II. Epidemiology

  1. Most common cause of premature Newborn Respiratory Distress
  2. Incidence: Affects 24,000 infants annually in the United States
  3. Prevalence varies by Gestational age
    1. Gestational age <28 weeks: Affects a majority of newborns
    2. Gestational age 28-34 weeks: Affects <33% of newborns
    3. Gestational age >34 weeks: Affects <5% of newborns

III. Pathophysiology

  1. Inadequate pulmonary surfactant (normally lowers alveolar surface tension)
  2. Leads to Atelectasis (increased alveolar surface tension, decreased compliance)
  3. Pulmonary vasculature responds with Vasoconstriction
  4. Lung hypoperfusion results in lung tissue ischemia
  5. Hyaline membranes form from epithelial cell destruction and infiltration of fluid and protein

IV. Risk factors

  1. Immature lung development at delivery
    1. Premature Infant (see Prevalence based on Gestational age above)
    2. Maternal Diabetes Mellitus (confers 6 fold increased risk)
    3. White race
    4. Family History of RDS in siblings
    5. Male gender
  2. Inadequate surfactant
    1. Cesarean birth without labor
    2. Premature Infant with perinatal asphyxia
      1. Antepartum Hemorrhage
        1. Abruptio Placentae
        2. Placenta Previa
      2. Second born Twin Gestation

V. Signs: Onset shortly after birth (and progressive over first 12-24 hours)

  1. Tachypnea
  2. Nasal flaring
  3. Grunting
  4. Central Cyanosis
  5. Intercostal muscle retractions
  6. Hypoxia

VI. Labs

  1. Antepartum Assessment
    1. Fetal Lung Maturity Assessment
  2. Newborn Assessment
    1. Blood Gas
      1. Hypoxemia
      2. Respiratory Acidosis
    2. Swallowed Amniotic fluid Shake Test (historical use)

VII. Imaging: Chest XRay

  1. Hypoinflated lungs
  2. Homogenous opaque infiltrates (Reticulogranular pattern, "ground glass" appearance)
  3. Air Bronchograms (contrast of air-filled Bronchi against airless lung tissue)

VIII. Management: General

  1. See Respiratory Distress in the Newborn
  2. See Newborn Resuscitation
  3. Artificial Surfactant Replacement (Exosurf)
    1. Surfactant delivery via Endotracheal Tube (200 mg/kg for initial dose)
    2. Followed by weaning Endotracheal Tube to N-CPAP
  4. Oxygen Delivery
  5. Body Temperature control
  6. Adequate hydration and nutrition
  7. Neonatal Sepsis Evaluation
    1. Administer antibiotics for 48 hours

IX. Management: Continuous Positive Pressure Airway Pressure

  1. Indications
    1. FIO2 0.3 - 0.5 required to maintain PaO2 50-80 mmHg
  2. Delivery device
    1. No abdominal distention
      1. Nasal canula (N-CPAP)
      2. Face mask
    2. Abdominal distention from hyperinflation
      1. Nasopharyngeal tube
      2. Endotracheal Tube
  3. Technique
    1. Starting Pressure: 5-7 cm H2O
    2. Titrate pressure by 1-2 cm H2O to PaO2 and effort
    3. Maintain Adequate Flow: 5-10 L/min
  4. Weaning
    1. Reduce FIO2 by 0.05 steps until FIO2 <0.40
    2. Reduce CPAP by 1-2 cm H2O (follow Arterial Blood Gas)
    3. Discontinue CPAP when pressure 4-6 cm H2O

X. Management: Mechanical Ventilation

  1. Indications
    1. PaCO2 >55mmHg
    2. PaO2 <50 mmHg despite FIO2 >0.6
  2. Initial Respirator Settings
    1. Continuous-flow
    2. Pressure-limited
      1. Identify pressure settings with Anesthesia Bag
        1. Use manometer to measure pressures required
      2. Peak inspiratory pressure: 20-25 cm H2O
      3. Positive End-Expiratory Pressure (PEEP): 4-6 cm H2O
    3. Time-cycled
      1. Respiratory frequency: 20-30 breaths per minute
      2. Inspiratory duration: 0.4 to 0.6 second
    4. FIO2: 0.5 to 1.0
  3. Maintenance
    1. Maintain PaCO2: 45-60 mmHg
    2. Follow Arterial Blood Gas
      1. Every 4 to 6 hours
      2. Following every respirator setting change by 15 min
  4. Weaning
    1. Lower inspiratory pressure by 2 cm H2O steps to 30
    2. Lower FIO2 by steps of 0.05 to 0.5-0.6
    3. Lower inspiratory pressure by 1-2 cm H2O steps to 20
    4. Lower PEEP to 5 cm H2O
    5. Slowly decrease FIO2 to 0.40 in steps
    6. Lower respirator rate by 2-4 bpm steps to >8 bpm

