Ghai Essential Pediatrics8th

		Newborn Infants -
	Table 8.17: Choice of initial antibiotic therapy
Clinical situation	Septicemia and pneumonia	Meningitis
Community acquired; resistant	Ampicillin or penicillin and gentamicin	Cefotaxime and gentamicin
strains unlikely	(First line)
Hospital acquired or when there is	Ampicillin or cloxacillin and amikacin	Cefotaxime and amikacin
a low to moderate probability of	(Second line)
resistant strains
Hospital acquired sepsis or when	Cefotaxime and amikacin	Cefotaxime and amikacin
there is a high probability of	(Third line)
resistant strains

Therapy might be modified based on culture report

gentamicin initially and is clinically well after 3 days, the physicianmayconsideranindividualbasisswitchingover to oral amoxycillin along with single-dose intramuscular gentamicin therapy for the rest of the course.

Monitoring

Intensive care and monitoring is the key determinant of improved survivalof neonates. The elements of monitoring in sepsis are not different from those in other life­ threatening conditions. Proper monitoring of sick babies enables care providers detection of complications at the earliest. The periodicity of documenting the various parameters should be individualized.

Prognosis

The outcome depends upon weight and maturity of the infant, type of etiologic agent, its antibiotic sensitivity pattern; and adequacyof specificand supportivetherapy. The early-onset septicemia carries higher risk of adverse outcomes. The reported mortality rates in neonatal sepsis in various studies from India ranges between 45-58%. The institutionof sepsis screen for early detection of infection, judicious and early antimicrobial therapy, close moni­ toring of vital signs and intensive supportive care are the most crucial factors responsible for a better outcome.

Suggested Reading

Sankar MJ, Agarwal R, Deorari AK, Paul VK. Sepsis in the new­ born. Indian J Pediatr. 2008 Mar;75:261-6

Necrotizing Enterocolitis

Necrotizing enterocolitis (NEC) occurs among smaller premature infants, often those less than 32 week. The clinical picture mimicks neonatal septicemia because of the presence of abdominal distension, apnea, bradycardia, instability of temperature, cyanosis and lethargy.

NEC is believed to result from interaction of several factors such as gut immaturity, mucosal injury due to hypoxia-ischemia, milk feeding and infection. Antenatal steroids and breastfeeding protect against NEC. Delaying enteral feeding does not prevent NEC.

Clinical Features

The illness usually develops after the first week of life. The course may be very fulminant with death occurring in a few hours, mortality rate being around 40-50%.

Clinicalmanifestations may bedescribedin three stages: Stage 1. Suspected NEC: Unstable temperature, apnea, bradycardia, lethargy, mild abdominal distension, vomiting. Frank or occult, blood may be present in stools. X-ray shows mild intestinal distension.

Stage 2. Clinical signs as similar to stage 1. Bowel sounds are diminished with or without abdominal tenderness. Pneumatosis intestinalis (gas in intestinal wall) and dilatation of intestines are seen on abdominal X-ray (Fig. 8.41).

Stage 3. In addition to the above, the infant is severely sick with hemodynamic instability. There are frank signs of peritonitis with abdominal wall redness. Pneumo­ peritoneum may occur due to intestinal perforation.

Management

Oral feeding should be withheld. A nasogastric tube is inserted to relieve distension and to aspirate stomach contents. Fluids and electrolytes in adequate quantities should be administered. Parenteral nutrition may be administered.

Fig. 8.41: Necrotizing enterocolitis showing dilated bowel loops and pneumatosis intestinalis (arrows)

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__________________________ _

The blood, cerebrospinal fluid, urine and stools are cultured. Shock is managed by replacement of fluids and use of vasopressoragents.Plasma andplatelettransfusion may be necessary to prevent bleeding tendency.

Perforation is suggested if there is free intra-abdominal gas and liver dullness is obliterated. Surgical intervention is required in these cases.

Sequelae

Intestinal strictures may develop in survivors. These manifest with bloody stools, vomiting and abdominal distention. Shortened bowel leads to malabsorption.

PERrNATAL ASPHYXfA

Table 8.20: Neurological patterns of hypoxic ischemic encephalopathy

Premature newborns

Selective subcortical neuronal necrosis

Periventricular leukomalacia

Focal and multifocal ischemic necrosis

Periventricular hemorrhage or infarction

Term newborns

Selective cortical neuronal necrosis

Status marmoratus of basal ganglia and thalamus Parasagittal cerebral injury

Focal and multifocal ischemic cerebral necrosis

Perinatal asphyxia is an insult to the fetus or newborn due to a lack of oxygen (hypoxia) and/or a lack of per­ fusion (ischemia) to various organs. It is often associated with tissue lactic acidosis and hypercarbia.

