with i perinatal mortality and morbidity; preterm delivery. Associated Major causes are placenta praevia, vasa praevia, placental abruption. Observatio n or immediate delivery performed depending on severity and gestation. Umbilical cord prolapse An obstetric emergency due to high risk of cord compression and per ina- tal asphyxia. Requires urgent delivery, usually by CS. Preterm prelabour rupture of the membranes \200 \200 In 80% preterm labor rapidly follows. In remaining 20% there is occurs before 20wks, neonat al pulmonary hypoplasia. Treatment Tocolysis is contraindicated. signi \201 cant risk of infection and, if PPROM : Give mother corticosteroids. Consider antibiotics. Preterm labor See b p.114 and p.116 06_McClure_06.indd 121 11/14/2012 11:22:41 PM CHAPTER 6 Neonatology 122 Failure to progress Neonatal and maternal morbidity increase with progressive delay. \200 Caused by: passage obstruction (malpresentation, cephalopelvic disproportion, abnormal pel vic, or cervical anatomy) or uterine dysfunction. Treatment synthetic oxytocin to hasten de livery. CS may be necessary. : arti \201 cial rupture of membranes (ARM), analgesia, and Disturbing/abnormal fetal heart rate patterns May signify hypoxia. Fetal acidosis results i f hypoxia prolonged or re- peated. Signs \200 \200 Loss of variability in baseline fetal heart rate (<5beats/min). Late decelerations (in hear t rate) (lowest fetal heart rate is >30s after peak uterine contraction). Repetitive severe , variable decelerations. Prolonged fetal deceleration (2 \200\2239min below established ba seline). Prolonged fetal bradycardia (<100/min). Persistent fetal tachycardia (>170/min). \200 \200 \200 Tests \200 (cid:1) 7.24 = \200\230Borderline \200\231 - repeat (cid:1) 30min; Fetal blood gas sampling (pH (cid:1)7.2 = \200\230Abnormal \200\231 \200\224consultant obs tetrician and delivery). Postnatal umbilical artery and venous blood gas are used to determ ine the actual level and nature of acidaemia. Malpresentation Most common form is breech (3% at term). \200 Types: knees \202 exed); footling (feet are presenting part). External and 36wks. Vaginal morbidity; CS is recommended. Other malpresentations are associated with labor and CS rate (obligatory for brow and transverse presentation). extended (hips \202 exed and knees extended); \202 exed (hips and cephalic version: may be successful in turning baby between 34 breech delivery: associated with i perinatal mortality and i risk of obstructed Shoulder dystocia Inability to deliver shoulders after head has been delivered. Cord com- p ression leads to rapid fetal asphyxia. \200 Treatment: maneuver ( \202 exion + abduction maternal hips, thighs on abdomen), suprapubic pressure, posterior fetal arm extraction, +/ \200\223 episiotomy : perinatal asphyxia, hum eral and clavicle fracture, Erb \200\231s palsy. Risks urgent delivery \200\224experienced obstetrician, McRobert \200\231s 06_McClure_06.indd 122 11/14/2012 11:22:41 PM OBSTETRIC PROBLEMS 123 06_McClure_06.indd 123 11/14/2012 11:22:41 PM CHAPTER 6 Neonatology 124 Maternal disorders causing neonatal disease Any maternal disease can adversely affect fetal and neonatal health. Certain maternal illnesses, e.g. CHD, also raise the risk of inherita nce in the newborn. Most common manifestations are: \200 \200 \200 Spontaneous abortion. Fetal death. IUGR and/or preterm delivery. Maternal drug ingestion Maternal medications or substance abuse can affect the newborn: \200 b p.951); \200 \200 \200 maternal anticonvulsants ( alcohol abuse and fetal alcohol syndrome ( tobacco; neonatal abs tinence syndrome ( b p.951); b p.186). Hypertensive diseases Pregnancy-induced hypertension (e.g. pre-eclampsia, eclampsia, haemol yt- ic anemia \200\223elevated liver enzymes \200\223low platelet count (HELLP) syndrome) i s associated with increased fetal loss, the need for preterm delivery, IUGR, neonatal leuco penia, and thrombocytopenia. Maternal drug treat- ment may cause neonatal hypoglycaemia and hypotension. Systemic lupus erythematosus Associated with: i \200 \200 \200 \200 Risk of spontaneous abortion. IUGR. Preterm delivery. Neonatal lupus syndrome (rare; associated with anti-Ro and \200 \223La antibodies): complete heart block, haemolytic anemia, leucopenia, thrombocytopenia, and discoid erythematous skin rash. Antiphospholipid syndrome Maternal antiphospholipid antibodies (e.g. lupus anticoagulant or anticardiolipin antibodies) are associated with spontaneous abortion, IUGR, fetal death, n eed for preterm delivery. Thyroid disease See b p.425. In 710% of women with Graves \200 \231s disease, thyroid-stimulating hormone (TSH) receptor-stimulator antibodies cross the p lacenta causing neonatal thyrotoxicosis. Fetus most likely to be affected if high maternal IgG se- rum level develops, or mother requires treatment