Principles of Treatment of Hemangiomas


Stage

Clinic

CCDS

I. Prodromal phase

Red/white spot; telangiectasia

Structureless; low-echo space

Blurred vision; swelling

No signs of pathological vessels

II. Initial phase

Loss of typical skin structure

Hyposonoric center

Increasing thickness and induration

Hypervascularization beginning at edges

III. Proliferation phase

Bright red cutaneous infiltration; flat spreading

Increasing intratumoral hyperperfusion

Subcutaneous growth of thickness;

Central vessel density

Infiltration of surroundings possibly even at organ borders

Nutrition of tumor vessels

Possible early primary central ulceration

Drainage veins with arterial flow profile

IV. Maturation phase

Pale and livid color

Declining central vessel density

Possible central exulceration

Increasing ectatic drainage veins

Decreasing growth

Declining arterialization of drainage veins

Possible late ulceration over drainage veins

Central increasing hypersonoric aspect

V. Regression phase

Hypopigmentation; wrinkled skin/telangiectasis

Circumscribed hypersonoric area

Surrounding subcutaneous drainage veins

Loss of typical tissue structure

Subcutaneous palpable induration

Nearly no central tumor vessels

Residuals supplying tumor arteries

Residuals of ectatic drainage veins


Correlation of clinic and CCDS. The diagnosis itself is clinical. But for a correct definition of the stage and the involved organ structures, CCDS is mandatory




Spontaneous Course


Because hemangiomas may involute spontaneously, waiting for spontaneous regression remains a viable therapeutic option. But the “wait-and-see” principle is always wrong (Table 10.2). If it means that treatment arrives too late, then “see and wait” as a control is correct, because at the first sign of progression of complication an action can be taken. Therefore, in case of small, uncomplicated infantile hemangiomas in non-problematic areas (extremities, body) without any tendency to proliferate, especially in cutaneous hemangiomas, one can “see and wait.” If delayed growth cannot be excluded, frequent controls are required. Clinical checkups alone may not be sufficient, as subcutaneous infantile hemangiomas may remain unnoticed as they grow deeply and are then only recognized after complications result. This is why a periodic duplex scan control is mandatory. For hemangiomas in the quiescent or regression phase, a “see-and-wait” attitude should normally be recommended. However, if complications are expected from ulcerations, treatment is also required for these forms. As therapy may cause adverse systemic or cutaneous side effects, particularly scarring, sometimes intervention has been reserved for patients with significant complications. Therefore, it is difficult to choose a therapy that eliminates hemangiomas before the development of complications and without systemic side effects [2]. For this reason, the following infantile hemangiomas must be considered as “problem hemangiomas,” for which active treatment is mandatory (Table 10.3):


Table 10.2
Grade 1 means uncomplicated hemangioma with no risks; Grade 2a hemangiomas with low risk factors need controls; Grade 2b with risk factors needs close controls and laser therapy if any progress is observed; Grade 3 has a laser indication due to risks of complication; and Grade 4 requires additional systemic therapy
























































Grade

Definition

Procedure

G1

Uncritical region/organ

No treatment

No action

Signs of/or final regression

No control

G2a Control

Uncritical region/organ

Control; if no progress or sign of regression, follow G1
 
No expectation of progress

If with slight progress follow G2b

G2b Close control

Although no progress expected but critical region/organ

If with slight progress and no complication, close control

If with major progress or slight progress but with complication, follow G3a
 
Uncritical region/organ but progress expected

Follow G3a

G3a Laser treatment

Progress, in case of further progress complication expected

If progress or no progress but remaining hemangioma causes functional impairment; start laser therapy to prevent complication
 
No further progress, but critical region/organ complication expected

If regression starts, follow G2b

If complication occurs, follow G3b

G3b Add. systemic treatment

Critical region/organ complication has occurred with early regression/reduction necessary

If primary complication due to critical localization and/or biological aggressivity occurs, perform additional laser therapy systemic therapy
 
Monotherapy not successful

If stabilization achieved, follow G3a



Table 10.3
Therapeutic algorithm for infantile hemangiomas and congenital hemangioendotheliomas

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The symbols are according to ISO 9000. The grading for infantile hemangioma is given in Table 10.2. Abbreviations: DIC disseminated intravascular coagulopathy, CCDS color-coded duplex sonography, cHE congenital hemangioendothelioma, iH infantile hemangioma, RICH rapid involuting congenital hemangioendothelioma, NICH non-involuting congenital hemangioendothelioma, KHE kaposiform congenital hemangioendothelioma, Thermogr. infrared thermography

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Sep 20, 2016 | Posted by in HEMATOLOGY | Comments Off on Principles of Treatment of Hemangiomas

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