There is no universally accepted definition of perinatal asphyxia. TheAmerican AcademyofPediatricsCommittee on Fetus and Newborn has suggested essential criteria (Tables 8.18 and 8.19) for defining perinatal asphyxia.

Table 8.18: Essential criteria for perinatal asphyxia

Prolonged metabolic or mixed acidemia (pH <7.0) on an umbilical arterial blood sample

Persistence of Apgar score of 0-3 for >5 min

Neurological manifestations, e.g. seizures, coma, hypotonia or hypoxic ischemic encephalopathy (HIE) in the immediate neonatal period

Evidence of multiorgan dysfunction in the immediate neonatal period

In the absence of such quantification, it is better to use the term 'neonatal depression', which refers to a condition of the infant in the immediate postnatal period (approxi­ mately 1st hr) without making any association with objective evidence.

NationalNeonatology Forum of India (NNF) and WHO use an Apgar of 0-3 and 4-7, at 1 min, to define severe and moderate birth asphyxia respectively (1985). For the community settings NNF defines asphyxia as absence of cry at 1 min and severe asphyxia as absent or inadequate breathing at five minutes.

Neuropathology

These differ according to gestation (Table 8.20) and are of the following main types:

Term

Selective neuronal necrosis involves cerebral cortex, hippocampus, basal ganglia, cerebellumandanteriorhorn cells of spinal cord. Seen predominantly in term infants and depending on site, this manifests clinically as diminished consciousness, seizures and abnormalities of feeding, breathing, etc. Parasagittal area is a watershed area for many arteries and is vulnerable to ischemia resulting in proximal limb weakness (upper >lower) that later may develop into spastic quadriparesis. Status marmoratus is a variant of selective neuronal necrosis involving basal ganglia and thalamus, having longterm sequelaesuchaschoreoathetosis, spastic quadriparesis and retardation. Focal necroses are commonly thrombo­ embolic and involve the left middle cerebral artery.

Preterm

Selectiveneuronalnecrosisisrare in preterms; diencephalic neuronal necrosis restricted to thalamus and brainstem with orwithouthypothalamus andlateralgeniculatebody is seen. Hypoxia and acidosis followed by hyperoxia demonstrates a unique pattern of injury involving pontine nucleus and subiculum of the hippocampus.

Periventricular leukomalacia (PVL) results from hypoxic-ischemic insult leading to coagulative necrosis and infarction of periventricular white matter that is the watershed area between various arteries. Two areas

frequently involved are the posterior white matter, involving the occipital radiation at trigone and anteriorly around the foramen of Munro. Relative sparing of the cerebral cortex is seen due to its rich supply of arteries. Longterm sequelae of PVL include spastic diplegia and

	Table 8.19: Multiorgan dysfunction in perinatal asphyxia
Central nervous system	Hypoxic ischemic encephalopathy, cerebral edema, longterm neurological sequelae
Pulmonary	Pulmonary hypertension, meconium aspiration, surfactant disruption
Renal	Acute renal failure
Metabolic	Metabolic acidosis, hypoglycemia, hypocalcemia, hyponatremia
Gastrointestinal	Necrotizing enterocolitis, hepatic dysfunction
Hematological	Thrombocytopenia, disseminated intravascular coagulation

Newborn Infants -

quadriplegia (lower limbs >upper limbs) and visual impairment. Posthemorrhagic infarcts are usually associated with severe intraventricular bleeds and result from venous infarction due to occlusion of medullary and terminal veins by the large bleed. Other lesions include small infarcts secondary to blocking of end arteries resulting in porencephaly, hydrancephaly or multicysti­ cencephalomalacia.

Diagnosis and Approach

Hypoxia is an evolving process that starts at the onset of the insult and continues after resuscitation and thereafter manifests in form of sequelae. Management thus depends on which point in this evolution it is detected; with the preventiveapproachbeginning in the prenatalperiod and then continuing in the form of a long followup much after the stabilization of the initial condition.

A wide spectrum of clinical manifestations is seen depending on the severity of injury. These manifestations change over time and are clinically noted in babies of gestational age more than 36 weeks by classification on the basis of Levenestages of HIE (Table 8.21).

HIE staging helps predict evolution of the disease and longterm outcome. Babies with stage 1 hasuniformlygood prognosis. Adverse neurological outcomes are present in 20% of babies with stage 2 HIE. In stage 3 HIE, half of the

neonates die and remaining half tend to have poor neuro­ development outcomes.

