Kaohsiung J Med Sci December 2009 • Vol 25 • No 12 680
The incidence of tracheobronchial anomalies in the pediatric population ranges from 1% to 12%, and congenital bronchial stenosis is very uncommon [1–3]. The clinical presentation of tracheobronchial anomalies varies, and diagnosis is therefore difficult without the aid of imaging tools such as tracheo-bronchoscopy or computed tomography [1,4]. This
report describes a 3-year-old girl who presented at our emergency department with shortness of breath, wheezing and cyanosis. Focal stenosis in the orifice of the right upper lobe bronchus was detected inci-dentally by flexible tracheobronchoscopy (FTB) and confirmed by multidetector-row spiral computed tomography (MDCT).
C
ASEP
RESENTATIONA 3-year-old girl with a history of intermittent wheez-ing visited our emergency department because of acute dyspnea. Her body temperature was 37.6°C, Received: Feb 10, 2009 Accepted: Mar 27, 2009
Address correspondence and reprint requests to: Dr Zen-Kong Dai, Department of Pediatrics, Kaohsiung Medical University Hospital, 100 Shih-Chuan 1stRoad, Kaohsiung 807, Taiwan. E-mail: [email protected]
F
OCAL
S
TENOSIS IN
R
IGHT
U
PPER
L
OBE
B
RONCHUS IN A
R
ECURRENTLY
W
HEEZING
C
HILD
S
EQUENTIALLY
S
TUDIED BY
M
ULTIDETECTOR
-
ROW
S
PIRAL
C
OMPUTED
T
OMOGRAPHY
AND
S
CINTIGRAPHY
I-Chen Chen,1Jiunn-Ren Wu,2,3Chia-Ying Lu,4Chien-Ming Tseng,1Jong-Hau Hsu,1and Zen-Kong Dai1,5
1Division of Pediatric Cardiology and Pulmonology, Department of Pediatrics, and 4Department of
Medical Imaging, Kaohsiung Medical University Hospital; Department of Pediatrics,
2Kaohsiung Municipal Hsiao-Kang Hospital, Kaohsiung Medical University; 3Department of
Pediatrics, Faculty of Medicine; and 5Department of Pediatrics Graduate Institute of Medicine,
College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan.
Lower respiratory tract infections associated with wheezing are not uncommon in infants and young children. Among the wheezing-associated disorders, allergic etiologies are more com-monly encountered than anatomic anomalies. We present a 3-year-old girl with a sudden attack of asthmatic symptoms including dyspnea, cyanosis and diffuse wheezing. Based on a history of choking, and atelectasis in the right upper lobe detected by chest films, flexible tracheobron-choscopy was arranged and incidentally detected a stenotic orifice in the right upper lobe bronchus. Multidetector-row spiral computed tomography and pulmonary scintigraphy subse-quently also disclosed the focal stenosis. She suffered from recurrent wheezing, pneumonia and lung atelectasis during 1 year of follow-up. We emphasize the diagnosis, clinical course and man-agement of focal stenosis in the right upper lobe bronchus.
Key Words:flexible tracheobronchoscopy, multidetector-row spiral computed
tomography, ventilation and perfusion scintigraphy (Kaohsiung J Med Sci 2009;25:680–4)
Focal stenosis in the right upper bronchus respiratory rate was 64 breaths per minute, oxygen
saturation was 89%, heart rate was 192 beats per min-ute, and blood pressure was 94/70 mmHg. Her fam-ily reported that she had developed severe coughing after consuming some nuts the day before admission. Slight subcostal retraction of the chest wall was noted on physical examination. Breath sounds included bilat-eral wheezing. A chest X-ray revealed upward devia-tion of the minor fissure and a wedge-shaped opacity in the right upper lobe, suggesting atelectasis in the right upper lobe. A small area of opacity in the right lower lung was also found, and bronchopneumonia was suspected (Figure 1). The patient was admitted to our pediatric intensive care unit with suspected for-eign body aspiration, based on the reported choking history.
Laboratory data revealed elevated C-reactive pro-tein (29.1 mg/L) and leukocytosis (white blood cells, 17,900/μL; Seg/Lym, 83%/12%). FTB was performed immediately. Severe stenosis of the orifice of the right upper lobe bronchus was detected, which did not allow the 2.0-mm bronchoscope to pass. There was no foreign body or structural anomaly noted in the bronchi. Due to suspected bacterial pneumonia and asthma attack, the patient was given empiric antibiotic therapy with ampicillin/sulbactam (150 mg/kg every 6 hours), bronchodilators, and an inhaled steroid. However, the wheezing persisted and was refractory to bronchodilators. MDCT was performed and re-vealed narrowing of the orifice of the right upper lobe bronchus, relative to the diameter of the remainder of
the right upper lobe bronchus, without external com-pression (Figure 2).
The patient also underwent ventilation and perfu-sion scintigraphy (V/Q scans). The perfuperfu-sion scan showed heterogeneous radiotracer distribution with defects in the bilateral pulmonary fields (Figure 3A). The ventilation scan showed prolonged radioaerosol alveolar epithelial permeability in the right upper lung field (114 minutes), indicating relative obstruc-tion compared with the left upper lung (85 minutes) (Figure 3B). She was discharged 2 weeks later in a rel-atively stable condition. However, her symptoms re-curred 7 months later, in the absence of any choking event. A second chest MDCT scan was performed, which showed the same focal narrowing of the right upper lobe bronchus. The patient suffered from four episodes of recurrent wheezing, pneumonia and lung atelectasis during the following year. Surgical resection of the right upper lobe was therefore sug-gested, but declined by the patient’s parents.
D
ISCUSSIONWheezing is one of the main clinical manifestations of small airway narrowing in infants and young chil-dren. The causes of airway narrowing are diverse, and include congenital causes, trauma, infection, in-halational burns, and amyloid deposition [5,6]. The presentation of airway abnormalities may mimic other respiratory diseases such as asthma, bronchi-olitis, and aspiration pneumonia. FTB is performed if there is a suspicion of foreign body aspiration or per-sistent wheezing despite treatment [7]. Compared with MDCT, which also plays an important role in the evaluation of tracheobronchial abnormalities in children, FTB can be used to perform therapeutic ma-neuvers and provide tissue biopsies, show bronchial mucosal morphology, and involves no radiation ex-posure [8]. However, unlike MDCT, it cannot provide three-dimensional information on adjacent soft tissue structures and the poststenotic airway [9–11].
Lung scintigraphy is another noninvasive tool that can be used for functional assessment of the lung in children [12]. In a study in dogs, artificial occlusion of a sublobar bronchus increased the ventilation to the obstructive lung, induced a shift of blood to the rest of the lung, and increased the ratio between mean ventilation and mean perfusion [13]. The increased
Figure 1.Chest X-ray shows right upper lobe atelectasis and mild opacity in the right lower lung, suggesting a possible infectious process.
A B
Figure 2.(A) Multidetector-row spiral computed tomography demonstrating that the orifice of the right upper lobe bronchus is nar-rowed relative to the diameter of the rest of the right upper lobe bronchus (white arrow). (B) The axial view reveals similar findings (arrowhead).
A B
Figure 3.Ventilation and perfusion scintigraphy (V/Q scan) reveals a mismatch in the right upper lobe. The perfusion image (A) appears normal, whereas the ventilation image (B) shows stasis in the right upper lobe.
ventilation to the obstructive lung may have been due to increased communications with collateral ven-tilation [13]. As in our patient, the stasis of venven-tilation in the focal stenotic area could possibly be explained by regional mismatching between ventilation and perfusion. In other words, hypoxia vasoconstriction phenomenon can explain the diversion of blood flow to oxygen-rich areas. However, the normal perfusion results in our patient may have been due to the par-tial, but not total, occlusion of the bronchus. Hauck et al demonstrated a positive correlation among V/Q scans, lung function parameters and gas exchange [14], which prompted us to follow-up the regional lung function in this patient with a V/Q scan. Subsequent
V/Q scans during the asymptomatic period, after anti-inflammatory medication with montelukast and inhaled budesonide, showed improvements in venti-lation and V/Q mismatch in the right upper lobes.
The clinical presentation of tracheobronchial anom-alies varies from asymptomatic to severe respiratory distress [1,15]. In this patient, recurrent lower respi-ratory tract infection, lung atelectasis and wheezing were observed at follow-up. Her symptoms could have been caused by stenosis of the right upper lobe bronchus. Frequent wheezing episodes since later infancy suggested that her condition was congenital in nature. To the best of our knowledge, congenital focal stenosis of the orifice in the right upper lobe
bronchus is rare, compared with acquired causes, such as trauma, infection, inhalational burns, and amyloid deposition [5,6]. In addition, chest film abnormalities during the recurrent episodes suggestive of pneumo-nia and atelectasis were also noted in the surround-ing lobes, includsurround-ing the right middle and lower lobes. In addition to mucoid impaction in the distal bronchus which can easily cause recurrent infection and lung atelectasis, some authors have hypothesized that the infectious process can migrate proximally to cause inflammation in the trachea and/or adjacent mainstem bronchus [6].
Management of airway stenosis is complex and depends on the degree, length and location of the stenosis. There are several strategies used to deal with tracheal and bronchial stenosis, including medical treatment, endoscopic dilatation, stent placement, and surgical resection [5,6,16]. Surgical intervention was suggested in this patient, but her parents refused. However, asymptomatic intervals could be achieved through the use of the anti-inflammatory agents telukast and inhaled budesonide. The effects of mon-telukast and inhaled budesonide on inflammatory airway narrowing have been proven [17,18], but their effects on congenital focal bronchial stenosis need further study.
In conclusion, this case highlights the importance of considering airway abnormalities in patients with periodic wheezing, recurrent pulmonary infections, or atelectasis on chest films. FTB is the first step in diagnosing airway abnormalities or sputum impaction of isolated lobe bronchus, though other diagnostic workups, including MDCT and V/Q scans, should be used as adjuvant tools for evaluating focal bronchial tree abnormalities.
R
EFERENCES1. Sanchez I, Navarro H, Mendez M, et al. Clinical char-acteristics of children with tracheobronchial anom-alies. Pediatr Pulmonol 2003;35:288–91.
2. Anton-Pacheco JL, Galletti L, Cabezali D, et al. Man-agement of bilateral congenital bronchial stenosis in an infant. J Pediatr Surg 2007;42:E1–3.
3. Ghaye B, Szapiro D, Fanchamps JM, et al. Congenital bronchial abnormalities revisited. Radiographics 2001; 21:105–19.
4. Vevecka E, De Boeck K, Moerman P, et al. Tracheal bronchus associated with congenital cystic adenoma-toid malformation. Pediatr Pulmonol 1995;20:413–6. 5. Beddow E, Rice AJ, Sheppard MN, et al. Idiopathic
tra-cheal and bronchial stenosis in two pediatric patients. Ann Thorac Surg 2004;77:1829–32.
6. Shipchandler TZ, Geelan-Hansen K, Krakovitz PR. Complete bronchial stricture and airway management challenges. Am J Otolaryngol 2008;29:195–7.
7. Aslan AT, Kiper N, Dogru D, et al. Diagnostic value of flexible bronchoscopy in children with persistent and recurrent wheezing. Allergy Asthma Proc 2005;26:483–6. 8. Haliloglu M, Ciftci AO, Oto A, et al. CT virtual bron-choscopy in the evaluation of children with suspected foreign body aspiration. Eur J Radiol 2003;48:188–92. 9. Lee EY, Siegel MJ. MDCT of tracheobronchial
narrow-ing in pediatric patients. J Thorac Imagnarrow-ing 2007;22:3009. 10. Hoppe H, Walder B, Sonnenschein M, et al.
Multi-detector CT virtual bronchoscopy to grade tracheo-bronchial stenosis. AJR Am J Roentgenol 2002;178: 1195–200.
11. Heyer CM, Nuesslein TG, Jung D, et al. Tracheobronchial anomalies and stenoses: detection with low-dose multidetector CT with virtual tracheo-bronchoscopy—comparison with flexible tracheobron-choscopy. Radiology 2007;242:542–9.
12. Ciofetta G, Piepsz A, Roca I, et al. Guidelines for lung scintigraphy in children. Eur J Nucl Med Mol Imaging 2007;34:1518–26.
13. Clercx C, van den Brom WE, Stokhof AA, et al. Pulmonary scintigraphy in canine lobar and sublobar airway obstruction. Lung 1989;167:213–24.
14. Hauck RW, Romer W, Schulz C, et al. Ventilation perfu-sion scintigraphy and lung function testing to assess metal stent efficacy. J Nucl Med 1997;38:1584–9. 15. Lodha R, Puranik M, Natchu UC, et al. Recurrent
pneumonia in children: clinical profile and underlying causes. Acta Paediatr 2002;91:1170–3.
16. Takahashi N, Horie T. Medical treatment for bronchial stenosis due to endobronchial tuberculosis. Kekkaku 1999;74:885–9. [In Japanese]
17. Bel EH, Timmers MC, Zwinderman AH, et al. The effect of inhaled corticosteroids on the maximal degree of airway narrowing to methacholine in asthmatic sub-jects. Am Rev Respir Dis 1991;143:109–13.
18. Joos S, Miksch A, Szecsenyi J, et al. Montelukast as add-on therapy to inhaled corticosteroids in the treat-ment of mild to moderate asthma: a systematic review. Thorax 2008;63:453–62.
