Pulm

Pyriform Aperture Stenosis

  • Definition: type of congenital airway obstruction characterized by narrowing of the bony structure at the anterior nasal opening (pyriform aperture).

Cause

  • Bony overgrowth at the anterior bony opening (pyriform aperture).

Clinical Features

  • Symptoms include noisy breathing and respiratory distress, especially worsening during feeding while improving with crying.

  • Associated with other conditions such as craniosynostosis and pituitary anomalies.

Diagnostic Testing

  • A CT scan showing a small nasal opening can confirm the diagnosis.

Treatment

  • Initial management may include nasal stenting, which can be effective.

  • Some patients may require tracheostomy until they are large enough to undergo surgical correction.

Nasal Foreign Body and Nasal Polyps

Nasal Foreign Body

  • Suspicion: Should be raised when a child presents with unilateral foul-smelling nasal discharge.

Nasal Polyps

  • Association: Consider cystic fibrosis (CF) when nasal polyps are observed.

  • Other Causes:

    • Chronic sinusitis

    • Allergic rhinitis

    • Samter's triad (asthma, nasal polyps, and aspirin sensitivity)

  • Treatment: Nasal steroids can be effective at reducing the size of nasal polyps.

Oropharynx Malformations

  • Includes:

    • Lingual Ankyloglossia

    • Lingual Thyroid

Lingual Ankyloglossia

  • Definition: A condition characterized by a restriction of tongue movement due to a prominent lingual frenulum, commonly referred to as "tongue tie."

    • Major Features:

    • More prevalent in males.

    • Can exacerbate or cause mouth breathing, leading to sleep issues.

    • Treatment: Frenotomy is a surgical option to correct this condition.

Lingual Thyroid

  • Definition: A condition resulting from the failure of thyroid tissue to descend into the neck, where thyroid tissue is found at the base of the tongue.

    • Clinical Features: Airway obstruction can occur due to the positioning of this tissue.

    • Treatment: Often, no treatment is required. In severe cases, surgery may be necessary, and lifelong thyroid hormone replacement will be indicated.

Larynx Malformations

  • Include:

    • Laryngeal Lesions

    • Laryngomalacia

    • Subglottic Stenosis

    • Laryngeal Webs

    • Laryngeal Atresia

Laryngeal Lesions

  • Clinical Features: Symptoms can include stridor (primarily inspiratory), hoarseness, aphonia, and feeding disorders.

Laryngomalacia

  • Definition: A condition characterized by the collapse of the supraglottic structures during inspiration. It is the most common congenital anomaly of the larynx.

    • Clinical Features:

    • Presents with inspiratory stridor in infants.

    • Symptoms worsen when the infant is supine, crying, or feeding.

    • Symptoms typically progress over weeks to months.

    • Treatment: Usually self-limiting, with spontaneous resolution by 12-18 months. In severe cases, referral to an ENT specialist is warranted, especially if there are symptoms like feeding difficulties, dyspnea, tachypnea, cyanosis, or apnea. Speech and swallow therapy may also be indicated.

Subglottic Stenosis

  • Definition: Narrowing of the cricoid lumen in the larynx.

    • Causes:

    • Congenital causes usually improve as the child grows.

    • Acquired causes often stem from trauma or prior instrumentation.

    • Clinical Features: Most commonly presents as recurrent croup but can cause biphasic stridor in severe newborn cases.

    • Diagnostic Testing: Direct visualization is required, with treatment for symptomatic patients.

Laryngeal Webs and Laryngeal Atresia

  • Laryngeal Atresia:

    • Definition: Causes complete upper airway obstruction, presenting as asphyxia at birth.

    • Treatment: Requires emergent tracheostomy.

  • Laryngeal Webs:

    • Definition: Rare congenital anomalies leading to incomplete separation of the vocal folds.

    • Clinical Features: Present in infancy with respiratory distress and an unusual cry.

    • Treatment: Surgical intervention is required.

Tracheal/Bronchial Malformations

  • Include:

    • Tracheal Atresia

    • Tracheal Stenosis

    • Tracheomalacia

    • Bronchomalacia

Tracheal Atresia and Tracheal Stenosis

  • Tracheal Atresia:

    • Definition: Very rare condition resulting in respiratory distress immediately after birth; infants often do not survive long. If proximal TEF (tracheoesophageal fistula) is present, intubation is possible via this route.

    • Prognosis: Generally poor.

  • Tracheal Stenosis:

    • Definition: Narrowing that can affect a short segment or the entire trachea, often presenting with restrictive symptoms such as sternal retractions, stridor, and a monophonic wheeze unreponsive to bronchodilators.

    • Diagnosis and Treatment: Diagnosis via bronchoscopy. Surgical intervention may be necessary.

Tracheomalacia

  • Definition: A condition characterized by abnormal flaccidity of the trachea during breathing that leads to collapse on expiration.

    • Clinical Features: May present with expiratory stridor at birth but often goes unnoticed until the child becomes more active or during respiratory infections.

    • Treatment: Many cases resolve spontaneously within 6-12 months. CPAP can help maintain tracheal patency in severe cases; surgery may be required.

Bronchomalacia

  • Definition: Characterized by weak bronchial cartilage during expiration, possibly leading to tracheobronchomalacia if combined with tracheal weakness.

  • Types:

    • Primary bronchomalacia: Congenital absence of cartilaginous rings.

    • Secondary bronchomalacia: Results from external compression (e.g., enlarged vessels, vascular rings, bronchogenic cysts).

    • Clinical Features: Recurrent respiratory infections and severe respiratory distress in severe cases.

    • Diagnostic Testing: Bronchoscopy indicated.

Lung Malformations

  • Include:

    • Congenital Lobar Emphysema

    • Pulmonary Hypoplasia

    • Pulmonary Arteriovenous Fistula

    • Bronchopulmonary Sequestration

Congenital Lobar Emphysema

  • Definition: A developmental anomaly of the lower respiratory tract leading to hyperinflation of one or more pulmonary lobes.

    • Clinical Features:

    • Most often affects the left upper lobe.

    • Symptoms can arise suddenly or have a more gradual onset including signs of respiratory distress, tachypnea, cyanosis, and wheezing.

    • Physical examination may reveal hyperresonance on percussion, displaced PMI.

    • Diagnostic Testing:

    • Chest X-ray showing distended lobe and mediastinal shift.

    • Treatment: Lobectomy for symptomatic patients; conservative management for asymptomatic individuals.

Pulmonary Hypoplasia

  • Definition: An uncommon condition represented by incomplete lung development.

    • Clinical Features: It is a common cause of neonatal death and typically presents with respiratory distress, hypoxia, and hypercarbia at birth.

    • Risk Factors: Premature rupture of membranes, premature delivery, congenital diaphragmatic hernia.

    • Prognosis: Dependent on severity and associated anomalies.

    • Treatment: Extracorporeal membrane oxygenation or mechanical ventilation until adequate lung growth.

Pulmonary Arteriovenous Fistula

  • Definition: An abnormal connection between pulmonary arteries and veins, leading to a pathological intrapulmonary right-to-left shunt and impaired gas exchange.

    • Clinical Features: Symptoms include dyspnea, hemoptysis, and exercise intolerance.

    • Risk Factors: Strongly associated with Hemorrhagic Hereditary Telangiectasia, also known as Osler-Weber-Rendu syndrome.

    • Treatment: Options include ablation by angiography or surgical removal if ablation fails.

Bronchopulmonary Sequestration

  • Definition: An anomaly characterized by a mass of non-functioning lung tissue without normal communication with the tracheobronchial tree, receiving its arterial supply from the systemic circulation.

    • Types:

    1. Intralobar: Contained within normal lung parenchyma and visceral pleura (75-86%), diagnosed later in life often due to chronic infections; surgery includes lobectomy.

    2. Extralobar: Separated from regular lung tissue and pleura (14-25%); diagnosed in utero via ultrasound; treated with resection.

Chest Wall Malformations

  • Include:

    • Kyphoscoliosis

    • Pectus Excavatum

    • Pectus Carinatum

Kyphoscoliosis

  • Definition: Excessive curvature of the thoracic spine causing severe lateral deformity.

    • Cause: Most cases are idiopathic.

    • Clinical Features: Curvature >50 degrees may lead to restrictive changes in pulmonary function tests (PFTs); curvature >90 degrees can cause cardiopulmonary compromise.

Pectus Excavatum / Pectus Carinatum

  • Pectus Excavatum:

    • Definition: Inward bowing of the midsternum, can be congenital or acquired. Commonly causes respiratory issues in severe cases.

    • Treatment: Usually surgical for cosmetic reasons.

  • Pectus Carinatum:

    • Definition: Protrusion of the sternum.

    • Treatment: Surgical correction is usually cosmetic as well.

Diaphragm Malformations

Congenital Diaphragmatic Hernia

  • Definition: A condition where bowel herniates through the diaphragm into the chest, halting lung development, leading to pulmonary hypoplasia.

    • Common Defect: Posterolateral defect (90%) on the left side.

    • Clinical Features: Presents with respiratory distress, barrel-shaped chest, and scaphoid abdomen at birth.

    • Diagnostic Testing: Chest X-ray may show loops of bowel in the thoracic cavity with mediastinal shift.

    • Treatment: Initial management includes intubation and NG tube with suction; definitive treatment requires surgical intervention.

Pulmonary Infections

Upper Respiratory Infection

  • Cause: Most commonly viral (shown from rhinovirus, coronavirus, adenovirus, and coxsackie virus).

  • Clinical Features: Common in fall/winter; patients may exhibit low-grade fever, rhinorrhea, cough, and nasal congestion. Viral shedding peaks at 2-7 days post infection.

  • Risk Factors: Exposure through daycare/schools, secondhand smoke, overcrowding.

  • Diagnostic Testing: Not needed unless RSV/Influenza/COVID-19 suspected.

  • Complications: Acute Otitis Media (AOM), sinusitis, asthma exacerbation, pneumonia.

  • Treatment: Supportive—hydration, saline, suction; recommend avoidance of OTC cold/cough meds in children <12 years.

Acute Bronchitis

  • Cause: Acute inflammation of the tracheobronchial tree, often self-limiting.

  • Clinical Features: Dry cough progressing to a productive cough with intermittent rhonchi.

  • Progression of Symptoms: Symptoms start as cold-like, worsening over a week, and persist for 2-4 weeks.

  • Diagnostic Testing: CBC may show normal WBC or viral shift; pulmonary function tests may indicate airway obstruction.

  • Treatment: Supportive care includes saline sprays and hydration.

Protracted Bacterial Bronchitis

  • Etiology: Follows a URI wherein bacteria (H. influenzae, S. pneumoniae) colonize airways and from biofilms impair mucociliary clearance.

  • Symptoms: Persistent wet cough lasting >4 weeks; worsens with exertion or at night with a