December, 2016/ Vol 4/Issue 12 ISSN- 2321-127X Case report International Journal of Medical Research and Review Available online at: www.ijmrr.in 2176 | Page Neonatal Kasabach-Merritt Syndrome (KMS): case report Rathia SK 1 , Phuljhele S 2 , Gandhi A 3 1 Dr. Santosh Kumar Rathia, Assistant Professor, Department of paediatrics, Pt. JNM Medical College, Raipur; 2 Dr. Sharja Phuljhele, Professor and Head, Department of Paediatrics, Pt. JNM Medical College, Raipur; 3 Dr. Animesh Gandhi, Junior Resident, Department of Paediatrics, Pt. JNM Medical College and Dr. Bhim Rao Ambedkar Memorial Hospital, Raipur (C.G), India. Address for Correspondence: Dr. Santosh Kumar Rathia, Email: drsantoshrathia84@gmail.com ……………………………………………………………………………………………………………………….............. Abstract A 2days baby girl presented with congenital reddish-blue swelling over chest and abdomen with thrombocytopenia. The clinical, imagining and laboratory findings suggested the diagnosis of KMS. Oral steroid was started with initially needed platelet and FFP transfusions. Large thoracic hemangioma was non-amenable to surgical and radiological interventions. Vincristine was initiated after 2week trial of Prednisolone for persistent thrombocytopenia and non-regressing haemangioma. Over a week the lesion shrunk with improving platelet count. Vincristine was stopped after 4weeks in view of no further tumour shrinkage. The patient was discharged on steroid advised for 6-12 months and showed significant tumour regression at 3month. Key words: Kasabach-Merritt syndrome, haemangioma, thrombocytopenia, corticosteroid, vincristine ……………………………………………………………………………………………………………………….............. Introduction Hemangiomas constitute the commonest paediatric neoplasm. The management of these benign lesions not only depends upon their size and site but also on vascular characteristics or certain complications [1]. The triad of hemangioma, thrombocytopenia and consumption coagulopathy is known as Kasabach- Merritt phenomenon [1]. Hypotheses suggest that exposure of sub endothelial elements or abnormal endothelium within the haemangioma results in aggregation and activation of platelets with a secondary consumption of clotting factors [2]. This case report describes a neonate with Kasabach- Merritt syndrome (KMS). The clinical presentation of the case and its outcome with possible treatment modalities has been discussed in the light of similar cases/studies reported previously [3–8] Case Report A female baby born to a non-consanguineous couple presented with a diffuse reddish blue swelling over chest and upper abdomen, present since birth. Although normally delivered at term with no perinatal insults/ trauma, the baby got admitted in private nursery in view of suspected hematoma or ? vascular lump (see figure 1). She was referred from there on 2 nd day to our NICU to rule out bleeding diathesis with thrombocytopenia (leading to enlarging hematoma). On examination at admission, baby had mild respiratory distress with RR-68/min, HR- 152/min, CFT<3sec, pulse- normal, Spo2- 99% with O2 with minimal retractions, but no lethargy. On local examination- reddish blue diffuse swelling with few petechiae was seen over chest and abdomen, with predominant bulge over right upper chest; the swelling was smooth, firm, non tender and warm to touch. Abdominal/other systemic examinations including growth parameters were normal. There were no dysmorphism/organomegaly/neurocutaneous markers. Manuscript received: 04 th December 2016 Reviewed: 12 th December 2016 Author Corrected: 20 th December 2016 Accepted for Publication: 31 st December 2016