Congenital Lung Malformations — MCQs
Majority of bronchogenic lung cysts occur in which?
A 2-week-old girl is found to have a harsh murmur along the left sternal border. The parents report that the baby gets "bluish" when she cries or drinks from her bottle. Echocardiogram reveals a congenital heart defect associated with pulmonary stenosis, ventricular septal defect, dextroposition of the aorta, and right ventricular hypertrophy. What is the appropriate diagnosis?
To visualize vascular sling causing tracheal or external airway compression, which of the following would you best prefer?
Bronchogenic sequestration is seen in which lobe?
What is the arterial supply to the segment involved in intralobar sequestration of the lung?
A child comes with cyanotic spells and chest X-ray was as shown below; What is the most probable diagnosis?
Branchial cleft anomalies are present at birth or shortly after birth. Which of the following is TRUE about branchial anomaly?
What is the most common variety of mesenteric cyst?
Earliest congenital malformation that may be detected on USG is
All of the following statements about laryngomalacia are true, except for:
Congenital Lung Malformations Indian Medical PG Practice Questions and MCQs
Question 1: Majority of bronchogenic lung cysts occur in which?
- A. Mediastinum (Correct Answer)
- B. Near the carina
- C. Peribronchial tissue
- D. Base of the lung
Explanation: ***Mediastinum*** - **Bronchogenic cysts** most commonly occur in the **mediastinum** (approximately 60-70% of cases), particularly in the **subcarinal** or **paratracheal** regions [1]. - These cysts develop from abnormal budding of the primitive foregut during embryonic development [1]. - The mediastinum is the most comprehensive answer, as cysts can occur in various mediastinal locations. *Near the carina* - The **subcarinal region** (near the carina) is indeed one of the most common specific sites for mediastinal bronchogenic cysts [1]. - However, this represents only one specific location within the mediastinum, whereas cysts also commonly occur in paratracheal, hilar, and other mediastinal regions. - "Mediastinum" is the more complete answer as it encompasses all these locations. *Peribronchial tissue* - While bronchogenic cysts are derived from bronchial tree anomalies, the term **peribronchial tissue** is too vague and not the standard anatomical classification used. - The majority are found in well-defined mediastinal compartments, not simply described as "peribronchial." *Base of the lung* - **Intrapulmonary bronchogenic cysts** account for only 30-40% of cases, making them less common than mediastinal cysts. - Intrapulmonary cysts may be mistaken for other lung pathologies like **pulmonary sequestration** or congenital cystic adenomatoid malformation.
Question 2: A 2-week-old girl is found to have a harsh murmur along the left sternal border. The parents report that the baby gets "bluish" when she cries or drinks from her bottle. Echocardiogram reveals a congenital heart defect associated with pulmonary stenosis, ventricular septal defect, dextroposition of the aorta, and right ventricular hypertrophy. What is the appropriate diagnosis?
- A. Atrial septal defect
- B. Tetralogy of Fallot (Correct Answer)
- C. Coarctation of aorta, postductal
- D. Coarctation of aorta, preductal
Explanation: ***Tetralogy of Fallot*** - The combination of **pulmonary stenosis**, **ventricular septal defect**, **dextroposition of the aorta** (overriding aorta), and **right ventricular hypertrophy** is the classic definition of Tetralogy of Fallot. - The "bluish" episodes (cyanosis) when crying or feeding are characteristic of **tet spells**, indicating right-to-left shunting and reduced pulmonary blood flow, exacerbated by activity. *Atrial septal defect* - An ASD primarily involves a **left-to-right shunt** and typically presents with a **fixed, split S2** and a **pulmonic flow murmur**, usually without cyanosis in infancy. - It does not involve the characteristic four defects seen in this patient, particularly the significant pulmonary stenosis and cyanosis. *Coarctation of aorta, postductal* - **Postductal coarctation** typically presents in older children or adults with **hypertension in the upper extremities** and **diminished or absent femoral pulses**, often without cyanosis. - This condition is a narrowing of the aorta **distal to the ductus arteriosus** and does not involve the four specific intracardiac defects described. *Coarctation of aorta, preductal* - **Preductal coarctation** can present in neonates with **heart failure** and **differential cyanosis** (upper body pink, lower body blue), or signs of shock if the ductus arteriosus closes. - This condition involves a narrowing of the aorta **proximal to the ductus arteriosus** and is not characterized by the four specific tetralogy defects.
