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The neonatal guidelines presented on this site were developed by clinicians primarily for use by medical and nursing personnel working in newborn special care units throughout Victoria. They detail the initial assessment and management of many common (and some rare but important) conditions encountered during the early newborn period. They do not constitute a textbook, rather, they are designed to acquaint the reader rapidly with the clinical problem and provide practical advice regarding assessment and management. These guidelines have been developed, where possible, by achieving consensus between practicing clinicians. They do not however necessarily represent the views of all neonatologists working in Melbournes Neonatal Intensive Care Units. The recommendations contained in these guidelines do not indicate an exclusive course of action, or serve as a standard of medical care. Variations, taking individual circumstances into account, may be appropriate. The authors of these guidelines have attempted to ensure the information upon which they are based is accurate and up to date. Users of these guidelines should confirm that the information contained within them, especially drug doses, is correct by way of independent sources. No responsibility is accepted for any inaccuracies or information perceived as misleading.
36. Intravenous Electrolyte Correction ..91 37. Intravenous Infusion For Scn Admissions ....92 38. Intubation .94 39. Jaundice In The First Two Weeks Of Life ...97 40. Listeria Monocytogenes Infection ..101 41. Meconium Aspiration Syndrome ....102 42. Meconium Stained Liquour, Delivery Room Management ..104 43. Meningomyelocele ..105 44. Metabolic Disease A Neonatal Approach .106 45. Necrotising Enterocolitis ......109 46. Normal Laboratory Values ....112 47. Osteopenia Of Prematurity ...116 48. Parvovirus Infection ...117 49. Percutaneous Central Venous Catheter Insertion .........................................................................118 50. Persistent Pulmonary Hypertension Of The Newborn ..121 51. Pneumothorax Drainage 123 52. Polycythaemia ..125 53. Respiratory Distress Syndrome (RDS) ..126 54. Resuscitation 128 55. Retinopathy Of Prematurity ..131 56. Small For Gestational Age Infants ..134 57. Shock ..136 58. Seizures .140 59. Sepsis .143 60. Single Umbilical Artery .....148 61. Small For Gestational Age Infants ..149 62. Supraventricular Tachycardia .....151 63. Surfactant Replacement Therapy ...154 64. Thrombocytopenia ..155 65. Thrombosis In Newborns ..157 66. Tracheo-Oesophageal Fistula/Oesophageal Atresia .159 67. Transfer Guidelines ....161 68. Transfusion ...164 69. Tuberculosis (TB) ...166 70. Umbilical Artery Catheterization ....167 71. Umbilical Cord Care ...169 72. Umbilical Hernias ....170 73. Umbilical Vein Catheterisation ....171 74. Undescended Testes (Cryptorchidism) ....173 75. Vomiting In The Newborn Infant .....174
A. Summary: - the abnormality should be covered with cling wrap - pay careful attention to thermoregulation and fluid management - refer early via NETS to tertiary referral centre with surgical facilities B. Introduction: - The diagnosis of exomphalos and gastroschisis is often but not invariably made antenatally by ultrasound. - These babies will usually be delivered at a tertiary referral centre. C. Gastroschisis: - small defect of the anterior abdominal wall to the right of the umbilicus through which bowel herniates - occurs in between 1:10,000-30,000 births - no covering sac, the surface of the bowel is usually oedematous and matted - associated anomalies are reported in up to 15% (mainly gastrointestinal) - prematurity and growth restriction are frequent - necrotising enterocolitis and malabsorption may occur - survival rates are about 90% D. Exomphalos: - a protrusion of intestinal contents through the abdominal wall at the umbilicus - covered by a thin membrane of amnion and peritoneum - herniation of the liver accompanies intestine if a large sac, omentum and intestine are present if a small
sac associated anomalies occur in 45 67% (eg trisomies, cardiac defects, G.I. and renal anomalies) survival rates are mainly dependent on whether other anomalies are present necrotising enterocolitis and malabsorption are associated complications
F. Management: - wrap abdomen of baby in cling film with gut lying well supported either on the abdomen if a small lesion or
supported by a foam rubber "doughnut". If in one position a length of bowel appears to have impaired blood supply or drainage i.e. looks purple or black, try gentle manipulation of the bowel into other positions to see if the circulation can be improved the bowel may need to be rotated on its pedicle to achieve a better circulation cotton wool covering or the use of moist packs is contraindicated. (Cotton wool adheres to the bowel wall, cannot be fully removed and causes peritoneal granulomas; moist packs rapidly become cold and lead to hypothermia) pass size 8 NG tube, place on continuous low-pressure suction or leave on free drainage and aspirate every 60 minutes place nil by mouth start IV infusion - Give usual Day 1 fluids infants with gastroschisis may loose large amounts of colloid fluid into the inflamed gut requiring vigorous colloid fluid replacement (eg Albumin 5% 10-20 mL/kg). Watch blood pressure
check blood glucose immediately and monitor closely because of the association of Beckwith-Wiedemann syndrome with exomphalos monitor temperature frequently. Patients with a ruptured exomphalos sac or gastroschisis may have major problems with temperature control due to evaporative heat loss contact paediatric surgeon and NETS to arrange transfer to surgical centre give antibiotics: Penicillin, Gentamicin (preferably after blood culture) take blood for FBE, electrolytes, blood culture, group and hold for cross match of blood
2. AMBIGUOUS GENITALIA
A. B. C. D. E. F. G. H.
Summary Introduction Management Clinical Evaluation Investigation
A. Summary:
Be very careful in your choice of words during the diagnostic period and do not sign the birth certificate until a definite decision as to the sex of rearing has been reached. - be aware of associated metabolic problems - palpable gonads imply the presence of testicular tissue - decisions as to sex of rearing may have no relationship to karyotypic, gonadal or genital status in isolation - there is some ongoing controversy as to when is the appropriate time to make decisions as to sex of rearing and who should be party to those decisions
B. Introduction:
Approximately 1 in 4,500 births are complicated by ambiguous genitalia. This situation is rarely anticipated and can be a source of great distress for parents, delivery room and nursery staff. Often there can be pressure on medical staff to "make it better" and assign a gender to the child arbitrarily in the first few hours after birth. This must be avoided at all costs. Staff must be careful in their choice of words when discussing the baby with parents. It is unnatural not to discuss a baby without using the terms "he" or "she" and it is easy to accidentally refer to the baby in a gender-orientated way. Parents who are often greatly distressed may assume that medical and nursing staff "know" what the gender of the baby "really is". Consequently any terminology used (deliberately or accidentally) will be given great emphasis by parents. This may lead to confusion and distress later if the suggested sex of rearing is at odds with initial "off the cuff" remarks. Parents may seek advice regarding the naming of their infant. The usual advice is to select non-ambiguous names (ie using gender specific names) since it is thought that by encouraging the use of ambiguous (non-gender specific) names, one is implying an ongoing sense of "ambiguity".
C. Management: - Be very careful in your use of terms when discussing the baby with ambiguous genitalia. Appropriate,
non-gender orientated terms are listed in Table 1. Never refer to the baby in question as "it" Table 1: Suggested phenomenology when dealing with babies with ambiguous genitalia.
FEMALE
She Clitoris Labia Ovaries Vagina, urethra
AMBIGUOUS
Your baby Phallus Folds Gonads Urogenital sinus
MALE
He Penis Scrotum Testes Urethra
The situation should be treated as a medical emergency, with paediatric endocrine advice being sought immediately
D. Clinical Evaluation:
Genital ambiguity can be quantified according to the Prader scale (Figure 1). Other relevant clinical details include: - gonads palpable in the labioscrotal or inguinal regions - size of the phallus - pigmentation of the genitalia - syndromic features - metabolic condition of the baby (paying particular attention to glucose, sodium and potassium) The baby's mother should also be examined for signs of hyperandrogenism. Care should be taken in the interpretation of examination findings in growth retarded or premature female neonates. These children may exhibit atrophic labia and clitoral oedema giving them an appearance of "pseudoambiguity". It is a moot point where the boundary lies between severe perineal hypospadias and genital ambiguity. Inability to palpate the gonads in this situation may be indicative of a diagnosis other than isolated hypospadias. Figure 1: Prader staging system for the degree of virilisation of the external genitalia.
Prader 2: Clitoromegaly with partial labial fusion forming a funnel-shaped urogenital sinus.
Prader 3: Increased phallic enlargement. Complete labioscrotal fusion forming a urogenital sinus with a single opening.
Prader 4: Complete scrotal fusion with urogenital opening at the base or on the shaft of the phallus.
(Prader Von, A. (1954). "Der genitalbefund beim Pseudohermaproditismus femininus des kongenitalen adrenogenitalen Syndroms. Morphologie, Hausfigkeit, Entwicklung und Vererbung der verschiedenen Genitalformen." Helv. Pediatr. Acta. 9: 231-248.)
E. Investigation:
Blood should be sent for: - electrolytes - gonadotropins (LH, FSH) - testosterone - urgent karyotype - serum 17-hydroxyprogesterone (17OHP) levels (after day 3 of life) Pelvic ultrasound (carried out by an experienced sonographer) should be undertaken as soon as possible. Other investigations which may or may not be subsequently relevant include: - sinugram - human chorionic gonadotropin stimulation test (to assess testosterone and dihydrotestosterone synthesis capability)
F. Differential diagnoses:
a) Gonads palpable, 46XY:
gonadal dysgenesis partial androgen insensitivity biosynthetic defect in either testosterone or dihydrotestosterone production b) Gonads impalpable, 46XX: - Congenital adrenal hyperplasia - gonadal dysgenesis - exogenous androgen exposure c) Mosaic karyotype: - gonadal dysgenesis
G. Ongoing Management:
Decision as to sex of rearing is made after opinions have been sought from the endocrine and surgical teams. It should be undertaken with the baby's parents after all the relevant investigation results have been discussed. The decision that will be influenced by an amalgam of:
the baby's karyotype gonadal status internal and external genital duct status potential for fertility and adequate sexual function cultural influences
Do not complete the baby's birth certificate until the sex of rearing has been decided. There is a 60 day period of grace between the birth of a child and when their birth certificate needs to be completed - hence there is no rush. If the "wrong" sex is entered on the form it is extremely difficult to correct and requires judicial intervention. Long term care:
families require long term medical and psychological support corrective surgery is usually undertaken within the first year of life infants with CAH and congenital syndromes have additional requirements for ongoing medical therapy disclosure to the patient as to their diagnosis is usually undertaken in mid to late adolescence when they have the ability to understand complex issues such as chromosomes, hormones etc, and possess some degree of emotional maturity
H. Further Reading:
Reiner WG Assignment of sex in neonates with ambiguous genitalia. Curr Opin Pediatr 1999 Aug;11(4):363-5. Ahmed SF, Hughes IA The genetics of male undermasculinization.Clin Endocrinol (Oxf) 2002 Jan;56(1):118
3. APNOEA
A. B. C. D. E. F. G.
Summary Introduction Differential Diagnosis Investigations Management Areas of Uncertainty in Clinical Practice References
A. Summary:
initially monitor all infants <34 weeks for breathing and heart rate apnoea on day 1 may not be due to idiopathic apnea of prematurity -consider sepsis or impending respiratory failure (esp when there is underlying surfactant deficiency) sudden increases in severity/frequency of apnoiec episodes suggests new pathology treatment requires acute resuscitation followed by diagnosis and treatment of specific causes symptomatic control with medication or ventilatory support may be required
B. Introduction:
Apnoea is defined as no inspiratory gas flow for 20 seconds or shorter if associated with bradycardia (<100bpm), cyanosis or pallor. Apnoea may be classified as
a) Central apnoea - a pause of alveolar ventilation due to immaturity of neurological controls. This may be
provoked by: - gavage feeding - aggressive pharyngeal suction - gastro-oesophageal reflux b) Obstructive apnoea - a pause in alveolar ventilation due to obstruction of the upper airway (usually at the level of the pharynx) - not detected by motion sensing monitors c) Mixed apnoea - a combination of central and obstructive apnoea - seen in over 50% of infants Apnoea is distinguished from periodic breathing (respiratory pauses > 3 seconds duration with less than 20 seconds of respiration between pauses) which may occur normally. Apnoea occurs in: - most infants <30wks - about 50% of infants at 30-32wks - about 10% of infants at 34wks Apnoea usually resolves by the time the infant is 36wks postconceptual age. There is evidence that apnoea of prematurity is not a risk factor for SIDS. There is no evidence that apnoea of prematurity causes subsequent neurodevelopmental morbidity although recurrent apnoea causes concern because of effects of repeated episodes of tissue hypoxia (especially on the gut and brain).
C. Differential Diagnosis:
Apnoea occurs with increasing frequency the more immature the infant. Various conditions may cause or aggravate apnoea:
Apnoea on day 1 is not normal. A sudden increase in severity/frequency of episodes suggests new pathology. The following lists important potential causes of apnoea according to infant age: a) Day 1-2: - sepsis - hypoglycemia - impending respiratory failure - polycythemia b) Days 3-6: - sepsis - impending respiratory failure - PDA - massive IVH - apnoea of prematurity c) Late: - sepsis - progressive post-extubation atelectasis - out grown dose of theophylline/caffeine - gastro-oesophageal reflux - presenting symptom of RSV infection
D. Investigations:
A thorough physical examination is mandatory with emphasis on cardiorespiratory and neurological status. Usually a septic screen and blood glucose estimation will be required. Further tests are determined by the need to look for specific conditions (see differential diagnosis) causing or aggravating apnoea.
