Four

Question 1

List 7 examples of congenital defects involving the larynx.

Answer 1

Absent Epiglottis

Bifid Epiglottis

Laryngeal Atresia

• Laryngeal Web

Laryngotracheoesophageal cleft

• Laryngomalacia

Subglottic stenosis

Question 2

What kind of diagnostic tests are useful for laryngeal congenital defects and what kinds of things can be seen?

Answer 2

Physical Exam –
stridor, ?absence of larynx in neck

Direct –
may see lesion at intubation

Radiography –
non specific

Contrast Rad –
may see aspiration

Endoscopy –
abnormality seen well
– rigid endoscope preferred in LTE cleft

CT/MRI –
not generally helpful unless paralaryngeal mass

Question 3

What is laryngeal atresia? What are the symptoms? Diagnostics? Treatment?

Answer 3

Laryngeal atresia-larynx doesn’t form

symp: At birth, extreme respiratory distress with sternal and costal retractions w/o detectable airflow

Diag: usually post-mortem but possible during laryngeal intubation

Treatment-Emergency tracheostomy

Question 4

What is laryngeal webs? What are the symptoms? Diagnostics? Treatment?

Answer 4

Laryngeal webs-partial failure of recanalization of the larynx. Thin incomplete membrane with posterior glottis open.

symp: Depends on severity: neonates-stridor, weak cry, resp. distress
Less severity (larger opening): Resp. distress w/ exercise or hoarseness. 

Diag: laryngoscopy

Treatment-Depends on severity

Question 5

What is laryngomalacia? What are the symptoms? Diagnostics? Treatment?

Answer 5

Laryngomalacia-Supraglottic structures prolapse into the airway during inspiration (they’re floppy, less elastic-omega shaped epiglottis or large arytenoids

symp: Most common: Healthy child with a harsh inspiratory noise that differs with activity. High pitched fluttering.
More severe: Failure to thrive due to cyanosis and feeding difficulties.

Diag: Physical and exam

Treatment-Grow out of it in second year of life
If severe, surgery.

Question 6

List 5 tracheal abnormalities.

Answer 6

• Tracheal Stenosis
• Tracheomalacia

Tracheal Atresia

• Tracheoesophageal Fistula
• Tracheal Bronchus

Question 7

What diagnostic tests can be used for tracheal abnormalities? What can they find?

Answer 7

Physical Exam –
no cervical trachea, absent breath sounds, wheezing

Radiology – airway films

Contrast Radiograms –

work well but not usually necessary

Endoscopy –

lesion seen well + larynx evaluated at same time +

Dynamic

CT/MRI –
fixed lesions and distal extent of severe stenosis well
defined

Question 8

What is tracheal atresia?

Answer 8

Trachea doesn’t form. Always leads to death.

Question 9

What is tracheal stenosis? What are the symptoms? Diagnostics? Treatment?

Answer 9

Tracheal Stenosis: The C rings of the trachea wrap all the way around it so there is no posterior membrane. Can involve a short part or can involve a much longer section.

symp: First years of life-recurrent wheezing w/o responding to bronchodilators. Narrower stenosis=younger age. Nonspecific symptoms

Diag: Plain chest radiograph–>bronchoscopy and CT scan

Treatment-Short segment=excision
Longer segment=Slide tracheoplasty

Question 10

What is tracheal bronchus? What are the symptoms? Treatment?

Answer 10

Tracheal Bronchus:Abnormal bronchus arising directly from the trachea usually in right upper lobe. May supply all or part of lobe or be an extra pathway to lobe.

symp: Usually an incidental finding. Generally asymptomatic but might have recurrent infection or atelectasis

Treatment-Lobectomy but only in the case of severe recurrent infections.

Question 11

What is tracheomalacia? What are the symptoms? Diagnostics? Treatment? What causes it/what are its associations?

Answer 11

Tracheomalacia: The C rings of the trachea are misshapen or don’t wrap all around trachea leading to increased flexibility of the posterior membrane. This leads to the trachea collapsing during respiration.

symp: During quiet breathing: Harsh central expiratory wheeze.
During forced expiration or cough: ant. and post. walls may touch leading to a characteristic harsh, barking sound
In cervical trachea: may lead to dynamic inspiratory collapse with stridor.

Associations/causes: Secondary: tracheoesophogeal fistulae, esophogeal atresia, CV anomalies, or external compression from other sources.
Primary: Premature infants, mechanical ventilation leads to stretching of the C rings.

Diag: Endoscopy as patient breathes spontaneously. Not with PA x-ray.

Treatment-Mild forms-Slow improvement with growth
Severe forms Tracheostomy with positive pressure support.

