Choanal Atresia: Types, Symptoms, and Diagnosis

Choanal Atresia

• Atresia means the closure of a normal opening.
• Choanal Atresia is a condition where the posterior nares are obstructed by membranous septum or bone, occurring either bilaterally or unilaterally.
• Most common congenital malformation of the nose.
• Occurs in 1:7,000 live births.
• 50% association with other congenital anomalies like craniofacial syndromes and skull-based defects termed encephalocele.
• More prevalent in females according to the text.
• Cause of choanal atresia is unknown.

Types of Choanal Atresia:

• Unilateral (usually on the right in about 60% of cases): can be undiagnosed until the child has persistent unilateral nasal drainage, particularly if a respiratory infection is present.
• Bilateral: may cause neonates to become apneic or cyanotic at birth, requiring resuscitation to prevent asphyxia/severe hypoxia.

Clinical Manifestations of Choanal Atresia

• Difficulty breathing after birth that may lead to cyanosis unless the infant is crying.
• Inability to nurse.
• Inability to breath at the same time.
• A catheter can't pass through either side of the nose into the throat.
• Show persistent one-sided nasal blockage/discharge.

Diagnosis of Choanal Atresia

• Generally, it's recognized shortly after birth, while the infant remains in the hospital.
• Physical Exam: Used for obstruction of the nose.
• Catheter Test: Confirmed by passing a firm catheter (Fr. 6) through each nostril 3-4 cm into the nasopharynx.
• Radiography: Using contrast material to determine the exact location of obstruction.
• Fiberoptic Endoscope: Used to look into the nose, ensure open airways, and assess healing following surgery.
• CT Scan: Done if feeding tube fails to pass through nasal passage and can detect composition and thickness of the atresia, depth of the nasopharynx, and any associated skull base anomalies.

Management of Choanal Atresia

• Airway Support Options: oral intubation or tracheostomy.
• Surgical Correction: aims to cure the obstruction. Surgery may be delayed until 2 or 3 years if only one nasal passage is blocked, allowing for better anatomy and easier access to the posterior choanae. Blood transfusion is not normally needed for repair at this age.
• Transnasal Approach: requires less operative time, lower morbidity but higher risk of nasal passages closing later.
• Transpalatal Approach: provides better exposure and more accurate bone removal.

Nasal Stents

• Small plastic tubes are stitched into the passages to keep them open.
• Stents are removed under general anesthesia after 6+ weeks.
• One possible complication is restenosis that requires dilatation and reoperation.

Nursing Care for Choanal Atresia

• Airway Support: Close observation with cardio respiratory monitoring and careful suctioning of mucus to maintain breathing, especially before feeding.
• Hydration and Nutrition: Use a feeding tube for bilateral choanal atresia until the defect is corrected, and for unilateral cases, feed the infant slowly with a small nipple in an upright position along with frequent pauses to allow breathing.

Croup

• Broad classification of upper airway illnesses due to inflammation and narrowing of the larynx, trachea and bronchi. Prevalent in children ages 5 and younger, specifically those born prematurely.
• Incidents peak in the 2nd year, with signs and symptoms that are generally most severe in children ages 3 as well as younger given their airways are narrower and more likely to experience blockage when infected.
• Croup usually occurs in the winter or early spring, and a child can get it more than once.

Causes of Croup

• Parainfluenza Virus is the most common cause of croup.
• Adenovirus, respiratory syncytial virus, influenza, and measles can also cause it.
• Can sometimes be caused by bacteria or an allergic reaction.

Upper Airway Changes in Croup

• The epiglottis and larynx swell, which occludes the airway.
• The trachea swells against the cricoid cartilage, causing restriction.

General Clinical Manifestations of Croup

• Croupy Cough: a loud, harsh, brassy or barking cough that is a result of swelling around the larynx and trachea where air is forced through this narrowed passage and vibrates.
• Dysponia: medical term of hoarseness, where laryngeal involvement dominates the clinical picture.
• Inspiratory Stridor: high-pitched or squeaking noise when breathing in due to narrowing of the airway.
• Respiratory Distress: due to swelling or obstruction of the larynx.

Types of Croup

• Acute Laryngitis involves the glottic area causing the least serious symptoms and is viral with an allergic/psychosomatic component.
• Spasmodic Croup typically exhibits sudden onset, especially at night and tends to recur.
• Laryngo-tracheo Bronchitis affects the subglottic area, progresses if untreated, most prevalent of the serious viral croup.
• Bacterial Tracheitis affects the upper trachea and requires close observation.
• Acute Epiglottitis affects the supraglottic area, is life-threatening, bacterial and rapidly progressive.

