At admission, she had tachycardia, tachypnea, grunting, prolonged capillary refill time, hypoxia (SpO2 85% on oxygen by prongs) and feeble pulses. Systemic examination revealed bilateral crepitations, grade III systolic murmur at upper right sternal border, and a palpable liver (2.5 cm). There was a large vascular malformation on the left thigh with splitting of overlying skin (Fig. 1). There was no other anomaly or dysmorphism. Investigations were suggestive of anemia (hemoglobin 7.5 g/dL) and severe thrombocytopenia (platelet count: 8×109/L). Total leucocyte count (13.3 ×109/L), serum electrolytes and serum calcium were normal; C-reactive protein was negative.

Fig.1 Large arterio-venous malformation affecting left thigh.

Fig. 2 Coronal image of CT angiography showing large soft tissue swelling of left thigh and multiple arterial feeders along with dilated femoral artery.

She received intravenous antibiotics, packed red cells, platelet concentrates and fresh frozen plasma. Intravenous furosemide was administered in view of congestive heart failure. By 12 hours of life, infant’s condition remained critical, and she required mechanical ventilation. X-ray chest showed cardiomegaly (CT ratio 0.7), and liver was palpable (3 cm below costal margin). Congestive cardiac failure further worsened, requiring digitalization and inotrope support to maintain perfusion. Echocardio-graphy revealed dilated right atrium, right ventricle and major pulmonary artery, mild to moderate TR, small ASD, and PDA. Doppler ultrasound of the enlarged left lower limb showed large arterio-venous malformation from left inguinal region including labia, extending upto foot. Computed tomography (CT) angiography of the affected left thigh revealed tufts of vessels in subcutaneous planes arising from superficial femoral artery, with enhancing soft tissue component and large draining veins sugges-tive of large arterio-venous malformation (Fig. 2). Urinary bladder was also grossly distended with bilateral hydronephrosis. Interventional radiologist suggested angio-embolization of the affected arterio-venous malformations, but the parents refused the procedure. Steroids were not administered because of fulminant sepsis. Child’s condition worsened and coagulation profile deteriorated further. She developed pulmonary hemorrhage and died on day four of life due to severe bleeding manifestations, overwhelming sepsis and cardiac failure.

Kasabach-Merritt syndrome, a potentially life-threatening coagulopathy characterized by enlarging hemangioma with severe thrombocytopenia [6], can complicate KTWS. Analysis of prenatal presentations and perinatal outcomes of KTWS suggested that the involvement of fetal thigh is rare [7]. Bilateral hydronephrosis is very rarely reported with KTWS [4]. Although urine output and renal function tests were normal, further workup of renal system involvement could not be done. Mortality rate in the neonatal period is high with complications of Kasabach-Merritt syndrome observed in about one-third of cases [7]. The causes of neonatal mortality in KTWS includes Kasabach-Merritt syndrome, cardiac failure, sepsis, and prematurity [7,8]. Treatment options include steroids, compression, pulsed-dye laser treatment, surgical reduction or embolization. Surgical therapy is often precluded by the risk of severe hemorrhage and high mortality in extensive lesions [7].