Case report
Multiple pulmonary arteriovenous fistulas in juvenile cirrhosis

https://doi.org/10.1016/0002-9343(56)90043-2Get rights and content

Abstract

Clinical and pathologic findings in a case of juvenile cirrhosis associated with cyanosis, clubbing of the digits, greatly elevated cardiac output and a long bruit are presented. Although careful examination of the lungs at necropsy, both grossly and microscopically, failed to reveal any significant pathology, injection of a plastic solution into the pulmonary vessels revealed the presence of numerous abnormal vascular channels connecting the pulmonary arteries and veins. Thus the presence of multiple arteriovenous fistulas in the lungs, suspected and sought for during the patient's life, was finally confirmed.

Pulmonary arteriovenous fistulas of the classical type may be multiple. The findings in the present case suggest an entirely different process. A review of the literature has failed to reveal similar reported cases, probably because injection studies of the pulmonary vascular tree have not been performed in instances of juvenile cirrhosis associated with cyanosis and clubbing.

References (39)

  • W.B. Bean

    Acquired palmar erythema and cutaneous vascular “spiders.”

    Am. Heart J.

    (1943)
  • S.M. Berggren

    The oxygen deficit of arterial blood caused by non-ventilating parts of the lungs

    Acta physiol. Scandinav.

    (1942)
  • H. Rolleston et al.

    Diseases of the Liver, Gall-bladder and Bile Ducts

    (1929)
  • H.R. Smith

    Seven cases of cirrhosis of the liver occurring in children and young adults

    Tr. Clin. Soc.

    (1898)
  • A. Sohval

    Hepatic complications in polycythemia vera, with particular reference to thrombosis of the hepatic and portal veins and hepatic cirrhosis

    Arch. Int. Med.

    (1938)
  • P.R. Evans et al.

    Biliary cirrhosis with cyanosis and finger clubbing

  • M. Flückiger

    Vorkommen von trommelschägelförmigen Fingerendphalangen ohne chronische Veränderungen an der Lungen oder am Herzen

    Wien. med. Wchnschr.

    (1884)
  • A. Gilbert et al.

    Rév. mensuelles des mal de l'euf

    Compt. rend. Soc. de Biol.

    (1895)
  • T. Perozzi

    Juvenile cirrhosis

  • A.M. Snell

    The effects of chronic liver disease of the liver on the composition and physiochemical properties of blood: changes in the serum proteins; reduction in the oxygen saturation of the arterial blood

    Ann. Int. Med.

    (1935)
  • A.M. Snell et al.

    Portal cirrhosis complicated by hyperthyroidism; report of studies on oxygen saturation and cardiac output

  • A. Keys et al.

    Respiratory properties of the arterial blood in normal man and in patients with disease of the liver: position of the oxygen dissociation curve

    J. Clin. Investigation

    (1938)
  • E.A. Gaensler

    Clinical pulmonary physiology

    New England J. Med.

    (1955)
  • D.A. Wood et al.

    The role of the dual pulmonary circulation in various pathologic conditions of the lungs

    J. Thorac. Surg.

    (1937–1938)
  • M.R. Hales et al.

    Collateral circulation to the lungs in congenital pulmonic stenosis

    J. Tech. Methods

    (1948)
  • A.A. Liebow et al.

    Enlargement of the bronchial arteries and their anastomoses with the pulmonary arteries in bronchiectasis

    Am. J. Path.

    (1949)
  • A.A. Liebow et al.

    The genesis and functional implications of collateral circulation of the lungs

    Yale J. Biol.

    (1949–1950)
  • P. Marchand et al.

    An anatomical study of the bronchial vascular system and its variations in disease

    Thorax

    (1950)
  • L. Cudkowicz et al.

    Observations on the normal anatomy of the bronchial arteries

    Thorax

    (1951)
  • Cited by (127)

    • Non-invasive assessment of intrapulmonary shunt and ventilation to perfusion ratio in children with hepatopulmonary syndrome before and after liver transplantation

      2021, Respiratory Medicine
      Citation Excerpt :

      The principal abnormality in HPS is the vasodilatation of the alveolar capillaries which results in an increased amount of blood flowing in normally ventilated alveoli and causes a mismatch in the ventilation to perfusion ratio (VA/Q) and hence arterial hypoxaemia [1]. Hypoxaemia in HPS is also caused by intrapulmonary vascular dilatation and shunting via arteriovenous communications [2–4]. HPS is associated with significant morbidity such as refractory hypoxaemia and need for liver transplantation (LT) [5].

    • Hepatopulmonary Syndrome

      2021, Encyclopedia of Respiratory Medicine, Second Edition
    • An unusual solitary endobronchial pulmonary arteriovenous malformation in a 10 year-old child

      2011, International Journal of Pediatric Otorhinolaryngology
      Citation Excerpt :

      The condition is primarily congenital with hereditary hemorrhagic telangiectasia (HHT), an autosomal dominant disorder also known as Osler–Weber–Rendu Syndrome, accounting for 50–70% of all PAVMs [6,7]. Acquired causes include trauma [8], mitral stenosis [8], actinomycosis [8], Fanconi syndrome [9], hepatic cirrhosis [10–14], systemic amyloidosis, schistosomiasis [15], and metastatic thyroid carcinoma [16]. Genetic linkage has been established and PAVMs are attributed to mutations in endoglin (ENG), a TGF-B binding protein, and activin receptor-like kinase (ALK-1), a serine-threonine kinase that binds TGF-B [17–24].

    • THE PULMONARY CIRCULATION IN LIVER DISEASE

      2023, Cardiovascular Complications of Liver Disease
    View all citing articles on Scopus

    This investigation was supported in part by a contract from the office of the Surgeon General, United States Army, Washington, D. C.

    1

    From the Departments of Pathology and Medicine, University of Minnesota, School of Medicine, Minneapolis, Minnesota.

    View full text