Superficial Vessel Anomalies

This article will discuss superficial vein anomalies that are frequently encountered in infants and children.

1- What are Lymphangiomas (Lymphovenous Malformations)?

Lymphangiomas are an anomaly resulting from the development of the lymphatic system being stalled and characterized by the expansion of lymphatic vessels. Today, they are called lymphatic malformations. They are the second most common benign vascular tumor of childhood. Their frequency is seen in approximately one in 60,000 babies. The frequency is equal in girls and boys. There is no familial predisposition.

What is the Lymphatic System? and What is its Function?

The lymphatic system, or lymphatic system, is an organ system formed by the lymphatic transport system and lymphoid tissue. The lymphatic system, which can be defined as a second circulatory system, is still very different from the circulatory system in terms of its structure. It works independently of the circulatory system, but in the last part it is connected to the general systemic circulation via veins. In general, it has two functions: to transfer interstitial fluids to the general circulatory system and to contribute to the body's immune system.

 

Are There Types of Lymphangioma?

Yes, there are; 1- Lymphangioma simplex, 2- Lymphangioma cavernosus 3- Cystic hygroma. All three of these are most commonly seen in the neck region. Since cystic hygroma is the most common, cystic hygroma will be discussed first.

Cystic hygromas (CH) are multilocular cystic (in the form of multiple cysts) structures lined with endothelial cells. Approximately 60% of cases are present at birth and are noticed. Approximately 80-90% are noticed by the age of 2. Approximately 70% of cases can be seen in the neck, and the rest in any anatomical region of the body. 20% of cases can be seen in the armpit, 5% in the breast tissue, abdomen, mediastinum, orbit, parathyroid and extremities. Cystic lymphangioma can be seen in only one region or in multiple regions. These lesions, which are generally small, can sometimes involve large areas.

How Does Cystic Hygroma Form?

During the 5th week of pregnancy, the baby's lymphatic system begins to form and develop. On the 40th day of pregnancy, a connection is formed between the vein and the previously formed lymphatic system. This connection takes on the task of draining the fluid in the lymphatic system into the venous system. If this connection is not present, cystic hygroma occurs. It connects to the fluid accumulation in the lymphatic system in the neck of the fetus and the cystic structure in question forms.

How is Cystic Hygroma Diagnosed?

Diagnosis is made by history and physical examination. In the history, cystic swelling is seen in the neck during an ultrasound performed in the womb and is suspected.

After the baby is born, the doctor and/or the family notices a swelling in the neck and the diagnosis is made during an ultrasound. Patients should be investigated for any other location. If it is very large and extends deep into the neck, MRI (Magnetic Resonance) imaging should be performed to determine its relationship with neighboring organs. Cystic hygroma or mesenteric lymphangiomas located in the abdomen can often be asymptomatic and are detected by ultrasonography during examinations for other reasons. Sometimes they can cause recurrent abdominal pain, and very rarely, they can cause acute abdomen due to rotation of the intestine, intussusception or perforation. And emergency surgery may be required.

 

 

 

Images: Lymphangioma Appearances in Different Anatomical Localizations

Cystic Hygroma and Chromosome Disorder

Cystic hygroma, which is rarely seen in infants, can also be accompanied by some chromosomal anomalies. Cardiac anomalies and structural anomalies can also be seen with this condition.

How is Cystic Hygroma Treated?

In the treatment, medication is applied or it is removed with surgery. The location and structure of the cyst and the age of the patient determine which one to apply. If the cyst is single, small and can be removed without damaging the neighboring organs, it can be excised. In addition, drug treatment can be preferred in cases that recur after surgery.

Local drug treatment can be applied, which should be preferred, especially in cysts that are widespread and surround neighboring organs. In recent years, it has been reported that successful results have been obtained with bleomycin and picibanil (OK-432) sclerosing treatment applications in the treatment of lymphangioma. Sometimes laser and even radiation therapy may be required in these patients. However, the last two applications have serious complications. The structure and location of the cyst, the patient's age, the relationship of the cyst or cysts to vital organs, and the physician's experience determine which treatment option to apply.

What are the Complications of Surgical Removal of Cystic Hygroma and Drug Treatment?

