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CLOVES syndrome (Plastic Surgery)

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Acute

What is CLOVES Syndrome?

Congenital Lipomatous Overgrowth, Vascular Malformations, Epidermal Nevis, Spinal/Skeletal Anomalies/Scoliosis (CLOVES) syndrome is 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.

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Large wide hands or feet, large fingers or toes, wide space between digits (sandal gap toe) and uneven size of extremities are common. 4. Spinal anomalies include scoliosis (curving of the spine), fatty masses and vessels pushing on the spinal cord and tethered cord (spinal cord fixed by abnormal band).

Features CLOVES syndrome*
Visceral/endocrine anomalies Renal agenesis/hypoplasia, Wilms tumor, spleen lesions, ocular and dental anomalies (rare)
Complications Scoliosis, hemoptysis, cardiac failure from AVM, pulmonary embolism, gastrointestinal bleeding/obstruction
Mutations Mosaic activation mutation in PIK3CA gene

Research by Matthew Warman, MD, director of Boston Children's Orthopedic Research Laboratories, and pathologist Kyle Kurek, MD, has revealed that CLOVES is caused by mutations in a gene called PIK3CA. These mutations arise spontaneously in the womb, while a baby is developing.

limb abnormalities (typically, wide feet or toes and large or extra fingers or 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.

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

How is it diagnosed?

How is it diagnosed?

Treatments for CLOVES include: medical therapy: some newer medicines, including sirolimus, can help control some effects of the syndrome. sclerotherapy: to shrink the vascular malformations and lymphatic spaces/cysts.

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How is it treated?

Treatment for acute myeloid leukemia is vital. It varies with the patient and stage of the disease. Treatment options include

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.

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