OBSOLETE: Congenital vascular bone syndrome with limb overgrowth

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ORPHA:235835
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Overview

Congenital vascular bone syndrome with limb overgrowth is a very rare condition that was previously listed as a distinct diagnosis but has since been marked as obsolete in medical classification systems. This means that the condition has been reclassified or merged into a broader category of vascular malformation and overgrowth syndromes. The condition is characterized by abnormal blood vessel formation (vascular malformations) that are present from birth, along with changes in bone structure and overgrowth of one or more limbs. Affected individuals may notice that one arm or leg is noticeably longer or larger than the other, and they may have visible birthmarks or skin discoloration caused by abnormal blood vessels. This condition falls within a group of disorders sometimes referred to as vascular malformation-overgrowth syndromes, which include conditions like Klippel-Trenaunay syndrome, Parkes Weber syndrome, and other PIK3CA-related overgrowth spectrum (PROS) disorders. Because the term is now obsolete, patients who were previously given this diagnosis may now be reclassified under one of these related conditions based on modern genetic testing and clinical criteria. Treatment is generally supportive and symptom-based, focusing on managing vascular malformations, addressing limb length differences, and preventing complications such as blood clots or bleeding. A multidisciplinary team of specialists is typically involved in care.

Key symptoms:

One limb noticeably longer or larger than the otherVisible birthmarks or port-wine stains on the skinAbnormal blood vessels visible under the skinSwelling in the affected limbBone overgrowth or thickeningSkin warmth over affected areasPain or heaviness in the affected limbVaricose veins appearing at a young ageBleeding from abnormal blood vesselsDifficulty walking or uneven gaitBlood clots in the affected limbSoft tissue overgrowth

Inheritance

Variable

Can be inherited in different ways depending on the underlying gene

Age of Onset

Neonatal

Begins at or shortly after birth (first 4 weeks)

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for OBSOLETE: Congenital vascular bone syndrome with limb overgrowth.

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No actively recruiting trials found for OBSOLETE: Congenital vascular bone syndrome with limb overgrowth at this time.

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No specialists are currently listed for OBSOLETE: Congenital vascular bone syndrome with limb overgrowth.

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Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

No travel grants are currently matched to OBSOLETE: Congenital vascular bone syndrome with limb overgrowth.

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Community

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Caregiver Resources

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Questions for your doctor

Bring these to your next appointment

  • Q1.Has my condition been reclassified under a more specific diagnosis, and if so, what is it?,Should genetic testing be done to look for a specific mutation like PIK3CA?,What imaging or monitoring schedule do you recommend for tracking limb growth and vascular changes?,Am I or my child at risk for blood clots, and should we take preventive measures?,Would alpelisib (Vijoice) or any other targeted therapy be appropriate in my case?,What surgical options are available if the limb length difference becomes significant?,Are there clinical trials or research studies I should consider enrolling in?

Common questions about OBSOLETE: Congenital vascular bone syndrome with limb overgrowth

What is OBSOLETE: Congenital vascular bone syndrome with limb overgrowth?

Congenital vascular bone syndrome with limb overgrowth is a very rare condition that was previously listed as a distinct diagnosis but has since been marked as obsolete in medical classification systems. This means that the condition has been reclassified or merged into a broader category of vascular malformation and overgrowth syndromes. The condition is characterized by abnormal blood vessel formation (vascular malformations) that are present from birth, along with changes in bone structure and overgrowth of one or more limbs. Affected individuals may notice that one arm or leg is noticeably

At what age does OBSOLETE: Congenital vascular bone syndrome with limb overgrowth typically begin?

Typical onset of OBSOLETE: Congenital vascular bone syndrome with limb overgrowth is neonatal. Age of onset can vary across affected individuals.