Sir: Venous malformations are common congenital anomalies of vascular morphogenesis characterized by slow progression.1 However, in a very small group of patients, the lesions could expand dramatically in a rather short time, which is caused by unusual intralesional hemorrhage. According to the data from our vascular anomalies center, it appeared in approximately 3 percent in all venous malformations patients. In this article, we describe the clinical manifestations, diagnosis, and other aspects of this phenomenon. Sixteen patients were treated over the past 4 years. Almost all lesions occurred in male patients (n = 15) aged younger than 20 years (n = 14) and were located in the head and neck region (n = 15), including the cheek, upper lid, parotid region, and temple. Only a small number of patients had a history of trauma (n = 6) or signs of venous malformation (n = 3) before the onset of hemorrhage. Some lesions exhibited remarkable enlargement even overnight, whereas others kept expanding over 2 to 25 days. No pain, burn sensation, or other special symptoms were reported by any of the patients. On physical examination, there were localized and swollen masses under normal or bluish skin. No pulsation or thrill could be detected on palpation (Fig. 1). Dark red, thick, stale blood could be aspirated easily by puncture (Fig. 2). Magnetic resonance imaging and direct injection venography are the most accurate imaging modalities in diagnosis of these lesions.2 Magnetic resonance imaging revealed irregular shape, intermediate or slightly high signal intensity on T1-weighted sequences, and inhomogeneous high signal intensity on T2-weighted sequences. Intensity change was shown on contrast-enhanced T1-weighted sequences. Magnetic resonance imaging was also used for therapeutic effect evaluation and follow-up. Direct injection venography was performed in two patients. Homogenous contrast-enhanced masses and draining veins were found. No biopsy was performed because of unfeasible surgical resection and cosmetic concerns.Fig. 1.: A large, bossed mass on the neck of a 16-year-old boy. The mass enlarged abruptly overnight after intense parallel bars exercises. He had never been diagnosed with venous malformations in this region before the onset of hemorrhage.Fig. 2.: Dark red blood was aspirated from the mass on the left cheek of a 3-year-old boy.Our diagnosis was also confirmed by the response to embolosclerotherapy and long-term follow-up results. Fourteen patients were treated successfully using percutaneous intralesional injection of absolute ethanol and bleomycin A5. In some cases, residual masses could still be detected by magnetic resonance imaging several months after the first procedure. Dark red blood could still be aspirated at this time. In addition, an 18-month-old child with a lesion involving his left upper lid and temple was not treated. Four years later, a soft, compressible, posture-related mass was evident at the original site. These lesions should be differentiated from other masses in the head and neck region, such as cystic lymphangiomas, branchial cleft cyst, and parotid cyst. The exact mechanism of hemorrhage in venous malformations is not clear. It might be associated with abnormal and vulnerable vessels, insufficient peripheral drainage,3 or altered hemodynamics stimulated by trauma or other unknown triggering factors. The head and neck region is the most affected. The possible reason is the dense vascular network of this region, the frequent temporomandibular joint movements, and the loose surrounding tissues. In summary, although intralesional hemorrhage in venous malformations is uncommon, it is not difficult to deal with if correct diagnosis can be established. The medical history, physical examination, diagnostic puncture, and magnetic resonance imaging are essential for achieving this goal. Hui Chen, M.D. Xiaoxi Lin, M.D. Yunbo Jin, M.D. Wei Li, M.D. Gang Ma, M.D. Department of Plastic and Reconstructive Surgery Shanghai Ninth People’s Hospital Shanghai Jiaotong University Medical School Shanghai, China DISCLOSURE None of the authors has a financial interest to disclose in relation to the content of this article.
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Nov 1, 2014
Weimin Shen + 4
Open Access