Chapter 23 - Vicky - Fall 16

Which of the following acronyms is associated with esophageal atresia and tracheoesophageal fistula? a. VACTERL b. PROM c. CHARGE d. PPHN

ANS: A For unknown reasons, a constellation of tracheal and esophageal abnormalities, which include esophageal atresia and tracheoesophageal fistula, may be associated with other midline vertebral, anal, cardiac, and renal or peripheral limb anomalies, which are referred to as the VACTERL (vertebral, anal, cardiac, tracheal, esophageal, renal, limb) syndrome. REF: p. 456

An infant presents with abdominal distension, intolerance to feeding, rectal bleeding, and abdominal wall erythema. Laboratory findings include thrombocytopenia, neutropenia, and metabolic acidosis. An abdominal radiograph reveals distended loops and pneumatosis intestinalis. Which of the following conditions is consistent with these findings? a. Necrotizing enterocolitis b. Gastroschisis c. Omphalocele d. Umbilical coelom

ANS: A Infants with NEC will present with abdominal distension, intolerance to feeds, rectal bleeding, and abdominal wall erythema. Laboratory values include thrombocytopenia, neutropenia, and metabolic acidosis. Severe acidosis may require intubation and mechanical ventilation. The diagnosis is confirmed radiographically. Distended loops will be seen and pneumatosis intestinalis is pathognomonic. Air may be seen in the portal system as well. Pneumoperitoneum confirms a perforated viscus necessitating operation. REF: p. 466

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

ANS: A The diagnosis of a bronchogenic cyst may be apparent radiographically in the newborn with respiratory distress where the radiograph reveals a circular or ovoid mass with smooth edges. Similarly, the radiograph can suggest the diagnosis in an older child who presents with stridor, wheezing, or recurrent pneumonia. REF: p. 463

Which of the following forms of tracheoesophageal fistula and esophageal atresia is most common? 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 b. An isolated esophageal atresia with a proximal blind-ending pouch and a "long gap" of missing esophagus above a small distal esophageal pouch c. An esophageal atresia with a proximal and distal tracheoesophageal fistula d. An isolated tracheoesophageal fistula presenting without atresia and usually occurring in the lower cervical or upper thoracic area

ANS: A The most common combination of lesions is esophageal atresia associated with a distal tracheoesophageal fistula. More than 85% of patients will present with this form of the anomaly, which results in a blind-ending upper esophageal pouch of variable length associated with a fistula from the lower trachea or main stem bronchi that leads into the distal esophagus. REF: p. 456

Which of the following conditions is consistent with micrognathia, glossoptosis, and cleft palate in a newborn? a. Pierre Robin syndrome b. Choanal atresia c. Treacher Collins syndrome d. Esophageal atresia

ANS: A The primary features of the Pierre Robin syndrome include micrognathia, glossoptosis (or posterior displacement of the tongue), and cleft palate. These features result in pharyngeal obstruction and respiratory distress. REF: p. 456

Why is pulmonary development often stifled in children who have asphyxiating thoracic dystrophy? a. Because of gas exchange problems b. Because the chest cavity is decreased c. Because the thorax is too compliant d. Because of a hypoplastic right ventricle

ANS: B Asphyxiating thoracic dystrophy, also known as Jeune's syndrome, is a rare genetic disorder with an autosomal recessive inheritance pattern. This disorder is an osteochondrodystrophy that may have mild to severe expression. The chest cavity is decreased in both the anteroposterior and superior and inferior orientations. Pulmonary development is often blunted because of decreased cavity size, and postpartum the lungs are not able to fully expand. Multiple associated defects including polydactyly, hypoplastic iliac wings, and fixed clavicles may be seen. REF: p. 460

Which of the following conditions is consistent with the presence of a scaphoid abdomen in a newborn with tachypnea? a. Neonatal pneumonia b. Congenital diaphragmatic hernia c. Tracheoesophageal fistula d. Esophageal atresia

ANS: B 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. REF: p. 458

What intervention should the therapist perform when an infant is born with choanal atresia? a. Perform nasotracheal intubation b. Recommend a tracheotomy procedure c. Insert an oropharyngeal airway d. Perform a cricothyroidotomy

