Congenital Airway Anomalies — MCQs
Most common cause of intermittent stridor in a 10-day-old child shortly after birth is:
Double aortic arch is associated with which syndrome?
The 'steeple sign' on X-ray neck (as shown in the image) is characteristically seen in which of the following diseases?
All of the following are clinical features suggestive of tracheoesophageal fistula except -
A 2 year child presented with low grade fever and stridor. What is the likely diagnosis?
What is the most common indication for performing a tracheostomy?
A chest CT shows 'doughnut sign' in mediastinum. Which additional finding would best support pulmonary artery sling?
In an infant brought with stridor, diagnosed with laryngomalacia, which of the following is NOT typically observed?
18 weeks pregnant female presents with no high risk of NTD and low risk of trisomy 21 on quad test. What is the most appropriate next step in management?
Identify the condition shown in the CT scan image.
Congenital Airway Anomalies Indian Medical PG Practice Questions and MCQs
Question 1: Most common cause of intermittent stridor in a 10-day-old child shortly after birth is:
- A. Hypertrophy of turbinate
- B. Laryngomalacia (Correct Answer)
- C. Foreign body
- D. Vocal nodule
Explanation: ***Laryngomalacia*** - This is the **most common cause** of congenital stridor, typically becoming noticeable within the first few weeks of life, consistent with a 10-day-old child. - The stridor is characteristically **intermittent**, worsens with crying or feeding, and improves when prone, due to the collapse of supraglottic structures. *Hypertrophy of turbinate* - While nasal issues can cause **stertor** (a snoring sound), hypertrophy of turbinates is not a common cause of stridor in an infant. - **Stridor** originates from the larynx or trachea, not the nasal passages. *Foreign body* - An aspirated foreign body would typically cause **acute-onset stridor** that is often continuous and associated with choking or coughing spells, rather than intermittent symptoms starting shortly after birth. - This is a less likely etiology for intermittent stridor developing within the first two weeks of life. *Vocal nodule* - Vocal nodules are generally associated with **hoarseness** or a change in voice quality, and typically require chronic vocal abuse to develop. - They are exceptionally rare in neonates and would not present as intermittent stridor.
Question 2: Double aortic arch is associated with which syndrome?
- A. DiGeorge syndrome (Correct Answer)
- B. CATCH 22 syndrome
- C. Shprintzen syndrome
- D. None of the options
Explanation: ***DiGeorge syndrome*** - **DiGeorge syndrome** is caused by a **22q11.2 deletion** affecting the development of the third and fourth pharyngeal pouches, leading to **thymic hypoplasia**, **parathyroid hypoplasia**, and **cardiac anomalies**. - Common cardiac defects include **interrupted aortic arch type B**, **truncus arteriosus**, **tetralogy of Fallot**, and **VSD**. - **Double aortic arch** can occur in 22q11.2 deletion syndrome, though it is less common than other cardiac anomalies; however, among the syndromes listed, this represents the most appropriate association. - The question tests recognition that various cardiac arch anomalies, including double aortic arch, may be seen in this genetic syndrome. *CATCH 22 syndrome* - This is an **acronym** for DiGeorge syndrome: **C**ardiac defects, **A**bnormal facies, **T**hymic hypoplasia, **C**left palate, **H**ypocalcemia, and **22q11 deletion**. - It is **essentially the same condition** as DiGeorge syndrome, just using different nomenclature. - While technically correct, "DiGeorge syndrome" is the more standard medical terminology currently used. *Shprintzen syndrome* - **Shprintzen syndrome** (also called **velocardiofacial syndrome or VCFS**) is caused by the **same 22q11.2 deletion** as DiGeorge syndrome. - It represents a **phenotypic variant within the 22q11.2 deletion syndrome spectrum**, with more emphasis on palatal and facial features. - Since it shares the same genetic basis, it can also present with similar cardiac anomalies, but "DiGeorge syndrome" is the more commonly recognized term for this genetic disorder. *None of the options* - This is incorrect because the three syndromes listed above (DiGeorge, CATCH 22, and Shprintzen) all refer to **22q11.2 deletion syndrome** or its variants, which can be associated with various cardiac anomalies including double aortic arch. - Among the listed options, **DiGeorge syndrome** is the most appropriate and widely recognized answer.
