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.
Date reviewed: August 2017