ANS: C 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. An oral airway should be inserted and maintained to relieve the airway obstruction. REF: p. 455

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. Thoracostomy tube insertion if necessary a. I and III only b. II and IV only c. I, II, and IV only d. II, III, and IV only

ANS: C After the diagnosis of congenital diaphragmatic hernia is made, the following interventions are implemented: 1. A large orogastric tube is placed to decompress the gastrointestinal tract. 2. Bag-mask ventilation is avoided to keep the stomach in the chest from becoming distended and causing tension pneumothorax pathophysiology. 3. An endotracheal tube is inserted, and the infant is mechanically ventilated, avoiding high airway pressures. 4. Barotrauma may be avoided by using high-frequency oscillatory ventilation. 5. Maintaining alkalosis reduces the amount of pulmonary vasospasm. Traditionally, PaCO2 values were kept between 25 and 30 mm Hg, but more recently a protocol of "permissive hypercapnia" with increased PaCO2 and decreased pH values seems to be safe. 6. If a pneumothorax is seen, chest tube placement is indicated. The pneumothorax is seen on the contralateral side and results from excessive ventilation pressures. REF: p. 459

What should a respiratory therapist do to confirm a diagnosis of choanal atresia? a. Recommend lateral mandibular radiographs b. Inspect the nasal cavities with a rhinoscope c. Attempt to insert an 8 French suction catheter through each nasal cavity d. Gently pinch the infant's nose closed until oral breathing occurs

ANS: C An 8 French catheter is the best diagnostic tool for atresia in the newborn intensive care unit. If the catheter fails to pass through the nose into the oropharynx, choanal atresia should be suspected. The neonate is stabilized by immediately inserting an oral airway. REF: p. 455

A newborn is brought to the emergency room for difficulty breathing. The mother of the baby reports constant drooling, coughing, and episodes of cyanosis. What should the therapist suspect this condition is? a. Congenital diaphragmatic hernia b. Tracheoesophageal fistula c. Esophageal atresia d. Pyloric dilation

ANS: C Drooling, along with frothing and bubbling at the nose and mouth, are the first symptoms in the majority of newborns with esophageal atresia. The first feedings result in choking, coughing, and episodes of cyanosis. The respiratory distress may be severe and progressive, which should prompt an immediate work-up for esophageal atresia. If esophageal atresia is suspected, a stiff nasogastric tube is introduced until resistance is met. The tube is connected to constant suction and irrigated with 1 to 2 mL of saline at frequent intervals. REF: p. 457

What is a possible consequence of inadvertent rupture of the systemic arterial supply in cases of pulmonary sequestration when this supply arises directly from the aorta? a. Hypoxemia b. Pulmonary hypoperfusion c. Significant blood loss or exsanguination d. Decreased anatomic shunt

ANS: C If a sequestration is suspected, an arteriogram can be diagnostic and helpful to define the arterial anatomy, which is crucial to successful resection. The systemic arterial supply may arise directly from the aorta, at times even through the diaphragm from the intra-abdominal aorta. Injury or inadvertent division of this vessel without proper control may result in a hemorrhagic catastrophe. REF: p. 464

What is the most common patient complaint associated with pectus carinatum? a. Cough b. Dyspnea at rest c. Cosmetic d. Cardiac palpitations

ANS: C This defect, which accounts for 5% of chest wall deformities, is the opposite of pectus excavatum. Pectus carinatum is usually seen later in life around a growth spurt. The protrusion is most commonly located on the lower sternum. Because the sternum is protruded, the underlying structures are not compressed. REF: p. 460

After surgical repair of a congenital diaphragmatic hernia, what is the potential problem generated by the rapid shift of the contralateral lung and mediastinum? a. Inducing a pneumothorax b. Causing pulmonary edema c. Rupturing vascular structures d. Tearing the sutured diaphragm on the ipsilateral side

ANS: C Too rapid a shift of the mediastinum and contralateral lung may cause lung rupture or obstruct the vascular structures. REF: p. 459

