Neonatal respiratory distress syndrome, also known as hyaline membrane disease, is a condition due to insufficient surfactant synthesis and secretion, that characterizes prematurely born infants. Consequences such as atelectasis, abnormal ventilation-perfusion ratio, decreased oxygenation with consecutive hypoxia and acidosis are seen.
Presentation
Neonatal respiratory distress syndrome (NRDS) more frequently affects the Caucasian boys, especially born to diabetic mothers by cesarean section, second born twins and children with a positive family history. On the other hand, maternal hypertension, antenatal steroid administration and prolonged membrane rupture seem to act as protective factors. Keeping this in mind, mothers should be counseled upon discharge in case they ever want to have another child.
The more prematurely the baby is born, the higher the chances of suffering from this ailment are. However, late prematurity is also associated with this disease [1]. The problem becomes apparent soon after birth, progressively worsens and its signs consist of tachypnea, increased respiratory effort with intercostal, suprasternal and substernal retractions, flaring of the nasal alae, expiratory grunting and cyanosis. In extreme situations, irregular breathing, apnea, and hypothermia are encountered. The consciousness state may be diminished and severe fatigability may be reported. In advanced cases, the patient requires ventilatory support and intensive care [2] [3].
Clinical examination reveals decreased breath sounds and peripheral pulses. Oliguria and peripheral edema can be encountered.
The surfactant deficiency may be secondary to several pathological processes, such as infectious or meconium aspiration pneumonia or pulmonary hemorrhage, intrapartum asphyxia, pulmonary hypoplasia or oxygen toxicity. Furthermore, the newborn may have other associated diseases, such as congenital diaphragmatic hernia, necrotizing enterocolitis or patent ductus arteriosus.
Entire Body System
- Swelling
This leads to problems including total body swelling. Severe swelling can interfere with how the body organs work. Nonimmune hydrops fetalis is more common. It accounts for up to 90% of cases of hydrops. [medlineplus.gov]
The severe swelling that occurs with hydrops can overwhelm the baby's organ systems. About 50% of unborn babies with hydrops don’t survive. Risks for other problems are also high for babies born with hydrops. [stanfordchildrens.org]
Hydrops fetalis, or hydrops, is a condition that occurs when large amounts of fluid build up in a baby’s tissues and organs causing extreme swelling. [childrenshospital.org]
Rh compatibility causes massive red blood cell destruction, which leads to several problems, including total body swelling. Severe swelling can interfere with how the body organs work. [rarediseases.info.nih.gov]
- Surgical Procedure
Estimation of DMC was done by combining the techniques of average costs for services and surgical procedures. For drug prescriptions and surfactant treatment microcosting was applied. [scielosp.org]
[…] for use in connection with surgical procedures for removing or relieving respiratory obstruction, in particular obstruction of the upper respiratory passages, respiratory failure, respiratory insufficiency, respiratory paralysis and retained secretion [et.glosbe.com]
- Underweight
For example, when: the mother has diabetes the baby is underweight the baby's lungs have not developed properly Around half of all babies born between 28 and 32 weeks of pregnancy develop NRDS. [nhs.uk]
If IRDS is diagnosed and treated promptly, the outcome is good Infants with low birth weight (under 1000 g or 2.2 lbs) may develop severe IRDS, and the mortality rate in underweight infants is reported to be nearly 50% Additionally, if serious complications [dovemed.com]
Respiratoric
- Pneumonia
Understand management strategies for TTN, pneumonia, RDS, and MAS. Implement up-to-date recommendations for the prevention of neonatal pneumonia, RDS, and MAS. [pedsinreview.aappublications.org]
