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Essential Neonatal Medicine

Sixth Edition

Sunil Sinha

Professor of Paediatrics
University of Durham
Consultant Neonatologist
James Cook University Hospital
Middlesbrough, UK

Lawrence Miall

Consultant Neonatologist, Leeds Children's Hospital
Honorary Senior Lecturer, University of Leeds
Leeds Teaching Hospitals NHS Trust
Leeds, UK

Luke Jardine

Senior Staff Specialist Neonatology, Mater Mothers' Hospital
Honorary Researcher, Mater Research
Associate Professor, The University of Queensland
Australia

This edition first published 2018
© 2018 John Wiley & Sons Ltd

Edition History
John Wiley & Sons (1e 1987; 2e 1993; 3e 2000); Wiley-Blackwell (4e 2008; 5e 2012).

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Library of Congress Cataloging-in-Publication Data
Names: Sinha, Sunil K., M.D., Ph.D., author. | Miall, Lawrence, author. | Jardine, Luke, author.
Title: Essential neonatal medicine / Sunil Sinha, Lawrence Miall, Luke Jardine.
Other titles: Essentials (Wiley-Blackwell (Firm))
Description: Sixth edition. | Hoboken, NJ : John Wiley & Sons Inc., 2018. |
    Series: Essentials | Includes bibliographical references and index.
Identifiers: LCCN 2017007280 (print) | LCCN 2017008052 (ebook) | ISBN
    9781119235811 (paper) | ISBN 9781119235774 (Adobe PDF) | ISBN
    9781119235750 (ePub)
Subjects: | MESH: Infant, Newborn, Diseases | Neonatology | Infant, Newborn
Classification: LCC RJ251 (print) | LCC RJ251 (ebook) | NLM WS 421 | DDC
    618.92/01—dc23
LC record available at https://lccn.loc.gov/2017007280