Question 3: The 'steeple sign' on X-ray neck (as shown in the image) is characteristically seen in which of the following diseases?
- A. Epiglottitis
- B. Croup (Correct Answer)
- C. Laryngomalacia
- D. Bronchiolitis
Explanation: ***Croup*** - The image suggests a **steeple sign** on the X-ray neck, which corresponds to the subglottic narrowing characteristic of croup. - Croup, primarily caused by **parainfluenza virus**, leads to inflammation and edema below the vocal cords reducing the airway. *Epiglottitis* - Epiglottitis is characterized by a **"thumb sign"** on lateral neck X-ray, indicating a swollen epiglottis. - Patients with epiglottitis typically present with rapidly progressing airway obstruction, high fever, and drooling, unlike the gradual onset and barking cough of croup. *Laryngomalacia* - Laryngomalacia involves the **collapse of supraglottic structures** during inspiration, causing inspiratory stridor. - It would not typically present with the subglottic narrowing seen in the image, but rather with dynamic airway changes visualized during endoscopy. *Bronchiolitis* - Bronchiolitis involves inflammation of the **small airways (bronchioles)** and is usually diagnosed clinically, with chest X-rays showing hyperinflation or peribronchial cuffing if at all. - It primarily affects the lower respiratory tract and would not produce findings like the steeple sign on a neck X-ray.
Question 4: All of the following are clinical features suggestive of tracheoesophageal fistula except -
- A. Fever (Correct Answer)
- B. Choking and Coughing
- C. Regurgitation
- D. Cyanosis
Explanation: ***Fever*** - **Fever is NOT a primary clinical feature** of tracheoesophageal fistula (TEF) itself. - While fever might occur as a **complication** if aspiration pneumonia develops, it is not a direct presenting sign of TEF. - The classic presentation of TEF occurs in **newborns within hours of birth** and involves the "3 Cs" - not fever. *Choking and Coughing* - Part of the classic **"3 Cs" triad** (Choking, Coughing, Cyanosis) of TEF presentation. - Occurs during the **first feeding attempt** when milk enters the trachea through the abnormal fistulous connection. - This is a **cardinal diagnostic feature** that should immediately raise suspicion for TEF. *Regurgitation* - **Immediate regurgitation** of feeds is characteristic, especially in TEF with esophageal atresia (most common type - Type C). - The **blind-ending proximal esophageal pouch** prevents normal passage of saliva and feeds, causing regurgitation. - Often accompanied by **excessive drooling and frothy secretions** from the mouth and nose. *Cyanosis* - The third component of the **"3 Cs" triad** and a key clinical feature. - Results from **aspiration of feeds or saliva** into the trachea and lungs, causing acute respiratory distress. - May also occur from **laryngospasm** as a protective reflex when fluid enters the airway.
Question 5: A 2 year child presented with low grade fever and stridor. What is the likely diagnosis?
- A. Acute Laryngotracheobronchitis (Correct Answer)
- B. Acute Bacterial Tracheitis
- C. Acute Epiglottitis
- D. Foreign Body aspiration
Explanation: ***Acute Laryngotracheobronchitis*** - The combination of **low-grade fever** and **stridor** in a 2-year-old child strongly suggests **croup**, which is medically known as acute laryngotracheobronchitis. - Croup is characterized by **inflammation** of the larynx, trachea, and bronchi, often presenting with a **barking cough** and inspiratory stridor. The X-ray image would show the characteristic **steeple sign**. *Acute Bacterial Tracheitis* - This is a more severe bacterial infection that can present with stridor but typically shows **higher fever**, **toxic appearance**, and rapid clinical deterioration. - Unlike croup, bacterial tracheitis patients appear **more ill** and may have **purulent secretions** requiring more aggressive management. *Acute Epiglottitis* - A serious condition characterized by **rapid onset of high fever**, **dysphagia**, drooling, and a **"tripod" position**, which are not indicated by the given symptoms. - The stridor in epiglottitis is typically quieter and may indicate more severe airway obstruction compared to the characteristic stridor of croup. *Foreign Body aspiration* - While foreign body aspiration can cause stridor, it is typically an **acute event** with a sudden onset of choking, coughing, and respiratory distress. - There is no mention of a choking episode or sudden onset, and a low-grade fever is less typical for an uncomplicated foreign body aspiration.
