What Is CLOVES Syndrome?

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.

What Are the Signs & Symptoms of CLOVES Syndrome?

Children who have CLOVES syndrome are born with it. So far, it's been identified in fewer than 200 children.

CLOVES syndrome 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)
• birthmarks (raised red or brown areas, port-wine stains)
• spinal curvature (scoliosis) or tethered cord (when the spinal cord is attached to the spinal column)
• kidney problems (unusual size, asymmetry, Wilms tumor)
• intestinal and bladder problems (bleeding)
• 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. It is not hereditary (passed down by parents to their children).

How Is CLOVES Syndrome Diagnosed?

CLOVES is typically diagnosed at birth based on a baby's physical problems. Careful examination and advanced imaging tests (MRI, CT scan, ultrasound) might be needed to confirm a CLOVES 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.

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. To diagnose CLOVES, doctors will look for the syndrome's combination of vascular, skin, and spinal abnormalities.

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, and others.

Treatments for CLOVES include:

• sclerotherapy: to shrink the vascular malformations and lymphatic spaces/cysts
• interventional radiology procedures: to shrink tumors or repair lymph and blood vessel malformations
• embolization: to block blood vessels or vascular channels of malformations
• debulking surgery: to remove some tissue overgrowth, which will maximize function for the child and decrease deformity
• surgery: general, urology, gastroenterology, thoracic
• orthopedic procedures: to correct foot, hand, spine, bone, and joint problems
• neurosurgery: to repair a tethered spinal cord, or spinal mass and craniofacial anomalies
• rehabilitation therapies: including physical therapy and occupational therapy

Looking Ahead

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 overall health.

Research into medicines and other treatments that can help kids with CLOVES is ongoing.

Reviewed by: Craig M. Johnson, DO
Date Reviewed: 31-08-2017