Question 12

What is tracheoesophageal fistula? What are the symptoms? Diagnostics? Treatment?

Answer 12

Tracheoesophogeal fistula: Abnormal anatomical connections b/w esophagus and trachea, usually w/ esophogeal atresia.

symp: Early in life: Feeding difficulties, drooling, and resp. distress. Possible abdominal distention. Proximal atresia—>aspirations. Should be suspected w/ recurrent aspirations when all other causes have been eliminated.

Diag: Proximal atresia: radiopaque feeding tube w/ chest x-ray. Contrast radiographic studies w/ care not to induce aspiration.

Question 13

List 3 congenital defects that can occur in the bronchi?

Answer 13

Bronchial Stenosis

• Bronchogenic Cyst

Bronchial Atresia

Question 14

How are bronchial congenital defects diagnosed? What exams are used and what findings are there?

Answer 14

Physical Exam –
focal abnormality in chest sounds

Radiology –
visible but often non specific

Contrast Radiology –
best definition of airways

Endoscopy –
proximal abnormalities are seen, aid with bronchogram,
culture

CT/MRI –
helps to delineate extent of parenchymal involvement, cysts

Question 15

What are bronchogenic cysts? What are the symptoms? Diagnostics? Treatment?

Answer 15

Bronchogenic cysts: thin-walled structures caused by
abnormal budding of respiratory tissue from the developing respiratory tract (Fig. 37.7). They can occur anywhere along the embryonic airways, including in paratracheal, subcarinal, and intrapulmonary locations. Cysts are often lined with respiratory mucosa (Chap. 2), including functional mucus-secreting cells.

Symptoms: Most bronchogenic cysts present in the second decade of life as an incidental finding on a chest x-ray done for other reasons. However, symptoms of cough, dyspnea, persistent wheeze, or recurrent infection can lead to their detection.

Diagnostics: On a chest radiograph, cysts appear as rounded masses that may cause airway compression observable by bronchoscopy. CT imaging demonstrates
most cysts as having well-demarcated borders with variable internal densities.

Treatment: Surgical therapy is curative, particularly
when well-circumscribed cystic lesions are involved that can be easily dissected from surrounding tissues.

Question 16

List 6 lung parenchyma congenital defects.

Answer 16

Congenital Lobar Emphysema

• Congenital Pulmonary Airway Malformation

Lymphangiectasia

• Sequestration

Hypoplasia

• Aplasia/Agenesis

Question 17

What diagnostic exams are useful for lung congenital defects? What is found in them?

Answer 17

Physical Exam-
focal change in breath sounds

Radiology –often visualized but non-specific

Contrast Radiography –not useful

Endoscopy - show absence of airway, culture

CT/MRI – defines the character (cystic,solid,etc) and the extent of the lesion well

Question 18

What are pulmonary agenesis/aplasia? What are the symptoms? Diagnostics? Prognosis?

Answer 18

Pulmonary agenesis or pulmonary aplasia: the absence
of functional lung parenchyma (Fig. 37.8). Agenesis denotes absent airways, vasculature, and lung tissue on the affected side, while aplasia refers to the presence of a rudimentary mainstem bronchus lacking vasculature and lung tissue on the affected side.

Symptoms: Most cases present in neonates with respiratory distress, cyanosis, stridor, diffi culty feeding, and an abnormal chest exam. However, some affected infants may be asymptomatic.

Diagnostics: Typically the AP chest x-ray reveals a dense homogeneous infiltrate on the affected side, a marked mediastinal shift toward it, and blurring or loss of the heart border. Pulmonary aplasia may be diffi cult to differentiate from massive atelectasis on such fi lms alone, while CT imaging can more accurately confi rm the presence or absence of pulmonary tissue on that side. Bronchoscopy permits evaluations for endobronchial lesions, compression of airways, and the presence of a bronchial stump, but may not be necessary if the results of the CT scan are conclusive. A barium swallow may be important to evaluate for the presence of a foregut communication that would require early surgical intervention.

Prognosis: The prognosis for unilateral pulmonary agenesis varies, having a reported mortality of 30%-50%, with most deaths occurring within the fi rst year of life. Survival longer than 5 years is generally consistent with a normal life span. There may be a worse prognosis with right-sided agenesis, likely due to the increased incidence of cardiovascular lesions seen with
anomalies on this side. Longitudinal pulmonary function testing reveals that with age, the patient’s FVC will mature to 50%-70% of predicted values. Coincident development of pulmonary hypertension is limited to those patients whose associated cardiovascular anomalies increase pulmonary blood fl ow as by left-to-right shunts. Most patients are asymptomatic at rest, but not surprisingly they demonstrate obvious pulmonary impairment during exercise testing (Chap. 12).

