Easy Pediatrics

Raynaud Phenomenon (RP) refers to a transient vasospasm of peripheral arteries and arterioles that classically results in triphasic color changes in the affected region. The initial artery and arteriole vasospasm causes pallor (white), followed by cyanosis (blue) due to dilation of the capillaries and venous stasis (deoxygenated blood), with continued artery and arteriole vasospasm. The arteries and arterioles then dilate, causing rapid return of blood flow (red, reactive hyperemia). The fingers are the most commonly affected region, and Raynaud phenomenon is typically triggered by cold exposure or stress.

Raynaud phenomenon can be primary (idiopathic), meaning no associated diseases are present, or secondary, meaning that another condition is believed to be the cause of the Raynaud phenomenon. Connective tissue diseases are the most common cause of secondary Raynaud phenomenon, but several medications and many other conditions are also associated with Raynaud phenomenon.

Pathophysiology

The pathophysiology of Raynaud phenomenon is not completely understood; the vasospasm that occurs in the digital arteries, precapillary arterioles, and cutaneous arteriovenous shunts involves both central and peripheral mechanisms. The pathophysiology of secondary Raynaud phenomenon also involves structural changes to the vasculature that worsens the severity of the Raynaud phenomenon. Raynaud phenomenon can be thought of as an exaggerated normal response to cold and emotional stress.

Clinical Presentation

History form the patient

Patient history should include affected sites, frequency and severity of attacks, duration of attacks, color pattern, triggers, seasonality, and associated symptoms (ie, numbness, paresthesia, pain). The most commonly affected sites are the fingers, toes, ears, nose, and, rarely, nipples.The triggers of Raynaud phenomenon in children are similar to those described in adults. Cold and emotional stresses are the most common triggers; primary Raynaud phenomenon can also be triggered by exercise.Episodes are more common in the winter than in the summer, and serious ischemia is also more common in the winter.

Patients and their parents should be queried about changes in digits such as pits, ulcers, or poor healing, and about the presence of infection in affected digits. They should also be asked about possible associated or precipitating factors including frostbite, drug or toxin exposure, infection, vibration injury, family history of Raynaud phenomenon, family history of connective tissue diseases, history of migraine,weight loss or eating disorders, and cardiovascular diseases.

Patients should also be questioned for any history suggestive of connective tissue disease such as fever, fatigue, rash (malar, vasculitic, dermatomyositis), morning stiffness, Continue reading »