Al-Qattan Mohammad M
Department of Surgery, King Saud University, Riyadh, Saudi Arabia.
Plast Reconstr Surg Glob Open. 2021 Jan 26;9(1):e3391. doi: 10.1097/GOX.0000000000003391. eCollection 2021 Jan.
Venous (cavernous) malformations are commonly seen in the upper limb. There is no consensus in the literature regarding the classification of venous malformations. Patients may be viewed as 2 clinical entities: patients with single or multiple lesions. Single venous malformations are sporadic and nonsyndromic, whereas the presence of multiple malformations indicates the presence of either an inherited or an overgrowth (noninherited) disorder. In this article, the author reviews multiple venous malformations of the upper limb, offers a novel classification, and describes their clinical entities along with their genetics and pathogenesis. These clinical entities will also be described by categorizing the cases as per the clinical presentation. Furthermore, the number of cases seen by the author (during an experience of 28 years of practice in Saudi Arabia) in each category will be reviewed to give the reader an overall view of the frequency of presentation of each category to the hand/plastic surgery clinic. Clinically, patients may present in 4 different presentations depending on the distribution of the lesions: the late-onset malformations confined to the upper limb; malformations involving the limbs/face/trunk with no mucosal lesions; widespread malformations of the skin, oral mucosa, and the intestine; and venous malformations presenting as a well-known syndrome. The author has seen a total of 84 patients, and the most 2 common presentations were late-onset type (n = 26) and malformations involving the limbs/face/trunk with no mucosal lesions (n = 36). This is the most comprehensive review of multiple venous malformations of the upper limb.
静脉（海绵状）畸形常见于上肢。关于静脉畸形的分类，文献中尚无共识。患者可被视为两种临床类型：单发或多发病变患者。单发静脉畸形是散发性的且无综合征表现，而多发畸形的存在表明存在遗传性或过度生长（非遗传性）疾病。在本文中，作者回顾了上肢的多发静脉畸形，提出了一种新的分类方法，并描述了它们的临床类型、遗传学和发病机制。这些临床类型也将根据临床表现对病例进行分类来描述。此外，作者（在沙特阿拉伯28年的临床经验中）将回顾每类中所见病例的数量，以便让读者全面了解每类病例在手外科/整形外科诊所的出现频率。临床上，根据病变分布，患者可能有4种不同表现：局限于上肢的迟发性畸形；累及四肢/面部/躯干但无黏膜病变的畸形；皮肤、口腔黏膜和肠道的广泛畸形；以及表现为一种知名综合征的静脉畸形。作者共诊治了84例患者，最常见的两种表现是迟发性类型（n = 26）和累及四肢/面部/躯干但无黏膜病变的畸形（n = 36）。这是对上肢多发静脉畸形最全面的综述。
