Pierre Robin Sequence and Airway Management — MCQs
Middle age diabetic with tooth extraction with ipsilateral swelling over middle one-third of sternocleidomastoid & displacement of tonsils towards contralateral -
A newborn male is noted to have difficulty feeding and "turns blue and chokes when drinking formula." The prenatal records reveal that the amniotic fluid appeared normal on ultrasound. A pediatric feeding tube is passed orally to 20 cm without difficulty, with gastric secretions aspirated. Which of the following is the most likely diagnosis?
A man takes peanut and develops tongue swelling, neck swelling, stridor, hoarseness of voice. What is the probable diagnosis?
A four-year-old child presents with a large face, large jaw, large ears, and macroorchidism. What is the most likely diagnosis?
Which one of the following life-threatening congenital anomalies in the newborn presents with polyhydramnios, aspiration pneumonia, excessive salivation and difficulty in passing a nasogastric tube?
Which malformation is associated with mutations in the HOX gene?
Statement 1 - A 59-year-old patient presents with flaccid bullae. Histopathology shows a suprabasal acantholytic split. Statement 2 - The row of tombstones appearance is diagnostic of Pemphigus vulgaris.
A child who underwent a tonsillectomy started bleeding while lying in the ward post-operatively. Which of the following is the most appropriate management step?
The first step on priority basis required in the management of status epilepticus is
As per the recent guidelines of resuscitation, what should be done if asystole is not responding to two consecutive doses of epinephrine?
Pierre Robin Sequence and Airway Management Indian Medical PG Practice Questions and MCQs
Question 1: Middle age diabetic with tooth extraction with ipsilateral swelling over middle one-third of sternocleidomastoid & displacement of tonsils towards contralateral -
- A. Ludwigs angina
- B. Parapharyngeal abscess (Correct Answer)
- C. Retropharyngeal abscess
- D. None of the options
Explanation: ***Parapharyngeal abscess*** - The **ipsilateral swelling** over the middle one-third of the sternocleidomastoid and **contralateral tonsil displacement** are classic signs of a parapharyngeal abscess, often secondary to an odontogenic infection. - This location involves the space lateral to the pharynx, which can expand and push structures like the tonsils medially. *Ludwig's angina* - Ludwig's angina is a **rapidly progressive cellulitis** of the submandibular, sublingual, and submental spaces, typically bilateral. - It presents with **brawny induration** of the neck and elevation of the tongue, but usually without a distinct mass displacing the tonsils. *Retropharyngeal abscess* - A retropharyngeal abscess develops in the space behind the posterior pharyngeal wall, usually presenting with **swelling of the posterior pharyngeal wall**. - It more commonly causes **dysphagia**, **stridor**, and neck stiffness, and does not typically result in significant ipsilateral external neck swelling or contralateral tonsil displacement like a parapharyngeal abscess. *None of the options* - The described clinical presentation, with a history of **tooth extraction**, ipsilateral sternocleidomastoid swelling, and contralateral tonsil displacement, is highly specific for a parapharyngeal abscess. - This option is incorrect because there is a clear and well-matched diagnosis among the choices.
Question 2: A newborn male is noted to have difficulty feeding and "turns blue and chokes when drinking formula." The prenatal records reveal that the amniotic fluid appeared normal on ultrasound. A pediatric feeding tube is passed orally to 20 cm without difficulty, with gastric secretions aspirated. Which of the following is the most likely diagnosis?
- A. Floppy epiglottis
- B. Tracheoesophageal fistula (Correct Answer)
- C. Zenker diverticulum
- D. Congenital heart disease
Explanation: ***Tracheoesophageal fistula*** - The combination of **feeding difficulties**, **cyanosis** ("turns blue"), and **choking** in a newborn, along with the ability to pass a feeding tube to 20 cm, strongly suggests a tracheoesophageal fistula (TEF), specifically a type where the **proximal esophagus ends in a blind pouch and the distal esophagus connects to the trachea**. - The **normal amniotic fluid** on prenatal ultrasound suggests that the fetus was able to swallow amniotic fluid, ruling out esophageal atresia without a fistula as the primary cause of polyhydramnios. Passage of the feeding tube to 20 cm and aspiration of gastric secretions indicates that the stomach is connected to the esophagus, but the reflux of gastric contents during feeding leads to aspiration into the trachea via the fistula. *Floppy epiglottis* - **Laryngomalacia**, or "floppy epiglottis," typically causes **stridor** (a high-pitched inspiratory sound), especially when crying or feeding, which is not the primary symptom described here. - While it can cause some feeding difficulties, **severe cyanosis and recurrent choking** during feeding are more characteristic of aspiration due to a different anatomical defect like TEF. *Zenker diverticulum* - A **Zenker diverticulum is a pharyngoesophageal pouch** that typically causes dysphagia, regurgitation of undigested food, and halitosis, primarily in **older adults**. - It is an acquired condition and **extremely rare in newborns**, making it an unlikely diagnosis for these symptoms in an infant. *Congenital heart disease* - While congenital heart disease can cause **cyanosis and feeding difficulties** due to increased metabolic demand or heart failure, it would not typically present with the specific description of **choking during feeding and successful passing of a feeding tube to the stomach with aspirated gastric secretions**. - The symptoms in this case point more directly to a **problem with the swallowing mechanism or the connection between the esophagus and the trachea**.