Preface to the Sixth Edition
Acknowledgements
Preface to the First Edition
Abbreviations
How to use your textbook
About the companion website
Chapter 1 The fetus, placenta and changes at birth
1. Introduction
2. Placental function
3. Fetal homeostasis
4. Fetal circulation
5. Assessment of fetal well-being
6. Screening during pregnancy
7. Fetal monitoring during labour
8. Fetal compromise
9. Acknowledgements
10. Further reading
Chapter 2 Perinatal epidemiology and audit
1. Introduction
2. Definitions of terms commonly used in perinatal medicine
3. The role of perinatal and neonatal audit
4. Classification of perinatal deaths
5. Factors affecting perinatal death rates
6. Prevention of perinatal mortality and  low birthweight
7. Changing trends
8. Further reading
Chapter 3 Multiple births
1. Introduction
2. Physiology of fertilization, implantation and placenta formation
3. Classification of multiple pregnancy
4. Assisted reproductive technology
5. Incidence of multiple pregnancies
6. Parental counselling
7. Complications of multiple pregnancy
8. Further reading
Chapter 4 Neonatal consequences of maternal conditions
1. Introduction
2. Congenital anomalies: malformations and deformations
3. Congenital anomalies associated with teratogens
4. Congenital malformation secondary to maternal infections
5. Consequences of maternal substance misuse
6. Neonatal manifestations of maternal medical diseases
7. Further reading
Chapter 5 Resuscitation at birth
1. Introduction
2. Fetal responses during labour
3. Fetal and neonatal responses to perinatal asphyxia
4. Perinatal asphyxia
5. Assessment of the infant at birth
6. Stabilization at birth
7. Resuscitation
8. Post-resuscitation care of the  asphyxiated infant
9. Further reading
Chapter 6 Examination of the newborn
1. Introduction
2. The newborn examination as a  screening test
3. Approach to the newborn examination
4. General appearance
5. Head and neck
6. Chest
7. Cardiovascular
8. Abdomen
9. Back
10. Extremities
11. Congenital abnormalities of the hips  and limbs
12. Skin disorders
13. Communication with parents
14. Further reading
Chapter 7 Birth injury
1. Introduction
2. Risk factors for birth injury
3. Injuries to the scalp, skull and brain
4. Bone and joint injuries
5. Peripheral nerve injuries
6. Soft-tissue injuries
7. Organ injuries
8. Injuries sustained in the neonatal intensive care unit (NICU)
9. Further reading
Chapter 8 Genetic disorders
1. Introduction
2. Gene structure
3. Commonly used investigations
4. Genetic variation
5. Multifactorial inheritance
6. Approach to the dysmorphic neonate
7. Prevention of congenital abnormalities
8. Further reading
Chapter 9 Infant feeding and nutrition
1. Introduction
2. Specific nutritional requirements
3. Breastfeeding
4. Artificial feeding/formulas
5. Techniques of artificial feeding
6. Feeding the preterm infant
7. Parenteral nutrition
8. Common feeding disorders
9. Further reading
Chapter 10 Infection in the newborn
1. Introduction
2. The immune system
3. Susceptibility of the neonate to infection
4. Congenital infection
5. Intrapartum (early-onset) infection
6. Postnatal (late-onset) infection
7. Further reading
Chapter 11 The extreme preterm infant
1. Introduction
2. Gestational age
3. Causes and management of preterm labour
4. Survival and outcome for the preterm infant
5. Preterm delivery at the margins of viability
6. Stabilization at birth and management in the ‘golden hour’
7. Common problems to be expected in the preterm infant
8. Supportive care on the NICU
9. Preparation for discharge home
10. Further reading
Chapter 12 The low-birthweight infant
1. Introduction
2. The infant who is small for gestational age
3. Causes of intrauterine growth restriction
4. Problems to be expected in the growth-restricted fetus and  SGA infant
5. Management of the low-birthweight infant
6. Further reading
Chapter 13 Respiratory physiology and respiratory support
1. Introduction
2. Fetal lung development
3. Pulmonary surfactants
4. Respiratory physiology
5. Assessment of respiratory function
6. Respiratory failure
7. Mechanical ventilation
8. Further reading
Chapter 14 Respiratory disorders
1. Introduction
2. Respiratory distress
3. Transient tachypnoea of the newborn
4. Respiratory distress syndrome (RDS)
5. Pneumonia
6. Pulmonary air leaks
7. Meconium aspiration syndrome
8. Pulmonary hypoplasia
9. Pulmonary haemorrhage
10. Congenital diaphragmatic hernia
11. Oesophageal atresia and  tracheo-oesophageal fistula
12. Congenital lobar emphysema
13. Congenital pulmonary airway malformation (CPAM) [formerly known as congenital cystic adenomatous malformation; CCAM]
14. Chronic lung disease and bronchopulmonary dysplasia (BPD)
15. Further reading
Chapter 15 Apnoea, bradycardia and upper airway obstruction
1. Introduction
2. Physiology
3. Apnoea
4. Acute life-threatening events (ALTEs)
5. Sudden and unexpected infant death and sudden infant death syndrome
6. Upper airway obstruction
7. Further reading
Chapter 16 Cardiovascular disorders
1. Introduction
2. Physiology of the cardiovascular system
3. Blood pressure
4. Hypertension
5. Congenital heart disease
6. Investigations
7. Cyanotic heart disease
8. Congestive heart failure
9. Left-to-right shunts
10. Obstructive lesions
11. Dysrrhythmias
12. Circulatory maladaptation at birth
13. Further reading
Chapter 17 Gastrointestinal and abdominal disorders
1. Introduction
2. Development of the gastrointestinal tract
3. Malformations
4. Abdominal wall defects
5. Necrotizing enterocolitis
6. Short bowel syndrome
7. Rectal bleeding
8. Hernia
9. Hydrocoele
10. Undescended testis
11. Hypospadias
12. Further reading
Chapter 18 Renal disorders
1. Introduction
2. Role of amniotic fluid
3. Renal physiology
4. Normal urine output
5. Investigation of renal disease
6. Presentation of renal disease
7. Acute kidney injury
8. Urinary tract infection
9. Renal masses
10. Cystic disease of the kidneys
11. Haematuria
12. Ectopia vesicae (bladder exstrophy)
13. Further reading
Chapter 19 Jaundice
1. Introduction
2. Physiology of bilirubin metabolism
3. Clinical assessment of the jaundiced infant
4. Unconjugated hyperbilirubinaemia
5. Conjugated hyperbilirubinaemia
6. Further reading
Chapter 20 Haematological disorders
1. Introduction
2. Placental transfusion
3. Anaemia
4. Hydrops fetalis
5. Aplasia
6. Polycythaemia
7. Bleeding and coagulation disorders
8. Thrombocytopenia
9. Haemorrhagic disease of the newborn (Vitamin K-deficient bleeding)
10. Disseminated intravascular coagulation (DIC)
11. Inherited disorders of coagulation
12. Congenital deficiency of anticoagulant proteins (hypercoagulable states)
13. Further reading
Chapter 21 Endocrine and metabolic disorders
1. Introduction
2. Glucose homeostasis and its abnormalities
3. Disorders of calcium, phosphate and magnesium metabolism
4. Disorders of magnesium metabolism
5. Disorders of sodium and potassium metabolism
6. 21.6.1 Box 21.3 Causes of neonatal hyponatraemia.
7. Endocrine gland disorders
8. Abnormalities of the adrenal gland
9. Inborn errors of metabolism
10. Further reading
Chapter 22 The central nervous system
1. Introduction
2. Brain development
3. Malformations of the central nervous system
4. Disorders of head size and shape
5. Intracranial haemorrhage (ICH)
6. Periventricular leukomalacia
7. Neonatal stroke
8. Hypoxic–ischaemic encephalopathy
9. Neonatal convulsions
10. Neonatal hypotonia (‘floppy infant’)
11. Further reading
Chapter 23 Neurodevelopmental follow-up and assessment of hearing and vision
1. Introduction
2. Neurodevelopmental outcome
3. Hearing impairment (deafness)
4. Visual impairment
5. Further reading
Chapter 24 Developmental care and the neonatal environment
1. Introduction
2. Thermoregulation
3. Skin care on the neonatal intensive care unit
4. Optimizing the neonatal environment
5. Procedural pain and analgesia
6. Developmental care
7. Further reading
Chapter 25 Organization of perinatal services
1. Introduction
2. Organization of perinatal services
3. Levels of perinatal care
4. Neonatal networks
5. Further reading
Chapter 26 Neonatal transport
1. Introduction
2. Transport in utero
3. Preparation for transport
4. Transport equipment
5. The role of a neonatal transport service
6. Further reading
Chapter 27 Discharge and follow-up of high-risk infants
1. Introduction
2. Discharge of high-risk infants
3. Immunization
4. Specialized follow-up clinics
5. Follow-up of preterm infants
6. Further reading
Chapter 28 Parent–infant attachment and support for parents of critically ill infants
1. Introduction
2. Parent–infant attachment (bonding)
3. Care of parents of critically ill infants
4. Family integrated care
5. Further reading
Chapter 29 Ethical issues and decision-making process in the treatment of critically ill newborn infants
1. Introduction
2. Principles of ethical reasoning
3. Decision-making processes
4. The role of the Institutional  Ethics Committee
5. Withholding and withdrawing  life-sustaining treatment
6. Common neonatal ethical dilemmas
7. Parents in the decision-making process
8. Further reading
Chapter 30 End-of-life care and palliative care
1. Introduction – why babies die
2. Unexpected deaths (including sudden unexpected postnatal collapse)
3. What is palliative care?
4. Expected deaths and care planning
5. Making a care plan
6. Place of death: hospice versus home versus hospital
7. Symptom control
8. Organ donation
9. Autopsy
10. Caring for parents – grief and bereavement
11. Caring for staff
12. Further reading
Index
EULA

