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Assessment of cyanosis in the newbornOverview

Summary

Aetiology

Emergencies

Urgent considerations

DiagnosisStep-by-step

Differential diagnosis

Guidelines

Resources

References

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SummaryTachypnoea 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.

AetiologyAny of the following 5 mechanisms may give rise to arterial oxygendesaturation:

Hypoventilation

Significant right-to-left intracardiac or intrapulmonary shunting

Ventilation perfusion unevenness

Diffusion impairment

Inadequate transport of oxygen by the haemoglobin.
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During assessment, it is helpful to break down the aetiology into differentsystems. 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.


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Pulmonary causes:[4]

Parenchymal

o Respiratory distress syndrome due to surfactant deficiency in pre-term and surfactant protein B

deficiency in full-term infantsView image

o

Transient tachypnoea of the newborn due to delayed clearance of fetal pulmonary fluidViewimage

o Aspiration (meconium,View image blood, mucus, or milk) can give rise to atelectasis or chemical

pneumonitis

o Pneumonia

o Pulmonary haemorrhage (seen in coagulopathy, asphyxia, or left to right shunts with pulmonary

oedema)

o Pulmonary oedema

o Pulmonary hypoplasia (history of oligohydramnios, or congenital diaphragmatic hernia)

o Pulmonary lymphangiectasia: a rare congenital condition with pulmonary subpleural, interlobar,

perivascular and peribronchial lymphatic dilatation, leading to significant respiratory distress at birth.

Non-parenchymal

o Tracheo-oesophageal fistula/oesophageal atresiaView image

o Congenital diaphragmatic herniaView image

o Congenital cystic adenomatoid malformationView image

o PneumothoraxView image

o Pleural effusionView image

o Upper airway obstruction (choanal atresia, laryngeal web, laryngomalacia, subglottic stenosis,

and vocal cord paralysis)

o Lobar emphysema

o Persistent pulmonary hypertension of the newborn.

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
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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 anyseverely ill infant when a range of other features are present (e.g., metabolicacidosis, seizures).

Urgent considerationsSee 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:

Fluids and vasopressors (dopamine, dobutamine, or epinephrine) should be given for hypotension and

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
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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 approachInitial 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
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Important features of maternal history predisposing to paediatric disordersinclude:

Diabetes

o Transient tachypnoea of newborn

o Respiratory distress syndrome

o Hypoglycaemia

o Large for gestational age

Asthma

o Transient tachypnoea of newborn

Use of opiates

o Respiratory depression from opiates

Pregnancy-induced HTN

o Intrauterine growth retardation

o Polycythaemia

o Hypoglycaemia

Polyhydramnios

o Tracheo-oesophageal fistula/oesophageal atresia

Delivery of previous sibling with respiratory distress syndrome

o Surfactant protein B deficiency

o Group B streptococcal pneumonia

Oligohydramnios

o Pulmonary hypoplasia.

Factors relating to labour and delivery


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Premature and prolonged rupture of membranes

o Sepsis

o Pneumonia

Epidural anaesthesia

o Fever

Anaesthesia/analgesia

o Respiratory depression

o Apnoea

o Cyanosis

Asphyxia

o Cerebral oedema

o Metabolic acidosis

Chorioamnionitis

o Sepsis

Caesarean section without labour

o Transient tachypnoea of the newborn

o Respiratory distress syndrome

o Persistent pulmonary hypertension of the newborn

Breech delivery (trauma)

o Erb's palsy with phrenic nerve palsy.

Physical examination: overviewCyanosis can develop immediately or several hours after birth.

Immediate:


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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 examinationIn a cold environment, acrocyanosis is common. Examination should beperformed under a radiant warmer in a well-lit room. Prolonged capillary refilltime is often evident when peripheral perfusion is suboptimal in:

Hypoplastic left heart syndrome

Sepsis/pneumonia

Polycythaemia

Acidosis

Hypovolaemic states

Hypothermia.

Respiratory examinationTachypnoea, retractions, nasal flaring, and grunting generally indicate apulmonary cause. However, in cardiac conditions with significant left-to-rightshunt these symptoms may also occur. In cyanotic heart disease andmethaemoglobinaemia, the respiratory rate may be normal. Apnoea andcyanosis may be due to sepsis, asphyxia, or seizures.


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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 atresiagenerally gives rise to significant retractions at birth, but the symptoms arerelieved by an oral airway. Cyanosis due to parenchymal lung disease isassociated 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 examinationNormal heart rate in full-term newborns is approximately 120 beats perminute (bpm), with a range of 100 to 140 bpm. During quiet states it may beas low as 90 bpm. Heart rates >160 bpm during quiet state are abnormal. Insupraventricular tachycardia, heart rates >200 bpm are usually seen. Heartrate variability is also important: in severe sepsis and asphyxia, the beat-to-beat 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.


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It is important to record the type and quality of murmurs. The location ofapical impulse should be noted to rule out dextrocardia. Precordial thrillindicates significant murmur of grade >3/6. Precordial hyperactivity isgenerally due to increased ventricular activity. It should be remembered thatnot all heart murmurs are pathological and that many cardiac conditions do

not cause murmurs.

Abdominal examinationThe abdomen may appear scaphoid in congenital diaphragmatic hernia.

Abdominal distension due to bowel obstruction or ascites can give rise torespiratory distress. Hepatosplenomegaly with ascites and hydrops fetalis, asseen in cases of severe haemolytic disease of the newborn, can lead tosevere respiratory distress. Hepatomegaly can co-exist with pulmonarycongestion 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 examinationHypotonia is one of the earliest signs of:

Sepsis

Asphyxia

Metabolic disorders.

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

CXR

CXR is integral to initial assessment of respiratory distress.[4] [5] [6] Thelocation of stomach, liver, and heart should be ascertained to rule outdextrocardia 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.
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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.

The commonly described cardiac silhouettes in congenital heart disease are:

o "Egg on end" appearance of transposition of the great vessels

o "Snowman" sign (a rounded, figure-of-eight-like cardiac contour) of total anomalous pulmonary

venous return

o 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 withinadequate ductal shunting. Decreased pulmonary vascular markings mayalso 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 onboth sides. Diaphragmatic paralysis (more commonly seen on the right side)is manifested by elevation of the right hemidiaphragm by more than 2intercostal spaces compared to the left side. This may simulate right lower

lobe atelectasis. Hyperinflated lung fields are seen occasionally in lobaremphysema or cystic lesions of lungs. The prevalence of spontaneous airleaks giving rise to pneumothorax View image and pneumomediastinum ViewimageView image in full-term newborns is approximately 1% to 2%.

Some characteristic pulmonary findings on CXR are associated with specificpathologies:

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
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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

image

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 thoracicdystrophy, the thoracic cage will be small and narrow. The thorax may have abell-shaped appearance in infants with severe hypotonia. Fractures of theribs, humerus, or clavicles should be looked for following difficult vaginaldeliveries, as infants may develop respiratory distress from the pain and

splinting of the chest.

UltrasoundUltrasound examination of diaphragmatic motion during spontaneousbreathing can detect paradoxical motion in diaphragmatic paralysis. Pleuraleffusion, eventration of the diaphragm, and size/location of the liver/spleencan also be determined.

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

Pulse oximetryPulse oximetry monitoring is recommended for all infants with respiratorydistress and cyanosis, since clinician assessment of colour at birth is wellrecognised to vary.[7] It is an accurate, reliable, and non-invasive method formonitoring oxygen saturation in infants.[8] [9][10] [11] In severe cyanosiswith respiratory distress, both preductal and postductal oxygen saturationsshould be monitored to detect the gradient across the ductus arteriosus. Forthis procedure, the pulse oximeter probes should be placed over the righthand and a lower extremity. Pulse oximetry screening has been shown to be
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helpful in early detection of congenital cardiovascular malformations in thenewborn nursery.[12]Acrocyanosis is a common finding in the first fewminutes after birth. This generally resolves spontaneously. If a low-pulseoximeter reading persists, it may be appropriate to proceed to a hyperoxiatest.

Pulse oximetry screening is helpful in the early detection of congenitalcardiovascular malformations in the newborn nursery, and may detect life-threatening malformations before symptoms develop.[12] [13] There is asignificant body of evidence suggesting that early detection of congenitalcardiovascular heart disease (CCHD) through pulse oximetry monitoring is aneffective strategy for reducing morbidity and mortality rates in young children.[13]A working group has identified strategies and made recommendations forhospitals and birthing centres to implement the use of pulse oximetry forscreening for CCHD in newborns, which should complement physical

examinations.[13] ]

Hyperoxia testThe hyperoxia test is a further clinical tool to differentiate between cardiacand pulmonary aetiologies. It is indicated if the pulse oximeter reading is


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Laboratory testsABG can help in determining the oxygenation, ventilation, and acid-basestatus of the infant. In methaemoglobinaemia, the PO2 is normal even withcyanosis.

FBC with differential count is an important test to rule out polycythaemia,anaemia, neutropenia, leukopenia, abnormal I:T (immature to total neutrophilcount) ratio, and thrombocytopenia as signs of sepsis.

If sepsis is suspected, a blood culture should be obtained and spinal tapperformed 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 metabolicacidosis with cyanosis and respiratory distress.

Calcium and magnesium levels should be obtained where other causes havebeen ruled out. Hypocalcaemia and hypomagnesaemia are both associatedwith CNS irritability and seizures.

Metabolic screening of urine and drug screening of urine/meconium shouldbe performed as clinically indicated.

ECGAn ECG is an important test, but of limited value except in certain specificconditions.[15] [16]Normally, there is right ventricular predominance in thenewborn, and many cases of cyanotic congenital heart disease (CHD) willhave similar findings. Left axis deviation with left ventricular dominance isseen in tricuspid atresia or pulmonary atresia with an intact ventricularseptum. Left axis deviation is frequently associated with right ventricularhypertrophy as seen in arteriovenous canal malformation. ECG is importantin the diagnosis of arrhythmias.

EchocardiogramEchocardiogram is the definitive test in the diagnosis of congenital cardiaclesions and pulmonary HTN. A technician who is trained in performingechocardiograms in newborns and a paediatric cardiologist are required forthis procedure.
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Differential diagnosisSort by: common/uncommonorcategory

Commonhide allTransposition of great arteries (TGA)

see our comprehensive coverage of Overview of congenital heart disease

History Exam 1st test

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

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

echocardiogram: definitive diagno

reveals abnormal position of the ao

pulmonary arteries, abnormal cardi

and function

CXR: "egg on a string" appearance

cardiomegaly and increased pulmo

markings

Tetralogy of Fallot (TOF)

see our comprehensive coverage of Tetralogy of Fallot


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, tracheo-

oesophageal 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

prominent right

ventricular heave and

systolic ejection

murmur at left sternal

border are usually

present

CXR: boot-shaped heart

echocardiogram: diagno

revealing characteristic c

and function

Pulmonary atresiasee our comprehensive coverage of Overview of congenital heart disease


variable presentation depends on the presence

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

murmur of patent

ductus arteriosus is

the only common

finding

CXR: may reveal decreased pulmonary

vascular markings

echocardiogram: definitive diagnostic test t

reveals pulmonary atresia, abnormal cardiac

anatomy and function

Respiratory distress syndrome (RDS)

see our comprehensive coverage of Acute respiratory distress syndrome


generally occurs in preterm infants due to surfactant deficiency;

antenatal history may reveal immature lung profile in the amniotic

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

tachypnoea, nasal flaring,

grunting, and retractions with

cyanosis; in severe cases,

diminished air entry is present

on chest auscultation

CXR: reticular g

bronchograms;

cases, decrease

volumeMore
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RDS are born to mothers who did not receive antenatal

corticosteroids

Transient tachypnoea of the newborn (TTN)


higher incidence among full-term newborns born by

elective caesarean section; tachypnoea is the presentingsymptom with increased FiO2 requirement; self-limiting

problem; generally resolves within the first 3 days;

cyanosis may occur in severe cases

tachypnoeic infant with

reasonable air entrybilaterally upon

auscultation

CXR: good lung volume wit

markings along with fissura

markings on the right horizo

fissureMore

Persistent pulmonary hypertension of the newborn (PPHN)


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

loud S2 and right

ventricular heave

CXR: depends on the precipitatin

aspiration syndrome, sepsis/pneu

diaphragmatic hernia

echocardiogram: definitive diag

pulmonary artery pressure, tricus

ventricular size and function

Pneumothorax

see our comprehensive coverage of Pneumothorax


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

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

CX

co

vis

ma

Aspiration pneumonia

see our comprehensive coverage of Aspiration pneumonia

History Exam 1st t

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

tachypnoea, grunting, retractions,

and cyanosis are present; on

auscultation diminished air entry,

rales, and rhonchi may be evident

Pneumonia

see our comprehensive coverage of Overview of pneumonia

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risk factors include a hx of prolonged rupture of

membranes, chorioamnionitis, or positive maternal

group B beta streptococcus (GBS) screen; congenital

pneumonia due to viruses or bacteria is rare; early-onset

GBS sepsis may present as GBS pneumonia with

apnoea and circulatory collapse within the first week of

life

respiratory distress with tachypnoea,

retractions, and grunting may be present;

auscultation often reveals rales, rhonchi,

and diminished air entry in a few cases;

apnoea may be the only presenting

symptom in some cases

CXR: diagnostic

GBS sepsis may

distress syndrom

FBC: abnormal;

thrombocytopen

amounts of imma

abnormal I:T (im

ratio (>0.2) is pre

blood culture: m

Pulmonary oedema

History Exam 1st test Othe

generally due to congestive cardiac

failure; underlying cardiac disease,

AV malformation, or severe anaemia

may be present

tachypnoea, tachycardia, and

hepatomegaly are usually present;

severe pallor or signs of AV

malformation present (bruit)

CXR: fluffy infiltrates or hazy

lung fields and associated

cardiomegaly

Congenital cystic adenomatoid malformation (CCAM)


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

tachypnoea and mild cyanosis are

present in many infants;

diminished air entry on the

affected side will be present

CXR: multiple c

opaque areas in

lungMore

Congenital diaphragmatic hernia (CDH)History Exam 1st test Othe

most cases are diagnosed by antenatal

ultrasound; more common on the left

side; most cases are symptomatic at

birth with severe cyanosis; some have

minimal symptoms and are diagnosed

later in the neonatal period or infancy

scaphoid abdomen at birth,

diminished air entry on the left side

of chest, with cyanosis at birth are

typical features; heart sounds may

not be heard on the left side of

chest due to mediastinal shift

CXR: diagnostic with intestinal

gas pattern in the left

hemithorax with mediastinal

shiftMore

ABG: severe hypoxia and

hypercarbia

Upper airway obstruction

see our comprehensive coverage of Central airway obstruction

History Exam 1st test Other t

most become symptomatic shortly after birth

with no significant cyanosis; symptoms worsen

during feeding, with stridorous breathing; those

with vocal cord paralysis will have a weak or

non-existent cry; cyanosis may develop; risk of

submandibular, suprasternal, and

supraclavicular retractions are

characteristic of upper airway

obstruction; infants with choanal

atresia or stenosis may have mild to

CXR and lateral

view of

neck:sometimes

the airway calibre
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aspiration during feeding; infants with vocal

cord paralysis may have CNS conditions, such

as meningomyelocele with Arnold-Chiari

malformation and hydrocephalus

moderate symptoms depending on

whether unilateral or bilateral;

inability to pass nasogastric tube is

diagnostic

can be

demonstrated

Polycythaemia

History Exam 1st te

polycythaemia is common in insulin-dependent

diabetes mellitus, small for gestational age

infants, and twin-to-twin transfusion; maternal hx

of diabetes and HTN during pregnancy are

frequent; slightly higher incidence of transienttachypnoea of the newborn may be present

either large for gestational age or small for gestational age infants

who may appear cyanotic but have normal ABGs; some infants may

have transient tachypnoea of the newborn or mild persistent

pulmonary HTN of newborn; infants appear ruddy and plethoric, and

have a higher incidence of acrocyanosis; clinical examination may benormal or findings of transient tachypnoea of the newborn may be

present

Asphyxia


hx of fetal distress with perinatal asphyxia and low

Apgar scores requiring vigorous resuscitation; hx of

seizures or apnoea may be the presenting symptom;

may lead to hypoxic ischaemic encephalopathy

neurologically depressed, hypotonia, subtle

seizure activity, with good aeration to both

lung fields; apnoea or hypoventilation

(bradypnoea) is the cause for cyanosis

CXR: normal

serum

creatinine: elev

liver function

tests: raised AL

Methaemoglobinaemia (met-Hb)


usually well in spite of characteristic skin

discoloration, termed pseudocyanosis; condition may

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

characteristic blue colour, dyspnoea, mental

state changes; arterial blood with elevated

methaemoglobin levels has a characteristic

chocolate-brown colour; pulse oximetry is

unreliable

ABG: normal

PaO2More

Hypoglycaemia

see our comprehensive coverage of Non-diabetic hypoglycaemia


either a small for gestational age or

large for gestational age infant born

to a diabetic or hypertensive woman

may be jittery or may have seizures; clinical examination may

be normal or may reveal hypo- or hypertonia; cyanosis is

usually due to apnoea or transient persistent pulmonary

hypertension of newborn

blood sugar: s

hypoglycaemic

(


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Neonatal sepsis

see our comprehensive coverage of Sepsis


hx of premature rupture of membranes,

chorioamnionitis, or intrapartum maternal

fever may be present; may present with

apnoea and cyanosis or with circulatory shock;

history of lethargy, poor feeding, or emesis,

temperature instability and hypo- or

hyperglycaemia may be present

infants may be hypotonic with normal or

poor peripheral perfusion and hypotension;

cyanosis in most cases is due to

hypoventilation or apnoea and in some cases

associated persistent pulmonary HTN of

newborn is also present; chest examination

is normal

FBC: leukopenia,

neutropenia, elevated

ratio, thrombocytopen

ESR: elevated

blood culture: pathog

organism cultured

Uncommonhide allTotal anomalous pulmonary venous return (TAPVR)



clinical condition depends on the

presence of pulmonary venous

obstruction; pulmonary congestion

(pulmonary oedema) with respiratory

distress and cyanosis can be mistaken

for interstitial pneumonitis

prominent right ventricular heave; widely

split S2; systolic ejection murmur at the left

upper sternal border may occur;

hepatomegaly is common; response to

alprostadil (prostaglandin E1) infusion is

generally ineffective to minimal

CXR: cardiomegaly; "snowma

congestionMore

echocardiogram: definitive d

reveals pulmonary venous dra

characteristic cardiac anatom

Hypoplastic left heart syndrome or single ventricle physiology states



most cases are diagnosed prenatally; more common in boys; if

unrecognised at birth, infants become symptomatic at the time of the

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

right ventricular

heave and single

S2 are present

CXR: cardiomegaly with s

pulmonary congestion

echocardiogram: definitiv

that reveals abnormal card

function

Tricuspid atresia

see our comprehensive coverage of Overview of congenital heart diseaseHistory Exam 1st test

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

systolic ejection

murmur at the left upper

sternal border and

prominent left

ventricular impulse

CXR: depends on the degree

blood flow and may present w

congestion

echocardiogram: definitive d

that reveals tricuspid atresia;
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when the ductus arteriosus closes atrium and dilated right ventri

Truncus arteriosus (TA)



mild to moderate cyanosis with

or without respiratory distress

may present in first week of

life; velocardiofacial syndrome

(22q deletion) is present in 30%

to 50% of TA cases

hyperdynamic precordium with loud single

S2; loud systolic and diastolic murmur with

wide pulse pressure and bounding pulses;

respiratory distress depends on the degree of

pulmonary congestion and worsens when

pulmonary vascular resistance drops after the

first 2 to 3 days; CHF may develop as a result

CXR: cardiomegaly and increased

pulmonary blood flow

echocardiogram: definitive diagn

may reveal abnormal morphology

functional derangement of the trun

Pulmonary haemorrhage


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

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

CXR: fluffy infiltrates

bilaterally

ABG: severe hypoxia a

hypercarbia

FBC: normal but in som

cases thrombocytopen

may be present

DIC screen: prolonged

and PTT; low fibrinogen

and low platelet count w

increase in D-dimers

Pulmonary hypoplasia

History Exam 1st test Oth

hx of oligohydramnios and respiratory

distress soon after birth are present;

higher incidence of pneumothorax in

these cases

tachypnoea, cyanosis, and fair to poor

aeration of lungs present; S2 is loud

with associated pulmonary

hypertension

CXR: decreased lung

volume

ABG: hypoxia and

hypercarbia

Pulmonary lymphangiectasia

History Exam 1st test Other tests

respiratory distress

and cyanosis in the

newborn

tachypnoea and cyanosis in a full-term

newborn; air entry will not be diminished;

occasionally rales may be heard

CXR: diffuse reticular

opacities bilaterally

CT

inv

lun

lym

inte

Tracheo-oesophageal fistula (TOF)/oesophageal atresia (OA)
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see our comprehensive coverage of Tracheo-oesophageal fistula


hx of polyhydramnios and large amount

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

lesions, TOF, renal and limb anomalies)

respiratory distress with frothy secretions

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 tracheo-

oesophageal fistula; respiratory distress is

due to secretions in the upper airway and

aspiration

CXR: diagnostic with an

indwelling orogastric tube

curled in the upper

oesophageal pouchMore

Congenital lobar emphysema


generally asymptomatic at birth;

respiratory distress becomes

progressively worse over the next

few days

tachypnoea and cyanosis are present;

diminished air entry over the affected

side (commonly over left upper chest)

CXR: hyperinflation of the affected lob

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


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

needle aspiration or chest tube insertion occursto remove the fluid; minor degrees of pleural

effusion are seen in pneumonitis, meconium

aspiration syndrome, and persistent pulmonary

HTN of newborn

in severe cases, diminished air

entry will be present on the

affected side; in bilateral

effusions, as seen in hydrops

fetalis, it will be difficult toventilate the lungs unless the

pleural fluid is evacuated

CXR: opacity on the lateral borde

with blunting of the cardiophrenic

unilateral effusions; in severe cas

mediastinal shift to contralateral s

effusion is unilateral; in severe bi

effusions, the whole chest is opaq

indistinguishable cardiac borderM

Arteriovenous malformation

History Exam 1st test Other tests

CHF, large head due to

hydrocephalus, seizures

bruit may be heard over the

cranium on auscultation

CXR: may show

cardiomegaly

angiogram

angiogram

1
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