Pneumonia is an infection of the lungs. It’s usually caused by a bacteria or virus. Some babies get pneumonia while they are still in the womb and must be treated at birth. Babies may also develop pneumonia several weeks after delivery. [healthline.com]
Pneumonia Pneumonia (lung infection) is commonly treated in newborn care, particularly in developing countries. Bacterial, viral and fungal agents can all cause pneumonia. [futurelearn.com]
Pulmonary 1- Transient tachypnea of newborn 2- Hyaline membrane disease 3- Meconium aspiration syndrome (MAS) 4- Pneumonia 5- Air Leak Syndromes 6. [slideshare.net]
- Tachypnea
Neonates with transient tachypnea are usually at term. [emedicine.com]
Convert to ICD-10-CM : 770.6 converts directly to: 2015/16 ICD-10-CM P22.1 Transient tachypnea of newborn Approximate Synonyms Neonatal transient tachypnea Transient tachypnea (rapid breathing) of newborn Transient tachypnea of newborn Clinical Information [icd9data.com]
Transient tachypnea is more likely to occur in babies who were: Born before 38 weeks gestation (early term) Delivered by C-section, especially if labor has not already started Born to a mother with diabetes Male Newborns with transient tachypnea have [medlineplus.gov]
Symptoms manifest shortly after birth and include tachypnea, tachycardia, increased breathing effort, and/or cyanosis. The diagnosis is suspected based on clinical features and confirmed by evaluating the extent of atelectasis via chest x-ray. [amboss.com]
- Nasal Flaring
The symptoms usually appear shortly after birth and may include tachypnea, tachycardia, chest wall retractions (recession), expiratory grunting, nasal flaring and cyanosis during breathing efforts. [orpha.net]
flaring and blue discoloration of the skin during breathing efforts. [imedpub.com]
Definition Respiratory distress in the newborn is characterized by one or more of the following: nasal flaring, chest retractions, tachypnea, and grunting. [pedsinreview.aappublications.org]
2016 Topics: - Introduction - Cause & Risk Factors - Role of Maternal Diabetes in RDS - Role of Male Gender in RDS - Role of Prematurity in RDS - Role of Surfactant - Composition of Surfactant - Pathogenesis - Morphology - Clinical Features - Cause of Nasal [youtube.com]
This infant will also probably have nasal flaring. What is most remarkable about the physical appearance of this baby? No. May increase P(a)O2, but any increase is merely transient. [quizlet.com]
- Respiratory Insufficiency
The ability to give multiple doses of surfactant to infants with ongoing respiratory insufficiency leads to improved clinical outcome and appears to be the most effective treatment policy. [ncbi.nlm.nih.gov]
The control group underwent later intubation and surfactant replacement if required for progressive respiratory insufficiency. [doi.org]
Rapid development of profound respiratory insufficiency unresponsive to standard treatment is a bad sign and is associated with high mortality. [histopathology-india.net]
- Decreased Breath Sounds
Clinical examination reveals decreased breath sounds and peripheral pulses. Oliguria and peripheral edema can be encountered. [symptoma.com]
decreased breath sounds Cyanosis due to peripheral hypoxic vasoconstriction References:[4][1][5] Diagnostics Chest x-ray: diffuse, fine, reticulogranular (ground-glass) densities, with low lung volumes and air bronchograms Blood gas analysis Hypoxia [amboss.com]
If the infant is on ventilatory support will have sudden onset of clinical deterioration (i.e., cyanosis, hypoxemia, hypercarbia & respiratory acidosis associated with decreased breath sounds and shifted heart sounds). 49. [slideshare.net]
[…] an asymmetric chest, decreased breath sounds, and shift of the cardiac apical pulse away from the affected side [ 38 ]. [intechopen.com]
Cardiovascular
- Cyanosis
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, [icd9data.com]
[…] neonatal respiratory distress syndrome a condition of the newborn marked by dyspnea with cyanosis, preceded by symptoms such as dilatation of the nostrils, grunting on exhalation, and retraction of the suprasternal notch or costal margins. [web.archive.org]
The symptoms usually appear shortly after birth and may include tachypnea, tachycardia, chest wall retractions (recession), expiratory grunting, nasal flaring and cyanosis during breathing efforts. [orpha.net]
Tachypnea (>60 breaths/min), nasal flaring, subcostal and intercostal retractions, cyanosis & expiratory grunting. Breath sounds may be normal or diminished and fine rales may be heard. Progressive worsening of cyanosis & dyspnea. [slideshare.net]
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. [fpnotebook.com]
- Tachycardia
The symptoms usually appear shortly after birth and may include tachypnea, tachycardia, chest wall retractions (recession), expiratory grunting, nasal flaring and cyanosis during breathing efforts. [orpha.net]
Symptoms manifest shortly after birth and include tachypnea, tachycardia, increased breathing effort, and/or cyanosis. The diagnosis is suspected based on clinical features and confirmed by evaluating the extent of atelectasis via chest x-ray. [amboss.com]
Urogenital
- Cesarean Section
section, and sometimes with no apparent predisposing cause. [icd9data.com]
Amniotic fluid samples were obtained by amniocentesis or by puncturing embryonic membranes during cesarean section. The presence of mild, moderate, and severe RDS was assessed by neonatologist. [ncbi.nlm.nih.gov]
Most affected babies either were born prematurely, have diabetic mothers, or were delivered by cesarean section ; sometimes there is no known cause. [web.archive.org]
- Oliguria
Oliguria and peripheral edema can be encountered. [symptoma.com]
Pneumothorax and/or pneumomediastinum PPHN in severe cases Hypoxia to other organs (e.g., seizures, oliguria) 36. Pathophysiology 37. Chest x-ray: Areas of hyperexpansion mixed with patchy densities and atelectasis 38. [slideshare.net]
These manifestations progress to apnea, flaccidity, unresponsiveness, mottling, peripheral edema, oliguria, hypotension, and bradycardia. [medical-dictionary.thefreedictionary.com]
Workup
An amniocentesis is able to predict if NRDS will occur after birth, based on the lecithin-to-sphingomyelin ratio the presence of phosphatidylglycerol in the amniotic fluid. If these tests are not available, birth is in progress at the time the mother comes to the hospital or fetal distress is seen and childbirth is required, the diagnosis is made after the delivery is done.
Once respiratory distress is noticed, the physician should determine its severity and arterial blood gases offer a good measurement tool. Central (umbilical) or peripheral arterial blood should be analyzed [4] and may show hypoxemia, hypercapnia and respiratory (caused by alveolar atelectasis) and metabolic (due to lactic acid accumulation) acidosis. If a congenital right to left shunt heart malformation coexists, hypoxia will be severe and the child is likely to be cyanotic. A patent ductus arteriosus can have the same results. Blood workup should include a blood glucose level, that is often low, a complete cell count, bilirubin level, renal and liver function and electrolytes.
Oxygenation may also be monitored using a less invasive method, pulse oximetry. Probes should be placed on the right wrist or palm in order to establish the preductal saturation and on either foot for postductal saturation. Saturation should be kept at 91-95% [5]. This is a good screening technique for congenital heart disease, but the diagnosis should finally be evaluated by echocardiography in selected cases.
A thoracic radiography is done in order to observe the pulmonary aspect. Pleural effusion, atelectasis or air bronchograms, interstitial emphysema, pneumothorax, bronchogenic cyst or pulmonary sequestration may be described by this exam. Cardiomegaly is often found and further characterization by echocardiography is required. Doppler echocardiography is reliable in determining if pulmonary hypertension is present. Hyperinflation (in a non-ventilated patient) or a normal aspect at 6 hours of age exclude the NRDS.
Pulmonary ultrasonography may be a reliable method [6] [7] that visualizes the existing pleural effusions [8]. However, pneumothorax is harder to find by lung ultrasonography [9].
If infection signs are detected, tracheal aspirate, cerebrospinal fluid and blood cultures should be obtained.
In situations where the outcome is unfavorable, a histology evaluation will describe the presence of hyaline membranes - an amorphous material, lining the alveolar spaces, collapsed alveoli alternating with hyper aerated ones and vascular congestion.
X-Ray
- Bilateral Atelectasis
atelectasis (grade IV); (5) Arterial blood gas analysis showing hypoxia, hypercapnia and oxygen tension/fraction of inspired oxygen ratio (PaO 2 /FiO 2 ) ≤ 26.7 kPa. [omicsonline.org]
atelectasis (grade IV); (4) arterial blood gas analysis showing hypoxia, hypercapnia, and oxygen tension/fraction of inspired oxygen ratio (PaO2/FiO2) ≤ 26.7 kPa. [hindawi.com]
Serum
- Hypercapnia
Central (umbilical) or peripheral arterial blood should be analyzed and may show hypoxemia, hypercapnia and respiratory (caused by alveolar atelectasis) and metabolic (due to lactic acid accumulation) acidosis. [symptoma.com]
Clinical evaluation ABG (hypoxemia and hypercapnia) Chest x-ray Blood, CSF, and tracheal aspirate cultures Diagnosis of RDS is by clinical presentation, including recognition of risk factors; ABGs showing hypoxemia and hypercapnia; and chest x-ray. [msdmanuals.com]
Surfactant deficiency → little or no reduction of alveolar surface tension → reduced pulmonary unfolding → atelectasis → decreased lung compliance and functional residual capacity → hypoxemia and hypercapnia References:[1][3] Clinical features History [amboss.com]
Treatment
[…] airway pressure (MAP) [ Time Frame: 72 hours after treatment ] Information from the National Library of Medicine Choosing to participate in a study is an important personal decision. [clinicaltrials.gov]
Sixty babies were recruited, with 29 in the bLES and 31 in the Survanta treatment group. [ncbi.nlm.nih.gov]
Curosurf for “early treatment” (the new treatment) was donated by Serono Nordic, Copenhagen, Denmark. [doi.org]
Treatment before birth Treatment for neonatal respiratory distress syndrome (NRDS) sometimes begins before birth. [your.md]
Prognosis
Also, gestational age has an important role in determining the prognosis, where late preterm infants usually have a better prognosis in comparison with early preterm infants. 5. [intechopen.com]
Prognosis Prevention Prevent premature birth if possible. See tocolysis in the Preterm labor and birth learning card. [amboss.com]
[…] ground-glass” opacification of both lungs with air bronchograms and hypoaeration Hypoaeration from loss of lung volume (may be counteracted by respiratory therapy) Fine granular pattern Prominent air bronchograms Bilateral and symmetrical distribution Prognosis [learningradiology.com]
Monitoring the changes of serum BMP-7 and TGF-β1 is very important for treatment and prognosis assessment of the NRDS. [ncbi.nlm.nih.gov]
Etiology
Etiology Impaired synthesis and secretion of surfactant Risk factors Premature birth Genetic predisposition Scheduled cesarean section Maternal diabetes mellitus Hydrops fetalis Multifetal pregnancies Rarely, hereditary References:[1] Pathophysiology [amboss.com]
The etiologies of NRD in the 334 newborns were dominated by neonatal infections (Figure 1). [panafrican-med-journal.com]
Etiology NIHF is the result of an increase in interstitial fluid production or, in turn, of an obstruction of lymphatic return. [orpha.net]
Etiologic diagnosis will guide prognosis and the selection of specific chemotherapy. [ncbi.nlm.nih.gov]
Epidemiology
Epidemiology References:[1][2] Epidemiological data refers to the US, unless otherwise specified. [amboss.com]
Knowledge of the epidemiology and clinical characteristics of maternal and fetal infection can be used to select a diagnostic approach. Etiologic diagnosis will guide prognosis and the selection of specific chemotherapy. [ncbi.nlm.nih.gov]
Published on May 25, 2018 In this video, the viewer will learn the differential diagnosis for newborn respiratory distress, and the epidemiology, pathophysiology, presentation, diagnosis, and management of its most common etiologies. [youtube.com]
In this chapter, we are going to have a rapid review on epidemiology, diagnosis and treatments of RDS. [intechopen.com]
Pathophysiology
[…] groups, the GT genotype and A allele were less frequent among the RDS groups in 26-32.9 weeks of gestational age and in a birth weight subgroup of CONCLUSION: Our study raises the possibility that a genetic variation of NOS3 could be implicated in the pathophysiology [ncbi.nlm.nih.gov]
Etiology Impaired synthesis and secretion of surfactant Risk factors Premature birth Genetic predisposition Scheduled cesarean section Maternal diabetes mellitus Hydrops fetalis Multifetal pregnancies Rarely, hereditary References:[1] Pathophysiology [amboss.com]
Historical Perspective: Failure of Oxygen Treatment Before discussing the pathophysiology of RDS and the current modalities of treatment, it is useful first to consider why the historical mode of treatment, administration of oxygen, was ... [accessmedicine.mhmedical.com]
Prevention
Prevention Preventing prematurity is the most important way to prevent neonatal RDS. Ideally, this effort begins with the first prenatal visit, which should be scheduled as soon as a mother discovers that she is pregnant. [healthmedicinet.com]
Calfactant (Infasurt) is a natural bovine surfactant which has been evaluated for intratracheal use in the prevention and rescue treatment of respiratory distress syndrome (RDS) in preterm infants. [ncbi.nlm.nih.gov]
Prevention Taking steps to prevent premature birth can help prevent neonatal RDS. Good prenatal care and regular checkups beginning as soon as a woman discovers she is pregnant can help avoid premature birth. [mountsinai.org]
Taking steps to prevent premature birth can help prevent neonatal RDS. Good prenatal care and regular checkups beginning as soon as a woman discovers she is pregnant can help avoid premature birth. [medlineplus.gov]
Controlled trial of artificial surfactant to prevent respitatory distress syndrome. Lancet 1984; i: 476. CrossRef Google Scholar 20. Wilkinson A, Jenkins PA, Jeffrey JA. [link.springer.com]
References
- Wang ML, Dorer DJ, Fleming MP, et al. Clinical outcomes of near-term infants. Pediatrics. 2004;114:372-6.
- Escobar GJ, Clark RH, Greene JD. Short-term outcomes of infants born at 35 and 36 weeks’ gestation: we need to ask more questions. Semin Perinatol. 2006;30:28-33.
- Jain L, Eaton DC. Physiology of fetal lung fluid clearance and the effect of labor. Semin Perinatol. 2006;30:34-43
- Billman GF, Hughes AB, Dudell GG, et al. Clinical performance of an in-line, ex vivo point-of-care monitor: a multicenter study. Clin Chem. 2002;48(11):2030-43.
- Tarnow-Mordi W, Stenson B, Kirby A, et al. Outcomes of Two Trials of Oxygen-Saturation Targets in Preterm Infants. N Engl J Med. 2016; 374(8):749-60.
- Liu J, Cao HY, Liu Y. Lung ultrasonography for the diagnosis of neonatal respiratory distress syndrome: a pilot study. Zhonghua Er Ke Za Zhi. 2013; 51(3):205-10.
- Vergine M, Copetti R, Brusa G, et al. Lung ultrasound accuracy in respiratory distress syndrome and transient tachypnea of the newborn. Neonatology. 2014;106(2):87-93.
- Ahuja CK, Saxena AK, Sodhi KS, et al. Role of transabdominal ultrasound of lung bases and follow-up in premature neonates with respiratory distress soon after birth. Indian J Radiol Imaging. 2012;22(4):279-83.
- Sawires HK, Abdel Ghany EA, Hussein NF, et al. Use of lung ultrasound in detection of complications of respiratory distress syndrome. Ultrasound Med Biol. 2015;41 (9):2319-25.