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La radiologia medica

, Volume 118, Issue 1, pp 1–13 | Cite as

Hepatic nodular regenerative lesions in patients with hereditary haemorrhagic telangiectasia: computed tomography and magnetic resonance findings

  • A. Scardapane
  • M. Ficco
  • C. Sabbà
  • F. Lorusso
  • M. Moschetta
  • N. Maggialetti
  • P. Suppressa
  • G. Angelelli
  • A. A. Stabile IanoraEmail author
Abdominal Radiology / Radiologia Addominale

Abstract

Purpose

This study was done to evaluate the prevalence of regenerative hepatic nodules in patients with hereditary haemorrhagic telangiectasia (HHT).

Materials and methods

Between February 2001 and December 2010, 171 consecutive HHT patients (95 men and 76 women) were studied with triphasic multidetector computed tomography (MDCT) in 91 cases, magnetic resonance imaging (MRI) in 34 cases and both in the remaining 46 cases. The presence of diffuse vascular abnormalities and focal liver lesions were recorded.

Results

Hepatic arteriovenous malformations (HAVMs) were found in 126/171 (74%) patients. Arteriovenous shunts were found in 24/171 (14%) cases, arterioportal shunts in 52/171 (30%), mixed shunts in 26/171 (15%), telangiectases in 84/171 (49%) and transient hepatic attenuation differences (THADs) in 70/171 (41%). Hepatic nodular lesions were found in 6/171 (3.5%) patients (three men; three women). In 5/6 cases, vascular abnormalities were also evident. Two patients had a single lesion; four had multiple lesions. No lesion showed a central scar.

Conclusions

Hyperenhancing hepatic regenerative lesions have a high prevalence in HHT patients, representing the response of liver parenchyma to hypoperfusion caused by HAVMs. These lesions are often multiple and may lead to nodular regenerative hyperplasia.

Keywords

Hereditary haemorrhagic telangiectasia MDCT MRI Nodular regenerative hyperplasia 

Rigenerazione nodulare epatica nei pazienti affetti da telangiectasia emorragica ereditaria: reperti di tomografia computerizzata e risonanza magnetica

Riassunto

Obiettivo

Scopo del presente lavoro è valutare la frequenza di lesioni nodulari rigenerative nei pazienti affetti da telangiectasia emorragica ereditaria (HHT).

Materiali e metodi

Nel periodo compreso tra febbraio 2001 e dicembre 2010 sono stati arruolati consecutivamente 171 pazienti (95 uomini, 76 donne) affetti da HHT. La presenza di alterazioni epatiche è stata ricercata in 91 casi con TCMD trifasica (fase arteriosa precoce, tardiva e fase venosa) e in 34 con angio-risonanza magnetica (RM) dinamica, i rimanenti 46 pazienti sono stati sottoposti ad entrambi gli esami.

Risultati

In 126/171 (74%) pazienti sono state diagnosticate alterazioni vascolari epatiche costituite da fistole artero-venose (24/171, 14%), fistole artero-portali (52/171, 30%), fistole miste (26/171, 15%), telangiectasie (84/171, 49%) e disordini di perfuzione (THADs) (70/171, 41%). In 6/171 casi (3 donne e 3 uomini, 3,5%) sono state descritte lesioni nodulari epatiche ipervascolarizzate associate in 5 pazienti ad altre lesioni vascolari. Due pazienti erano portatori di una lesione singola mentre in quattro casi sono state dimostrate lesioni multiple. In nessun caso è stata riconosciuta una cicatrice centrale.

Conclusioni

Le lesioni epatiche rigenerative hanno un’elevata prevalenza nei pazienti affetti da HHT e costituiscono una risposta del fegato all’ipoperfusione determinata dagli shunt artero-venosi intraparenchimali. Tali lesioni si presentano spesso in forma multipla e possono determinare un quadro di iperplasia nodulare rigenerativa diffusa.

Parole chiave

Telangiectasia emorragica ereditaria TCMD RM Iperplasia nodulare rigenerativa 

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Copyright information

© Springer-Verlag Italia 2012

Authors and Affiliations

  • A. Scardapane
    • 1
  • M. Ficco
    • 1
  • C. Sabbà
    • 2
  • F. Lorusso
    • 1
  • M. Moschetta
    • 1
  • N. Maggialetti
    • 1
  • P. Suppressa
    • 2
  • G. Angelelli
    • 1
  • A. A. Stabile Ianora
    • 1
    • 3
    Email author
  1. 1.Sezione di Diagnostica per Immagini, Centro Interdipartimentale per lo Studio dell’HHTUniversità degli Studi di Bari, Azienda Universitario-Ospedaliera Consorziale “Policlinico”BariItaly
  2. 2.Sezione di Medicina Interna “Augusto Murri”, Di.M.I.M.P. — Centro Interdipartimentale per lo Studio dell’HHTUniversità degli Studi di Bari, Azienda Universitario-Ospedaliera Consorziale “Policlinico”BariItaly
  3. 3.Istituto di RadiologiaUniversità degli Studi di Bari, Azienda Universitario-Ospedaliera Consorziale “Policlinico”BariItaly

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