Question 19

What is CCAM or CPAM? How is it classified? What are the symptoms? Diagnostics? Treatment? Prognosis?

Answer 19

Congenital cystic adenomatoid malformation (CCAM) or Congenital Pulmonary Airway Malformation (CPAM):
a mass of abnormally developed respiratory tissue composed mainly of terminal bronchial elements that lack distal alveolar structures. Such CCAMs are classifi ed by one of two systems: (1) as macrocystic versus microcystic; and (2) as type 1 (several large cysts), type 2 (multiple small cysts), and type 3 (a largely homogeneous, nearly solid mass). Histologically, these CCAM types correlate with the stage of development when the abnormality arose: larger cysts begin later in gestation.

Symptoms/Diagnostics: Because CCAM is often associated with polyhydramnios, it may be noted by fetal ultrasound, allowing subsequent prenatal planning to optimize lung growth. Type 1 and macrocystic CCAMs present most commonly within the fi rst several months in term infants exhibiting respiratory distress, cyanosis, and feeding diffi culties. Postnatal expansion of a CCAM with air or fl uid can create a surgical emergency due to compression of lung and cardiac tissue. Expansion by partial aeration of a thin-walled macrocystic lesion makes it diffi cult to distinguish on plain x-ray from congenital lobar emphysema, diaphragmatic hernia, or lung abscess. However, CT imaging usually reveals a CCAM’s characteristic cystic appearance.

Treatment: When discovered prenatally and of extensive size, CCAMs are occasionally treated by intrauterine aspiration to decrease cyst size or by fetal surgery to remove the mass (Fig. 37.9).Such interventions provide space for normal lung growth to resume. In the absence of hydrops fetalis, monitoring CCAM over time is preferred since some resolve spontaneously. Postnatally,
surgical resection of CCAM is often done because
lesions may become recurrently infected, and their aeration and expansion may further compromise surrounding lung tissue. Lobectomy has become the surgical therapy of choice to avoid post-resection air leaks when smaller tissue volumes are removed, but extensive CCAMs may require pneumonectomy.

Prognosis: Type 1 or macrocystic CCAMs generally have good surgical outcomes. Infants with microcystic or types 2 and 3 CCAM often have associated fetal hydrops, pulmonary hypoplasia, or other anomalies that signifi cantly worsen prognosis.

Question 20

What is congenital lobar emphysema? Symptoms? Diagnostics? Treatment?

Answer 20

Congenital lobar emphysema is an idiopathic hyperinfl
ation of one or more lobes of the lung, excluding lesions
caused by extrinsic compression of the bronchus.

Symptoms: Most cases present in the fi rst 6 months of life with symptoms of respiratory distress, tachypnea, or cyanotic spells.

Diagnostics: On physical exam there are decreased breath sounds on the affected side, as found in other forms of emphysema (Chap. 14). Plain chest x-rays
reveal a characteristic hyperinfl ated lobe with decreased vascular markings. The hyperinfl ated lobe usually compresses surrounding lung tissue and often causes mediastinal shift. Although any lobe can be involved, the right middle and left upper lobes accounted for 74% of cases in one reported pediatric series. Chest CT imaging may be useful in differentiating diffi cult cases.

Treatment: Asymptomatic patients may be followed without intervention, while symptomatic patients undergo surgical resection that is essentially curative. In at least one study, however, surgery offered no signifi cant benefi t to long-term pulmonary function over medical management alone.

Question 21

What is pulmonary sequestration? Types? Symptoms? Diagnostics? Treatment?

Answer 21

Pulmonary sequestration describes lung tissue that has
lost its communication with the central airways and receives its principal vascular supply from the systemic rather than pulmonary circulation (Fig. 37.10). A sequestered lung segment that is invested by its own pleura is termed extralobar, whereas sequestered lung tissue within the visceral pleura of otherwise normal lung is termed intralobar. A sequestered portion of lung that communicates directly with the gut is termed a
bronchopulmonary foregut malformation.

Symptoms: Because of their abnormal bronchial communication, many lung sequestrations are prone to recurrent infection with poor drainage that often
leads to cystic degeneration and possible hemorrhage. The most common presentation is a patient evaluated for recurrent chest infections, although some sequestrations are discovered as densities on routine chest fi lms.

Treatment: Extralobar sequestrations are easily removed in their own pleural investment. Intralobar sequestrations are generally treated by lobectomy or segmental resection. In either situation, careful attention must
be paid to termination of the vascular supply as it arises from an abnormal location.

Diagnostics: Bronchopulmonary foregut malformations
are sometimes identifi ed by a barium esophagram that shows communication between the gut and bronchi.
Other sequestrations might be seen on routine chest films.