List of Illustrations

Chapter 1
1. Figure 1.1 Diagram of placental structures showing blood perfusion.
2. Figure 1.2 Diagram of the fetal circulation through the heart and lungs, showing the direction of flow through the foramen ovale and ductus arteriosus.
3. Figure 1.3 A timeline for fetal assessment and monitoring during pregnancy.
4. Figure 1.4 Doppler measurement of blood flow in the fetal umbilical artery. The left-hand panel shows normal forward flow throughout the cardiac cycle. The right-hand panel shows pathological reversed flow during diastole (see arrow).
5. Figure 1.5 Cleft lip. Illustration courtesy of Dr Jason Ong.
6. Figure 1.6 Fetal MRI scan (coronal view) showing large cystic hygroma on the left side of the neck (arrow) and an associated pleural effusion (arrow). Illustration courtesy of Dr Mike Weston.
7. Figure 1.7a CTG showing fetal heart rate accelerations.

Chapter 2

Figure 2.1 2014 ANZNN survival data to discharge home (with 95% CI) (Full data are available in Table 30 in Chow, S.S.W., Le Marsney, R., Haslam, R., Lui, K. (2016) Report of the Australian and New Zealand Neonatal Network 2014. ANZNN, Sydney.

Chapter 3

Figure 3.1 Twin peak or lambda sign. Illustration courtesy of Dr Scott Peterson, Mater Mothers’ Hospital. Reproduced with permission of Dr Peterson.

Chapter 4

Figure 4.1 Problems leading to joint contractures.

Figure 4.2 Infant with typical features of fetal alcohol syndrome. From Lissauer, T. and Fanaroff, A. A. (2011) Neonatology at a Glance, 2nd edition. © 2011, Blackwell Publishing Ltd. Reproduced with permission of John Wiley & Sons.

Chapter 5

Figure 5.1 The physiological effect of acute asphyxia and the response to resuscitation. Illustration courtesy of Dr Sam Richmond.

Figure 5.2 Algorithm for resuscitation. Reproduced with permission from the Resuscitation Council UK (2015).

Figure 5.3 Mask inflation with the head in the neutral position.

Figure 5.4 (a) T-piece (Fischer Pykell Health Care). (b) Face masks designed for use in face mask ventilation of term and preterm newborns.

Figure 5.5 Laryngoscopy. The laryngoscope blade displaces the tongue and lifts the epiglottis anteriorly to expose the cords  (Source: Baillière Tindall).

Figure 5.6 The stages of intubation. (a) Visualization of the uvula and oropharynx. (b) The epiglottis is seen with the oesophagus beyond it. (c) The cords are also seen.

Figure 5.7 Cardiac compressions performed by encircling the chest, whilst ventilation breaths are given by bag-valve-mask in a 3:1 ratio.

Chapter 6

Figure 6.1 Head-to-toe examination sequence. From Miall, L. (2009) The Newborn Examination. Paediatrics at a Glance, 3rd edition,  Wiley-Blackwell. Reproduced with permission of John Wiley & Sons.

Figure 6.2 Sagittal synostosis. (a) The baby has a palpable ridge on their skull. (b) 3D CT scan of the same child showing fusion of the sagittal suture (arrow).

Figure 6.3 This baby (who is being examined under anaesthetic) has a normal red reflex in their left eye, but an absent red reflex in the right eye (arrow) due to congenital cataract.

Figure 6.4 Severe micrognathia (with tracheostomy). This patient has cerebro-costo-mandibular syndrome.

Figure 6.5 Cystic hygroma of the neck (trans-illuminated).

Figure 6.6 (a) Teratoma of the neck. This child was intubated while still connected to the placental circulation (EXIT procedure) before having surgical excision. (b) MRI scan showing the same lesion (arrow).

Figure 6.7 A radiograph showing multiple vertebral anomalies (arrows).

Figure 6.8 Eliciting the Moro reflex.

Figure 6.9 (a) Talipes equinovarus; (b) talipes calcaneovalgus.

Figure 6.10 Ortolani’s test. The hip cannot be abducted because of posterior dislocation of the femoral head. The hip is pulled upwards and the head clunks into the acetabulum, permitting abduction. Barlow’s test. The adducted hip is pushed downwards and laterally to see whether it is dislocatable. Source: Lawrence Miall, Mary Rudolf, Dominic Smith. Paediatrics at a Glance, 4th Edition. May 2016, ©2016, Wiley-Blackwell. Reproduced with permission of John Wiley & Sons.

Figure 6.11 Arthrogryposis multiplex.

Figure 6.12 Thanatophoric dwarf.

Figure 6.13 Vascular haemangioma.

Figure 6.14 Congenital melanocytic naevus involving the buttock and loin.

Figure 6.15 Mongolian blue spot.

Figure 6.16 Harlequin ichthyosis with severe deep skin cracking.

Figure 6.17 Transient neonatal pustular melanosis.

Figure 6.18 Cutis aplasia of the scalp. Note the extensive area of hair loss, some of which is scabbed over.

Chapter 7

Figure 7.1 Anatomic location of injuries to the head. Source: Tom Lissauer, Avroy A. Fanaroff, Lawrence Miall, Jonathan Fanaroff. Neonatology at a Glance, 3rd Edition. Wiley-Blackwell. Reproduced with permission of John Wiley & Sons.

Figure 7.2 Cephalhaematoma. Note the swelling over the right parietal bone. This child also has hypotonia with a characteristic drooping appearance to the mouth.

Figure 7.3 Right frontal depressed skull fracture (see arrow).

Figure 7.4 Right-sided clavicular fracture in a child born after shoulder dystocia. The baby is also receiving mechanical ventilation.

Figure 7.5 Left-sided facial nerve palsy.

Figure 7.6 Right-sided Erb’s palsy showing the typical ‘waiter’s tip’ position of the hand. Note the unilateral Moro reflex on the left.

Figure 7.7 Bruising to the foot from SaO₂ probe.

Figure 7.8 Chemical burn from aqueous 2% chlorhexidine used prior to UAC insertion in an extreme preterm baby. Reproduced with permission from Lashkari, H.P., Chow, P., Godambe, S. (2011) Aqueous 2% chlorhexidine-induced chemical burns in an extremely premature infant. Archives of Diseases in Childhood: Fetal and Neonatal Edition, 97, F64.© 2011, BMJ Publishing Group Ltd.

Chapter 8

Figure 8.1 Diagram of a DNA double helix.

Figure 8.2 Normal chromosome pattern and number after Giemsa staining. This is an example of a male karyotype. The hashed horizontal line is at the centromere and divides the chromosome into short (p) and long (q) arms.

Figure 8.3 Image showing FISH probes for chromosome 21 (red) and chromosome 13 (green). There are three red signals (abnormal) and two green signals (normal); this patient therefore has trisomy 21.

Figure 8.4 A family pedigree showing autosomal dominant inheritance.

Figure 8.5 A family pedigree showing autosomal recessive inheritance.

Figure 8.6 A family pedigree showing X-linked recessive inheritance.

Chapter 9

Figure 9.1 Total body water and extracellular fluid expressed as percentages of body weight. Redrawn from Dear (1984), with permission from Reed Business Publishing.

Figure 9.2 Hormonal maintenance of lactation. PIF, prolactin-inhibiting factor; PRF, prolactin-releasing factor.

Chapter 10

Figure 10.1 Schematic representation of the clinical features of prenatal TORCH infections.

Figure 10.2 Routes of neonatal cross-infection.

Figure 10.3 Showing position of infant during lumbar puncture while taking care to avoid excessive bending.

Figure 10.4 Equipment required for insertion of a percutaneous intravenous central catheter (PICC). Illustration courtesy of Dr Emmanuel Erinaugha.

Chapter 11

Figure 11.1 Outcomes of all extreme preterm babies born in the UK in 2006.

Chapter 12

Figure 12.1 Monozygotic twins born at 32 weeks. The smaller twin weighed 750 g and the larger 1700 g. The smaller twin shows features of IUGR with relative head sparing (asymmetric IUGR).

Chapter 13

Figure 13.1 Stages of fetal lung development. Reproduced with permission from Attar, M.A., Donn, S.M. (2002) Mechanism of ventilator-induced lung injury in premature infants. Seminars in Neonatology, 7, 353–360; © 2002, Elsevier.

Figure 13.2 Oxygen dissociation curve for fetal haemoglobin (upper red line) and adult haemoglobin (lower blue line).

Figure 13.3 Pressure–volume loop showing compliance of the lung.

Figure 13.4 Flow–volume loop, showing resistance to airflow. The loop on the left shows increased resistance causing impedance to airflow, which has improved after treatment as shown in the loop on the right.

Figure 13.5 Lung volumes. TLC, total lung capacity; VC, vital capacity; RV, residual volume; IC, inspiratory capacity; FRC, functional residual capacity; IRC, inspiratory respiratory capacity; ERC, expiratory respiratory capacity; TV, tidal volume.

Chapter 14

Figure 14.1 Transient tachypnoea of the newborn (TTN). Note streaky bilateral shadows, fluid in the transverse fissure (arrows) and relative cardiomegaly.

Figure 14.2 Incidence of RDS related to gestational age.

Figure 14.3 Schematic representations of two alveoli, demonstrating the Laplace law (see text for details).

Figure 14.4 Chest radiograph showing the characteristic ‘ground glass’ appearance of RDS. Note the ‘air bronchogram’.

