Download Recurrent Hemoptysis Following a Systemic-to

of tuberculosis appeared to be unlikely, and oral corticosteroid therapy was begun, leading to a rapid normalization of the symptoms and to a resolution of the radiographic abnormalities. Corticosteroids were discontinued
after a 12-month treatment period, and regular clinical,
radiologic, and sonographic controls failed to detect a
recurrence of the disease for ⬎ 1 year.
Multiple primary disorders have been associated with
pulmonary reactions and pathologic features of BOOP,
including respiratory infections with various bacterial and
viral agents, toxic inhalants, adverse drug reactions, or
collagen vascular disease. In a large proportion of cases,
however, no etiology may be apparent (idiopathic BOOP).
On the basis of the available serologic data, the patient
may have had a respiratory infection, presumably caused
by C pneumoniae, as has previously been observed.5
The present case represents an unusual radiographic
manifestation of BOOP, with an almost symmetrical involvement of the two apical lung segments. Comparable
abnormalities affecting both upper lobes associated with
BOOP have not been reported previously. Differential
diagnosis of bilateral apical consolidations is primarily
suggestive of tuberculosis, which could be excluded in the
patient. The case presented herein demonstrates that
BOOP can both clinically and radiographically mimic
tuberculosis.
the left pulmonary artery. Following this procedure,
he developed recurrent episodes of hemoptysis,
cough, and left upper lobe consolidation. Treatment
resulted in clinical but no radiologic resolution. At
age 6, a new right systemic-to-pulmonary anastomosis was needed, as the left one was no longer functioning. After placement of the second shunt, the
hemoptysis disappeared. At age 14, flexible bronchoscopy revealed a foreign body granuloma at the
left secondary carina. Rigid bronchoscopy and laser
photoresection showed it to be the left vascular
prosthesis, placed 10 years before. Surgery failed to
remove it.
(CHEST 2000; 118:865– 867)
References
A 14-year-old boy with history of single ventricle, left type, left
pulmonary artery stenosis, and left great vessels transposition,
diagnosed by cardiac catheterization during the postnatal period,
was sent to the Pulmonary Department for chest radiograph
abnormalities.
At age 4, the patient developed worsening cyanosis and
decreased exercise tolerance. A cardiac catheterization was performed, confirming the previous diagnosis and showing the
development of collateral circulation, in the form of a great
number of vessels branching from the descending aorta toward
both lungs, particularly the left one. Bronchial circulation was
poor bilaterally, particularly in the left lung where it was almost
absent. A left systemic-to-pulmonary anastomosis was performed
(from the left subclavian artery to the left pulmonary artery; Fig
1), using a tubular 6-mm polytetrafluoroethylene prosthesis
(Gore-Tex; W.L. Gore; Flagstaff, AZ). In the immediate postoperative period, a new catheterization showed increase in blood
flow to the left lung.
Nine months after the surgery, the patient presented an
episode of cough and hemoptysis, and chest radiography showed
consolidation and atelectasis on the left upper lobe (LUL). The
patient was treated with antibiotics, and aspirin was discontinued.
The picture improved both clinically and radiologically, although
a small opacity persisted in the LUL in the following chest
radiographs. This abnormality was further investigated by CT of
the chest, which only showed lung consolidation. Similar clinical
1 Epler GR, Colby TV, McLoud TC, et al. Bronchiolitis
obliterans organizing pneumonia. N Engl J Med 1985; 312:
152–158
2 Davidson AG, Heard BE, McAllister WAC, et al. Cryptogenic
organizing pneumonia. Q J Med 1983; 52:382–393
3 Bartter T, Irwin RS, Nash G, et al. Idiopathic bronchiolitis
obliterans organizing pneumonia with peripheral infiltrates
on chest roentgenogram. Arch Intern Med 1989; 149:273–
279
4 Izumi T, Kitaichi M, Nishimura K, et al. Bronchiolitis obliterans organizing pneumonia: clinical features and differential
diagnosis. Chest 1992; 102:715–719
5 Diehl JL, Gisselbrecht M, Meyer G, et al. Bronchiolitis
obliterans organizing pneumonia associated with chlamydial
infection. Eur Respir J 1996; 9:1320 –1322
Recurrent Hemoptysis
Following a Systemic-toPulmonary Anastomosis in a
Child With a Complex
Congenital Cardiomyopathy*
Jose Pablo Diaz-Jimenez, MD, PhD; Alicia N. Rodriguez, MD;
and Martin Anselmo Andres, MD
A 14-year-old boy with a history of congenital cardiopathy is presented. At age 4, a left systemic-topulmonary fistula was performed, using a tubular
prosthesis to anastomose the left subclavian artery to
Key words: foreign body; hemoptysis; laser photoresection;
vascular prosthesis
Abbreviation: LUL ⫽ left upper lobe
is a frequent complication in children with
H emoptysis
congenital cardiopathies undergoing surgery. We
present an unusual case of hemoptysis in a 4-year-old child
after the performance of a systemic-to-pulmonary anastomosis secondary to a severe congenital cardiopathy. The
possible cause of the hemoptysis was found 10 years later,
after a flexible bronchoscopy.
