Korean J Gastroenterol.  2016 Apr;67(4):220-223. 10.4166/kjg.2016.67.4.220.

Hepatic Hemangioma with Kasabach-Merritt Syndrome in an Adult Patient

• Oak CY
• Jun CH
• Cho EA
• Lee DH
• Cho SB
• Park CH
• Joo YE
• Kim HS
• Rew JS
• Choi SK

• KMID: 2373429
• DOI: http://doi.org/10.4166/kjg.2016.67.4.220

Abstract

Hemangiomas are the most common benign tumors of the liver. They are generally asymptomatic, but giant hemangiomas can lead to abdominal discomfort, bleeding, or obstructive symptoms. Kasabach-Merritt syndrome is a rare but life-threatening complication of hemangioma, characterized by consumptive coagulopathy with large vascular tumors. More than 80% of Kasabach-Merritt syndrome cases occur within the first year of life. However, there are few reports of Kasabach-Merritt syndrome with giant hepatic hemangioma in adults and, as far as we know, no reports of Kasabach-Merritt syndrome with hepatic hemangioma treated with first line medical treatment only. The most important treatment for this syndrome is removal of the large vascular tumor. However, surgical treatment entails risk of bleeding, and the patient's condition can mitigate against surgery. We herein present a case of unresectable giant hepatic hemangioma with disseminated intravascular coagulopathy. The patient was a 60-year-old woman who complained of hematochezia, ecchymosis, and abdominal distension. She refused all surgical management and was therefore treated with systemic glucocorticoids and beta-blockers. After two weeks of steroid therapy, she responded partially to the treatment. Her laboratory findings and hematochezia improved. She was discharged on hospital day 33 and observed without signs of bleeding for three months.

Keyword

Kasabach-Merritt syndrome; Hemangioma; Disseminated intravascular coagulation

MeSH Terms

Abdomen/diagnostic imaging
Ecchymosis/etiology
Female
Gastrointestinal Hemorrhage/etiology
Hemangioma/complications/*diagnosis
Humans
Kasabach-Merritt Syndrome/complications/*diagnosis/drug therapy
Middle Aged
Prednisone/therapeutic use
Propranolol/therapeutic use
Tomography, X-Ray Computed
Prednisone
Propranolol

Figure

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  Fig. 1. Physical examination at admission showed distended abdomen and extremity ecchymosis.

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  Fig. 2. Abdominal CT scan at presentation with several centripetal enhancing exophytic masses in both hepatic lobes (maximum size, 32.3×27.5×25.4 cm). (A) Cross section image. (B) Coronal section image. (C) Cross section image of abdominal CT 8 years prior (maximal size, 22.3 cm).

Reference

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