Paediatrics: Blood vessel disorders

Blood vessel disorders

Telangiectasia

Telangiectasias are permanently dilated small vessels. Commonest are spi-der naevi (dilated capillaries radiating from central arteriole). Less than 5 are considered normal. Laser or cautery of central vessel is rarely re-quired. Five or more telangiectasias may be part of:

·  Hereditary haemorrhagic telangiectasia (autosomal dominant genetic disorder with telangiectasia on lip, tongue, nasal epithelium, risking recurrent epistaxis +/– GI haemorrhage).

·  Ataxia telangiectasia.

·  Hereditary benign telangiectasia.

Vascular malformations

Salmon patches 

Occur on nape of neck, glabella, eyelids and other sites.

Often resolve or improve with age.

Port wine stain

Naevus flammeus. A capillary vascular malformation evi-dent at birth, which persists with age. Vivid red or purple macule. May af-fect any site, but face and neck commonest. Involvement of the eyelid may be associated with glaucoma; segmental ophthalmic branch of trigeminal nerve involvement is associated with a risk of Sturge–Weber syndrome (seizures, hemiplegia, mental retardation). Treatment is pulse dye laser.

Klippel–Trenaunay syndrome

A complex venous-lymphatic malformation of the limb associated with limb hypertrophy and varicose veins. Patients may be at increased risk of DVT/pulmonary embolism (PE). Treatment is compression stockings. Lymphatic leakage may be treated with pulse dye laser or CO₂ laser.

Haemangiomas

·  There are several types, but the most common by far is the infantile haemangioma (strawberry naevus).

·  These occur in 10% of infants. They often present in the first few weeks of life and are more common in females.

·  May have a superficial (red colour) and deep (blue colour) component.

·  They often undergo a rapid proliferative phase between 4–9mths of age and then slowly involute over years. Complications include ulceration, bleeding and infection.

·  If segmental over the face and perineum, they may be associated with PHACES (posterior fossa abnormalities, haemangioma, arterial anomalies, cardiac anomalies, eye abnormalities, sternal cleft or supra-umbilical raphe) and PELVIS (perineal haemangioma, external genital anomalies, lipomyelomeningocele, vesicorenal abnormalities, imperforate anus and skin tags) syndromes respectively.

Those in the beard area may cause airway obstruction and those in the lumbar midline may be associated with spinal dysraphism. Multiple cutaneous haemangiomas may be associated with internal organ haemangiomas (especially liver and brain).

·Infantile haemangiomas have no risk of Kassabach-Merritt Syndrome (comprises thrombocytopaenia and a consumptive coagulopathy; only occurs in tufted angiomas and kaposiform haemangioendotheliomas).

Treatment

Reassurance and monitoring in most cases is all that is required. However, those in critical or cosmeticially sensitive sites may now be treated with oral propranolol by a dermatologist. Pulse dye laser is a useful treatment for ulceration and residual telangiectasia after involution. Surgical correc-tion may be required to remove the fibrofatty residual after involution of large haemangiomas. Segmental, midline and multiple haemangiomas may need further investigation.

Perniosis (chilblains)

Abnormal reaction to cold with localized, inflammatory, red-blue lesions on extremities (e.g. digits, ears). On rewarming there is pain or itching. Lesions may ulcerate. Resolves spontaneously. Prevented by warm cloth-ing and housing!

Raynaud’s syndrome

Episodic artery spasm causes digital ischaemia. The condition is precipi-tated by cold, finger constriction (e.g. shopping bags), or emotion. Most cases improve with age. Syndrome may be idiopathic (Raynaud’s disease) or s (Raynaud’s phenomenon) to:

·Systemic sclerosis.

·Arterial occlusion (e.g. cervical rib).

·Occlusive arterial disease.

Presentation

Fingers ache, burn, or tingle with colour changes of pallor (ischaemia), blue (cyanosis), and red (reactive hyperaemia).

Treatment

·Treat underlying disease.

·Local warmth.

·Nifedipine.

Consider sympathethectomy if severe or recurrent.