Malformasi Limfatik, Kapiler, dan Vaskular Kombinasi

Malformasi Limfatik, Kapiler, dan Vaskular Kombinasi
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Definition

Voscalar malformation is an abnormal development or formation of blood vessels. This disorder can occur in pulses (arterials), back blood vessels (venes), 'clear fluid' lymphic vessels, capillary blood vessels, or a combination of several blood vessels.

 

Limfatik Malformation

Elimphatic malformation usually appears to be a lump/mass (unnormal growth) like a clot forming an irregularly growing (kista). The lymphphate is better known as a lymph lining, which contains lymph fluid and white blood cells. The lymph fluid functions in the body's defense system, such as fighting bacteria or germs that enter the body. The mass in lymphatic malformation is tame, and does not include cancer.

 

Capillary Malformation

Capillary malformation is the smallest growth disorder in blood vessels (so-called capillaries) that experience widening in the skin. This disorder appears to be a change in color to pink, red, or purple in the skin. Capillary Malformation is expected to occur in 1 in 300 newborns, and is often considered a sign of birth'' that can grow slowly with age.

 

Combination Vascalar Malformation

Combinatorial vascular malformation is a growth disorder involving two or more types of blood vessels, whether a vessel, veins, capillary, or a lymphatic vessel. Most combination malformation occurs in the arm or leg, but basically it can occur in any area of the body.

 

Cause

Limfatik Malformation

The exact cause of the emergence of lymphatic malformation is not yet known. This disorder is a congenital disease that was formed at the beginning of pregnancy. During the process of its formation, lymphatic vessels may be clogged and enlarged so that it can cause lymphatic fluids to accumulate. Most cases of lymphatic malformation are considered to occur randomly during the development of the baby in the uterus. Several cases of lymphatic malformation are suspected to be related to the presence of genetic mutations. However, these mutations are also considered to occur randomly and are not inherited in the family.

 

Capillary Malformation

Capillary malformation is thought to be caused by genetic disorders and is often relative (derived in the family). This malformation occurs when your child is still in the womb, where the capillary blood vessels expand and widen so that it allows more blood than normal amounts, flows through it and gives the skin a red warma.

Combinatorial vascular malformation is also associated with genetic abnormalities and in some cases can be inherited in the family.

 

Risk Factors

The risk of vascular malformation, such as malformation of lymphatic, capillary, or combination vessels, will increase if a baby is born from an older mother. Babies with certain chromodynamic disorders will also have a higher risk, such as in Down syndrome, Turner syndrome, and▁hatiin syndrome.

 

Symptoms

Limfatik Malformation

Most symptoms of lymphatic vessel malformation can arise and be seen since the baby is born. If symptoms have not arisen at birth, symptoms may begin to appear before the baby is 2 years old. Symptoms can vary on each child depending on the size of the malformation and where it lies in the body. Possible symptoms include:

  • Slow and gentle lumps or masses. These lumps occur most frequently in the neck, but they can also appear on the head, mouth, tongue, eyes, chest, stomach, arms, feet, or cretum (bags of sickles) and your child's penis.
  • Lumbulan or mass yang membesar dengan cepat. Hal ini mungkin karena adanya bleeding atau infeksi.
  • Signs of infection, such as redness, warm feeling when touching, pain, swelling, bleeding, and flow disorders in lymphatic vessels.
  • Malformation in the chest can cause difficulty breathing and swallowing.
  • Malformation in the eyes can cause difficulty seeing.

 

Capillary Malformation

Symptoms of capillary malformation can differ according to their type, namely:

  • Naevus symplectics, which usually appears to be small spots with a flat surface on the skin that is pink or pink with unclear boundaries. This type is commonly found on the neck (also mentioned stork bite, ethiteme nuchae), on the forehead between eyebrows (also mentioned angel's Kiss) or on the eyelids. This malformation will appear clearer when the child cries.
  • Port-wine statin, which is usually in the form of large purple or dark red spots with firm boundaries. When born, the surface of the patch is usually flat, but over time it will turn into waves. The area of the face becomes the location most often exposed even though spots can occur in any part of the body.

