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Capillary Malformations

1. Essentials of Diagnosis

• Present at birth
• Distribution remains constant, although the color may darken
• Overlying skin is unaffected in childhood but may change in adulthood with the development of a nodular skin surface and ectatic dermal vessels
• Capillary malformations must be differentiated from commonplace fading macular stains of infancy (neveus flammeus neonatorum), which are referred to as stork bites, angel kisses, or salmon patches
• Lesions roughly follow cutaneous sensory nerve distributions

2. General Considerations
     Capillary malformations are the most common of the vascular malformations and occur in 0.3% of newborns. These lesions, which are also known as nevus flammeus, or port wine stains. Magnetic resonance imaging with contrast is the most useful of all radiologic modalities for evaluations of vascular malformations, although usually unnecessary for most lesions.

3. Pathogenesis
     The improper sympathetic neuronal control of the capillaries may lead to chronic dilatation of dermal capillaries and their development into ectatic vessels. Clinical observation provides evidence that these lesions somewhat follow cutaneous sensory nerve distributions. While an autosomal dominant mode of inheritance with variable penetrance has been suggested, this finding is not observed in most clinical situations.

4. Clinical Findings
     Although usually not associated with other abnormalities, a capillary malformation may point to other problems. When capillary malformations are associated with other vascular malformations, these combined situations are recognized as syndromes. A facial capillary vascular malformation in the ophthalmic distribution of the trigeminal nerve (CN V) may indicate the patient has Sturge-Weber syndrome. This syndrome is a congenital condition consisting of the aforementioned cutaneous vascular malformation associated with a similar malformation of the underlying meninges and cortex. Children with Sturge-Weber syndrome are at increased risk to develop seizures and glaucoma as well as soft-tissue and bony overgrowths in the midface. Children with a capillary malformation located in the V1 division (the first division of the trigeminal nerve) should have both an MRI scan of the brain and screening ophthalmologic exams.

     A capillary malformation that overlies a deep venous or lymphatic malformation (a mixed vascular malformation) of the extremity is referred to as Klippel-Trenaunay syndrome. The overlying skin is often involved with ulceration and infection. The underlying bone becomes overgrown, adding to limb hypertrophy and often necessitating surgical intervention. A capillary malformation that overlies a deep high-flow arteriovenous malformation is referred to as Parkes-Weber syndrome. Lumbo-sacral capillary malformations may indicate that spinal cord abnormalities exist and should also be investigated further.

5. Differential Diagnosis 
     The typical capillary malformation must be differentiated from the commonplace fading macular stains of infancy (eg, “stork bite”). These lesions, in contrast to a true capillary malformation, will fade by the age of one and are usually seen in the nuchal region, the eyelid, the glabella, or the lips.  Location is the best clue to help differentiate.

6. Complications
     The primary complications of capillary malformations are skin changes and bleeding. If untreated, a significant percent of patients will manifest a change in the surface appearance of the skin. The skin can become nodular and the increasingly dilated and ectatic dermal vessels may bleed spontaneously.

7. Treatment
     The treatment of choice for a capillary malformation is laser photocoagulation. Both cosmetic improvement and the prevention of complications in adulthood are possible with laser therapies. These therapies often require multiple treatments and are more efficacious when started early in life. The flashlamp pulsed dye laser is reported to give a 50–70% response rate. These rates range from complete to partial resolution. In a previously untreated adult patient with both progression of the lesion to a nodular appearance and troublesome bleeding, skin grafting may be necessary. 

Breugem CC, van der Horst CMAM, Hennekam RCM. Progress toward understanding vascular malformations. Plast Reconstr Surg. 2001;107:1509. [PMID: 11335828] (A detailed review of the current understanding of the pathogenesis of these birth defects.) Enjolras O, Mulliken JB. The current management of vascular birthmarks. Pediatr Dermatol. 1993;10:311.[PMID: 8302734] (A review of current treatments of vascular birthmarks.)