Post-Resuscitation Management of an Asphyxiated

Baby (Fig. 8.42)

i.Temperature: Maintainnormaltemperatureofthebaby and avoid hyperthermia. In resourceful setting, moderate induced hypothermia (core temperature of 33° to 34° C) reduces the death or severe neuro­ developmental handicap. However, the efficacy and safetyoftherapeutichypothermiahasnot beenproved in resource restrictedsetting (in absence of intensive care).

ii.Oxygen: Both hypoxia and hyperoxia can damage neurons. Oxygen saturations are maintained between

90% to 95%. CO2 concentration in ventilated babies should be maintained between 40 and 50 mm Hg as hypocarbia as well as hypercarbia are detrimental to brain.

iii.Perfusion: Cerebral perfusion in asphyxiated babies is in 'pressure passive' state means there is loss of auto­ regulation and blood supply to the brain is entirely dependant on BPs; it decreased when BP falls and increases when BP rises. Therefore, to maintain normal perfusion pressure, a systemic mean arterial

	Table 8.21: Levene classification for hypoxic ischemic encephalopathy
Feature	Mild	Moderate	Severe
Consciousness	Irritablity	Lethargy	Comatose
Tone	Hypotonia	rviarked hypotonia	Severe hypotonia
Seizures	No	Yes	Prolonged
Sucking/respiration	Poor suck	Unable to suck	Unable to sustain spontaneous respiration

Modified from: Levene MI.The asphyxiated newborn infant. In Levene Ml, Lilford RJ, ed. Fetal and neonatal neurology and neurosurgery. Churchill Livingstone, Edinburgh 1995;405-26

Need for positive pressure ventilation for :"..30 seconds, chest compression or adrenaline

Apgar <7 at 5 minutes

			[±ransfer to NICU a d monitor the baby
		:t
					Abnormal tone, activity, or
rHemodynamicauy stable; norma1
l_:one, activity, sensorium and no seizures					sensorium and presence of seizures
	-	i

Maintain normal body temperature. avoid hyperthermia Maintain normal oxygenation and ventilation by judicious use of oxygen and/or mechanical ventilation Ensure normal perfusion by saline boluse(s) and/or vasopressors, as required

Maintain normal blood glucose by infusion of IV dextrose Treat for seizures, if present

Monitor the baby (neurological and cardiorespiratory examination: renal function)

Fig 8.42: Post-resuscitation management of an asphyxiated baby

- Essential Pediatrics

pressureof 45-50 mm Hg (term), 35-40 (1-2kg weight) and 30-35 mm Hg (<1 kg weight) is required. Judicious use offluid boluses anduseof vasopressors help maintain BP. Hyperviscosity due to poly­ cythemia should be corrected by partial exchange.

iv.Glucose: Levels between 75-100 mg/dl are recom­ mended. Hyperglycemia enhances cerebral edema and compromise perfusion, while hypoglycemia potentiates excitotoxic damage. Hypoglycemia is commonly seen in asphyxiated infants and the infant must be regularly monitored.

v.Metabolic profile: Hypocalcemia and electrolyte disturbances should be regularly looked for until stabilization of baby and corrected as indicated.

vi.Seizures: 20%-50% of infants with HIE develop sei­ zures during day 1 or 2. Seizures are commonly subtle or focal or multifocal. Metabolic disturbances such as hypoglycemia, hypocalcemia and hyponatremia must be ruled out. Seizures should be treated with anti­ epileptic drugs (AEDs) such as phenobarbitone and phenytoin. The seizures may be intractable initially but usually tend to burn out by 48 hr. Subtle seizures lasting for brief duration need not be treated.

Once the baby is seizure free for 3-4 days, AEDs are stopped in the same order as they were started, except phenobarbitone. Phenobarbitone is stopped at discharge if neurological examination is normal and baby is feeding well on breast. If neurological examination is not normal, then phenobarbitone is continued until one month. At one month if baby is normal neurologically, phenobarbitone is tapered off over a couple of days. If neurological function is abnormal but EEG shows no seizure activity, tapering of phenobarbitone may still be tried. If EEG shows seizure activity, reevaluation is done at 3 months.

Prognosis

The following features predict a poor outcome:

•Lack of spontaneous respiratory effort within 20-30 minutes of birth is associated with almost uniform mortality

•HIE stage 3

•Abnormal neurological findings persisting beyond the first 7-10 days of life

•Oliguria (<1 ml/kg/day) during the first 36 hr

Thus all thesebabiesshouldhave regularfollowup with monitoring of neurodevelopmental milestones to detect any deficits early and to intervene effectively.

Suggested Reading

Agarwal R Jain A, Deorari AK, Paul VK.Post-resuscitation manage­ ment of asphyxiated neonates. Indian J Pediatr 2008;75:175--80

RESPIRATORY DISTRESS

Respiratory distress in the neonate is a common problem and it can be a serious neonatal emergency. Respiratory distress is said to be present when tachypnea (RR >60 per min) is accompanied by chest retractions and or grunt. It can be due to respiratory (Table 8.22) and non-respiratory causes (Table 8.23). Early recognition and prompt treatment is essential to improve outcomes.

