Venular (Capillary) Vascular Malformation of Maxillofacial Region: Portwine Stain

Background: Vascular Malformations (VMs) are localized defects of vascular morphogenesis resulting in formation of tortuous enlarged vascular channels. They become clinically visible during early childhood and persist throughout life. Case description: Large reddish-pink macule involving left maxillary facial skin and mucosa from birth which was clinically misdiagnosed as hemangioma. The macule extended from the facial midline and involved the left forehead, bridge of the nose, and the left philtrum. The left half of upper lip was thick and prominent, sagging and redder in color compared to the right half and the lower lip. She gave a history of progressive complete loss of vision from the left eye without restriction of ocular movements. Clinical implications: It is important that the correct nosologies for various VMs are followed, and they should be differentiated from and never wrongly termed as hemangioma. It is important that we adhere to correct clinical diagnosis as the therapeutic guidelines and follow-up of these lesions differ.