during pregnancy. Take cord blood for TSH, fT4, and TSH receptor antibody (TRAB). Repeat at D5 if results abnormal. Myasthenia gravis In 710% transplacental passage of IgG antibodies to motor end-plate ace- tylcholine receptors causes transient neonatal myasthenia gravis. Diabetes mellitus See b p .112 Maternal infection See b pp.184 \200\223185 06_McClure_06.indd 124 11/14/2012 11:22:41 PM MATERNAL DISORDERS CAUSING NEONATAL DISEASE 125 06_McClure_06.indd 125 11/14/2012 11:22:41 PM CHAPTER 6 Neonatology 126 Birth trauma Risk factors LGA, cephalic \200\223pelvic disproportion, malpresentation, prec ipitate delivery, instrumental delivery, shoulder dystocia, prematurity. Head \200 Caput succedaneum: particularly large following ventouse delivery (chignon). Rapidly resolv es. Cephalhaematoma: bleed(s). Most often occur over parietal bones. Swelling limited by su ture lines. Resolves over weeks. Subaponeurotic haematoma: periostium, so can be large and life-threatening. Presents as \202 uctuant scalp swelling, not limited by suture lines. oedema of the presenting scalp. Can be common \202 uctuant swelling(s) due to subperiostial rare; bleeding not con \201 ned by skull Skin \200 \200 Traumatic cyanosis: Lacerations: pH sampling, or scalpel wounds during CS. Close with Steri -Strips or suture if required. bruising and petechiae of presenting part. caused by forceps, ventouse cap, scalp electrode s, scalp Nerve palsies \200 Brachial plexus: result from dif \201 cult assisted delivery (e.g. shoulder dystocia); the arm is \202 accid with pronated forearm and \202 exed wrist (waiter \200\231s tip posi tion). Complete recovery occurs within 6wks in two-thirds of cases. X-ray clavicle to exclu de fractures. Refer to physiotherapy for assessment and follow-up. Facial nerve palsy: spin e or forceps. Presents as facial asymmetry that is worse on crying (affected side shows lac k of eye closure and lower facial movement; mouth is drawn to normal side). Majority recove r in 1 \200\2232wks. May require eye care with methylcellulose and specialist referral. commonest is Erb \200\231s palsy (C5 \200\223C6 nerve routes). May follows pressure on face from either maternal ischial Fractures \200 Clavicle \200 Long bone fractures: usually lower avulsion fractures of t he femoral or tibial epiphyses, or mid-shaft fractures of the femur or humerus. Infant pres ents as unsettled, with affected limb pseudo-paralysis, or obvious deformity or swelling. C on \201 rm by X-ray. Skull fracture: treatment unless depressed in which case neurosurgica l referral is required. Treatment: analgesia; limb immobilization (arm inside baby-grow), o ften do not require orthopedic intervention, healed in a few weeks. Rapid healing and remod eling usually occur. (commonest). associated with forceps delivery and usually require no 06_McClure_06.indd 126 11/14/2012 11:22:41 PM BIRTH TRAUMA 127 Soft tissue trauma \200 Sternocleidomastoid tumor: haematoma. Subsequent contraction of muscle results in non-tende r \200\230tumor \200\231 and torticollis (head turns away from affected muscle). Physiothe rapy almost always curative (see also b p.883). Possible indication of malposition in-utero \200\224consider increased risk of developmental dysplasia of the hip (DDH) (b p.748). Fat necrosis over bony prominences, e.g. forceps. It usually resolves spontaneously. May be ex tensive with risk of i Ca2+ and so there is a need to monitor serum level. overstretching of muscle leads to Tender, red, subcutaneous swelling caused by pressure 06_McClure_06.indd 127 11/14/2012 11:22:41 PM CHAPTER 6 Neonatology 128 Non-speci \201 cally ill neonate Early recognition of serious neonatal illness is an impor tant skill. The nurse or parent may say that the infant is just \200\230not right \200\231 . Listen, examine the baby carefully, and act if in any doubt! Any serious disease can pres ent non-speci \201 cally. Major causes \200 Infection (e.g. group B streptococcus or Guillain \200\223Barr syndrome (GBS), septicaemi a, meningitis; b p.180). Hypothermia (may be sign of infection). Metabolic (e.g. hypoglycae mia, b p.187). Cardiac (e.g. congenital heart disease, arrhythmias; GI (e.g. NEC; CNS (e.g. intracranial hemorrhage, seizures; \200 b p.132; inborn errors of metabolism \200 \200 b pp.232, 250). b p.178). b pp.134, 176). Presentation \200 Skin: re \201 ll >2secs; rash; jaundice. Temperature: CNS: jittery, seizures. Respiratory: (>60breaths/min), intercostal or subcostal recession, tracheal tug. CVS: or hypotension sh ould be considered late/pre-terminal signs). GI: abdominal tenderness; bilious vomit or asp irate. Metabolic: pallor, mottling, peripheral cyanosis, cool peripheries; capillary \200 i or d. lethargy, weak or unusual cry, generalized hypotonia, irritability, apnoea, expiratory grunting, \202 aring nostrils, tachypnoea tachycardia (>160/min), weak or absent pulses, (bradycardia <80 vomiting, distended abdomen (ileus), diarrhea, bloody stools; i or d blood glucose. 