XI. Prevention

  1. Prenatal Corticosteroids at 24-34 weeks gestation (up to 2 courses of steroid)
    1. Reduces Respiratory Distress Syndrome risk (NNT 11)
    2. Effective for 7 days if given >24 hours before delivery

XII. Complications: Short-term

XIII. Complications: Long-term

  1. Bronchopulmonary Dysplasia (5-10%)
  2. Recurrent Wheezing through childhood and more complicated Asthma Exacerbations
  3. Retinopathy of Prematurity
  4. Neurologic Impairment

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Ontology: Hyaline Membrane Disease (C0020192)

Definition (MSH) A respiratory distress syndrome in newborn infants, usually premature infants with insufficient PULMONARY SURFACTANTS. The disease is characterized by the formation of a HYALINE-like membrane lining the terminal respiratory airspaces (PULMONARY ALVEOLI) and subsequent collapse of the lung (PULMONARY ATELECTASIS).
Concepts Disease or Syndrome (T047)
MSH D006819
ICD10 P22.0
SnomedCT 26168007, 206281003, 46775006
English Disease, Hyaline Membrane, Diseases, Hyaline Membrane, Hyaline Membrane Disease, Hyaline Membrane Diseases, HYALINE MEMBRANE DIS, Hyaline Membrane Disease [Disease/Finding], distress newborns respiratory, respiratory distress newborn, distress newborn respiratory, hyaline membrane disease, newborn rds, newborn respiratory distress, newborns rds, respiratory distress of newborn, Hyaline membrane disease (disorder), Hyaline membrane disease, HMD - Hyaline membrane disease, Pulmonary hyaline membrane disease
Dutch hyaliene membraanziekte, Hyaliene-membranenziekte, Ziekte, hyaliene-membranen-
German Hyalinmembrankrankheit, Hyaline-Membranen-Krankheit, Surfactantmangelsyndrom
Portuguese Doença de membrana hialina, Doença da Membrana Hialina
Spanish Enfermedad de la membrana hialina, enfermedad de la membrana hialina, enfermedad de la membrana hialina (concepto no activo), Enfermedad de la Membrana Hialina
Japanese 肺硝子膜症, ハイショウシマクショウ
Swedish Hyalinmembransjukdom
Czech nemoc hyalinních membrán, Syndrom hyalinních blanek
Finnish Hyaliinimembraanisairaus
Russian PNEVMONIIA ASFIKTICHESKAIA, GIALINOVYKH MEMBRAN BOLEZN', GIALINOVO-MEMBRANNAIA BOLEZN' NOVOROZHDENNYKH, ГИАЛИНОВО-МЕМБРАННАЯ БОЛЕЗНЬ НОВОРОЖДЕННЫХ, ГИАЛИНОВЫХ МЕМБРАН БОЛЕЗНЬ, ПНЕВМОНИЯ АСФИКТИЧЕСКАЯ
Croatian HIJALINA MEMBRANA, BOLEST
Polish Zespół błon szklistych, Choroba błon szklistych noworodków, Zespół błon hialinowych
Hungarian Hyalinmembrán-betegség
Norwegian Surfaktantmangelsyndrom, Hyalin membran-sykdom, Hyalinmembransykdom
French Maladie des membranes hyalines
Italian Malattia delle membrane ialine

Ontology: Respiratory Distress Syndrome, Newborn (C0035220)