Case Presentation
*From the Endoscopy and Laser Unit (Drs. Diaz-Jimenez and
Rodriguez), Hospital Duran i Reynals, L’Hospitalet de Llobregat,
Barcelona; and Pulmonary Pediatric Section (Dr. Andres), Hospital Infantil Universitario Virgen del Rocio de Sevilla, Spain.
Manuscript received June 28, 1999; revision accepted March 1,
2000.
Correspondence to: Jose Pablo Diaz-Jimenez, Endoscopy and
Laser Unit, Pulmonary Department, Ciutat Sanitària de Bellvitge, Feixa Llarga s/n, E-08907 L’Hospitalet de Llobregat,
Barcelona, Spain; e-mail: [email protected]
CHEST / 118 / 3 / SEPTEMBER, 2000
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Figure 3. Endobronchial image showing sutures, granulation
tissue, and part of the vascular prosthesis.
Figure 1. Location of the left systemic-to-pulmonary anastomosis.
episodes recurred occasionally, with the same characteristics of
cough, hemoptysis, and worsening of the LUL radiologic abnormalities.
Two years after the surgery, at age 6, a new systemic-topulmonary anastomosis was performed, this time to the right
pulmonary artery, since the previous one had stopped working.
After the second operation, the episodes of hemoptysis diminished progressively and finally disappeared, but the consolidation-like image was persistent in the LUL.
At age 14, the patient was scheduled for cardiac surgery; as a
part of the presurgical evaluation, he underwent a flexible
bronchoscopy in order to clarify the persistent chest radiograph
image (Fig 2). Flexible bronchoscopy showed a tracheal bronchus
as an incidental finding, a normal right tracheobronchial tree, and
a granulomatous-type lesion located at the level of the left
secondary carina. Since some sutures were visible, a presumptive
diagnosis of foreign body granuloma was made, and the patient
was transferred to us for laser bronchoscopy evaluation.
The patient underwent rigid bronchoscopy under general
anesthesia. After removal of the granulation tissue, we found a
mesh-like foreign body emerging from the lateral wall of the left
upper bronchus, which appeared to be the endovascular prosthesis (Fig 3). No attempt was made to remove it, and the patient
was referred to thoracic surgery for evaluation.
A left thoracotomy was performed and was complicated by
excessive fibrous adhesions and a cardiac arrest. It was not
possible to remove the prosthesis.
Since then, the patient has remained clinically stable. The
episodes of hemoptysis have not recurred, and the LUL chest
radiograph abnormalities have not resolved.
Discussion
Figure 2. Chest radiograph at age 14, showing the persistent
consolidation image at the LUL, and atelectasis.
A single ventricle is a rare and complex congenital
cardiopathy, characterized by the presence of one ventricle, which receives two atrioventricular valves and empties
into both the aorta and the pulmonary artery.1 Transposition of the great arteries and pulmonary stenosis are
common accompanying malformations. Hemodynamically, there is bidirectional shunting at the ventricular
level. Surgical correction is very difficult, but palliation of
the cyanosis can be achieved by the creation of a systemicto-pulmonary anastomosis by means of a tube graft, or
without using vascular prosthesis. This will increase the
pulmonary blood flow, bypassing the pulmonary stenosis.2
Causes of hemoptysis in children with congenital cardiopathies, whether they undergo surgery or not, are
many: extracorporeal circulation, pulmonary infections,
coagulopathies, vascular disorders. A vascular prosthesis as
a cause of bronchial obstruction and hemoptysis is very
unusual, and reports in the literature are scant. Some cases
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Selected Reports
of chronic hemoptysis are associated with the development of aortobronchial fistulas involving prosthetic aortic
grafts.3 Artery bands can migrate and produce bronchial
obstruction. A case of a 4.5-kg boy was presented by Parry
et al.4 This patient developed cough after placement of a
pulmonary band, and episodes of consolidation were seen
on the chest radiographs. Two bronchoscopies failed to
reveal any abnormality other than purulent drainage coming from the right middle and lower lobes. The intact
Silastic band (Dow Corning; Auburn, MI) used for the
pulmonary banding was recovered from the right lung at
age 5. Some other reports involving pulmonary band
migrations through the vascular wall have been published,
most of them associated with infections at the site of the
prosthesis followed by vascular erosion.5–7
To our knowledge, this is the first report of a vascular
prosthesis migrating and eroding the bronchial wall. We
presume that the mechanism is the same as the one
involved in the aortobronchial fistula formation: chronic
inflammation secondary to foreign body reaction, and/or
direct pressure necrosis of the wall.
