Question 1

Which abnormality describes the overdistention of one or more lobes of the lung, most frequently the left upper lobe?&#10;A) Congenital lobar emphysema&#10;B) Bronchopulmonary sequestration&#10;C) Congenital pulmonary airway malformation&#10;D) Bronchogenic cyst

Accepted Answer

Congenital lobar emphysema (CLE) is defined by overdistention of one or more lobes of the lung, most frequently the left upper lobe.

Question 2

What is the most common patient complaint associated with mild pectus excavatum?&#10;A) Cough&#10;B) Dyspnea at rest&#10;C) Cosmetic&#10;D) Cardiac palpitations

Accepted Answer

The most common congenital disorder of the chest wall is pectus excavatum, comprising almost 90% of chest wall deformities. Chest wall and thoracic malformations can have significant impact on a child's self-esteem, respiratory performance, and overall quality of life. They can range from mild deformities that have primarily cosmetic implications with the attendant impact on self-esteem to severe deformities with nontrivial morbidity.

Question 3

Which of the following interventions are used to treat congenital diaphragmatic hernia?
I) High-frequency oscillatory ventilation
II) Gastrointestinal tract decompression
III) Bag-mask ventilation immediately after birth
IV) A mean arterial blood pressure goal greater than 50 mm Hg

A) I and III only
B) II and IV only
C) I, II, and IV only
D) II, III, and IV only

Accepted Answer

After diagnosis, resuscitation must begin immediately in the delivery room. The aim is to prevent hypoxia, or significant hypercapnia and acidosis. A large orogastric tube is placed to decompress the stomach and gastrointestinal tract. Bag-valve mask ventilation is avoided when possible, in order to prevent the intrathoracic stomach and intestine from becoming distended and causing tension physiology, and to avoid barotrauma. An endotracheal tube (ideally cuffed, if feasible) is inserted without delay and the infant is placed on mechanical ventilation, while avoiding high peak inspiratory pressures that may, again, cause barotrauma to the hypoplastic lungs. Close attention must be paid to acid-base balance and PaCO₂. A "permissive hypercapnia" strategy is typically adopted, exchanging mildly increased PaCO₂ and decreased pH for gentler ventilation and decreased potential for barotrauma. Conventional and alternative ventilation strategies are discussed below. Lines are placed for central venous access and arterial blood pressure monitoring. A mean arterial blood pressure goal greater than 50 mmHg may be needed to assist the right heart in overcoming potential pulmonary vascular resistance. In cases of cardiopulmonary collapse, ECMO is used as rescue therapy.

Question 4

Why is pulmonary development often stifled in children who have asphyxiating thoracic dystrophy?&#10;A) Because of gas exchange problems&#10;B) Because the chest cavity is decreased&#10;C) Because the thorax is too compliant&#10;D) Because of a hypoplastic right ventricle

Accepted Answer

Jeune syndrome or asphyxiating thoracic dystrophy is a rare osteochondrodystrophy with an autosomal recessive inheritance pattern. The development of the cartilage and bones is affected, impacting the rib cage, pelvis, and extremities. Symptoms can range from mild to severe. It is a type of thoracic insufficiency syndrome as the chest cavity is decreased in both the anteroposterior and superoinferior orientations. This leads to pulmonary hypoplasia as a result of the decreased cavity size, and after birth the lungs are not able to fully expand. Multiple associated defects, including polydactyly, hypoplastic iliac wings, and fixed clavicles, may be seen.

Question 5

Which of the following is the most significant postoperative complication following primary esophageal anastomisis?&#10;A) Diaphragmatic hernia&#10;B) Esophageal stricture&#10;C) Choanal atresia&#10;D) Pyloric dilation

Accepted Answer

The most significant postoperative complications following primary esophageal anastomosis are anastomotic leak, esophageal stricture, and recurrent TEF formation.