Question 6: What is the most common indication for performing a tracheostomy?
- A. Severe obstructive sleep apnea
- B. Tracheal stenosis
- C. Vocal cord paralysis
- D. Prolonged mechanical ventilation (Correct Answer)
Explanation: ***Prolonged mechanical ventilation*** - Maintaining an **endotracheal tube** for an extended period carries risks like **tracheal injury**, **vocal cord damage**, and difficulty with oral intake. - A tracheostomy provides a more comfortable and stable airway for **long-term respiratory support**, facilitates weaning from the ventilator, and reduces the risk of **ventilator-associated pneumonia**. *Severe obstructive sleep apnea* - While tracheostomy can effectively treat severe OSA by bypassing the upper airway obstruction, it is generally considered a **last resort** after less invasive treatments have failed. - The most common initial treatments for OSA include **CPAP**, weight loss, and oral appliances. *Tracheal stenosis* - Tracheal stenosis itself is a **structural narrowing** of the trachea that may or may not require tracheostomy, depending on its severity and location. - While a tracheostomy can bypass a severe stenosis, surgical repair of the trachea is often the definitive treatment for **severe tracheal stenosis**. *Vocal cord paralysis* - Unilateral vocal cord paralysis typically causes **hoarseness** and may not always necessitate a tracheostomy. - Bilateral vocal cord paralysis can lead to **airway obstruction**, but intervention usually involves vocal cord lateralization procedures or, in severe cases, a tracheostomy for airway patency.
Question 7: A chest CT shows 'doughnut sign' in mediastinum. Which additional finding would best support pulmonary artery sling?
- A. Right aortic arch (Correct Answer)
- B. Patent ductus arteriosus
- C. Cardiac dextroposition
- D. Rightward course of left pulmonary artery
Explanation: ***Right aortic arch*** - A **right aortic arch** is a common associated anomaly with **pulmonary artery sling**, increasing the likelihood of **tracheal compression** and airway symptoms. - The combination of a right aortic arch with the characteristic "doughnut sign" (trachea encircled by vascular structures) is highly suggestive of pulmonary artery sling. *Patent ductus arteriosus* - A **patent ductus arteriosus (PDA)** is a common congenital heart defect but does not specifically contribute to the characteristic vascular encirclement of the trachea seen in pulmonary artery sling. - While a PDA can cause left-to-right shunting and pulmonary vascular changes, it is not directly involved in the anatomical sling mechanism. *Cardiac dextroposition* - **Cardiac dextroposition** refers to the heart being positioned on the right side of the chest, often due to extrinsic factors or lung hypoplasia, but it does not inherently relate to the specific vascular anomaly of a pulmonary artery sling. - It doesn't explain the "doughnut sign" or the aberrant course of the pulmonary artery around the trachea. *Rightward course of left pulmonary artery* - **Pulmonary artery sling** itself is defined by the **left pulmonary artery** originating abnormally from the right pulmonary artery and passing between the trachea and esophagus, often giving it a "rightward course" relative to its usual position. - This option essentially describes the pathology of a pulmonary artery sling rather than an *additional* finding that would help *support* the diagnosis. The "doughnut sign" already implies this abnormal course, and we are looking for a separate associated anomaly.
Question 8: In an infant brought with stridor, diagnosed with laryngomalacia, which of the following is NOT typically observed?
