriovenous malformation (CM-AVM) syndrome, may be attributed to misdiagnosis as port-wine stains (PWSs). Objective: To identify the ultrasound features of patients with exacerbation of erythema following PDT for a definite diagnosis. Methods: A retrospective single-center study was conducted on PWS patients with exacerbation of erythema following PDT. The clinical and ultrasonic features were reviewed and analysed. One low-frequency and two high-frequency ultrasound (HFUS) devices were used to examine the lesions and the contralateral normal skin. Results: The clinical characteristics of 16 patients were consistent with those of high-flow vascular stains (HFVS). Ultrasound findings revealed skin and hypodermis thickening, increased blood flow signals with an arteriovenous malformation (AVM) waveform and deeper, larger blood vessels in the dermis and hypodermis of pink macules (PMs) compared to normal skin. In the absence of genetic diagnosis, PWS patients with exacerbation of erythema following PDT were diagnosed as having probable CM-AVM syndrome. Limitations: Retrospective single-center design and small sample size. Conclusion: Patients with PWSs are advised to undergo low-frequency ultrasound examinations to avoid misdiagnosis. HFUS can serve as a complementary method to promptly identify underlying AVMs beneath the PMs. We do not recommend PDT treatment for patients with probable CM-AVM syndrome.
Study Type
OBSERVATIONAL
Enrollment
16
Prior to treatment, patients or their guardians provided written informed consent. Then patients were then prepared for the procedure by fully exposing the treatment area and shielding the surrounding normal skin with a black cloth. Hemoporfin (Shanghai Fudan-Zhangjiang Bio-Pharmaceutical) was administrated intravenously at a dosage of 5.0 mg/kg. Subsequently, patients underwent light radiation at 532 nm (Wuhan Yage Optic and Electronic Technique, Wuhan, China) for 18 to 25 minutes, with a power density randing form 75 to 110 mW/cm2.
Ultrasound examinations were performed using three types of devices: a Paragon XHD equipped with a L38-22 probe operating at a frequency of 20-30 MHz (Kolo Medical Co., Ltd, Suzhou, China), a DUB Skin Scanner with a 22 MHz probe (Taberna Pro Medicum, German), or a Mindray Resona 7 featuring an L14-5WU linear probe set to a frequency range of 11-12 MHz frequency (Mindray Bio-Medical Electronics Co., Ltd, Shenzhen, China).
The Second Affiliated Hospital of Xi'an Jiaotong University
Xi'an, Shaanxi, China
ultrasound characteristics
After cleaning and disinfecting the mesured area, the probe, coated with 1-2 millimeters of coupling agent, was positioned vertically on the skin surface. Measurements of skin thickness, density, vessel diameter, and depth were obtained for both the lesions and the contralateral normal skin. The color Doppler (CD) mode of high-frequency ultrasound was employed to assess local blood perfusion in both lesions and normal skin, while the pulsed-wave (PW) Doppler mode was used to detect the blood flow spectrum. The average of three measurements at the same site was calculated.
Time frame: The efficacy evaluation will be conducted 3 months after the completion of the 1st HMME-PDT treatment.
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