Research Article: Thrombotic Storm With Budd-Chiari Syndrome in a Pediatric Patient With Ulcerative Colitis

Date Published: October 25, 2019

Publisher: Wolters Kluwer

Author(s): Rohit Josyabhatla, Diane Hsu, Michael McGuire, Sharon D’Mello.


Ulcerative colitis is associated with an increased risk of thromboembolic phenomena. Thrombotic storm defined by the development of multiple thrombi in multiple locations within a short period of time is a rare condition that is potentially life threatening. We present a 14-year-old adolescent boy with an ulcerative colitis flare complicated by Budd-Chiari syndrome and thrombotic storm.

Partial Text

Ulcerative colitis (UC) is a type of chronic inflammatory bowel disease (IBD). Multiple genetic and environmental factors have been implicated as probable triggers.1 IBD often predisposes patients to a procoagulant state, leading to thromboembolic events (TEs).2 Multiple factors including thrombocytosis, upregulation of coagulation factors, downregulation of fibrinolytic factors, endothelial dysfunction, and the presence of procoagulant microvesicles have been suggested as possible explanations.2 Episodes of acute severe UC require hospitalization that is often accompanied by dehydration, immobilization, infection, and possible surgery, which further increase the risk of thrombosis.3 We present a 14-year-old adolescent boy with UC who presented with acute severe colitis and went on to develop Budd-Chiari syndrome (BCS) with thrombotic storm (TS).

A 14-year-old adolescent boy with UC presented with a 2-week history of multiple episodes of bloody diarrhea, tenesmus, rectal pain, fever, fatigue, and 10-pound weight loss. He was diagnosed with UC 3 years before presentation, and his course so far had been uncomplicated while on treatment with sulfasalazine. He was admitted for management of his acute severe colitis. His hospital course was complicated by ongoing hematochezia, severe anemia (hemoglobin 8.3 gm/dL), and hypoalbuminemia (2.2 g/dL). Early in his course, he developed chest pain, shortness of breath, and hypotension refractory to fluids. Chest x-ray showed interstitial markings and small pleural effusions with a paucity of vascular markings. Electrocardiography showed ST depression in the inferior leads. Troponin (8.06 ng/mL) and brain natriuretic peptide (1,440 pg/mL) levels were elevated. Echocardiogram revealed mild left ventricular dilation with reduced ejection fraction of 40% with normal right ventricular size and function. The findings were attributed to demand ischemia. He was treated with blood transfusions and isotonic crystalloid infusions. Methylprednisone was initiated to better control the UC flare, and piperacillin/tazobactam was empirically added to cover for possible bacterial colitis. Colonoscopy was performed, and the colon biopsy showed ulcerated granulation tissue consistent with active colitis. The biopsy was negative for cytomegalovirus immunostaining.

A study by Nylund et al found that the relative risk for thromboembolism in hospitalized children and adolescents with IBD was 2.36 when compared with hospitalized children without IBD.4 One large retrospective analysis of a single hospital database found that thromboembolic complications occurred in 1.3% of the study population with UC.5 They reported that deep vein thrombosis (DVT) and pulmonary embolism were the most common culprits contributing to 60% of the cases, with a mortality rate as high as 25%. In pediatric IBD, a recent systematic review of the literature found that UC was more frequently associated with TE than with Crohn’s disease.6 They also found that the most common sites for TE in pediatric IBD were cerebral (54.3%) and limbs (26%), while abdominal TE contributed to 13%. Only 6 pediatric cases of BCS with UC have been reported so far.7–12 What made our patient unique was the concurrent development of portal thrombi, hepatic venous thrombi, and renal infarcts.

Author contributions: R. Josyabhatla wrote the manuscript. D. Hsu edited the manuscript. S. D’Mello revised the manuscript and is the article guarantor.




Leave a Reply

Your email address will not be published.