Anaesthetic management of a neonate with Kasabach-Merritt syndrome
A 2.5 kg 1-month-old boy, delivered at term, afflicted with KMS presented for excision and split skin grafting of a giant haemangioma over the left thigh, extending from the inguinal region to the knee, with a circumferential girth of 32.5 cm []. Patient had received intravenous propranolol[] and prednisolone[] for 10 days and intralesional bleomycin injection once, which produced a slight, but insignificant reduction in the size of haemangioma. Apart from pallor, examination was unremarkable and there were no other co-morbidities. The patient had been transfused 150 mL of packed RBCs, 250 mL platelet concentrates and 110 mL of fresh frozen plasma but the anaemia, thrombocytopenia and coagulopathy persisted. Pre-operative haemoglobin was 7.6 g/dL, platelets 19,000/cumm, bleeding time 9 min and international normalized ratio (INR) was 1.6. Other biochemical investigations including arterial blood gas analysis were all within normal limits. Appropriate radiological investigations were carried out to rule out haemangioma in other visceral organs.\nIntravenous access was secured with two 24 G intravenous cannulae. The neonate was pre-medicated with injection hydrocortisone 10 mg IV and injection fentanyl 2.5 μg IV. After standard induction using sevoflurane and atracurium, an uncuffed endotracheal tube, size 3.0 mm ID was inserted. Paracetamol 70 mg suppository was administered for post-operative analgesia. Normothermia was maintained with the use of Mediprema® Infant Operating Table and HOTLINE® Blood and Fluid Warmer. Since, the application of a conventional tourniquet was technically impossible in an attempt to minimize blood loss, we requested the surgeon to tie a Foley's catheter around the proximal most part of thigh (tourniquet time 35 min). The haemangioma was excised and raw area was covered with a skin graft taken from the excised haemangioma itself, in an attempt to further decrease the blood loss. Intraoperative blood loss was 300 mL and was appropriately replaced with 150 mL packed cells, 50 mL fresh frozen plasma and 50 mL platelet concentrates. Patient was haemodynamically stable throughout the procedure and had adequate urine output. Arterial blood gas analysis and serum electrolytes performed prior to extubation were within normal limits. Recovery from anaesthesia was smooth and uneventful. In the post-operative period, patient received additional 200 ml of packed cells and 50 mL of platelet concentrates. On the second post-operative day, the haemoglobin was 9.9 g/dL, platelets had increased to 64,000 cells/cumm and INR had improved to 1.3. Further post-operative course was uneventful.