Pediatric Vascular Anomalies

At Children's Memorial Hermann Hospital, we stand at the forefront of caring for unique and rare vascular conditions that affect the lymphatic system and blood vessels throughout the body. Leveraging a multidisciplinary approach, we provide comprehensive and personalized care for complex congenital conditions, utilizing disease-modifying medications in combination with the most advanced laser technology, surgical methods and minimally invasive radiological techniques available today.

Our patients receive customized care through a coordinated and streamlined approach, including timely scheduling of appointments, periodic imaging and complex therapeutic procedures. This patient-centered treatment helps us provide swift, seamless care across various specialties to diagnose and treat children in a supportive environment.

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Patients benefit from Children’s Memorial Hermann Hospital’s collaborative, multidisciplinary care and the combined experience, of affiliated specialists in general pediatrics and pediatric specialties such as plastic surgery, dermatology, interventional radiology, pediatric surgery, otolaryngology, pathology, hematology, genetics and neurosurgery. This multidisciplinary approach thoroughly assesses and addresses every aspect of the patient’s condition in a cohesive manner, exemplifying our commitment to delivering care in a supportive environment.

Because of the care we provide for vascular anomalies at Children’s Memorial Hermann Hospital, we are also designated as a NORD Rare Disease Center of Excellence, highlighting our efforts to expand access and advance care and research for rare disease patients across the country.

 As part of the Memorial Hermann Health System, we extend care beyond pediatric patients to adults. Many of our affiliated providers are dual-certified in both pediatric and adult care, enabling them to provide a continuity of care that transcends the traditional transition from pediatric to adult health care services. This continuum of care can help provide a seamless experience for our patients as they grow, eliminating the stress and uncertainty often associated with transitioning to adult care providers. Our goal is to provide care that evolves with each patient, providing lifelong support and guidance.

At Children’s Memorial Hermann Hospital, we're not just treating vascular anomalies; we're nurturing brighter futures through high-quality care and advanced treatments.

We offer a wide array of treatments and services that address a comprehensive range of conditions with precision and compassion. From innovative therapies to specialized surgical interventions, we're dedicated to providing care that supports the health and well-being of our young patients at every step.