Definition (NCI_FDA) A condition of the newborn marked by dyspnea with cyanosis, most frequently occurring in premature infants, children of diabetic mothers and infants delivered by cesarean section, and sometimes with no predisposing cause.
Definition (NCI) A condition of the newborn marked by dyspnea with cyanosis, most frequently occurring in premature infants, children of diabetic mothers and infants delivered by cesarean section, and sometimes with no predisposing cause.
Definition (MSH) A condition of the newborn marked by DYSPNEA with CYANOSIS, heralded by such prodromal signs as dilatation of the alae nasi, expiratory grunt, and retraction of the suprasternal notch or costal margins, mostly frequently occurring in premature infants, children of diabetic mothers, and infants delivered by cesarean section, and sometimes with no apparent predisposing cause.
Definition (CSP) a condition of the newborn marked by dyspnea with cyanosis, heralded by such prodromal signs as dilatation of the alae nasi, expiratory grunt, and retraction of the suprasternal notch or costal margins, most frequently occurring in premature infants, children of diabetic mothers, and infants delivered by cesarean section, and sometimes with no apparent predisposing cause.
Concepts Disease or Syndrome (T047)
MSH D012127
ICD9 769
ICD10 P22.0 , P22, P22.9
SnomedCT 46775006, 157107007, 206281003, 3774009
English Respiratory distress syndrome in newborn, RESP DISTRESS SYNDROME NEONATAL, IRDS - Idiopath resp dist synd, Pulmonary hypoperf.syn.newborn, Resp distress synd of newborn, Respiratory distress of newborn, unspecified, RDS-Resp distress synd newborn, idiopathic respiratory distress of newborn, respiratory distress syndrome of newborn (diagnosis), respiratory distress syndrome of newborn, Surfactant deficiency syndrome neonatal, Infant respiratory distress syndrome, Resp distress syndrome neonatal, Respiratory distress syndrome (neonatal), Distress respiratory syndrome newborn, Respiratory distress syndrome newborn, Syndrome respiratory distress newborn, Respiratory distress syn, Neonatal Respiratory Distress Syndrome, Infantile Respiratory Distress Syndrome, Respiratory Distress Syndrome, Infant, Newborn Respiratory Distress Syndrome, Idiopathic respiratory distress syndrome [IRDS or RDS] of newborn, Respiratory distress of newborn, Respiratory Distress Syndrome, Newborn [Disease/Finding], distress respiratory syndrome, distress respiratory syndromes, newborn rds, newborns rds, respiratory syndrome distress, respiratory distress syndrome, Respiratory distress syndrome in neonate, respiratory distress newborn (diagnosis), respiratory distress newborn, Respiratory distress syndrome of newborn (disorder), Idiopathic respiratory distress syndrome (disorder), infant respiratory distress syndrome, SYNDROME OF NEWBORNS (RDS), RESPIRATORY DISTRESS, NEWBORNS (RDS), RESPIRATORY DISTRESS SYNDROME OF, RDS, RESPIRATORY DISTRESS SYNDROME OF NEWBORNS, Respiratory Distress Syndrome in the Newborn, RDS OF NEWBORNS, RESPIRATORY DISTRESS SYNDROME OF NEWBORNS, RESPIRATORY DISTRESS SYNDROME OF NEWBORNS (RDS), Respiratory Distress Syndrome, Newborn, Idiopathic respiratory distress syndrome of newborn, Cardiorespiratory distress syndrome of newborn, Congenital alveolar dysplasia, IRDS of newborn, Pulmonary hypoperfusion syndrome of newborn, RDS of newborn, Respiratory distress syndrome in the newborn, Wet lung disease of newborn, IRDS - Idiopathic respiratory distress syndrome, Respiratory distress syndrome of newborn, Neonatal respiratory distress syndrome, RDS - Respiratory distress syndrome of newborn, Idiopathic respiratory distress syndrome, Respiratory distress syndrome in the newborn (disorder), distress; respiratory, newborn, distress; respiratory, syndrome (idiopathic) (newborn), respiratory; distress, syndrome (idiopathic) (newborn), Idiopathic respiratory distress syndrome, NOS
Italian Sindrome da distress respiratorio del neonato, Sindrome da distress respiratorio neonatale, Sindrome da sofferenza respiratoria (neonatale), Sindrome da sofferenza respiratoria nel neonato, Sindrome da sofferenza respiratoria neonatale, Sindrome da deficit di fattore surfactante neonatale, Sindrome da distress respiratorio
