Autor : Ubal, Leonardo German1, Farieri, Verónica María1, Tisera Castanié, Candelaria2
1 Hospital Privado Universitario de Córdoba, Pulmonology Service, Córdoba City, Province of Córdoba, Argentina. 2Hospital Privado Universitario de Córdoba, Diagnostic Imaging Service, Córdoba City, Province of Córdoba, Argentina.
https://doi.org/10.56538/ramr.IPNV6463
Correspondencia : Leonardo Ubal E-mail: leoub6@hotmail.com
Received: 04/06/2025
Accepted: 05/01/2025
CASE REPORT
61-year-old
male who works as a supervisor at a nuclear power
plant. The patient is an
active smoker with a 43
pack-year history, overweight, and currently going through the
immediate postoperative period after umbilical hernioplasty.
Medical
history: more than 6 episodes of hemoptysis with bright red blood and clots of approximately 200 ml each, with acute onset.
The patient denies other symptoms.
He reports recurrent
epistaxis during childhood
and adolescence.
Hemodynamically stable,
with basal crackles on the right
side. Mucocutaneous telangiectases observed on the bridge of the nose, cheeks,
forehead, lips, tongue, and hands. Laboratory results: Hb (hemoglobin): 15g/dl, Hto (hematocrit): 44.5%,
C-reactive protein: 0.26mg/dl, D-dimer:
1.15ugr/ ml, normal coagulogram.
Initialchest HRCT without contrast showed ground-glass infiltrates (GGI) and a right
basal cobblestone pattern (Image 1). Two hours
later, a chest HRCT with contrast revealed
progressive GGI extension (Image 2).


Bronchoalveolar lavage
was performed (BAL), revealing the presence
of hemosiderophages, and confirming
pulmonary hemorrhage. Results from molecular biology, microbiology, and anatomic pathology ruled out other
diagnoses.
ADMISSION DIAGNOSIS
Focal
alveolar hemorrhage, suspected
pulmonary arteriovenous malformation (PAVM)
CLINICAL DISCUSSION
In
cases of focal alveolar hemorrhage, it is important
to rule out localized lesions such as bronchiectasis, chronic bronchitis, infections, tumors, trauma-related causes,
vasculitis, and PAVM, among others.1
In this patient,
with alveolar hemorrhage confirmed by BAL, along with physical
examination findings, recurrent epistaxis, and a paternal history
of Rendu-Osler-Weber Syndrome, the primary
diagnostic suspicion is PAVM. A selective bronchial arteriography was performed (Image 3).

PAVM
secondary to Hereditary Hemorrhagic Telangiectasia (HHT) is confirmed. The
pathological artery is embolized, and stops the bleeding.
HHT
or Rendu-Osler-Weber Syndrome: is
an autosomal dominant disorder with a global prevalence of
1/5,000-8,000. It is characterized by cutaneous and mucosal telangiectases, recurrent
epistaxis, and arteriovenous malformations
(AVMs) in the brain, liver, gastrointestinal tract, and lungs.2-4
PAVMs occur in 20-50% of patients with HHT, creating systemic-to-pulmonary arteriovenous shunts. They are typically unilateral and located
in the lower lobes. They can cause hypoxemia, paradoxical emboli, pulmonary hemorrhage, hemoptysis, and hemothorax. Selective embolization is the treatment of choice, with a 15% recanalization rate. CT follow-up is recommended.
Diagnosis through contrast-enhanced
high-resolution computed
axial tomography (HRCAT) requires
visualization of a feeding artery and a draining vein. PAVMs with
focal pulmonary hemorrhage
are observed with GGO and areas of consolidation, due to the presence
of alveolar blood, along with superimposed interlobular septal lines that can form a “crazy paving
pattern.”1,5 Also notable is the “dark bronchus
sign”, caused by filling and prominence of segmental and subsegmental bronchi. Hemoptysis is always
a diagnostic challenge. In
this case, we want to highlight the importance of CT findings as the first step in the
diagnostic evaluation, along with the
BAL to confirm focal pulmonary
hemorrhage.
Conflict of interest
Authorshave no conflict of interest to declare that are relevant to the content of this article.
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