Figure 14.5 Chest radiograph showing right-sided tension pneumothorax. Note this has occurred despite a chest drain being in place, suggesting a massive air leak or a blocked chest drain.

Figure 14.6 Chest radiograph showing pneumomediastinum.  The heart and thymus are outlined by gas.

Figure 14.7 Chest radiograph showing extensive PIE. Note the overinflated chest with flattened diaphragm.

Figure 14.8 Chest radiograph showing left-sided PIE. The mediastinum and right lung are compressed by the overinflated  left lung.

Figure 14.9 (a) Left-sided pneumothorax in a preterm baby. (b) The same baby after insertion of a 10 Fr pigtail catheter.

Figure 14.10 Chest radiograph showing meconium aspiration syndrome (MAS). There is extensive discrete shadowing throughout both lung fields and hyperinflation.

Figure 14.11 Chest radiograph showing a left-sided diaphragmatic hernia.

Figure 14.12 Variants of tracheo-oesophageal fistula with or without oesophageal atresia. Type (c) accounts for 85% of cases, the others being equally uncommon.

Figure 14.13 Chest radiograph showing severe bronchopulmonary dysplasia.

Chapter 15

Figure 15.1 Neuromuscular pathway for control of respiration.

Figure 15.2 Suggested protocol for the management of apnoea alarm.

Figure 15.3 A normal upper airway. Reproduced with permission from South, M., Isaacs, D. (eds) Practical Paediatrics, 7th edition. Elsevier Health Sciences, London.

Figure 15.4 Nasopharyngeal tube used for micrognathia. Reproduced with permission from South, M., Isaacs, D. (eds) Practical Paediatrics, 7th edition. Elsevier Health Sciences, London.

Chapter 16

Figure 16.1 Upper and lower centiles for (a) systolic and (b) diastolic blood pressure against gestational age. (c) The change in mean arterial blood pressure (MABP) with postnatal age at different gestational age bands.

Figure 16.2 Flow diagram showing a suggested graded management response to neonatal hypotension.

Figure 16.3 Real-time, two-dimensional echocardiograms of the normal neonatal heart. (a) Parasternal long-axis view. RV, right ventricle; LV, left ventricle; Ao, aorta; LA, left atrium. (b) Parasternal short-axis view showing colour Doppler (left-to-right) flow through patent ductus arteriosus (white arrow). Illustration courtesy of Dr J. Wyllie. (c) Apical four-chamber view,  RA, right atrium; RV, right ventricle; LV, left ventricle; LA, left atrium.

Figure 16.4 A diagnostic approach to cyanotic CHD.

Figure 16.5 Schematic diagram of simple transposition of the great vessels without VSD.

Figure 16.6 Tetralogy of Fallot.

Figure 16.7 A large mid-muscular ventricular septal defect.

Figure 16.8 Schematic diagram of a hypoplastic left heart.

Chapter 17

Figure 17.1 Cleft lip. (a) At birth the infant has a right-sided cleft lip. (b) The same infant following repair. Pictures courtesy of Mr Alistair Smyth. Reproduced with permission of John Wiley & Sons.

Figure 17.2 Duodenal atresia. Abdominal radiograph showing the ‘double bubble’ appearance.

Figure 17.3 Omphalocoele. Illustration courtesy of Dr Lawrence Miall. Reproduced with permission of Dr Lawrence Miall.

Figure 17.4 Gastroschisis being gradually reduced using a silo.

Figure 17.5 Congenital ascites and umbilical hernia.

Figure 17.6 Schema for the development of NEC.

Figure 17.7 Radiological appearance of NEC. The image shows extensive intramural gas in the bowel and dilated loops of small bowel.

Figure 17.8 Left-sided inguinal hernia.

Figure 17.9 Hypospadius and potential urethra opening sites.

Chapter 18

Figure 18.1 Longitudinal ultrasound view of fetal abdomen showing bilateral renal pelvocalyceal dilatation. Illustration courtesy of Dr R. Cincotta.

Figure 18.2 Management of fetal renal pelvis dilatation.

Figure 18.3 Suprapubic aspiration of urine from the bladder. The needle should be aimed slightly superiorly in the midline and 0.5 cm above the pubis.

Chapter 19

Figure 19.1 Summary of neonatal bilirubin metabolism.

Figure 19.2 Phototherapy for hyperbilirubinaemia.

Figure 19.3 NICE clinical guideline: treatment threshold for babies with neonatal jaundice ≥38 weeks’ gestation.

Figure 19.4 Approach to the jaundiced infant.

Chapter 20

Figure 20.1 Physiological anaemia. The two graphs show the normal fall in haemoglobin with postnatal age in mature and premature infants.

Figure 20.2 The interrelationship between polycythaemia and hyperviscosity and their contribution towards clinical signs. CNS, central nervous system; GFR, glomerular filtration rate.

Chapter 21

Figure 21.1 Metabolic pathways involved in gluconeogenesis.

Figure 21.2 Characteristic appearance of the macrosomic infant of a poorly controlled diabetic mother. Note the right-sided brachial plexus injury (Erb’s palsy), arising from shoulder dystocia.

Figure 21.3 Radiograph of an infant’s forearm and wrist, showing the metaphyseal flaring of neonatal rickets (arrow).

