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HomePediatrics: SurgerySurgery for Congenital Arterial, Venous, and Lymphatic Anomalies

Surgery for Congenital Arterial, Venous, and Lymphatic Anomalies


Embryologic studies by Woodward et al at the turn of the 20th century shed light on the understanding of vascular congenital anomalies. These anomalies are encountered infrequently in everyday practice. They represent a heterogeneous group of isolated or multiple abnormalities that are sometimes associated with complex congenital syndromes. Most vascular anomalies affect the skin, though any organ system can be involved. Nearly all cutaneous congenital vascular abnormalities are evident either at birth or within the first few weeks of life.

The presence of such lesions at birth and early childhood invokes concern and fear in parents and, in some cases, starts a protracted process of multiple visits to various specialists. Thus, it is mandatory to take the time and diagnose lesions appropriately early on and to ensure that a multidisciplinary team approach be used if the disease process warrants. The first step toward this goal is to obtain a careful history and physical examination, as these can distinguish between vascular tumors and malformations with a diagnostic accuracy exceeding 90%.

A great deal of confusion surrounds the nomenclature and classification of congenital vascular abnormalities, and as a result, prompt proper diagnosis and appropriate treatment for patients are often lacking. Alarmingly, as reported in one study, as many as one half of patients referred to specialty clinics for vascular abnormalities were diagnosed and monitored incorrectly.

Accordingly, as put forth by Mulliken et al,
an appropriate start to any discussion of congenital vascular abnormalities should include the distinction between vascular tumors (eg, hemangiomas) and vascular malformations (eg, capillary or lymphatic); the two entities are decidedly different (see Pathophysiology).

The topic of vascular anomalies is quite broad. This article serves as a superficial review covering the major tumors and malformations that the general surgeon or practitioner may encounter. To minimize confusing nomenclature and to organize the discussion of the topic, this article adheres to the Mulliken-Glowacki schema.

Finally, it should be mentioned that many vascular anomalies are found in association with syndromes. A full discussion of such syndromes is outside the scope of this article, but a brief litany of predominant syndromes is mentioned in later sections.

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