Assessment of cyanosis in the newborn

Overview
Summary Aetiology

Emergencies
Urgent considerations

Diagnosis
Step-by-step Differential diagnosis Guidelines

Resources
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Summary
Tachypnoea and cyanosis are frequently encountered in the neonatal period. The prevalence of respiratory distress in newborns ranges from 2.9% to 7.6%. Cyanosis can result from a range of disorders, including cardiac, metabolic, neurological, and parenchymal/non-parenchymal pulmonary disorders. In all, 4.3% of newborns may require supplemental oxygen therapy because of cyanosis. [1] [2] [3] Cyanosis is dependent on the absolute concentration of the reduced haemoglobin and not on the ratio of reduced haemoglobin to oxyhaemoglobin. Cyanosis is classified into central and peripheral cyanosis. When present throughout the body, including the mucous membranes and tongue, the condition is termed central cyanosis. When limited to the extremities, it is termed peripheral cyanosis or acrocyanosis.

Aetiology
Any of the following 5 mechanisms may give rise to arterial oxygen desaturation:
Hypoventilation Significant right-to-left intracardiac or intrapulmonary shunting Ventilation perfusion unevenness Diffusion impairment Inadequate transport of oxygen by the haemoglobin.

During assessment, it is helpful to break down the aetiology into different systems. Cyanosis may be due to: Cardiac causes (memorised as the 5Ts mnemonic):
Transposition of great arteries: The great arteries (the aorta and the pulmonary artery) have their origins transposed so that the aorta originates from the right ventricle and the pulmonary artery originates from the left ventricle. Thus, deoxygenated blood is in the aorta, giving rise to severe cyanosis. Generally, these infants have either an intra-cardiac shunt, such as a ventricular septal defect (VSD) or an atrial septal defect (ASD). It becomes an emergency if there is no intra-cardiac shunt. Tetralogy of Fallot: The 4 common problems are: right ventricular hypertrophy, infundibular pulmonary stenosis, overriding of the aorta, and a VSD. Most infants will be cyanotic at birth but a few of them will have adequate oxygen saturation depending on the pulmonary blood flow and shunt. Total anomalous pulmonary venous return: The pulmonary veins drain into the right atrium (instead of the left atrium), and this condition leads to cyanosis and pulmonary congestion. The veins may also drain into the superior vena cava, inferior vena cava, or hepatic veins. The condition is generally associated with an ASD for intra-cardiac shunting. The condition can be partial or total, depending on whether all 4 pulmonary veins drain into the right side or not. Truncus arteriosus: 1 large vessel originates from both ventricles, instead of 2 vessels (the aorta and the pulmonary artery). It is associated with a VSD. This gives rise to cyanosis and increased pulmonary blood flow. Tricuspid atresia: right atrial blood is forced to shunt through foramen ovale or ASD to the left atrium; most patients also have a VSD. This condition is usually associated with a hypoplastic right ventricle.

Other less common cardiac causes:

Pulmonary atresia: generally associated with a VSD but may occur with intact ventricular septum, in which case pulmonary blood flow depends on the patent ductus arteriosus (PDA). Ebstein's anomaly: the tricuspid valve is abnormal with 2 of its leaflets displaced towards the right ventricle with subsequent atrialization of the right ventricle. The right atrium is significantly enlarged and there is regurgitation of the tricuspid valve; shunt takes place from right atrium through foramen ovale to left atrium. Left-to-right shunt with pulmonary oedema: generally this takes place if there is a VSD or PDA. Cyanosis is less severe than in conditions where there is right to left shunt but they have more severe respiratory distress. Single ventricle states (hypoplastic left or right heart) Low cardiac output states - CHF.

Pulmonary causes: [4]

o o image o pneumonitis o o oedema) o o o Pulmonary oedema Pulmonary hypoplasia (history of oligohydramnios, or congenital diaphragmatic hernia) Pulmonary lymphangiectasia: a rare congenital condition with pulmonary subpleural, interlobar, perivascular and peribronchial lymphatic dilatation, leading to significant respiratory distress at birth. o o o o o o Non-parenchymal Tracheo-oesophageal fistula/oesophageal atresiaView image Congenital diaphragmatic herniaView image Congenital cystic adenomatoid malformationView image PneumothoraxView image Pleural effusionView image Upper airway obstruction (choanal atresia, laryngeal web, laryngomalacia, subglottic stenosis, and vocal cord paralysis) o o Lobar emphysema Persistent pulmonary hypertension of the newborn. Pneumonia Pulmonary haemorrhage (seen in coagulopathy, asphyxia, or left to right shunts with pulmonary Aspiration (meconium,View image blood, mucus, or milk) can give rise to atelectasis or chemical Parenchymal Respiratory distress syndrome due to surfactant deficiency in pre-term and surfactant protein B deficiency in full-term infantsView image Transient tachypnoea of the newborn due to delayed clearance of fetal pulmonary fluidView

Other important conditions to consider include:

Methaemoglobinaemia Sepsis giving rise to apnoea. Pulmonary haemorrhage may be seen in severe cases, in the presence of disseminated intravascular coagulation Hypoglycaemia leading to apnoea or seizures

Polycythaemia, usually does not result in central cyanosis, but may give rise to increased pulmonary vascular resistance and delayed clearance of fetal pulmonary fluid; acrocyanosis is frequently seen

Asphyxia leading to cerebral oedema and intra-cranial hemorrhage. Cyanosis is a result of the subsequent hypoventilation

Arteriovenous malformation leading to (high output) CHF.

