Massive haemoptysis after living donor liver transplantation

Abstract

A 27 year old man with hereditary haemorrhagic telangiectasia who developed progressive liver dysfunction underwent living related right lobe transplantation. Pulmonary arteriography did not reveal arteriovenous malformation or abnormal intrapulmonary venous channels. The postoperative course was characterised by persistent hypoxaemia and respiratory failure developed. On day 6, a massive haemoptysis developed and the patient died shortly thereafter. The native liver showed a nodular pseudocirrhotic transformation, with highly dilated and irregularly interconnected vein-like or arterial-like structures in the fibrous septa. Pathological examination of both lungs showed irregular thickening of the wall of the arteries, secondary to eccentric and/or concentric myointimal hyperplasia. This case suggests that massive haemoptysis can develop even when arteriovenous malformations are undetectable by pulmonary arteriography, and it questions the role and the appropriateness of living donor liver transplantation in high risk patients.

Figure 1

Native liver histology: general architecture. (A) Extremely large fibrous septa entrapping near normal “hepatic macrolobules” containing occasional portal tracts (P) and veins (V); large bile ducts (D) are irregularly embedded in the surrounding fibrous tissue. Chromotrope aniline blue method; original magnification, ×40. (B) Native liver histology: interesting features. Fibrous band containing small veins (Ve) and tangentially cut arterial vessels (A). Greatly dilated sinusoids (S) are found in the surrounding parenchyma. The black arrow points to an irregular, thin walled vascular space, directly abutting the hepatocyte cords. In addition, dilated vein-like vessels (V) are sparse among the hepatocyte cords. Haematoxylin and eosin stained; original magnification, ×40.

Figure 2

Lungs and tracheobronchial tree viewed from behind, after dissection of the pars membranacea of the trachea and of the main left and right bronchi, showing large blood clots occluding the airways.

Figure 3

Massive alveolar haemorrhage and the main vascular lesions. (A) Eccentric intimal thickening of the dilated medium arteries (arrows; haematoxylin and eosin stained; original magnification, ×100) and (B) myointimal hyperplasia of small sized pulmonary arteries (arrows; haematoxylin and eosin stained; original magnification, ×200).