Figure 21.4 A simplified diagram to illustrate the synthesis of adrenal hormones. The asterisk represents the enzyme 17-α-hydroxydehydrogenase.

Figure 21.5 Flow diagram showing a scheme for investigating infants with ambiguous genitalia.

Figure 21.6 Representation of metabolic pathways with a negative feedback loop.

Figure 21.7 Metabolism of phenylalanine. The broken arrow represents the enzyme defect in phenylketonuria.

Chapter 22

Figure 22.1 The sequence of brain development.

Figure 22.2 Occipital encephalocoele, prior to surgical repair.

Figure 22.3 The varieties of spina bifida.

Figure 22.4 Lumbosacral myelomeningocoele. Note the baby has talipes.

Figure 22.5 Premature suture closure leading to craniostenosis. (a) Scaphocephaly (sagittal suture); (b) turricephaly (coronal suture); (c) plagiocephaly (single lambdoid suture). The dotted line indicates different sutural synostosis, Coronal and lambdoid sutures can be involved on one or both sides, giving different shapes.

Figure 22.6 Diagram to show intracerebral drainage of cerebrospinal fluid. Reproduced from Levene 1987, with permission of Churchill Livingstone, Elsevier.

Figure 22.7 Coronal ultrasound scan showing massive dilatation of both lateral ventricles and the third ventricle.

Figure 22.8 Indication for intervention for significant ventriculomegaly. The lower line is the 97th centile for normal ventricular size. The upper line defines ventricular dilatation severe enough to require treatment.

Figure 22.9 Post-mortem specimen showing bilateral intraventricular haemorrhage with ventricular dilatation.

Figure 22.10 Coronal ultrasound scan showing massive left-sided IVH with venous infarction of the left parietal lobe with porenchephalic cyst developing (arrow).

Figure 22.11 Cystic periventricular leukomalacia (PVL). (a) Cerebral ultrasound showing PVL; there is bilateral periventricular ‘flare’ with cysts on the left side (arrowed) which appeared at 14 days of life; (b) T2-weighted MRI scan on the same patient 6 days later shows extensive bilateral cystic PVL (arrows).

Figure 22.12 Neonatal stroke. MRI scan showing ischaemic infarction of the brain (dark) in the territory of left middle cerebral artery.

Figure 22.13 The prognostic values of different forms of aEEG tracings in babies with hypoxic–ischaemic encephalopathy. While the top and middle tracings are mostly indicative of good prognosis, the suppressed amplitude with continuous low voltage with seizure activity (burst suppression) as seen in the bottom panel is invariably associated with a worst prognosis in terms of death and neurodisability.

Figure 22.14 Abnormality in the thalamic nuclei (arrows) in a term baby indicating a poor prognosis following acute intrapartum asphyxia.

Figure 22.15 Trace from a cerebral function monitor. There are frequent electroconvulsive seizures (red arrows). The clinically evident seizures are marked in the upper panel with black arrows, showing a degree of electroconvulsive dissociation. The blue arrow indicates the onset of seizure activity on the raw EEG panel. The bottom panel shows the raw EEG trace present at the point in time marked by the black arrow on the top panel. This shows the start of a seizure

Figure 22.16 An infant with severe hypotonia, showing the characteristic ‘frog’ posture.

Chapter 23

Figure 23.1 Stage 3 retinopathy of prematurity (ridging and vascular proliferation) with plus disease (tortuosity of posterior retinal vessels).

Chapter 24

Figure 24.1 Heat loss. (a) By conduction; (b) by convection; (c) by radiation; (d) by evaporation. Reproduced from Warren, I. (2010) Nursing the Neonate, 2nd edition, Wiley Blackwell.

Figure 24.2 Demonstration of the use of a plastic wrap and hat to aid thermoregulation in the newborn preterm infant. Reproduced with permission of Dr Wood.

Figure 24.3 (a) Neutral thermal environment during the first week of life, calculated from the measurements. Dewpoint of the air 18 °C, flow 10 l min^–1; (b) Neutral thermal environment (°C) from day 7 to day 35. Dewpoint of the air 18 °C. flow 10 l min^–1. Body weight is current weight. Values for body weight >2.0 kg are calculated by extrapolation. Source: Sauer PJ, Dane HJ, Visser HK. New standards for neutral thermal environment of healthy very low birthweight infants in week one of life. Arch Dis Child. 1984 Jan;59(1):18-22. Reproduced with permission of BMJ Publishing Group Ltd.

Figure 24.4 Three designs of intensive care incubator. (a) Closed incubator; (b) hybrid; (c) open platform.

Figure 24.5 Developmental care within the neonatal nursery.

Figure 24.6 (a) Kangaroo care. Reproduced with permission of Rady Children’s Hospital – San Diego; (b) Skin-to-skin contact. Reproduced with permission from Neama Firth.

Chapter 27

Figure 27.1 Corrected postnatal ages at which disabilities become evident in VLBW infants.

Chapter 30

Figure 30.1 A typical Limitation of Treatment Agreement (LOTA) agreement.

Figure 30.2 Memory box. Source: Tom Lissauer, Avroy A. Fanaroff, Lawrence Miall, Jonathan Fanaroff. Neonatology at a Glance, 3rd Edition August 2015, ©2014, Wiley-Blackwell. Reproduced with permission of John Wiley & Sons.

