AJR 2004; 183:1115-1116
© American Roentgen Ray Society
Congenital HIV and Tracheal Diverticulosis
Terry L. Levin1,
Laura Weingart2,
Henry M. Adam2 and
Alfin G. Vicencio2
1 Department of Radiology, Montefiore Medical Center, Bronx, NY 10467.
2 Department of Pediatrics, Section of Respiratory Medicine, Children's Hospital
at Montefiore Medical Center, 3415 Bainbridge Ave., Bronx, NY 10467.
Received December 5, 2003;
accepted after revision January 21, 2004.
Address correspondence to A. G. Vicencio
(dvicenci.montefiore.org).
Introduction
Respiratory symptoms in HIV-infected individuals may be associated with a
variety of infectious and noninfectious entities. We report a case of tracheal
diverticulosis in a 19-year-old man with congenital HIV infection, severe
airflow obstruction, and persistent cough.
Case Report
A 19-year-old man with congenital HIV and asthma was referred for pulmonary
evaluation because of worsening respiratory symptoms. The patient reported
that his asthma, which had been previously controlled with high-dose inhaled
corticosteroids, had worsened over the past year. At the time of consultation,
he had difficulty walking up two flights of stairs and complained of daily
cough, productive of sputum. He denied experiencing hemoptysis, chest pain,
fever, or recent weight loss.
Past medical history was significant for lymphocytic interstitial
pneumonitis (treated with corticosteroids) and recurrent bacterial pneumonia.
The results of the yearly testing for purified protein derivative were
negative, and the patient had no history of Pneumocystis carnii
pneumonia. Results of sputum cultures were repeatedly negative for the
presence of acid-fast bacilli and fungi. The patient was treated with a highly
active antiretroviral therapy regimen of five drugs; his viral load was
undetectable, and his CD4+ T-cell count was 264/µL (11% of predicted
value).
At physical examination, the patient was thin but well nourished. His
oxygen saturation level was 93% on room air. Examination of the chest revealed
poor aeration and crackles posteriorly. There was moderate digital clubbing.
Pulmonary function testing showed severe obstructive disease and air trapping
(forced vital capacity [FVC] 38% of the predicted value; forced expiratory
volume in 1 sec [FEV1], 31% of the predicted value;
FEV1/FVC ratio, 75; forced expiratory flow at 25–75% of FVC,
22% of the predicted value; and residual volume, 256% of predicted value).
Radiography and CT of the chest showed bilateral extensive emphysematous
changes, scarring, and bronchiectasis. CT also revealed numerous small right
paratracheal and subcarinal air collections that communicated with the airway
(Fig. 1A). Fiberoptic
bronchoscopy revealed multiple diverticula along the right side of the
posterior tracheal wall (Figs.
1B and
1C). A culture of secretions
from the diverticula yielded Haemophilus parainfluenzae
organisms.
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Fig. 1A. —19-year-old man with congenital HIV infection, severe airflow
obstruction, and persistent cough. On chest CT scan, numerous tracheal
diverticula are visualized. Their communication with trachea via air-filled
necks is clearly seen.
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Fig. 1B. —19-year-old man with congenital HIV infection, severe airflow
obstruction, and persistent cough. Fiberoptic bronchoscopic images reveal that
trachea and carina have numerous well-circumscribed abnormalities along right
posterior tracheal wall (arrows, B). Image obtained at close
range in one lesion shows opening of diverticulum into trachea (C).
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Fig. 1C. —19-year-old man with congenital HIV infection, severe airflow
obstruction, and persistent cough. Fiberoptic bronchoscopic images reveal that
trachea and carina have numerous well-circumscribed abnormalities along right
posterior tracheal wall (arrows, B). Image obtained at close
range in one lesion shows opening of diverticulum into trachea (C).
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Review of a chest CT scan obtained when the patient was 10 years old showed
no tracheal diverticula. However, chest CT obtained when the patient was 15
years old revealed small air-filled tracheal diverticula with no clear
communication with the tracheobronchial tree.
Discussion
Tracheal diverticulosis is a rare finding usually diagnosed on radiographic
or bronchoscopic examination
[1,
2]. The few reported cases of
tracheal diverticulosis in the literature speculate regarding the etiology and
clinical significance of such abnormalities. Although some reports have
suggested postsurgical or congenital origins
[3–5],
others have identified severe chronic lung disease as a common denominator for
the development of diverticula
[6–7].
For patients with severe chronic lung disease, the combined effect of
weakening of the tracheal musculature due to recurrent tracheitis and
increased intratracheal pressures due to chronic cough may lead to the
formation of diverticula in a manner similar to that of the pulsion
diverticula of the esophagus. The preponderant right-sided location of the
diverticula (described in past reports as well as in ours) may reflect the
relatively weak structural support on the right side of the trachea. The
esophagus and aortic arch provide support to the left side of the airway and
thus make the left tracheal wall less susceptible to the development of
diverticula [7]. The
possibility that the diverticula are postinflammatory, however, is not
entirely excluded, given the presence of lymphocytic interstitial
pneumonitis–related mediastinal adenopathy on early images.
Although tracheal diverticula have been described in the past, to our
knowledge, ours is the first report of tracheal diverticulosis in a patient
congenitally infected with HIV. Because the initial generation of congenitally
infected patients is just now coming of age, there is little information
regarding the long-term pulmonary sequelae of congenitally acquired HIV
infection. This patient's history, coupled with the reported association of
diverticula with chronic lung disease, however, suggests that his
abnormalities are not the result of HIV infection alone but rather the result
of repeated pulmonary insults and chronic cough. Whether congenital HIV
infection increases the likelihood of developing such abnormalities has yet to
be determined, but individuals with HIV who have a history of recurrent
pneumonias are known to exhibit chronic pulmonary function abnormalities and
cough [8]. Currently, the
prevalence of tracheal diverticulosis in this patient population is
unknown.
The diagnosis of tracheal diverticula is best made on CT. In our patient,
the findings were not noted on chest radiography because of the marked
concurrent lung disease. The paratracheal air collections were small and could
not be distinguished from the extensive medial lung disease. CT, however,
clearly defined the abnormalities and displayed their communication with the
tracheobronchial tree. Fiberoptic bronchoscopy confirmed the findings and
allowed direct visualization and sampling of the diverticula.
The clinical significance of tracheal diverticulosis is unknown. The
abnormalities do not appear to progress rapidly. In the patient presented, CT
scans showed a small increase in the number of diverticula over the span of
approximately one decade, although there was not a noticeable change in the
patient's overall clinical condition. In select cases, surgical resection of
similar lesions has been performed to minimize the possibility of mucous
retention and recurrent infection
[5,
9]. Whether the diverticula act
as a reservoir for bacteria and contribute to repeated pulmonary infections
and eventual airflow obstruction is unknown. Indeed, numerous other airway
abnormalities such as bronchiectasis are frequently associated with recurrent
pneumonia.
In conclusion, tracheal diverticulosis should be considered in any person
with a long history of recurrent pulmonary insults and chronic cough. As the
population of patients with congenital HIV who survive into adulthood
increases, one may expect to encounter other airway and pulmonary
manifestations.
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Introduction
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