Approach

Respiratory distress in a neonate can be recognized by the presence of varying combinations of tachypnea (RR >60/min), chest retractions, grunting, flaring of ala enasi and cyanosis. The gestation, age at onset, severity ofdistress andpresence of associatedclinicalfeatures help in arriving at diagnosis. It should be noted that chest retractions are mild or absent in respiratory distress due to non-respiratory causes.

Respiratory causes. Conditionslisted in Tables 8.22 and 8.23 can occur both in preterm and term babies. However, if a preterm baby has respiratory distress within the first few hours of life the most likely cause is respiratory distress syndrome (RDS).Similarlyif a term baby born to a mother with meconium stained liquor develops respiratory distress within the first 24 hr, the most likely cause is meconium aspiration syndrome (MAS). Atermbabywith uncomplicatedbirthdevelopingtachypnea in the first few hours of birth is likely to have transient tachypnea of newborn. Presence of suprasternal recessions with or without stridor indicates upper airway obstruction.

	Table 8.22: Pulmonary causes of respiratory distress
Cause	Time ofonset	Remarks
Respiratory distress syndrome	First 6 hr of life	Common in preterm neonates
Meconium aspiration syndrome	First few hr of life	Common in term, post-term and small for date babies; history
		of meconium stained liquor
Pneumonia	Any age	Often bacterial
Transient tachypnea of newborn	First 6 hr after birth	Tachypnea with minimal distress; lasts for 48-72 hr
Persistent pulmonary hypertension	Any age	Severe distress; cyanosis
Pneumothorax	Any age	Sudden deterioration; usually during assisted ventilation
Tracheoesophageal fistula,	Any age	May show associated malformations; polyhydramnios in
diaphragmatic hernia, lobar		esophageal atresia
emphysema

Table 8.23: Non-pulmonary causes of rapid breathing

Cardiac	Congestive heart failure; congenital heart
	disease
Metabolic	Hypothermia, hypoglycemia, metabolic
	acidosis
Central nervous	Asphyxia, cerebral edema, hemorrhage
system
Chest wall	Asphyxiating thoracic dystrophy,
	Werdnig-Hoffman disease

Newborn Infants -

Clinical Features

Respiratory distress usually occurs within the first 6 hr of life. Clinical features include tachypnea, retractions, grunting, cyanosis anddecreased air entry. Diagnosis can be confirmed by chest X-ray. Radiological featuresinclude reticulogranular pattern, ground glass opacity, low lung volume, air bronchograrn (Fig. 8.43) and white out lungs in severe disease.

Cardiac disease. Cardiac etiology for respiratory distress shouldbe suspectedif a neonate with distress hascyanosis or hepatomegaly. Congenital heart disease and cardio­ myopathiesorrhythmdisorderscanpresent as congestive cardiac failure in the neonatal period. Transposition of great vessels (TGV) and hypoplastic left heart syndrome usuallypresent on day one with progressive distress. Most other cardiac conditions present after the first week of life. A preterm neonate having a systolic murmur with tachy­ pnea and hepatomegaly is likely to have patent ductus arteriosus (PDA).

Neurological causes. Neonates with birthasphyxia,cerebral hemorrhage, or meningitis can present with tachypnea and respiratory distress. These neonates are usually lethargic with poor neonatal reflexes.

Respiratory Distress Syndrome (RDS) or

Hyaline Membrane Disease (HMD)

RDS is common in preterm babies less than 34 weeks of gestation. The overall incidence is 10-15% but can be as high as 80% in neonates <28 weeks. In addition to prematurity, asphyxia, acidosis, maternal diabetes and cesarean section can increase the risk of RDS.

Etiopathogenesis

In RDS, the basic abnormality is surfactant deficiency. Surfactant is a lipoprotein containing phospholipids like phosphatidylcholine and phosphatidylglycerol and proteins. Surfactant is produced by type II alveolar cells of lungs and helps reduce surface tension in the alveoli. In the absence of surfactant, surface tension increases and alveoli tend to collapse duringexpiration. During inspira­ tion more negative pressure is needed to keep alveoli patent. There is inadequate oxygenation and increased work of breathing. Hypoxernia and acidosis result in pul­ monary vasoconstriction and right to left shunting across the forarnen ovale. This worsens the hypoxernia and the neonate eventually goes into respiratory failure. Ischernic damage to the alveoli causes transudation ofproteins into the alveoli that forms hyaline membrane. Surfactant pro­ duction starts around20 weeks of life andpeaks at 35 week gestation.Thereforeanyneonate lessthan 35 week isprone to develop RDS.

Fig. 8.43: Moderate to severe hyaline membrane disease. Note homogenous opacification of lungs obscuring heart borders and presence of air bronchogram (arrows)

Management

Neonates suspected to have RDS need to be cared for in neonatal intensive care unit with IV fluids and oxygen. Mild to moderate RDS can be managed with continuous positive airway pressure (CPAP). CPAP is a non invasive modality of support where a continuous distending pressure (5-7 cm of water) is applied at nostril level to keep the alveoli open in a spontaneously breathing baby (Fig. 8.44). This is an excellent modality of respiratory support which minimizes lung injury and other compli­ cations such as air leak and sepsis. Preterm babies developing severe RDS often require mechanical ventilation. Preterm babies are at risk of lung injury by excessive pressure and high oxygen. High saturations of oxygen (above 95%) can produce retinopathy of pre­ maturity (ROP) which can blind the infant.

Since surfactant deficiency is the basis of RDS, exogenous surfactant is recommended as the treatment of choice in neonates with RDS. Surfactant is indicated in all neonates with moderate to severe RDS. The route of administration is intratracheal. It can be given as a rescue treatment (when RDS actually develops) or prophylac­ tically (all neonates less than 28 weeks irrespective of presenceorabsence of RDS).Surfactant decreases duration

- Essentlal Pedlatrl:cs____________________________

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distressin thefirst fewhoursof life thatoften deteriorates in subsequent 24-48 hr. If untreated, distress can progress to respiratory failure. Complications include pneumo­ thorax, other air leak syndromes (pneumopericardiurn, pneumomediastinurn) and persistent pulmonary hyper­ tension. Chest X-ray shows bilateral heterogeneous opacities, areas of hyperexpansion and atelectesis and air leak (Fig. 8.45).

Fig. 8.44: Continuous positive airway pressure being provided to a preterm baby

and level of support of ventilation in neonates and therefore improves outcome. Many babies can be INtubated, given SURfactant and rapidly Extubated (lnSurE approach) to CPAP (Fig. 8.44). This avoids the need for mechanical ventilation in many neonates.

RDS has generally a good prognosis if managed appropriately. Survival is as high as90% in verylowbirth weight babies (<1500 g). In the absence of ventilatory support, most neonates with severe disease will die.

Fig. 8.45: Meconium aspiration syndrome. Note hyperexpansion of lungs and heterogeneous opacities in right lung

Prevention of RDS

Administration ofantenatalsteroids tomothersinpreterm labor (<35 week) has been a major breakthrough in management of preterm infants. Antenatal steroids reduces RDS, intraventricular hemorrhage and mortality in preterm neonates (Table 8.24).

Table 8.24: Benefits ofadministering antenatal glucocortlcoids

Reduction in neonatal mortality by 40% Reduction in respiratory distress by 50% Reduction in intraventricular hemorrhage by 50%

Reduction in occurrence of patent ductus arteriosus, necrotizing enterocolitis, hemodynamic instability

Meconium Aspiratrion Syndrome (MAS)

Meconiurn staining of amniotic fluid (MSAF) occur in 10%-14% of pregnancies. Neonates born through MSAF can aspirate the meconium into the lungs and develop respiratory distress (meconium aspiration syndrome; MAS). Aspirated meconium can blockthe large and small airway causingareas of atelectasis and emphysema which can progress to develop air leak syndromes like pneumo­ thorax. Presence of atelectasis and emphysema can cause ventilation perfusion mismatch in these babies that can progress to respiratory failure. Meconium also induces chemical pneumonitis.

Clinical Features and Course

MAS usually occurs in term or post term babies and small for dates babies. Infants usually develops respiratory

Management

Clinical course in these babies can be complicated by severe pulmonary hypertension. A good supportive care in terms of maintenance of normal body temperature, blood glucoseand calcium levels, ensuringanalgesiaand avoiding unnecessary fiddling pay good dividends. Oxygenation and ventilation is maintained by judicious use ofoxygenand mechanical ventilation.With ventilatory support, 60-70% neonates survive, but in the absence of ventilatory support, mortality is high in severe disease.

Persistent Pulmonary Hypertension (PPHN)

Itiscausedby apersistentelevationinpulmonaryvascular resistance resulting in rightto left shuntacrossthe forarnen ovaleand/or ductus.Thedisease is more common in term and post-term babies and occurs as a result of persistent hypoxia and acidosis. Hypoxia and hypercarbia cause pulmonary vasoconstriction. This increases pulmonary vascular pressure and results in right to left shunting.

Common causes include asphyxia, respiratory distress due to MAS, RDS, diaphragmatic hernia, etc. Primary pulmonary hypertension can also occur because of an abnormal pulmonary vasculature secondary to chronic intrauterine hypoxia.

The neonate usually presents with severe respiratory distress and cyanosis. It is often difficult to differentiate PPHN from cyanotic congenital heart disease. Echo­ cardiography helps in ruling out congenital heart disease and maydemonstrate rightto leftshunt acrossthe forarnen ovale.

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Ventilatory support is mandatory. Nitric oxide, a selective pulmonary vasodilator is an effective therapy.

Pneumonia

Pneumonia is a common cause of respiratory distress in both term and preterm babies and is caused by bacteria such E. coli, S. aureus and K. pneumoniae. Neonatal pneumonia may be due to aspiration or occasionally due to viral or fungal infection. Though group B streptococcal pneumonia is common in the West, it is uncommonly reported in India.

Theneonatehasfeaturessuggestive of sepsis inaddition to respiratory distress. Chest X-ray shows pneumonia (Fig. 8.46), blood counts are raised and blood culture may be positive. Treatment includes supportive care and specific antibiotic therapy. Ampicillin or cloxacillin with gentamicin is usually used. If the pneumonia is due to hospitalacquired infection, antibiotics like cephalosporins with amikacin may have to be used.

Fig. 8.46: Pneumonia. Note heterogeneous opacities in both the lung fields

Transient Tachypnea of Newborn (TTN)

Transient tachypnea of the newborn is a benign self­ limiting disease occurring usually in term neonates and is due to delayed clearance of lung fluid. These babies have tachypnea with minimal or no respiratory distress. Chest X-ray may show hyperexpanded lung fields, prominent vascular marking and prominent interlobar fissure (Fig. 8.47). Oxygen treatment is often adequate. Prognosis is excellent.

Surgical Problems

Tracheoesophageal fistula (TEF) should be suspected in any neonate with excessive frothing. Diagnosis can be confirmed by a plain X-ray with a red rubbercatheter (not infant feeding tube, it is soft and gets coiled up) inserted in stomach; the catheter generally stops at 10th thoracic

Fig. 8.47: Transient tachypnea of newborn. Note hyperinflated lungs, prominent bronchovascular markings and horizontal fissure (arrow)

vertebrae in presence of esophageal atresia. Presence of gastric bubble suggest concomitant TEF.

Diaphragmatic hernia should be suspected in any neonates who has severe respiratory distress and has a scaphoid abdomen. This condition can be detected during antenatal ultrasonography. Chest X-ray shows presence of bowel loops in the thoracic cavity.

Chronic Lung Disease (CLO) or Bronchopulmonary Dysplasla (BPD)

CLD occurs because of barotrauma and oxygen toxicity that causes damage to the alveolar cells, interstitium and blood vessels. Inflammatory mediators are released and there is increased permeability causing leakage of water and protein. In later stages, there is fibrosis and cellular hyperplasia. Severe lung damage leads to respiratory failure.Thesebabies continue to requireprolonged oxygen therapy or ventilatory support.

Pneumothorax

Presence of air in the pleural cavity (pneumothorax) is most common in babies with meconium aspiration syndrome and those being ventilated (Fig. 8.48). Transillumination of the chest can help in diagnosis. Needle aspiration or chest tube drainage is a life saving procedure in this situation.

Apnea

Apnea isdefined as cessation of respiration for 20 seconds with or without bradycardia and cyanosis or for shorter periods if it is associated with cyanosis or bradycardia. Apnea is a common problem in preterm neonates. It could be central, obstructive or mixed.

Apnea of prematurity occurs in preterm neonates between the second to fifth days of life and is because of theimmaturity of the developing brain. Central apnea can also occur because of pathological causes like sepsis, metabolic problems (hypoglycemia, hypocalcemia),

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Fig. 8.48: Tension pneumoth orax on right side displacing the mediastinum and pushing down the diaphragm

temperature instability, respiratory distress, anemia and polycythemia. Obstructive apnea can occur because of block to the airway by secretion or improper neck positioning.

Treatment is supportive and involves correction of underlying cause. Apnea of prematurity is treated with aminophylline or caffeine. Prognosis is good in apnea of prematurity. In other cases it depends on the underlying cause.

Suggested Reading

Bhutani VI<.Differential diagnosis ofneonatal respiratory disorders. In: Intensive of the Fetus and Neonate. Ed Spitzer AR. Mosby Year Book 1996;494-505

Greenough A, Roberton MRC. Respiratory distress syndrome. In: Neonatal Respiratory Disorders. Eds Greenough A, Roberton NRC, Milner AD. Arnold 1996;238-79

Singh M,Deorari AK. Pneumonia in newborn babies.IndianJ Pediatr 1995; 62:293-306

JAUNDICE

Jaundice is an important problem in the first week of life. High bilirubin levels may be toxic to the developing central nervous system and may cause neurological impairment even in term newborns. Nearly 60% of term newborn becomes visibly jaundiced in the first week of life. In most cases, it is benign and no intervention is required. Approximately 5-10% of them have clinically significantjaundice requiringuse ofphototherapyorother therapeutic options.

Physiological Versus Pathological Jaundice

Physiological jaundicerepresents physiologicalimmaturity of the neonates to handle increased bilirubin production. Visible jaundice usually appears between 24-72 hr of age.

