4 - Congenital Respiratory Disorders

choanal atresia

blockage/narrowing of one or both nares

choanal atresia

• diagnosis

• inability to pass NG tube or suction catheter

• CT scan

• nasal endoscopy

choanal atresia

• manifestations

• respiratory distress

• stridor

• increased WOB

• paradoxical cyanosis

• ear infection

• nasal discharge in obstructive area

• OSA

• cor pulmonale and/or PHTN

• craniofacial abnormalities

choanal atresia

• management

• transnasal endoscopy and puncture

• transpalatal resection

• trans-septal

• sublabial

mandibular hypoplasia

small jaw causing tongue to fall back in mouth

mandibular hypoplasia

• associated conditions

• Pierre Robin sequence

• Treacher Collins

• Stickler syndrome

mandibular hypoplasia

• manifestations

• stridor

• OSA

• cyanosis that worsens when supine

mandibular hypoplasia

• RT role

• prone position

• nasal trumpet/CPAP

• prep for surgery

• family education

macroglossia

large tongue

macroglossia

• causes

• Beckwith-Wiedemann

• Down syndrome

• congenital hypothyroidism

macroglossia

• RT role

• prone or side position

• suction

• CPAP

• sedation cautiously

• airway action plan

tracheoesophageal fistula and esophageal atresia

trachea and esophagus are improperly connected

tracheoesophageal fistula and esophageal atresia

• manifestations

• drooling

• choking

• coughing with feeding

• air in stomach

• cyanosis

tracheoesophageal fistula and esophageal atresia

• diagnosis

• NG tube unable to pass

• CXR shows coiling of trachea and esophagus

tracheoesophageal fistula and esophageal atresia

• RT role

• avoid bag mask

• suction pouch

• O₂

• post-op ventilation

congenital diaphragmatic hernia (CDH)

herniation of abdominal organs into thoracic cavity

• most common: left side (Brochdalek)

congenital diaphragmatic hernia (CDH)

• pathophysiology

lungs and heart displaced → pulmonary hypoplasia and PHTN

congenital diaphragmatic hernia (CDH)

• manifestations

• respiratory distress

• scaphoid abdomen

• barrel chest

• decreased breath sounds

congenital diaphragmatic hernia (CDH)

• diagnosis

• prenatal ultrasound

• CXR: bowel gas in thorax, heart shift

congenital diaphragmatic hernia (CDH)

• RT role

• avoid bag mask

• MV stat

• low PIP with permissive hypercapnia

• HFOV if needed

• monitor for PPHN (iNO)

• ECMO if severe hypoxemia present

surfactant protein deficiency

genetic disorder of proteins SP-B, SP-C, and ABCA3 causing surfactant deficiency

surfactant protein deficiency

• manifestations

• term infant with distress similar to RDS

• poor response to surfactant therapy

• progressive hypoxemia

• tachypnea

surfactant protein deficiency

• diagnosis

• exclusion: genetic testing, lung biopsy

• CXR: diffuse infiltrates, white out

surfactant protein deficiency

• RT role

• lung protective ventilation (ie, PC-AC)

• HFOV if severe

• surfactant trial

• lung transplant in severe cases

• prevent infection

• nutrition support

• corticosteroids if inflammation present

pulmonary hypoplasia and agenesis

underdeveloped or absent lung or respiratory parts

pulmonary hypoplasia and agenesis

• etiology

• CDH

• oligohydramnios

• renal anomalies

• skeletal dysplasia

pulmonary hypoplasia and agenesis

• manifestations

• severe distress

• cyanosis

• chest asymmetry

• refractory hypoxemia

pulmonary hypoplasia and agenesis

• diagnosis

• CXR/CT

• echocardiogram

pulmonary hypoplasia and agenesis

• RT role

• gentle ventilation

• avoid overdistention

• avoid high pressures

• O₂