Other disorders of haemoglobin (haemoglobin M and sulfhaemoglobinaemia) should be considered in rarer instances. Cyanosis can also result in any severely ill infant when a range of other features are present (e.g., metabolic acidosis, seizures).

Urgent considerations
See Differential Diagnosis for more details All cases of newborn cyanosis persisting beyond the first 5 minutes postpartum require immediate attention. Most cases of persisting newborn cyanosis are due to cardiopulmonary causes that require rapid intervention with a goal of maintaining oxygen saturation >90%. Oxygen administration should occur prior to evaluation of the underlying cause. Oxygen saturation should be maintained >90%. If hyperoxia test is positive (i.e., a rise in arterial PO2 after 15 minutes exposure to 100% oxygen), assisted ventilation (nasal CPAP or intubation and ventilation) may be required for infants with severe cyanosis. Vascular access should be established for infusion of drugs and/or fluids: shock. Antibiotics should be given if there is evidence of sepsis or pneumonia. Prostaglandins for patency of ductus arteriosus in suspected ductal-dependent congenital cardiac conditions. Administration of alprostadil (prostaglandin E1) as an IV infusion is life-saving in infants with ductal-dependent cyanotic cardiac lesions. Treatment should therefore be initiated without delay if this cause is suspected, keeping in mind that apnoea results from alprostadil administration. Cyanotic infants should be transferred to a tertiary care centre immediately for further management where balloon septostomy and/or surgery may be necessary. Symptomatic hypoglycaemia and hypocalcaemia should Fluids and vasopressors (dopamine, dobutamine, or epinephrine) should be given for hypotension and

be corrected with IV administration of glucose or calcium, as these conditions can give rise to apnoea (with or without seizures).

Red flags
Transposition of great arteries (TGA) Congenital diaphragmatic hernia (CDH) Upper airway obstruction Asphyxia Hypoglycaemia Neonatal sepsis Total anomalous pulmonary venous return (TAPVR) Hypoplastic left heart syndrome or single ventricle physiology states Tricuspid atresia Pulmonary haemorrhage Pleural effusion Arteriovenous malformation

Step-by-step diagnostic approach

Initial assessment of infants with cyanosis should include: [4]
History Physical examination CXR FBC with differential count Blood glucose Calcium (hypocalcaemia associated with CNS irritability/cyanotic heart disease) Pulse oximetry ABG/hyperoxia test Blood culture/sepsis screen ECG and echocardiogram.

Maternal history

Important features of maternal history predisposing to paediatric disorders include:

o o o o o o o o o o o o o Asthma Transient tachypnoea of newborn Use of opiates Respiratory depression from opiates Pregnancy-induced HTN Intrauterine growth retardation Polycythaemia Hypoglycaemia Polyhydramnios Tracheo-oesophageal fistula/oesophageal atresia Delivery of previous sibling with respiratory distress syndrome Surfactant protein B deficiency Group B streptococcal pneumonia Oligohydramnios Pulmonary hypoplasia. Diabetes Transient tachypnoea of newborn Respiratory distress syndrome Hypoglycaemia Large for gestational age

Factors relating to labour and delivery

 o o o o o o o o o o o o o

Premature and prolonged rupture of membranes Sepsis Pneumonia Epidural anaesthesia Fever Anaesthesia/analgesia Respiratory depression Apnoea Cyanosis Asphyxia Cerebral oedema Metabolic acidosis Chorioamnionitis Sepsis Caesarean section without labour Transient tachypnoea of the newborn Respiratory distress syndrome Persistent pulmonary hypertension of the newborn Breech delivery (trauma) Erb's palsy with phrenic nerve palsy.

Physical examination: overview

Cyanosis can develop immediately or several hours after birth. Immediate:

Transient tachypnoea of newborn Respiratory distress syndrome Pneumothorax or air leak Meconium aspiration syndrome Congenital diaphragmatic hernia Congenital cystic adenomatoid malformation.

Onset hours after birth:

Cyanotic congenital heart disease Aspiration Tracheo-oesophageal fistula.

General examination
In a cold environment, acrocyanosis is common. Examination should be performed under a radiant warmer in a well-lit room. Prolonged capillary refill time is often evident when peripheral perfusion is suboptimal in:
Hypoplastic left heart syndrome Sepsis/pneumonia Polycythaemia Acidosis Hypovolaemic states Hypothermia.

Respiratory examination
Tachypnoea, retractions, nasal flaring, and grunting generally indicate a pulmonary cause. However, in cardiac conditions with significant left-to-right shunt these symptoms may also occur. In cyanotic heart disease and methaemoglobinaemia, the respiratory rate may be normal. Apnoea and cyanosis may be due to sepsis, asphyxia, or seizures.

Upper airway obstruction may be evident from stridorous breathing due to:
Laryngotracheomalacia Subglottic stenosis Vocal cord paralysis Glossoptosis with micrognathia (Pierre-Robin syndrome).

Upper airway obstruction can also give rise to supraclavicular, submandibular, and suprasternal retractions. Bilateral choanal atresia generally gives rise to significant retractions at birth, but the symptoms are relieved by an oral airway. Cyanosis due to parenchymal lung disease is associated with intercostal and subcostal retractions. Absent/poor aeration of one side of chest on auscultation may be due to:
Pneumothorax Pleural effusion Atelectasis Congenital diaphragmatic hernia (CDH).

Cardiovascular examination
Normal heart rate in full-term newborns is approximately 120 beats per minute (bpm), with a range of 100 to 140 bpm. During quiet states it may be as low as 90 bpm. Heart rates >160 bpm during quiet state are abnormal. In supraventricular tachycardia, heart rates >200 bpm are usually seen. Heart rate variability is also important: in severe sepsis and asphyxia, the beat-tobeat variability will be lost. S2 is loud and narrowly split in pulmonary HTN. A single S2 is generally indicative of:
Severe pulmonary stenosis Pulmonary atresia Abnormal single valve position (transposition of great arteries) A large semilunar valve, as in truncus arteriosus.

It is important to record the type and quality of murmurs. The location of apical impulse should be noted to rule out dextrocardia. Precordial thrill indicates significant murmur of grade >3/6. Precordial hyperactivity is generally due to increased ventricular activity. It should be remembered that not all heart murmurs are pathological and that many cardiac conditions do not cause murmurs.

Abdominal examination
The abdomen may appear scaphoid in congenital diaphragmatic hernia. Abdominal distension due to bowel obstruction or ascites can give rise to respiratory distress. Hepatosplenomegaly with ascites and hydrops fetalis, as seen in cases of severe haemolytic disease of the newborn, can lead to severe respiratory distress. Hepatomegaly can co-exist with pulmonary congestion in conditions such as total anomalous pulmonary venous return. Absence of bowel sounds indicates ileus and is associated with sepsis, gangrenous bowel, or peritonitis.

CNS examination
Hypotonia is one of the earliest signs of:
Sepsis Asphyxia Metabolic disorders.

Hypertonia is often seen in narcotic withdrawal. Phrenic nerve paresis/paralysis can precipitate respiratory distress and is associated with Erb's palsy following traumatic vaginal delivery. Excessive traction on the neck or vaginal breech extraction are the common aetiologies.

CXR
CXR is integral to initial assessment of respiratory distress. [4] [5] [6] The location of stomach, liver, and heart should be ascertained to rule out dextrocardia and situs inversus. The size and shape of the heart may yield some clues to the diagnosis:
A small heart may be due to hypovolaemia, adrenal insufficiency from asphyxia or adrenal haemorrhage, significant pulmonary interstitial emphysema, and congenital lobar emphysema.

Severe cardiomegaly is present in Ebstein's anomaly. Moderate cardiomegaly is seen in infants with diabetic mothers (hyperinsulinaemia) and in cardiomyopathy (infections, metabolic disorders, or asphyxia) and congestive cardiac failure. CHF without intrinsic cardiac anomaly is seen in coarctation of the aorta and arteriovenous malformation.

o o

The commonly described cardiac silhouettes in congenital heart disease are: "Egg on end" appearance of transposition of the great vessels "Snowman" sign (a rounded, figure-of-eight-like cardiac contour) of total anomalous pulmonary venous return

Boot-shaped heart of tetralogy of Fallot.

Increased pulmonary vascular markings and pulmonary congestion are indicative of left-to-right shunt, while decreased pulmonary vascular markings (oligaemic lung fields) indicate pulmonary stenosis or pulmonary atresia with inadequate ductal shunting. Decreased pulmonary vascular markings may also occur in persistent pulmonary hypertension of the newborn. The expansion of lungs (lung volume) on both sides should be checked. Normal inspiratory films should have 8 intercostal spaces of lung fields on both sides. Diaphragmatic paralysis (more commonly seen on the right side) is manifested by elevation of the right hemidiaphragm by more than 2 intercostal spaces compared to the left side. This may simulate right lower lobe atelectasis. Hyperinflated lung fields are seen occasionally in lobar emphysema or cystic lesions of lungs. The prevalence of spontaneous air leaks giving rise to pneumothorax View image and pneumomediastinum View imageView image in full-term newborns is approximately 1% to 2%. Some characteristic pulmonary findings on CXR are associated with specific pathologies:
Transient tachypnoea of the newborn: lung fields may appear hazy with normal lung volume and increased parahilar markings, frequently with fluid in the horizontal fissure.View image Respiratory distress syndrome (RDS): CXR may not be reliable in the initial stages. As RDS worsens, the characteristic reticular granular pattern and air bronchograms become evident. Lung volume is reduced significantly in severe RDS.View image Meconium aspiration syndrome: fluffy infiltrates, patchy areas of atelectasis, and areas of hyperinflation due to air trapping may be evident.View image

