Hemangioma and Shock

An 11 months old, one of twin girls of Syrian Nationality whose other twin is normal was admitted to our hospital 8 months ago and I was called for consultation. I saw her and took over her care. She was admitted as a case of haemangioma involving both sides of the head, neck and shoulders anteriorly down to the clavicles and posteriorly to the lower part of the scapula. Before coming to our hospital she was seen at variable private clinics and hospitals in the city and the parents were told that this a self limiting condition and does not need any treatment. My first impression when I saw her was Kasabach-Merritt syndrome. I put her on prednisolone tablets 2mg per kg per day in 3 divided doses. Her weight at that time was 5kg. She was discharged from the hospital on prednisolone same dose as above. Unfortunately the parents stopped the prednisolone tabs without medical advice. Soon after that the hemangioma increased rapidly in size and she was brought again few days later to our hospital ER in a state of shock. Her Hemoglobin was 7 and the platelets were in the 70,000 range. She was admitted to the ICU, ventilated for about 1 week and weaned to Oxygen by nasal prongs 2 liters per min and till now maintaining saturation of 100 percent. I don`t have a facility to give drugs like interferon or methotrexate. Her weight now is only 4.4kg, she is on ensure formula and taking 70 to 80 ml every 3 hours. She is hemodynamically stable, with BP 90 by 60mmHg, capillary refill time of 2 seconds, she has a grade 2 systolic murmur over the precordium {Echocardiography not done}. There is no hepatomegaly or splenomegaly. Her respiratory system and CNS were not remarkable. Her hemangioma is obliterating the left ear completely and now extending to the lateral margin of the ipsilateral eye. The CT scan brain with contrast showed vascular malformation involving left hemifacial and scalp with abnormal intracranial draining veins. No leptomeningeal affection. No choroidal hemangioma {Intra orbital affection}. CT scan abdomen with contrast showed no hemangioma.