E. Management:
1. The acute apnoeic episode: - Stimulate e.g. tickle or flick the feet or stroke the abdomen - Aspirate airway if no response, briefly suction the oropharynx then repeat stimulation - Bag and mask ventilation if still no response, using the amount of oxygen the infant was receiving prior to the apnoea (not 100%). Only increase the concentration of oxygen (by steps of 5-10%) if the infant's condition fails to improve despite effective bag and mask ventilation - If still no response, Positive Pressure Ventilation is required 2. Management of specific causes: Treatments will depend on the specific cause of the apnoea. 3. Symptomatic management: Various methods can be used to provide symptomatic control of apnoea until the infant 'out grows' this problem. When is symptomatic treatment useful? There is no 'right' answer to this question. The following suggestions fall within the spectrum accepted at most neonatal units. Episodes needing brief stimulation for cyanosis + bradycardia: >6 every 12hrs Episodes needing vigorous stimulation +/- oxygen: >1 every 24hrs Episodes needing PPV +/- oxygen: >1 episode every 24hrs
Attention should be given to posturing the infant to avoid obstruction of the upper airway Feeds may be given more frequently as smaller boluses to avoid excessive distension of the stomach Some infants benefit from maintaining their thermal environment in the lower part of the neutral thermal range Low flow oxygen into the incubator (approx 23-24%) may help when levels of oxygenation between apnoeas are borderline satisfactory. If used continuous saturation monitoring is needed to avoid risks of hyperoxia Medications
Both caffeine and theophylline are effective in short term reduction of symptoms - caffeine may have advantages because of its higher therapeutic ratio, once daily dosing, lack of need to assay blood levels. Longer term outcomes have not been thoroughly examined. Prophylactic use of caffeine has not been shown to be effective. Currently, caffeine is only available upon application to Canberra under the Special Access System.
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Aminophylline/ Theophylline 2.5 to 4mg/kg/dose 12 hourly(2.5mg wk1, 3mg wk2, 4mg>wk2) Commence maintenance 24hrs after loading dose for infants <=1kg, 12hrs after loading dose for infants>1kg
Medication is usually stopped when the infant is >=34wks gestation and apnoea free for 1 to 2 weeks. Monitoring is continued for a further week after medication is stopped.
Proposed mechanisms of action: - prevents pharyngeal collapse by splinting the nasopharynx, stabilizes the chest wall musculature, alters various reflexes (Herring-Breuer, Intercostal inspiratory inhibitory). Nasal CPAP may be given by various techniques - most simply via a cut down endotracheal tube inserted 2cm into one nostril. Initial pressure settings for nasal CPAP are 5 -7 cm H2O which may be adjusted according to clinical response. Possible side effects - barotrauma, nasal irritation, abdominal distension and feed intolerance.
When uncontrolled by other means intubation and positive pressure ventilation will be required. Initial ventilator settings will use short inspiratory times and minimal PIP pressures to minimise risk of lung injury.
b) The place of blood transfusion in treatment of apnoea: Presence and severity of apnoea correlate
poorly with the presence of anaemia. Theophylline has been shown superior to blood transfusion in improving symptoms of apnoea in anaemic infants (but in a very small study). The clinical benefits from transfusion appear greater the more severe the level of anaemia (the effects are trivial when the haemoglobin is about 100g/L). c) Kinesthetic stimulation (e.g. oscillating mattresses) to treat apnoea: Not recommended for either prophylactic or symptomatic control of apnoea. d) Doxapram as medication for symptomatic control of apnoea: May reduce apnoea in the first 48 hours of treatment, longer term risk and outcomes have not been established, It has similar short term effect to the methylxanthines in obtaining symptomatic control but must be given as a continuous IV infusion limiting any usefulness. The long term safety of doxapram has not been well studied. e) Caffeine and Prematurity (CAP) Trial: Currently in Melbourne infants are being enrolled in a trial testing the use of caffeine against placebo in the management of apnoea. The main outcome is long-term survival free of major disability. f) Use of oxygen by nasal cannulae to control apnoea: High oxygen flows given by nasal cannulae may achieve significant positive distending pressures. Possible side effects include inadequate heating and humidification leading to temperature control problems and increased nasal irritation. Pressures generated are not able to be monitored with this system.
G. References:
Infantile Apnea and Home Monitoring, National Institutes of Health Consensus Development Conference Statement 1986 http://consensus.nih.gov/cons/058/058_intro.htm Effect of blood transfusion on apnoea, bradycardia and hypoxemia in preterm infants C.F.Poets, U.Pauls, B.Bohnhorst Eur J Pediatr (1997)156: 311-316 Transfusion-induced changes in the breathing pattern of healthy preterm anemic infants P.Sasidharan, R.Heimler Ped. Pulmonlogy (1992)12(3):170-3 Relationship between determinants of oxygen delivery and respiratory abnromalities in preterm infants with anemia E.M.Bifano, F.Smith, J.Borer J.Pediatr 120(2Pt1):292-6, 1992 Feb High-Flow Nasal Cannulae in the Management of Apnea of Prematurity: A comparison with conventional nasal continuous positive airway pressure C.Sreenan, R.P.Lemke, A. Hudson-Mason, H.Osiovich Pediatrics 2001 107(5) 1081-3
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A. Summary: - history, physical examination and determining whether the neonate is "well" or "sick" is helpful in
assessment of a bleeding neonate simple and widely available lab tests are useful in investigating a bleeding neonate treatment depends on the cause of bleeding and clinical condition of the neonate "treat the baby not the numbers" consult a Paediatric Haematologist if in doubt
B. Introduction:
Neonates are susceptible to bleeding for various reasons: - immaturity of the haemostatic system because of quantitative and qualitative deficiency of coagulation factors - maternal disease and drugs - birth trauma - other conditions eg sepsis and asphyxia
C. Clinical presentation:
Bleeding in neonates may present with: - oozing from the umbilicus or stump - cephalhaematoma - bruising more than that anticipated after delivery - bleeding from peripheral venipuncture or procedure sites - bleeding into scalp - bleeding following circumcision - petechiae - intracranial haemorrhage - bleeding from mucous membranes - unexplained anaemia and hypotension A detailed history and complete examination is essential in the assessment of a bleeding neonate. Particular points in the history include: - maternal diseases such as ITP, preeclampsia and diabetes - maternal exposure to drugs such as aspirin, anticonvulsants, rifampicin and isoniazid - family history of bleeding disorders - previous affected siblings Physical examination will determine whether the neonate is "well" or "sick", which is very useful as the differential diagnosis is very different in the two circumstances.
D. Differential diagnosis:
Causes of bleeding in a "well" neonate: - immune thrombocytopenia (alloimmune or autoimmune (maternal ITP)) - vitamin K deficiency - inherited coagulation factor deficiencies such as haemophilia - bleeding from anatomic lesions such as a haemangioma, A-V malformation Causes of bleeding in a "sick" neonate: - DIC - usually associated with sepsis, asphyxia, severe RDS or NEC - consumption thrombocytopenia without depletion of coagulation factors - liver failure
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Bleeding at a single site is more likely to have an anatomic or structural component. Major bleeding from any primary cause may induce a secondary DIC, which may mask the original pathology.
E. Laboratory investigations:
first line tests include: platelet count APTT PT fibrinogen d-dimer
The results should be interpreted in the context of normal laboratory values for gestation and postnatal age.
F. Management:
Management depends on: - the cause of bleeding and - clinical condition of the neonate 1. DIC: treat the primary condition blood products i.e. platelets and FFP are used on clinical grounds: 10-15 ml/ kg of FFP can be given to correct coagulation abnormality if platelets <50 and actively bleeding, transfuse platelets 2. Vitamin K deficiency: - IV vitamin K1, 1mg is usually effective within hours - also give FFP 10-15 mls/kg to immediately increase the levels of clotting factors 3. Thrombocytopenia: - Refer to Thrombocytopenia 4. Inherited factor deficiency: - initial therapy with FFP after blood taken for specific factor assays - specific factor replacement when diagnosis known
G. Further Reading:
Christensen, MD : Hematologic problems of the neonate. W.B. Saunders Company , first edition; 2000.
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A. Introduction:
Blood gases are helpful in determining the adequacy of respiratory function of the baby (oxygenation and ventilation) as well as the baby's acid/base balance. Blood gases can be performed from arterial (either a stab or via an arterial line), venous (through an intravenous cannula) or capillary (heel prick) specimens. Repeated arterial stabs are strongly discouraged, as they are painful and do not represent oxygenation as well as pulse oximetry. Arterial stabs may be taken from the radial artery (provided there is also a palpable ulnar pulse) or from the brachial artery, although this is in close proximity to the median nerve. All three specimens will give a good assessment of acid/base status and pCO2, whereas arterial specimens are required to assess pO2. It is always important to note the FiO2 (percentage inspired oxygen) when interpreting blood gases. Each unit should have their own reference ranges.
B. pH:
The pH is a negative logarithm of hydrogen ion concentration [H+], that is a decrease in pH from 7.0 to 6.0 represents a ten-fold increase in [H+]. Although each unit will define neonatal pH a little differently, if, say a normal neonatal pH is defined as 7.30 to 7.40, then:
The pH is proportional to HCO3 (or base excess), therefore: an abnormal increase in HCO3 (or base excess) increases the pH (metabolic alkalosis) an abnormal fall in HCO3 (or base excess) decreases the pH (metabolic acidosis)
The pH is inversely proportional to pCO2, therefore: an abnormal increase in pCO2 decreases the pH (respiratory acidosis) an abnormal decrease in pCO2 increases the pH (respiratory alkalosis)
C. Acid (H+):
Many organic acids are produced during normal metabolism. Sometimes they can accumulate in the blood (e.g. lactic acid). The hydrogen ion (H+) may be 'mopped up' by buffers including bicarbonate (HCO3). Bicarbonate is unique because it can be converted to CO2, which can be blown off by the lungs (provided the baby is not in respiratory failure). The following bi-directional equation demonstrates this:
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F. Metabolic Acidosis: (e.g. HCO3 < 17 mmol/L or B.E. < minus 6.0 mEq/L, pH < 7.30)
This may occur where there is a rise in free H+ ions that cannot be totally buffered. In this case the anion gap is increased. Causes include lactic acidosis secondary to tissue hypoxia (e.g. hypotension, sepsis and PDA) or the inability to excrete/buffer accumulated organic acids (e.g. protein loading and renal immaturity). Another common cause of metabolic acidosis, particularly in the extremely premature infant is excessive loss of HCO3 in the urine or gut. In this case the anion gap is normal. Metabolic acidosis is rarely due to an inborn error of metabolism. The treatment of a metabolic acidosis is to treat the underlying cause, consider volume expansion (e.g. 10 mls/kg of normal saline) if the baby is thought to be hypovolaemic or to administer NaHCO3 if the metabolic acidosis is severe (controversial) or refractory (e.g. bicarbonate wasting). Bicarbonate should not be given if the pCO2 is elevated as the pH will not change (according to the above formula, a metabolic acidosis is merely being replaced by a respiratory acidosis).
G. Metabolic Alkalosis: (e.g. HCO3 > 28 mmol/L or B.E. > plus 4.0 mEq/L, pH > 7.40)
This occurs where the plasma HCO3 or base excess is abnormally high. Causes include hypochloraemia (the level of bicarbonate and chloride in plasma are reciprocally related), which may be due to diuretic therapy or upper gastrointestinal obstruction (e.g. pyloric stenosis). The baby may also be trying to compensate for a respiratory acidosis, although the pH will never become alkalotic (as the baby will never over-compensate). The treatment of a metabolic alkalosis is to treat the underlying cause (e.g. chloride replacement) or the underlying cause of the respiratory acidosis.
H. Base Excess:
This is one way of looking at the metabolic component. It refers to the 'amount of base that would have to be added to one litre of the baby's blood at 40 mmHg pCO2 to return the pH to normal. It is a calculated value and will be erroneous if the pCO2 is not normal. In these circumstances, the 'metabolic' component of the blood gas should be assessed using the plasma HCO3 level.
I. Acid-Base Disorders:
Any one of the above four scenarios can occur in isolation, with or without compensation. These are classified as simple acid-base disorders. When a combination of simple acid-base disturbances occurs, the baby has a mixed acid-base disorder. When there is a mixed disorder, it is sometimes difficult to know which is the primary and which is the compensatory component. In such circumstances a helpful principle is that normal physiological processes never over-compensate. The pH can be relatively normal in the following situations - respiratory acidosis with metabolic compensation - metabolic acidosis with respiratory compensation - metabolic alkalosis with respiratory compensation - respiratory alkalosis with metabolic compensation The fourth is extremely unusual in neonates.
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L. References:
Ganong WF. Review of Medical Physiology, 19th Ed. 1999, p. 697-704. Appleton & Lange, Stanford, Connecticut Taeusch HW, Ballard RA (Eds). Avery's Diseases of the Newborn 7th Ed. W.B. Saunders Company, Philadelphia. 1998
6. BLOOD PRESSURE
A. B. C. D. E.
Introduction Method of Blood Pressure Measurement 'Normal' Blood Neonatal Blood Pressure Values Areas of Uncertainty in Clinical Practice References
A. Introduction:
The recognition and treatment of hypotension are particularly important to avoid complications such as cerebral ischaemic injury or intraventricular haemorrhage. On the other hand, hypertension in the newborn is increasingly seen as a complication in infants with bronchopulmonary dysplasia and who are receiving steroid treatment. Arterial blood pressure (BP) is determined by: cardiac output peripheral vascular resistance In general hypotension indicates inadequate systemic blood flow or left ventricular output and therefore inadequate tissue perfusion, although this is not always the case.
