Wheezing - Case 1-1: 8-Month-Old Girl
Wheezing - Case 1-1: 8-Month-Old Girl: Excerpt from Pediatric Complaints and Diagnostic Dilemmas
I. History of Present Illness
An 8-month-old girl presented to the emergency department for the third
consecutive day with parental complaints of wheezing and cough. Two days before
admission she was seen in the emergency department, diagnosed with
bronchiolitis and otitis media, and discharged on amoxicillin, nebulized
albuterol, and prednisolone. One day before admission, she was again evaluated
in the emergency department for continued wheezing and cough, which improved
with nebulized albuterol. A chest roentgenogram demonstrated hyperinflation and
peribronchiolar thickening. There was no cardiomegaly or pleural effusion. On
the day of admission, her cough was accompanied by two episodes of perioral
cyanosis. She had decreased oral intake and urine output and was febrile to
39.7
°C at home.
II. Past Medical History
Her past history was remarkable for frequent episodes of wheezing since 5 months
of age. She had received nebulized albuterol intermittently, including every 4
hours for the past month, without significant improvement in her wheezing. Her
cough was worse at night but did not seem to be worse with feeding or supine
positioning. Her birth history was unremarkable, and the prenatal ultrasound
was reportedly normal.
III. Physical Examination
Temperature (T), 38.3°C; respiration rate (RR), 60/min; heart rate (HR), 110 bpm; blood pressure (BP),
110/55 mm Hg; and pulse oximetry (SpO
2), 100% in room air
Height, 25th percentile; weight, 25th percentile; head circumference, 25th
percentile
Initial examination revealed a well-nourished, acyanotic infant in moderate
respiratory distress. Physical examination was remarkable for purulent
rhinorrhea and buccal mucosal thrush. Moderate intercostal and subcostal
retractions were present. There was fair lung aeration with diffuse expiratory
wheezing. No murmurs or gallops were heard on cardiac examination, and femoral
pulses were palpable. No hepatomegaly or splenomegaly was present.
IV. Diagnostic Studies
Laboratory analysis revealed 14,600 white blood cells (WBCs)/mm3, with 38% segmented neutrophils, 53% lymphocytes, and no band forms. The
hemoglobin was 11.0 g/dL, and there were 580,000 platelets/mm
3. Electrolytes, blood urea nitrogen, and creatinine were normal. Polymerase
chain reaction performed on nasopharyngeal aspirate was negative for
Bordetella pertussis. Antigens of adenovirus; influenza A and B viruses; parainfluenza virus types 1,
2, and 3; and respiratory syncytial virus (RSV) were not detected by
immunofluorescence of nasopharyngeal washings. However, RSV subsequently grew
in viral culture of the nasopharyngeal aspirate. Blood and urine cultures were
negative.
V. Course of Illness
The patient was diagnosed with bronchiolitis. Her tachypnea and wheezing
improved with nebulized albuterol and oral prednisolone. She was discharged
after 3 days of hospitalization, receiving albuterol every 4 hours. A
radionuclide milk scan was scheduled on an outpatient basis to assess for the
presence of GER and pulmonary aspiration.
Ten days later, the patient returned to the emergency room with increased
wheezing and recurrence of her fever. She had poor oral intake, which had not
improved significantly since the last admission and was now accompanied by
frequent emesis. She was admitted for treatment and further evaluation. Her
radionuclide milk scan, which had been performed between admissions, revealed
GER without pulmonary aspiration. During her current admission, careful
examination of the chest radiograph suggested the diagnosis (Fig. 1-1A,B).
Magnetic resonance imaging (MRI) of the chest confirmed this diagnosis (Fig.
1-1C).
Discussion: Case 1-1
I. Differential Diagnosis
The causes of recurrent or persistent wheezing in the infant are diverse. Common
causes of recurrent wheezing in infancy include bronchiolitis, reactive airways
disease, and GER with microaspiration. Less commonly, recurrent wheezing is
caused by congenital abnormalities of the lung or respiratory tract (cystic
adenomatous malformations, TEF), diaphragmatic abnormalities (paralysis of the
diaphragm, diaphragmatic hernia), cystic fibrosis, or immunologic defects
(congenital absence of thymus, DiGeorge syndrome, chronic granulomatous
disease, gammaglobulin deficiencies). Rarely, anomalies of the major arterial
branches of the aorta or pulmonary blood vessels compress the trachea and
bronchi of the infant, causing acute or progressive respiratory distress. The
features of this case that prompted additional evaluation included recurrent
episodes of wheezing, incomplete resolution of wheezing despite prolonged
β-agonist therapy, and episodes of cyanosis.
