Imaging Pearls ❯ Liver ❯ HHT
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- Results In patients with abdominal vascular abnormalities, the liver was the most commonly involved organ, with 180 out of 333 (54.1%) patients demonstrating at least one hepatic vascular abnormality (telangiectasia, confluent vascular mass, transient perfusion abnormalities, and hepatic shunts), with most (70.0%) demonstrating multiple hepatic vascular abnormalities. The other most common organs involved included the pancreas (18.0%), spleen (6.3%), and small bowel (4.5%).
Conclusion In patients with the clinical diagnosis of HHT, greater than half demonstrate an abdominal vascular abnormality, with the most commonly involved organ being the liver. These may be under recognized on routine or single phase contrast- enhanced CT of the abdomen. This supports the use of optimized multiphasic abdominal CT exams as an important tool for the evaluation and screening of patients with HHT.
Abdominal manifestations of hereditary hemorrhagic telangiectasia: a series of 333 patients over 15 years
Welle CL et al.
Abdominal Radiology (2019) 44:2384–2391 - Hereditary hemorrhagic telangiectasia (HHT), also known as Osler-Weber-Rendu disease, is an autosomal dominant inherited disorder with an estimated prevalence of 1–2 individuals per 10,000. It is characterized by vascular abnormalities consisting of dilated, disorganized venules, with direct connection to arterioles (a.k.a. arteriovenous malformations or telangiectasias). The disease affects multiple organs and vascular beds throughout the body, with the skin, mucous membranes, lungs, central nervous system, gastrointestinal tract and liver among the most common sites of involvement.
Abdominal manifestations of hereditary hemorrhagic telangiectasia: a series of 333 patients over 15 years
Welle CL et al.
Abdominal Radiology (2019) 44:2384–2391 - “Clinical diagnosis of HHT is made via the Curacao criteria. The four criteria are: (1) spontaneous and recurrent epistaxis, (2) characteristic mucocutaneous telangiectasias (lips, oral cavity face and fingers), (3) visceral (brain, lung, liver, bowel) arteriovenous malformations (AVM), and (4) diagnosis of HHT in a first degree relative utilizing these same Curacao criteria. Patients with ≥ 3 criteria are labeled as “definite HHT” while those with 2 of the 4 criteria are labeled as “possible” or “suspected” HHT.”
Abdominal manifestations of hereditary hemorrhagic telangiectasia: a series of 333 patients over 15 years
Welle CL et al.
Abdominal Radiology (2019) 44:2384–2391 - Telangiectasias were the most common hepatic vascular abnormality, present in 165 (49.5%) of the patients. Large confluent vascular masses, perfusion abnormalities, and hepatic shunts were all found in lesser but similar numbers. Large confluent masses were seen in 76 (22.8%), perfusion abnormalities were seen in 77 (23.1%), and hepatic shunts were seen in 68 (20.4%).
Abdominal manifestations of hereditary hemorrhagic telangiectasia: a series of 333 patients over 15 years
Welle CL et al.
Abdominal Radiology (2019) 44:2384–2391 - “In patients with the clinical diagnosis of HHT, greater than half demonstrate an abdominal vascular abnormality, with the most commonly involved organ being the liver. These may be under recognized on routine or single phase con- trast-enhanced CT of the abdomen. This supports the use of optimized multiphasic abdominal CT exams as an important tool for the evaluation and screening of patients with HHT.”
Abdominal manifestations of hereditary hemorrhagic telangiectasia: a series of 333 patients over 15 years
Welle CL et al.
Abdominal Radiology (2019) 44:2384–2391 - “Hepatic lesions were sub- categorized into four groups: Telangiectasias, large confluent vascular masses, hepatic perfusion abnormalities, and hepatic shunts according to standard nomenclature. Telangiectasias were defined as typically round, 9 mm or less, arterial hyperenhancing foci which are due to a direct communication between arterioles and postcapillary venules. Typically these are most conspicuous in the early arterial phase and are often subtle or imperceptible in the portal venous phase. Large confluent vascular masses appear similar to telangiectasias, but are defined as ≥ 10 mm in diameter. Because of their larger size, these lesions can sometimes remain visible into the portal venous phase.”
Abdominal manifestations of hereditary hemorrhagic telangiectasia: a series of 333 patients over 15 years
Welle CL et al.
Abdominal Radiology (2019) 44:2384–2391
- ”Hereditary hemorrhagic telangiectasia (HHT), also known as Osler-Weber-Rendu disease, is an autosomal dominant inherited disorder with an estimated prevalence of 1–2 individuals per 10,000. It is characterized by vascular abnormalities consisting of dilated, disorganized venules, with direct connection to arterioles (a.k.a. arteriovenous malformations or telangiectasias). The disease affects multiple organs and vascular beds throughout the body, with the skin, mucous membranes, lungs, central nervous system, gastrointestinal tract and liver among the most common sites of involvement.”
Abdominal manifestations of hereditary hemorrhagic telangiectasia: a series of 333 patients over 15 years
Christopher L. Welle et al.
Abdominal Radiology https://doi.org/10.1007/s00261-019-01976-7 - “Clinical diagnosis of HHT is made via the Curacao criteria. The four criteria are: (1) spontaneous and recurrent epistaxis, (2) characteristic mucocutaneous telangiectasias (lips, oral cavity face and fingers), (3) visceral (brain, lung, liver, bowel) arteriovenous malformations (AVM), and (4) diagnosis of HHT in a first degree relative utilizing these same Curacao criteria. Patients with ≥ 3 criteria are labeled as “definite HHT” while those with 2 of the 4 criteria are labeled as “possible” or “suspected” HHT
Abdominal manifestations of hereditary hemorrhagic telangiectasia: a series of 333 patients over 15 years
Christopher L. Welle et al.
Abdominal Radiology https://doi.org/10.1007/s00261-019-01976-7 - “In patients with the clinical diagnosis of HHT, greater than half demonstrate an abdominal vascular abnormality, with the most commonly involved organ being the liver. These may be under recognized on routine or single phase contrast- enhanced CT of the abdomen. This supports the use of optimized multiphasic abdominal CT exams as an important tool for the evaluation and screening of patients with HHT.”
Abdominal manifestations of hereditary hemorrhagic telangiectasia: a series of 333 patients over 15 years
Christopher L. Welle et al.
Abdominal Radiology https://doi.org/10.1007/s00261-019-01976-7 - “In patients with abdominal vascular abnormalities, the liver was the most commonly involved organ, with 180 out of 333 (54.1%) patients demonstrating at least one hepatic vascular abnormality (telangiectasia, confluent vascular mass, transient perfusion abnormalities, and hepatic shunts), with most (70.0%) demonstrating multiple hepatic vascular abnormalities. The other most common organs involved included the pancreas (18.0%), spleen (6.3%), and small bowel (4.5%).”
Abdominal manifestations of hereditary hemorrhagic telangiectasia: a series of 333 patients over 15 years
Christopher L. Welle et al.
Abdominal Radiology https://doi.org/10.1007/s00261-019-01976-7