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In clinical practice, the infant's blood pressure is generally considered to be adequate as long as urine output (> 1ml/kg/hr) and capillary refill (< 3 seconds) are within normal limits and there is no metabolic acidosis. However, these are not reliable indicators of tissue perfusion. Arbitrary definitions of hypertension are as follows: term infant: systolic > 90 mmHg, diastolic > 60 mmHg preterm infant: systolic > 80 mmHg, diastolic > 50 mmHg
Day
1 2 3 4 5 6 7
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Term infants
Age
Systolic (mmHg)
Diastolic (mmHg)
Mean (mmHg)
1 hour 12 hour Day 1 (Asleep) Day 1 (Awake) Day 3 (Asleep) Day 3 (Awake) Day 6 (Asleep) Day 6 (Awake) Week 2 Week 3 Week 4
E. References:
Nuntnarumit P, Yang W, Bada-Ellzey HS. Blood pressure measurements in the newborn. Clin Perinatol 1999;26:981-996 Rennie JM, Roberton NRC (Eds). Textbook of Neonatology, 3rd Ed. Churchill Livingstone, Edinburgh, 1999. Taeusch HW, Ballard RA. Avery's Diseases of the Newborn 7th Ed. W.B. Saunders Company, Philadelphia. 1998
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7. BOWEL OBSTRUCTION
A. B. C. D. E. F. G. H. I. J.
Summary Introduction Differential Diagnosis Duodenal Atresia Midgut Malrotation and Volvulus Jejunoileal Atresia Meconium Ileus Hirschsprungs Disease Investigation of Bowel Obstruction Management
A. Summary:
delay in carrying out surgery may result in the loss of large amounts of bowel not all infants with bowel obstruction require transfer by the NETS team. Infants diagnosed early and without fluid or electrolyte problems may be safely transferred with local ambulance services. However, it is advisable to discuss such infants with the receiving hospital or the NETS team
B. Introduction:
Signs of bowel obstruction can include:
vomiting with or without bile stained material, therefore never ignore bile-stained vomiting in the newborn gastric residuals before feedings failure to pass meconium in the first 24 hours of life abdominal distension (particularly with low level obstruction)
C. Differential Diagnosis:
Intestinal obstruction without bilious vomiting: duodenal atresia (if obstruction proximal to Ampulla of Vater 20% of cases) duodenal stenosis annular pancreas pyloric stenosis (usually presents at 4-6 weeks of life but may present as early as the first week) Intestinal obstruction with bilious vomiting: malrotation and volvulus duodenal atresia (if obstruction distal to Ampulla of Vater 80% of cases) jejunoileal atresia meconium ileus necrotising enterocolitis (see Necrotising Enterocolitis) Intestinal obstruction with marked abdominal distension: ileal atresia Hirschsprung disease meconium ileus meconium plug imperforate anus
D. Duodenal Atresia:
Duodenal atresia may take the form of either a membranous or interrupted-type lesion at the level of the papilla of Vater. In 80% the papilla of Vater opens into the proximal duodenum causing the vomiting to be bilious:
obstruction due to failure of recanalisation of the 2nd part of the duodenum during foetal development occurs in 1:5,000-10,000 live births commoner in males associated with Down syndrome in 25% hydramnios is seen antenatally X-ray usually shows a characteristic "double-bubble" appearance
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F. Jejunoileal Atresia:
caused by a mesenteric vascular accident during fetal life abdominal distention with bilious vomiting is observed within the first 24 hours after birth. The more proximal the lesion, the earlier the bile-stained vomiting X-ray shows air-fluid levels proximal to the lesion. Calcification due to meconium peritonitis may be present
G. Meconium Ileus:
thick tenacious meconium in the bowel (ileum, jejunum or colon) causes obstruction 50% have associated: volvulus jejunoileal atresia bowel perforation and/or meconium peritonitis meconium ileus occurs in 15% of newborns with cystic fibrosis, and at least 90% of patients with meconium ileus have cystic fibrosis presentation includes: early marked bowel distension bilious vomiting remarkable abdominal distention, tenderness and/or erythema of the abdominal skin may indicate perforation on rectal examination mucus plugs may be evacuated after withdrawal of the examination finger (fifth finger) X-ray shows: distended loops of intestine with thickened bowel walls a large amount of meconium mixed with swallowed air produces the so-called "ground-glass" sign typical of meconium ileus, a characteristic feature but often absent calcification, free air or very large air-fluid levels suggest bowel perforation
Patients with uncomplicated meconium ileus may be successfully treated with hypertonic enemas performed while adequate intravenous fluid is maintained. Immediate surgery is indicated for infants with complicated meconium ileus or where conservative treatment fails.
H. Hirschsprungs Disease:
causes 15-20% of newborn intestinal obstructions 80% of cases present in the first 6 weeks of life 4:1 male:female ratio presents with failure to pass meconium in the first 24 hours plus gradual onset of abdominal distension and vomiting. Distal short segment disease can present later in life with persistent and progressive constipation the most serious complication is enterocolitis. This occurs as a result of progressive colonic dilation with decreased ileal and colonic fluid resorption, stasis with bacterial overgrowth and mucosal ischaemia which may lead to massive acute fluid loss into the bowel with diarrhoea, shock and dehydration. Enemas should be avoided during episodes of enterocolitis because of the possibility of perforating the colon definitive diagnosis is made by a full thickness rectal biopsy showing a lack of ganglion cells in the myenteric plexus of the colon
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J. Management:
place infant in an incubator for close observation and temperature control nurse supine or on the right side with the head elevated place an orogastric tube (8 - 10FG) on low pressure suction (or aspirate with a syringe every 60 minutes and leave on free drainage). The amount and type (eg bile-stained, faeculent) of fluid aspirated should be recorded place nil by mouth commence IV fluids. Give maintenance fluids plus ml for ml replacement of NG aspirate with normal saline obtain abdominal x-rays (include supine and erect or decubitus view). Note that a relatively gasless abdomen is compatible with mid-gut volvulus consult with a paediatric surgeon or NETS to arrange transfer to an appropriate surgical centre it may be appropriate to commence antibiotics preferably after blood culture taken(discuss with the receiving unit or NETS) obtain blood for FBE, electrolytes, blood grouping and hold for X match (and blood cultures if commencing antibiotics) frequently, these infants have associated problems of acidosis and shock
8. BREASTFEEDING ISSUES
A. B. C. D. E. F. G. H. I. J. K. L. M. N. O. P.
Summary Introduction Benefits of breast milk Storage of Breast milk for Infant Use Maintenance of supply of breast milk Developmental issues Prematurity and nutritional adequacy of human milk Human Milk Fortifier Breast milk substitutes Infections Maternal medications Drugs of addiction Herbal preparations Jaundice Drugs in pregnancy and/or lactation References
A. Summary:
Breast milk is the milk of first choice in neonates, whether term or preterm there are significant clinical benefits to providing breast milk in the preterm infant expressed breast milk can be safely frozen for later use human milk fortification should be considered in babies < 1500g or < 30 weeks' gestation very few maternal medications contraindicate breastfeeding maternal Hepatitis C does not preclude breastfeeding (unless nipples are cracked)
B. Introduction:
The Innocenti Declaration (WHO/UNICEF, 1990) recognised that breastfeeding is a unique process that provides ideal nutrition for infants and contributes to their healthy growth and development. The Paediatrics and Child Health Division of the Royal Australasian College of Physicians encourages and supports the promotion of breastfeeding (see website).
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The many benefits of mother's own milk are well known. Breastmilk is a unique 'living" fluid. It contains: anti-infective factors hormones enzymes specialised growth factors anti-inflammatory mediators specific nutrients Colostrum is a high density; low-volume feed high in immunoglobulins, which evolves into mature milk between 3 and 14 days postpartum. Breastmilk feeding for both preterm and unwell term infants assists recovery and has major health benefits. As the unwell or preterm infant may not be able to breastfeed mothers are encouraged to provide fresh expressed milk daily; if this is not possible breast milk can be stored in a refrigerator or freezer.
Breast milk
Freshly expressed into closed container
Freezer
2 weeks in freezer compartment inside a refrigerator 3 months in freezer section of refrigerator with separate door 6 - 12 months in deep freeze (- 180C) Do not refreeze
Thawed outside For completion of refrigerator in warm water feeding Infant has begun feeding Only for completion of feeding then discard
Hold for 4 hours or until Do not refreeze next feeding Discard Do not refreeze Discard
There is controversy regarding the use of sterile vs clean washed containers for storage of expressed breast milk (EBM) intended for preterm infants. There is limited published evidence to support the use of clean containers One Level 3 nursery in Melbourne does provide sterile containers which families re-use after washing in the dishwasher and air-drying.
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the enteromammary pathway by which a mother produces antibodies in response to antigens in the infant's environment If supply lags, technique and frequency of expression needs review before resorting to galactogues. Commencement of an oral contraceptive agent may also contribute to reduction in supply. In addition
Galactogues:
These are substances, which stimulate the supply of breast milk. Both pharmacological and herbal preparations are available. - Published evidence supportive of herbal preparations is limited. Fenugreek is the most widely recognised however there is no data regarding transmission in breast milk or safety for preterm infants. - Metoclopramide (Maxalon) will stimulate breast milk supply in the lactating mother. The safety of this medication has been established for preterm infants. Mothers should be advised about the possibility of dystonic reactions. In some women use exacerbates symptoms of depression. Controversy exists over the dosage regime and duration. A suggested regime is 10mg tds for 5 days and then tapering over the next 5 days. Some women benefit from repeated courses but little data exists on the safety of such practice. Metoclopramide often results in dramatic increase in supply, which may not be sustained once medication is withdrawn. - Domperidone also acts a galactogue and is safe for preterm infants. There appears to be a slower onset of action but the increase in supply is better maintained than with Metoclopramide. Unfortunately Domperidone is not approved as a galactogue on the Australian Pharmaceutical Benefits Scheme and the quantity required can prove costly. The dose required is 10 - 20 mg qid. Domperidone is better tolerated by mothers as a long term stimulant of breast milk supply.
F. Developmental issues:
Initiating breast-feeding in preterm infants does not require a demonstrated ability to breastfeed. Kangaroo care is a good introduction to mother's breasts for the preterm or unwell infant. Studies have shown that preterm infants show greater cardio-respiratory stability when breast feeding than bottle feeding. Infants exhibit sucking movements as early as 11 weeks gestation. By 32 weeks there is coordination of sucking and swallowing, but this is not sustained until closer to term. Controversy exists over the issue of nipple confusion. Ultrasonography has shown that the sucking action used at the breast is different from that used for an artificial teat. A randomised controlled trial comparing artificial teats and cup feeding in preterm infants did not demonstrate any difference in time to achieve breastfeeding. (personal communication). If it is to be mentioned then the technique of cup feeding needs brief explanation Similarly controversy exists between the advantages of indwelling naso-gastric feeding tubes and intermittent orogastric tube feeds. Sandra Lang in her book "Breastfeeding Special Care Babies" addresses these issues in depth.
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supplementation is introduced. However, there are a number of potential complications with fortification. These include:
an increase in regurgitation an increase in feed intolerance glycosuria in extremely of preterm infants hypercalcemia in extremely preterm infants
As the fortifier is usually cow's milk based there is a theoretical advantage in using a product in which the protein has been hydrolysed. Infants fed fortified human milk receive less volume, but greater intakes of protein and minerals and experience greater weight gain and incremental linear growth than infants fed unfortified milk. The growth of infants fed fortified breastmilk is still less than infants fed on preterm formula. However the quality of the milk and its many advantages far outweigh any growth disadvantage. In general, fortifier can be discontinued once the infant reaches a corrected age of term and prior to discharge from hospital.
J. Infections:
Maternal HIV is the only infection in which breastfeeding is contra-indicated in the developed world Hepatitis C has been reported to have a 5% risk of transmission. Most probably this occurs at times of active disease (PCR positive women). It is generally advised that HCV positive mothers do not breastfeed when nipples are cracked. Hepatitis B is not transmitted through breast milk CMV is transmitted through breast milk. The burden of disease acquired from breast milk is not well established. It is presumed that preterm infants are more vulnerable and likely to exhibit more severe clinical illness, such as pneumonitis, than term infants. However women who are CMV positive are not discouraged from breastfeeding as the other benefits are thought to outweigh the risk. Herpes Simplex is not transmitted through breast milk. Should there be an open sore on the breast the mother would be advised to avoid feeding from that breast. With Varicella and Herpes Zoster (shingles) maternal antibodies transmitted through breast milk will be protective. However in the case of Varicella if the mother develops chicken pox within 5 days of birth the infant is at risk and should be protected with VZV immunoglobulin. Breast-feeding can then continue, provided there are no lesions on or near the nipple. (link) bacterial infections almost never transmit disease through breast milk
K. Maternal medications:
absolutely contraindicated: chemotherapeutic agents
radioactive drugs
citalopram cyclosporin
Most psychoactive medications are now generally considered safe, although dosage needs to be considered. Infants should always be carefully monitored for effects of sedation when their mother's are using psychoactive drugs. Be aware that drug companies are very cautious in their recommendation of safety for the breastfed infant. Preferably consult specific reference texts or drug advisory services experienced in lactation.
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L. Drugs of addiction:
Methadone passes in small quantities into breast milk and generally the benefits of breast milk overcome the disadvantages. In situations of very high maternal dosage (e90mg daily) the infant is at risk of sedation. Buprenorphine is a long acting narcotic agonist and antagonist used to replace methadone in opiate addicts. Little information is available regarding the pharmacology of this drug in lactation. Sedative effects are of concern. Marijuana passes into breast milk and the relative dose is concentrated. Infants are at risk of sedation, feeding difficulties and poor weight gain.
M. Herbal preparations:
Scientific data is limited. Given the variability in standards of preparation of herbal supplements it is recommended that breastfeeding mothers avoid such products.
Q. References:
J Akre. Infant Feeding: the physiological basis. WHO 1994. Bulletin (Suppl.) 67:25 J Akre. Infant Feeding Guidelines for Health Workers. 1996. NHMRC Sosa R, Barness L. Bacterial growth in Refrigerated Human Milk. Am J Dis Child. 1987: 141; 111-112 Whitelaw A. Kangaroo baby care: just a nice experience or an important advance for preterm infants? Pediatrics 1990; 85: 604-605 Ehrenkranz RA et al. Metoclopramide effect on faltering milk production by mothers of premature infants. Pediatrics. 1986;78:614-20. Da Silva et al. Effect of Domperidone on milk production in mothers of premature newborns: a randomised, double-blind, placebo controlled trial. CMAJ. 2001; 164: 17-21. Blaymore Bier JA et al. Breastfeeding infants who were extremely low birth weight. Pediatrics 1997;100:E3 Lang S. Breastfeeding Special Care Babies. 1997. Bailliere Tindall. London. Schanler RJ, Hurst NM, Lau C. The use of human milk and breastfeeding in premature infants. Clin Perinatol. 1999;26:379 - 398 El-Mohandes AE et al. Use of human milk in the intensive care nursery decreases the incidence of nosocomial sepsis. J Perinatol. 1997;17:130 - 134 Lucas A, Cole TJ. Breast milk and necrotising enterocolitis. Lancet. 1990; 336: 1519-1523 Lucas A, Morely R, Cole TJ et al. Breastmilk and subsequent intelligence quotient in children born preterm. Lancet. 1992; 339:261-264 K Simmer. Choice of formula and human milk supplement for preterm infants in Australia. J Paediatr Child Health. 2000;36:593-595. Ruff. Infection and breastmilk. Semin Perinatol. Howard CR, Lawrence RA. Drugs and breastfeeding. Clin Perinatol. 1999;26: 447 - 478 Maisels MJ, Gifford K. Normal serum bilirubin levels in the newborn and the effect of breastfeeding. Pediatrics. 1986;78:837-843 De Carvalho M, et al. Frequency of breastfeeding and serum bilirubin concentration. Am Dis Child 1982;136:747-748 Clinical Aspects of Human Milk and Lactation. Clin Perinatol June 1999;26:2
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9. BRONCHOPULMONARY DYSPLASIA
A. B. C. D. E. F. G. H. I. J. K.
Summary Introduction Risk Factors Clinical Features Management of BPD in Level 3 Units Respiratory Criteria for Transfer to a Level 2 Hospital Management in the Level 2 SCN Oxygen Home oxygen Follow up References
A. Summary:
the most severely affected babies are the most premature, particularly 23-26 week gestation babies diuretics and corticosteroids are effective in achieving short-term improvement in the status of ventilator dependant babies. Safety issues of steroid use are unresolved. There is no place for long term therapy with diuretics in level 2 SCN's there is no consensus on how to wean oxygen in babies with BPD the transition from a tertiary hospital nursery to a level 2 SCN is a difficult time for parents as they adjust to different staff and practices
B. Introduction:
In line with the recommendations from a recent workshop in North America the term bronchopulmonary dysplasia (BPD) will be used in this chapter rather than Chronic Lung Disease. The definition is complicated, however in this chapter BPD refers to a premature baby who has been in oxygen for > 28 days. BPD is the single most important factor determining length of stay in babies born at less than 29 weeks. The most severely affected babies are the most premature, particularly 23 - 26 week gestation babies.