Diagnosis of Croup Includes

• Patient History: fever, cold symptoms, recent viral infection and clinical manifestation.
• X-ray: used to rule out foreign body obstruction or epiglottitis can show a steeple sign indicating the top of airway narrows to a point.
• WBC count: used to differentiate between bacterial and viral illnesses.

Management of Croup Focuses on

• Goal: maintain a patent airway and adequate respiratory exchange.
• Humidity: high humidity with cool mist helps decrease swelling of laryngeal tissue and mild cases can be managed at home with a cool-air vaporizer
• Sudden exposure to cold air can relieve spasms and warm mist from a hot running shower can be helpful. However, a hot steam in a pan should be avoided. Also use a mist tent for severe cases.
• Medications: racemic epinephrine nebulized to cause mucosal vasoconstriction decreasing edema, corticosteroids for anti-inflammatory effects against subglottic edema, antibiotics for bacterial croup, and antipyretics to reduce fever.
• Hydration: IVF during fever and sore throat.
• Artificial Airway: ET intubation/tracheostomy is needed and must be extubated when spasm and edema is no longer a problem.

Nursing Diagnoses for Croup

• Ineffective Airway Clearance: related to laryngeal edema, mucosal inflammation, and fatigue.
• Altered Tissue Perfusion: related to partially obstructed airway.
• High Risk for Fluid Volume Deficit: related to inability to meet body requirements and increased metabolic demands.
• Fear/Anxiety: related to acute illness, hospitalization, and uncertain course of illness and treatment.
• Knowledge Deficit: related to diagnosis, treatment, prognosis, and home care needs.

Nursing Care for Croup Implies

• Maintaining patent airway and respiratory exchange by assessing respiratory status every 2-4 hours, keep a suction and tracheostomy intubation tray at the bedside, and caring for child in a croupette (device for cool humidification with O2).
• Keeping the child dry and linens changed, tucking lose edges under the mattress, and maintaining the room near the nurses station/resuscitation equipment.
• Administering prescribed medications, avoiding visual mouth/throat inspections unless needed by a professional, and positioning the head of the bed up.
• Promoting balanced fluid and nutrition by restricting NPO in severe respiratory distress and assessing for dehydration.
• Promoting rest and comfort by providing a quiet environment in order to allay and anxiety.

Bronchiolitis

• Lower respiratory illness where an infecting agent causes inflammation and obstruction in the bronchioles.

Causes of Bronchiolitis:

• Respiratory Syncytial Virus (RSV) is easily spread through direct contact/droplet and is generally viral.
• Others are adenovirus and parainfluenza.
• Mycoplasma pneumonia is bacterial.

Incidence of Bronchiolitis

• Age Affected: Mainly affects infants with the most common age being 6 months, and is rare after 2 years of age.
• Sex: More often affects boys.
• Time of Year: Epidemics at late fall, early spring (October to March).

Risk Factors for Bronchiolitis

• Children in day care are more likely to get it.
• Those exposed to cigarette smoke.
• Babies in day care are less likely to get it than those at home with a parent who smokes.
• Formula-fed infants are more prone to bronchiolitis.
• Those at high risk for asthma appear prone to bronchiolitis and most kids have been exposed to an adult or another child with a cold in the previous week.

Pathophysiology of Bronchiolitis

• RSV invades mucosal cells lining the bronchioles → invaded cells die when virus bursts and spread to adjacent cells → cell debris clogs and obstructs bronchioles which irritates the airway causing swelling and increased mucus production resulting in bronchospasm → creating partial obstruction which leads to air trapping and complete obstruction. This causes emphysema and atelectasis leading to respiratory acidosis as well as hypoxia.

Clinical Manifestations of Bronchiolitis:

• Preceded by URI manifested as nasal stuffiness and mild fever progressing to increased respiratory distress after 1-2 days.
• Tachypnea
• Paroxysmal cough
• Irritability
• Wheezing
• Retraction
• Dyspnea
• Diminished breath sound
• Apnea

Diagnostic Tests for Bronchiolitis

• History: Exposure to cold previous week plus physical exam.
• Nasal or Nasopharyngeal Culture: Presence of RSV through ELISA/immunoflourescent antibody, and isolate/room together if result is positive to minimize spread.
• Chest X-ray: Shows hyper aeration and consolidation, reveals alveoli/atelectasis collapse and is a risk factor for COPD later in life.
• Pulse Oximetry
• ABG: Respiratory acidosis=Hypercapnea, hypoxemia.
• CBC: Leukocytosis and elevated ESR.