When removed surgically; if the cyst or cysts are injured by vital organs or infiltration, nerve damage, long-term lymphatic drainage requirement, infection, scar formation and recurrence, aesthetic problems may cause complications. In addition, recurrence after surgery is around 5-15%. Especially in multiple ones, it may not be possible to remove all of the cyst or cysts.

There is no serious complication detected with OK-432 treatment yet. However, Bleomycin may have complications, although very rare. These complications may occur in the short or long term, you should definitely be informed by your doctor about this.

!!! The above treatment methods for mesenteric lymphangiomas located in the abdomen can be performed by laparoscopy or open surgery.

2- Hemangiomas

What is Hemangioma?

It is a benign tumoral structure characterized by the proliferation of endothelial cells found in the innermost layer of the vessels. Having a tendency to bleed and being in the vicinity of vital organs can be vital. It is usually seen in superficial capillaries (capillaries). Its incidence is approximately 1 in every 100 live births, this rate is more common in low birth weight and premature babies.

Hemangiomas, which are tumors of vascular origin that grow by cellular proliferation, are the most common tumors of childhood. They constitute 7% of all benign tumors. It is present in one in 10 children around the age of one. These lesions, which are usually not seen at birth, are observed in the first few months of life. 70-90% are seen in the first 1-4 weeks. 80% are single, 20% are multiple lesions. Hemangiomas show a proliferation, then stabilization and finally involution (regression) phases. Around 50% of cavernous hemangiomas undergo involution around the age of 5, 70% around the age of 7, and others around the age of 10-12. The earlier the involution begins, the more complete it is.

Hemangiomas may be located in the skin, internal organs, or both. It is rare in blacks. 60% are located in the head and neck, 25% in the trunk, and 15% in the extremities. The female/female ratio is 3/1 or 4/1.

 

 

 

Figure: Various examples of hemangiomas

Why Does Hemangioma Occur?

It is a congenital anomaly. It is not known exactly how it occurs. Because some of these lesions are present at birth while others appear within the first few weeks of life. There are a number of theories on this subject. As a result, hemangiomas emerge as a developmental defect of the embryonic mesoderm of peripheral blood vessels. Hemangiomas are a condition characterized by the proliferation of endothelial cells, the innermost layer of the vessels. It is thought that this is caused by some angiogenetic factors.

Are There Types of Hemangioma?

Yes, there are. Hemangiomas can be very simple anomalies, as well as very complex anomalies accompanied by syndromes. The most common types are as follows;

• Port-wine stain; superficial stain like
• Cherry-strawberry; small vascular moles
• Large vascular mole (cavernous hemangioma) very large
• Rose spot (spider hemangioma)
• Angel kiss (looks like a spot on the forehead)
• Organ hemangiomas (liver, brain etc.)
• Stork bite (seen on the nape of the neck, very common, heals on its own)
• Hemangiomatosis (covering almost the entire body)
• Red moles on the body; are red dots that are in the chery class, occur mostly on the trunk, and appear after the age of 40.

Strawberry Hemangioma:

The most common congenital scars, seen at a rate of 4-5%. Usually 70% are noticed at birth, the rest are seen in a few weeks during the neonatal period. The rate is higher in premature babies. The F/M ratio is like 2/1 or 3/1. It is rare in black race. 56% of all strawberry hemangiomas occur in the head and neck, 23% in the trunk, 19% in the extremities and 2% in the genital area.

In general, strawberry hemangiomas grow rapidly in the first 6-8 months, plateau in 6-12 months and regress in 12-18 months. Regression is characterized by lightening of the color in the center of the lesion and a blue-gray color change; and its gradual spread to the surrounding area. In a study, it was found that 49 of 77 patients with 82 hemangiomas had their hemangiomas completely; It was determined that 38 of them partially disappeared. Different authors gave different rates for regression that will occur in the first 5 years. Wallace 97%, Simpson 55%, Bowers 98%, Walter 96%.

How is Hemangioma Diagnosed?

It is usually diagnosed visually during physical examination. However, it should be distinguished from similar formations. Hemangiomas usually appear in the few weeks or months following birth. It should definitely be distinguished from structures such as telangiectatic nevi, A-V malformations and pyogenic granuloma. Hemangiomas continue to proliferate and grow after they are first detected, but they later begin to regress. This is not the case for other pathologies. Additional examinations are usually not required for superficial pathologies, but doppler ultrasound, magnetic resonance and angiography may be required for large and complex structures. These examinations are performed especially for deep lesions.

