Wheezing - Case 1-4: 15-Month-Old Girl
I. History of Present Illness
The patient was a 15-month-old girl hospitalized for respiratory distress. She
was well until 3 days before admission, when she began coughing during a meal.
She had moderate respiratory distress initially but gradually improved. On the
day of admission, she had a fever to 39.5
°C and worsening tachypnea. She received albuterol with some improvement in her
respiratory status.
II. Past Medical History
The patient was born at term after an uncomplicated pregnancy and delivery. She
was diagnosed with reactive airways disease in infancy after three
hospitalizations for wheezing. She received nebulized albuterol and prednisone
for 5 days during each of those episodes. Mild GER was diagnosed by a pH probe
study at 7 months of age; an upper gastrointestinal barium study performed at
that time was normal. At 11 months of age, ranitidine and metoclopramide were
started, after which her symptoms of chronic cough improved. She was treated
with intravenous antibiotics for a right middle lobe pneumonia at 12 months of
age. At the time of presentation, she was receiving metoclopramide for GER, as
well as nebulized albuterol for wheezing approximately twice per week. A
distant relative was diagnosed with cystic fibrosis a decade ago and died
during infancy. There was no family history of atopy or asthma.
III. Physical Examination
T, 37.4°C; RR, 44/min; HR, 110 bpm; BP, 103/65 mm Hg; SpO2, 96% in room air
Weight, 16.5 kg (10th percentile); height, 105 cm (25th percentile)
Physical examination revealed a thin child in mild respiratory distress. There
was no conjunctival infection. The sinuses demonstrated symmetric
transillumination. The oropharynx was clear. There was no cervical
lymphadenopathy. There were mild intercostal retractions with good aeration and
mild diffuse wheezing. Breath sounds were slightly diminished in the right
lower lobe. The cardiac examination was normal. There were no rashes or skin
lesions. The remainder of the examination, including the neurologic
examination, was normal.
IV. Diagnostic Studies
Her WBC count was 18,300 mm3, with 9% band forms, 78% segmented neutrophils, and 13% lymphocytes. Her
hemoglobin and platelet counts were normal. A blood culture was obtained and
subsequently was found to be negative. Chest radiography revealed a right
middle lobe density. There was no hyperinflation or peribronchial thickening.
V. Course of Illness
A repeat upper gastrointestinal barium study was performed (Fig. 1-6) while
other diagnostic possibilities were considered.
Discussion: Case 1-4
I. Differential Diagnosis
The most common cause of recurrent wheezing in a young infant is GER with
pulmonary aspiration. Other causes of recurrent aspiration include
cricopharyngeal incoordination, submucosal cleft palate, seizures,
neuromuscular disorders, and TEF. Esophageal obstruction due to webs or
strictures may also predispose to recurrent aspiration.
Although bronchiolitis and poorly controlled reactive airways disease remain a
consideration, the frequency of wheezing episodes and the recurrent pneumonia
warrant further investigation. Cystic fibrosis should be excluded, particularly
in light of the family history. The differential diagnosis also includes
extrinsic obstructing lesions such as mediastinal lymphadenopathy,
diaphragmatic hernia, and vascular ring. Intraluminal obstructing lesions can
occur in this age group and include aspirated foreign body, bronchial papilloma
or lipoma, and segmental bronchomalacia. The history of recurrent pneumonia may
be a sign of underlying primary immunodeficiency
—for example, an agammaglobulinemia, a dysgammaglobulinemia, or a phagocytic
defect such as chronic granulomatous disease, which occasionally exhibits
autosomal recessive inheritance. Infectious causes of recurrent or persistent
pneumonia, such as
Coxiella burnetii (Q fever), Histoplasma capsulatum, and Mycobacterium tuberculosis, are less likely in this age group.
II. Diagnosis
On very direct questioning, her mother stated that the child's cough, although chronic, seemed worse when she drank liquids. This
information, combined with a history of right middle lobe pneumonia, chronic
cough, and recurrent wheezing, suggested chronic aspiration. The sweat test was
negative, but the contrast esophagogram (Fig. 1-6) revealed filling of the
trachea through a small esophageal fistula.
The diagnosis is TEF without esophageal atresia (H-type fistula). In retrospect, this patient's symptomatic improvement at 11 months of age coincided with her transition from
a predominantly liquid to predominantly solid diet.
III. Incidence and Epidemiology
TEF with or without esophageal atresia occurs as a congenital pulmonary
abnormality in 1 of every 3,000 to 5,000 live births. The most common form of
TEF, proximal esophageal atresia with distal TEF, occurs in 85% of cases of
TEF. Communication between the trachea and esophagus with an otherwise normal
esophagus, known as an H-type TEF, occurs in 3% to 5% of cases. Approximately
10% of cases of esophageal atresia are not associated with TEF.
The anomalies arise from defective differentiation of the primitive foregut into
trachea and esophagus. The role of genetic factors is unclear; TEF has been
described in siblings and identical twins. Autosomal dominant transmission has
been reported in a few kindreds. Approximately 40% of infants with a TEF have
associated congenital anomalies, usually cardiac or gastrointestinal, including
anal atresia, pyloric stenosis, duodenal obstruction, and malrotation. One
cluster of congenital abnormalities, the VATER association (
Vertebral anomalies, Anal anomalies, Tracheoesophageal fistula with Esophageal atresia, and Renal and Radial limb anomalies), is seen most often among infants of diabetic mothers.
