A 37-year-old woman with Osler-Weber Rendu syndrome presents with dyspnea and fatigue. The chest x-ray is shown. The best test for establishing a diagnosis is
A. chest computed tomography.
B. lung perfusion scan.
C. percutaneous needle biopsy.
D. pulmonary arteriogram.
E. thoracoscopic lung biopsy.
Answer A
The chest x-ray shows a well-demarcated opacity within the right lower lobe which, combined with the
history and clinical presentation, is suggestive of a large pulmonary arteriovenous malformation
(AVM). Chest computed tomography (CT) using contrast usually demonstrates these lesions
sufficiently well to be diagnostic and may reveal other AVMs that are not identified by chest
radiograph. CT is diagnostic in 98% of pulmonary AVMs compared to only 60% identified by
pulmonary arteriogram. A lung perfusion scan will not aid in the diagnosis, and attempted tissue
biopsy by a percutaneous needle or thoracoscopy may result in significant bleeding or systemic
air emboli.
Puskas JD, Allen MS, Moncure AC, Wain JC Jr, Hilgenberg AD, Wright C, Grillo HC, Mathisen DJ. Pulmonary arteriovenous malformations:
therapeutic options. Ann Thorac Surg 1993;56:253-7.
We have treated 21 patients (13 female, 8 male) with pulmonary arteriovenous
malformations (PAVMs). Mean age at diagnosis was 37.5 years (range, 15 to 72 years). Presenting
symptoms included dyspnea on exertion (67%), hereditary hemorrhagic telangiectasia (57%), and
major neurologic events (33%). In our early experience, 8 patients had no specific treatment;
their case histories illustrate the major neurologic complications of untreated PAVMs. Nine
patients (8 primarily, 1 after recurrence) underwent conservative surgical excision; 4 had
lobectomy, and 5 had segmentectomy or subsegmental excision. One patient underwent staged
bilateral thoracotomies for multiple bilateral lesions. The arterial oxygen tension was found to
increase after excision of large or solitary PAVMs. All surgically treated patients were relieved
of dyspnea, and none had postoperative recurrence of PAVMs or neurologic complications related to
PAVMs. Five patients underwent balloon occlusion of PAVMs. Two patients chose to have solitary
PAVMs occluded rather than undergo thoracotomy. One underwent surgical excision 5 years later,
and the other required repeat balloon embolization 4 years later when recanalization of the PAVMs
was documented. Three patients with numerous PAVMs received palliation with multiple balloon
embolizations. The high incidence of associated major neurologic complications mandates
aggressive treatment of PAVMs whenever feasible. Conservative surgical resection remains the
treatment of choice. Balloon embolization offers an alternative therapy for patients who are poor
surgical risks or those whose lesions are too numerous to resect.
White RI Jr, Pollak JS, Wirth JA. Pulmonary arteriovenous malformations: diagnosis and
transcatheter embolotherapy. J Vasc Interv Radiol 1996;7:787-804.
The recent long-term studies from England, France, and the Netherlands, as well as our own,
indicate that transcatheter embolotherapy is definitive treatment for PAVM. More recently, Puskas
et al have questioned transcatheter embolotherapy as a primary treatment for patients with PAVM
(4,56). Their opinion was based on two recurrences among five patients treated with transcatheter
embolotherapy. It is not clear why one of the late recurrences in the series by Puskas et al
happened, and the other recurrence could have been due to early deflation of the balloon.
Nevertheless, we believe that the collective experience in the larger series reporting on
transcatheter embolotherapy of PAVM supports the use of embolotherapy as a primary modality of
treatment. Because many patients have bilateral pulmonary malformations and many pulmonary
malformations will grow with time, repeated surgical intervention is not ideal therapy. The
recurrence rate of 8% reported by Remy et al using coils, and 2% reported by Pollak et al using
balloons and coils supports our contention that transcatheter embolotherapy is durable and should
be the initial treatment. Also, recurrences are easily retreated by transcatheter embolotherapy
with durable results (54). We favor detachable balloons over coils for occluding PAVMs because
immediate cross-sectional occlusion of the segmental artery is obtained in a position that
preserves the most normal branches. The necessity for repeated introduction of coils, when using
the coil method, contributes to longer procedure times with an increased risk of air introduction
and, in our experience, a greater risk of postprocedure pleurisy. At the same time, we appreciate
that approximately 70% of PAVMs can be occluded equally well with balloons or coils. We also
believe that coils have unique advantages over balloons in specific anatomic situations including
oversized arteries (where coils are the only option) and for occlusion of the aneurysm of a PAVM.
As with all forms of embolotherapy, the interventionalist is best served by having more than one
option of treatment, which for PAVM includes both balloons and coils. In summary, PAVMs are
effectively managed by means of transcatheter embolotherapy. This therapy has been demonstrated
to be safe and durable. Careful technique with modifications depending on the angioarchitecture
of the PAVM is required. Patients with PAVMs require follow-up at 1 month and 1 year. While
observations documenting serial growth of small PAVMs are somewhat limited, there is published
evidence to support their growth with time (35,36). Because of these reports and our unpublished
observations, we believe that patients with treated PAVM need long-term follow-up every 5 years
to detect growth of small PAVMs that will ultimately reach a size where they may cause
paradoxical embolization and stroke (1).
Remy J, Remy-Jardin M, Wattinne L, Deffontaines C. Pulmonary arteriovenous malformations:
evaluation with CT of the chest before and after treatment. Radiology 1992;182:809-16.
A total of 109 single or multiple pulmonary arteriovenous malformations (PAVMs) were evaluated
with computed tomography (CT) of the chest in 40 patients separated into three groups to study
the usefulness of CT (a) in the diagnosis and pretherapeutic management of PAVMs by comparison
with selective pulmonary angiography of each lung (group 1: 20 patients), (b) in the follow-up of
patients who received treatment (group 2: 27 patients), and (c) as an isolated diagnostic
procedure in elderly patients (n = 3) or family members with Osler-Weber-Rendu disease (n = 8)
(group 3: 11 patients). Follow-up ranged from several weeks to 10 years (mean follow-up, 4
years). In group 1, conventional and dynamic CT enabled identification of 107 PAVMs (98.2%) (vs
65 PAVMs [59.6%] identified with angiography), with confident segmental location in 56 of 65
PAVMs (86%) and reliable analysis of angioarchitecture in 17 PAVMs (26%) (vs 39 PAVMs [60%]
analyzed with angiography). In group 2, progressive aneurysmal retraction was associated with
successful occlusion. In group 3, CT enabled noninvasive evaluation of patients unable to undergo
treatment and detection of PAVMS in family members.