Does Hemangioma Grow?

Yes, it can grow like an independent organ, but it may not grow and may shrink on its own later.

What Complications Can Hemangiomas Cause If They Are Not Treated?

It usually does not cause any effect other than cosmetic problems. However, it can cause bleeding in 10-20%, ulceration in 5%, as well as infection, necrosis, respiratory distress, heart failure, blood clotting problems, thrombocytopenia and additional problems depending on the anatomical location of the lesion.

How is Hemangioma Treated?

The treatment is determined by the type of lesion, its behavior, anatomical location and whether it will regress on its own.

Treatment options include; monitoring, compression, injection of sclerosing agent, radiotherapy, chemotherapy, laser, embolization and surgical removal.

In Some Cases, Can Treatment Be Required Without Waiting for the Regression Phase?

Yes, it may be necessary, we call these Early treatment indications;

Rapid tissue destruction during the growth phase
Danger of obstruction of some formations; Airway, oral cavity, etc.
Any obstruction in the visual field
Disseminated intravascular coagulopathy may develop due to thrombocytopenia in massive hemangiomas. (Kasabach-Merritt Syndrome)

Treatment alternatives:

Observation: 90% of the cases will regress during the observation period. Opaque cosmetic creams (Covermark) can be tried in the meantime.
Corticosteroid treatment: Systemic steroid treatment should only be used in selected infants with hemangiomas.
This treatment:

Those with cervicofacial lesions causing distortion
Lesions showing recurrent bleeding, ulceration and/or infections
Patients with impaired physiological functions (such as vision, respiration)
In platelet depletion coagulopathies (Kasabach-Merritt syndrome)
It should be used in cases complicated by high cardiac output.
A hemangioma in the proliferative phase is more sensitive to this treatment than one in involution.

Corticosteroid treatment scheme:

Prednisone (Prednol tablet [4 and 16 mg] or Deltacortil tablet [5 mg] 2-3 mg/kg) is administered orally for 2-3 weeks.

If there is a response; (softening, lightening of color, slowing of growth within 7-10 days) The dose is reduced to 1 mg/kg or given every other day. Then the dose is reduced to 0.75 mg/kg.

Steroids are generally applied for 4-6 weeks. A break is given. Re-treatment varies according to age, response to the first treatment, and localization. If necessary, a second course of 2-3 weeks is given at 1 mg/kg daily or every other day.

If there is no response or no change in the lesion, treatment is discontinued.

Treatment is not required for hemangiomas that have undergone involution.

Compression Therapy:
In some cases, it has been argued that compression therapy can accelerate the regression of the hemangioma.

It is simple, non-invasive, and effective.

Surgery:
When the decision for active treatment is made, surgery is the most effective treatment. Small lesions can be excised. Diffuse cavernous hemangiomas under the lip mucosa can be ligated circumferentially with multiple chronic catgut to create premature involution. In other lesions, closure with a graft or flap is required after excision.

Laser Treatment:

Some successes have been achieved in the treatment of strawberry-type hemangiomas using argon laser. Surgery is necessary to remove the contracted lesion later. Laser treatment is more effective in superficial capillary hemangiomas.

Radiation Therapy:
Although radiation is strongly recommended in the treatment of some hemangiomas, there is not much evidence supporting success in safe doses. However, regression is considerably accelerated in doses exceeding 800 rad, but radiation dermatitis, late malignant degeneration, suppression of dental epiphyseal, breast and gonadal development are possible.

Cryotherapy;
Partial treatments can be made in superficial lesions with liquid air carbon dioxide snow, dry ice sticks, ethyl chloride. However, cryotherapy is applied in the treatment of intraoral and deeply invasive hemangiomas. Excessive blood loss can be prevented with cryotherapy.

*** The information provided here, the content of the website, is arranged for the purpose of informing visitors, especially families. No information should be considered as advice by visitors and should not lead to any decision or action. The patient should definitely be examined by a pediatric surgeon, and a decision should be made by consulting him/her and consulting his/her personal knowledge.