Presentacion Hemangiomas Infantiles.pptx

Presentacion de los hemangiomas infantiles mas comunes

1. Infantile Haemangioma The Cardiac Counseling Tips Reza Miri , MD, Fetal-Youth Cardiologist

2. Infantile Haemangioma(IH ) is The most common benign tumours of infancy

3. clinical course 1) A rapidly dividing endothelial cell proliferation followed by 2) Involutional phase, most IH are resolved by age 9 yr.

4. IH Pathology Composed of proliferating plumped endothelial cells

5. Most small lesions resolve spontaneously without any or with minimal scarring. Large lesions usually leave behind residual life-long lesions : Telangiectasia, Fibro-fatty Deposits, Depigmentation ,Scars Residual changes are seen in 25 to 69% of untreated IH

6. The treatment History in 2008 when a child with secondary HCOM ( induced by systemic corticosteroid) managed with propranolol , the patient nasal capillary IH rapidly regressed

7. Treatment aims Stopping the growth Preventing the sequel Life-threatening HI (causing heart failure ,respiratory distress) Functional risks (visual obstruction, amblyopia, feeding difficulties) Severe anatomic or cosmetic distortion( on the face) Significant complications such as thrombocytopenia Ulceration, bleeding, obstruction of vital structures . Treatment Indications

8. The first line of the medical treatment are Beta Blockers The treatment modalities different based on the morbidity other complementary l therapeutic options include Wound care Topical antiseptics Antibiotics Corticosteroids Lasers

9. Beta-blockers & Angiogenesis Beta-blockers Decrease Vascular Endothelial Growth Factor (VEGF)

10. Beta-blockers Cardiac Counseling ?

11. Cardiac Counseling ? CHD are more common than the general population

12. CHD & IH . 1)From July 2009 through January 2013, 239 consecutive patients 12 months of age or younger, 50 patients (21%) had an abnormal echocardiogram: 39 ASD,6 VSD, 2 PDA, 1 COA, 1 PS, 1 ARSA. Two of seven patients with PHACE syndrome had cardiac anomalies. 2)Thirty-nine of 155 patients had structural abnormalities (26.7%), 9 had VSD, 9 ASD; 8 PDA, 6 PS, 6 MR, 2 AI (BAV), one COA ,1 mesocardia 18 aneurysm of the IAS . the percentage of reported CHD is slightly different, may be related to patient age ; which may change the number of abnormalities detected, as the majority of cases of PDA, ASD, and small VSD close naturally over time.

13. Cardiac Counseling ? Cardiac contraindication to use propranolol: Low BP Arrhythmia: Sick sinus syndrome, Heart block (2nd or 3rd degree) Myocarditis Significant AS Coa Evaluation for Cardiac failure result of the high flow within the lesions during the proliferation phase with increase in shunting Visceral Involvement more than 5 cutaneous lesions =increased chance of visceral IH

14. Cardiac Counseling ? Cardio vascular anomalies are part of the PHACE syndrome P osterior Fossa Malformations Hemangioma Arterial Anomalies Coarctation Of The Aorta And Cardiac Defects E ye Abnormalities Occasionally Sternal Defects PHACE(S) 70% of affected patients have only 1 extracutaneous manifestation

15. Cardiac Counseling ? Special attention is required in patients at risk of cardiac compromise Large Hemangiomas PHACES Syndrome PELVIS Syndrome (lumbosacral or genital area hemangiomas) Miliary Hemangiomatosis

16. Cardiac Counseling ? Consumptive Hypothyroidism Probability reported with hepatic & bulky cutaneous IH it is appears be secondary to high activity of the type 3 iodothyronine deiodinase enzyme in hemangioma tissue, which is responsible for degradation of T4 to reverse T3 . Thyroid function tests should be ordered in the appropriate clinical setting

17. Cardiac Counseling ? The Ball's In Cardiologist Court Parents Awareness about beta blocker adverse events : paleness, sweating, reduced feeding, agitation,bronchospasm Guide discontinuation the beta-blocker cardiac hypersensitivity may occur 24-48 hr. after propranolol discontinuation Infants increase their weight and thus need the dose adjustments Beta blocker selection

18. Beta-blockers selction Propranolol is a lipophilic nonselective beta-blocker that crosses the blood-brain barrier , sleep disturbances have been associated with its use Being less frequent with hydrophilic drugs such as atenolol and nadolol. Acebutolol, Atenolol are selective cardiac beta-blockers and may decrease possible respiratory side effects Atenolol??

19. The pretreatment cardiac evaluation Physical examination of the heart, lungs Heart rate Blood pressure Electrocardiography Echocardiogram ( PHACE syndrome, other high risk situation) HR & BP are monitored after every dose escalation.

20. The dosing recommendation Propranolol at 1 mg/kg in 2 divided doses under pediatric observation for 2 hr. monitoring BP,HR,1 and 2 hours after the first dose The dose is then gradually increased , up to 2 mg/kg daily the second week of treatment up to 3 mg/kg daily the third week

21. Beta-blockers side effects Mean blood pressure reduction with treatment is significant only after achieving the maintenance dose 2 mg/kg body weight The hypotension and bradycardia seen is often asymptomatic Bradycardia is often associated with the first dose These follow-up evaluations should consist of: monitoring for symptoms secondary to the beta-blocker dosage adjustment for weight gain

22. Beta-blockers side effects Hypoglycemia Bronchospasm/Bronchiolitis Restless Sleep Nightmares Agitation Diarrhea Peripheral Coldness hypothermia

23. continuation of the beta-blocker The shrinkage is rapidly observed with systemic or /and topical beta blockers about 4 weeks Treatment is continued for at least 3 mo prior to determining that there is no effect from the beta-blocker If there is a significant decrease in size of the hemangioma,the drug continued for 6 months or longer after the end of the proliferation phase to prevent rebound growth ,. If there is a significant decrease in size of the hemangioma but not complete resolution, this can be continued for 12 mo. Consider a gradual taper over 2 weeks, rather than abrupt discontinuation. Cardiac hypersensitivity (tachycardia) may occur 24-48 hours after propranolol is discontinued (peaks at 4-8 d).

24. The other medicine ACEIs and ARBs Block Transforming Growth Factor β ( angioproliferative properties) Regulatory Role Of AT I & AT II In Promoting Cell Proliferation

25. The lab studies Complete Blood Cell Count Liver Function Tests Coagulation Studies Urinalysis Stool Examination For Occult Blood Thyroid Function Test

26. Imaging & IH Imaging is not necessary unless: the diagnosis is uncertain there are five or more cutaneous infantile hemangiomas present there is suspicion of anatomic abnormalities Chest Radiograph Abdominal Imaging CNS Imaging MRI with and without intravenous gadolinium is the imaging modality of choice to delineate the location and extent of both cutaneous and extracutaneous hemangiomas.

27. IH Risk Factors 1) Hypoxic stress which modify gene expression stimulating angiogenesis 2) Embolization of placental stem cells to receptive fetal tissues supported by the unique immunohistochemical similarities between the placenta and the IHs endothelium Multiple Gestations Female Sex (3:1 Ratio) Maternal Age In Vitro Fertilization Low Birth Weight(23% For Born At <1000 G ,1–4% For Nl) Prematurity Gestational Hypertension Pre-eclampsia Gestational Diabetes Mellitus Use Of Progesterone During Pregnancy Invasive Antepartum Procedures ( 21% Of Cases) Placental Ischemic Abnormalities

28. Thank You