Guide

Cover

Table of Contents

Preface

Preface to the Sixth Edition

Neonatology is coming of age as a speciality — when the First Edition of this book was published 30 years ago, neonatal medicine was evolving rapidly and the emphasis was rightly on improving survival, especially at the margins of extreme prematurity. Now, survival is greater than 90% down to 28 weeks, and survival at 24 weeks — previously regarded as the threshold of viability — exceeds 60%.

With this improvement in survival, emphasis has begun to turn to the quality of care, quality of family support, and to the longer-term outcomes of graduates of the neonatal intensive care unit. Parents and siblings are now routinely welcomed into the nursery, whereas 30 years ago they may have been restricted in their visiting, and family-centred and family integrated care is becoming the normal. There is an increasing emphasis on risk reduction and minimizing harm — whether through hospital-acquired infections, injury from lines and procedures, or preventing ventilator-associated lung injury with the use of minimally invasive ventilation. There is also a greater recognition of the subtle but significant developmental and health challenges faced by only moderately pre-term babies, who are considerably greater in number than the extreme preterm babies.

To reflect this evolution this book has also evolved, with new chapters on palliative and end-of-life care, a greater emphasis on developmental and family care, and comprehensively updated chapters to include the latest developments in diagnostic imaging and genetic testing available. We believe that Essential Neonatal Medicine offers a comprehensive introduction to modern neonatology for trainee doctors, neonatal nurses, nurse practitioners and allied health professionals. We thank the many colleagues who have made it possible.

Dr Sunil Sinha
Dr Lawrence Miall
Dr Luke Jardine

Acknowledgements

We would like to thank all the many colleagues and families who have contributed to this edition. In particular, Mr Andrew Breeze for reviewing the obstetric chapter, and Dr Jayne Shillito, Dr Mike Weston,
Dr Fiona Wood, Dr Shalabh Garg, Dr Sam Richmond, Dr Jonathan Wyllie, Mr Roly Squire, Mr Vernon Long, Dr Scott Peterson and Dr Liz McKechnie for providing clinical images.

This edition of the book would also not have been possible without the efforts of many ‘behind the scenes’ individuals, including Jennifer Seward (Senior Project Editor) and Loan Nguyen (Senior Editorial Assistant), and the editors are grateful to them for their patience and guidance.

We would especially like to thank our families for their support with this project and their understanding during the many evenings we spent writing this book.

And finally, we are indebted to the babies and their families that it has been our privilege to treat, who have taught us so much over the years.

Preface to the First Edition

There has been an explosion of knowledge over the last decade in fetal physiology, antenatal management and neonatal intensive care. This has brought with it confusion concerning novel methods of treatment and procedures as well as the application of new techniques for investigating and monitoring high-risk neonates. The original idea for this book was conceived in Brisbane, and a Primer of Neonatal Medicine was produced with Australian conditions in mind. We have now entirely rewritten the book, and it is the result of cooperation between Australian and British neonatologists with, we hope, an international perspective.

We are aware of the need for a short book on neonatal medicine which gives more background discussion and is less dogmatic than other works currently available. We have written this book to give more basic information concerning physiology, development and a perspective to treatment which will be of value equally to neonatal nurses, paediatricians in training, medical students and midwives. Whilst collaborating on a project such as this we are constantly aware of the variety of ways for managing the same condition. This is inevitable in any rapidly growing acute speciality, and we make no apologies for describing alternative methods of treatment where appropriate. Too rigid an approach will be to the detriment of our patients!

A detailed account of all neonatal disorders is not possible but common problems and their management are outlined giving an overall perspective of neonatology. Attention has been given to rare medical and surgical conditions where early diagnosis and treatment may be lifesaving. It is easy to be carried away with the excitement of neonatal intensive care and forget the parents sitting at the cotside. Our approach is to care for the parents as well as their baby, and we have included two chapters on parent–infant attachment as well as death and dying. The final chapter deals with practical procedures and gives an outline of the commonly performed techniques used in the care of the high-risk newborn. We have also provided an up-to-date neonatal Pharmacopoeia as well as useful tables and charts for normal age-related ranges.

Malcolm I. Levene
David I. Tudehope
M. John Thearle

Abbreviations

ABR auditory brainstem response

ADHD attention deficit hyperactivity disorder

ALTE acute life-threatening events

ART assisted reproductive technology

ASD atrial septal defect

BE base excess

BPD bronchopulmonary dysplasia

CAH congenital adrenal hyperplasia

CCAM congenital cystic adenomatous malformation

CDH congenital diaphragmatic hernia

CFM cerebral function monitoring

CHARGE coloboma, heart defects, choanal atresia, retardation, genital and/or urinary abnormalities, ear abnormalities