 

Combination Vascalar Malformation

Symptoms of combined vascular malformation can be a combination of several other symptoms of vascular malformation. This condition also often appears as a collection of symptoms (syndrom), such as:

  • Klippel-Trenaunay Syndrome (KTS), if there are two of the three criteria, namely port-wine statin, excess bone and/or soft tissue growth, and slow flow venous vessel / lymphatic malformation. Vessel malformation in KTS can be very broad and involve the bones, joints and muscles, as well as the underlying skin and fat.
  • Parkes-Weber Syndrome, which is very similar to KTS, but in this syndrome involves high flow arteriovenosa malformation on the arm or leg.
  • Blue Rubber Bleb Nevus Syndrome, or also known as Bean Syndrome, refers to the presence of several slow flow vessel maluformations in the skin and tissues below, as well as in the intestines and other internal organs.
  • CLOVES (Congenital Lipomatous Overgrowth, Vascular Malformations, Epidermal Nevi and Spine Deformities), which is a condition that affects babies and small children where excessive fat tissue is growing throughout the body. This disorder usually occurs in the body, legs, arms, head, or neck. Some children have dark red rashes that look like port-wine statin. CLOVES sufferers may also have spinal shape disorders such as scoliosis and high-flux arteriovenosa malformation affecting the spinal cord.
  • Heritary Hemorrhagic Telangectasias (HHT), or also known as Osler-Webe-Rendu Syndrome, ie sebuah kondisi yang diberitakan oleh orang tua kepada anak-anak yang ditandai dengan malformation pada urat nade kecil. Symptomsnya meliputi kurung dan munculnya sampah kulit, terutama pada palm tangan dan kaki.

 

Diagnosed

In diagnosing vascular malformation in lymphatic, capillary, or combination vessels, doctors will start by conducting interviews with parents or caregivers. Doctors will ask what symptoms have been experienced, since when symptoms arise, the history of the baby's condition at birth, as well as other child's medical history. Subsequently, doctors will perform physical examinations by looking directly at the area of the affected body. Doctors may also touch the surface of the affected skin area. Basically, the diagnosis of vascular malformation in lymphatic vessels, capillaries, or combinations can be enforced based on clinical findings from the results of interviews and physical examinations. However, to further ascertain the diagnosis and types of blood vessels exposed, doctors may conduct supporting checks, such as ultrasound examinations, X-rays X-ray, CT-scan atau CT angiography (Inspection CT-scan in blood vessels), and MRI.

 

Laksana Procedure

Limfatik Malformation

Treatment and malformation therapy of lymphatic vessels will depend on symptoms, age, general health of children, and how severe their condition is. Some of the options include:

  • Observation, where doctors will wait and monitor the development of malformation without special treatment. Doctors will monitor signs of infection, bleeding, or increased size
  • Medicines, such as giving antibiotics if you have an infection.
  • Badminton, to cut the masses or bumps that arise.
  • cyclootherapy, by giving a special injection into the mass to destroy it.
  • Laser therapy or radiofrequency abundance, to destroy the masses with lasers or radio waves.

 

Capillary Malformation

Capillary malformation is usually treated with laser therapy, like pulse dye laser, which directly targets capillary blood vessels in the skin.

 

Combination Vascalar Malformation

Procedures for combinatorial vascular malformation may require a wide range of combined therapies, such as:

  • Embolization, that is a procedure to cover abnormal blood vessels from the inside using a special 'lem' or particle.
  • Laser therapy
  • cyclootherapy
  • Badminton
  • Medicines

 

Complications

Limfatik Malformation

Malformation of untreated lymphatic vessels can cause complications that vary depending on size and location. Some of them include:

  • Large malformation in the neck or chest can interfere with breathing and swallow so that it can be life-threatening.
  • Bleeding
  • Infection
  • Disorders in the form of soft tissues and bones
  • Surgery can harm nearby tissue or cause bleeding.

 

Capillary Malformation

Capillary blood vessel malformation rarely causes serious complications. Malformation of this type more often causes aesthetic problems in sufferers.

 

Combination Vascalar Malformation

The compounds of vascular malformation will vary depending on the type. Complications tend to occur more often when they are large and many types of blood vessels are involved.

 

Prevention

Voscalar malformation in lymphatic, capillary, or combination vessels is a congenital disorder born and is often associated with genetic mutations so it is not yet known the exact way to prevent it.

 

When To Go To A Doctor

Consult your child to the doctor if you have symptoms of vascular malformation. Doctors will assist in determining the right diagnosis and implementation.

 

Want to know information about other diseases? Check here, yes!

 

 

Writer : dr Dedi Yanto Husada
Editor :
  • dr Anita Larasati Priyono
Last Updated : Kamis, 13 April 2023 | 22:58

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