C. Risk Factors:
prematurity peripartum inflammation/infection associated with preterm labour and/or clinical or subclinical chorioamnionitis postnatal lung Injury due to volutrauma, oxygen or infection
D. Clinical Features:
Tertiary unit babies with BPD (actual or evolving) fall into three broad groups:
babies dependant on endotracheal mechanical ventilation (MV) babies dependant of Nasal CPAP babies who are oxygen dependant, usually by nasal prongs
The most common clinical scenario is the 23 - 26 week gestation baby who, over a period of 4 - 10 weeks, progresses from MV, NCPAP through to requiring supplementary oxygen. These babies are usually transferred to a level 2 Special Care Nursery (SCN) for ongoing care. Although some of these babies spend many weeks on NCPAP it is common to see rapid improvement in their respiratory stability once weaned from NCPAP. Respiratory stability off NCPAP is the single most important criterion that determines suitability for transfer to a SCN. It is important to understand the tertiary unit's experience with babies at these gestations as it has a significant impact on decision making with respect to transfer to level 2 SCNs. Issues include:
all tertiary units experience late deaths of extremely premature babies due to chronic lung disease when prolonged MVis needed there are often many weeks before one can reassure the family with confidence that the baby is likely to survive. Once a baby is showing consistent growth associated with an oxygen requirement less than 40% the recovery process is likely to be successful some babies cope for many months on NCPAP in high (more than 40%) oxygen concentrations before dying. Fortunately this group is rare
These factors make the care of babies with BPD extremely demanding for babies and their families, as well as for nursing, medical and ancillary staff.
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there is an intense focus on minimizing ventilator associated lung injury from the moment a baby is placed on a ventilator. Synchronised modes of MV with close monitoring of tidal volumes are key features of current practice. In addition there is a more liberal approach to carbon dioxide control, allowing CO2 to rise into the 50's and 60's providing the pH remains better than 7.25 oxygen damages delicate lung tissue as well as the immature retina. Pulse oximetry targets are typically setx etween 88 to 93% in the first weeks after birth endotracheal ventilation is being increasingly replaced by NCPAP, even for the tiniest babies while it is clear that the aggressive early use of NCPAP can avoid the need for endotracheal intubation and MV in babies who in the past would have been electively intubated, it is not yet clear what the effects of this practice are on survival and short and long term morbidity. Randomised trials are in progress to determine best practice in this area babies who require endotracheal ventilation are aggressively weaned and extubated to NCPAP often within 1-2 days of birth
Corticosteroids:
dexamethasone is effective in achieving short-term improvement in the status of ventilator dependant babies there is now low level evidence showing that dexamethasone in the first week of life is associated with an increased risk of cerebral palsy in survivors safety of corticosteroids used later in the course of evolving BPD between 14 - 28 days is unresolved. RCT's are in progress frequency of the use of steroids for BPD in NICUs has dramatically declined in the past 2 years it is recommended that corticosteroids be used only within the context of a RCT. Otherwise a "low" dose regimen (eg 0.15 - 0.25mg/kg/day) weaned and ceased over a 7 - 10 day period is recommended typical clinical scenarios where steroids would be considered are a baby > 2 weeks of age who is unable to be weaned from endotracheal MV there is no place for the use of steroids in the treatment of BPD outside a tertiary neonatal unit early use of inhaled steroids is ineffective in preventing BPD
Diuretics:
insufficient studies of suitable size reporting on important outcomes exist to strongly support the use of diuretics for the treatment of BPD diuretics are an effective short term therapy for ventilated babies There is no evidence for efficacy in non ventilated babies therefore diuretic therapy should be weaned and ceased once babies are stable off mechanical ventilation typical combinations include hydrochlorothiazide and spironolactone chronic frusemide administration is generally avoided, as it has been associated with the development of nephrocalcinosis and hyperchloremic metabolic alkalosis NaCl and KCl supplementation are commonly required there is no place for long term therapy with diuretics in level 2 SCN's
3. Nutrition:
provision of adequate calories in a nutritionally appropriate form is critical caloric requirements of babies with moderate to severe lung disease can be as high as 130 - 150 calories/kg/day babies are modestly fluid restricted (150 - 160mL/kg/day) and fed fortified breast milk or low birthweight formula growth is closely monitored and caloric intake titrated against growth Vitamin A supplementation has a statistically significant benefit in reducing oxygen requirements at 36 weeks corrected gestational age however most consider this effect to be clinically insignificant babies sufficiently stable to transfer to a level 2 unit should not require a caloric density of > 24 cal/30mls
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4. Oxygen Therapy:
oxygen is the one constant in the treatment of BPD but it has been poorly studied once weaned from NCPAP, oxygen is delivered by nasal prongs using low flow (<0.5L/min) there is no consensus on how to wean oxygen in babies with BPD weaning is dictated to some extent by the equipment available. Some tertiary centres have only recently moved from having flow meters with a lower limit of 0.25L/min/O2. Other units use flow meters that allow weaning down to as low as 0.005mL/min/O2. Most believe that weaning should be slow, but what this means varies from institution to institution babies with BPD have a degree of pulmonary hypertension, and are therefore likely to benefit from a more generous oxygen administration regimen rather than from a restrictive policy. Whilst there is some logic to this approach, there is indirect evidence from randomized controlled trials conducted for other reasons suggesting that a more liberal oxygen policy in these babies can actually increase the pulmonary morbidity. RCT's are in progress to address this question
Nutrition:
Aim to achieve an intake of 180mls/kg/day Breast milk fortifier or a low birthweight formula can be continued until at least term without adverse effects if caloric supplementation is required For babies on tube feeds oral feeds should be cautiously introduced eg. initially one oral feed/day then 2 etc as the baby copes with the previous increment
H. Oxygen:
1. General considerations:
Aim to move to intermittent oximetry rather than continuous once it is clear the baby is not having apnoea or bradycardia Saturation goal should be 93-98% Brief periods where the saturation drops to the low 90's are not of concern During feeding and certain parts of sleep are the times where a baby's oxygen demand is higher. Therefore if one is looking to see if a baby will manage in less oxygen saturations should be monitored over several feed periods or for several hours during sleep
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For babies in headbox/tent oxygen do not wean by more than FiO2 0.01 in a 24 hour period even if the saturations are 100% For babies on low flow subnasal oxygen do not wean by more than 10mls/24 hours If the lowest accurate flow deliverable is 0.25L/min then the only option is to periodically (eg twice/week) turn off the oxygen and carefully monitor the saturations Blood gas monitoring is not required in stable babies The indications for blood transfusion in a growing stable baby with a mild oxygen requirement are unclear and currently the subject of study, but most practice a conservative policy i.e accept Hb concentrations down to 8g/dl particularly if there is a good reticulocyte response
2. Ceasing oxygen:
A prolonged period (8-12 hours) of saturation monitoring should be undertaken that captures extended periods of sleep Babies with BPD should not be discharged until at least 72 hours after ceasing oxygen
I. Home oxygen:
This will be the subject of a separate chapter, however some general points can be made.
Appropriate social/home environment including reasonable accessibility to medical care Baby is on 4 hourly or demand oral feeding regimen Baby is normothermic in an open cot Satisfactory growth All babies discharged from tertiary units on home oxygen have specific Paediatric Thoracic specialist follow up. It is strongly recommended that Paediatricians manage babies on home oxygen in collaboration with a Paediatric Thoracic physician
Baby must pass an "air test". The oxygen is turned off, the nasal prongs removed and the baby monitored over 30 minutes If saturations are maintained >86% for 30 minutes the test should be repeated in 48 hours. If a second test is satisfactory the baby is eligible for discharge on home oxygen on respiratory grounds. In other words the baby has demonstrated a reasonable level of respiratory reserve
J. Follow up:
These babies require term follow up throughout childhood There is an increased pulmonary morbidity in the first 2 years of life. Parents should be counselled about this morbidity and ways to minimise it. Influenza vaccine is not officially recommended for these babies RSV prophylaxis is not routinely recommended
K. References:
Jobe A, Bancalari E. Bronchopulmonary Dysplasia, NICHD/NHLBI/ORD Workshop Summary. Am J Respir Crit Care Med. 163 1723-1729, 2001 Barrington KJ and Finer NN. Treatment of Bronchopulmonary Dysplasia - A Review. Clinics in Perinatology 25 1 March 1998 177-202 Brion L et al. Diuretics acting on the distal renal tubule for preterm infants with (or developing) chronic lung disease. Cochrane Neonatal group, Cochrane database of systematic reviews, Issue 3, 2001. Shah V, Ohlsson A, Hallidah HL, Dunn MS. Early administration of inhaled corticosteroids for prevention of chronic lung disease in ventilated VLBW preterm neonates. Cochrane Neonatal group, Cochrane database of systematic reviews, Issue 3, 2001. Halliday HL and Ehrenkranz RA. Early postnatal corticosteroids for the prevention of chronic lung disease in preterm babies. Cochrane Neonatal group, Cochrane database of systematic reviews, Issue 3, 2001. Chronic Lung Disease. Department of Neonatal Medicine Protocol Book, Royal Prince Alfred Hospital, Sydney, NSW. www.cs.nsw.gov.au/rpa/neonatal Oxygen Therapy. Department of Neonatal Medicine Protocol Book, Royal Prince Alfred Hospital, Sydney, NSW. www.cs.nsw.gov.au/rpa/neonatal
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A. Introduction:
The Varicella-zoster virus is a herpes virus causing chickenpox as the primary infection or herpes zoster after reactivation from its latent form in dorsal root ganglia.
Risk is higher when maternal chickenpox occurs before 20 weeks and is estimated at approximately 2%. CVS is associated with:
cicatricial skin lesions limb hypoplasia or paresis microcephaly (secondary to cortical atrophy) ophthalmic lesions (chorioretinitis, microphthalmia, atrophy and cataracts)
1. Management:
give ZIG (6mL IMI) within 72 hours of significant exposure to the pregnant woman if sero-negative or if there is a negative history and sero-testing is unavailable continue monitoring, including with ultrasound, since ZIG reduces the clinical attack rate in the pregnant woman but may not eliminate fetal risk negative amniotic fluid PCR correlates well with a good outcome but positive PCR correlates poorly with congenital varicella syndrome development
termination of pregnancy would not usually be offered but may require discussion
1. Management:
give the infant ZIG (2mL IMI) as soon as possible after delivery or onset of maternal illness. ZIG must be given within 72 hours while in hospital, a mother and/or infant with lesions should be isolated from other patients. A mother with lesions does not need to be isolated from her own infant continue to encourage breastfeeding unless lesions are on or near the nipple admit infant into hospital isolation room if rash develops give IV aciclovir (20mg/kg every 8hours) to infants who develop chickenpox and:
did not receive ZIG prophylaxis within 24 hours are immunocompromised are premature (less than 28weeks gestation at birth)
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The high risk period for severe infection varies between authorities. These conservative recommendations follow the published Australian guidelines
1. Management:
give ZIG (2mL IMI) immediately if: mother is seronegative her sero-status cannot be determined and history is negative infant born at less than 28weeks gestation or <1000gm birthweight</li/> encourage normal care. Do not exclude infant from family contact admit for aciclovir treatment if infant becomes unwell
E. References:
The Management of varicella-zoster virus exposure and infection in pregnancy and the newborn period A. Heuchan, D. Isaacs on behalf of the Australasian Subgroup in Pediatric Infectious Diseases of the Australasian Society for Infectious Diseases MJA 174 2001 288-291
A. Summary:
early feeding intervention and education from a Speech Pathologist and a Lactation Consultant fosters establishment of an effective feeding regimen a dysfunctional feeding pattern may indicate other congenital anomalies when breast feeding a baby with a soft palate cleft, unilateral or bilateral cleft, supplementary breast milk feeding is usually required assisted feeding techniques are used with bottle feeding oromotor development, adequate nutritional intake, positive caregiver/infant interaction and consistent carers are necessary for effective feeding
B. Introduction:
Cleft lip and palate occurs in approximately 1 per 1000 births. This may occur sporadically or in the setting of a family history. Babies born with a cleft may present with a range of feeding difficulties according to the type and severity of the cleft, however, a direct relationship between cleft type and feeding problem does not seem to exist. Sucking efficiency varies but is reduced on both the bottle and breast. The baby is at risk of failing to thrive as oral intake efficiency is reduced and the baby fatigues during lengthy feeds.
C. Diagnosis:
Obvious in more severe cases. Careful examination with a bright light and a tongue depressor is required in the case of a posterior soft palate cleft or submucous cleft of the palate. Difficulties in feeding may lead to a later diagnosis.
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D. Investigation:
A small percentage of affected children have abnormalities elsewhere in the face or in other systems.
