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Background Bleeding and progressive coagulopathy are established symptoms of early cholestasis, including extrahepatic biliary atresia. We present a patient with bleeding secondary to vitamin K deficiency and cholestasis-induced fat malabsorption with unusual presentation of mediastinal mass. Objective Describe a case of late-onset vitamin K deficiency and cholestasis ¬¬presenting as jaundice and coagulopathy with associated bleeding into thymic structures Design/Method Case report Results A 4-week-old term male infant was brought to the ED with fussiness and bruising. Prior to presentation, he was afebrile and followed regularly with his primary care provider for jaundice below phototherapy thresholds. Infant received vitamin K at birth. In the ED, infant was tachypneic with retractions and grunting. Physical examination was notable for diffuse jaundice and two 1 cm bruises along shoulder and midline thoracic spine. No hepatosplenomegaly was noted. Chest x-ray was significant for mildly widened mediastinum. Labs demonstrated elevated WBC 19.68 x10(3)/mcL, Hgb 10.8 gm/dL, Platelet count 287 x10(3)/mcL. LFT notable for mixed direct/indirect hyperbilirubinemia with total bilirubin 11.1 mg/dL, direct bilirubin 5.6 mg/dL, indirect bilirubin 5.5 mg/dL. DAT was negative. Coagulation studies were significantly abnormal, with PT >125 sec and aPTT >250 sec; INR was unable to be completed. Fibrinogen was 406 mg/dL. A repeat set of coagulation studies were similarly abnormal. Infant received 2 mg vitamin K for three days, 50 IU/kg KCentra, and 15 mg/kg fresh frozen plasma. Factors VII and IX were low, factor VIII was high, and factor V was normal consistent with late-onset vitamin K deficiency bleeding. Coagulation studies normalized after these interventions. An echocardiogram and computed tomography scan of the chest were obtained due to respiratory distress identifying an anterior mediastinal mass bifurcating the thymus. Due to concern for solid appearance of the mass, a biopsy was completed demonstrating only normal thymic tissue, suggestive of bleeding into his thymic structures as cause of mass. He was able to be discharged with ongoing stability in coagulation labs but with ongoing fat malabsorption and persistent cholestasis. He followed closely with hepatology outpatient and was ultimately diagnosed with biliary atresia with Kasai procedure completed at 8 weeks of age. Conclusion Bleeding associated with Vitamin K deficiency and cholestasis may present variably in neonates, including intracranial bleeding, bruising, and coagulopathy; in this case, our patient presented with thymic bleeding and mediastinal mass. Vitamin K deficiency is correctable in the acute setting, however, early identification and further evaluation is indicated to address underlying causes of cholestasis.


Hematology | Pediatrics


Presented at the 2024 American Society of Pediatric Hematology/Oncology (ASPHO) Conference; April 2-5, 2024; Seattle, WA.

Early Cholestasis and Vitamin K Deficiency Secondary to Biliary Atresia presenting as Coagulopathy and Thymic Hemorrhage