Polycythaemic Newborn - Management of the

Publication: 26/08/2010  --
Last review: 24/05/2018  
Next review: 24/05/2021  
Clinical Guideline
CURRENT 
ID: 2160 
Approved By: Trust Clinical Guidelines Group 
Copyright© Leeds Teaching Hospitals NHS Trust 2018  

 

This Clinical Guideline is intended for use by healthcare professionals within Leeds unless otherwise stated.
For healthcare professionals in other trusts, please ensure that you consult relevant local and national guidance.

Management of the Polycythaemic Newborn

Background

Polycythaemia affects 2-5% of babies and is commonly defined as a venous haematocrit greater than or equal to 65%.2,4

  • The haematocrit typically peaks at 4-6 hours after birth and the declines over the next 12-18 hours so that by 24 hours of age it is equivalent to that at birth.2
  • Capillary samples have been proven to over-estimate PCV and the gold standard for diagnosis is arterial or umbilical venous sample.
  • In the clinical setting a free flowing sample from a large vein is sufficient for diagnostic criteria.2

Hyperviscosity occurs in about 50% of infants with polycythaemia3 (blood viscosity greater than 2 standard deviations above the mean; >13 cps at a shear rate of 11.25 sec.1) This results in increased internal friction of blood, which may produce sludging and impair end organ perfusion.

In the newborn the principle determinant of blood viscosity is the haematocrit.2 Although variable, hyperviscosity increases markedly when haematocrit exceeds 65%.

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Aetiology

Several disease states produce polycythaemia through a variety of mechanisms. As a feature of this, babies who are polycythaemic may be hypovolaemic, normovolaemic or have an increased intravascular compartment. See table 1.2,4

Table 1. Causes of polycythaemia

Erythrocyte transfusion (passive)

Delayed clamping of the umbilical cord (eg, >2 minutes after birth)

Uncontrolled or precipitous delivery

Intrapartum hypoxia

Twin-to-twin transfusion (10 to 15 percent of monochorionic twins)

Maternal-fetal transfusion (rare)

Increased intrauterine erythropoiesis (active)

Placental insufficiency

Preeclampsia

 

Other hypertensive disorders

 

Other vascular disorders

Maternal hypoxemia due to cardiac or pulmonary disorders

Cardiac or pulmonary disorders

 

Drugs (eg, propranolol)

 

Smoking

 

High altitude

 

Post term delivery

Infant risk factors

Large for gestational age

 

Maternal diabetes mellitus

 

Beckwith-Wiedemann syndrome

 

Endocrine abnormalities (congenital adrenal hyperplasia, hypothyroidism, hyperthyroidism)

 

Chromosomal anomalies (trisomy 21, 18, and 13)

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Clinical Features

Acute

Intra-vascular sludging affects multiple organ systems and symptomatic polycythaemic babies can present with a variety of clinical features including plethora, tremors, lethargy, irritability, poor feeding and vomiting. Some of these features result from hypoglycaemia, others from the direct effect of polycythemia. See table 2.2,3

Table 2. Common clinical features

System

Mechanism

Clinical finding

Skin

Increase red cells

Plethoric, jaundice

Respiratory

Decreased pulmonary blood flow

Respiratory distress

Gastro-intestinal

Decreased intestinal blood flow
Low enzyme levels

Poor feeding/vomiting/ increased risk NEC

CNS

Hypoglycaemia
Physiological reduction in cerebral perfusion

Irritability/jittery/lethargy/seizures
Not thought to cause ischaemia.

Metabolic

Increased glucose consumption
?Reduced gluconeogenesis
? increased CGRP

Hypoglycaemia
Hypocalcaemia

Renal

Depends on PCV and blood volume

ARF if hypovolaemia or normovolaemia

Asymptomatic babies are detected co-incidentally when FBCs are measured for other clinical indications.

Long-term
Whether neonatal polycythemia or its treatment affect long-term outcome is uncertain and more likely to be related to the associated conditions or causation e.g. hypoglycaemia, IUGR.