Question 3: A man takes peanut and develops tongue swelling, neck swelling, stridor, hoarseness of voice. What is the probable diagnosis?
- A. FB in larynx
- B. Angioneurotic edema (Correct Answer)
- C. Parapharyngeal abscess
- D. FB bronchus
Explanation: Andioneurotic edema - The combination of **tongue swelling**, **neck swelling**, **stridor**, and **hoarseness of voice** following peanut ingestion is highly suggestive of **angioneurotic edema**, a severe allergic reaction that can lead to airway obstruction [1]. - This is a life-threatening condition requiring immediate medical intervention, often associated with generalized **anaphylaxis** when triggered by allergens [2]. *FB in larynx* - While a **foreign body (FB) in the larynx** can cause stridor and hoarseness, the widespread swelling of the tongue and neck points away from a localized laryngeal obstruction [3]. - A laryngeal FB would typically be associated with a more sudden onset of choking and coughing, not diffuse edema [3]. *Parapharyngeal abscess* - A **parapharyngeal abscess** would typically present with **fever**, **severe throat pain**, and **trismus** (difficulty opening the mouth), which are not mentioned in this scenario. - The acute, rapid onset of symptoms after peanut consumption is inconsistent with the slower progression of an abscess. *FB bronchus* - A **foreign body in the bronchus** would primarily cause **coughing**, **wheezing**, and possibly **respiratory distress**, often unilateral, rather than severe global swelling of the tongue and neck. - Inspiratory stridor and hoarseness are more indicative of upper airway involvement than bronchial obstruction.
Question 4: A four-year-old child presents with a large face, large jaw, large ears, and macroorchidism. What is the most likely diagnosis?
- A. Down syndrome
- B. Cri du chat syndrome
- C. Fragile X syndrome (Correct Answer)
- D. McCune-Albright syndrome
- E. Klinefelter syndrome
Explanation: ***Fragile X syndrome*** - This syndrome is characterized by **macroorchidism** (enlarged testes) and distinct facial features such as a **large face**, **prominent jaw**, and **large ears**. - It is an X-linked dominant disorder caused by a **FMR1 gene mutation** leading to an increased number of CGG trinucleotide repeats. *Down syndrome* - Characterized by **upslanting palpebral fissures**, **epicanthic folds**, a **flat nasal bridge**, and a **single palmar crease**, which are not described here. - While associated with intellectual disability, it does not present with macroorchidism or the specific facial features mentioned. *Cri du chat syndrome* - This syndrome is known for a distinctive **high-pitched cry** resembling a cat's meow in infancy, along with **microcephaly** and **hypertelorism**. - It results from a deletion on the **short arm of chromosome 5** and does not typically present with macroorchidism or a large jaw. *McCune-Albright syndrome* - This syndrome is characterized by a triad of **fibrous dysplasia of bone**, **café-au-lait spots** (irregularly shaped hyperpigmented skin lesions), and **precocious puberty**. - It is caused by a somatic mutation in the **GNAS gene** and does not involve the specific facial features or macroorchidism described. *Klinefelter syndrome* - This syndrome (47,XXY) presents with **tall stature**, **gynecomastia**, and **small, firm testes** (not macroorchidism), typically noted after puberty. - Patients may have mild developmental delay but lack the distinctive facial features (large jaw, large ears) seen in this case.
Question 5: Which one of the following life-threatening congenital anomalies in the newborn presents with polyhydramnios, aspiration pneumonia, excessive salivation and difficulty in passing a nasogastric tube?
- A. Choanal atresia
- B. Gastroschisis
- C. Diaphragmatic hernia
- D. Tracheo-esophageal fistula (Correct Answer)
Explanation: ***Tracheo-esophageal fistula*** - This condition presents with **polyhydramnios** due to the fetus being unable to swallow amniotic fluid, **excessive salivation** from accumulated secretions in the blind-ending esophageal pouch, and difficulty passing a **nasogastric tube** because of the esophageal obstruction. - **Aspiration pneumonia** is a common complication as saliva and gastric contents can be aspirated into the lungs through the fistula. *Choanal atresia* - Characterized by **blocked nasal passages**, leading to **cyclical cyanosis** relieved by crying, but not typically associated with polyhydramnios or excessive salivation in this manner. - While it can cause respiratory distress, it does not involve esophageal obstruction or directly cause aspiration pneumonia from swallowed fluids. *Gastroschisis* - This is an **abdominal wall defect** where intestines protrude outside the body, unrelated to swallowing difficulties, polyhydramnios caused by inability to swallow, or excessive salivation. - It does not involve difficulty in passing a nasogastric tube or directly cause aspiration pneumonia. *Diaphragmatic hernia* - Involves **abdominal contents herniating into the chest cavity**, leading to **pulmonary hypoplasia** and respiratory distress. - It does not explain polyhydramnios due to impaired swallowing, excessive salivation, or the characteristic inability to pass a nasogastric tube.
Question 6: Which malformation is associated with mutations in the HOX gene?
- A. Polysyndactyly (Correct Answer)
- B. Holoprosencephaly
- C. Mayer Rokitansky syndrome
- D. Gorlin syndrome
Explanation: ***Polysyndactyly*** - The **HOX gene** plays a critical role in limb development and is associated with the malformation of **polysyndactyly**, which is characterized by extra fingers or toes [1]. - This condition is due to the disruption of the normal **patterning** during limb formation, directly involving the action of HOX genes [1]. *Gorlin syndrome* - Gorlin syndrome is primarily caused by mutations in the **PTCH1 gene**, linked to **basal cell carcinoma** and other abnormalities. - It does not involve HOX gene mutations, hence is **not** related to limb malformations. *Holoprosencephaly* - Holoprosencephaly is a developmental condition often linked to **chromosomal anomalies** and abnormal embryonic development, **not specifically** HOX gene mutations. - It refers to the incomplete separation of the forebrain, distinct from the **limb malformations** associated with HOX genes. *Mayer Rokitansky syndrome* - Mayer-Rokitansky syndrome involves **agenesis** or **hypoplasia** of the uterus and upper two-thirds of the vagina, which is due to other genetic factors. - This condition is not related to the functions of the **HOX genes** in limb or skeletal development. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Bones, Joints, and Soft Tissue Tumors, p. 1186.
Question 7: Statement 1 - A 59-year-old patient presents with flaccid bullae. Histopathology shows a suprabasal acantholytic split. Statement 2 - The row of tombstones appearance is diagnostic of Pemphigus vulgaris.
- A. Statements 1 & 2 are correct, 2 is not explaining 1 (Correct Answer)
- B. Statements 1 and 2 are correct and 2 is the correct explanation for 1
- C. Statements 1 and 2 are incorrect
- D. Statement 1 is incorrect
Explanation: ***Correct: Statements 1 & 2 are correct, 2 is not explaining 1*** **Analysis of Statement 1:** - A 59-year-old patient with **flaccid bullae** and **suprabasal acantholytic split** on histopathology is the classic presentation of **Pemphigus vulgaris** - The flaccid (easily ruptured) nature of bullae distinguishes it from tense bullae seen in bullous pemphigoid - The suprabasal location of the split (just above the basal layer) with acantholysis (loss of cell-to-cell adhesion) is pathognomonic - **Statement 1 is CORRECT** ✓ **Analysis of Statement 2:** - The **"row of tombstones" or "tombstone appearance"** is indeed a diagnostic histopathological feature of Pemphigus vulgaris - This appearance results from basal keratinocytes remaining attached to the basement membrane while suprabasal cells separate due to acantholysis - The intact basal cells standing upright resemble a row of tombstones - **Statement 2 is CORRECT** ✓ **Does Statement 2 explain Statement 1?** - Statement 2 describes a **histopathological appearance** (tombstone pattern) that is a **consequence** of the suprabasal split - However, it does NOT explain the **underlying cause** of the flaccid bullae or the suprabasal split - The true explanation involves **IgG autoantibodies against desmoglein 3 (and desmoglein 1)**, which attack intercellular adhesion structures (desmosomes), causing **acantholysis** - Therefore, **Statement 2 does NOT explain Statement 1** ✗ *Incorrect: Statement 2 is the correct explanation for Statement 1* - While both statements describe features of Pemphigus vulgaris, the tombstone appearance is a descriptive finding, not an explanatory mechanism *Incorrect: Statements 1 and 2 are incorrect* - Both statements are medically accurate descriptions of Pemphigus vulgaris features *Incorrect: Statement 1 is incorrect* - Statement 1 correctly describes the cardinal clinical and histopathological features of Pemphigus vulgaris
Question 8: A child who underwent a tonsillectomy started bleeding while lying in the ward post-operatively. Which of the following is the most appropriate management step?
- A. Take to OT, remove the clot & re-ligation (Correct Answer)
- B. Conservative management
- C. Take to OT & pressure packing
- D. Cautery
Explanation: ***Take to OT, remove the clot & re-ligation*** - **Post-tonsillectomy bleeding** is a surgical emergency requiring immediate intervention to prevent airway compromise and significant blood loss. - The most definitive management involves returning to the **operating theatre** for direct visualization, removal of any obstructing clots, and **re-ligation** of the bleeding vessel. *Conservative management* - **Conservative management** is generally insufficient for significant post-tonsillectomy bleeding, as it does not address the source of hemorrhage and can lead to severe complications. - While minor oozing might be observed, active bleeding often indicates a larger vessel injury that requires **surgical hemostasis**. *Take to OT & pressure packing* - While **pressure packing** can temporarily slow bleeding, it is not a definitive long-term solution as it does not directly identify and treat the bleeding vessel. - Furthermore, pharyngeal packing in a child carries a risk of **airway obstruction** and aspiration, making it less suitable than direct re-ligation. *Cautery* - **Cautery** is a method of hemostasis, but it is typically performed in the operating theatre under direct vision, often after clot removal. - It is not a stand-alone initial management step in the ward for active bleeding without proper surgical assessment and preparation.
Question 9: The first step on priority basis required in the management of status epilepticus is
- A. i.v. Phenytoin
- B. i.v. Phenobarbitone
- C. Airway maintenance (Correct Answer)
- D. i.v. Diazepam
Explanation: ***Airway maintenance*** - Maintaining a **patent airway** is the absolute first step in any emergency, especially in status epilepticus where respiratory depression and aspiration risk are high [2]. - Ensuring adequate **oxygenation and ventilation** is critical for preventing brain hypoxia and further complications [1]. *i.v. Phenytoin* - While an important drug for the long-term management and prevention of recurrent seizures, **phenytoin** has a delayed onset of action and is not the first-line agent for acute seizure termination in status epilepticus. - It is typically administered after initial first-line agents like benzodiazepines have been given. *i.v. Phenobarbitone* - **Phenobarbitone** is a potent anticonvulsant and can be used in refractory status epilepticus, but it is not the very first step. - Its use often comes with significant **sedation and respiratory depression**, necessitating close airway monitoring. *i.v. Diazepam* - **Intravenous diazepam** is a rapid-acting benzodiazepine and is usually the first-line medication to **terminate acute seizures** in status epilepticus. - However, airway maintenance precedes even medication administration to ensure patient safety before drug effects take hold [1], [2].
Question 10: As per the recent guidelines of resuscitation, what should be done if asystole is not responding to two consecutive doses of epinephrine?
- A. Administer another dose of epinephrine.
- B. Continue high-quality CPR and consider advanced airway management. (Correct Answer)
- C. Administer vasopressin as a second-line drug.
- D. Defibrillation with 200J.
Explanation: ***Continue high-quality CPR and consider advanced airway management.*** - For **asystole** that is unresponsive to initial epinephrine doses, maintaining **high-quality CPR** is the cornerstone of resuscitation efforts, ensuring vital organ perfusion. - **Advanced airway management** (e.g., endotracheal intubation) should be considered early to secure the airway and facilitate ventilation, optimizing oxygen delivery during CPR. *Administer another dose of epinephrine.* - While epinephrine is the primary drug for asystole, repeating doses beyond the initial recommended schedule without other interventions is not indicated if there is no response, as it may not improve outcomes. - The focus shifts to identifying and treating reversible causes while maintaining high-quality CPR, rather than escalating epinephrine frequency. *Administer vasopressin as a second-line drug.* - **Vasopressin** is no longer recommended in adult cardiac arrest resuscitation algorithms, including for asystole, according to current guidelines from organizations like the American Heart Association. - Its use has not been shown to improve survival to hospital discharge or neurological outcomes compared to epinephrine. *Defibrillation with 200J.* - **Defibrillation** is only indicated for shockable rhythms such as **ventricular fibrillation (VF)** or **pulseless ventricular tachycardia (pVT)**. - Asystole is a **non-shockable rhythm**, meaning there is no electrical activity to defibrillate, and administering a shock is ineffective and can be harmful.
Want unlimited practice?
Get full access to all questions, explanations, and performance tracking.
Start For Free