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Paeds Topicsfetal-neonatal-and-perinatal

Paeds · fetal-neonatal-and-perinatal

Respiratory distress syndrome of prematurity

Also known as Hyaline membrane disease · Surfactant deficiency disease · Neonatal respiratory distress syndrome · RDS of prematurity

Fellowship guide to respiratory distress syndrome of prematurity: surfactant deficiency as the central mechanism, the gestational-age incidence ladder, clinical and radiographic features, the differential from transient tachypnoea and sepsis, and the full management bundle of antenatal corticosteroids, early nasal CPAP, surfactant replacement, oxygen targeting, mechanical ventilation and caffeine.

high12 referencesUpdated 12 July 202619 min readVerification in progress

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Target exams

RACP General PaediatricsRACP DWERACP DCEMRCPCH TheoryMRCPCH ClinicalABP General PediatricsRCPSC Pediatrics

Red flags

  • Rising inspired oxygen requirement on nasal CPAP beyond the first hour in a preterm infant
  • Sudden deterioration with asymmetrical breath sounds suggesting a pneumothorax
  • Persistent metabolic acidosis or poor perfusion despite respiratory support
  • Pink frothy secretions from the airway suggesting a pulmonary haemorrhage
  • Preterm infant who fails CPAP and needs intubation for rising FiO2 over 0.30
  • Differential cyanosis or cyanosis unresponsive to oxygen suggesting duct-dependent congenital heart disease

Life stages

fetalneonate

Care settings

delivery-roomnicuretrievalrural-remote

Clinical exam formats

written-onlyracp-dce-short-casemrcpch-communicationrcpsc-structured-oral

Board mappings

  • Surfactant deficiency as the mechanism of neonatal respiratory distress
  • Recognition and initial management of the preterm infant with respiratory distress
  • Surfactant replacement therapy, CPAP and mechanical ventilation in RDS
  • Prevention and recognition of bronchopulmonary dysplasia and complications of prematurity
  • Antenatal corticosteroids and oxygen targeting in the preterm
  • Surfactant doses, thresholds and the respiratory support ladder
  • Long case: extremely preterm graduate with bronchopulmonary dysplasia
  • Communication: counselling parents before an anticipated very preterm delivery
  • Neonatology: respiratory distress syndrome of prematurity
  • Respiratory support, surfactant and oxygen targeting in the newborn
  • Fetal and neonatal lung physiology and surfactant
  • Pharmacology: surfactant preparations and caffeine citrate
  • Neonatal respiratory distress scenario station
  • Communication: antenatal counselling and breaking news of prematurity
  • Neonatal respiratory distress: diagnosis and management
  • Surfactant replacement therapy and neonatal respiratory support
  • Patient Care: respiratory support of the preterm infant
  • Medical Knowledge: surfactant physiology and oxygen targeting
  • Systems-Based Practice: regional neonatal retrieval networks
  • Medical Expert: respiratory distress syndrome of prematurity
  • Collaborator: multidisciplinary neonatal team
  • Health Advocate: regionalisation of perinatal care
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Practise this topic

  • Short-answer question1
  • Viva station1
  • Clinical case1

Target exams

RACP General PaediatricsRACP DWERACP DCEMRCPCH TheoryMRCPCH ClinicalABP General PediatricsRCPSC Pediatrics

Red flags

  • Rising inspired oxygen requirement on nasal CPAP beyond the first hour in a preterm infant
  • Sudden deterioration with asymmetrical breath sounds suggesting a pneumothorax
  • Persistent metabolic acidosis or poor perfusion despite respiratory support
  • Pink frothy secretions from the airway suggesting a pulmonary haemorrhage
  • Preterm infant who fails CPAP and needs intubation for rising FiO2 over 0.30
  • Differential cyanosis or cyanosis unresponsive to oxygen suggesting duct-dependent congenital heart disease