06_McClure_06.indd 128 11/14/2012 11:22:41 PM NON-SPECIFICALLY ILL NEONATE 129 Management of non-speci \201 cally ill neonate \200 Quickly assess ABC. Secure airway, give O ventilatory support if needed Transfer to neonata l unit as soon as safe to do so (get a nurse/ midwife to accompany you on the move) Obtain vascular access (IV/UV/IO) and give bolus 0.9% saline 10 \200\22320mL/kg if circulatory com promise. Repeat if necessary Monitor breathing, SpO temperature Measure BP, blood glucose, urea and electrolytes (U&Es), FBC, blood gas. Consider clotting studies and C-reactive prot ein (CRP). Ventilate early if respiratory failure Full septic screen: blood culture; CXR/AX R; LP (only postpone if baby very unstable) for CSF, M, C&S, protein, glucose; stool cultur e and virology; urine (suprapubic, or midstream urine (MSU) if antibiotics can be delayed) Consider cranial ultrasound scan if preterm/at risk Start broad-spectrum antibiotics. IV be nzylpenicillin and an aminoglycoside (e.g. gentamicin) unless possible listeria infection i n which case substitute ampicillin for benzylpenicillin. If >48hr old, and particularly if indwelling lines were present before illness, consider \202 ucloxacillin, or vancomycin, and gentamicin. If meningitis ensure broad spectrum cover and good CSF penetration \200\224 e.g. cefotaxime. If all cultures are negative and index of suspicion of sepsis is low, anti biotics can be stopped after 48hr. If not, treat for 5 \200\2237 days, changing antibiotics according to sensitivities of signi \201 cant identi \201 ed pathogens. Treat for 14 \200\22321 days if meningitis present. If any doubt consult microbiologist Speci \201 c tr eatment as appropriate, e.g. correct hypoglycaemia, inotropic support if persistently hypot ensive, blood transfusion if signi \201 cant hemorrhage, clotting factors to correct disse minated intravascular coagulation (DIC) 2 if required, and provide 2, heart rate, BP (consider arterial access), \200 06_McClure_06.indd 129 11/14/2012 11:22:42 PM CHAPTER 6 Neonatology 130 Neonatal jaundice See also b p.314. Jaundice is common (60% term, 80% preterm in \201 rst week), and is usually unconjugated. Signi \201 cant jaundice may indicate un- derlying di sease. High serum unconjugated free bilirubin is neurotoxic and can cause kernicterus (deaf ness, athetoid cerebral palsy (CP), seizures). Physiological jaundice Common and appears after 24hr, peaks around day 3 \200\2234, and usu ally re- solves by 14 days. It is due to immaturity of hepatic bilirubin conjugation, but p oor feeding (particularly in breast-fed infants) can also contribute. Jaundice progresses i n a cephalic-caudal direction. Measure bilirubin (transcutaneous or serum) in babies with jaundice. Action is required whe n serum bilirubin (SBR) is above gestation and age cut-offs (e.g. >300 mol/l in term infan t at 72hr) or rapidly rising. Causes of elevated SBR Exaggerated physiological jaundice (e.g. preterm, bruising); sepsis; haemolytic disorders; hepatic disease. Treatment of elevated SBR \200 \200 \200 b p.195). Stop bilirubin rising to level that may cause kernicterus ( Treat any underlying cause, e.g . sepsis. Start \200\230blue light \200\231 phototherapy (converts bilirubin to water-solu ble form that can then be excreted in urine). Use age/gestation speci \201 c charts to det ermine level to start phototherapy (see Fig. 6.2). Be aware of risk factors (family history , exclusive breast feeding, Rh or blood group incompatibility). Measure SBR frequently (4 \200\22324-hourly depending on circumstances) and stop when falls below treatment level. En sure adequate hydration. d or i temperature; eye Cover eyes (phototherapy side effects: dam age; diarrhea; dehydration; rash; separation from mother). Exchange transfusion intraven ous immunoglobulin (IVIG) if very high SBR (e.g. >450 mol/L in term infant at 48hr) or rap id rise (>8.5 mol/L/hr). In the UK the National Institute of Clinical Excellence (NICE) ha s produced guidance on investigation and management of newborn jaundice (see Fig 6.2) \200 \224the full guideline also includes gestation speci \201 c treatment thresholds and chart s: M http://guidance.nice.org.uk/CG98. \200 Jaundice in the \201 rst 24hrs Assume it is pathological. Start phototherapy. Check SBR, FBC, direct coombs test (DCT), and blood group. Consider septic screen/ TORCH. Causes Haemolysis (e.g. Rh disease), red cell enzyme defects (e.g. G6PD de \201 ciency), r ed cell membrane defects (congenital spherocytosis, ellipto- cytosis), sepsis, severe bruis ing. 06_McClure_06.indd 130 11/14/2012 11:22:42 PM NEONATAL JAUNDICE 131 Date of birth Baby \200\231s name Direct Antiglobulin Test