Question 3: To visualize vascular sling causing tracheal or external airway compression, which of the following would you best prefer?
- A. MRI
- B. CT (Correct Answer)
- C. Catheter angiography of aorta and pulmonary artery
- D. PET-CT
Explanation: ***Correct: CT (CT Angiography)*** - **CT angiography is the gold standard** for diagnosing vascular rings and slings causing airway compression - Provides **excellent spatial resolution** with multiplanar and 3D reconstruction capabilities to clearly demonstrate the anatomical relationship between anomalous vessels and the trachea/bronchi - **Fast acquisition time** minimizes motion artifacts, particularly important in pediatric patients who are the typical population affected by vascular slings - Superior for **surgical planning** due to detailed visualization of vascular anatomy and the exact site and degree of airway compression - More readily available and cost-effective compared to MRI for this specific indication *Incorrect: MRI* - While MRI provides excellent soft tissue contrast and avoids ionizing radiation, it has **longer acquisition times** leading to increased risk of motion artifacts, especially in children - Lower spatial resolution compared to CT for vascular structures - May be used as a **complementary modality** when radiation avoidance is critical or for follow-up imaging, but not the first-line preferred modality *Incorrect: Catheter angiography of aorta and pulmonary artery* - **Invasive procedure** with associated risks including vascular injury, bleeding, and contrast reactions - Provides excellent vascular detail but **no information about airway compression** or surrounding soft tissue structures - Reserved for cases requiring **intervention** (embolization, stent placement) or when non-invasive imaging is inconclusive - Has been largely replaced by non-invasive CTA for diagnostic purposes *Incorrect: PET-CT* - Primarily assesses **metabolic activity**, used for oncology staging, infection, and inflammatory conditions - **Not indicated** for structural vascular anomalies or their anatomical relationships to airways - Does not provide the necessary vascular or airway detail for evaluating vascular slings
Question 4: Bronchogenic sequestration is seen in which lobe?
- A. Left lower lobe (Correct Answer)
- B. Right upper lobe
- C. Left upper lobe
- D. Right middle lobe
Explanation: ***Left lower lobe*** - **Bronchopulmonary sequestration**, particularly the **intralobar type**, most commonly affects the **posterior basal segment of the left lower lobe**. [1] - This congenital malformation involves a segment of lung tissue that lacks normal communication with the tracheobronchial tree and receives its blood supply from a systemic artery. [1] *Right upper lobe* - While sequestration can occur anywhere, the **right upper lobe** is a much less common location for bronchopulmonary sequestration compared to the lower lobes. - Sequestration in the upper lobes is rare and usually associated with specific anatomical variations. *Right middle lobe* - The **right middle lobe** is also an infrequent site for bronchopulmonary sequestration. - The typical presentation involves the basal segments of the lower lobes due to embryonic developmental patterns. *Left upper lobe* - **Left upper lobe** involvement in bronchopulmonary sequestration is uncommon. - The majority of cases are found in the lower lobes, especially the left lower lobe.
Question 5: What is the arterial supply to the segment involved in intralobar sequestration of the lung?
- A. Thoracic or abdominal aorta (Correct Answer)
- B. Internal mammary artery
- C. Splenic artery
- D. None of the options
Explanation: ***Thoracic or abdominal aorta*** - **Intralobar lung sequestration** is characterized by an abnormal arterial supply arising directly from the **systemic circulation**, most commonly from the **thoracic aorta** (60-70% of cases) or **abdominal aorta** (20-30% of cases). - This anomalous arterial supply provides high-pressure systemic blood flow to the sequestered lung tissue, distinguishing it from normal pulmonary circulation supplied by pulmonary arteries. - The sequestered segment is located within the normal lung parenchyma and shares the visceral pleura with adjacent normal lung tissue. *Internal mammary artery* - The internal mammary artery primarily supplies the **anterior chest wall** and mediastinal structures. - While rarely reported in atypical cases, it is not a typical source of anomalous arterial supply for intralobar lung sequestrations. *Splenic artery* - The splenic artery supplies the **spleen** and portions of the stomach and pancreas. - It has no anatomical connection or developmental role in supplying lung tissue, whether normal or anomalous. *None of the options* - This option is incorrect because the thoracic or abdominal aorta represents the well-established arterial source for intralobar lung sequestration. - Identifying the specific anomalous vessel is crucial for preoperative imaging, surgical planning, and management of the condition.
