CLOVES syndrome is a very rare genetic
disorder. It's named for the combination of vascular, skin, spinal, and bone or joint
abnormalities that make up the syndrome:
C: Congenital (present at birth)
L: Lipomatous (referring to lipomas, soft fatty masses often
found on one or both sides of the back and belly)
O: Overgrowth (of fatty tissue in some body areas, which grow
much more quickly than others)
V: Vascular malformations (problems with lymph
and blood vessels)
E: Epidermal nevi (skin lesions)
S: Spinal/skeletal anomalies and/or Scoliosis (a
tethered spinal cord)
CLOVES and other rare conditions that can cause similar symptoms are often called
overgrowth disorders. These are part of a larger group of similar
disorders called PIK3CA-related overgrowth spectrum (PROS).
What Are the Signs & Symptoms of CLOVES Syndrome?
Children who have CLOVES syndrome are born with the condition. It can cause:
fatty tissue masses on the belly, back, or sides
problems with feet and hands (large, wide hands and feet; large fingers or toes;
unusual spacing between toes and fingers)
dilated veins (chest, arms, legs, and feet with a risk of blood clots)
asymmetric growth (one side grows faster than the other, such as the arms and
legs or head)
CLOVES syndrome symptoms vary widely in combination and severity. Some children
have mild symptoms, while others can have serious, life-threatening problems.
What Causes CLOVES Syndrome?
CLOVES is caused by a genetic
mutation (an error in a person's genes). Sometimes, like with CLOVES, genes can
mutate (change) on their own, with no known reason. This is called a sporadic mutation.
It is not hereditary (passed down by parents to their children).
How Is CLOVES Syndrome Diagnosed?
Doctors usually diagnose CLOVES at birth based on a baby's physical problems. Careful
examination and advanced imaging tests (MRI,
CT scan, ultrasound) might be needed to confirm the diagnosis. Fetal diagnosis (while
a baby is still in the womb) is sometimes possible. But because CLOVES is so rare,
it's still unknown to many health care providers. There is a genetic test for
the underlying genetic mutation, which requires a biopsy
to take a small piece of the abnormal tissue.
CLOVES syndrome can cause problems that are similar to those of other rare disorders.
Klippel-Trenaunay syndrome, Proteus syndrome, and hemihypertrophy also cause blood
vessel overgrowth and abnormalities in certain body areas. While these syndromes can
look the same, they're caused by different genetic mutations. To diagnose CLOVES,
doctors will look for its combination of vascular, skin, and spinal problems.
How Is CLOVES Syndrome Treated?
There is no cure for CLOVES, but a team of health care specialists can help families
manage the conditions and symptoms it causes. These specialists can include a vascular
interventional radiologist, a general surgeon, a hematologist, a genetics doctor,
a pathologist, a radiologist, a plastic surgeon, an orthopedic specialist, a neurosurgeon,
Treatments for CLOVES include:
medical therapy: some newer medicines, including sirolimus, can
help control some effects of the syndrome
sclerotherapy: to shrink the vascular malformations and lymphatic
interventional radiology procedures: to shrink tumors or repair
lymph and blood vessel malformations
embolization: to block blood vessels or vascular channels of
debulking surgery: to remove some tissue overgrowth, which will
help the affected area function and decrease deformity
surgery: general, urology, gastroenterology, thoracic
orthopedic procedures: to correct foot, hand, spine, bone, and
neurosurgery: to repair a tethered spinal cord, or spinal mass
and craniofacial anomalies
Early diagnosis is very important for kids with CLOVES. Evaluation and treatment
with a multidisciplinary team specializing in vascular anomalies and vascular malformations
should start as soon as possible to help manage a child's specific symptoms and health.
Research into medicines and other treatments that can help kids with CLOVES is
ongoing. You also can find information and support online at: