Respiratory distress syndrome

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Respiratory distress syndrome (RDS) is a relatively common condition that occurs in preterm neonates resulting from insufficient production of surfactant ~~that occurs in preterm neonates~~.

~~On imaging, the condition generally presents as bilateral and relatively symmetric diffuse ground glass lungs with low volumes and a bell-shaped thorax.~~

Terminology

RDS is also known as hyaline membrane disease (not favoured as reflects non-specific histological findings), neonatal respiratory distress syndrome, lung disease of prematurity (both non-specific terms),or ~~as some authors prefer~~ surfactant~~-deficiency~~ deficiency disorder ².

Epidemiology

The incidence is estimated at 6 per 1000 births ². Uncommon after 36 weeks' gestation due to development of pneumocyte surfactant production around 35 weeks ⁵.

Clinical presentation

Respiratory distress presents in the first few hours of life in a premature baby. Signs include tachypnoea, expiratory grunting, and nasal flaring. The infant may or may not be cyanosed. Substernal and intercostal retractions may be evident.

Risk factors include maternal diabetes, greater prematurity, perinatal asphyxia, and multiple gestations.

Associated ~~abnormalities~~conditions are those that can occur in prematurity: germinal matrix haemorrhage, necrotising enterocolitis, patent ductus arteriosus, delayed developmental milestones, hypothermia, and hypoglycaemia.

Pathology

Immature type II pneumocytes cannot produce surfactant. The lack of surfactant increases the surface tension in alveoli causing them to collapse. Patients have a decreased lecithin to sphingomyelin ratio. Damaged cells, necrotic cells, and mucus line the alveoli.

Although most cases are related to prematurity alone, rarely patients may have genetic disorders of surfactant production and can present in a similar clinical and radiological manner ⁶.

As the alveoli are collapsed (microscopically), the lungs are collapsed macroscopically as well. It is a diffuse type of adhesive atelectasis.

Radiographic features

Plain radiograph

low lung volumes ~~are mandatory (atelectasis - lung collapse).~~
diffuse ~~granular opacities (ground glass)~~, bilateral and symmetrical ~~- "white lungs".~~granular opacities
bell-shaped thorax
air bronchograms may be evident.
~~bell-shaped thorax.~~
~~hyperinflation (in a non-ventilated patient) excludes~~

Hyperinflation makes the diagnosis~~; if~~ less likely, unless the patient is intubated ~~there might be hyperinflation~~.

~~RDS can be safely excluded if the neonate has a normal chest radiograph at six hours after birth.~~

If treated with surfactant therapy, there may be an asymmetric improvement as more surfactant may reach certain parts of the lungs than others.

Ultrasound

On transabdominal ultrasound, retrodiaphragmatic hyperechogenicity can be seen. If this hyperechogenicity does not resolve by day 9-18 on follow up ultrasound, it helps in the prediction of risk of development of bronchopulmonary dysplasia ^ref.

Treatment and prognosis

Exogenous surfactant administration is an effective treatment, traditionally administered via endotracheal tube, though less invasive methods of surfactant administration such as via laryngeal mask airway are becoming more common ⁷. Supportive oxygen therapy is typically required for a period of time.

Complications

Acute

persistent patent ductus arteriosus (PDA) due to reduced oxygen stimulus
pulmonary interstitial emphysema ~~(from treatment~~ or air leak (secondary to requirement for mechanical ventilation)
oxygen toxicity (from treatment)
pulmonary haemorrhage ~~(can also be included in the differential diagnosis)~~
~~air leak (barotrauma related to ventilation)~~

Chronic

bronchopulmonary dysplasia
recurrent pulmonary infection
subglottic stenosis ~~(from~~(secondary to intubation)

Differential diagnosis

Consider:

congenital heart disease
neonatal pneumonia (particularly group B ~~Streptococcal pneumonia~~Streptococcus)
pulmonary haemorrhage
pulmonary oedema / pulmonary venous congestion
neonatal pneumonia
transient tachypnea of the newborn: lung volumes are normal to slightly hyperinflated in TTN and decreased in RDS

-<p><strong>Respiratory distress syndrome</strong> (<strong>RDS</strong>) is a relatively common condition resulting from insufficient production of surfactant that occurs in preterm neonates. </p><p>On imaging, the condition generally presents as bilateral and relatively symmetric diffuse ground glass lungs with low volumes and a bell-shaped thorax.</p><h4>Terminology</h4><p>RDS is also known as <strong>hyaline membrane disease</strong> (not favoured as reflects non-specific histological findings), <strong>neonatal</strong> <strong>respiratory distress syndrome</strong>, <strong>lung disease of prematurity </strong>(both non-specific terms),<strong> </strong>or as some authors prefer <strong>surfactant-deficiency disorder </strong><sup>2</sup>. </p><h4>Epidemiology</h4><p>The incidence is estimated at 6 per 1000 births <sup>2</sup>. Uncommon after 36 weeks' gestation due to development of pneumocyte surfactant production around 35 weeks <sup>5</sup>.</p><h4>Clinical presentation</h4><p>Respiratory distress presents in the first few hours of life in a premature baby. Signs include tachypnoea, expiratory grunting, and nasal flaring. The infant may or may not be cyanosed. Substernal and intercostal retractions may be evident. </p><p>Risk factors include <a href="/articles/fetal-conditions-associated-with-maternal-diabetes">maternal diabetes</a>, greater prematurity, perinatal asphyxia and multiple gestations.</p><p>Associated abnormalities are those that can occur in prematurity: <a href="/articles/germinal-matrix-haemorrhage">germinal matrix haemorrhage</a>, <a href="/articles/necrotising-enterocolitis-1">necrotising enterocolitis</a>, <a href="/articles/patent-ductus-arteriosus">patent ductus arteriosus</a>, delayed developmental milestones, hypothermia and hypoglycaemia.</p><h4>Pathology</h4><p>Immature type II pneumocytes cannot produce <a href="/articles/surfactant">surfactant</a>. The lack of surfactant increases the surface tension in alveoli causing collapse. Patients have a decreased lecithin to sphingomyelin ratio. Damaged cells, necrotic cells, and mucus line the alveoli.</p><p>Although most cases are related to prematurity alone, rarely patients may have genetic disorders of surfactant production and can present in a similar clinical and radiological manner <sup>6</sup>.</p><p>As the alveoli are collapsed (microscopically), the lungs are collapsed macroscopically as well. It is a diffuse type of adhesive atelectasis.</p><h4>Radiographic features</h4><h5>Plain radiograph</h5><ul>
~~-<li>low lung volumes are mandatory (atelectasis - lung collapse).</li>~~
~~-<li>diffuse granular opacities (ground glass), bilateral and symmetrical - "white lungs".</li>~~
~~-<li>air bronchograms may be evident.</li>~~
~~-<li>bell-shaped thorax.</li>~~
~~-<li>hyperinflation (in a non-ventilated patient) excludes the diagnosis; if the patient is intubated there might be hyperinflation.</li>~~
-</ul><p>RDS can be safely excluded if the neonate has a normal chest radiograph at six hours after birth. </p><p>If treated with surfactant therapy, there may be an asymmetric improvement.</p><p>On transabdominal ultrasound, retrodiaphragmatic hyperechogenicity can be seen. If this hyperechogenicity does not resolve by day 9-18 on follow up ultrasound, it helps in the prediction of risk of development of bronchopulmonary dysplasia <sup>ref</sup>.</p><h4>Treatment and prognosis</h4><p>Exogenous surfactant administration. Supportive oxygen therapy.</p><h5>Complications</h5><h6>Acute</h6><ul>