Time of birth >=38 weeks gestation Hospital number shade for phototherapy Multiple Single 550 500 Exchange transfusion 450 ) e r t i l / l o m o r c m 400 Phototherapy 350 300 i ( 250 n b u r i l i i 200 b m u r e s 150 100 50 l a t o T 0 7 8 9 10 11 12 13 14 0 1 2 3 4 5 6 Days from birth Baby \200\231s blood group Mother \200\231s blood group Fig. 6.2 Bilirubin thresholds for phototherapy and exchange transfusion in infants with hyp erbilirubinaemia. Reproduced from: Neonatal Jaundice. National Collaborating Center for Wom en \200\231s and Children \200\231s Health, May 2010, with the permission of the Royal Coll ege of Obstetricians and Gynecologists. Prolonged jaundice (>14 days in term infant; >21 days in preterm) All infants require inves tigation and measurement of conjugated bilirubin. If conjugated hyperbilirubinaemia present , further specialized investigation will be required. Ask about pale stools/dark urine. Causes Breastfeeding (benign, self-limiting, and usually resolves by 12wks), enclosed bleed ing (e.g. cephalhaematoma), prematurity, haemolysis, sep- sis, hypothyroidism, conjugated j aundice, hepatic enzyme disorders (e.g. Crigler \200\223Najjar Syndrome, Lucy \200\223Drisc oll disease). Initial investigations SBR (total and conjugated), U&E, FBC, DCT, blood group, thyroid func tion test (TFTs), LFTs, and glucose. Treatment Depends on cause. Rarely phototherapy is bene \201 cial, e.g. Crigler \200\223Na jjar Syndrome. Conjugated jaundice (conjugated SBR >25 mol/L)) Stools may be clay-colored in obstructive jaundice. Causes Sepsis, TPN, biliary tract obstruction (e.g. biliary atresia, choledo- chal cyst), v iral hepatitis; TORCH infections, 1-antitrypsin de \201 ciency, cystic \201 brosis, i nspissated bile syndrome after haemolytic disease, galac- tosaemia, other inherited metabol ic disease, idiopathic giant cell hepatitis. Initial investigations As for prolonged jaundice. Further investigations include radiology, enzyme testing, viral serology, liver biopsy, histology. Treatment Depends on cause. 06_McClure_06.indd 131 11/14/2012 11:22:42 PM CHAPTER 6 Neonatology 132 Hypoglycaemia \200 Measurement of blood glucose using glucose reagent strips is unreliable. Use blood glucose analyzer or laboratory measurement. In newborn period de \201 ned as <2.6mmol/L. Blood glu cose drops naturally in \201 rst few hours after birth before normalizing \200\224newborn s have increased ability to utilize ketones/lactate for energy. All infants should be encou raged to feed in \201 rst hour if well enough. At risk groups for hypoglycaemia include; infant of diabetic mother; <2500g or <3rd centile for weight; <37/40 gestation; maternal be ta- blockers; birth asphyxia. Check blood glucose in all infants who are unwell/lethargic/j ittery. \200 \200 Causes \200 Reduced metabolism (IEM) (e.g. galactosaemia). Increased polycthaemia, haemolytic disease, seizures. Hyperinsulinism: hyperplasia, transient. Miscellaneous: infusion. Other rare adre nal insuf \201 ciency. glucose stores: preterm, IUGR, LBW, inborn errors of glucose consumption: sepsis, hypothermia, perinatal hypoxia, maternal diabetes mellitus, BWS, pancreatic islet cell maternal \237 blockers, tissued or malfunctioning IV causes: fetal alcohol syndrome, pituitary insuf \201 ciency, Presentation Commonly asymptomatic. Jitteriness, apnoea, poor feeding, drowsiness, seizures , cerebral irritability, hypotonia, macrosomia (if hyperinsulinism). Investigation Blood glucose should be measured in \201 rst hour in all high risk infants. Apart from regular blood glucose measurements, further investigation is not usually requir ed if cause evident (e.g. IDM). Suspicious patterns of hypoglycaemia meriting investigation include; Recurrent hypoglycaemi a in term infant despite functioning intravenous infusion (IVI) of glucose 10%. Severe (<1m mol/L) and/or recurrent (>1) hypoglycaemia. Symptomatic hypoglycaemia. High glucose require ment (>8mg/kg/min). Hypoglycaemia and prolonged jaundice (panhypotpituitarism) or sodium ab normalities (adrenal problems). Hypoglycaemia with genital or midline abnormalities. \200 \200 \200 Calculating IV glucose infusion rate (mg/kg/min) Infusion rate (mg/kg/min) equals: IV rate (mL/hr) \227 % glucose in IV infusion x 0.167 weight in kg 06_McClure_06.indd 132 11/14/2012 11:22:42 PM HYPOGLYCAEMIA 133 First line tests (taken when hypoglycaemic): \200 Blood for glucose, insulin, growth hormone, cortisol, -hydroxybuyrate, free fatty acids, amino acids (consider C-peptide, lactate, and ammonia). Urine for urinalysis (ketones), ami no and organic acids. Further investigations as guided by results/clinical biochemist. \200 Prevention of hypoglycaemia in at-risk infants (see list of causes on b pp.98, 132). \200 \200 Adequate feed soon after birth (<1hr) and then at least 3-hourly. Monitor