E. Management:
1. Early:
genetic counselling and information about the Cleft Pals Association who can arrange a parent visit if appropriate feeding assessment by a Speech Pathologist within 24 hours of birth to discuss feeding options with the parents referral to a Lactation Consultant to discuss breast feeding and/or expressing techniques all mothers encouraged to put their baby to the breast for skin to skin contact, bonding and to maximise the opportunity to stimulate milk flow parental education about infant feeding cues:
Breast feeding: A cleft palate can interfere with breast feeding as it precludes generation of suction during feeding. Milk extraction from the breast is inefficient and the required strong attachment to the breast is absent. The potential for a baby with a cleft palate to receive adequate nutrition from being exclusively breast fed in the traditional manner is limited. Mothers wishing to breast feed should consult a lactation consultant for support as breast feeding an infant with an unrepaired cleft is demanding. When breast feeding a baby with a soft palate cleft, unilateral or bilateral cleft compressing the breast to express the milk into the babies mouth will facilitate milk flow and the baby's suck swallow reflex. As the necessary negative intra oral pressure for sucking will rarely be produced for adequate volume intake, a "top up" with expressed breast milk via a supply line or bottle will be required. However, breast feeding a baby with an incomplete cleft lip can be achieved by pressing part of the breast into the cleft to obtain lip seal.
Bottle feeding: Assisted breast milk feeding using squeezable bottles requires less support and intervention than using a rigid bottle, after initial instruction. A Haberman Feeder or Pigeon teat (cross cut) with a Cleft Pals or Soft Plas squeeze bottle are recommended. Before feeding, practice squeezing the bottle and compressing the teat to be familiar with rate of flow and pressure required. When feeding a baby with a cleft lip and palate, hold the baby in a semi upright position to minimise the nasal regurgitation of milk. Squeeze the bottle rhythmically only when the baby sucks (every two, three of five sucks). Burp the baby regularly as the cleft allows extra air to be ingested. The feed should be completed within 30-40 minutes.
Audiology:
regular testing during childhood until risk of effusion/ otitis media subsides, removing need for ventilation tubes
lip repair early or up to 3 months palate repair at approximately 6 months bone graft to the cleft alveolus (gum) 9-11 years ventilation tubes to ears at time of palate repair and subsequently adjustments of lip and nose shape as required until fully grown pharyngoplasty to improve speech age 5-6 years in 10-15% of patients with cleft palate
lifetime dental care orthodontics before and after alveolar (gum) bone graft jaw surgery to correct malocclusions in some teenagers
Speech therapy:
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G. References:
Converse, Plastic & Reconstructive Surgery Vol.4, p. 1930 Saunders Bannister P. Early Feeding Management. In:Watson ACH, Sell DA, Grunwell P (eds) .Management of Cleft Lip and Palate. London: Whurr; 2001 pp. 137-147. Clarren, SK. Anderson, B. Wolf, LS. Feeding infants with cleft lip, cleft palate, or cleft lip and palate. Cleft Palate Journal 1987 Jul; 24(3): 244-249. Glass RP, Wolf LS, Feeding Management of Infants with Cleft Lip and Palate and Micrognathia. Inf Young Children. 1999; 12(1): 70-81. Shaw WC, Bannister RP, Roberts CT, Assisted feeding is more reliable for infants with clefts- a randomised controlled trial. Cleft Palate Craniofacial J. 1999; 36(3):262-8.
A. Summary:
most cases of brachial plexus palsy resolve spontaneously. Infants without signs of recovery at 1 month of age should be referred to a plastic surgeon metatarsus adductus with a rigid foot, 'fixed' talipes equinovarus, polydactyly and syndactyly should be referred for orthopaedic opinion
B. Introduction:
Anomalies of the musculoskeletal system may be evident as: - the absence of a part - extra parts - malformed - malfunctioning tissue Anomalies may be congenital or acquired (eg birth trauma) and usually affect the infant's movement, muscle tone, or posture.
C. Upper Extremities:
Brachial palsy: is seen most often in large babies who are vulnerable to stretching injuries to the components of the brachial plexus. Erb's palsy and total plexus palsy are the two most common types of injury. Erb's palsy: involving C5, C6 and sometimes C7 causes the affected arm to be adducted and internally rotated, with extension at the elbow, pronation of the forearm, and flexion of the wrist ('waiter's tip' position). Paralysis of the upper arm is more common than paralysis of the lower arm or of the entire arm. The grasp reflex remains intact, but the Moro reflex is absent on the affected side. Complete brachial plexus palsy: presents with a limp, dangling appendage, without any trace of movement. Klumpke's palsy: involving C7-8 and T1, is purely a lower brachial plexus palsy that presents as a clawed hand with function at the shoulder and elbow. There is also an associated dilation of the pupil on the side of the injury (the nerves which dilate the pupil leave the spinal cord on the C8 and T1 nerves and then travel with the artery to the brain and eye).
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Management of Brachial Plexus Palsy: - X-ray of the clavicle and upper arm on the affected side, and a chest X-ray to exclude ipsilateral diaphragmatic paralysis - where the nerve roots are not disrupted, most resolve spontaneously with physiotherapy as the only modality of treatment - babies without evidence of recovery by 1 month should be referred to the Erb's palsy and Brachial Plexus Injury Clinic at The Royal Children's Hospital (9345 5391). With the advent of new reconstructive techniques, useful function can be obtained by children who have failed conservative therapy, provided that timely treatment is given
D. Lower Extremities:
1. Developmental dysplasia of the Hip (DDH): Metatarsus Adductus: The most common congenital foot anomaly, metatarsus adductus is caused by intrauterine positioning. It may be a positional (flexible) deformity with no bony abnormality involved or a structural deformity. In a structural deformity, the forefoot usually cannot be abducted beyond the midline (neutral position) and the heel (hindfoot) is in a valgus position. In a positional deformity, the forefoot is very mobile and can be easily abducted. A positional deformity will correct without treatment. In a rigid foot, an orthopaedic consultation is necessary for early treatment. Clubfoot ( Talipes Equinovarus): Clubfoot is one of the most common congenital anomalies with an incidence of approximately 1 per 1000 live births. In Caucasians, males are affected twice as often as females. The involvement is bilateral in 50% of cases. A thorough examination should be made for other anomalies, especially the effects of fetal hypokinesia There are variations in the severity of clubfoot. Some are relatively flexible and correctable with serial exercises and casting. Treatment of fixed deformities can often be started in the nursery; an orthopaedic consultation should be initiated as soon as possible after birth. Syndactyly: This is frequently a familial tendency. The severity varies from minimal 'bridging' between adjacent fingers/toes to complete webbing of the hand/foot. Syndactyly of the toes does not interfere with function but may be unacceptable cosmetically. Treatment for syndactyly of the fingers depends on the severity and the presence of bony abnormalities. An early orthopaedic consultation is needed. Polydactyly: Extra digits are common abnormalities affecting both the hands and the feet, with a familial tendency. The most common type is a floppy digit or skin tag on the lateral side of the hand. It may involve the duplication of a normal looking digit. All digital remnants should be surgically removed.
E. References:
Tappero EP. 1996. Musculoskeletal system assessment. In Physical Assessment of the newborn, 2nd ed., Tappero EP, and Honeyfield ME, eds. Petaluma, California: NICU Ink, 117-136. Rennie JM, Roberton NRC, eds. 1999. Textbook of Neonatology , 3rd ed., Edinburgh: Churchill, 275-280. Shenaq et al. 1998. Brachial plexus birth injuries and current management. Clinics in Plastic Surgery;25(4):527-535 Committee on Quality Improvement, Subcommittee on Developmental Dysplasia of the Hip. 2000. Clinical practice guideline: early detection of developmental dysplasia of the hip. Pediatrics;105(4 Pt1):896-905.
Further Reading:
Ponseti IV, 1996. Congenital clubfoot. Fundamentals of treatment. Oxford University Press, Oxford, New York. Levene M, Tudehope D. 1993. Essentials of neonatal medicine. 2nd ed., Oxford: Blackwell, 315-324.
Web Links:
http://www.ubpn.org The home page of the United Brachial Plexus Network. Very interesting
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A. Summary: - a paediatric endocrinologist should be involved in all cases - families will need referral to endocrinologist/geneticist for appropriate counselling - affected children usually remain metabolically stable for the first two weeks of life - adrenal crises (usually heralded by vomiting and failure to thrive) are often sudden and life threatening - salt replacement, in addition to both glucocorticoid and mineralocorticoid replacement is usually required
in the first months of life
B. Introduction:
Congenital adrenal hyperplasia (CAH) is an autosomal recessive condition with an incidence of approximately 1 in 12,500 births. Approximately 95% of cases are due to a deficiency of the enzyme 21-hydroxylase which catalyzes the conversion of progesterone and 17-hydroxyprogesterone (17OHP) to deoxycorticosterone and 11-deoxycortisol respectively. This section will focus upon this most common form of CAH.
C. Diagnosis:
In the neonatal period CAH may present in one of four ways - a virilised female neonate The clue that neonates with ambiguous genitalia may have CAH is the presence of pigmentation of the areolae and genital skin. There may also be: other affected siblings a history of parental consanguinity significant metabolic derangement: hyperkalaemia hyponatraemia metabolic acidosis hypoglycaemia (rarely) Related symptoms (vomiting, failure to thrive, haemodynamic collapse) do not usually occur until the second to fourth week of life. Hence it is unusual for affected females to present in critical condition, having most commonly been diagnosed in the first few days of life. Milder degrees of CAH may present in more subtle ways (isolated clitoromegaly, virilisation in early childhood etc) and are usually not associated with metabolic compromise in the neonatal period. Until the diagnosis of CAH is established such neonates should be managed as for the neonate with ambiguous genitalia. - a male neonate with metabolic and haemodynamic collapse, aged 2-4 weeks Presentation can vary from mild (failure to thrive) to catastrophic (sudden infant death). Frequently, presenting males are assumed to have sepsis. The major clinical clues to CAH are the presence of pigmented genitalia and nature of the electrolyte disturbance. - an antenatally diagnosed case of CAH (due to a previously affected sibling) The mother will usually have received pre-natal dexamethasone therapy if the fetus is 46XX. If the antenatal treatment has been successful, affected female neonates should not be virilised, however this is not always the case. - detection in unambiguous and metabolically stable males from newborn screening programs
D. Investigation:
the diagnosis is usually made after an elevated serum 17OHP levels is found. This metabolite is normally elevated in the fetus during the last trimester and in the immediate postpartum period. Therefore results can be difficult to interpret in premature infants and term infants less than three days of age. Ideally assessment of 17OHP levels should be deferred until after day 3 of age where it is available, a confirmatory urinary steroid profile is extremely useful elevation in adrenal androgens and ACTH levels may also be helpful, but are usually not measured if the electrolyte pattern and 17OHP levels are concordant
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E. Management:
if the patient is haemodynamically compromised, resuscitation with intravenous normal saline and hydrocortisone is required hypoglycaemia may need correction with intravenous dextrose but care should be taken with the water load exacerbating hyponatraemia once stabilized, oral hydrocortisone (10-15 mg/m2/day, given tds) and fludrocortisone (0.15 mg/ m2/day, given bd) therapy should be instituted. Additional salt (0.5-1.0 gm/10 kg/day) replacement is usually required although there is some variance in opinion regarding this
Tablets should be ground up between two teaspoons and mixed with a few drops of milk. This solution should be transferred to a small, plastic feeding spoon and deposited on the back of the tongue immediately prior to feeds. Hydrocortisone solutions/suspensions should be avoided, as they are notoriously unstable and inconstant in dose delivery.
F. Reference:
Merke DP, Bornstein SR, Avila NA, Chrousos GP. Future Directions in the Study and Management of Congenital Adrenal Hyperplasia due to 21-Hydroxylase Deficiency. Ann Intern Med 2002 Feb 19; 136(4):320-34
A. Summary:
if unexpected delivery occurs in a Level II nursery, resuscitation must be undertaken and supervised by the most experienced clinician available ventilation using a bag and mask should be minimised the baby with a best preductal pO2 <70 mmHg, or a persistent respiratory or metabolic acidosis despite 36 hours of expert effort to optimise ventilatory and circulatory support has a poor prognosis</p/>
B. Introduction:
Congenital diaphragmatic hernia is a serious congenital abnormality associated with: - pulmonary hypoplasia, worse on the ipsilateral side - structural and functional lung immaturity - a reduction in pulmonary arteriolar cross sectional area - muscular hyperplasia of remaining pulmonary arterioles - an association with other major anomalies, chromosomal and non-chromosomal, in up to 20% of cases 15 - 25 cases occur each year in Victoria corresponding to an incidence of approximately 1:3000. In the early 1990's few were prenatally detected. With the virtual universal occurrence of mid trimester ultrasound examination for fetal abnormality more than 85% of cases are now prenatally detected. The survival rate for all cases diagnosed is approximately 50-60%. If a coexistent significant abnormality is present survival is <10%. This illustrates the importance of thorough evaluation, from the time of suspected fetal diagnosis, by a multidisciplinary team familiar with all aspects of the diagnosis and management of CDH.</p/> Reported survival for live born infants ranges from 50-90%. The international CDH Study Group database with over 1600 patients reports an overall survival of 66%. The experience for babies managed at RCH, Melbourne shows the survival for babies with isolated lesions to be 75% based on more than 200 infants. The difference in survival for all cases versus liveborns reflects the high rate of termination of pregnancy (TOP) where other anomalies are detected, a lower rate of TOP where an isolated lesion is present, as well as a relatively low incidence of stillbirth.
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1. Objectives:
establish an accurate description of the abnormalities present specify ultrasound features relevant to CDH using a standard form: degree of mediastinal shift liver/stomach position lung:head ratio liquor volume fetal echocardiogram performed by a Paediatric cardiologist, if associated congenital heart disease suspected establish a fetal karyotype, if informed parental consent given provide informed and supportive counselling for the family including a written summary of any discussion with the parents. This summary should include a description of the abnormalities and most likely diagnoses, management options available, and possible outcomes
2. Ongoing Management:
referral to a Paediatric Thoracic Surgeon if not previously arranged regular general obstetric surveillance repeat Ultrasounds at 24, 30 and 34 weeks to assess fetal growth and features of the CDH as defined above
3. Intervention:
prenatal steroid therapy - recommended according to usual indications. Clinical trials are under consideration for use at mature gestations fetal operative intervention (tracheal ligation) is an unproven therapy. It is not available in Australia and is not recommended outside of clinical trials (currently in progress overseas)
4. Delivery Strategy:
- to achieve a normal vaginal delivery following spontaneous onset of labour at term Women who live more than 1 hour from the perinatal centre are encouraged to move to Melbourne at 35-36 weeks gestation. For pragmatic reasons induction of labour at 38-39 weeks is offered to these women.
