1) A one-month-old male infant presented with respiratory distress for 24 hours including stridor, retractions, and coughing. He received albuterol and steroids prior to arriving at the emergency department.
2) Initial management included oxygen, racemic epinephrine, labs, IV access, and imaging. Imaging revealed a double aortic arch causing extrinsic airway compression.
3) A double aortic arch is a vascular ring anomaly where both aortic arches persist, potentially compressing the trachea. Surgical correction is usually required to prevent long-term respiratory issues.
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A case in respiratory distress
1. A Case in Respiratory
Distress
G. Patrick Daubert, MD
Emergency Medicine Resident
Detroit Receiving Hospital
2. Presentation
One month old male infant presenting to the
emergency department for respiratory distress
Parents state that the patient has had difficulty
breathing for the past 24 hours which has
progressively gotten worse
Patient was seen by primary medical doctor this
morning and received albuterol nebulizers and 1
mg of Decadron intramuscularly prior to being
transferred to the emergency department
Patient arrived to the emergency department in
moderate to severe respiratory distress with
stridor
3. History of Present Illness
Infant was born at 38 weeks gestational age
via a spontaneous vaginal delivery without
complication.
Over the past week he has developed post-
prandial respiratory distress. Distress
described as stridor, retractions, and cough.
Night before presentation, the infant had an
attack of heavy coughing with short apneic
episode.
4. Primary Physician
Yes – Pediatrician
Medical History
None,
Surgical History
None
Birth History
38 weeks gestation
Mother G4P4004
Apgar scores of 8/9
No complications with the
pregnancy; 8lb2oz
HBV Vaccine at birth
Allergies
None known
Family History
No congenital defects
known
Social History
3 siblings, No day care,
no exposure to smoke in
the home, no sick
contacts
Normal prenatal care
Past History
5. Review of Systems
Constitutional: No history of fever, acting
apporpriate for age.
Eyes: No redness or drainage.
Ears, nose, Mouth, Throat: No congestion or
drainage.
Cardiovascular: Good colour. No history of
congenital cardiac disease, no difficulty with
nippling
Respiratory: Post-prandial cough, positive stridor,
positive retractions
Gastrointestinal: One episode of vomiting 4 days
PTA, (white in colour), no diarrhea, breast milk
feeding,
Genitourinary: 6-7 wet diapers/day, stool 2-3/day
Musculoskeletal: No swelling, deformity, nor change
in movements or posture.
Skin: No swelling, rash, no lesions.
7. Physical Exam
VS 38.0 rectally, P 172, RR 72, 5 kg, sat 91% RA
General: Patient in respiratory distress with
audible wheezing and notable stridor. The infant
was moving all four extremities and did not appear
to be lethargic.
HEENT: PERRL. EOMI. Anicteric sclerae, pink
conjunctivae. Oropharynx was clear. There was no
tonsillar exudate or enlargement. Moist mucous
membranes.
Neck, supple, no JVD, positive stridor.
8. Physical Exam Continued…
RESP: Stridorous. Mild, late wheezing, IC/SC
retractions noted. No grunting.
CVS: Regular rhythm and rate, no murmurs.
Abdomen, soft, nontender to palpation, no
hepatosplenomegaly.
MS: Pulses were equal upper and lower
extremities bilaterally. Capillary refill is two
seconds, no peripheral edema.
SKIN: warm and dry, no petechiae or bruising;
There is no rash on the palms or soles of the feet.
NEUROLOGIC: Patient moving all four
extremities. Arousable and consolable.
10. Initial Management in the ER
Patient was given humidified oxygen which
improved his saturation to 99%
Patient was given racemic epinephrine 0.5
mg nebulizer with nearly complete
resolution of his stridor
CBC, electrolytes, and blood cultures
ordered
IV was established
Chest and Neck soft tissue x-rays
obtained...
16. Diagnosis..
.
There was a double aortic arch with a
ligamentum arteriosum.
The arches were approximately equal in
size, measuring about 5 mm in diameter
each.
The left (anterior) arch clearly gave off the
left subclavian artery and the left common
carotid artery
Double Aortic Arch
18. Determining the Site of
Obstruction…
5 important clinical observations
Rate and depth of respirations
Presence of retractions
Inspiratory stridor
Expiratory wheezing
Grunting
20. Arterial Blood Gases
Obstructions above the carina
ABG reflects overall alveolar
hypoventilation
This manifests as PaCO2 and
proportionate PaO2
Since there is no parenchymal disease
causing R L shunting, hypoxia responds
well to supplemental O2
21. Arterial Blood Gases
Intrapulmonary airway obstruction
ABG reflect ventilation/perfusion imbalance and
venous admixture - caused by non-uniform
obstruction throughout the lungs
In mild cases hyperventilated areas predominate
resulting in a PaCO2
However, the PaO2 in these areas cannot
compensate for the PaO2 in hypoventilated areas
resulting in venous admixture and a total PaO2
and PaCO2
Response to O2 depends on the amount of venous
admixture and illness severity
22. Double Aortic Arch
Double aortic arch is the most common
vascular abnormality requiring operation
In this condition, the trachea or esophagus
are completely encircled by the bilateral aortic
arches owing to persistence of both right and
left embryologic fourth aortic arches
Both aortic arches may be patent, or one may
be atretic and remaining as a fibrotic cord
23. Double Aortic Arch
First surgical correction in 1945
The incidence of anomalies of this type is not
known, as many are asymptomatic
There is no predilection of these anomalies
for either sex
Abnormal vascular structures causing
extrinsic compression of the trachea is
uncommon, but is a common cause of
serious, persistent respiratory problems in
infancy
24. Double Aortic Arch
Clinical manifestations may vary
considerably depending on the type of
lesion and severity of encroachment on
trachea, bronchi or esophagus
The most common presentation is the
picture of airway obstruction, mainly
stridor, wheezing, or excessive
secretion
25. Double Aortic Arch
In the newborn, respiratory symptoms are usually
mild and rarely recognized
Symptoms usually become apparent during the first
few months of life
Patients often have a history of recurrent respiratory
embarrassment, or infection, requiring frequent
medical attention
Some have been referred for evaluation of a possible
allergic problem such as asthma
Symptoms are frequently exacerbated by crying or
exertion
26. Double Aortic Arch
All cases reported to date have had a left side
ductus arteriosus
The luminal size of the two arches in relation to
each other varies considerably
Occasionally double aortic arch has been
reported to be found in combination with other
congenital cardiac anomalies
In one series of 16 cases of double aortic arch
associated with intracardiac defects, 15 of the 16 were
cyanotic and included Tetralogy of Fallot and
transposition of the great arteries.
Several cases with coarctation in one of the aortic
branches have been reported.
27. Double Aortic Arch
A chest roentgenogram with barium filling the
esophagous is the simplest and yet the most
informative procedure in the evaluation of
patients who are suspected of having a
vascular abnormality
Most aortic arch anomalies causing vascular
rings have a posterior esophageal indentation
Careful review of the standard chest xray may
be helpful initially to evaluate tracheal or
bronchial abnormalities such as an abnormal
shift or constriction
28. Double Aortic Arch
If airway compromise is significant, surgical
intervention should be undertaken without
delay
Operative mortality in recent series is ~5%
The prognosis for survival is generally good in
most patients with aortic arch anomalies
Some patients may have such significant
damage or distortion of the tracheal bronchial
tree owing to chronic compression that
severe respiratory symptoms may persist
postoperatively