blood glucose lev els (pre-feed), keep warm, support feeding. Prognosis Profound/prolonged hypoglycaemia can cause neurological damage \200\224 exact lev el/duration after which this may occur is unclear. Treatment of hypoglycaemia Symptomatic or severe hypoglycaemia (glucose<1.0mmol/L) \200 \200 IV bolus 3 \200\2235mL/kg of glucose 10% Follow with 10% glucose infusion IV (4 \200\2236mg /kg/min) Asymptomatic (glucose <2.0mmol/L or 2.0 \200\2232.6mmol/L on 2 occasions) \200 Enterally fed infants: \200 \200 \200 \200 \200 Infants on IV \202 uids: \200 \200 inspect feed chart (frequency/volume, etc.) if reluctant to feed \200\224consider NGT if no t tolerating milk \200\224consider IV give early milk feed (consider larger volume) monitor with pre-feed blood glucose levels check IV line is working if glucose <1.0mmol/L \200\224give bolus then increase infusion ra te/ concentration if glucose >1.0mmol/L \200\224increase infusion rate/concentration Resistant hypoglycaemia (glucose requirement >8mg/kg/min) \200 \200 \200 Seek specialist advice, as hyperinsulinism likely Increase background glucose infusion (cen tral IV access needed) Glucagon 0.5mg IM can be given in emergency \200\224rebound increase d insulin secretion will occur Treatment options include: \200 \200 \200 \200 Enter al feeding promotes normality. Aim to wean off IV as soon as able High concentrations of gl ucose (>12.5%) require central IV access Monitor plasma sodium if on IV \202 uids diazoxide (given with chlorthiazide to counteract \202 uid retention) somatostatin (octre otide) nifedipine surgery (subtotal pancreatectomy) \200 06_McClure_06.indd 133 11/14/2012 11:22:42 PM CHAPTER 6 Neonatology 134 Neonatal seizures See also b p.508. Incidence 2 \200\2234/1000 live births. Usually occur 12 \200\22348hr after delivery. Can be generalized or focal, and tonic, clonic, or myocloni c. Subtle seizure patterns (lip-smacking, limb-cycling, eye deviation, apnoeas, etc.) can b e dif \201 cult to identify or differentiate from other benign condi- tions that may mimic seizures: \200 Startle or Moro re \202 exes; \200 Normal \200\230jittery \200\231 mo vements ( \201 ne, fast limb movements that are abated by holding affected limb); Sleep my oclonus (REM movements). A large proportion of clinically diagnosed seizures are not associated with electrical seiz ure activity and many electrical seizures do not manifest clinically. Causes \200 Brain injury: \200 \200 \200 \200 \200 CNS infection: \200 \200 Cerebral malf ormation. Metabolic: \200 \200 \200 \200 \200 Neonatal withdrawal Kernicterus Rar e syndromes: \200 \200 hypoxic ischaemic encephalopathy (HIE); intracranial hemorrhage; cerebral infarction (ischa emic or haemorrhagic); cerebral oedema; birth trauma. meningitis (e.g. GBS, coliforms); encephalitis (e.g. HSV, CMV). \200 hypoglycaemia; hypo- or hypernatraemia; hypocalcaemia, hypomagnesia; pyridoxine dependent s eizures; non-ketotic hyperglycinaemia. \200 \200 from maternal medication or substance abuse. (b p.195). benign familial neonatal seizures (autosomal dominant); early myoclonic encephalopathy. With improved access to neuroimaging, fewer infants are being catego- rized as \200\230ben ign \200\231 or \200\230idiopathic \200\231 seizures. Neonatal stroke is increasingly reco gnized. Investigation and management This should include family history, history of pregnancy and d elivery, com- plete examination, evaluation for infection, serum electrolytes, calcium, mag nesium, glucose, and blood gas. If available, cerebral function analysis monitoring (CFAM) should be commenced. 06_McClure_06.indd 134 11/14/2012 11:22:42 PM NEONATAL SEIZURES 135 If appropriate, further investigation may include radiological evaluation, e.g. cranial MRI , toxicology screening, serum ammonia, urine organic acids, serum amino acids, karyotype, a nd TORCH screening. Treatment of neonatal seizures \200 Immediate: give O2, maintain airway, insert IV, treat underlying cause. When to start antic onvulsants is controversial because risks and bene \201 ts of treatment have not been prop erly evaluated; usual indication is >3seizures/hr or single seizure lasting >3 \200\2235min particularly if evidence of cardio-respiratory compromise. First-line anticonvulsant: IV p henobarbital (10 \200\22320mg/kg bolus; give further 10 \200\22315mg if seizures persist af ter 30min; maintenance dose 5mg/day). Second-line For intractable seizures pyridoxine (50mg ). (cid:1) Depending on cause probably safe to stop treatment after a few days of no seizur es, but many clinicians prefer to wait until several months before ceasing. \200 IV clonazepam, IV midazolam, or IV phenytoin. consider therapeutic trial of parenteral Prognosis Prognosis varies with the cause of seizures, but is generally good for idi- opath ic seizures, sleep myoclonus, hypocalcemia, and benign familial ne- onatal seizures. There is a signi \201 cant risk of adverse neurodevelopmental outcome after meningitis, HIE, hyp oglycemia, cerebral infarction, hypo- or hypernatraemia, cerebral malformations, kernicteru s, and some inborn er- rors of metabolism. 