Approach to clinically significant vascular anomalies in children

Vascular anomalies consist of tumours or malformations made up of abnormal growth or collections of blood vessels that can result in functional or cosmetic problems. While many vascular anomalies are present at birth, some do not appear until later in life, making diagnosis more challenging. Although many vascular anomalies are benign, some are associated with serious complications and may involve multiple organ systems. This article highlights the important features of clinically significant vascular anomalies to help physicians promptly identify and refer these cases to a specialised multidisciplinary team for evaluation and management. The discussion includes the various presenting complaints of vascular anomalies in children, namely, rapidly growing birthmarks, painful lesions, seizures/neurological manifestations, bleeding diathesis, cardiac/airway abnormalities and part of an overgrowth syndrome.

Classification of Vascular Anomalies Using Clinico- Radiological Parameters – Is It Sufficient?

To classify vascular anomalies using clinico-radiological parameters. Settings: Government Medical College Srinagar and Army Hospital Research and Referral New Delhi. Methods: 129 cases of vascular anomalies were studied and classified using clinical and radiological methods from March 2011 to June 2018. Outcome: Vascular anomalies were broadly classified into vascular tumours and vascular malformations and these anomalies were further sub-classified based on clinical and radiological parameters. Results: Among 129 vascular anomalies studied there were 51 vascular tumours which included 50 hemangiomas (H) and 1 case of pyogenic granuloma (PG). Among Hemangiomas there were 34 Hemangiomas of Infancy (HOI) and 16 Congenital Hemangiomas (CH). Among HOI there were 26 Simple (Localized-17, Segmental-2, Indeterminate-6, Multifocal-1), 8 complex visceral hemangiomas (Liver-2, Subglottic-2, Parotid-4). Among Congenital Hemangiomas there were 4 Rapidly Involuting Congenital Hemangiomas (RICH) and 12 Non involuting congenital hemangiomas (NICH). There were 78 vascular malformations which included 51 slow flow malformations and 27 fast flow malformations based on colour doppler studies. Among slow flow there were 42 Simple {2 Capillary Malformations, 26 Venous Malformations (VM), 14 Lymphatic Malformations (LM), 4 Combined (2 each of Lymphohemangiomas - LH and Lymphovenous Malformations – LVM} and 5 Complex-combined (4 Klipple Trenaunay Syndrome – KTS and 1 case of Blue Rubber Bleb Nevus Syndrome - BRBNS). Among fast flow there were 6 simple (Nasopharyngeal Angiofibromas), 20 combined (Arteriovenous Malformations - AVM) and 1 Complex combined malformation (Sturge Weber Syndrome - SWS). Conclusion: Newborn with birthmark should always be documented by the attending health care provider at birth and referral to an expert for proper evaluation and careful parental/Guardian counseling. Doppler US should be the first line of investigation in broadly classifying vascular anomalies whereas CT Scan, MRI/MRA/MRV, Angiography, Venography help in further sub-classification. Some confusion still persists in classifying few lesions like Lympho-hemangioma (LH), Complex combined Malformations (Syndromes) like Sturge Weber Syndrome (SWS), Blue Rubber Bleb Nevus Syndrome (BRBNS), and Nasopharyngeal Angiofibroma (NPA).

Cutaneous tumours of vascular origin: two rare entities

Skin and subcutaneous tissue are the most common location of benign and malignant vascular tumors. The benign or malignant nature of the lesion may not always be identified clinically because of the varying presentations. The histology of the lesion, especially in malignancies may not exhibit definite vascular pattern.  In such cases, clinical presentation has to be correlated with histopathological picture as well as immunohistochemical characteristics to reach a specific diagnosis. Judicious use of immunohistochemical markers proves to be invaluable.  Two rare cases of cutaneous vascular tumors which presented at our institution are cited, the diagnosis of which involved correlation of these modalities.