II. Diagnosis
The chest radiographs revealed a midline trachea with bilateral indentations in
the anteroposterior projection (Fig. 1-1A) and anterior bowing of the trachea
on the lateral projection (Fig. 1-1B). These findings suggested the diagnosis
of double aortic arch. MRI of the chest showed the bifurcation of this double
arch as the
“horseshoe”-appearing structure surrounding the trachea in the center of the image (Fig.
1-1C). There were no associated structural defects of the heart.
The diagnosis is double aortic arch.
III. Incidence and Anatomy of Vascular Rings and Slings
Vascular anomalies, commonly referred to “vascular rings and slings,” can cause tracheal or esophageal compression leading to respiratory symptoms or
feeding difficulty. The term
vascular ring refers to any aortic arch anomaly in which the trachea and esophagus are
completely surrounded by vascular structures. The vascular structures need not
be patent. For example, a ligamentum arteriosum may complete a ring. A vascular
or pulmonary sling is an anomaly in which vascular structures only partially
surround the lower trachea but cause tracheal compression. Vascular rings are
seen in fewer than 1% of congenital cardiac anomalies.
The most commonly occurring rings and slings are described in the following
paragraphs (Fig. 1-2).
Double aortic arch. This is the most common clinically recognized form of vascular ring; as the name
implies, both right and left aortic arches are present.
“Left” and “right” refer to which bronchus is crossed by the arch, not which side of the midline
the aortic root ascends. The ascending aorta divides anterior to the trachea
into left and right arches, which then pass on either side of the trachea. The
right arch is usually higher and larger and gives rise to the right common
carotid and right subclavian arteries. The right arch travels posteriorly and
indents the right side of the trachea and the right and posterior portions of
the esophagus, as it passes behind the esophagus to join the left arch at the
junction of the left-sided descending aorta. The left arch gives rise to the
left common carotid and left subclavian arteries. The left arch is located
anteriorly and indents the left side of the trachea and esophagus as it joins
the descending aorta. Double aortic arch is rarely associated with congenital
heart disease. If congenital disease is present, tetralogy of Fallot is the
most common condition, and transposition of the great arteries is occasionally
seen. Surgical division of one of the arches, usually the smaller one, is
curative. Respiratory symptoms may persist for months postoperatively due to
prolonged deformity of the tracheobronchial tree.
Aberrant right subclavian artery. This condition is also known as left aortic arch with retroesophageal right
subclavian artery. It is the most common aortic arch malformation noted on
postmortem examination. The incidence of this abnormality in the general
population is approximately 0.5%. Aberrant right subclavian artery was found in
0.9% of 3,427 consecutive patients undergoing cardiac catheterization at The
Children
's Hospital of Philadelphia; it represented 20% of aortic arch anomalies found at
catheterization. It is also seen in approximately one third of patients with
Down syndrome and congenital heart disease. The left aortic arch has a normal
course to the left and anterior to the trachea. However, the right subclavian
artery arises as the last branch of the arch and runs posteriorly from the
descending thoracic aorta to reach the right arm, passing obliquely up to and
right behind the esophagus, indenting it posteriorly. Although most patients
with this anomaly are asymptomatic, an older patient may complain of dysphagia.
Symptomatic anterior tracheal compression results if there is a common origin
of both carotid arteries in conjunction with a retroesophageal aberrant right
subclavian artery. Rarely, an anomalous right subclavian artery in association
with a left aortic arch, retroesophageal descending aorta, and right ligamentum
arteriosum produces a symptomatic vascular ring.
Right aortic arch with anomalous left subclavian artery and left ductus
arteriosus or ligamentum arteriosum.
The aortic arch passes to the right of the trachea, becomes retroesophageal, and
descends on left. The first branch is the left carotid artery; the second, the
right carotid artery; the third, the right subclavian artery; and the fourth,
the left subclavian artery, which arises from the descending aorta. The ductus
arteriosus originates from a retroesophageal diverticulum of the descending
aorta, courses to the left, and connects to the pulmonary artery. Patients are
usually asymptomatic. However, some patients present with wheezing or stridor
due to tracheal compression and require surgical division of the ligamentum
arteriosum. Older children with dysphagia may require relief of esophageal
compression by actual division of the aortic arch. The retroesophageal portion
is mobilized, and reanastomosis of ascending and descending portions of the
aorta is completed with the use of a graft.
Aberrant left pulmonary artery (pulmonary sling). A normal pulmonary artery is absent, and the left lung is supplied by an
anomalous left pulmonary artery that arises from the distal right pulmonary
artery. The vessel courses to the right of the trachea and then passes between
the trachea and the esophagus, causing compression of the right main stem
bronchus, trachea, and esophagus. The resulting compression of the right main
stem bronchus and trachea leads to airway obstruction, primarily affecting the
right lung. Two thirds of affected infants present in the first month of life
with wheezing, stridor, or apnea. Dysphagia is rare. There may be associated
collapse or hyperinflation of the right lung. Aberrant left pulmonary artery is
frequently associated with complete cartilaginous rings in the distal trachea,
resulting in tracheal stenosis. It usually appears as an isolated abnormality
but can be associated with other congenital cardiac defects, particularly
tetralogy of Fallot. Surgical repair involves division of the left pulmonary
artery from the right and their reanastomosis in front of the trachea.