Question 6: A child comes with cyanotic spells and chest X-ray was as shown below; What is the most probable diagnosis?
- A. Tricuspid atresia
- B. Tetralogy of Fallot (Correct Answer)
- C. TAPVC
- D. Pulmonary atresia with intact ventricular septum
Explanation: ***Tetralogy of Fallot*** - The **boot-shaped heart** (Coeur en sabot) seen on the chest X-ray is virtually pathognomonic for **Tetralogy of Fallot** due to right ventricular hypertrophy and a concave pulmonary artery segment. - **Cyanotic spells** (hypercyanotic or "tet" spells) are characteristic clinical features resulting from increased right-to-left shunting across the ventricular septal defect. *Tricuspid atresia* - While tricuspid atresia causes **cyanosis**, the classic radiographic finding is usually a **small right ventricle** and a large left ventricle, affecting heart size and shape differently, often with normal or decreased pulmonary vascularity. - A **boot-shaped heart** is not a typical finding; it might show a normal or slightly enlarged heart with distinct chamber enlargement patterns. *TAPVC* - Total anomalous pulmonary venous connection (TAPVC) typically presents with **cyanosis** and can have increased pulmonary vascular markings on CXR, but the classic X-ray finding is a **"snowman" or "figure-of-8" heart** in the supracardiac type due to dilated SVC and anomalous vertical vein. - A **boot-shaped heart** is not associated with TAPVC. *Pulmonary atresia with intact ventricular septum* - This condition presents with **severe cyanosis** at birth. The chest X-ray typically shows a **markedly enlarged right atrium** due to severe tricuspid regurgitation and a small, hypoplastic right ventricle, along with decreased pulmonary vascular markings. - A **boot-shaped heart** is not a characteristic finding; the cardiac silhouette is often described as globular or enlarged differently.
Question 7: Branchial cleft anomalies are present at birth or shortly after birth. Which of the following is TRUE about branchial anomaly?
- A. Fistulas are more common than cysts
- B. For sinuses surgery is not always indicated
- C. Most commonly due to 2nd branchial remnants (Correct Answer)
- D. Cysts present with dysphagia and hoarseness of voice
Explanation: ***Most commonly due to 2nd branchial remnants*** - **Second branchial cleft anomalies** are the most prevalent type, accounting for approximately **90-95%** of all branchial anomalies. - They typically present as cysts, sinuses, or fistulas along the anterior border of the **sternocleidomastoid muscle**. *Fistulas are more common than cysts* - **Cysts** are actually the most common presentation of branchial anomalies, often appearing as solitary masses. - While fistulas and sinuses can occur, they are generally **less frequent** than isolated cysts. *For sinuses surgery is not always indicated* - **Surgical excision** is generally indicated for all branchial anomalies, including sinuses, due to the risk of **infection**, recurrence, and potential for an underlying fistula. - Conservative management is typically reserved for infected cysts (drainage and antibiotics) before definitive surgical removal. *Cysts present with dysphagia and hoarseness of voice* - **Dysphagia** (difficulty swallowing) and **hoarseness of voice** are not typical symptoms of branchial cleft cysts, as these cysts are usually located laterally in the neck. - These symptoms are more commonly associated with congenital anomalies affecting the **pharynx**, **larynx**, or **thyroid gland** (e.g., thyroglossal duct cysts when large or infected).
Question 8: What is the most common variety of mesenteric cyst?