Which of the following conditions is the best explanation for the presentation of symptoms beyond infancy in patients with bronchogenic cysts or congenital cystic adenomatoid malformations? a. Compromised immune system b. Administration of antibiotics against resistant strains c. Noncompliance with prescribed medications and bronchial hygiene techniques d. Lack of communication between cysts and the tracheobronchial tree

ANS: D After infancy the most common presentation is with repeated or prolonged episodes of pneumonia despite adequate antibiotic coverage. The infectious course arises because the contents of the cyst do not communicate with the tracheobronchial tree, allowing bacteria and debris to accumulate in the cyst. This material cannot be evacuated and acts as a nidus for infection, which often spreads to adjacent healthy tissue and lymph nodes in the hilum of the involved lung. Thus, a small infected cyst can result in pneumonia with fever and purulent cough. Although antibiotics are helpful, they are not curative, and the underlying cause, that is, the cyst, must be resected to prevent recurrence of pulmonary infections. REF: p. 462

Which of the following patterns are commonly found in the presentation of lung bud anomalies? I. Appear early in the newborn period II. Emerge later in childhood III. Frequent respiratory infections IV. Systemic hypertension a. I and III only b. II and III only c. II and IV only d. I, II, and III only

ANS: D Although lung bud anomalies have exceptionally different histopathologies, patterns in clinical presentation are common to all. The presentation seems to follow two distinct patterns: (1) the condition becomes obvious in the early newborn period and is manifested by respiratory distress; and (2) the condition occurs later in childhood and is characterized by repeated infections. REF: p. 460

What is the role of radiography in the determination of tracheoesophageal fistula and esophageal atresia? a. The esophageal pouch can be observed. b. The opening into the airway can be located. c. The presence of left-to-right shunt can be seen. d. The location of the nasogastric tube may confirm obstruction of the proximal esophagus caused by atresia.

ANS: D Chest and abdominal radiographs are critical to the diagnosis of esophageal atresia and tracheoesophageal fistula. Several important radiographic findings are specific for esophageal atresia. The course and end point of the nasogastric tube confirm obstruction of the proximal esophagus by atresia and determine the relative position of the upper esophageal pouch. The lung fields must be examined to determine the presence of parenchymal changes resulting from aspiration. Changes in mediastinal structures give an early indication of congenital heart disease, and a careful search is made for the aortic arch to determine its left- or right-sided position. REF: p. 457

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

ANS: D In esophageal atresia, the ability to successfully perform esophageal anastomoses has been lifesaving. The most significant complication of primary esophageal anastomosis is stricture or recurrent fistula formation. These complications have important implications for postoperative respiratory care. Even infants with successful anastomoses have persistent respiratory problems. With or without complications, postoperative respiratory symptoms have been noted in up to 50% of patients. Complications range from apnea and bradycardia to aspiration, recurrent pneumonia, and even respiratory arrest. REF: p. 458

Which of the following interventions is used to treat macroglossia? a. Positioning the patient appropriately corrects most cases. b. Most cases are corrected surgically. c. Polysomnography to determine any effect on sleep is all that is required. d. Treatment is based on the severity of airway obstruction and etiology.

ANS: D Treatment for macroglossia should be individualized, depending on the severity of respiratory obstruction and etiology. For isolated macroglossia, prone positioning usually relieves mild cases. More severe cases may require one of the many surgical techniques to reduce tongue size. Lymphangiomas and small hemangiomas of the tongue may require excision, and large congenital hemangiomas frequently respond to systemic corticosteroid or interferon therapy. Chronic hypoxia and carbon dioxide retention are frequent sequelae of macroglossia and require close follow-up. Immediate intubation may be necessary along with tracheotomy to temporize for reduction surgery. REF: p. 456

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

ANS: D With congenital diaphragmatic hernia, the herniated contents cause compression of the developing ipsilateral lung bud. The contralateral lung may be compressed as well from shifting of the mediastinum. The lung tissue is hypoplastic, including the pulmonary vasculature, even on the contralateral side. Histologic studies demonstrate increased musculature in the media of the arterioles. REF: p. 458