+<p><strong>Respiratory distress syndrome</strong> (<strong>RDS</strong>) is a relatively common condition that occurs in preterm neonates resulting from insufficient production of surfactant. </p><h4>Terminology</h4><p>RDS is also known as <strong>hyaline membrane disease</strong> (not favoured as reflects non-specific histological findings), <strong>neonatal</strong> <strong>respiratory distress syndrome</strong>, <strong>lung disease of prematurity </strong>(both non-specific terms),<strong> </strong>or <strong>surfactant deficiency disorder </strong><sup>2</sup>. </p><h4>Epidemiology</h4><p>The incidence is estimated at 6 per 1000 births <sup>2</sup>. Uncommon after 36 weeks' gestation due to development of pneumocyte surfactant production around 35 weeks <sup>5</sup>.</p><h4>Clinical presentation</h4><p>Respiratory distress presents in the first few hours of life in a premature baby. Signs include tachypnoea, expiratory grunting, and nasal flaring. The infant may or may not be cyanosed. Substernal and intercostal retractions may be evident. </p><p>Risk factors include <a href="/articles/fetal-conditions-associated-with-maternal-diabetes">maternal diabetes</a>, greater prematurity, perinatal asphyxia, and multiple gestations.</p><p>Associated conditions are those that can occur in prematurity: <a href="/articles/germinal-matrix-haemorrhage">germinal matrix haemorrhage</a>, <a href="/articles/necrotising-enterocolitis-1">necrotising enterocolitis</a>, <a href="/articles/patent-ductus-arteriosus">patent ductus arteriosus</a>, delayed developmental milestones, hypothermia, and hypoglycaemia.</p><h4>Pathology</h4><p>Immature type II pneumocytes cannot produce <a href="/articles/surfactant">surfactant</a>. The lack of surfactant increases the surface tension in <a title="Alveoli" href="/articles/alveoli">alveoli</a> causing them to collapse. Patients have a decreased lecithin to sphingomyelin ratio. Damaged cells, necrotic cells, and mucus line the alveoli.</p><p>Although most cases are related to prematurity alone, rarely patients may have genetic disorders of surfactant production and can present in a similar clinical and radiological manner <sup>6</sup>.</p><p>As the alveoli are collapsed (microscopically), the lungs are collapsed macroscopically as well. It is a diffuse type of <a title="Adhesive atelectasis" href="/articles/adhesive-atelectasis">adhesive atelectasis</a>.</p><h4>Radiographic features</h4><h5>Plain radiograph</h5><ul>
+<li>low lung volumes</li>
+<li>diffuse, bilateral and symmetrical granular opacities </li>
+<li>bell-shaped thorax</li>
+<li>air bronchograms may be evident</li>
+</ul><p>Hyperinflation makes the diagnosis less likely, unless the patient is intubated.</p><p>If treated with surfactant therapy, there may be an asymmetric improvement as more surfactant may reach certain parts of the lungs than others.</p><h5>Ultrasound</h5><p>On transabdominal ultrasound, retrodiaphragmatic hyperechogenicity can be seen. If this hyperechogenicity does not resolve by day 9-18 on follow up ultrasound, it helps in the prediction of risk of development of bronchopulmonary dysplasia <sup>ref</sup>.</p><h4>Treatment and prognosis</h4><p>Exogenous surfactant administration is an effective treatment, traditionally administered via endotracheal tube, though less invasive methods of surfactant administration such as via laryngeal mask airway are becoming more common <sup>7</sup>. Supportive oxygen therapy is typically required for a period of time.</p><h5>Complications</h5><h6>Acute</h6><ul>
~~-<a href="/articles/pulmonary-interstitial-emphysema">pulmonary interstitial emphysema</a> (from treatment)</li>~~
+<a href="/articles/pulmonary-interstitial-emphysema">pulmonary interstitial emphysema</a> or air leak (secondary to requirement for mechanical ventilation)</li>
~~-<a href="/articles/pulmonary-haemorrhage">pulmonary haemorrhage</a> (can also be included in the differential diagnosis)</li>~~
~~-<li>air leak (barotrauma related to ventilation)</li>~~
+<a href="/articles/pulmonary-haemorrhage">pulmonary haemorrhage</a> </li>
~~-<a href="/articles/subglottic-stenosis">subglottic stenosis</a> (from intubation)</li>~~
+<a href="/articles/subglottic-stenosis">subglottic stenosis</a> (secondary to intubation)</li>
~~-<li><a href="/articles/group-b-strep-pneumonia">group B Streptococcal pneumonia</a></li>~~
+<li>
+<a title="Neonatal pneumonia" href="/articles/neonatal-pneumonia">neonatal pneumonia</a> (particularly group B <em>Streptococcus</em>)</li>