Management of Bronchiolitis

• Can be managed at home, but should be hospitalized if it becomes worse.
• Cardiorespiratory Monitor and Pulse Oximetry
• Oxygen Therapy via mask, nasal canula or high humidity mist tent.
• Hydration – may be on NPO until acute crisis has passed.
• Medications: Nebulizer Therapy, Antipyretics (Acetaminophen), mild sedatives, antibiotics (secondary bacterial infection), and antiviral for high-risk infants.

Prevention of Bronchiolitis Includes

• RSV-IGIV (less preferred): IV infusion that interferes with MMR or varicella vaccines.
• Palivizumab (preferred for high risk): IM monoclonal antibody that does not interfere with MMR and varicella.

Nursing Diagnoses for Bronchiolitis

• Ineffective airway clearance due to increased airway secretions, decreased energy from coughing and dyspnea.
• Ineffective breathing pattern due to inflamed tacheobronchial tree and progression.
• High Risk for Fluid Volume Deficit related to inability to meet the requirements of metabolic demands.
• Fear/Anxiety related to illness, treatment and home care.

Nursing Care for Bronchiolitis Consists Of

• Maintaining respiratory function and promoting rest/comfort.
• Promoting balanced fluid/nutrition when breathing is difficult.
• Using infection control measures like precautions and hygiene.

Cystic Fibrosis

• A chronic multi system, inherited as an autosomal recessive disorder characterized by exocrine gland dysfunction.
• The mucus produced by the exocrine glands abnormally thick causing obstruction of the small passageways and both parents are unaffected but carry trait and occurs in 1 of 4 children
• Equally affects male and females wherein the affected individual married to unaffected will have all kids be carriers.

Occurrence Rate of Cystic Fibrosis

• Occurs in 1:3000 live births with high occurrence among Caucasians and median age of survival is almost 37 years.
• Caused by a defect in a gene called the cystic fibrosis transmembrane conductance regulator (CFTR).

Manifestations of Cystic Fibrosis Include

• Progressive chronic lung disease
• Pancreatic enzyme deficiency, wherein acinar cells are primarily affected.
• Sweat gland dysfunction which is an electrolyte composition.

Cystic Fibrosis Symptoms

• Colonization of bacteria in the airway: S aureus, aeroginosa and H influenza which results in multiple and continuous episode of bronchitis and pneumonia.
• Emphysema: atelectasis occurs in the airways and chronic hypoxemia causes contraction of muscle fibers in pulmonary arteries leading to pulmonary hypertension or Cor Pulmonale.
• Dyspnea, Cyanosis, Clubbing of fingers and toes
• Earliest manifestation: Meconium ileus, small intestine blockage with thick putty like tenancious producing abdominal distention without passage of stool.

Skin Presentation

• Abnormally increase concentration of Na and Cl in sweat, can taste salty when kissed and dehydration and electrolyte imbalance due to heat.
• Can cause both delayed puberty in females and males are often sterile due to plugged vas deferens. And mucus plug can disrupt conception.

Diagnostic Tests for Cystic Fibrosis

• History and clinical manifestations.
• Diagnostic Tests: Sweat chloride test, Fecal Fat test/quantitative stool fat determination, Secretin stimulation and serum.
• Chromotrypsin in stool, upper GI small intestine and IRT tests are also diagnostic with the addition of chest X-ray.
• Electrolyte concentration found within sweat.

Sweat Choloride Test

• Also known as: Sweat Test, Sweat Choloride, Iontophoretic Sweat Test, Pilocarpine Iontophoresis.
• Done via stimulated stimulation, sweat collection and electrolyte measured using low level electric current applied to test area.
• Normal range is less than 40 MEq/L and if it is greater than 60 MEq/L it is postive and if inbetween these rates, it is highy suggestion and needs to be repeated.

Fecal Fat Test

• 24 hr collection used to test fats and indications.
• Need bile from galbladder from liver and enzymes to absorb them.
• Use normal meal of eating around 100 mg of fat of about 3 day period.
• No substances or inteferring products such as laxatives and need a less than 7 g of fat per day.

Tryspin Test

• Young children who may use fibrotic test most of time to scren them but do not diagnose it.
• When pancrease function decreased, the tryprin enyme is decreased and the emulisfied stool is placed to see if trypsin occur.

Medical Management for Cystic Fibrosis

• Therapy for respiration that includes the mucoltics, dilators, alpha medications and chest physical therapy and oxygen administration.
• Antibiotics Based: based on Sputum results.
• Nutrition: enzyme supplements, high caloric, protein and moderate fat diet.
• Lung Transplant is the final option.

Nursing Diagnoses for Cystic Fibrosis

• Ineffective airway.
• Risk of infections from mucus and alterations in nutrition.

Nursing Implementation for Cystic Fibrosis

• Administer medication and implement cautios administration of oxygen. Also promote fluids.
• Encourage physical activity such as enzymes and supplements.