 image

Pleural effusion (due to chylothorax or other causes): visible on lateral aspect of lung fields as a linear opacity. In large effusions, a whole lobe may appear opaque, with mediastinal shift to the contralateral side.View Lobar atelectasis: mediastinal shift towards the ipsilateral side is seen. In pneumonic consolidation, which may simulate atelectasis, no mediastinal shift is present.

Congenital diaphragmatic hernia: soon after birth, a large area of opacity may be the only finding instead of the classical finding of bowel gas in the thorax.View image This should be differentiated from congenital cystic adenomatoid malformation.View image

It is important to check the bony thoracic cage. In asphyxiating thoracic dystrophy, the thoracic cage will be small and narrow. The thorax may have a bell-shaped appearance in infants with severe hypotonia. Fractures of the ribs, humerus, or clavicles should be looked for following difficult vaginal deliveries, as infants may develop respiratory distress from the pain and splinting of the chest.

Ultrasound
Ultrasound examination of diaphragmatic motion during spontaneous breathing can detect paradoxical motion in diaphragmatic paralysis. Pleural effusion, eventration of the diaphragm, and size/location of the liver/spleen can also be determined.

Other imaging
CT scan of the chest is helpful if diagnosis is not clear, and it can identify congenital abnormalities and tumours of the mediastinum, lungs, and heart. Feeding-associated cyanosis may be due to incoordination of sucking and swallowing, vocal cord paralysis, laryngeal cleft, or severe birth asphyxia. Upper GI contrast studies should be obtained to rule out severe gastrooesophageal reflux and oesophagitis.

Pulse oximetry
Pulse oximetry monitoring is recommended for all infants with respiratory distress and cyanosis, since clinician assessment of colour at birth is well recognised to vary. [7] It is an accurate, reliable, and non-invasive method for monitoring oxygen saturation in infants. [8] [9][10] [11] In severe cyanosis with respiratory distress, both preductal and postductal oxygen saturations should be monitored to detect the gradient across the ductus arteriosus. For this procedure, the pulse oximeter probes should be placed over the right hand and a lower extremity. Pulse oximetry screening has been shown to be

helpful in early detection of congenital cardiovascular malformations in the newborn nursery. [12] Acrocyanosis is a common finding in the first few minutes after birth. This generally resolves spontaneously. If a low-pulse oximeter reading persists, it may be appropriate to proceed to a hyperoxia test. Pulse oximetry screening is helpful in the early detection of congenital cardiovascular malformations in the newborn nursery, and may detect lifethreatening malformations before symptoms develop. [12] [13] There is a significant body of evidence suggesting that early detection of congenital cardiovascular heart disease (CCHD) through pulse oximetry monitoring is an effective strategy for reducing morbidity and mortality rates in young children. [13] A working group has identified strategies and made recommendations for hospitals and birthing centres to implement the use of pulse oximetry for screening for CCHD in newborns, which should complement physical examinations. [13] ]

Hyperoxia test
The hyperoxia test is a further clinical tool to differentiate between cardiac and pulmonary aetiologies. It is indicated if the pulse oximeter reading is <85% in both room air and 100% oxygen. It is not recommended in preterm infants. ABG should be obtained from the right radial artery. A transcutaneous oxygen tension monitor may also be used for this purpose, but pulse oximetry is inadequate. Arterial samples are taken both before and 15 minutes after exposure to 100% oxygen. Providing that there is no significant right-to-left shunt, the 100% inhaled oxygen leads to an increase in alveolar PO2, which causes pulmonary venous PO2 and subsequently arterial PO2 to rise, although this might be partially dependent on level of ventilation. This rise in arterial PO2 is considered a positive result. In cyanotic congenital heart disease, there is little or no rise in PO2 with 100% oxygen inhalation. The exception is when there is a significant increase in pulmonary blood flow due to decreased pulmonary vascular resistance. [14] In this instance, the increase in pulmonary blood flow will cause an increase in QP/QS (pulmonary to systemic flow ratio) and may result in an increase in PO2 (although not as high as would be expected in an infant with pulmonary parenchymal disease). In persistent pulmonary hypertension of the newborn, the hyperoxia test may not be helpful in differentiating from cyanotic congenital heart disease.

Laboratory tests
ABG can help in determining the oxygenation, ventilation, and acid-base status of the infant. In methaemoglobinaemia, the PO2 is normal even with cyanosis. FBC with differential count is an important test to rule out polycythaemia, anaemia, neutropenia, leukopenia, abnormal I:T (immature to total neutrophil count) ratio, and thrombocytopenia as signs of sepsis. If sepsis is suspected, a blood culture should be obtained and spinal tap performed before antibiotic therapy is started. Significant metabolic acidosis may indicate cardiac failure, sepsis, asphyxia, or metabolic disorders. Closure of the ductus arteriosus in an infant with a ductal-dependent cardiac lesion can lead to shock and severe metabolic acidosis with cyanosis and respiratory distress. Calcium and magnesium levels should be obtained where other causes have been ruled out. Hypocalcaemia and hypomagnesaemia are both associated with CNS irritability and seizures. Metabolic screening of urine and drug screening of urine/meconium should be performed as clinically indicated.

ECG
An ECG is an important test, but of limited value except in certain specific conditions. [15] [16]Normally, there is right ventricular predominance in the newborn, and many cases of cyanotic congenital heart disease (CHD) will have similar findings. Left axis deviation with left ventricular dominance is seen in tricuspid atresia or pulmonary atresia with an intact ventricular septum. Left axis deviation is frequently associated with right ventricular hypertrophy as seen in arteriovenous canal malformation. ECG is important in the diagnosis of arrhythmias.

Echocardiogram
Echocardiogram is the definitive test in the diagnosis of congenital cardiac lesions and pulmonary HTN. A technician who is trained in performing echocardiograms in newborns and a paediatric cardiologist are required for this procedure.

Differential diagnosis
Sort by: common/uncommon or category Commonhide all Transposition of great arteries (TGA) see our comprehensive coverage of Overview of congenital heart disease

History
incidence is higher in male infants and infants of diabetic mothers; cyanosis appears within the first 24 hours, sometimes with no respiratory distress; if TGA is present with an intact ventricular septum, the infant will be very cyanotic, requiring immediate intervention

Exam
prominent right ventricular heave and a single second heart sound (a loud A2) are usually present; a systolic murmur due to increased pulmonary blood flow may be heard in a few cases

1st test

echocardiogram: definitive diagnostic test; reveals abnormal position of the aorta and

pulmonary arteries, abnormal cardiac anatom and function

CXR: "egg on a string" appearance; slight

cardiomegaly and increased pulmonary vasc markings

Tetralogy of Fallot (TOF) see our comprehensive coverage of Tetralogy of Fallot

History
cyanosis depends on the degree of right ventricular outflow tract obstruction, which ranges from very mild to severe; the age of symptom onset is variable; VACTERL (vertebral anomalies, imperforate anus, cardiac lesions, tracheooesophageal fistula, renal and limb anomalies) are seen in approximately 15% of infants who have TOF; infants may be referred for mild cyanosis or presence of a murmur at birth

Exam
prominent right ventricular heave and systolic ejection murmur at left sternal border are usually present

1st test

CXR: boot-shaped heart

echocardiogram: diagnostic study

revealing characteristic cardiac ana and function

Pulmonary atresia see our comprehensive coverage of Overview of congenital heart disease

History
of ventricular septal defect (VSD) and ASD for adequate mixing of blood at the atrial or ventricular level; mild to moderate cyanosis becomes worse when ductus arteriosus closes

Exam
ductus arteriosus is the only common finding

1st test

CXR: may reveal decreased pulmonary vascular markings

Othe

variable presentation depends on the presence murmur of patent

echocardiogram: definitive diagnostic test that reveals pulmonary atresia, abnormal cardiac anatomy and function

Respiratory distress syndrome (RDS) see our comprehensive coverage of Acute respiratory distress syndrome

History
generally occurs in preterm infants due to surfactant deficiency; fluid; higher incidence has been noted in infants of diabetic mothers; full-term infants with RDS have been reported to have surfactant protein B deficiency; majority of preterm infants with

Exam
tachypnoea, nasal flaring, cyanosis; in severe cases, diminished air entry is present on chest auscultation

1st test

CXR: reticular granularity bronchograms; in severe cases, decreased lung volumeMore

antenatal history may reveal immature lung profile in the amniotic grunting, and retractions with

RDS are born to mothers who did not receive antenatal corticosteroids

Transient tachypnoea of the newborn (TTN)

History
higher incidence among full-term newborns born by elective caesarean section; tachypnoea is the presenting symptom with increased FiO2 requirement; self-limiting problem; generally resolves within the first 3 days; cyanosis may occur in severe cases

Exam
tachypnoeic infant with reasonable air entry bilaterally upon auscultation

1st test

CXR: good lung volume with perihila markings along with fissural fluid markings on the right horizontal fissureMore

Persistent pulmonary hypertension of the newborn (PPHN)

History
occurs in full-term and post-term infants commonly; aetiology is variable; risk factors include history of asphyxia, meconium aspiration syndrome, sepsis, congenital diaphragmatic hernia, pulmonary hypoplasia

Exam
loud S2 and right ventricular heave

1st test

CXR: depends on the precipitating factors,

aspiration syndrome, sepsis/pneumonia, c diaphragmatic hernia

echocardiogram: definitive diagnostic tes

pulmonary artery pressure, tricuspid regurg ventricular size and function

Pneumothorax see our comprehensive coverage of Pneumothorax

History
can occur spontaneously at birth in full-term newborns or following resuscitation with positive pressure ventilation; occurs frequently in preterm infants with respiratory distress syndrome; assisted ventilation and CPAP contribute to the development of pneumothorax; higher incidence in meconium aspiration syndrome, pulmonary hypoplasia, and congenital diaphragmatic hernia

Exam
tachypnoea and cyanosis are usually present; depending on severity (proportionate to the amount of free air in the pleural space), breath sounds and heart sounds may be faint/distant; mediastinal shift to the contralateral side may occur; transillumination of the chest will be positive in most cases

1st test

CXR: media

contralatera

visceral pleu

markingsMo

Aspiration pneumonia see our comprehensive coverage of Aspiration pneumonia

History
may be due to meconium aspiration syndrome, blood, milk aspiration, or amniotic fluid; meconium aspiration is commonly seen in post-term infants and those who develop fetal distress; a hx of thick meconium-stained amniotic fluid at the time of rupture of membranes is characteristic; most infants become symptomatic with respiratory distress at birth; in the case of blood aspiration, there may be a hx of antepartum haemorrhage; in the case of milk aspiration, emesis following feeding and subsequent development of respiratory distress is usually present

Exam
tachypnoea, grunting, retractions, and cyanosis are present; on auscultation diminished air entry, rales, and rhonchi may be evident

1st test

CXR:

infiltra

marki

ABG:

PCO2

Pneumonia see our comprehensive coverage of Overview of pneumonia

History

Exam

1st test

risk factors include a hx of prolonged rupture of membranes, chorioamnionitis, or positive maternal group B beta streptococcus (GBS) screen; congenital GBS sepsis may present as GBS pneumonia with life

respiratory distress with tachypnoea, retractions, and grunting may be present; auscultation often reveals rales, rhonchi, apnoea may be the only presenting

CXR: diagnostic in most c

GBS sepsis may have fea

pneumonia due to viruses or bacteria is rare; early-onset and diminished air entry in a few cases; apnoea and circulatory collapse within the first week of symptom in some cases

distress syndrome on x-ra

FBC: abnormal; leukopen

thrombocytopenia may be

amounts of immature neu

abnormal I:T (immature to

ratio (>0.2) is present in m

Pulmonary oedema

blood culture: may be po

History
generally due to congestive cardiac failure; underlying cardiac disease, may be present

Exam
tachypnoea, tachycardia, and hepatomegaly are usually present; malformation present (bruit)

1st test

CXR: fluffy infiltrates or hazy lung fields and associated cardiomegaly

Other tests

ABG:

echoc

AV malformation, or severe anaemia severe pallor or signs of AV

conge

FBC:

Congenital cystic adenomatoid malformation (CCAM)

History
most of these cases are diagnosed by antenatal ultrasound; the severe forms may lead to hydrops in the fetus; milder forms may have mild respiratory distress or no symptoms at birth; many infants are diagnosed in childhood when they present with recurrent infections

Exam
tachypnoea and mild cyanosis are present in many infants; diminished air entry on the affected side will be present

1st test

CXR: multiple cysts with opaque areas in the lungMore

Congenital diaphragmatic hernia (CDH)

History
ultrasound; more common on the left side; most cases are symptomatic at

Exam
diminished air entry on the left side of chest, with cyanosis at birth are

1st test

CXR: diagnostic with intestinal gas pattern in the left hemithorax with mediastinal shiftMore

Other tests

most cases are diagnosed by antenatal scaphoid abdomen at birth,

echoc

also s

birth with severe cyanosis; some have typical features; heart sounds may minimal symptoms and are diagnosed not be heard on the left side of later in the neonatal period or infancy chest due to mediastinal shift

ABG: severe hypoxia and hypercarbia

Upper airway obstruction see our comprehensive coverage of Central airway obstruction

History
most become symptomatic shortly after birth

Exam
submandibular, suprasternal, and

1st test

CXR and lateral view of neck:sometimes the airway calibre

Other tests

laryngo

with no significant cyanosis; symptoms worsen supraclavicular retractions are during feeding, with stridorous breathing; those characteristic of upper airway with vocal cord paralysis will have a weak or obstruction; infants with choanal non-existent cry; cyanosis may develop; risk of atresia or stenosis may have mild to

laryngos

broncho

diagnost

aspiration during feeding; infants with vocal as meningomyelocele with Arnold-Chiari malformation and hydrocephalus

moderate symptoms depending on inability to pass nasogastric tube is diagnostic

can be demonstrated

malacia, web

cord paralysis may have CNS conditions, such whether unilateral or bilateral;

CT scan atresia

Polycythaemia

CT scan flexible

changes

History
polycythaemia is common in insulin-dependent diabetes mellitus, small for gestational age of diabetes and HTN during pregnancy are frequent; slightly higher incidence of transient tachypnoea of the newborn may be present

Exam
either large for gestational age or small for gestational age infants who may appear cyanotic but have normal ABGs; some infants may pulmonary HTN of newborn; infants appear ruddy and plethoric, and have a higher incidence of acrocyanosis; clinical examination may be normal or findings of transient tachypnoea of the newborn may be present

1st test

ABG: CXR:

infants, and twin-to-twin transfusion; maternal hx have transient tachypnoea of the newborn or mild persistent

of tran

the ne

FBC:

haema

Asphyxia

History

Exam

1st test

CXR: normal serum creatinine: elevated

hx of fetal distress with perinatal asphyxia and low neurologically depressed, hypotonia, subtle Apgar scores requiring vigorous resuscitation; hx of seizure activity, with good aeration to both seizures or apnoea may be the presenting symptom; lung fields; apnoea or hypoventilation may lead to hypoxic ischaemic encephalopathy (bradypnoea) is the cause for cyanosis

liver function tests: raised ALT

Methaemoglobinaemia (met-Hb)

History
usually well in spite of characteristic skin worsen with SOB and CNS features including seizures; may be congenital or acquired; acquired form may result from exposure to drugs/toxins known to cause met-Hb

Exam
characteristic blue colour, dyspnoea, mental methaemoglobin levels has a characteristic chocolate-brown colour; pulse oximetry is unreliable

1st test

ABG: normal PaO2More

Oth

discoloration, termed pseudocyanosis; condition may state changes; arterial blood with elevated

Hypoglycaemia see our comprehensive coverage of Non-diabetic hypoglycaemia

History
either a small for gestational age or

Exam
may be jittery or may have seizures; clinical examination may

1st test

blood sugar: severely

large for gestational age infant born be normal or may reveal hypo- or hypertonia; cyanosis is to a diabetic or hypertensive woman usually due to apnoea or transient persistent pulmonary hypertension of newborn

hypoglycaemic <1.4 mm (<25 mg/dL)More

Neonatal sepsis see our comprehensive coverage of Sepsis

History
hx of premature rupture of membranes, chorioamnionitis, or intrapartum maternal fever may be present; may present with history of lethargy, poor feeding, or emesis, temperature instability and hypo- or hyperglycaemia may be present

Exam
infants may be hypotonic with normal or poor peripheral perfusion and hypotension; cyanosis in most cases is due to associated persistent pulmonary HTN of newborn is also present; chest examination is normal

1st test

FBC: leukopenia, neutropenia, elevated I:T ratio, thrombocytopenia

apnoea and cyanosis or with circulatory shock; hypoventilation or apnoea and in some cases

ESR: elevated blood culture: pathogenic organism cultured

Uncommonhide all
Total anomalous pulmonary venous return (TAPVR) see our comprehensive coverage of Overview of congenital heart disease

History
clinical condition depends on the presence of pulmonary venous obstruction; pulmonary congestion

Exam
prominent right ventricular heave; widely split S2; systolic ejection murmur at the left upper sternal border may occur;

1st test

CXR: cardiomegaly; "snowman" pulmo congestionMore

(pulmonary oedema) with respiratory hepatomegaly is common; response to distress and cyanosis can be mistaken alprostadil (prostaglandin E1) infusion is for interstitial pneumonitis generally ineffective to minimal

echocardiogram: definitive diagnostic reveals pulmonary venous drainage,

characteristic cardiac anatomy and fun

Hypoplastic left heart syndrome or single ventricle physiology states see our comprehensive coverage of Overview of congenital heart disease

History
most cases are diagnosed prenatally; more common in boys; if closure of the ductus arteriosus; when the ductus arteriosus closes, respiratory distress develops, shock and cyanosis with poor peripheral perfusion generally occur within the first 3 days of life; severe cyanosis can occur at birth when the foramen ovale is closed or restrictive

Exam
right ventricular S2 are present

1st test

CXR: cardiomegaly with some degr pulmonary congestion

unrecognised at birth, infants become symptomatic at the time of the heave and single

echocardiogram: definitive diagno

that reveals abnormal cardiac anato function

Tricuspid atresia see our comprehensive coverage of Overview of congenital heart disease

History
most cases are diagnosed antenatally by fetal echo; symptoms depend on the presence of ventricular septal defect (VSD); if there is a large shunt through the VSD, many infants may have mild cyanosis or none in the neonatal period; many infants present with CHF later; if the VSD is very restrictive, severe cyanosis may develop

Exam
systolic ejection murmur at the left upper sternal border and prominent left ventricular impulse

1st test

CXR: depends on the degree of pulmo

blood flow and may present with pulmo congestion

echocardiogram: definitive diagnostic

that reveals tricuspid atresia; enlarged

when the ductus arteriosus closes

atrium and dilated right ventricle

Truncus arteriosus (TA) see our comprehensive coverage of Overview of congenital heart disease

History
or without respiratory distress may present in first week of

Exam
S2; loud systolic and diastolic murmur with wide pulse pressure and bounding pulses;

1st test

CXR: cardiomegaly and increased pulmonary blood flow

mild to moderate cyanosis with hyperdynamic precordium with loud single

life; velocardiofacial syndrome respiratory distress depends on the degree of (22q deletion) is present in 30% pulmonary congestion and worsens when to 50% of TA cases pulmonary vascular resistance drops after the first 2 to 3 days; CHF may develop as a result

echocardiogram: definitive diagnostic test may reveal abnormal morphology and

functional derangement of the truncal valve

Pulmonary haemorrhage

History
most cases occurring in full-term infants will give a hx of severe asphyxia or coagulopathy; in preterm infants who have haemodynamically significant patent ductus arteriosus with left-to-right shunt, pulmonary haemorrhage may develop; most of these infants are ventilated post-surfactant instillation

Exam
sudden appearance of cyanosis or increased requirement of oxygen when infant is being ventilated is the earliest sign; bloody secretions from the trachea may be suctioned from the endotracheal tube; rales or diminished air entry found on the affected side, but condition is generally bilateral

1st test

CXR: fluffy infiltrates bilaterally

Oth

ABG: severe hypoxia and hypercarbia

FBC: normal but in some cases thrombocytopenia may be present

DIC screen: prolonged PT and PTT; low fibrinogen and low platelet count with increase in D-dimers

Pulmonary hypoplasia

History
hx of oligohydramnios and respiratory distress soon after birth are present; higher incidence of pneumothorax in these cases

Exam
tachypnoea, cyanosis, and fair to poor aeration of lungs present; S2 is loud with associated pulmonary hypertension

1st test

CXR: decreased lung volume

Other tests

echo

be pr

ABG: hypoxia and hypercarbia

Pulmonary lymphangiectasia

History
respiratory distress and cyanosis in the newborn

Exam
tachypnoea and cyanosis in a full-term newborn; air entry will not be diminished; occasionally rales may be heard

1st test

CXR: diffuse reticular opacities bilaterally

Other tests

CT scan: su involvement

lung biopsy

lymphatic sit

interlobular s

Tracheo-oesophageal fistula (TOF)/oesophageal atresia (OA)

see our comprehensive coverage of Tracheo-oesophageal fistula

History
of pharyngeal secretions present after birth; inability to pass an orogastric or nasogastric tube is diagnostic; OA associated with VACTERL (vertebral anomalies, imperforate anus, cardiac

Exam
at the mouth following birth is usually present; conducted upper airway sounds will be heard on auscultation; most forms of TOF have OA with/without tracheooesophageal fistula; respiratory distress is aspiration

1st test

CXR: diagnostic with an indwelling orogastric tube curled in the upper oesophageal pouchMore

Other te

hx of polyhydramnios and large amount respiratory distress with frothy secretions

lesions, TOF, renal and limb anomalies) due to secretions in the upper airway and

Congenital lobar emphysema

History
generally asymptomatic at birth; respiratory distress becomes progressively worse over the next few days

Exam
tachypnoea and cyanosis are present; diminished air entry over the affected side (commonly over left upper chest)

1st test

CXR: hyperinflation of the affected lobe present; in severe cases, there may be herniation to the opposite side

ABG: hypoxia and hypercarbia

Pleural effusion see our comprehensive coverage of Pleural effusion

History
most pleural effusions present at birth are seen in infants with hydrops fetalis and are diagnosed by antenatal ultrasound; in severe cases it will be difficult to ventilate unless to remove the fluid; minor degrees of pleural effusion are seen in pneumonitis, meconium aspiration syndrome, and persistent pulmonary HTN of newborn

Exam
in severe cases, diminished air entry will be present on the affected side; in bilateral effusions, as seen in hydrops ventilate the lungs unless the pleural fluid is evacuated

1st test

CXR: opacity on the lateral border of the c with blunting of the cardiophrenic angle in unilateral effusions; in severe cases, mediastinal shift to contralateral side if the

needle aspiration or chest tube insertion occurs fetalis, it will be difficult to

effusion is unilateral; in severe bilateral ple

effusions, the whole chest is opaque with a indistinguishable cardiac borderMore

Arteriovenous malformation

History
CHF, large head due to hydrocephalus, seizures

Exam
bruit may be heard over the cranium on auscultation

1st test

CXR: may show cardiomegaly

Other tests

angiogram or MRA (

angiogram): may rev