Pediatric Otolaryngology Practice Questions

Q: All are seen in Treacher Collins syndrome except?

Choanal atresia. **Explanation:** **Treacher Collins Syndrome (TCS)**, also known as **Mandibulofacial Dysostosis**, is an autosomal dominant disorder caused by mutations in the *TCOF1* gene. It results from the failure of migration of neural crest cells into the **first and second branchial arches**. **Why Choanal Atresia is the Correct Answer:** While TCS involves extensive craniofacial malformations, **Choanal atresia** is not a characteristic feature of this syndrome. Choanal atresia is more classically associated with **CHARGE syndrome** (Coloboma, Heart defects, Atresia choanae, Retardation, Genitourinary anomalies, and Ear abnormalities). **Analysis of Incorrect Options:** * **Conductive Deafness:** This is a hallmark of TCS. It occurs due to malformation of the ossicles (incus and malleus) and/or **meatal atresia** (narrowing or absence of the external auditory canal). * **Cleft Palate:** Approximately 30% of patients with TCS present with a cleft palate, often accompanied by macrostomia (large mouth). * **Mandibular Hypoplasia:** This is a defining feature. Patients exhibit a "bird-like" facies due to micrognathia (small jaw) and malar (cheekbone) hypoplasia. **High-Yield Clinical Pearls for NEET-PG:** * **Antimongoloid slant:** The eyes show a downward slant of the palpebral fissures. * **Coloboma:** Notch-like defects are typically seen in the **outer third of the lower eyelids**. * **Fish-mouth appearance:** Due to the combination of mandibular hypoplasia and macrostomia. * **Inheritance:** Autosomal Dominant (most common) but can be sporadic. * **Inner Ear:** Usually remains normal as it develops from the otic capsule, not the branchial arches.

Q: An 8-month-old infant presented with stridor and respiratory difficulty, which worsened on crying. Laryngoscopy revealed a red mass in the subglottic area. All the following are used in the treatment of this condition EXCEPT:

Radiotherapy. ### Explanation The clinical presentation of an 8-month-old with stridor and a red subglottic mass is classic for a **Subglottic Hemangioma**. This is the most common benign vascular neoplasm of the pediatric airway. **Why Radiotherapy is the Correct Answer (The "Except"):** Radiotherapy is **contraindicated** in the treatment of subglottic hemangioma. While it was historically used to shrink vascular masses, it is now strictly avoided due to the high risk of inducing secondary malignancies (like thyroid carcinoma) and causing permanent damage to the developing laryngeal cartilage and growth plates in infants. **Analysis of Other Options:** * **Corticosteroids:** Historically the first-line medical therapy, steroids help reduce the size of the hemangioma by inducing vasoconstriction and inhibiting angiogenesis. * **CO2 Laser Vaporization:** This is a surgical option used for small, localized, or pedunculated lesions. It allows for precise removal with minimal bleeding. * **Tracheostomy:** This is a life-saving procedure reserved for severe cases where the airway is critically compromised and medical or conservative surgical management fails. It secures the airway until the hemangioma naturally undergoes its involution phase. **High-Yield Clinical Pearls for NEET-PG:** * **Gold Standard Treatment:** Currently, **Oral Propranolol** (a non-selective beta-blocker) is the first-line treatment of choice for subglottic hemangiomas, replacing steroids in most protocols. * **Biphasic Nature:** These lesions typically follow a growth phase (proliferation) for the first 12–18 months, followed by a slow regression phase (involution). * **Association:** Approximately 50% of children with subglottic hemangiomas also have cutaneous "strawberry" hemangiomas, often in a "beard distribution." * **Diagnosis:** Definitive diagnosis is made via direct laryngoscopy and bronchoscopy. Biopsy is usually avoided due to the high risk of hemorrhage.

Q: Pierre Robin syndrome is characterized by which of the following features, or the absence thereof?

Coloboma of the iris. **Explanation:** **Pierre Robin Sequence (PRS)** is a clinical triad resulting from a developmental failure during embryogenesis. The primary defect is **mandibular hypoplasia** (micrognathia), which prevents the tongue from descending, leading to **glossoptosis** (posterior displacement of the tongue). This, in turn, prevents the palatal shelves from fusing, resulting in a **U-shaped cleft palate**. **Why "Coloboma of the iris" is the correct answer:** The question asks for a feature that is **not** part of the syndrome (or the absence thereof). **Coloboma of the iris** is a characteristic feature of **CHARGE syndrome**, not Pierre Robin Sequence. While PRS can be associated with ocular issues in syndromic forms (like Stickler syndrome), iris coloboma is not a defining or typical feature. **Analysis of Incorrect Options:** * **Mandibular hypoplasia (Option D):** This is the initiating event in the sequence. The small jaw is the hallmark physical finding. * **Respiratory distress (Option B):** This is a critical clinical manifestation. Glossoptosis causes upper airway obstruction, leading to inspiratory stridor and cyanotic spells, especially when the infant is supine. * **Hearing loss (Option A):** Children with PRS frequently have Eustachian tube dysfunction due to the cleft palate, leading to chronic otitis media with effusion and subsequent **conductive hearing loss**. **NEET-PG High-Yield Pearls:** * **Sequence vs. Syndrome:** It is called a "Sequence" because one initial insult (micrognathia) triggers a cascade of secondary events. * **Management:** The immediate priority is maintaining the airway. This is often achieved through **prone positioning**, nasopharyngeal airways, or, in severe cases, mandibular distraction osteogenesis or tracheostomy. * **Associated Syndrome:** The most common syndrome associated with PRS is **Stickler Syndrome** (look for high myopia and joint hypermobility in questions).

Q: What is the commonest organism causing acute epiglottitis?

Haemophilus influenzae. **Explanation:** Acute epiglottitis is a life-threatening medical emergency characterized by rapid-onset inflammation of the epiglottis and supraglottic structures [2]. **1. Why Haemophilus influenzae is correct:** Historically and currently, **Haemophilus influenzae type b (Hib)** is the most common causative organism. Although the incidence has significantly decreased in regions with high Hib vaccination rates, it remains the leading pathogen identified in acute cases [2]. It typically causes a severe, fulminant bacterial infection leading to the classic "cherry-red epiglottis." **2. Why the other options are incorrect:** * **Clostridium:** These are anaerobic bacilli primarily associated with tetanus, botulism, or gas gangrene; they do not cause acute supraglottic infections. * **Streptococcus pneumoniae:** While it is a common cause of pneumonia and otitis media, and can occasionally cause epiglottitis in vaccinated populations, it is less common than Hib. * **Respiratory Syncytial Virus (RSV):** This is the most common cause of **Bronchiolitis** and a frequent cause of Croup (Laryngotracheobronchitis), but epiglottitis is almost exclusively a bacterial, not viral, disease. **Clinical Pearls for NEET-PG:** * **Classic Presentation:** The "4 Ds"—Drooling, Dysphagia, Dysphonia, and Distress [1]. * **Positioning:** The child often assumes the **"Tripod position"** (leaning forward on hands) to maintain the airway. * **X-ray Finding:** Lateral neck X-ray shows the **"Thumb sign"** (swollen epiglottis) [1]. * **Management:** The priority is **airway maintenance** (intubation or tracheostomy) [1]. Never examine the throat with a tongue depressor in a non-controlled setting, as it may trigger fatal laryngospasm [1].

Question 1

All are seen in Treacher Collins syndrome except?

Accepted Answer

Choanal atresia

Answer

Conductive deafness

Answer

Cleft palate

Answer

Mandibular hypoplasia

Question 2

Which of the following statements are true about pediatric tracheostomy?

Accepted Answer

The 3rd and 4th tracheal rings are incised.

Answer

Most common early complication is subcutaneous emphysema.

Answer

It is easy to remove the tracheostomy tube.

Answer

A complete tracheal ring is removed.

Question 3

An 8-month-old infant presented with stridor and respiratory difficulty, which worsened on crying. Laryngoscopy revealed a red mass in the subglottic area. All the following are used in the treatment of this condition EXCEPT:

Accepted Answer

Radiotherapy

Answer

CO2 laser vaporization

Answer

Corticosteroids

Answer

Tracheostomy

Question 4

Pierre Robin syndrome is characterized by which of the following features, or the absence thereof?

Accepted Answer

Coloboma of the iris

Answer

Hearing loss

Answer

Respiratory distress

Answer

Mandibular hypoplasia

Question 5

What is the commonest organism causing acute epiglottitis?

Accepted Answer

Haemophilus influenzae

Answer

Clostridium

Answer

Streptococcus pneumoniae

Answer

Respiratory Syncytial Virus (RSV)

Pediatric Otolaryngology — MCQs

Pediatric Otolaryngology — MCQs

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