Total serum bilirubin (TSB) level usually peaks by 3 days of age and then falls in termneonates. TSBlevels are below the designated cut-offs for phototherapy. It does not require any treatment.

Pathological jaundice is referred to as an elevation of TSB levels to the extent where treatment of jaundice is more likely to result into benefit than harm. There is no clear cut demarcation between pathological and physio­ logical jaundice. TSB levels have been arbitrarily defined as pathological if it exceeds 5 mg/dl on first day, 10 mg/ dl on second day, or 15 mg/dl thereafter in term babies. Such jaundice warrants investigation for the cause and therapeuticinterventionsuchasphototherapy. Appearance of jaundice within 24 hr, TSB levels above the expected normal range, presence of clinical jaundice beyond 3 weeks and conjugated bilirubin (dark urine staining the nappy) would be categorized under this category.

Breastfeeding Jaundice

Exclusively breastfed infants have a different pattern of physiological jaundice as compared to artificially-fed babies. Jaundice in breastfed babies usually appears between 24-72 hr of age, peaks by 5-15 days of life and disappears by the third week of life. One-third of all breastfed babiesaredetectedtohavemildclinical jaundice in the third week of life, which may persist into the 2nd to 3rd month of life in a few babies. This increased frequency ofjaundicein breastfedbabiesisnotrelatedtocharacteristics of breast milk but rather to inadequate breastfeeding (breastfeeding jaundice). Ensuringoptimumbreastfeeding would help decrease this kind of jaundice.

Breast Milk Jaundice

Approximately 2-4% of exclusively breastfed term babies have jaundice in excess of 10 mg/dl beyond third-fourth weeks of life. These babies should be investigated for prolonged jaundice. A diagnosis of breast milk jaundice should be considered if this is unconjugated (not staining nappies); and other causes for prolongation such as inadequate feeding, continuing hemolysis, extravasated blood, G6PD deficiency and hypothyroidism have been ruled out. Mothers should be advised to continue breastfeeding at frequent intervalsandTSBlevels usually decline over a period of time. Some babies may require phototherapy. Breastfeeding should not be stopped either for diagnosis or treatment of breast milk jaundice.

Clinical Estimation

Originally described by Kramer, dermal staining of bilirubin may be used as a clinical guide to the level of jaundice. Dermal staining in newborn progresses in a cephalocaudal direction. The newborn should be examined in good daylight. The skin of forehead, chest, abdomen,thighs,legs,palmsandsolesshould be blanched with digital pressure and the underlying color of skin and subcutaneous tissue should be noted.

Serum levels of total bilirubin are approximately 4-6 mg/dl (zone 1), 6-8 mg/dl (zone 2), 8-12 mg/dl (zone 3), 12-14 mg/dl (zone 4) and >15 mg/dl (zone 5) (Fig. 8.49). Yellow staining of palms and soles is a danger sign and requires urgent serum bilirubin estimation and furthermanagement.Ingeneral,theestimationofbilirubin levels by dermal zones is unreliable particularly at higher TSB levels, after phototherapy and when it is carried out by an inexperienced observer. Total serum bilirubin can be assessed non invasively by a transcutaneous handheld device.

Fig. 8.49: Dermal zones for estimation of total serum bilirubin levels

Measurement of Billrubin Levels

Newborns detected to have yellow discoloration of the skin beyond the legs, or when their clinically assessed TSB levels approach phototherapy range, should have lab confirmation of total serum bilirubin. TSB assessment has a marked interlaboratory variability.

Causes

Important causes of jaundice in neonates include:

i.Hemolytic: Rh incompatibility, ABO incompatability, G6PD deficiency, thalassemias, hereditary sphero­ cytosis

ii.Non-hemolytic: prematurity, extravasated blood, inadequate feeding, polycythernia, idiopathic, breast milk jaundice

Risk factors for development of severe hyper biliru­ binernia include:

i.Jaundice observed in the first 24 hr

ii.Blood group incompatibility with positive direct antiglobulin test, otherknownhemolytic disease (e.g. G6PD deficiency).

Newborn Infants -

iii.Gestational age 35-36 weeks.

iv.Previous sibling received phototherapy.

v.Cephalohematoma or significant bruising.

vi.Ifbreastfeeding isinadequatewithexcessiveweight loss

Approach to a Jaundiced Neonate

All the neonates should be visually inspected for jaundice every 12 hr during initial 3 to 5 days of life (Fig. 8.50). Transcutaneous bilirubin (TcB) can be used as an aid for initial screening of infants. Visual assessment (when performed properly) and TcBhave reasonable sensitivity for initial assessment of jaundice.

As a first step, serious jaundice should be ruled out. Phototherapy should be initiated if the infant meets the criteria for serious jaundice. Total serum bilirubin should be determined subsequently in these infants to determine further course of action.