CHD congenital heart disease

CLD chronic lung disease

CPAP continuous positive airway pressure

CVP central venous pressure

DDH developmental dysplasia of the hip

DIC disseminated intravascular coagulation

EBM expressed breast milk

ELBW extremely low birthweight

FASD fetal alcohol spectrum disorder

FES fractional excretion of sodium

FHR fetal heart rate

FRC functional residual capacity

GFR glomerular filtration rate

GIFT gamete intrafallopian transfer

GORD gastro-oesophageal reflux disease

HCV hepatitis C virus

HIE hypoxic–ischaemic encephalopathy

HMF human milk fortifiers

ICH intracerebral haemorrhage

IDM infants of diabetic mothers

IPPV intermittent positive pressure ventilation

ITP idiopathic thrombocytopenic purpura

IUGR intrauterine growth restriction

IVF in vitro fertilization

IVH intraventricular haemorrhage

LBW low birthweight

LMP last menstrual period

LVH left ventricular hypertrophy

MAS meconium aspiration syndrome

NAS neonatal abstinence syndrome

NCPAP nasal continuous positive airway pressure

NICU neonatal intensive care unit

NIPPV non-invasive positive pressure ventilation

NTD neural tube defects

PCV pneumococcal conjugate vaccine

PDA patent ductus arteriosus

PEEP positive end-expiratory pressure

PET pre-eclampsia

PICC peripherally inserted central catheter

PIE pulmonary interstitial emphysema

PIP peak inspiratory pressure

PMR perinatal mortality rate

PPHN persistent pulmonary hypertension of the newborn

PROM premature rupture of membranes

RDS respiratory distress syndrome

ROP retinopathy of prematurity

RVH right ventricular hypertrophy

SGA small for gestational age

SIDS sudden infant death syndrome

SLE systemic lupus erythematosus

TAR thrombocytopenia with absent radii

TGA transposition of the great arteries

ToF tetralogy of Fallot

TORCH toxoplasmosis, other infections, rubella, cytomegalovirus, herpes simplex virus

TPN total parenteral nutrition

TSH thyroid-stimulating hormone

TTN tachypnoea of the newborn

TTTS twin-to-twin transfusion syndrome

UAC umbilical arterial catheter

UVC umbilical venous catheter

VACTERL vertebral anomalies, anal atresia, cardiovascular anomalies, tracheoesophageal fistula, oesophageal atresia, renal and/or radial anomalies, limb defects

VAPS volume-assured pressure support

VCV volume-controlled ventilation

VILI ventilator-induced lung injury

VLBW very low birthweight

VSD ventricular septal defect

VUR vesico-ureteric reflux

WHO World Health Organization

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Further reading list

ABR	auditory brainstem response
ADHD	attention deficit hyperactivity disorder
ALTE	acute life-threatening events
ART	assisted reproductive technology
ASD	atrial septal defect
BE	base excess
BPD	bronchopulmonary dysplasia
CAH	congenital adrenal hyperplasia
CCAM	congenital cystic adenomatous malformation
CDH	congenital diaphragmatic hernia
CFM	cerebral function monitoring
CHARGE	coloboma, heart defects, choanal atresia, retardation, genital and/or urinary abnormalities, ear abnormalities
CHD	congenital heart disease
CLD	chronic lung disease
CPAP	continuous positive airway pressure
CVP	central venous pressure
DDH	developmental dysplasia of the hip
DIC	disseminated intravascular coagulation
EBM	expressed breast milk
ELBW	extremely low birthweight
FASD	fetal alcohol spectrum disorder
FES	fractional excretion of sodium
FHR	fetal heart rate
FRC	functional residual capacity
GFR	glomerular filtration rate
GIFT	gamete intrafallopian transfer
GORD	gastro-oesophageal reflux disease
HCV	hepatitis C virus
HIE	hypoxic–ischaemic encephalopathy
HMF	human milk fortifiers
ICH	intracerebral haemorrhage
IDM	infants of diabetic mothers
IPPV	intermittent positive pressure ventilation
ITP	idiopathic thrombocytopenic purpura
IUGR	intrauterine growth restriction
IVF	in vitro fertilization
IVH	intraventricular haemorrhage
LBW	low birthweight
LMP	last menstrual period
LVH	left ventricular hypertrophy
MAS	meconium aspiration syndrome
NAS	neonatal abstinence syndrome
NCPAP	nasal continuous positive airway pressure
NICU	neonatal intensive care unit
NIPPV	non-invasive positive pressure ventilation
NTD	neural tube defects
PCV	pneumococcal conjugate vaccine
PDA	patent ductus arteriosus
PEEP	positive end-expiratory pressure
PET	pre-eclampsia
PICC	peripherally inserted central catheter
PIE	pulmonary interstitial emphysema
PIP	peak inspiratory pressure
PMR	perinatal mortality rate
PPHN	persistent pulmonary hypertension of the newborn
PROM	premature rupture of membranes
RDS	respiratory distress syndrome
ROP	retinopathy of prematurity
RVH	right ventricular hypertrophy
SGA	small for gestational age
SIDS	sudden infant death syndrome
SLE	systemic lupus erythematosus
TAR	thrombocytopenia with absent radii
TGA	transposition of the great arteries
ToF	tetralogy of Fallot
TORCH	toxoplasmosis, other infections, rubella, cytomegalovirus, herpes simplex virus
TPN	total parenteral nutrition
TSH	thyroid-stimulating hormone
TTN	tachypnoea of the newborn
TTTS	twin-to-twin transfusion syndrome
UAC	umbilical arterial catheter
UVC	umbilical venous catheter
VACTERL	vertebral anomalies, anal atresia, cardiovascular anomalies, tracheoesophageal fistula, oesophageal atresia, renal and/or radial anomalies, limb defects
VAPS	volume-assured pressure support
VCV	volume-controlled ventilation
VILI	ventilator-induced lung injury
VLBW	very low birthweight
VSD	ventricular septal defect
VUR	vesico-ureteric reflux
WHO	World Health Organization