CLOVES Syndrome

By Dr. Anukriti Singh|2 - 3 mins read| June 03, 2024

What is CLOVES Syndrome ?

CLOVES Syndrome is a rare genetic disorder which is present at birth. It involves multiple organ systems such as the vascular, nervous system, skin, bones and joints. CLOVES is a short form which refers to Congenital Lipomatous Overgrowth, Vascular Malformations, Epidermal Nevis, Spinal/Skeletal Anomalies/Scoliosis. CLOVES Syndrome is a part of overgrowth disorders which are a part of a wider collection of disorders known as PROS or PIK3CA related overgrowth syndromes.

What causes CLOVES Syndrome?

Genetic mutations are responsible for CLOVES Syndrome. The specific gene responsible for the development of the syndrome is PIK3CA. The genetic errors are spontaneous in nature and will happen while the baby is developing in the womb. There is no known risk factor which can makes the development of CLOVES Syndrome likely. It is not an inherited condition which means that it does not pass from the parents to the child.

What are the signs and symptoms of CLOVES Syndrome?

There are a wide range of symptoms of CLOVES Syndrome. Depending on the extent and severity of the disease, these may vary and may or may not be noticeable at birth. For a child to be diagnosed with CLOVES Syndrome, he or she must have one or more of the following:

  • You may notice some fatty tissue mass on the belly, back, or sides of child.
  • There may abnormal shape or size of feet and hands. It can be large, wide hands and feet or large fingers or toes or unusual spacing between toes and fingers.
  • The child may be visible superficial dilated veins over chest, arms, legs, and feet.
  • There may be birthmarks due to abnormal vasculature which appears as port-wine stains.
  • Abnormal spinal curvature or scoliosis may be present .
  • There can be a tethered spinal cord.
  • The child may face kidney problems, intestinal and bladder problems.
  • There can be asymmetric growth of the arms and legs or head.

How is CLOVES Syndrome managed?

There is no definitive cure of CLOVES Syndrome but certain interventions can be performed to prevent any recurrent or long term complications. Ateam of multispecialists is needed to efficiently manage CLOVES Syndrome which includes doctors as radiologist, vascular surgery, neuro surgery, orthopedics, hematology, pediatric surgery and so on. Some procedures which may be needed in a child with CLOVES Syndrome are-

  • Medical therapy
  • Sclero therapy
  • Embolisation
  • IVC filter
  • Joint or bone reconstruction
  • Rehabilitation

What should I be aware of as a parent?

  • Early diagnosis and treatment is very important for CLOVES Syndrome.
  • It is important to consult your doctor and allow specific interventions as soon as possible to prevent complication.
  • You should know that management of CLOVES Syndrome is long term and repeated follow ups and interventions may be needed.
  • You may want to join centres which provide information about CLOVES Syndrome to be better informed.

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About The Author:

Dr. Anukriti Singh

Last Updated: Mon Jun 03 2024

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