Caesarean section without labour is not recommended unless there is a clear medical indication.
1. Resuscitation:
Resuscitation will be individualised according to the condition of the baby and the response to initial steps in resuscitation. Ventilation using a bag and mask should be minimised. If endotracheal intubation is required make sure the tube is not inserted too far. Positive pressure ventilation should be accompanied by inspiratory pressure or volume monitoring, when available. IPPV, if required, should be provided by a mechanical ventilator at the earliest opportunity. A large bore nasogastric tube (Ryles tube 10F) should be passed once the baby has been stabilised in the delivery room. Physical contact with the parents should be facilitated before transfer. Preductal saturation monitoring should be undertaken during any transfers. Saturations in the 70's and 80's are satisfactory provided sufficient ventilatory support is provided to ensure adequate tidal volume. This is the most difficult situation to get right and in the absence of portable volume monitors a highly experienced clinician must be supervising this period.
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achieve acceptable gas exchange: saturations > 75% pCO2 at a level that allows the pH to be > 7.20 while minimising the chances of inducing lung injury or air leak. monitor preductal saturations apply a transcutaneous pCO2 monitor consult with NETS and level 3 centre
Units reporting very high survival concentrate on minimising lung injury especially in the initial hours after birth and rarely exceed PIP of 25. If available use a synchronised mode of ventilation
3. Ongoing Stabilisation:
establish venous access - if the baby's condition is poor and the circulation compromised rapidly place a UV catheter measure blood pressure and assess the circulation to determine the need for volume support obtain a chest X Ray if the baby's condition remains poor obtain a blood gas by the most expedient route (arterial stab) and adjust support accordingly arterial access if urgent - cannulate the UA if non urgent then either the procedure can be deferred until the baby has been transferred, or a right radial (i.e. preductal) line sited do not waste time (and arteries) if the first attempt is unsuccessful sedate and muscle relax the baby who remains in poor condition despite attempts at optimising ventilation. In particular muscle relaxants should be considered if the baby requires high pressure IPPV (e.g. a mean airway pressure of > 14) surfactant administration (survanta, one ampoule) is optional. Some babies with CDH tolerate this procedure very poorly
The baby with a best preductal pO2 <70 mmHg, or a persistent respiratory or metabolic acidosis despite 3-6 hours of attempting to optimise ventilatory and circulatory support has a very poor prognosis.</p/>
4. Continuing Management:
requires a team of neonatologists, paediatric surgeons and paediatric intensivists continuous monitoring of transcutaneous pCO2, tidal and minute volumes the lowest FiO2 that results in a preductal saturation > 85% is maintained, especially in the initial hours of care. Permissive hypercarbia is strongly advocated preductal arterial access is desirable if the infant's condition is marginal assessment for dysmorphism - including echocardiography, renal and cranial sonography, a karyotype should be considered an ongoing metabolic acidosis requiring repeated large doses of base suggests myocardial ischaemia, sepsis or strangulated bowel the principles of management and escalation of therapy include: use of muscle relaxants and sedatives synchronised ventilation (SIMV or A/C) with tidal/minute volume monitoring HFOV +/- Inhaled nitric oxide if the baby has unsatisfactory gas exchange after a reasonable trial of conventional ventilation, or there is an ongoing need for high ventilatory pressures or FiO2 jet ventilation if there is overt gas trapping or air leak ECMO - less than 10% of babies need this surgery - after ventilatory and circulatory support weaned to satisfactory levels (e.g. FiO2 < 0.4 and mean airway pressure < 14) discharge to a level II unit considered after full enteral nutrition established for at least one week respiratory status indicates significant reserve audiology - arranged prior to discharge if possible long term follow up required
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Introduction:
There are a number of organisms that can cause congenital neonatal illness - often with devestating long term consequences. Rubella embryopathy was the first documented neonatal congenital infection, being recognised in 1941 by an Australian Ophthalmologist - Sir Norman Grigg. Congenital infection can occur during pregnancy or the peri-partum period. Primary infection in the mother, generally, results in greater risk of consequences to the developing fetus compared with 're-activation'. The timing of infection is important in regards to the severity of neonatal illness and in relation to the organism involved.
A. TOXOPLASMOSIS:
1. 2. 3. 4. 5. 6. 7.
Summary Introduction Clinical features Investigation Management Outcome Prevention
1. Summary:
Congenital Toxoplasmosis occurs in between 0.2 to 10 per thousand pregnancies Risk of fetal infection is lowest in early pregnancy but most fetuses infected early have severe consequences Specific therapy is available to treat congenital toxoplasmosis
2. Introduction:
Toxoplasma gondii is a parasitic organism. The domestic cat is the primary host. Infection can be contracted by: - ingesting oocytes present in faecal material of infected hosts - eating pseudocysts present in undercooked meat. Most women have no symptoms. Although 15% of women report acute flu-like illness with lymphadenopathy. Risk of fetal infection is lowest in early pregnancy but most fetuses infected early have severe consequences.
10% 60%
Infection usually affects the neurological and haemopoietic systems. The classical tetrad described by Sabin in 1942 includes: - hydrocephalus/microcephaly - chorioretinitis - convulsions (link) - other evidence of CNS involvement (including calcification) Haemopoietic manifestations include: - hydrops due to anaemia - rash due to thrombocytopenia purpura - blueberry muffin appearance (seen in 25% of generalised infection) - lymphadenopathy
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- hepatosplenomegaly Neurological manifestations include: - convulsions - hydrocephalus with bulging fontanelle - microcephaly - chorioretinitis can be present early or develop later Generalised features include: - lethargy and malaise - poor feeding - vomiting - diarrhoea - temperature instability - jaundice
4. Investigation:
Antenatal diagnosis can be performed using fetal blood sent for: - PCR - IgM assay - culture Postnatal investigations include; - specific IgM or IgA in cord or baby's blood - FBE (anaemia/thrombocytopenia) - liver function tests - culture by inoculation of blood/placenta in mice - cranial US (hydrocephalus and calcification) - CT scan (more sensitive than ultrasound at identifying calcification) - ophthalmological review
5. Management:
Treatment consists of prolonged therapy for the first year of life with: - pyrimethamine (1mg/kg orally daily) - sulphadiazine (50mg/kg orally, twice daily) - folinic acid (1ml/kg, orally twice weekly) is added Bone marrow suppression and hepatotoxicity can develop, and fortnightly blood tests are needed. An alternative regime aimed at minimising toxicity may be used: - 4 x 21 day cycles of pyrimethamine, sulphadiazine and folinic acid - spiramycin (50mg/kg, orally twice daily) used for 30 days in between The addition of steroids in severe infection has been suggested, but no evidence exists for this practice. Ongoing opthalmological and developmental follow up is mandatory.
6. Outcome:
Infants symptomatic at birth have high incidence of long term difficulties: - chorioretinitis (over 90%) - developmental delay (50%) - seizures (40%) - microcephaly (20%) - deafness - hydrocephalus Outcome data for infants who are asymptomatic at birth is scant. In 'asymptomatic' neonates, it appears that a significant number develop long-term sequelae if left untreated. Up to 92% develop long-term problems, usually due to ophthalmological disease. Chorioretinitis may not become evident for many years. Although prolonged therapy reduces the incidence of sequelae compared to untreated infants sequelae may still occur (over 80%).
7. Prevention:
Prevention of Toxoplasmosis is aimed at preventing ingestion of infected material. Pregnant women should be warned to avoid foods/products that may be contaminated with the oocytes - including care with the family cat (there may be a role for advocating against acquiring a new cat in households with pregnant women).
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B. RUBELLA:
1. 2. 3. 4. 5. 6.
Introduction Clinical features Investigations Management Prevention Outcome
1. Introduction:
Most people develop immunity (if not immunised) during childhood. In non-immunised populations, 10-20% of women of child bearing age are susceptible. Re-infection occurs in around 2% of people but is generally subclinical. Cases of congenital infection have been described with maternal re-infection. Rubella vaccination is effective in almost totally eliminating congenital rubella infection - provided coverage remains high.
2. Clinical features:
Most congenital infection is the result of primary maternal infection. The mother may have had little, if any, symptoms of infection.
Fetal Complications Congenital rubella syndrome (>90%) Sensorineural deafness (20%) Rare
Congenital rubella syndrome is a severe, disabling condition featuring: eye disorders (cloudy cornea/cataracts, salt and pepper chorioretinitis, microphthalmia) sensorineural deafness cardiovascular (pulmonary stenosis and PDA) microcephaly growth restriction haemopoietic disorders: hepatosplenomegaly lymphadenopathy thrombocytopenia anaemia extramedullary heamopoiesis (blueberry muffin skin appearance) long bones radiolucencies seen on Xray pneumonitis with associated respiratory signs renal tract abnromalities
3. Investigations:
Diagnosis is usually demonstrated by evidence of maternal seroconversion or rising IgG titres. This occurs some 10 days after contact. IgM assay is useful where exact 'contact time' is not known. IgM persists for around 2 months after primary infection. Re-infection can be identified by seeing a four-fold or more rise in IgG titres. Fetal diagnosis is possible from: - cord blood IgM - rubella PCR of amniotic fluid Postnatal diagnosis is by: - IgM - isolation of rubella virus (possible form many sites including NPA, eye, throat, CSF, stool and urine for up to 12 months) Other test include: - FBE - renal function and electrolytes - liver function tests
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cranial ultrasound (looking for discrete calcification) echocardiography (looking particularly for Pulmonary Stenosis and PDA) renal ultrasound LP (pleocytosis with elevated protein) CXR (indicated if the baby has respiratory symptoms) long Bone Xrays hearing assessment is mandatory, even in babies with no overt disease at birth.
Deafness may be progressive, and therefore serial hearing assessments over the 1st few years of life are essential. Ophthalmological assessment is also essential and progressive retinal damage can be seen. Endocrine problems can occur in the long term including diabetes mellitus and hypothyroidism .
4. Management:
There is no specific treatment. Management is supportive and aimed at addressing specific problems present (developmental/sensory/endocrine/cardiac/etc ).
5. Prevention:
Rubella immunisation is offered to all children in combination with measles and mumps vaccination at 1 year of age. This also reduces the 'viral pool' in the population and helps protect susceptible pregnant women. New arrivals into Australia also are a potential group of susceptible individuals. All women should be screened at first antenatal clinic appointment, and if found to be rubella susceptible, offered immunisation in the post-partum period. If rubella infection is confirmed in the pregnant woman, then appropriate counselling is essential to provide the woman with information regarding the likely effects on the unborn child and options for management.
6. Outcome:
Children with congenital rubella syndrome are likely to have severe developmental issues. Ongoing hearing and vision assessments are essential in babies whose mothers contracted rubella after 12 weeks gestation. Rarely, a form of subacute sclerosing panencephalitis, with demonstration of raised rubella antibodies in CSF, has been documented.
1. Introduction:
CMV is a ubiquitous herpes virus that usually it causes only mild disease. It is commonly acquired in infancy and childhood through 'saliva sharing', particularly in less developed countries where over 90% are infected in childhood compared to around 60% in Western countries.
2. Clinical features:
Overall, around 5 to 10 per 1000 babies are born with congenital CMV infection - with only 5-10% being symptomatic. Congenital CMV can develop from maternal primary infection or re-activation, with primary infection more likely to result in sequelae. Risk for sequelae is highest after fetal infection during the first trimester. Around 1% of non-immune women develop primary CMV in pregnancy, approximately 50% of their fetuses become infected. In women with prior CMV infection, re-activation can occur in pregnancy with 5% of babies developing infection inutero. Perinatal and postnatal infection can occur through birth canal secretions and breast milk or blood. If affected the clinical syndrome is usually mild and self-limiting. Severe infection can result in transfusion-acquired disease. Use of CMV negative donor blood or deglycerolised frozen blood transfused through a leucocyte filter prevents this problem.
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Symptomatic babies can be variously affected including: - growth restriction - haemotological problems: thrombocytopenia and purpura are common anaemia, neutropenia, lymphocytosis are occasional hepatosplenomegaly lymphandenopathy - neurological problems: poor tone and poor suck seizures microcephaly chorio-retinitis cerebral calcification (classically periventricular) deafness (can be a late manifestation and is the most common cause of sensori-neural hearing loss) - pneumonitis - colitis - hepatitis - dental defects
3. Investigations:
A high index of suspicion is needed. Investigation is warranted in: - babies whose mothers have developed primary infection in pregnancy or have 're-activated' - growth restricted infants with low platelet count Tests performed include: - IgM assayed from cord/baby blood ( tests have poor sensitivity) - viral culture from urine (the test of choice) - throat/saliva swab and NPA sent for PCR can give a more rapid answer. Culture and PCR should be performed as soon as possible in the first 2 weeks of life as CMV detected after this time can indicate peri/post natal infection.
4. Management:
Attention to 'general' measures include management of: - nutrition - haemotological disturbances (low platelets) - respiratory disease Specific treatment with foscarnet of ganciclovir has not been shown to be associated with long-term benefits in congenital infection. There is a role for their use in life-threatening CMV infection (particularly post-natal disease in preterm babies) or in the setting of chorio-retinitis but this will not reverse damage that has already been done. Long-term follow-up is essential with attention to: - management of neurological sequelae - hearing testing, continued for the first 2 to 5 years of life, due to the risk of late-onset deafness
5. Prevention:
Two groups require consideration: - hospitalised newborn infants: prevention of transfusion acquired infection is achieved by use of leucocyte filters or CMV negative blood standard precautions and aseptic measures (particularly hand-washing, especially after nappy changing) is important to prevent nosocomial spread of infection - potentially 'at-risk' pregnant women After infection viral shedding can either persist for months or occur transiently and then recur intermittently. Testing for viral shedding will not guarantee a baby is not shedding virus at other times. Seronegative staff members have a low risk (around 1-5%) of developing a primary infection. Attention to prevention through use of standard precautions is important. Likewise, it is difficult to advise parents of babies with documented CMV infection as to what to tell friends/family. Re-assurance and explanation about prevention of spread through scrupulous attention to hand washing after performing baby care are the most valuable tools.
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1. Introduction:
HSV 1 and HSV 2 are uncommon, but important, causes of neonatal illness. Neonatal infection is usually the result of HSV 2 as this is the main virus associated with genital infection. The overall rate of genital HSV infection varies from country to country and many women (amd men) remain asymptomatic, with no history of infection despite shedding virus. HSV can remain latent for long periods, with shedding or re-activation occurring at any time. There is a 1% chance of a women with a past history of genital HSV infection shedding virus at the time of delivery. In cases of neonatal infection, most women do not give a history of active genital herpes at the time of delivery. Babies born to mothers with a primary genital infection at the time of delivery have a 50% risk of developing infection, compared with <5% in cases of recurrent infection present at the time of delivery.
2. Clinical features:
Neonatal HSV usually presents within 2 weeks of birth Infection occurs in less than 5 per 100,000 deliveries (up to 10 times more in the USA). - 90% are acquired during passage through the birth canal or through ascending infection - 5% have 'congenital' HSV infection - 5% have post-natally acquired infection Usual clinical presentations are: - skin/eye/mouth (SEM) localised disease: untreated, >70% will progress to disseminated disease 25% will have virus in CSF at initial presentation isolated vesicles or 'crops occassionally other skin reactions can be present, including zoster-like eruptions keratoconjuncitivitis with dendritic ulcers choriodoretinitis single of multiple oral vesicular lesions can be present - disseminated disease: poor prognosis, with over 70%mortality untreated non-specific presentation with: lethargy poor feeding fever convulsion apnoea respiratory distress hepatomegaly jaundice DIC - Pneumonitis: tends to occur day 4 to 7 respiratory distress and can develop into haemorrhagic pneumonitis CXR shows diffuse pnuemonic change rare but dissemination is common if untreated - meningo-encephalitis: isolated or part of dissemnated disease presents with: encephalopathy (mean 11 days of age) seizures are common and often intractable absent gag-reflex is a particular feature - EEG shows characteristic temporal/parieto-temporal focus with periodic slow and fast waves. - brain imaging (CT/MRI) shows disease particularly affecting the temporal areas. Later calcification and cerebral atrophy can develop.
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3. Investigations:
Neonatal HSV infection is uncommon and a high level of vigilance is needed, particularly since most affected newborns are born to mothers with no history of current genital HSV lesions. The unwell baby should be examined for vesicles including oral. Specimens from lesions, throat and eye swabs should be performed. The use of immunoflourescence can provide rapid evidence of infection. Viral culture can take 5 days to demonstrate typical cytopathic changes. Lumbar puncture is mandatory, with CSF sent for PCR and culture. However, negative PCR testing on CSF does not completely rule out HSV infection, and the clinical picture of herpes encephalopathy is important in determining treatment. EEG and brain imaging are useful adjuncts in cases where diagnosis of CNS infection is in doubt. Serological studies are of little value early on as IgM may take 2 weeks to appear and IgG titres may not rise in babies and may reflect maternal antibody status.
4. Management:
Specific early treatment is with: - Acyclovir 10mg/kg IV three times daily for a total of 14 to 21 days - Vidarabine 15mg/kg 12 hourly, IV is also effective but is more cumbersome Supportive care as always is vitally important with attention to general care. Eye lesions require topical treatment (eg. Idoxuridine) and ophthalmological referral is essential.
5. Prognosis:
Mortality and morbidity rates for disseminated and CNS disease are very high, even with early and aggressive treatment. Even in the setting of localised SEM disease, 10% have long-term neurological sequelae. Recurrent skin and eye eruptions can occur. Oral acyclovir has a role in this setting.
6. Prevention:
Babies born to mothers with active genital herpes lesions, particulary primary infection, at the time of delivery are at high risk. LUSCS should be performed as soon as possible, particularly within 6 hours of ROM. Swabs from the baby's eyes and throat should be sent and if positive, the baby treated with acyclovir sysemtically. However, consideration to treatment even in the setting of CS should be given - especially if there delay in getting results occurs. Babies born vaginally in the face of active genital lesions should be treated systemically. Nosocomial infection can occur and vigorous infection control measures should be instituted. Relatives or staff with 'cold-sores' should be discouraged from handling newborn infants as there is a risk of infection. Mothers with cold-sores present a low-risk to the infant as passive antibody protection of the baby should be present, but in this setting the wearing of masks/rigorous handwashing and topical cold-sore treatment should be advocated.
E. SYPHILIS:
1. 2. 3. 4. 5. 6.
Introduction Clinical features Investigations Management Follow-up Prevention
1. Introduction:
Congenital syphilis is rare in Australia, disadvantaged groups are at higher risk. Screening at the initial antenatal visit is part of routine obstetric care since women may have asymptomatic latent disease. Untreated maternal syphilis can result in: - stillbirth/perinatal death - premature delivery - about half of survivors have long-term neurological sequelae. Transmission rates are around 50% for primary, secondary or early latent syphilis. In established latent syphilis, vertical transmission occurs in around 10%. Treponema pallidum infection remains treatable with penicillin.
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2. Clinical features:
The infected baby can: - be asymptomatic at birth - have disseminated sepsis with: reticulo-endothelial/haemotogical features: generalised lymphadenopathy and hepatosplenomegaly seen in over 50% haemolytic anaemia/thrombocytopenia/pancytopenia can occur occassional leucocytosis jaundice (unconjugated/conjugated or mixed) is common mucosal features: rhinitis (snuffles) develops at 1 week and worsens. Initially clear then progressively purulent and blood stained mucous 'patches' seen on palate and lips perioral and perianal condylomata ulceration of nasal mucosa can lead to 'saddle-nose deformity' in longer term cutaneous features: maculo-papular eruption over buttocks and lower torso, palms and soles bullous eruptions (pemphigus syphiliticus) which mimic staphyloccal infection desquamation bone involvement: osteo-chondritis, periostitis, osteitis is very common (>75% of cases). Usually asypmtomatic initially but can lead to deformity and pathological fracture neurosyphilis (rare at birth): meningitis eye involvement: gluacoma chorioretinitis chancres uveitis myocarditis pneumonitis renal (nephrotic) involvement if untreated, late features include: Hutchison's teeth and other dental deformity Sabre tibia keratitis and blindness nerve deafness saddle nose deformity frontal skull bossing scarring development impairment
3. Investigations:
Pregnant women are screened with non-specific treponemal tests (RPR and VDRL titre). If positive, then a specific TPHA/FTA-Abs titre will be performed. Serum IgM in newborn babies with congenital syphilis is positive in around 90%. Diagnosis of congenital infection can be confirmed by demonstration of treponema pallidum on dark ground microscopy on specimens from lesions on skin, placenta or other tissues. A fourfold rise in the baby's antibody titre over the first 3 months is considered diagnostic. Other tests:
FBE liver function tests urinalysis for proteinuria X-rays of long bones lumbar puncture: CSF abnormalities should be considered suggestive of CNS infection a positive CSF VDRL titre or treponema PCR is diagnostic of CNS involvement
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4. Management:
Babies born to mothers who have not been adequately treated should be considered as infected. Infants with suspected or confirmed infection should be treated with penicillin for at least 10 days. Benzyl penicillin, 30mg/kg per dose 12 hourly, IM or IV or Procaine penicillin, 30mg/kg daily, IM Infants with low antibody levels whose mother was treated appropriately and has evidence of falling RPR/VDRL titres, is unlikely to be at risk. Ongoing follow up will be needed and if follow up cannot be assured, the baby should be treated.
5. Follow up:
Babies should be evaluated at 3-monthly intervals over the first year of life, with serological tests performed at each visit.. In cases of neurosyphilis, ongoing serum and CSF analysis should be undertaken 6-monthly for the first 5 years of life. Re-treatment is needed if titres do not fall, or clincial signs of disease persist or develop.
6. Prevention:
Prevention relies upon adequate antenatal services and screening facilities.
F. Further reading:
Isaacs D, Moxon ER. "Handbook of Neonatal Infections - a practical guide". WB Saunders, London. 1999. Remington JS, Klein JO. "Infectious Diseases of the Fetus and Newborn Infant" 5Th Ed. WB Saunders, Philadelphia. 2000. Davies EG, Elliman DAC, et al. "Manual of Childhood Infections". WB Saunders, London, 1996. Jeffries DG, Hudson CN. "Viral infections in Obstetrics and Gynaecology". Arnold, London, 1999. Jones JL. Lopez A. Wilson M. Schulkin J. Gibbs R. Congenital toxoplasmosis: a review. Obstetrical & Gynecological Survey. 56(5):296-305, 2001 May Beazley DM. Egerman RS. Toxoplasmosis. Seminars in Perinatology. 22(4):332-8, 1998 Aug. Jacobs RF. Neonatal herpes simplex virus infections. Seminars in Perinatology. 22(1):64-71, 1998 Feb. Murph JR. Rubella and syphilis: continuing causes of congenital infection in the 1990s. Seminars in Pediatric Neurology. 1(1):26-35, 1994 Sep. Riley LE. Herpes simplex virus.Seminars in Perinatology. 22(4):284-92, 1998 Aug. Hollier LM. Cox SM. Syphilis. Seminars in Perinatology. 22(4):323-31, 1998 Aug
A. Summary: - echocardiography is the gold standard for the assessment of congenital heart disease in infancy - all infants with central cyanosis should be commenced on parental antibiotics early until further
investigation is possible. differentiating PPHN from ductal dependent pulmonary cardiac lesions can be very difficult; if uncertainty exists a PG infusion is generally the safest option for transport
B. Introduction:
Central cyanosis affects 3-4% of all newborns and is a marker of significant disease. The causes are varied including: - primary pulmonary disease - cardiac malformations - persistant pulmonary hypertension - sepsis - anaemia - asphyxia - metabolic conditions - methaemaglobinaemia
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Tachypnoea, grunting and Primary Pulmonary recession Disease Cardiac PPHN Sepsis Tachypnoea, slow/deep breathing Tachypnoea, recession and grunting may be present Respiratory distress may be present
Present +/+/-
D. General Approach to the Cyanotic Infant: - Confirm central cyanosis with arterial blood gas (ABG) in room air, if possible, a sample from the right arm
is the best site Assess the history and examination for cause, including four limb BP: an upper to lower limb systolic difference of > 10mmHg is significant and suggestive of Coarctation of the Aorta hypotension in a cyanotic infant is a serious finding Correct metabolic acidosis and systemic hypoperfusion if present with fluid boluses and bicarbonate Hyperoxia Test (HT): 100% FiO2 into headbox for > 10 min monitor SaO2 Repeat ABG: PaO2 > 100mmHg or SaO2 increase by 15%: pulmonary disease likely PaO2 < 70mmHg, rise by < 30mmHg or SaO2 unchanged: cardiac cause or PPHN likely Total Anomalous Pulmonary Venous Drainage (TAPVD) and Hypoplastic Left Heart syndrome may respond Pulmonary disease with a massive intrapulmonary shunt may not respond Limitations of HT: HT is not as reliable as an echocardiogram and is not as important as resuscitation and attendance to cardiorespiratory support, especially if acidosis or respiratory distress is present HT has many limitations especially when only saturations are measured and not arterial PaO2 and should only be used in conjunction with a thorough clinical assessment Assess right and left sided SaO2 for any ductal difference CXR and ECG if possible Intubation and paralysis if significant distress Two IV lines ideally or UVC Parental antibiotics (preferably after blood cultures taken) Prostaglandin E1 infusion if duct dependent cardiac disease is suspected Consider inotrope support to maintain BP/improve cardiac contractility Infants should be transferred to a centre with Paediatric Cardiology and Cardiac Surgery facilities readily available
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PPHN results from an altered pulmonary vasoreactivity and raised pulmonary vascular resistance, this causes a right to left ductal shunt. Most infants present with respiratory distress and cyanosis. Usually the infant is tachypnoeic, shows labile oxygenation and has evidence of right ventricular strain (prominent right ventricle impulse and tricuspid regurgitation murmur). There is usually a pre- and post-ductal difference of 10-15% in SaO2 and 10-15mmHg PaO2. Despite this a degree of clinical variability exists depending on the severity, stage of disease and underlying pathology. If PPHN is suspected hyperventilation for 10 minutes is a useful clinical test when other investigative options are unavailable. Infants with PPHN show improved oxygenation (PaO2 increasing by >30mmHg) when pH is raised to 7.55. Prolonged hyperventilation is not recommended due to the cerebral effects of prolonged hypocapnia. Differentiating PPHN from ductal dependent pulmonary cardiac lesions can be very difficult; if uncertainty exists a PG infusion is generally the safest option for transport and infants with PPHN may show some improvement on infusions of 50-60 nanog/kg/min.
F. Cardiac Causes:
Generally an infant with cyanosis and a murmur has a high probability of a cardiac cause, absence of pulses further raises the probability. Infants presenting with cyanosis due to a cardiac cause have a high probability of a ductal dependent lesion involving either: - Ductal Dependent Pulmonary Circulation presenting with: cyanosis tachypnoea without respiratory distress adequate perfusion initially such as: critical pulmonary stenosis transposition of the great vessels - Ductal Dependent Systemic Circulation (critically obstructed systemic circulation) presenting as: cardiac failure with systemic hypoperfusionhypoperfusion poor or absent peripheral pulses increasing metabolic acidosis cyanosis may not develop until the latter stages of the clinical course Such as: coarctation of the aorta hypoplastic left heart syndrome critical aortic stenosis - Management: Aim to maintain adequate tissue perfusion and ductal patency rather than correcting the cyanosis. minimise pulmonary blood flow: moderate PEEP (4-6 cm H20) ventilate in air if possible aim for a CO2 of 37 - 45 mmHg SaO2 75 - 85% maximise tissue perfusion: fluid resuscitation (saline 10ml/kg boluses) low dose inotropes (dopamine/dobutamine) - further details in section on management of shock sodium bicarbonate if BE > -10 (dose (mmol) = BE *wt/4) consider paralysis if infant distressed achieve ductal patency with Prostaglandin E1
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Dose:
When the duct is still open the priority is to prevent further loss of patency, usually a starting dose of 10 ncg/kg/min infusion in 5% dextrose or Normal Saline is adequate. If no improvement in SaO2 then increase dose by 10 nanogram/kg/min increments up to 50 nanogram/kg/min until SaO2 improves. Prostaglandin can be given via a peripheral line, UVC or UAC. Infants in extremis will usually have a closed duct and a higher starting dose of 100 ncg/kg/min will be required to reopen the duct, when saturations improve then dose can be decreased to a dose to maintain ductal patency.