References changed:

1. Blickman J, Parker B, Barnes P. Pediatric Radiology. (2009) ISBN: 9780323031257 - <a href="http://books.google.com/books?vid=ISBN9780323031257">Google Books</a>
2. Agrons G, Courtney S, Stocker J, Markowitz R. From the Archives of the AFIP: Lung Disease in Premature Neonates: Radiologic-Pathologic Correlation. Radiographics. 2005;25(4):1047-73. <a href="https://doi.org/10.1148/rg.254055019">doi:10.1148/rg.254055019</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/16009823">Pubmed</a>
3. Donnelly L & Frush D. Localized Radiolucent Chest Lesions in Neonates: Causes and Differentiation. AJR Am J Roentgenol. 1999;172(6):1651-8. <a href="https://doi.org/10.2214/ajr.172.6.10350310">doi:10.2214/ajr.172.6.10350310</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/10350310">Pubmed</a>
4. Swischuk L & John S. Immature Lung Problems: Can Our Nomenclature Be More Specific? AJR Am J Roentgenol. 1996;166(4):917-8. <a href="https://doi.org/10.2214/ajr.166.4.8610573">doi:10.2214/ajr.166.4.8610573</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/8610573">Pubmed</a>
5. Liszewski M & Lee E. Neonatal Lung Disorders: Pattern Recognition Approach to Diagnosis. AJR Am J Roentgenol. 2018;210(5):964-75. <a href="https://doi.org/10.2214/AJR.17.19231">doi:10.2214/AJR.17.19231</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/29489412">Pubmed</a>
6. Wert S, Whitsett J, Nogee L. Genetic Disorders of Surfactant Dysfunction. Pediatr Dev Pathol. 2009;12(4):253-74. <a href="https://doi.org/10.2350/09-01-0586.1">doi:10.2350/09-01-0586.1</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/19220077">Pubmed</a>
7. Smee N, Boyd D, Conetta H, O'Shea J. Laryngeal Mask Airway Surfactant Administration: Case Series of 60 Infants. Arch Dis Child Fetal Neonatal Ed. 2021;106(3):342. <a href="https://doi.org/10.1136/archdischild-2020-320438">doi:10.1136/archdischild-2020-320438</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/33246968">Pubmed</a>
1. Blickman JG, Parker BR, Barnes PD. Pediatric radiology, the requisites. Mosby Inc. (2009) ISBN:0323031250. <a href="http://books.google.com/books?vid=ISBN0323031250">Read it at Google Books</a> - <a href="http://www.amazon.com/gp/product/0323031250?ie=UTF8&tag=radiopaediaor-20&linkCode=as2&camp=1789&creative=9325&creativeASIN=0323031250">Find it at Amazon</a><div class="ref_v2"></div>
2. Agrons GA, Courtney SE, Stocker JT et-al. From the archives of the AFIP: Lung disease in premature neonates: radiologic-pathologic correlation. Radiographics. 25 (4): 1047-73. <a href="http://dx.doi.org/10.1148/rg.254055019">doi:10.1148/rg.254055019</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/16009823">Pubmed citation</a><div class="ref_v2"></div>
3. Donnelly LF, Frush DP. Localized radiolucent chest lesions in neonates: causes and differentiation. AJR Am J Roentgenol. 1999;172 (6): 1651-8. <a href="http://www.ajronline.org/cgi/content/citation/172/6/1651">AJR Am J Roentgenol (citation)</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/10350310">Pubmed citation</a><div class="ref_v2"></div>
4. Swischuk LE, John SD. Immature lung problems: can our nomenclature be more specific? AJR Am J Roentgenol. 1996;166 (4): 917-8. <a href="http://www.ajronline.org/cgi/content/abstract/166/4/917">AJR Am J Roentgenol (abstract)</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/8610573">Pubmed citation</a><div class="ref_v2"></div>
5. Liszewski MC, Lee EY. Neonatal Lung Disorders: Pattern Recognition Approach to Diagnosis. (2018) AJR. American journal of roentgenology. 210 (5): 964-975. <a href="https://doi.org/10.2214/AJR.17.19231">doi:10.2214/AJR.17.19231</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/29489412">Pubmed</a> <span class="ref_v4"></span>
6. Wert SE, Whitsett JA, Nogee LM. Genetic disorders of surfactant dysfunction. (2009) Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society. 12 (4): 253-74. <a href="https://doi.org/10.2350/09-01-0586.1">doi:10.2350/09-01-0586.1</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/19220077">Pubmed</a> <span class="ref_v4"></span>

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