Bronchoscopy is performed at the time of surgical repair because of the
frequent association of complete cartilaginous rings causing tracheal stenosis.
IV. Clinical Presentation of Vascular Rings and Slings
Most infants present with symptoms in early infancy. Superimposed viral
infection with edema of the trachea or bronchi may account for or contribute to
the respiratory symptoms. Asymptomatic infants, particularly those with
aberrant right subclavian artery, are sometimes diagnosed incidentally on the
basis of a chest roentgenogram taken during a viral respiratory illness.
The symptoms of a vascular ring or sling are caused by tracheal compression and,
to a lesser degree, esophageal compression. Symptoms of tracheal compression
include wheezing, stridor, and apnea. Some infants hyperextend their necks to
reduce tracheal compression. Symptoms related to esophageal compression include
emesis, choking, and nonspecific feeding difficulties in infants and dysphagia
in older children. Less severe obstructions may cause recurrent respiratory
infections as a result of aspiration or inadequate clearing of respiratory
secretions.
V. Diagnostic Approach
Clinicians should have a high index of suspicion for a vascular anomaly in the
evaluation of an infant with recurrent wheezing. Chest roentgenography and
barium esophagography should be considered in the initial evaluation.
Chest roentgenogram. The diagnosis of a vascular ring may be suspected before a barium esophagogram
is performed. The chest roentgenogram should be examined to assess laterality
of the aortic arch and for evidence of tracheal or bronchial compression. The
following features on chest roentgenogram are suggestive of a vascular anomaly
and require additional evaluation. (a) A midline trachea in which there is no
rotation of the patient or a sharp indentation on the right side of the trachea
above the carina suggest a right aortic arch; the normal infant
's trachea is slightly displaced to the right by the normal left arch. (b)
Lateral displacement of the right mediastinal pleural line indicates a right
descending aorta. (c) Anterior bowing of the trachea rather than a normal
posterior convexity on the lateral view indicates compression. Generalized or
focal areas of hyperinflation due to tracheal or bronchial compression can be
mistakenly diagnosed as a foreign body aspiration.
Barium esophagogram. Posterior (vascular ring) or anterior (pulmonary sling) indentation of the
esophagus is frequently seen and suggests the diagnosis.
Magnetic resonance imaging. MRI has been shown to give excellent anatomic detail and is helpful in planning
reconstructive procedures.
Angiogram and transthoracic echocardiogram. In the absence of any other cardiac defect, angiography is probably unnecessary
for diagnosis. It may be helpful in delineating the anatomic detail necessary
for surgical correction. Transthoracic echocardiography is important to detect
associated congenital cardiac defects but is less reliable at delineating
vascular and tracheal anatomy.
Bronchoscopy. This allows direct visualization of compression on the trachea and is indicated
for suspected tracheal stenosis present.
VI. Treatment
Surgical management is necessary to relieve symptomatic obstruction of trachea
and esophagus. Surgery should also be considered if the infant has frequent
respiratory infections or is failing to thrive. The infant with severe
preoperative respiratory symptoms is likely to have postoperative
tracheomalacia from prolonged compression by the vascular ring. However,
feeding difficulties resolve rapidly.
VII. References
1. Berdon WE, Baker DH. Vascular anomalies and the infant lung: rings, slings,
and other things.
Semin Roentgenol 1972;7:39–63.
2. Edwards JE. Malformations of the aortic arch system manifested as “vascular rings.” Lab Invest 1953;2:56–75.
3. Goldstein WB. Aberrant right subclavian artery in mongolism. Am J Roentgenol 1965;95:131–134.
4. Hawker RE, Celermajer JM, Cartmill TB, et al. Double aortic arch and complex
cardiac malformations.
Br Heart J 1972;34:1311–1313.
5. Moes CAF, Freedom RM. Rare types of aortic arch anomalies. Pediatr Cardiol 1993;14:93–101.
6. Weinberg PM. Aortic arch anomalies. In: Emmanouilides GC, Riemenschneider TA,
Allen HD, et al., eds.
Moss and Adams' heart disease in infants, children, and adolescents, including the fetus and
young adult,
5th ed. Baltimore: Williams & Wilkins, 1995:810–837.
Pictures
Book Source Details
- Book Title: Pediatric Complaints and Diagnostic Dilemmas
- Author(s): Samir S Shah MD; Stephen Ludwig MD
- Year of Publication: 2003
- Copyright Details: Pediatric Complaints and Diagnostic Dilemmas, Copyright © 2003 Lippincott Williams & Wilkins.
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