Management

Investigations

The aim ofperforminginvestigations is toconfirm the level of jaundice, identify the cause and follow response to treatment.

First line

•Total serum bilirubin (and its fractions, if jaundice is prolonged or there is yellow staining of nappies): All caseswithsuspectedpathological levels eitherclinically or by trancutaneous measurements need confirmation by blood examination of serum bilirubin levels.

•Blood groups of mother and baby (if the mother is 'O' or Rh negative): detects any incompatibility

•Peripheral smear: evidence of hemolysis

Second line

•Direct Coombs test: detects presence of antibody coating on fetal RBC

•Hematocrit: decreased in hemolysis

•Reticulocyte count: increased in hemolysis

•G6PD levels in RBC

•Others: sepsis screen; thyroid function test; urine for reducing substances to rule out galactosernia; specific enzyme/genetic studies for Crigler-Najjar, Gilbert and other genetic enzyme deficiencies

Physiological Jaundice

The parents should be explained about the benign nature of jaundice. The mother should be encouraged to breastfeed frequently and exclusively. Mother should be told to bring the baby to the hospital if the baby looks deep yellow or palms and soles have yellow staining. There is no use to expose the baby to direct sunlight to reduce hyperbilirubinemia.

Any newborn discharged prior to 72 hr of life should be evaluated again in the next 48 hr for assessment of adequacy of breastfeeding and progression of jaundice.

E				s		___________	_
__	s s ent 1a 1	P ed1a t r1c			__________		_
	_ _ _ _ _ _	_ _ _ _	_ _ _	______

Perform visual assessment of Jaundice: every 12 h during initial 3 to 5 days of life.

visual assessement can be supplemented with transcutaneous bilirubinometry (TcB), if available

Step 1: Does the baby have serious jaundice*?

Step 2: Does the infant have significant jaundice to require serum billirubin measurement'?

Measure serum bilirubin and determine if baby requires Continued observation every 12 hr phototherapy or exchange transfusion (refer to Table 8.25)

Step 3: Determine the cause of jaundice and provide supportive and followup care

*Serious jaundice

a.Presence of visible jaundice in first 24 hr

b.Yellow palms and soles anytime

c.Signs of acute bilirubln encephalopathy or kernicterus: hypertonia, abnormal posturing

such as arching, retrocollis, opisthotonus or convulsion, fever, high pitched cry

Measure serum blllrubln If

a.Jaundice in first 24 hr

b.Beyond 24 hr: If on visual assessment or by transcutaneous

bilirubinometry, total bilirubin Is likely to be more than 12-14 mg/di or approaching phototherapy range or beyond

c.If you are unsure about visual assessment

Fig 8.50: Approach to an infant with jaundice

Pathological Jaundice

Term and near term neonates The AmericanAcademy of Pediatrics (AAP), has laid down criteria for managing babies with elevated serum bilirubin (Figs 8.51 for phototherapyand8.52forexchange transfusion). Both the Figs have age in hours on the X-axis and TSB levels on Y-axis. There are three curves on each Fig. representing three risk categories of babies defined by gestation and other risk factors. Risk factor refer to hemolysis, asphyxia, acidosis,lowalbuminlevel, G6PDdeficiency,hypothemia and sickness.

Preterm neonates Table 8.25 provides cutoffs for exchange transfusion and phototherapy in preterm neonates below 35 weeks of gestation.

Table 8.25: Suggested TSB cut-offs for phototherapy and exchange transfusion In preterrn Infants <35 weeks

Gestation (completed	Phototherapy	Exchange transfusion
weeks)
<28	5-6	11-14
28 to 29	6-8	12-14
30 to 31	8-10	13-16
32 to 33	10-12	15-18
34	12-14	17-19

Use postmenstrual age (for phototherapy for example, when a 29 week infant is 7 days old, use the TSB level for 30 weeks).

(Adapted with permission from Maisels et al, Jour Perinatal, 2012)

	25			25
	20	1		20
r	15			15
-	10			10 1
E			i
	5			5
F	0			0
	Birth	24 h 48 h	72 h Age96 h	5 days 6 days 7 days

Fig. 8.51: Guidelines for phototherapy in hospitalized infants of 35 or more weeks' gestation. - Infants at lower risk (>38 week and well) - Infants at medium risk (>38 week + risk factors or 35-37 6/7 week and well) - Infants at higher risk (35-37 6/7 week + risk factors)

Prolonged Jaundice Beyond 3 Weeks

This is defined as persistence of significant jaundice (10 mg/dl) beyond three weeks in a term baby. The common causes include inadequate feeding, breast milk jaundice, extravasatedblood (cephalohematoma), ongoing hemolytic disease, G6PD deficiency and hypothyroidism. One should rule out cholestasis by noting the urine and stool color and checking the level of direct bilirubin. If the baby has dark urine or significant jaundice, investigations should be initiated to rule out: