CLOVES Syndrome Symptoms & Causes

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What does CLOVES stand for?

CLOVES is an abbreviation for “Congenital Lipomatous Overgrowth Vascular Malformations Epidermal Nevis Spinal/Skeletal Anomalies/Scoliosis.” 

When and how was CLOVES syndrome discovered?

CLOVES syndrome was first discovered in 2006 by Boston Children’s Hospital interventional radiologist and Vascular Anomalies Center Co-Director Ahmad Alomari, MD, MSc, FSIR.

Dr. Alomari spent more than a year reviewing decades’ worth of data and photos of children who had been given different diagnoses for a specific combination of symptoms. Although these cases were few and far between, he noticed a definite pattern when comparing them. 

In 2009, Dr. Alomari published a paper describing his findings in the medical journal Clinical Dysmorphology. This established CLOVES syndrome as a recognized pediatric disease.

What are some of the conditions CLOVES can be confused with?

Children with CLOVES syndrome are often mistakenly diagnosed as having other disorders that cause overgrowth of the blood vessels and abnormalities in certain parts of the body, such as:

  • Hemihypertrophy, a condition in which the structures on one side of a child’s body are larger than on the other
  • Klippel-Trenaunay syndrome, a rare disease that causes abnormal fatty growths of the leg, dilated veins, lymphatic malformations and port-wine stain birthmarks
  • Proteus syndrome, which causes progressive deformities of the bone, skin and soft tissue

The main difference between all of these conditions and CLOVES is that CLOVES causes vascular, skin and limb/torso abnormalities and truncal/spinal abnormalities (like fatty masses, scoliosis or tethered cord).

Can CLOVES be cured?

Unfortunately, there is no known cure for CLOVES. The good news is that many of the associated problems can be managed or prevented successfully with the right medical and surgical care.

What is the long-term picture for my child?

It depends very much on her age at the time of diagnosis (the earlier treatment is started, the better), her specific symptoms and her overall health.

Many children with CLOVES do very well when the disease is mild and the diagnosis and management of symptoms are not delayed. Your child’s doctor will give you specific information about her recommended plan of care and her long-term outlook.


Why was my child born with CLOVES syndrome?

Though CLOVES is a recently discovered and rare disorder, Matthew Warman, MD, director of Boston Children’s Orthopedic Research Laboratories, pathologist Kyle Kurek, MD, and their colleagues have made a major discovery about the genetic basis for CLOVES. The team found that between six and 60 percent of cells in each individual’s affected tissues contained mutations in a gene called PIK3CA

Learn more.

Signs and symptoms

What are the symptoms of CLOVES syndrome?

Symptoms can vary a great deal from child to child. They are always present at birth, but might not be noticeable right away, depending on how extensive they are.

Generally, in order to be diagnosed with CLOVES syndrome, a child will have one or more:

  • soft, fatty masses on his torso, or “trunk” (usually on his back, flank or abdomen)
  • vascular (blood vessel) abnormalities—most often, a “port wine stain” birthmark, lymphatic malformation, arteriovenous malformation and/or enlarged veins
  • limb abnormalities (typically, wide feet and/or toes and large or extra fingers/toes)
  • spinal problems (in many cases, scoliosis or tethered spinal cord). The presence of these abnormalities often indicates that a child is more likely to have CLOVES syndrome instead of a similar disorder.
  • skin abnormalities (most often, raised, wart-like bumps called epidermal nevi)

Less common symptoms can include:

  • problems with knee or hip joints
  • kidneys that are unusually small or uneven in size
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