CLOVES Syndrome | Diagnosis & Treatment

How is CLOVES syndrome diagnosed?

Because symptoms of CLOVES syndrome can be subtle or obvious, it’s critical to seek a diagnosis from an experienced vascular anomalies specialist.

To diagnose CLOVES syndrome, doctors will usually combine these steps:

CLOVES is still not widely known and so rare that an ultrasound is not a foolproof way to detect it. A clinical exam must be performed after birth.

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: a rare condition that causes progressive deformities of the bone, skin and soft tissue
  • The main difference between these conditions and CLOVES is that CLOVES causes a combination of vascular, skin and limb/torso abnormalities and truncal/spinal abnormalities (like fatty masses, scoliosis or tethered cord).

What are the treatment options for CLOVES syndrome?

There is no cure for CLOVES syndrome, but experienced vascular anomaly specialists can manage or prevent symptoms with the right medical and surgical care.

Some of the treatments your child's care team might recommend include:

  • Drug therapy with sirolimus: Also known as rapamycin, this oral medication suppresses the immune system and slows the growth of abnormal lymphatic vessels that cause the vascular anomalies present in children with CLOVES. It can also improve symptoms, including pain.
  • Sclerotherapy: This non-surgical procedure can help reduce the size of the vascular anomalies associated with CLOVES, as well as the pain they may cause.
  • Embolization: This minimally invasive procedure is used to reduce the size of arteriovenous malformations and other vascular anomalies associated with CLOVES syndrome.
  • Debulking surgery: Some children need debulking surgery to remove a portion of the overgrown tissue and blood vessels caused by CLOVES syndrome. Though debulking is a major, invasive operation, it can be life-changing for children who have limited mobility due to limb abnormalities or painfully overgrown veins.
  • IVC Filter: A device called an inferior vena cava (IVC) filter can prevent pulmonary embolisms, life-threatening clots that can form and travel through the bloodstream and into the lungs of children with CLOVES. The filter, which is surgically implanted, sits in the inferior vena cava (the main abdominal vein responsible for transporting blood from a child's lower body) and traps any clots, before they reach the heart and lungs.

What is the long-term outlook for CLOVES syndrome?

CLOVES is a rare disease that affects each child differently. Your child's long-term outlook will depends on many factors, including:

  • Age at diagnosis (the earlier treatment is started, the better)
  • Specific symptoms
  • Overall health

Many children with CLOVES do very well when the disease is mild and diagnosed early. Your child’s doctor will give you specific information about a recommended plan of care and long-term outlook.