- A. Chylolymphatic (Correct Answer)
- B. Dermoid
- C. Enterogenous
- D. Urogenital remnant
Explanation: **Chylolymphatic** - These cysts originate from **lymphatic tissue** and are the most prevalent type of mesenteric cyst, often containing **chylous fluid**. - They are typically **thin-walled, unilocular or multilocular**, and can be found anywhere in the mesentery. *Enterogenous* - These cysts are **duplications of the alimentary tract** and are lined by **gastrointestinal mucosa**, often presenting with symptoms related to their connection with the bowel. - They are less common than chylolymphatic cysts and may contain **mucinous material**. *Dermoid* - Dermoid cysts are **teratomas** containing various **ectodermal elements** like hair, teeth, or sebaceous material. - While they can occur in various locations, they are a **very rare** type of mesenteric cyst. *Urogenital remnant* - Cysts arising from urogenital remnants are exceedingly rare in the mesentery, as their primary location would be in close proximity to the **genitourinary organs**. - These cysts typically originate from **embryological structures** that fail to regress completely.
Question 9: Earliest congenital malformation that may be detected on USG is
- A. Down's syndrome
- B. Hydrocephalus
- C. Sacral Agenesis
- D. Anencephaly (Correct Answer)
Explanation: ***Anencephaly*** - **Anencephaly** is a severe form of **neural tube defect** characterized by the absence of a major portion of the brain, skull, and scalp. - This malformation is often detectable as early as the first trimester (10-14 weeks) on **ultrasound scans** due to the absence of the normal cranial vault and brain tissue. *Down's syndrome* - While Down's syndrome (Trisomy 21) can have numerous ultrasound markers (e.g., nuchal translucency, absent nasal bone, heart defects), these are **not structural malformations in themselves** but indicators for genetic testing. - It's a **chromosomal abnormality**, and its features are often subtle and develop over time, becoming more apparent later in gestation or requiring specific measurements rather than gross structural absence. *Hydrocephalus* - **Hydrocephalus** (excess cerebrospinal fluid in the brain) is typically a progressive condition where ventricle enlargement becomes significant enough to be detected later in gestation, usually in the late second or third trimester. - While theoretically possible in the first trimester, it is **rarely the earliest congenital malformation** detectable as marked ventricular dilation usually takes time to develop. *Sacral Agenesis* - **Sacral agenesis** (caudal regression syndrome) involves the abnormal development of the lower spine and sacrum. - This condition is usually detected in the **second or third trimester** when skeletal structures are more ossified and clearly visible, making it less likely to be the earliest identifiable malformation compared to severe neural tube defects.
Question 10: All of the following statements about laryngomalacia are true, except for:
- A. It is associated with an omega shaped epiglottis
- B. Surgical Tracheostomy is the treatment of choice (Correct Answer)
- C. It is the most common congenital anomaly of the larynx
- D. Stridor is increased on crying and relieved on lying prone
Explanation: ***Surgical Tracheostomy is the treatment of choice*** - While laryngomalacia is the most common cause of **stridor** in infants, most cases are **mild and self-limiting**, resolving spontaneously by **12 to 18 months of age**. - **Surgical tracheostomy** is reserved for severe cases with significant **airway obstruction**, **failure to thrive**, or **life-threatening apneic spells** after failed conservative management and supraglottoplasty. *It is the most common congenital anomaly of the larynx* - This statement is **true**; laryngomalacia is indeed the most frequent congenital anomaly of the larynx, affecting approximately **60% of all congenital laryngeal anomalies**. - It is the most common cause of **inspiratory stridor** in infants. *It is associated with an omega shaped epiglottis* - This statement is **true**; the characteristic finding in laryngomalacia is the collapse of the **supraglottic structures** during inspiration. - This often includes a **long, curled, 'omega-shaped' epiglottis**, which contributes to the airway obstruction. *Stridor is increased on crying and relieved on lying prone* - This statement is **true**; the inspiratory stridor in laryngomalacia is typically **worsened by agitation, crying, feeding, or supine positioning**. - Lying **prone** or **side-lying** positions can help relieve the stridor by allowing the supraglottic tissues to fall forward and open the airway.
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