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CLOVES Syndrome

  • Overview

    In 2006, Boston Children’s Hospital interventional radiologist and Vascular Anomalies Center Co-Director Ahmad Alomari, MD, MSc, FSIR, discovered a very rare congenital (present at birth) disorder characterized by a combination of vascular (involving the blood vessels), skin, spinal and bone or joint abnormalities.

    Dr. Alomari named this complex condition Congenital Lipomatous Overgrowth, Vascular Malformations, Epidermal Nevis, Spinal/Skeletal Anomalies/Scoliosis syndrome—“CLOVES” for short.

    CLOVES syndrome can be very difficult to diagnose correctly—not only because it is so rare, but also because its signs and symptoms vary quite a bit in the way they occur and how severe they are.

    In general, children with CLOVES have some type of:

    • fatty mass in their torso, or “trunk”
    • vascular anomalies
    • skin abnormality
    • overgrowth or deformities in their arms/hands and/or their legs/feet
    • scoliosis or other spinal problem

    CLOVES can also cause small or missing kidneys and problems with the joints.

    The long-term picture for a child with CLOVES depends on how serious his symptoms are and how old he is when he is diagnosed. The sooner the disease is detected and the proper course of treatment is started, the better the general outlook.

    That’s why it is essential to seek treatment from pediatric doctors with specialized expertise in CLOVES syndrome—experts like those on the Vascular Anomalies Center team at Boston Children’s Hospital. We are the world leaders in diagnosing and treating CLOVES.

    How Boston Children’s Hospital approaches CLOVES syndrome

    Not only was CLOVES syndrome first discovered by a Boston Children’s physician; our hospital also has a long and distinguished history of caring for infants, children, teens and young adults with all kinds of complex vascular anomalies.

    Our Vascular Anomalies Center clinicians are renowned for managing disorders involving the blood vessels—ranging from very rare syndromes like CLOVES to more common conditions, such as hemangiomas and lymphatic, venous and arteriovenous malformations. Learn more about the services we offer.

    Because CLOVES can affect so many different parts of a child’s body, a multidisciplinary approach is critical. Here at Boston Children’s, doctors in our Vascular Anomalies Center work closely with colleagues in:

    This allows us to offer comprehensive, compassionate care that will not only address your child’s medical and surgical needs, but will also provide emotional and educational support for your entire family.

    CLOVES syndrome: Reviewed by Ahmad Alomari, MD, MSc, FSIR
    © Boston Children’s Hospital; posted in 2012

    »
    Boston Children's Hospital
    300 Longwood Avenue
    Fegan Building, 3rd Floor
    Boston MA 02115

     617-355-5226



  • In-Depth

    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.

    Causes

    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
  • Tests

    How is CLOVES syndrome diagnosed?

    Because symptoms of CLOVES syndrome can be subtle and hard to notice right away (or very obvious and causing clear problems in day-to-day life, depending on the particular child), it’s critical to seek a diagnosis from an experienced pediatric professional.

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

    Genetic tests may become available in the future.

    Can a prenatal ultrasound detect CLOVES syndrome?

    Yes. However, because CLOVES is so rare and still not a widely known disorder that doctors look for in utero, an ultrasound is not a foolproof way to detect it; a clinical exam must be performed after birth.

  • What are the treatment options for a child with CLOVES?

    It's important to remember that every child with CLOVES syndrome is very different, and must be treated as an individual. Since the disorder can involve so many parts of the body, in different ways, treatment has to be customized at every stage of the process. 

    Symptoms should always be addressed as early in a child's life as possible. (Enlarged veins, for example, are much easier to operate on in a young child than in an older, bigger child or adult.)

    Sclerotherapy

    Sclerotherapy is a non-surgical procedure that can help reduce the size and pain associated with vascular anomalies occurring with CLOVES, such as lymphatic and venous malformations.

    During sclerotherapy, an interventional radiologist injects a toxic agent (called a sclerosant) into the malformation. This causes it to scar over, relieving symptoms and possibly shrinking the anomaly at the same time.
     
    Embolization

    Embolization is a minimally invasive procedure used to reduce the size of arteriovenous malformations and other vascular anomalies associated with CLOVES syndrome.

    During embolization, an interventional radiologist carefully moves a thin tube called a catheter up through the child's arteries and to the exact location of the problem. The doctor then injects a special substance to close the abnormal vessels. 

    Debulking surgery

    Some children need debulking surgery to remove a portion of the overgrown tissue and blood vessels caused by CLOVES syndrome. This involves multiple lengthy and complex procedures performed over a period of time. 

    Though debulking is a major, invasive type of surgery, it can be life-changing for kids who are essentially crippled by their overgrown limbs or painfully overgrown veins. 

    Preventing pulmonary embolisms

    To reduce the risk of pulmonary embolisms—life-threatening clots that travel through the bloodstream and into the lungs; they are a significant risk for children with vascular problems related to CLOVES—a device called an inferior vena cava (IVC) filter can be inserted before surgery.

    The interventional radiologist uses special imaging technology to guide the device into the inferior vena cava (the main abdominal vein responsible for transporting blood from a child's lower body). Once in place, the filter can trap any clots that are attempting to move through, stopping them from reaching the heart and lungs. 

    Managing spine, bone/joint and kidney problems

    Your child's treating clinician will refer her to specialists to manage her particular spinal, bone and joint issues, as well as any kidney problems related to her CLOVES syndrome.

    Why is it so important to have a multidisciplinary team treating CLOVES?

    CLOVES is a complex disorder that can cause a number of serious symptoms, impacting multiple organs and functions. As a result, the disease requires a team approach by pediatric experts who are skilled and experienced in treating particular types of problems in particular parts of the body—and who will work closely together at every step of the way.

    Here at Boston Children's Hospital, our CLOVES syndrome experts:


    Coping and support 

    In addition to the clinical information provided on this webpage, Boston Children's offers several other resources designed to give your child and family comfort, support and guidance. 

    Resources at Boston Children's

    • Boston Children's Center for Families is dedicated to helping families locate the information and resources they need to better understand their child's particular condition and take part in their care. All patients, families and health professionals are welcome to use the center's services at no extra cost. The Center for Families is open Monday through Friday from 8 a.m. to 7 p.m., and on Saturdays from 9 a.m. to 1 p.m. Please call 617-355-6279 for more information. 
       
    • Boston Children's Behavioral Medicine Clinic helps children who are being treated on an outpatient basis at the hospital—as well as their families—understand and cope with their feelings about:
      • being sick
      • facing uncomfortable procedures
      • handling pain
      • taking medication
      • preparing for surgery
      • changes in friendships and family relationships
      • managing school while dealing with an illness
         
    • The Experience Journal was designed by Boston Children's psychiatrist-in-chief, David DeMaso, MD, and members of his team. This online collection features thoughts, reflections and advice from kids and caregivers about living with a variety of medical conditions, the “befores” and “afters” of surgery and going through many other medical experiences.
       
    • Boston Children's Department of Psychiatry offers a free booklet, “Helping Your Child with Medical Experiences: A Practical Parent Guide.” (Adobe Acrobat required to view and download) Topics in the booklet include:
      • talking to your child about her condition
      • preparing for surgery and hospitalization
      • supporting siblings
      • taking care of yourself during your child's illness
      • adjusting to life after treatment
          
    • The Boston Children's chaplaincy is a source of spiritual support for parents and family members. Our program includes nearly a dozen clergy members—representing Episcopal, Jewish, Lutheran, Muslim, Roman Catholic, Unitarian and United Church of Christ traditions—who will listen to you, pray with you and help you observe your own faith practices during your child's treatment.
       
    • Boston Children's International Center is a resource for patients and families from countries outside the United States. The center can provide assistance with everything from reviewing medical records to setting up appointments and locating lodging. Contact the center by phone at 01-617-355-5209 or via e-mail at international.center@childrens.harvard.edu.  

    General guide for patients and families

    Read our guide to essential information across the hospital.

    Helpful links 

    Please note that neither Boston Children's Hospital nor the Vascular Anomalies Center at Boston Children's unreservedly endorses all of the information found at the sites listed below. These links are provided as a resource.

    • CLOVES Syndrome Community
      An exhaustive informational resource, featuring an active community that supports, educates, empowers and improves the lives of those affected by CLOVES syndrome.
       
    • CLOVES Syndrome Community Facebook Page
       
    • CLOVES Syndrome Foundation
      Dedicated to improving the lives of CLOVES syndrome patients by funding overgrowth and vascular anomaly research, raising public awareness and providing member support while establishing support networks within the medical and patient family communities.
  • Research & Innovation

    • Matthew Warman, MD, director of Boston Children’s Orthopedic Research Laboratories, and Kyle Kurek, MD, staff pathologist, are leading an initiative that has already revealed the genetic basis for CLOVES: a mutation in a gene known as PIK3CA.

      Next, the team hopes to develop models to understand how mutations in PIK3CA cause malformation and overgrowth, and to determine which drugs and other therapies can be used safely and successfully to improve the lives of individuals with CLOVES and other conditions with similar clinical characteristics. Learn more.
       
    • Boston Children’s has been identified as the second national site for a clinical trial of a drug called sirolimus—which has proven effective in slowing the growth of certain tumors—in children with complex vascular anomalies, such as those found in CLOVES syndrome.

      The Boston Children’s trial will be led by Cameron C. Trenor III, MD, co-director of the hospital’s Cerebrovascular Disorders and Stroke Program. Learn more.
       
    • Ahmad Alomari, MD, MSc, FSIR, co-director of Boston Children’s Vascular Anomalies Center, and his colleagues have collected data characterizing many clinical findings related to CLOVES syndrome. They are now beginning the data analysis phase. 
  • Your Story

     
    Riley's story

    Riley is like most other kids. She likes to swim, read, dance and sing. However, the one thing that makes her different than most kids is she was born with CLOVES syndrome. 

     

     Follow Riley's journey:


    Riley's mom's story

    "Becoming a parent is an act of love and faith that no amount of planning can ever really prepare you for. And once you begin raising your child, the changes you go through as a person are profound. Raising a child with a rare disease is even more life altering."

    Riley and care team members

    Riley’s mother Kristen talks about what’s it’s like to raise a child with such a rare medical condition in a post on Thriving, Boston Children's blog for patients and families.

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The future of pediatrics will be forged by thinking differently, breaking paradigms and joining together in a shared vision of tackling the toughest challenges before us.”
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