- A. Stridor will be inspiratory
- B. Hoarseness (Correct Answer)
- C. Prominent arytenoids
- D. Floppy aryepiglottic folds
Explanation: ***Correct: Hoarseness*** - **Laryngomalacia** primarily involves the collapse of supraglottic structures during inspiration, leading to inspiratory stridor - Hoarseness is NOT typically observed because laryngomalacia does **not directly affect the vocal cords** - Hoarseness indicates pathology at the level of the **vocal cords** themselves (such as vocal cord paralysis or inflammation), which is a different entity - The supraglottic collapse in laryngomalacia occurs above the vocal cords, leaving vocal cord function intact *Incorrect: Stridor will be inspiratory* - **Inspiratory stridor** is the hallmark feature of laryngomalacia - The collapse of supraglottic structures during inspiration creates a narrow airway, producing the characteristic high-pitched sound on inhalation - This is the most common presenting symptom in affected infants *Incorrect: Prominent arytenoids* - Laryngoscopy in laryngomalacia often reveals **prominent or redundant arytenoid mucosa** - The collapse of redundant tissue over the arytenoids makes them appear more prominent due to inward movement during inspiration - This contributes to the airway obstruction seen in the condition *Incorrect: Floppy aryepiglottic folds* - **Floppy, shortened aryepiglottic folds** are a hallmark anatomical feature of laryngomalacia - These folds collapse inward during inspiration, obstructing the laryngeal inlet - This collapse is the primary mechanism causing the inspiratory stridor in laryngomalacia
Question 9: 18 weeks pregnant female presents with no high risk of NTD and low risk of trisomy 21 on quad test. What is the most appropriate next step in management?
- A. Repeat non-invasive screening test.
- B. Perform invasive diagnostic testing.
- C. Perform amniotic fluid analysis.
- D. Perform a detailed fetal ultrasound. (Correct Answer)
Explanation: ***Perform a detailed fetal ultrasound.*** - A **detailed fetal ultrasound** (often referred to as an **anatomy scan**) at around 18-22 weeks is a standard component of prenatal care for all pregnant women, regardless of screening test results. - This ultrasound evaluates fetal anatomy for structural anomalies, assesses fetal growth, and confirms gestational age, providing crucial information even with low-risk screening. *Repeat non-invasive screening test.* - Repeating a non-invasive screening test (like another quad screen or NIPT) is generally **not indicated** when initial results show a low risk and there are no other clinical concerns. - Such tests are primarily for screening purposes, and a second low-risk result would offer little additional actionable information, as their positive predictive value is low. *Perform invasive diagnostic testing.* - **Invasive diagnostic testing**, such as **amniocentesis** or **chorionic villus sampling (CVS)**, carries a risk of miscarriage and is reserved for situations with a high risk of chromosomal abnormalities or genetic conditions. - Given the low-risk quad screen results for trisomy 21 and no high risk for NTDs, invasive testing is **not warranted** at this stage. *Perform amniotic fluid analysis.* - **Amniotic fluid analysis** is part of an amniocentesis, an **invasive diagnostic procedure** designed to detect chromosomal abnormalities or genetic disorders. - This procedure is typically reserved for cases where screening tests indicate a high risk or there is a clinical suspicion of a genetic condition; it's **not a routine step** after a low-risk quad screen.
Question 10: Identify the condition shown in the CT scan image.
- A. None of the options
- B. Dandy-Walker malformation (Correct Answer)
- C. Cerebellar vermis hypoplasia
- D. Mega cisterna magna
Explanation: ***Dandy-Walker malformation*** - The image shows an enlarged posterior fossa with **cystic dilation of the fourth ventricle** and **absence/hypoplasia of the cerebellar vermis**, which are classic features of Dandy-Walker malformation. - The elevated tentorium and upward displacement of the transverse sinuses are also characteristic, contributing to the distinct appearance. *Cerebellar vermis hypoplasia* - While cerebellar vermis hypoplasia is a component of Dandy-Walker malformation, it is not the sole, defining feature. - Dandy-Walker also includes cystic dilation of the fourth ventricle and an enlarged posterior fossa, which are evident in the image and go beyond isolated vermis hypoplasia. *Mega cisterna magna* - A **mega cisterna magna** is a benign enlargement of the cisterna magna, which is the space between the cerebellum and the medulla oblongata. - Unlike in Dandy-Walker malformation, a mega cisterna magna usually does not involve displacement of the tentorium or hypoplasia of the cerebellar vermis, and the fourth ventricle is typically normal in shape and size. *None of the options* - The image clearly displays the diagnostic hallmarks of Dandy-Walker malformation, making this option incorrect.
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