Long-term neuro-developmental impairment may be seen in both symptomatic and asymptomatic babies regardless of whether measures were taken to reduce PCV in neonatal period or not.5 6 It is believed that it is the in-utero environment resulting in the polycythaemic state, rather than the polycythaemia per se, that is responsible for the poor long-term outlook in these babies.5

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Management

At present there is no evidence to support the treatment of asymptomatic babies (or those babies with only mild symptoms) with partial exchange transfusion (PET) regardless of their PCV.4,5,6  There is no indication to check a PCV in an asymptomatic baby who is plethoric.

Studies have failed to show any long-term neuro-protective effect of PET in symptomatic infants (typical RR of neuro-developmental delay at 18-30 months 1.45, (95% CI 0.83, 2.54))6 and some authors reported increased rates of NEC among those receiving PET. 8,9

Current recommendations

In the absence of further evidence those

  • Babies with significant neurological symptoms should be treated with PET.5
  • No evidence to support use of PET in babies with no or only mild symptoms5,6
  • PET should be done with saline or other crystalloid rather than colloid solutions.10

From a practical point of view it is important to subdivide affected babies into symptomatic and asymptomatic, and hypovolaemic or normovolaemic as these affect the treatment modalities offered.

See flow chart.

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Partial Exchange Transfusion

This should always be discussed with the consultant.

The desired PCV is 50-55%, to achieve this 20ml/kg of blood is exchanged for normal saline.

Prior to procedure:

  • Place baby nil by mouth
  • Insert NGT and empty stomach
  • Treat hypoxia, hypoglycaemia, acidosis etc before commencing procedure
  • Cannulate umbilical vein, ensure tip not in liver (can also be done via peripheral arterial line/peripheral vein if no umbilical lines)
  • Investigations
    • FBC, U+E LFT, Calcium, glucose, gas

Procedure:     

  • Performed in NICU with full monitoring
  • Ensure clear documentation of procedure
  • Set infusion of normal Saline 20ml/kg running over 1 hour
  • In same hour remove 4 aliquots of 5ml/kg of blood. (every 15 minutes, each cycle to take 5 mins)
  • Full aseptic procedure
  • Monitoring of ECG, BP, saturations, RR and temp every 15 minutes
  • Check FBC, U+E, Calcium gas BM at end

After procedure:

  • Continue to monitor for 2 hours
  • Repeat FBC after 4 hours
  • Restart feeds 4 hours after exchange unless clinical concerns raised. (i.e abdominal distension/discolouration, abnormal NG aspirate, abnormal stools.)

Complications

  • Hypothermia-ensure fluids are warmed
  • Desaturation
  • Haemodynamic- fluid overload/fluid loss
  • Electrolyte disturbance
  • Infection
  • Line complications- haemorrhage, embolism, infection etc

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Flowchart

Always check a PCV with a free flowing venous sample. DO NOT rely on capillary sample results

Ensuring adequate enteral intake

  • Supplementation of breast feeds using formula should not be routinely prescribed.
  • In order to ensure adequate fluid intake, breastfeeding support should be prioritised and feeds observed in order to ensure that they are effective.
  • Mothers should be given information in order to ensure they are aware of the signs of adequate milk transfer and signs of good attachment including a reassuring suck-swallow pattern, and urine output and stooling appropriate to age.
  • Urine and stooling should be documented on the feed chart and output evaluated on a day to day basis.
  • Feed frequency should be monitored and documented, and mothers made aware that the baby should feed at least 8 times in a 24 hour period.
  • Where it is considered medically necessary to provide supplementation of breastfeeds, the use of mother’s breast milk should be prioritised above formula. Every attempt should be made to support mothers to express
  • Supplements should be administered via a nasogastric tube or cup. Teats should not be introduced to babies establishing on breastfeeds without documented parental informed consent

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Appendix A: RCT’s of PET vs routine care in polycythaemic babies

Citation

Study type

Study group

Intervention in polycythaemic babies

Long term Outcomes Assesed

Key results

Comments

Black

RCT

Randomized 93 “term” symptomatic and asymptomatic babies with PCV > 65% to PPET or routine care

Inclusion Criteria: clear
Randomization: clear
Blinded: unclear
PPET: FFP via umbilical vein to PCV of 50%

Follow up at 1 and 2 yrs with BSID and neurological assessment

62% 2 yr follow up
RR NDI at 2 yrs
1.34 (0.40, 4.49)
RR neuro diagnosis
0.45 (0.22, 0.93)

Increased rates of NEC PPET group
RR 11.18 (1.49, 83.64)

Bada

RCT

Randomized 28 asymptomatic babies with PCV >63% (gestation not stated) to PPET or routine care

Inclusion Criteria: clear
Randomization: clear
Blinded: yes
PPET: plasmanate to PCV 55%, route unclear.

