CLOVES syndrome


CLOVES syndrome is an extremely rare condition. It is a disorder that is present at birth (congenital). This disorder causes abnormalities in blood vessels, skin, spine, joints and bones.

CLOVES stands for Congenital Lipomatous assymetric Overgrowth, Vascular malformations, Epidermal nevi and Skeletal and spinal anomalies. This is similar to fibroadipose hyperplasia (FH) with more prominent blood vessel overgrowth, nevi (moles) and bone and spine problems. This condition may be noted on prenatal ultrasound or at birth.  This may also progress with time. There may be life-threatening problems due to the overgrowth pushing on vital organs or because the increased blood vessel growth can lead to heart failure.

The syndrome affects about 150 people worldwide.

Causes and risk factors

This is caused by a mutation in the PIK3CA gene during early development of the embryo in the womb

Symptoms and types

Symptoms can vary greatly from one child to the next, which can make this syndrome difficult to diagnose. These symptoms are always present at birth, but might not be noticeable right away.

 In general, children have some type of :

  • Fatty mass in their trunk (torso)
  • Deformities of the blood vessels
  • Skin abnormality such as scoliosis or tethered spinal cord (tissue is attached to the spinal cord so that it cannot move freely within the spinal canal)
  • Overgrowth of, or deformities in, arms, hands, legs or feet
  • Curvature of the spine (scoliosis or other spinal problem)

Less common symptoms:

  • Problems with knee or hip joints
  • Kidneys that are unusually small or uneven in size

Diagnosis and tests

The sooner a child with CLOVES syndrome is diagnosed and treatment started, the better the general outlook.

To diagnose, a doctor will:

Treatment and care

Because CLOVES syndrome can affect so many different parts of the body, it is essential to approach treatment in a multidisciplinary way.

A child’s care team should consist of doctors who treat the different areas of the body affected by CLOVES syndrome. These specialists may include:

  • pediatric general surgeon
  • hematologist
  • interventional radiologist and radiology
  • nephrologist
  • neurosurgeon
  • orthopedic specialist
  • plastic surgeon

Treatment may consist of:

  • Sclerotherapy. This treatment involves injecting a solution directly into the venous mass to make it shrink.
  • Embolization. This procedure blocks blood vessels or vascular channels of malformations.
  • Debulking surgery. This procedure partially removes tissue to make subsequent therapy with drugs, radiation or other measures more effective.
  • Managing problems with the spine, bones, joints and kidneys

Living and managing

The management of CLOVES syndrome can be very challenging. It is important to consider having your child treated by a doctor who specializes in blood vessel and skeletal disorders. The doctor will put together a treatment plan for your child.

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