Unfortunately, it is not possible at present to know the
exact situation of the prosthesis. After the attempted
prosthesis removal and the cardiac arrest, the patient had
a complicated postoperative period, and the family decided not to perform any additional studies or procedures.
We speculate that the prosthesis may be still connected to
the anastomosis. It is possible that the erosion of the
bronchial wall and kinking of the vessels was the reason
why the systemic-to-pulmonary bypass stopped working 2
years after the surgery. The graft most probably caused
endobronchial obstruction and episodes of postobstructive
infections.
It is important to consider prosthesis migration in any
patient with a thoracic vascular prosthesis who develops
recurrent hemoptysis and/or recurrence of other respiratory symptoms and nonresolving radiologic abnormalities.
ACKNOWLEDGMENT: The authors thank Dr. Mark Mercurio
for reviewing and correcting the manuscript.
References
1 Ionescu MI, Macartney FJ, Wooler GH. Intracardiac repair
of single ventricle with pulmonary stenosis. J Thorac Cardiovasc Surg 1973; 65:602– 607
2 Holden MP, Macartney FJ, Ionescu MI. Surgical palliation of
single ventricle with pulmonary stenosis. Ann Thorac Surg
1973; 15:578 –587
3 Wheeler AP, Loyd JE. Fatal hemoptysis: aortobronchial
fistula as a preventable cause of death. Crit Care Med 1989;
17:1228 –1230
4 Parry RL, Gordon LS, Sherman NJ. Pulmonary artery band
migration producing endobronchial obstruction. J Pediatr
Surg 1997; 32:48 – 49
5 Albus RA, Trusler GA, Izukawa T, et al. Pulmonary artery
banding. J Thorac Cardiovasc Surg 1984; 88:645– 653
6 Romer J, Brom AG, Nauta J. Bands inside the pulmonary
artery: a complication of the Damman-Muller procedure.
Ann Thorac Surg 1967; 3:449 – 454
7 Kumar RV, Roughneen PT, deLeval MR. Mycotic pulmonary
artery aneurism following pulmonary artery binding. Eur
J Cardiothorac Surg 1994; 8:665– 666
Normal Diffusing Capacity in
Patients With PiZ ␣1Antitrypsin Deficiency, Severe
Airflow Obstruction, and
Significant Radiographic
Emphysema*
Jeffrey S. Wilson, MD; and Jeffrey R. Galvin, MD, FCCP
␣1-Antitrypsin deficiency is usually suspected clinically in young adults with irreversible airflow obstruction that is out of proportion to their smoking
history. Many patients with ␣1-antitrypsin deficiency
receive an initial diagnosis of asthma or chronic
bronchitis. Measurement of the diffusing capacity of
the lung for carbon monoxide (DLCO) has been
recommended as a way to help distinguish emphysema from asthma and chronic bronchitis. In this
article, we describe four patients with severe ␣1antitrypsin deficiency, each of whom had a repeatedly normal DLCO despite having a significant component of fixed airway obstruction and prominent
panacinar emphysema on high-resolution CT scan
(HRCT). Each patient also demonstrated significant
bronchodilator responsiveness, and two patients received an initial diagnosis of asthma. Potential explanations for these findings are discussed. We report
these findings to illustrate the limitations of DLCO in
this setting. ␣1-Antitrypsin deficiency should be considered in patients with fixed airway obstruction that
is out of proportion to their age and smoking history,
regardless of their diffusing capacity and response to
bronchodilators.
(CHEST 2000; 118:867– 871)
Key words: ␣1-antitrypsin deficiency; chronic obstructive lung
disease; CT; diffusing capacity; emphysema
Abbreviations: ␣1-AT ⫽ ␣1-antitrypsin; Dlco ⫽ diffusing capacity
of the lung for carbon monoxide; HRCT ⫽ high-resolution CT
evere ␣ -antitrypsin (␣ -AT) deficiency may result in
S the
development of premature emphysema. It is usu1
1
ally suspected clinically in young adults with irreversible
airflow obstruction that is out of proportion to their
smoking history.1–3 Early diagnosis is important to prevent
disease progression, through implementation of smoking
cessation.4 –5 The data supporting the efficacy of ␣1-AT
*From the Departments of Internal Medicine and Radiology,
University of Iowa, Iowa City, IA.
Manuscript received June 26, 1997; revision accepted March 13,
2000.
Correspondence to: Jeff Wilson, MD, University of Iowa, Department of Internal Medicine, C33 GH, 200 Hawkins Dr, Iowa City,
IA 52242-1081; e-mail: [email protected]
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