Follow up at 30 months with BSID and neurological assessment.

67% 30 month follow up
RR NDI at 30 months
1.22 (0.51, 2.92)

 

Increased GI symptoms in PPET group.

Goldberg

RCT

Randomized 20 asymptomatic and symptomatic babies with PCV> 64 to PPET or routine care

Inclusion criteria: clear
Randomization:  unclear
Blinded:yes
PPET: FFP via umbilical vein to PCV 50%

BSID and Neurological assessment at 8 months

100% PPET, 60% routine care follow up at 8 months
No difference between groups for MDI or PDI scores.

 

Van der Elst

RCT

49 asymptomatic and symptomatic babies with PCV> 65% randomized to PPET or routine care.

Inclusion criteria: clear
Randomization: unclear
Blinded: yes
PPET: FFP via umbilical vein to PCV 50%

Neurological assessment at 8 months

86% follow up at 8 months
No difference between the groups in any area of development

Increased NEC seen in PPET group

Ratrisawadi

Quasi RCT

Alternately assigned 105 asymptomatic infants with PCV > 65% to PPET or routine care.

Inclusion Criteria: clear
Randomization: not truly random
Blinded: unclear
PPET: to PCV 60%, route not stated

Follow up at 18-24 months with Gassel development scales.

38% at 18-24 months
No difference seen in incidence of developmental delay

 

Kumar

RCT

45 asymptomatic babies with PCV >70% randomized to PPET or routine care.

Inclusion criteria:
Clear
Randomization: clear
Blinded: unclear
PPET: to PCV 55% with saline via peripheral route.

Follow up at 18 months with DDST-II

29% at 18 months
No difference in incidence of developmental delay.

 

Provenance

Record: 2160
Objective:
Clinical condition: Polycythaemia in the newborn baby
Target patient group: Neonates
Target professional group(s): Secondary Care Doctors
Adapted from:

Evidence base

Evidence base

  1. Bada HS, Korones SB, Pourcyrous M, et al. Asymptomatic syndrome of polycythaemic hyperviscosity: effect of partial plasma exchange tranfusion. Journal
    Pediatrics 1992;120:579–85.
  2. Sarkar S, Rosenkrantz TS. Neonatal polycythaemia and hyperviscosity. Seminars in fetal and neonatal medicine. 2008;13:248-55.
  3. Garcia-Prats JA. Neonatal polycythaemia. UpToDate accessed 24th May 2018.
  4. Oh W, Neonatal polycythaemia and hyperviscosity. Paediatric clinics of North America. 1986;33(3):523-32.
  5. Dempsey EM, Barrington K. Short and longterm outcomes following partial exchange transfusion in the polycythaemioc newborn: a systematic review. Archives of disease in childhood. 2006;91:F2-F6
  6. Ozek E, Soll R, Schimmel MS. Partial exchange transfusion to prevent neurodevelopmental disability in infants with polycythaemia. Cochrane Database of Systematic Reveiws 2010, Issue 1. Art No: CD005089. DOI:10.1002/14651858. CD005089.pub2
  7. Host A, Ulrich M. Late prognosis in untreated neonatal polycythaemia with minor or no symptoms. Acta Paediatrica 1982;71:629-633.
  8. Black VD, Lubchenco LO, Koops BL, et al. Neonatal hyperviscosity: randomized study of effect of partial plasma exchange transfusion on long-term outcome. Pediatrics 1985;75:1048–53.
  9. Van der Elst CW, Molteno CD, Malan AF, Heese H de V. The management of polycythaemia in the newborn infant. Eraly Human Development 1980, 4(4):393-403.
  10. Dempsey EM, Barrington K. Crystalloid or colloid for partial exchange transfusion in neonatal polycythaemia: A systematic review and metanalysis, Acta Paediatrica, 94:11;1650-1655

Approved By

Trust Clinical Guidelines Group

Document history

LHP version 1.0

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