[A case of congenital hepatic hemangioendothelioma treated with prednisone: the echographic changes and Doppler
C Poggiani1, A Auriemma, C Bellan
1Divisione di Patologia Neonatale, Ospedali Riuniti, Bergamo.
Insights
This case study highlights imaging findings in a term infant with hepatic hemangioendothelioma. Doppler sonography proved crucial for monitoring therapy and assessing vascular compromise.
Area of Science:
- Pediatric Radiology
- Vascular Anomalies
- Medical Imaging
Background:
- Multicentric hepatic hemangioendothelioma is a rare liver tumor in infants.
- Early diagnosis and monitoring are crucial for managing potential complications like congestive heart failure.
Observation:
- A term infant presented with feeding difficulties, dyspnea, cardiac bruit, congestive heart failure, and hepatomegaly.
- Physical examination revealed multiple small skin hemangiomas.
- Abdominal sonography showed multiple liver lesions, enlarged hepatic vessels, and reduced aortic caliber.
Findings:
- Magnetic Resonance (MR) imaging revealed multiple, well-circumscribed nodules with high signal intensity on T2-weighted images and fast flow.
- Whole-body scintigraphy with 99mTc-labeled red blood cells was used to detect other visceral hemangiomas.
- Doppler sonography demonstrated significant changes in hepatic, cerebral, and renal vascular flow patterns and velocities, indicating compromise.
Implications:
- Imaging, particularly Doppler sonography, plays a vital role in diagnosing hepatic hemangioendothelioma and monitoring treatment response.
- Vascular compromise, including reduced diastolic flow and increased Resistance Index in cerebral and renal arteries, can occur with hepatic hemangioendothelioma.
- Furosemide and steroid therapy showed positive response, evidenced by changes in echogenicity on serial ultrasound scans.
Abstract:
Imaging investigations and other findings observed in a term infant with a multicentric hepatic hemangioendothelioma, admitted to the Intensive Care Unit at the age of 13 days because of non specified feeding difficulties and dyspnoea, are presented. Physical examination revealed cardiac bruit and congestive heart failure with marked hepatomegaly; in addition there were multiple small skin hemangiomas. Echocardiography was negative, abdominal sonography showed multiple round lesions of mixed echogenicity in the liver, large vascular channels, a right hepatic artery and hepatic veins enlarged, a caliber of the aorta below the level of the superior mesenteric artery reduced. The infant was additionally investigated by whole-body scintigraphy with 99mTc-labeled red blood cells to determine the possibility of coexistence of other visceral hemangiomas and by MR, in which the tumor manifested as multiple well-circumscribed space-occupying nodules of high signal intensity on T2-weighted images with evidence of fast flow. The baby underwent furosemide and steroid therapy: serial two-dimensional US scans showed change in echogenicity, responding to therapy. Doppler sonography has proven to be also very useful in the monitoring therapy determining changes in flow pattern and velocity at the level of hepatic, cerebral and renal vessels: before therapy we observed a reduction of the diastolic flow until the zero line through the internal carotid artery and renal artery with an increase of the Resistance Index. It means that this important component can be compromised in the presence of a hepatic hemangioendothelioma.