Side Effects:
Most frequent side effects include: fever 12% apnoea 12% flushing 10% hypotension Apnoea will rarely occur at 10 ncg/kg/min and apnoea is not an indication to decrease the dose if the infant is responding clinically, rather respiratory support is warranted. The likelihood of apnoea is very high at a dose of 100 ncg/kg/min and most infants on this dose should have ventilatory support. Close observation is mandatory following commencement of PG infusion, and assisted ventilation and volume expansion or inotrope infusion are frquently required.
Who to intubate?
The threshold to intubate an infant on a PG infusion will be lower in a transport or remote setting. Factors to consider include presence of apnoea the distance to the receiving hospital gestation of the infant clinical state of the infant (metabolic acidosis, shock, severe distress and tachypnoea) high PG dose required to achieve ductal patency Thus in a stable infant with a PG responsive ductal lesion transport without intubation maybe appropriate. Conversely, it would generally be appropriate to electively intubate an infant requiring high dose PG (although these infants usually require respiratory support for other reasons).
H. References:
Penny DJ, Shekerdemian LS. Management of the neonate with symptomatic congenital heart disease. Arch Dis Child Fetal Neonatal Ed 2001; 84: F141 - F145. Hellstr?m-Westas L et al. Long-distance transports of newborn infants with congenital heart disease. Pediatr Cardiol 2001; 22: 380-384 Buck ML. Prostaglandin E1 treatment of congenital heart disease: use prior to neonatal transport. DICP Ann Pharmacother 1991; 25: 408 - 9 Barry PW, Ralston C. Adverse events occurring during interhospital transfer of the critically ill. Arch Dis Child 1994; 71: 8-11 Jaimovich DG, Vidyasagar D (Ed). Handbook of pediatric and neonatal transport medicine. Ch 8. 2nd Ed. 2002. Hanley & Belfus. Pp 93 - 125 Myung K Park. Pediatric cardiology for practitioners. 3rd Ed. 1996. Mosby-Year Book (St Louis)
Web Sites:
Cyanosis in the newborn: The Cyanotic Newborn Infant (University of Minnesota Neonatology Lecture) http://www.kumc.edu/kumcpeds/cardiology/cardiology.html Prostaglandin: http://www.cs.nsw.gov.au/rpa/neonatal/html/listview.asp?DrugID=36
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A. Summary: - premature neonates are born before brain maturation is complete - the stressful and abnormal environment of the nursery may contribute to altered brain development - modification of the nursery environment may reduce long term morbidity - light protection should not prevent adequate visualisation of the infant B. Introduction:
While advances in biomedical technology and improvements in care have led to a decrease in mortality rates in premature and extremely low birth weight neonates, there has not been a corresponding change in morbidity. Comprehensive long term follow up of these infants has lead to the recognition of new morbidities:
including learning and attention deficit disorders language comprehension and speech problems and visual and motor impairments
The focus of neonatal care must now extend beyond simply achieving survival. Tthe challenge now is to optimise infants developmental course and long term outcome. Developmental problems resulting from damage to the cerebral cortex may not become evident for some months or even years after birth. Infants "at risk" therefore require long term developmental follow up.
reduce stress and prevent agitation preserve energy and promote growth enhance recovery facilitate self regulatory capabilities promote C.N.S. organisation
Individualised strategies such as the "Neonatal Individualised Developmental Care and Assessment Program" (NIDCAP) have also been used.
protect from light: constant bright light in the nursery can interfere with the development of natural diurnal rhythms and have an arousing effect on the C.N.S. reducing light levels may prevent sensory overload and facilitate rest cover incubator hoods to reduce exposure to bright overhead lights dim the lights at night to assist in establishing day/ night patterns
Light protection should not preclude adequate visualisation of the sick or potentially sick infant.
protect from noise: noise (above 80-85decibels) has the potential for damage to the cochlear & hearing loss in adults. The immature cochlear is more sensitive to damage closing portholes with a "snap", dropping the head of the mattress and tapping or placing bottles on the Plexiglas top of the incubator all have a sound level above 80db noise may also cause agitation, irritability and crying, which may result in increased intra-cranial pressure and decreased oxygen saturation
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interventions to reduce noise include: turn the radio down or off have a designated "quiet time" daily avoid banging bin lids remove bubbling water in oxygen/ventilator tubing close incubator portholes gently give 'handover' away from the infants bedside avoid talking loudly, especially across open care beds
protect from over-stimulation: handling can effect physiological stability and cause hypoxaemia, especially in the extremely premature, unstable or ill neonate provide "time out"/ recovery time when the infant demonstrates avoidance or "stress" behaviour. Signs of stress behaviour include: colour changes: mottled, dusky, cyanosed apnoea, bradycardia, desaturation hiccoughing, sneezing, yawning, gagging, regurgitating feeds tremors, twitches, frantic activity, arching, frowning, gaze averting completely flaccid trunk, extremities & face easy fatiguability (take care as these behaviours may have serious underlying causes) introduce sensory stimuli slowly: eg one toy or picture in the incubator (too many are overwhelming) and be sensitive to the infants response alter patterns of care to allow maximum time for sleep and growth: clustering of cares/ minimum handling approach positioning: prone or side lying to enhance flexion, bring the shoulders forward and the hands to the midline provide boundaries and use rolls/nesting to maintain desired posture, reduce agitation, conserve energy and create a feeling of "security" for the infant avoid moving an infant who has sought out his own boundaries (eg foot against a porthole door) establish day and night patterns (diurnal rhythm): dim the lights at night and turn the radio off remind staff to talk & walk quietly around the nursery avoid non-emergency interventions during the night: eg. bathing & weighing normalise parent expectations: promote parent understanding of their infants behaviour, including signs and manifestations of stress provide opportunities for Kangaroo Care, Non-nutritive sucking and other forms of sensory stimuli as the infant matures and is able to maintain physiological homeostasis
E. Areas Of Uncertainty In Clincial Practice: - Does developmental care lead to the hypothesised measurable outcomes of:
reduction in incidence and severity of developmental delay improved weight gain decreased length of hospital stay less days of mechanical ventilation less days of oxygen dependence?
A Cochrane review of 31 eligible randomised control trials found evidence of some benefit from developmental interventions, with no major harmful effects reported. However, there were a large number of outcomes for which conflicting effects or no effects were demonstrated.
Broad interventions such as reducing light and noise in a nursery are easily implemented, of negligible cost and not harmful; however implementing and maintaining a formal developmental care program (such as NIDCAP) has a significant economic impact. Symington & Pinelli (2001) suggest that further evidence of the efficacy of developmental care is required before a clear direction for practice can be supported.
F. References:
Als, H., Lawhon, G., Brown, E., Gibes, R., Duffy, F.H., McAnulty, G., & Blickman, J.G. (1986). Individualised behavioural and environmental care for the very low birth weight preterm infant at high risk of Bronchopulmonary Dysplasia: Neonatal Intensive Care Unit and developmental outcome. Pediatrics. 78(6): 1123-1132.
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Blackburn, S.T. & VandenBerg, K.A.(1993). Assessment and management of neonatal neurobehavioral development. In: Kenner, C., Brueggemeyer,A. & Gunderson, L.P. (eds). Comprehensive Neonatal Nursing: A Physiological Perspective. Philadelphia: WB Saunders, 1094-1134. Buehler,D.M., Als, H., Duffy, F.H., McAnulty,G.B. & Liederman, J. (1995). Effectiveness of individualised developmental care for low risk preterm infants: Behavioural and electrophysiologic evidence. Pediatrics. 96(5): 923-932. Symington, A. & Pinelli, J. ((2001). Developmental care for promoting development and preventing morbidity in preterm infant (Cochrane Review). The Cochrane Library, 2, 2001. Oxford: Update Software. VandenBerg, K.A. (1995). Behaviourally supportive Care for the Extremely Premature Infant. In: Gunderson, L.P. & Kenner, C. (eds). Care of the 24-25 Week Gestational Age Infant. 2nd Ed. Petaluma: NICU INK, 145-171. Web Links: Developmental Progress Clinic at Emory University
A. Screening Algorithm: - all newborn infants should have the Ortolani and Barlow tests performed by a trained examiner as part of
the routine newborn examination Unfortunately, infants who are unwell after delivery, and who require admission to neonatal intensive or special care units, often have this important part of their newborn examination omitted. It must always be documented that the examination has been performed both in the infant's medical record and child health record. infants, in whom the examiner is uncertain of the findings, should be re-examined by a more experienced clinician prior to discharge Inexperienced examiners who are unsure whether what they are feeling is a 'click' or a 'clunk' are advised to enlist expert help. infants in whom either test is positive should be assessed by an orthopaedic surgeon prior to discharge and fitted with a Pavlik harness or Von Rosen splint. There is nothing to be gained by performing X-rays or ultrasonography in these infants. In addition, there is no evidence to support the use of double or triple napkins until definitive treatment is instituted high risk infants in whom examination is normal should have ultrasonography performed within 4-6 weeks of birth High risk infants are those with: breech presentation history of DDH in a first degree relative (parent or sibling) neuromuscular disease since DDH can develop over time, all infants (both high and low risk) with normal newborn examinations should have their hips regularly re-examined during the first year of life
B. Introduction:
Developmental dysplasia of the hip (DDH) is the preferred term for the disease previously referred to as congenital dislocation of the hip since it recognises that presentation can follow a normal examination of the hips in the newborn period. DDH refers to a spectrum of disorders of hip instability due either to the femoral head being able to move outside the acetabulum (luxation or dislocation), or abnormally within the acetabulum (subluxation or partial dislocation). Early detection is vital since if DDH is left untreated the hip joint develops abnormally and surgical reduction is required. By contrast, early conservative management with splinting (either a Pavlik harness or a Von Rosen splint) allows the hip joint to develop normally and avoids the need for surgery in most cases. Pathogenic factors for DDH include: - abnormal rotation of the developing hip during the first trimester - neuromuscular disease, especially in the second trimester - abnormal mechanical forces e.g. oligohydramnios, breech presentation (particularly frank breech), in the third trimester - female infants (who are more susceptible to the maternal hormone relaxin) - postnatal mechanical forces associated with swaddling (African infants strapped to their mothers' backs with hips abducted have a very low incidence of DDH)
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The relative risk for infants with a history of DDH in a first degree relative is 1.7 and when born after breech presentation (all types) is 7.0.
D. Screening:
There is no 'gold standard' diagnostic test for DDH. The Ortolani and Barlow tests are widely used for screening: The Ortolani test detects a dislocated hip reducing during the examination. The Barlow test detects a hip dislocating or subluxing during the examination.
A positive Ortolani or Barlow test is one in which a distinctive 'clunk' is felt. 'Clicks' are often felt while performing these tests, are not predictive of DDH, but cause considerable confusion. Readers who wish to learn the tests should: - consult an authoritative text - be shown how to perform both tests by an expert - practice the tests on the 'Baby Hippy' manikin - practice the tests on many infants to perfect their technique
E. Additional Investigations:
X-rays are unhelpful in assessment as the femoral head is cartilaginous until six months of age.
G. References:
American Academy of Pediatrics. Clinical Practice guidelines: Early detection of developmental dysplasia of the hip. Pediatrics 2000; 105:896-905. Griffin PP, Robertson WW Jr. Orthopedics. In: Avery GB, Fletcher MA, MacDonald MG, editors. Neonatology: Pathophysiology and management of the newborn. Philadelphia: Lippincott, Williams & Wilkins, 1999:1270.
A. Summary: - a thorough history and examination is required - ancillary investigations may be useful - chromosome and gene tests may be warranted in certain circumstances - parental communication is important - potentially 'offensive' terms should be avoided
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B. Introduction:
A dysmorphology assessment of a newborn focuses on aspects of history, examination and investigations that may lead to a syndrome diagnosis. This assessment should be carried out in any child with any of the following - congenital abnormality - growth abnormalities - dysmorphic features Below are checklists for history and examination with a dysmorphology focus as well as investigations that the paediatrician should consider as part of a dysmorphology work-up. For many doctors, the discussion of issues relating to syndrome diagnosis and dysmorphism can be difficult, and some suggestions are outlined.
C. History Checklist: - pregnancy history, noting particularly exposure to teratogens, amniotic fluid volume - results of ultrasound and amniocentesis/CVS - fetal movements - maternal illness - delivery history - family history of abnormalities - consanguinity D. Examination Checklist:
The following focuses on the examination for dysmorphic features in a baby. A thorough examination of all other systems is vital when considering a syndrome diagnosis. a) Growth: Birth weight, length and head circumference. Assess whether the baby's growth parameters are in proportion as well as the percentiles
b) Ectodermal Features:
skin - texture and colour, birthmarks, redundancy, defects hair - scalp hair and body hair: colour and distribution. Note position of anterior and posterior scalp hairline
c) Skull:
shape, symmetry sutures (over-riding/normal/widely open) fontanelle size and number d) Face: In examining the face, it can be useful to first gain an overall impression of the facial appearance. Sometime, an overall gestalt can be diagnostic (e.g. Down syndrome). If no diagnosis is made, it is then important to divide the face into sections to examine it thoroughly. You may divide the face into the forehead, midface and oral region. It can sometimes help to cover parts of the face with your hand, in order to isolate the section of the face you are assessing. In assessing the face, it is important to view the face from the front and from the lateral view. The depth or height of structures such as the nasal bridge, the position of the mandible relative to the maxilla and the development of the midface are best assessed by the lateral view. - Overall face shape, symmetry, facial muscle movement - Forehead region: forehead shape - (broad/bitemporal narrowing/tall) eyes: palpebral fissure length (short/long) palpebral fissure slant (up/down) epicanthic folds - a fold of skin which arcs from below the eye into the upper lid eye spacing (use a rough guide of 1:1:1 for the ratio of left palpebral fissure length: inner canthal distance: right palpebral fissure length) palpebral fissure shape iris colour pupil shape retina globe position (assessed from lateral view: protuberant vs deep set globes) - Midface region: nose divide the nose into 3 sections from the lateral view from superior to inferior into the nasal root, bridge and tip. root bridge (depressed/prominent/broad)
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