IV. Clinical Presentation
Infants with TEF and esophageal atresia are symptomatic from birth. They
accumulate large amounts of oral secretions, which precipitate coughing,
choking, emesis, and respiratory distress. Abdominal distention results from
accumulation of intestinal air via the TEF. A flat, gasless abdomen suggests
esophageal atresia either without a TEF or with an obliterated TEF that still
requires surgical repair.
Infants with an H-type TEF do not present in the neonatal period. Instead, their
symptoms are mild or moderate and persistent. Symptoms in infants with H-type
fistulas include coughing, choking, and cyanosis with feedings. Because the
tracheoesophageal connection is small, these symptoms usually occur with liquid
or formula feedings. There is no dysphagia. Children with H-type TEF may have
improvement of their symptoms when they make the transition from formula to
more solid foods. Many children have recurrent episodes of pneumonia or
pneumonitis due to aspiration of gastrointestinal contents through the fistula.
On examination, abdominal distention occurs after crying as air traverses
through the fistula into the stomach. Diffuse wheezing may be related to
aspiration.
V. Diagnostic Approach
Nasogastric tube placement and chest radiography. Esophageal atresia with or without TEF is easily detected by attempted passage
of a radiopaque 5 Fr or 8 Fr nasogastric tube. The tube will coil in the
proximal pouch and can be seen on chest radiograph. H-type TEF is more
difficult to detect. Chest radiography may show evidence of recurrent pulmonary
aspiration, particularly in the right upper or right middle lobe.
Contrast esophagogram. H-type TEF can be visualized by this technique, although the study must be
carefully performed. As in this case, an H-type TEF may be missed on the
initial study; therefore, a high level of suspicion is required.
Endoscopy. Endoscopic visualization may reveal an H-type TEF not demonstrated on the
esophagogram. The tracheal aspect of the fistula is located in the upper third
of the posterior tracheal wall and appears as a pit or crescent-shaped hole.
Dyes (e.g., methylene blue) instilled into the trachea may be detected in the
esophagus by endoscopy.
VI. Treatment
An H-type TEF requires surgical ligation. Esophageal atresia is treated with
end-to-end or end-to-side anastomosis of the esophagus.
Tracheoesophageal fistula with esophageal atresia. Immediate postoperative complications include leakage at the anastomotic site
causing mediastinitis or sepsis. Strictures at the anastomotic site may develop
at any time and require repeated esophageal dilatation. Tracheomalacia at the
fistula site is common and results in a brassy cough and impaired clearance of
secretions. Esophageal dysmotility and GER are common.
Recurrence of the TEF occurs in 4% to 10% of cases. The manifestations of a
recurrent TEF are similar to the presentation of children with H-type TEF.
Recurrent TEFs do not close spontaneously; therefore, they also require
surgical ligation. The survival rate is 95% among those patients with good
respiratory function and no major congenital anomalies. The survival rate among
infants with moderate pneumonia or congenital anomalies in addition to the TEF
is approximately 70%. In a series of 82 patients with TEF and esophageal
atresia, Holder and Ashcraft found that 79% of patients were alive and taking
food by mouth 3 to 15 years postoperatively.
H-type tracheoesophageal fistula. Postoperative complications such as tracheomalacia, strictures, and
mediastinitis are uncommon after repair of an H-type fistula. The prognosis in
children with H-type fistula is excellent. Morbidity and mortality are related
to the extent of chronic pulmonary disease in infants diagnosed later in life.
Infants with multiple anomalies and those with severe respiratory disease have
greater morbidity.
VII. References
1. Berseth CL. Disorders of the esophagus. In: Taeusch HW, Ballard RA, eds. Avery's diseases of the newborn. Philadelphia: WB Saunders, 1998:908–913.
2. Holder TM, Ashcraft KW. Developments in the care of patients with esophageal
atresia and tracheoesophageal fistula.
Surg Clin North Am 1981;61:1051–1061.
3. Lierl M. Congenital abnormalities. In: Hilman BC, ed. Pediatric respiratory disease: diagnosis and treatment. Philadelphia: WB Saunders, 1993:457–498.
4. Quan L, Smith DW. The VATER association: vertebral defects, anal atresia,
T-E fistula with esophageal atresia, radial and renal dysplasia, a spectrum of
associated defects.
J Pediatr 1973;7:104–107.
5. Touloukian RJ, Pickett LK, Spackman T, et al. Repair of esophageal atresia
by end-to-side anastomosis and ligation of the tracheoesophageal fistula: a
critical review of 18 cases.
J Pediatr Surg 1974;9:305–310.
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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Copyright Details: Pediatric Complaints and Diagnostic Dilemmas, Copyright © 2008 Williams & Wilkins.
More About Causes of Wheezing
» Next page: Wheezing - Case 1-5: 5-Week-Old Boy (Pediatric Complaints and Diagnostic Dilemmas)
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