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Port-wine stain in Child
See also in: External and Internal Eye
Other Resources UpToDate PubMed

Port-wine stain in Child

See also in: External and Internal Eye
Contributors: Brea Prindaville MD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

A port-wine stain (also termed nevus flammeus) is a type of vascular malformation known as a capillary malformation. In the past, port-wine stains and salmon patches were considered to be variations of capillary malformation; it is now known that port-wine stains are truly malformations of capillaries that may be associated with systemic complications and will never spontaneously improve. Furthermore, salmon patches represent dilatation of capillaries rather than a true malformation. These often fade with time if located on the face and are not associated with any other abnormalities.

Port-wine stains are typically congenital. Some port-wine stains darken over time, especially if located on the lower face. They may also become thicker and more nodular, and can develop pyogenic granulomas within them. This usually occurs in early adulthood.  

When a port-wine stain occurs on the forehead, Sturge-Weber syndrome (SWS) should be considered. SWS can include eye abnormalities (choroidal vascular anomalies, buphthalmos, and glaucoma) and leptomeningeal and brain abnormalities.

Port-wine stains on the lower back that cross the midline may be associated with dimples, sinuses, lipomas, faun-tail deformities (see lumbosacral skin lesion), and, most importantly, tethered cord. In these locations, neurological consultation and ultrasound or MRI is indicated.

Klippel-Trenaunay syndrome (angio-osteohypertrophy) consists of a triad of vascular stain, hemihypertrophy, and venous varicosities. In the past, the vascular stain has been thought to be a pure capillary malformation; however, it is now recognized that it is more commonly a combined capillary, lymphatic, and/or venous malformation. Some specialists prefer to characterize this syndrome with a description of the specific malformations found in each patient.  

Phakomatosis pigmentovascularis is characterized by the coexistence of a capillary malformation and a cutaneous pigmented lesion. Both are typically present at birth.

Codes

ICD10CM:
Q82.5 – Congenital non-neoplastic nevus

SNOMEDCT:
416377005 – Port-wine stain of skin

Look For

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Salmon patch – These are small capillary dilatations commonly occurring in the midline on the head (stork bites, angel kisses). They decrease in color with age (except for those on the nape of the neck, which tend to persist) and usually do not require treatment.
  • Early Infantile hemangioma in children
  • Sturge-Weber syndrome
  • Klippel-Trenaunay syndrome
  • Cutaneomeningospinal angiomatosis
  • Proteus syndrome
  • Rubinstein-Taybi syndrome
  • Megalencephaly-capillary malformation-polymicrogyria syndrome (Macrocephaly-capillary malformation)

Best Tests

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Management Pearls

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Therapy

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References

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Last Reviewed:11/16/2017
Last Updated:12/03/2017
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Patient Information for Port-wine stain in Child
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Port-wine stain in Child
See also in: External and Internal Eye
A medical illustration showing key findings of Port-wine stain : Vascular plaque
Clinical image of Port-wine stain - imageId=3351600. Click to open in gallery.  caption: 'A violaceous patch at the supraclavicular fossa.'
A violaceous patch at the supraclavicular fossa.
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