*From the Departments of Surgery (Mr. Guerrero and Dr. Karmy-Jones), Radiology (Dr. Hoffer), and Pulmonary Medicine (Dr. Hudson), Harborview Medical Center, Seattle, WA; and Willis-Knighton Health System and Christus-Shumpert Medical Center (Dr. Schuler), Shreveport, LA.
Correspondence to: Riyad Karmy-Jones, MD, Department of Surgery, Box 359796, Harborview Medical Center, 352 Ninth Ave, Seattle, WA 98104; e-mail: firstname.lastname@example.org
We present the case of a 32-year-old woman with high-grade right
pulmonary artery stenosis secondary to fibrous mediastinitis. The
patient was managed with balloon angioplasty and stent placement. Only
15 cases of this nature have been reported in the literature, and this
is one of the first to be managed with endovascular stent
mediastinitis is a rare condition first described in 1855 by Nathan
Oulmont.1It is typically associated with mycobacterial
and mycotic infections.2Patients typically present with
superior vena cava (SVC) syndrome.3–4 Most cases have a
benign clinical course until the lesion constricts a major mediastinal
structure.2 Fifteen other cases of fibrous mediastinitis
with pulmonary artery compression have been described in the
literature.5–12 In the past 10 years, endovascular
approaches have provided an effective treatment option for those
individuals suffering from SVC syndrome.13–14 Recent
advances in endovascular techniques have allowed the dilation and
stenting of the pulmonary artery. To our knowledge, only one other case
of fibrous mediastinitis has been managed with pulmonary artery
A 32-year-old woman from the Mississippi Valley presented with
severe shortness of breath and chronic cough. Physical examination
demonstrated distended neck veins and symptoms consistent with SVC
syndrome. Chest radiograph revealed an area of calcification in the
right parasternal region at the level of the third rib (Fig 1
). A pulmonary perfusion scan was performed that indicated essentially
no perfusion to the right lung. A CT scan was performed that revealed a
lesion adjacent to the right main pulmonary artery. The patient
underwent two mediastinoscopies. Frozen section evaluation and cultures
demonstrated a fibrous histology with no evidence of mycobacterial or
fungal infection. The patient was prescribed an anti-inflammatory
steroid regimen, with some resolution of the SVC syndrome. A CT scan
demonstrated a mass approximately 3.0 cm by 2.5 cm by 2.0 cm and SVC
stenosis with significant collateral veins. Peripheral administration
of contrast was unable to visualize the right pulmonary circulation, so
a catheter was employed to administer the contrast proximal to the
right pulmonary artery (Fig 2
). This showed an abrupt stenosis of the right main pulmonary artery. An
angiographic study revealed 95% stenosis of the right pulmonary artery
at its junction with the right lower pulmonary artery, and total
occlusion of the right upper pulmonary artery (Fig 3
). The stenosis was dilated and stented with a Palmaz 424 medium
catheter (Cordis/Johnson and Johnson; Miami, FL) over a 5.8F 10-mm
balloon catheter. A poststent angiogram confirmed the appropriate
placement of the stent and patency of the right lower and middle
pulmonary arteries (Fig 4
). The patient was prescribed aspirin, 5 grains tid, and discharged.
Shortly after the stent placement, the patient had complete resolution
of symptoms. At 3-month follow-up, she had continued absence of
symptoms, and a pulmonary perfusion scan indicated appropriate
ventilation and perfusion to the right lower and middle lobes.
Fibrous mediastinitis is a rare condition that was recognized as a
cause of SVC obstruction by Osler in 1903.3 Cases may be
idiopathic or associated with tuberculosis, histoplasmosis,
sarcoidosis, silicosis, or other fungal infections.2
Mediastinoscopy is warranted to distinguish this inflammatory process
from a malignancy. Patients with fibrous mediastinitis generally have a
benign clinical course until a mediastinal structure is
compressed.15 The first and most common structure affected
is the SVC, but involvement of the pulmonary artery, phrenic nerve,
recurrent laryngeal nerve, and pulmonary veins have also been
SVC stent placement is an effective approach to managing SVC syndrome
associated with fibrous mediastinitis.12–13 In addition,
steroid therapy may be an appropriate option in patients with mild
obstruction, or as an adjunct to other treatment approaches, although
this has not been established.16
Endovascular pulmonary artery angioplasty has been used
extensively in pediatric patients with tetrad of Fallot.17
The use of this approach in treating pulmonary stenosis with fibrous
mediastinitis has been employed only recently.11 We
believe it is an appropriate option that should be considered when
presented with such a condition.
Abbreviation: SVC = superior vena cava
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