Question 6

Which of the following acronyms is associated with esophageal atresia and tracheoesophageal fistula?&#10;A) VACTERL&#10;B) PROM&#10;C) CHARGE&#10;D) PPHN

Accepted Answer

Laryngeal cleft, or an abnormal connection between the larynx-trachea and hypopharynx-esophagus, occurs in 1 in 10,000-20,000 live births. Most cases are sporadic; however, many laryngeal clefts occur in conjunction with syndromes such VACTERL (a syndrome including vertebral defects, anal atresia, cardiac defects, tracheoesophageal fistula, renal anomalies, and limb abnormalities), Pallister-Hall, and CHARGE syndromes. The embryology is not fully understood, although some authors postulate that TEF results from an anomalous outgrowth of the early respiratory tract. EA and TEF are commonly accompanied by additional anomalies in the distribution of VACTERL association.

Question 7

Which of the following conditions is consistent with the presence of a scaphoid abdomen in a newborn with tachypnea?&#10;A) Neonatal pneumonia&#10;B) Congenital diaphragmatic hernia&#10;C) Tracheoesophageal fistula&#10;D) Esophageal atresia

Accepted Answer

Infants with congenital diaphragmatic hernia (CDH) usually develop respiratory distress shortly after birth. The diagnosis of CDH is confirmed by chest radiography but is suggested in a tachypneic newborn with a scaphoid abdomen.

Question 8

What intervention should the therapist perform when an infant is born with choanal atresia in respiratory distress?&#10;A) Perform nasotracheal intubation.&#10;B) Recommend a tracheotomy procedure.&#10;C) Insert an oral airway.&#10;D) Perform a cricothyroidotomy.

Accepted Answer

A newborn infant is an obligate nasal breather, so the presence of complete nasal obstruction caused by choanal atresia results in immediate respiratory distress and possible death by asphyxia. During the newborn's first breaths, the tongue becomes directly associated with the hard and soft palates, creating a vacuum. Bilateral choanal atresia in a neonate with severe respiratory distress may require emergent management. The neonate in distress will be stabilized by immediately inserting an oral airway.

Question 9

Symptoms of a bronchogenic cyst are often related to which of the follwoing?&#10;A) Compromised immune system&#10;B) Administration of antibiotics against resistant strains&#10;C) Noncompliance with prescribed medications and bronchial hygiene techniques&#10;D) Airway compression

Accepted Answer

The bronchogenic cyst is the most common foregut duplication cyst and can occur throughout the trachea and bronchi. They are embryologically related to other types of foregut cysts such as esophageal duplication cysts, gastric and duodenal duplication cysts, and neurenteric cysts and thus may have mixed characteristics shared with these lesions. Most commonly, bronchogenic cysts are lined by respiratory epithelium. However, the lumen is not connected to the bronchial tree, and the lesions are nonfunctional. They can be located within or outside the thoracic cavity. When intrathoracic, the cyst may be located in the bronchial wall, the pleura, the mediastinum, or the lung parenchyma. Presenting symptoms and timing of diagnosis are often dictated by the anatomic level of the lesion. Most bronchogenic cysts are discovered within the first few years of life. The diagnosis may be apparent radiologically in the newborn with respiratory distress or an older child with recurrent pulmonary infection. Symptoms are often related to airway compression and include dyspnea, wheezing, and recurrent pneumonias.

Question 10

A newborn is brought to the emergency room for difficulty breathing. The mother of the baby reports constant drooling, choking, and coughing episodes. What should the therapist suspect this condition is?

A) Congenital diaphragmatic hernia
B) Tracheoesophageal fistula
C) Esophageal atresia
D) Pyloric dilation

Accepted Answer

An isolated tracheoesophageal fistula may present as late as early adulthood with subtle symptoms of wheezing and recurrent respiratory infections. More typically, choking, drooling, and coughing episodes will be evident shortly after birth, and inability to pass an orogastric tube into the stomach confirms the diagnosis.