Conditions

• Arteriovenous Malformations (AVMs): An arteriovenous malformation (AVM) consists of a complex web of arteries and veins in the brain, leading to potential blood flow disruption and increased risk of bleeding.
• Birthmarks: These are visible discolorations or raised areas on the skin, present from birth or appearing soon after, varying widely in size, shape, and color.
• Blue Rubber Bleb Nevus Syndrome (BRBNS): This rare vascular disorder involves multiple venous malformations (blebs) that affect the skin and internal organs, often recognizable by their soft, blue, rubbery appearance.
• CLOVES Syndrome: A very rare congenital condition, CLOVES syndrome is marked by complex vascular anomalies, overgrowth of tissues and organs, and distinctive skin markings.
• Capillary Malformation: Also known as a port-wine stain, this type of birthmark is characterized by a reddish-purplish discoloration of the skin caused by an abnormal capillary network.
• Cavernous Malformations: These are clusters of malformed, dilated blood vessels that create a mass in the brain or spinal cord, potentially leading to neurological symptoms.
• Central Conducting Lymphatic Anomaly (CCLA): A rare lymphatic disorder characterized by abnormal development of the central lymphatic system, leading to dilatation and dysfunction of the lymphatic channels. This condition can result in fluid accumulation, swelling, and other complications in the chest and abdominal cavities.
• Craniofacial Anomalies: These are structural deformities of the skull and face bones, often present at birth, that can affect a child's appearance, brain development, and overall health.
• Congenital Nevus: Also known as a mole, this is a type of pigmented birthmark that appears at birth or during a baby’s first year.
• Cutis Marmorata Telangiectatica Congenita (CMTC): A rare vascular birthmark presenting as a marbled or mottled pattern on the skin, which typically fades as the child grows.
• Fibro Adipose Vascular Anomaly (FAVA): This condition involves painful, fibrous, and fatty overgrowths within muscle tissue, leading to functional impairment and discomfort.
• Generalized Lymphatic Anomaly: A condition characterized by the widespread abnormal formation of lymphatic vessels, affecting multiple body areas including lungs, bones, and soft tissues.
• Glomuvenous Malformation (GVM): A vascular malformation containing specialized smooth muscle cells (glomus cells), leading to localized, bluish skin lesions that are often painful.
• Gorham-Stout Disease: A rare disorder where progressive bone loss occurs due to an overgrowth of lymphatic vessels, leading to significant skeletal and soft tissue complications.
• Hemangioma: This common benign tumor of infancy appears as a bright red birthmark due to an abnormal accumulation of blood vessels in the skin or internal organs.
• Hemihyperplasia: A condition causing asymmetric growth of one side or part of the body, potentially leading to functional and cosmetic concerns.
• Hereditary Hemorrhagic Telangiectasia (HHT): A genetic disorder affecting blood vessels, leading to easy bleeding as well as arteriovenous malformations in various organs.
• Juvenile Nasopharyngeal Angiofibroma (JNA): A benign, but locally aggressive vascular tumor that typically affects adolescent males, causing nasal obstruction and epistaxis.
• Kaposiform Hemangioendothelioma (KHE): A rare, benign vascular tumor that may be associated with Kasabach-Merritt phenomenon, involving the skin, deep soft tissues, or internal organs.
• Kaposiform Lymphangiomatosis (KLA): KLA is a rare, aggressive vascular disorder involving multiple systems but primarily affecting the lymphatic system. KLA is characterized by lesions that resemble both capillary malformations and kaposiform hemangioendothelioma.
• Kasabach-Merritt Phenomenon (KMP): A life-threatening condition involving a vascular tumor with severe platelet trapping, leading to localized clotting and bleeding risk.
• Klippel-Trenaunay Syndrome (KTS): A congenital vascular disorder characterized by port-wine stains, varicose veins, and bone and soft tissue growth leading to limb enlargement.
• Lymphatic Malformation: A cluster of abnormal lymph vessels forming a mass that can occur anywhere in the body, often evident as a soft, fluid-filled swelling.
• Lymphedema: A chronic condition caused by inadequate lymph drainage, leading to swelling, most commonly in the limbs, and associated with a risk of infection and discomfort.
• Macrocephaly Capillary Malformation (MCAP): A rare genetic disorder characterized by abnormal overgrowth of body parts, distinctive facial features, capillary malformations on the skin, and increased head size (macrocephaly). It may also involve neurological and developmental issues.
• Parkes Weber Syndrome: An extremely rare vascular condition similar to Klippel-Trenaunay Syndrome but includes arteriovenous fistulas, leading to a high flow state in the affected limb.
• PIK3CA Overgrowth Spectrum: This spectrum covers a range of disorders caused by mutations in the PIK3CA gene. These mutations lead to abnormal growth and development of various body parts, including the skin, skeleton, and brain. Conditions under this spectrum include overgrowth syndromes like MCAP, Klippel-Trenaunay Syndrome, and isolated lymphatic malformations.
• PTEN Mutations: PTEN is a tumor suppressor gene, and mutations in PTEN can lead to uncontrolled cell growth and division, contributing to the development of tumors. PTEN mutations are associated with several genetic disorders and syndromes, including Cowden syndrome, which increases the risk of developing various types of cancer.
• Pyogenic Granuloma: This is a common, benign skin growth that presents as a small, red, and often bleeding bump on the skin.
• Spider Telangiectasias: Small, spider-web-like capillary blood vessels visible on the skin's surface, typically harmless and often of cosmetic concern only.
• Sturge-Weber Syndrome (SWS): A neurological disorder marked by a facial port-wine stain, eye and brain abnormalities, leading to seizures and other complications.
• Tufted Angioma: A rare, benign skin lesion that may appear as a red-brown, tender patch or plaque, sometimes associated with Kaposiform hemangioendothelioma.
• Venous Malformation: A common type of vascular malformation characterized by abnormally dilated and tortuous veins, leading to bluish skin lesions that can be painful or problematic.

