CLOVES Syndrome

What is CLOVES syndrome?

CLOVES syndrome is a very rare condition. It affects the blood vessels, spine, skin, joints and bones. CLOVES is named for the conditions that are part of the syndrome:

• Congenital: a condition a baby is born with
• Lipomatous: soft fatty masses that grow on the body
• Overgrowth: the fatty tissue in some areas of the body grows faster than in other areas · Vascular malformations: problems with the blood vessels
• Epidermal nevi: bumps on the skin
• Spinal or skeletal problems: often scoliosis or a tethered spinal cord

CLOVES syndrome is a type of overgrowth disorder. Fewer than 200 people worldwide have been diagnosed with the condition.

What are the signs and symptoms of CLOVES syndrome?

The symptoms of CLOVES syndrome can vary from one child to another, and can range from mild to severe. Although it's a condition a child has from birth, some children may not have symptoms right away. Common symptoms in children include:

• Fatty tissue masses growing on the torso
• Large hands, feet, fingers or toes
• Extra fingers or toes
• Blood vessels that are not formed normally (vascular malformations)
• Birthmarks (port-wine stains) or raised bumps on the skin
• A curve in the spine (scoliosis) or a tethered cord
• Kidney problems

What causes CLOVES syndrome?

CLOVES syndrome is caused by a mutation in a gene called PIK3CA. It is a mutation that occurs spontaneously while a baby is developing during pregnancy. CLOVES syndrome is not passed from a parent to their child. Experts do not know what causes some babies to have this mutation.

How is CLOVES syndrome treated?

There is no cure for CLOVES syndrome, so treatment is based on caring for symptoms. Your care team at Arkansas Children's will work with you to create the best treatment plan for your child. Treatment options may include:

• Medicines, such as a drug called sirolimus, can help control pain and abnormal growth of some lymphatic vessels
• Sclerotherapy, a procedure that injects a medicine into vascular malformations to make them shrink
• Embolization, is a procedure that can help block or reduce vascular malformations
• Debulking surgery to remove overgrown blood vessels and tissue