Dutch "respiratory-distress syndrome" pasgeborene, respiratory distress syndrome pasgeborene, respiratory-distress syndrome bij pasgeborene, verminderd surfactant syndroom neonataal, "respiratory-distress syndrome" zuigeling, respiratory-distress syndrome (neonataal), "respiratory-distress syndrome" bij pasgeborene, "respiratory-distress syndrome" (neonataal), distress; respiratoir, pasgeborene, distress; respiratoir, syndroom (idiopathisch) (pasgeborene), respiratoir; distress, syndroom (idiopathisch) (pasgeborene), 'Respiratory distress syndrome' van pasgeborene, Respiratoire nood van pasgeborene, niet gespecificeerd, neonataal "respiratory distress syndrome", Respiratoire nood van pasgeborene, Respiratory distress syndrome, Syndrome, respiratory distress, Distress, respiratory, syndrome, Hyaliene-membraanziekte, Infantiele 'respiratory distress syndrome', Syndrome, infantiele respiratory distress
French Syndrome de carence en surfactants du nouveau-né, Syndrome de détresse respiratoire chez le nouveau-né, Syndrome de détresse resp. du nouveau-né, SYNDROME SOUFFR RESP NOUVEAU-NE, SDRN, Syndrome de détresse respiratoire des nouveau-nés, Syndrome de détresse respiratoire du nouveau-né, Syndrome de détresse respiratoire néonatale
German resp Distress-Syndrom beim Neugeborenen, respiratorisches Distress-Syndrom beim Neugeborenen, Distress-Syndrom respiratorisch beim Saeugling, Distress-Atemwegsyndrom beim Neugeborenen, Surfactantmangelsyndrom beim Neugeborenen, respiratorisches Distress-Syndrom (Neugeborenes), Syndrom, respiratorischer Distress beim Neugeborenen, ATEMNOTSYNDROM NEUGEBORENES, Atemnot [Respiratory distress] beim Neugeborenen, nicht naeher bezeichnet, Atemnot [Respiratory distress] beim Neugeborenen, Atemnotsyndrom [Respiratory distress syndrome] des Neugeborenen, Atemnotsyndrom Neugeborenes, Respiratory-Distress-Syndrom, Atemnotsyndrom des Neugeborenen
Portuguese Síndrome de dificuldade respiratória neonatal, Síndrome de dificuldade respiratória do recém-nascido, Sindrome de dificuldade respiratória do bebé, Síndrome de carência de surfactante neonatal, Síndrome de dificuldade respiratória (neonatal), SINDROME DE DIFICULDADE RESPIRATO, Síndrome da Angústia Respiratória do Recém-Nascido, Síndrome do Desconforto Respiratório em Recém-Nascidos, Síndrome de sofrimento respiratório neonatal, Síndrome do Desconforto Respiratório do Recém-Nascido
Spanish Síndrome de distrés respiratorio infantil, Síndrome de distrés respiratorio en el recién nacido, Síndrome de déficit de surfactante neonatal, Síndrome de distrés respiratorio (neonatal), Síndrome neonatal de distrés respiratorio, DISTRESS RESPIRATORIO DEL RECIEN, síndrome de distrés respiratorio neonatal, Síndrome de sufrimiento respiratorio neonatal, Síndrome de distrés respiratorio del recién nacido, síndrome disneico idiopático, síndrome disneico idiopático (concepto no activo), Síndrome de Dificultad Respiratoria del Recién Nacido, SDR del recién nacido, SDRI del recién nacido, displasia alveolar congénita, enfermedad del pulmón húmedo del recién nacido, síndrome de dificultad cardiorrespiratoria en el recién nacido, síndrome de dificultad respiratoria en el recién nacido (trastorno), síndrome de dificultad respiratoria en el recién nacido, síndrome de dificultad respiratoria idiopática en el recién nacido, síndrome de distrés respiratorio en el recién nacido, síndrome de hipoperfusión pulmonar en el recién nacido, Síndrome Disneico Respiratorio del Recién Nacido
Japanese 呼吸窮迫症候群(新生児), 新生児サーファクタント欠乏症候群, コキュウキュウハクショウコウグンシンセイジ, シンセイジコキュウキュウハクショウコウグン, シンセイジサーファクタントケツボウショウコウグン, 呼吸切迫症候群-新生児, 呼吸促迫症候群-新生児, 呼吸困難症候群-新生児, 呼吸窮迫症候群-新生児, 新生児呼吸窮迫症候群, 新生児呼吸困難症候群, 新生児呼吸障害症候群, 特発性呼吸窮迫症候群
Swedish Andnödssyndrom hos nyfödda
Czech syndrom dechové nedostatečnosti, syndrom respirační tísně novorozenců, respirační distres syndrom, Syndrom dechové tísně novorozence, Syndrom nedostatku surfaktantu u novorozence
Finnish Vastasyntyneen hengitysvaikeusoireyhtymä
Russian ДЫХАТЕЛЬНЫХ РАССТРОЙСТВ СИНДРОМ НОВОРОЖДЕННЫХ, RESPIRATORNYI DISTRESS-SINDROM NOVOROZHDENNYKH, РЕСПИРАТОРНЫЙ ДИСТРЕСС-СИНДРОМ НОВОРОЖДЕННЫХ, DYKHATEL'NYKH RASSTROISTV SINDROM NOVOROZHDENNYKH, RESPIRATORNYI DISTRESS-SINDROM, РЕСПИРАТОРНЫЙ ДИСТРЕСС-СИНДРОМ
Korean 상세불명의 신생아의 호흡 곤란, 신생아의 호흡 곤란, 신생아의 호흡 곤란 증후군
Croatian Not Translated[Respiratory Distress Syndrome, Newborn]
Polish Zaburzenia oddychania u noworodka, Zespół zaburzeń oddychania noworodków, Zespół uszkodzenia układu oddechowego płodu, Zaburzenia oddechowe noworodka
Hungarian Neonatalis respiratorikus distress syndroma, Újszülöttkori surfactant-hiány syndroma, Újszülöttkori respiratorikus distress syndroma, Újszülöttkori légzési distress syndroma, Újszülött respiratorikus distress syndroma, Légzési distress syndroma az újszülöttben, Légzési distress syndroma (újszülöttkori), Csecsemő respiratorikus distress syndromája, Újszülöttek respiratorikus distress syndromája
Norwegian Neonatalt åndenødssyndrom, Åndenødssyndrom hos nyfødte, Akutt lungesviktsyndrom hos nyfødte