Treatments

• CO2 Laser Treatment: Utilizes concentrated carbon dioxide lasers to precisely target and treat skin abnormalities and lesions, including lymphatic malformations. This method minimizes damage to surrounding tissues, making it an efficient option for precise dermatological interventions.
• Cryoablation for Fibro-Adipose Vascular Anomaly (FAVA): Employs extreme cold to destroy fibrous and fatty tissues causing pain or dysfunction. This treatment is particularly effective in managing FAVA conditions, offering a non-invasive alternative to traditional surgical methods.
• Endovenous Laser Therapy: Targets symptomatic superficial varicosities and extensive lymphatic and venous malformations using laser energy delivered through a catheter placed in the vein. This therapy is minimally invasive and helps in reducing varicose vein symptoms and improving cosmetic appearance.
• Lymphangiography: An imaging technique to visualize the lymphatic system, including lymph nodes and vessels. It is used to identify abnormalities or leaks, playing a crucial role in diagnosing lymphatic disorders.
• Lymphoscintigraphy: A diagnostic test that produces images of the lymphatic system using a radioactive tracer to detect blockages, leaks, or other abnormalities. It is essential for understanding the functional status of the lymphatic system.
• Primary Endovascular Treatment (Embolization) of Arteriovenous Malformations: Involves the use of endovascular techniques to block or reduce blood flow to arteriovenous malformations. This treatment can prevent complications associated with these malformations and improve patient outcomes.
• Pulsed Dye Laser: Targets blood vessels in the skin to effectively reduce or eliminate vascular birthmarks, such as port-wine stains. This laser treatment is known for its precision and minimal risk of scarring or skin texture changes.
• Sclerotherapy: Involves injecting a sclerosing agent into vascular malformations to cause shrinkage and eventual closure. It is effective for treating venous and lymphatic malformations, offering a balance between therapeutic benefit and a low rate of side effects and complications.
• Surgical and Complex Reconstruction: Performed for the primary resection of anomalies or secondarily after the success of sclerotherapy. This approach involves detailed planning and execution to restore function and appearance, particularly in cases where non-invasive treatments are insufficient.
• Vascular Imaging: Includes ultrasonography, angiography, venography, and lymphatic mapping. These imaging modalities are essential for diagnosing vascular conditions, guiding treatment planning and monitoring therapeutic outcomes.
• Venography: An imaging test that uses contrast dye and X-rays to visualize veins. It is often utilized to diagnose conditions like deep vein thrombosis or venous malformations, providing detailed insights into venous anatomy and function.

Medical Therapies

• Beta Blockers: Beta blockers, such as propranolol, work by blocking the effects of adrenaline (epinephrine) and other stress hormones. They can help reduce the size and prominence of vascular lesions, particularly in infantile hemangiomas, which are the most common type of vascular tumor in infancy.
• mTOR Inhibitors: The mammalian target of rapamycin (mTOR) is a key protein that regulates cell growth, proliferation, and survival. mTOR inhibitors, such as sirolimus (also known as rapamycin), work by inhibiting the mTOR pathway, which is often dysregulated in complex vascular anomalies. Sirolimus is used to treat complex vascular anomalies that are unresponsive to other treatments, such as complicated lymphatic malformations, venous malformations and conditions associated with overgrowth syndromes.
• PI3Kα Inhibitor: Phosphoinositide 3-kinase alpha (PI3Kα) is an enzyme that plays a crucial role in the PI3K/AKT/mTOR signaling pathway, which is involved in cell growth, proliferation, and survival. Alpelisib specifically inhibits the PI3Kα isoform, thereby blocking the signaling pathway that leads to abnormal vascular growth.
• Other Targeted-Therapy Agents Based on Genetic Testing: The advancement in genetic testing has allowed for the identification of specific mutations involved in various vascular anomalies, which has in turn paved the way for the use of targeted therapy to specifically inhibit the proteins or signaling pathways dysregulated by these genetic mutations. This approach allows for personalized medicine, tailoring treatments to the specific genetic makeup of an individual's vascular anomaly.

Prenatal Treatment with The Fetal Center

Some vascular anomalies can now be identified before birth. In these cases, we collaborate closely with The Fetal Center at Children’s Memorial Hermann Hospital to educate expectant parents about these conditions early on, helping to prepare them prior to the birth of their child. Additionally, prenatal diagnosis helps aid in a seamless coordination of care at the time of delivery and throughout the neonatal period. Our affiliated teams have also started to introduce innovative therapies specifically designed for the fetal management of vascular anomalies.

Visit The Fetal Center at Children’s Memorial Hermann Hospital »

Texas Cranio-facial Team and Pediatric Plastic Surgery Clinic
6410 Fannin Street, Suite 950
Houston, TX 77030

Plastic & Reconstructive Surgery Clinic: (832) 325-7234
Texas Cranio-facial Team: (713) 500-7302