06_McClure_06.indd 135 11/14/2012 11:22:43 PM CHAPTER 6 Neonatology 136 The \202 oppy infant See also b pp.538 \200\223547 and p.944. It is often helpful to divi de causes into those that are \200\230central \200\231 involving the CNS (so-called \200 \230 \202 oppy strong \200\231) and \200\230peripheral \200\231 involving lower motor neu rons, neuromuscular junction (NMJ), or primary muscle disease ( \200\230 \202 oppy weak \200\231). Range of clinical features \200 Common \200\230frog-leg \200\231 posture; respiratory failure; obstetric problems (e.g. po lyhydramnios due to impaired swallowing, breech presentation); HIE. Central strength; i or normal tendon re \202 exes. Peripheral myotonia, fasciculations, or fatiguing); d or norma l tendon re \202 exes; little facial expression; micrognathia; high arched palate; ptosis; undescended testes; limb contracture/deformities (severe is arthrogryposis multiplex conge nital); hip dislocation. to \200\230central \200\231 and \200\230peripheral \200\231 diseases: generalized hypoton ia; conditions: encephalopathy; dysmorphism; reasonable muscle causes: normal conscious level; muscle signs (weakness, Management \200 Exclude treatment. any respiratory failure with O2 or ventilatory support as required. Trea t Examine for above clinical features to help distinguish cause. Examine both parents for p ossible disease, e.g. maternal myasthenia gravis or myotonic dystrophy (possibly undiagnose d!). Elicit (e.g. polyhydramnios). \200\230Central \200\231 screen; ESR/CRP; TFT; karyotyp e; cranial ultrasound; CT/MRI; EEG; IEM screen; maternal drug screen; genetics opinion if d ysmorphic. \200\230Peripheral \200\231 cause: consider \200\224serum creatinine phosphokin ase; speci \201 c cytogenetics (e.g. myotonic dystrophy); electromyogram (EMG), nerve cond uction studies; muscle or sural nerve biopsy; muscle ultrasound; edrophonium 20micrograms/k g test dose l followed 30s later (if no adverse reaction) with 80micrograms/kg IV (causes d ramatic improvement in some forms of myasthenia gravis); echocardiogram (storage diseases). Spinal cord damage (rare): consider in the infant who has a \202 accid paralysis from bi rth. Associated with rotational forceps delivery. Immobilize neck. Seek specialist advice. MRI. Refer to pediatric neurologist. severe systemic illness: e.g. sepsis that requires prompt \200 family history (e.g. maternal myotonic dystrophy); antenatal history cause: consider \200\224blood glucose; U&E; Ca2+; Mg2+; septic Prognosis Causation-dependent (see Box 6.1) and very variable. Some causes are fatal, e.g. type 1 SMA. 06_McClure_06.indd 136 11/14/2012 11:22:43 PM THE FLOPPY INFANT 137 Box 6.1 Causes \200\230Floppy strong \200\231 or \200\230central \200\231 involving CNS \200 \200 \200 \200 \200 \200 IEM \200 \200 \200 \200 \200 \200 \200 Prematurity HIE Hypoglycaemia Sepsis Electrolyte disturbance Drug-related Hypothyroidism Chromosomal disorders (e.g. trisomy 21) CNS malformations Benign congenital hypotonia Underlying syndrome (e.g. Prader \200\223Willi syndrome) Cervical spinal cord tra uma (birth injury) \200\230Floppy weak \200\231 or \200\230peripheral \200\231 involving lower neurology, NM J, or primary muscle disease \200 Spinal muscular atrophy (SMA), particularly type 1 (previously known as Werdnig \200\223Hof fman disease) Myasthenia gravis (transient or congenital) Congenital myotonic dystrophy (au tosomal dominant inheritance from mother) Congenital muscular dystrophies Congenital myopat hies Metabolic myopathies Peripheral neuropathies Spinal cord injury \200 \200 \200 \200 \200 06_McClure_06.indd 137 11/14/2012 11:22:43 PM CHAPTER 6 Neonatology 138 Hydrops foetalis Abnormal accumulation of \202 uid in skin and body compartments. Results from rate of production of interstitial \202 uid exceeding absorption. Characterized in the fetus by: Gross generalized oedema; Ascites; Pleural pericardial ef fusions. \200 \200 If still present at birth, it results in severe illness. Incidence 71/2500 to 1/4000 births. Causes Hydrops is due to underlying disease, singularly or in combination result- ing in: i capillary hydrostatic pressure; d colloid osmotic pressure; lym- phatic obstruction; capil lary leaking (see Box 6.2 for summary). Box 6.2 Some causes of hydrops foetalis Immune Haemolytic disease of the newborn: alloimmune, Rh, Kell, other. Non-immune \200 Cardiac: \200 structure \200\224 hypoplastic left or right heart arrythmia \200\224 SVT, atrial \202 ut ter cardiomyopathies \200\224 Ebsteins anomaly, in-utero closure of ductus arteriosus, \200 Genetic: Fetal anemia: maternal hemorrhage Infection: Malformation: bowel atresia AV malformation Lymphatic (cystic hygroma) Idiopathic TORCH or other viral infection \200 Turners, Trisomy (21,13,18, etc.) twin-twin transfusion, Thalassaemia, foeto- \200 TORCH, Parvovirus B19 congenital cystic adenomatoid malformation (CCAM), \200 \200 Associated complications \200 Intrauterine/perinatal death. \200 Obstetric complication s, e.g. shoulder dystocia. \200 Preterm labor. \200 Pulmonary hypoplasia (pleural effus ions). \200 Perinatal asphyxia. 06_McClure_06.indd 138 11/14/2012 11:22:43 PM HYDROPS FOETALIS 139 Management Disorders treatable antenatally \200 Intrauterine blood transfusion (IUT) for haemolytic disease/ parvovirus infection Anti-arrh ythmia drugs to treat fetal SVT Laser ablation of fetal vessels (twin-twin transfusion synd rome) \200 Birth planning Before birth organize expert help \200 If anemia likely, have available fresh CMV \200\223ve, O \200\223ve blood, irradiated (if previous IUT), cross-matched blood against the mother Prepare for full resuscitation, vent ilation, UVC insertion, paracentesis (ascites), or pleural effusion drainage Neonatal management \200 Resuscitation: \200 \200 \200 Supportive management: \200 cardiac support \200\224 \200 respiratory support \200\224 \200 chest tube placement, drainage of ascites \200 \202 uid and electrolyte management \200 treatment of anemia: blood transfusion or par tial exchange \200 transfusion \200 treatment of infections \200 octreotide to treat chylothorax and ascites ventilation, intubation paracentesis, thoracentesis blood transfusion or partial exchange t ransfusion pressors, ionotropes mechanical ventilation Prognosis For fetuses or infants with non-immune hydrops, the survival rates are variable, in the range of 50%. Higher survival is reported in infants with SVT, chylothorax, and parv ovirus infections, and lower rates in those with chromosomal abnormalities. Survival rates with immune hydrops is > 80%. Neurodevelopmental outcome depends on cause. 06_McClure_06.indd 139 11/14/2012 11:22:43 PM CHAPTER 6 Neonatology 140 Routine care of the newborn Routine measurements Measure within 1hr of birth: \200 73.5kg ); Weight (term mean \200 Head circumference (mean 750cm). \200 Body length (mean 735cm); Usually babies are not weighed again until day 3 \200\2235 and then alternate daily whilst they remain in hospital. It is normal to lose weight after birth due to water loss, but wei ght loss should not exceed 10% of birth weight. Birth weight should generally be regained b y day 7. Subsequent mean growth is 20 \200\22330g/day until age 6mths. Vitamin K (phytomenadione) To prevent haemorrhagic disease of the newborn, vitamin K1 is ro utinely given within 48hr of birth. Dose 1mg IM (preferred) in term infants or alternatively 2mg orally on days 1 and 7, and, i f breastfeeding, also on day 28. Cord care Immediately after birth clamp the cord with a purpose-made device. Keep the umbil icus clean and dry. Antibiotic powders or sprays are not rou- tinely required. The cord usu ally detaches after 7 \200\22310 days. If umbilical granulomas develop, clean with alcohol wipes and consider chemical cau- tery (silver nitrate stick). Thermal care Babies should be delivered in a warm room, rapidly dried with a warm towel, an d then immediately wrapped or placed skin to skin on the moth- er \200\231s front and then covered with a warm towel and a hat. Routine observations Record soon after birth and then daily whilst in hospital. Mean pulse is 120 \200\223160beats/min, respiratory rate 35 \200\22345breaths/min, and temperature 36. 9*C. Infants should be nursed in an ambient temperature of 20 \200\223 22*C. Bathing Not required until day 2 or 3. Use tepid water. Genitalia should be cleaned super \201 cially only. Do not retract foreskin; it is attached to the glans. Biochemical screening In the UK, all infants should undergo a screening heel prick blood te st placed on a speci \201 c card between day 3 and 10 ( \200\230Guthrie \200\231 test). Re gional variation exists, but commonly screened diseases include: \200 i phenylalanine); congenital hypothyroidism(i TSH); Phenylketonuria ( cystic \201 brosis (i immune-reactive trypsin). Medium chain acetyl-CoA de \201 ciency. Sickle cell disease. \200 06_McClure_06.indd 140 11/14/2012 11:22:43 PM ROUTINE CARE OF THE NEWBORN 141 Positive tests require follow-up and more detailed testing. Newborn hearing screening All infants in the UK will have their hearing screened (otoacoust ic emis- sion \200\224OAE) within the \201 rst 4wks of life. Automatic auditory brainstem re- sponse (AABR) testing is carried out if any uncertainty in OAE response. Neonatal immunization See also b pp.728 \200\223729. In several countries hepatitis B immun iza- tion of all newborns is recommended. In the UK, immunization is only offered to infant s of seropositive mothers. Similarly, in the UK, Bacille Calmette \200\223Gu rin (BCG) sho uld be offered to babies born to parents of ethnic groups or communities with a high incide nce of close contact with a sputum-positive case for tuberculosis (TB). 