Changing Faces website for families with facial disfigurement.
Portwine stains are the result of a segmental, absolute, or relative deficiency in autonomic innervation of the post-capillary venules in the papillary dermis. They do not have a proliferative or regressive phase like strawberry naevi. Laser treatment has become the standard of care. The vast majority of portwine stains lighten significantly with treatment, but only 15–20% clear completely. Early treatment is probably better; laser is particularly good for ulceration. Moreover, a recent report confirms that they can recur. Two of the most important considerations are the depth of the vessels and their diameter. Cooling the skin surface with a cryogen spray just prior to the delivery of the laser pulse reduces the likelihood of epidermal damage and pain.
The results of treatment of congenital nevi with Q-switched ruby lasers have been disappointing. All appear to eventually recur.
Facial - Sturge Weber? - Do MRI with contrast
A large capillary naevus can be associated with underlying venous and lymphatic abnormalities, leading to bone/soft tissue hypertrophy, lymphoedema, pain. Weber described associated arteriovenous malformation (AVM), which can then be associated with Kasabach-Merritt syndrome as below (the limb is usually warm). Although congenital, the manifestations are progressive. Sporadic.
Lesions on the abdomen or perineum may be associated with visceral abnormalities so may warrant MR angiography. Potential complications include haematuria, GI haemorrhage.
Management is supportive. The varicose veins are not easily improved by surgery.
Develop within 3 months of birth (occasionally present at birth), proliferate rapidly for about a year, plateau and then start to involute. Treatment is necessary for the following reasons:
Refer to a dermatologist for consideration of laser treatment. Intralesional steroids or oral steroids are occasionally used. By 5yrs 50% have involuted, 70% by 7y, 90% by 9y. Some involute completely without any remnant but the majority leave some stigmata eg telangiectases, a fibrofatty residuum or atrophy.
Distinguish the unwell child from the unwell child.
Multicystic, transilluminable lymphoid mass. Usually presents by age 2 but can be missed if unusual location. Typically in the neck, but can be mediastinal or axillary. Complications are from mass effect, or from infection.
SCFN is a panniculitis that develops in fatty areas during the first weeks of life after foetal distress or perinatal complications. Prognosis is generally good with complete regression, but it can be complicated by metabolic abnormalities like hypoglycemia, hypertriglyceridemia, thrombocytopenia, and also potentially life-threatening hypercalcemia.
Group of inherited conditions in which there is dry, scaly skin (ichthyosis). In severe autosomal recessive forms, the skin does not grow normally in utero and the baby is born encased in a hard case called collodion, often with developmental/deformational abnormalities. In other forms, skin at birth may be normal but collodion rapidly forms, potentially causing respiratory difficulties and temperature instability. As it cracks, the baby is susceptible to superficial infection.
Management is with use of incubator humidity, surveillance swabs, 50:50 white/soft paraffin, Mepitel dressings, sterile handling, monitoring of sodium/fluid balance and weight. Inside out baby grow may reduce skin trauma. Wean humidity over 1-2weeks.
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