Question 11

Which of the following forms of tracheoesophageal fistula and esophageal atresia is most common?&#10;A) Blind-ending upper esophageal pouch of variable length associated with a fistula from the lower trachea or main stem bronchi leading into the distal esophagus&#10;B) An isolated esophageal atresia with a proximal blind-ending pouch and a &#34;long gap&#34; of missing esophagus above a small distal esophageal pouch&#10;C) An esophageal atresia with a proximal and distal tracheoesophageal fistula&#10;D) An isolated tracheoesophageal fistula presenting without atresia and usually occurring in the lower cervical or upper thoracic area

Accepted Answer

The answer of Which of the following forms of tracheoesophageal...

Question 12

If a bronchopulmonary sequestration is suspected, what tool is used to help define the vascular anatomy for operative planning?&#10;A) Chest radiography&#10;B) Ventilation perfusion scan&#10;C) CT angiogram&#10;D) Electrocardiogram

Accepted Answer

The answer of If a bronchopulmonary sequestration is suspected, what...

Question 13

After surgical repair of a congenital diaphragmatic hernia, what is the potential problem generated by the rapid shift of the contralateral lung and mediastinum?&#10;A) Inducing a pneumothorax&#10;B) Causing pulmonary edema&#10;C) Rupturing vascular structures&#10;D) Tearing the sutured diaphragm on the ipsilateral side

Accepted Answer

The answer of After surgical repair of a congenital diaphragmatic...

Question 14

Which of the following clinical manifestations characterize congenital diaphragmatic hernia?
I) The herniated contents cause compression of the developing ipsilateral lung.
II) The ipsilateral side may be compressed from shifting of the mediastinum.
III) Histologic studies demonstrate increased musculature in the media of the arterioles.
IV) Lung tissue is hypoplastic, including the pulmonary vasculature, even on the contralateral side.

A) I and III only
B) II and III only
C) III and IV only
D) I, III, and IV only

Accepted Answer

The answer of Which of the following clinical manifestations characterize...

Question 15

Which of the following conditions is consistent with micrognathia, glossoptosis, and cleft palate in a newborn?&#10;A) Pierre Robin syndrome&#10;B) Choanal atresia&#10;C) Treacher Collins syndrome&#10;D) Esophageal atresia

Accepted Answer

The answer of Which of the following conditions is consistent...

Question 16

Which of the following are considered abdominal wall defects?
I) Gastroschisis
II) Congenital lobar emphysema
III) Omphalocele
IV) Pectus excavatum

A) I and III only
B) II and III only
C) III and IV only
D) I, III, and IV only

Accepted Answer

The answer of Which of the following are considered abdominal...

Question 17

About half of infants diagnosed with choanal atresia also have ______________________.&#10;A) Abdominal compartment syndrome&#10;B) VACTERL syndrome&#10;C) CHARGE syndrome&#10;D) Beckwith-Wiedemann syndrome

Accepted Answer

The answer of About half of infants diagnosed with choanal...

Question 18

A newborn presents with respiratory distress and has a chest radiograph that reveals a circular or ovoid mass with smooth edges. Which of the following lung bud anomalies is consistent with these clinical features?

A) Bronchogenic cysts
B) Congenital cystic adenomatoid malformations
C) Pulmonary sequestration
D) Congenital lobar emphysema

Accepted Answer

The answer of A newborn presents with respiratory distress and...

Question 19

What are some of the complications associated with performing esophageal anastomoses to repair esophageal atresia that the therapist needs to be aware of?
I) Apnea
II) Bradycardia
III) Hyperventilation
IV) Recurrent pneumonia

A) I and II only
B) III and IV only
C) I, II, and III only
D) I, II, and IV only

Accepted Answer

The answer of What are some of the complications associated...

Question 20

Which of the following interventions is used to treat the most severe cases of macroglossia?&#10;A) Prone feeding positioning&#10;B) Surgical intervention&#10;C) Polysomnography to determine any effect on sleep is all that is required&#10;D) Supine positioning

Accepted Answer

The answer of Which of the following interventions is used...

Deck 23: Surgical Disorders in Childhood That Affect Respiratory Care