Life stages

fetalneonate

Care settings

delivery-roomnicuretrievalrural-remote

Clinical exam formats

written-onlyracp-dce-short-casemrcpch-communicationrcpsc-structured-oral

Board mappings

  • Surfactant deficiency as the mechanism of neonatal respiratory distress
  • Recognition and initial management of the preterm infant with respiratory distress
  • Surfactant replacement therapy, CPAP and mechanical ventilation in RDS
  • Prevention and recognition of bronchopulmonary dysplasia and complications of prematurity
  • Antenatal corticosteroids and oxygen targeting in the preterm
  • Surfactant doses, thresholds and the respiratory support ladder
  • Long case: extremely preterm graduate with bronchopulmonary dysplasia
  • Communication: counselling parents before an anticipated very preterm delivery
  • Neonatology: respiratory distress syndrome of prematurity
  • Respiratory support, surfactant and oxygen targeting in the newborn
  • Fetal and neonatal lung physiology and surfactant
  • Pharmacology: surfactant preparations and caffeine citrate
  • Neonatal respiratory distress scenario station
  • Communication: antenatal counselling and breaking news of prematurity
  • Neonatal respiratory distress: diagnosis and management
  • Surfactant replacement therapy and neonatal respiratory support
  • Patient Care: respiratory support of the preterm infant
  • Medical Knowledge: surfactant physiology and oxygen targeting
  • Systems-Based Practice: regional neonatal retrieval networks
  • Medical Expert: respiratory distress syndrome of prematurity
  • Collaborator: multidisciplinary neonatal team
  • Health Advocate: regionalisation of perinatal care
The fellowship answer

Respiratory distress syndrome of prematurity is the lung disease caused by surfactant deficiency, and surfactant is the lever that moves the whole problem. The immature type II pneumocyte cannot keep alveoli open, the lung collapses at end-expiration, and the infant sinks into hypoxaemia, hypercapnia and acidosis within the first hours of life. Prevent it where you can with antenatal corticosteroids, then treat it early with warmth, early nasal CPAP, surfactant when the inspired oxygen climbs past 0.30, mechanical ventilation only when CPAP fails, and caffeine to protect the drive and aid extubation. Target saturations at 91 to 95 percent beyond the first minutes, because both lower and higher targets do harm. [1] [5]

Overview & Definition

A 27-week infant is grunting and retracting in the first hour of life, the inspired oxygen creeping upward. That is the opening scene of respiratory distress syndrome of prematurity, the prototypical lung disease of the preterm newborn, and one of the defining problems of neonatology. It is caused primarily by deficiency of pulmonary surfactant, the phospholipid-rich mixture secreted by the type II pneumocyte that lines alveoli and lowers surface tension at the air-liquid interface. Without enough of it, alveoli collapse at end-expiration, the lung becomes stiff and poorly aerated, and the infant works hard and fails to oxygenate. [1] [3]

The names carry the history. "Hyaline membrane disease" describes the eosinophilic alveolar lining seen at autopsy in the era before surfactant therapy. "Surfactant deficiency disease" names the mechanism. The modern term, respiratory distress syndrome of prematurity, keeps the mechanism central while signalling that the syndrome is graded, modifiable, and largely preventable. [3]

~60%Under 28 weeksdevelop RDS
~30%28–31 weeksdevelop RDS
<5%32–36 weeksdevelop RDS
rareTermthink TTN or sepsis

Two facts shape every decision. First, severity and incidence scale inversely with gestational age: the smaller and earlier the infant, the more likely and worse disease. Second, antenatal corticosteroids given to the mother are the single most powerful modifier, reducing both incidence and severity. Hold those two together and you have the strategic frame for the whole topic. [1] [4]

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References12ShowHide
  1. [1]Sweet DG European Consensus Guidelines on the Management of Respiratory Distress Syndrome: 2022 Update. Neonatology, 2023.PMID 36863329
  2. [2]Polin RA Surfactant replacement therapy for preterm and term neonates with respiratory distress. Pediatrics, 2014.PMID 24379227
  3. [3]Jobe AH Pulmonary surfactant therapy. N Engl J Med, 1993.PMID 8441430
  4. [4]Roberts D Antenatal corticosteroids for accelerating fetal lung maturation for women at risk of preterm birth. Cochrane Database Syst Rev, 2006.PMID 16856047
  5. [5]Askie LM Association Between Oxygen Saturation Targeting and Death or Disability in Extremely Preterm Infants in the Neonatal Oxygenation Prospective Meta-analysis Collaboration. JAMA, 2018.PMID 29872859
  6. [6]Carlo WA Target ranges of oxygen saturation in extremely preterm infants. N Engl J Med, 2010.PMID 20472937
  7. [7]Morley CJ Nasal CPAP or intubation at birth for very preterm infants. N Engl J Med, 2008.PMID 18272893
  8. [8]Schmidt B Long-term effects of caffeine therapy for apnea of prematurity. N Engl J Med, 2007.PMID 17989382
  9. [9]Stenson BJ Oxygen saturation and outcomes in preterm infants. N Engl J Med, 2013.PMID 23642047
  10. [10]Verder H Surfactant therapy and nasal continuous positive airway pressure for newborns with respiratory distress syndrome. N Engl J Med, 1994.PMID 8090164
  11. [11]Jobe AH Bronchopulmonary dysplasia. Am J Respir Crit Care Med, 2001.PMID 11401896
  12. [12]Finer NN Early CPAP versus surfactant in extremely preterm infants. N Engl J Med, 2010.PMID 20472939

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