06_McClure_06.indd 141 11/14/2012 11:22:43 PM CHAPTER 6 Neonatology 142 Milk feeding Methods \200 Bottle. \200 Tube feeding (if too ill/immature to suck). \200 \200 Naso/oro-gastic tube. Silastic naso-jejunal tube (severe gastro-oesophageal re \202 ux (GO R), aspiration, or recurrent apnoeas). Gastrostomy (if required long term, older children). Breastfeeding Breast feeding is a learned skill for both mother and baby. Establishing feed ing can take time, and it is vital that good support is available (breast feeding advisors or midwifes with appropriate training). Advantages d \200 \200 i \200 d \200 \200 d \200 d \200 d \200 Maternal post partum hemorrhage. maternal contraceptive effect. Mild Bonding. Maternal breast cancer risk. Cheap. Infant mortality (less relevant in developed world). GI and respiratory infection rate. Lat er autoimmune disease incidence (e.g. type I diabetes mellitus, atopic diseases). i later IQ. (cid:1) \200 Problems \200 \200 Cracked/sore nipples. Maternal anxiety (breast fed babies can gain weight slower than their bottle-fed couterparts \200\224give reassurance and support). Small risk of hypernatraemic dehydration if low milk intake (suspect if weight loss >10%). Contraindications \200 \200 \200 \200 \200 +ve maternal HIV status (in developed countries). Certain maternal medications (e.g. amioda rone). Maternal herpes zoster over breast. Infantile galactosaemia or phenylketonuria (PKU) . Primary lactose intolerance (very rare). Expressed breast milk (EBM) Usually mother \200\231s own breast milk, but some units have d onor breast milk banks which can be of value, particularly in extreme preterm infants. EBM is usually used to establish feeding in preterm infants, but is also useful when top-up feeds are required, if mother and baby are separated for any reason, or if there are other maternal problems e.g. cracked/sore nipples or breast engorgement. Once expressed, can refrigerate and use within 24 \200\22348hr, or freeze and use for up to 3mths. 06_McClure_06.indd 142 11/14/2012 11:22:43 PM MILK FEEDING 143 Formula milk Normal volume required is 150mL/kg/day. See Box 6.3 for different types of for mula milk available. Advantages \200 \200 Paternal involvement. Milk intake determined. Problems \200 \200 \200 Constipation. Oral thrush. Milk bezoars. Box 6.3 Types of formula milk available \200 Cow \200\231s milk formula: standard milk used. Extensively modi \201 ed, e.g. to d solute load, i iron and vitamins. Formula used from birth is predominantly whey protein, whilst \200\230follow on \200\231 milks (for \200\230hungrier \200\231 infants >6mths old) are pr edominantly casein-based Soya milk: or lactose intolerance, but use not now recommended due to high levels of phyto-oestrogens and availability of other alternatives Hydrolysed cow \200\231s milk formula: peptides. Indicated for prophylaxis or treatment of cow \200\231s o r soya milk protein allergy. Elemental formulas: hydrolysed to amino acids. Indicated for s evere milk protein allergy or malabsorption. In addition, many specialized formula milks ex ist for conditions such as: preterm/LBW infants; gastro-oesophageal re \202 ux (thickened with cornstarch); metabolic diseases; lactose intolerance (lactose- free milk); poor growth (high energy formulas); malabsorption, e.g. Pregestimil . previously recommended for cow \200\231s milk protein allergy e.g. Nutramigen . Contains short e.g. Neocate . Cow \200\231s milk protein is fully Trophic feeding (gut-priming) The term describes practice of feeding small milk volumes (0. 5 \200\2231mL/kg/hr of EBM) to enhance gut structure and function in infants too ill or imm ature to tolerate substantive milk feeds. Evidence suggests that in the preterm infant it i mproves GI motility and function, as well as achieving clinical outcomes of i weight gain, i head growth, d infection risk, and improved later milk tolerance. 06_McClure_06.indd 143 11/14/2012 11:22:43 PM CHAPTER 6 Neonatology 144 Routine neonatal examination Each baby must be examined at least once in the \201 rst wee k, usually on day 1 after birth. Such child health surveillance can be done by a hospital p ediatrician, advanced neonatal nurse practitioner, general practitioner, or specially train ed midwife/nurse. Purpose \200 \200 \200 \200 Maternal reassurance. Health education; explaining common variations. Detecting asymptomati c problems, e.g. congenital heart disease, DDH. Screening for rare, but serious conditions. Order of examination \200 \200 