Hydrops Foetalis - Diagnosis, Management and Investigation

Publication: 26/07/2018  --
Last review: 01/01/1900  
Next review: 26/07/2021  
Clinical Guideline
CURRENT 
ID: 5616 
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.

Hydrops Foetalis - Diagnosis, Management and Investigation

Summary of Guideline

Hydrops foetalis is a clinical diagnosis of an accumulation of fluid in two or more compartments of the developing foetus. There are many different causes, and often the baby will require extensive investigation.
Babies with hydrops foetalis can be very sick at delivery and so their stabilisation and on-going management is key.

Back to top

Background

Hydrops foetalis (HF) was first described in 1892. It is an accumulation of fluid in two or more compartments of the developing foetus. These abnormal fluid accumulations include: pleural effusions, pericardial effusions, ascites and skin oedema (often defined as >5mm). It can also be associated with polyhydramnios and placental thickening.

HF is, in itself, not a diagnosis but a symptom of an underlying pathology. There are two distinct forms of HF: immune and non-immune. Prior to the introduction of routine passive immunisation of Rhesus (Rh) negative mothers, the most common cause of HF was the breakdown of red cells due to Rh alloimmunisation. Since the widespread use of “Anti-D” immunoglobulin the number of babies who develop immune hydrops foetalis has fallen significantly. In the developed health systems NIHF accounts for >90% of the cases of HF, and for this reason our review will focus on NIHF.

NIHF occurs when the foetus becomes unable to manage fluid correctly. The main causes are:

  1. cardiovascular
  2. haematological (e.g. from thalassemia or infections causing anaemia)
  3. genetic/chromosomal
  4. idiopathic

The incidence of NIHF is thought to be between 1 in 1500 to 1 in 3800 births. The mortality of NIHF is approximately 81%. Once a hydropic infant is born, the main prognostic factors are gestation at birth and albumin level.

Back to top

Diagnosis

The diagnosis of NIHF is clinical and, in some cases, can be made antenatally, thus allowing time to prepare for the delivery, as outlined below. There are also cases when a baby will be delivered with features of HF without any indication antenatally.

Back to top

Investigation

Investigations are complex and diverse. It is important to elucidate the cause of the NIHF, both for the management of the case, but also for the management of any future pregnancies.

Antenatal Investigations
Where there is an antenatal concern regarding possible HF, women should be referred to tertiary Foetal Medicine for antenatal care and delivery of the infant.

Postnatal Investigations
This will depend in part upon the results of antenatal investigations; a probable diagnosis may already have been elucidated. Therefore not all babies will require all these investigations, but this may be used as a guide.

See “Investigation Sheet for Non-Immune Hydrops Foetalis”

Back to top

Treatment / Management

Treatment will depend to a degree on the underlying cause. However initial management and resuscitation, in conjunction with clear, two-way communication with the obstetric team, is key. Information concerning antenatal management, including in-utero transfusion, should be recorded in the baby’s notes.

Antenatally
If the diagnosis has been made antenatally, then the mother will have been referred to tertiary Foetal Medicine for management and extensive investigations.
Preterm delivery occurs more commonly in NIHF; if preterm labour is likely antenatal steroids should be offered.

Immediately prior to delivery
An incubator or platform should be set up ready. A ventilator should be prepared, bearing in mind that babies with HF often go on to require nitric oxide and sometimes high frequency oscillation.

On Delivery Suite
At minimum, the neonatal registrar, SHO and senior nurse should be in attendance. The consultant on-call should be present. As well as the standard equipment needed for neonatal resuscitation (the red 'emergency bag') there should be:

  • chest and ascitic drain equipment
  • central line equipment
  • Group O rhesus negative blood

Ask the midwife to obtain cord blood for:

  • Blood group
  • Direct Coomb's test (DCT)
  • Cord gases

In addition, request that at least 5cm of umbilical stump be left, to enable insertion of lines later.

When the baby is born, resuscitate as per the NLS algorithm, as required by the baby.
*Do not allow the baby to get cold.* Apply a hat promptly, ensure the resuscitaire heater is on and wrap the baby in warm towels.

AIRWAY
Due to potential laryngeal oedema, pleural effusions, RDS and cardiac failure, babies with HF are more likely to require endotracheal intubation. Laryngeal odema may make the intubation more technically challenging, therefore it may be advisable for a tier 2 person to have 1st attempt.

BREATHING
Ventilatory support is made more difficult by the presence of pleural effusions and/or ascites. High inspiratory pressures (>/=35cmH2O) may be required for adequate lung expansion.
If the insertion of chest and/or ascitic drains can wait, these should be inserted in the more controlled environment of the neonatal unit, and under sterile conditions.
However, it may be necessary to insert the drains on delivery suite, for example when there is poor chest movement despite adequate ventilatory pressures and a confirmed ETT position. To insert a chest drain, follow the process as outlined in the neonatal procedures guideline. Pleural fluid should be sent for analysis (see investigations sheet below).
There is evidence that NIHF affects surfactant production. This coupled with the fact that hydropic infants are more likely to deliver before term, makes respiratory distress syndrome (RDS) more likely. Surfactant should be given to infants in whom surfactant deficiency appears to play a role in their need for ventilatory support [evidence level D].

CIRCULATION
Central intravenous access is necessary for all hydropic infants, and should be ready for insertion at the delivery. It is preferable to insert a UVC (or alternative CVC) under sterile conditions on the neonatal unit if possible.  The baby may require fluid resuscitation, either with 0.9% sodium chloride, 4.5% HAS, or blood.

On the Neonatal Unit
After showing the baby to the parents and updating them, bring the baby promptly to the neonatal unit on the resuscitaire.
Babies with HF often require blood pressure support; please see the neonatal guideline on inotropes.

FLUIDS
It may be necessary to fluid restrict to 40ml/kg/day, discuss this with the consultant on-call.
A 20% human albumin (HAS) infusion with furosemide may be required. This can cause intravascular fluid depletion, and so should only be done following senior discussion.
Drain losses should be replaced, usually 2/3rds of the volume are replaced, using 4.5% HAS [evidence level D].

If there are risk factors for early-onset neonatal infection antibiotics should be commenced in line with NICE guidance on early neonatal infection. If the aetiology of the NIHF is still not known at birth, it is reasonable to commence antibiotics pending negative cultures and reassuring inflammatory markers, although there is no evidence to support their routine use in NIHF.

Once the infant has been stabilised the focus of the medical team should be to identify the cause of the NIHF.

Death
If the baby dies and the aetiology of the HF remains unknown, a post-mortem examination should be recommended. Post-mortem examinations should be offered sensitively and by a senior doctor, emphasising the potential benefits to the family that they know the exact aetiology of the NIHF. It may also be appropriate to ask for cells to be stored for future biochemical or genetic testing.

Back to top

Provenance

Record: 5616
Objective:

Aims

To improve the diagnosis and management of non-immune hydrops foetalis.

Objectives

To provide evidence-based recommendations for appropriate diagnosis, investigation and management of non-immune hydrops foetalis.

Clinical condition:

Hydrops Foetalis

Target patient group: Neonates
Target professional group(s): Secondary Care Nurses
Secondary Care Doctors
Adapted from:

Evidence base

References:

  1. Ballantyne JW. The Diseases and Deformities of the Fetus. Edinburgh: Oliver and Boyd, 1892.
  2. Am J Med Genet A. 2009 May;149A(5):844-51. doi: 10.1002/ajmg.a.32655.
    Etiology of nonimmune hydrops fetalis: a systematic review. Bellini C1, Hennekam RC, Fulcheri E, Rutigliani M, Morcaldi G, Boccardo F, Bonioli E.
  3. Am J Med Genet A. 2012 Mar;158A(3):597-605. doi: 10.1002/ajmg.a.34438. Epub 2012 Feb 2.
    Non-immune hydrops fetalis: a short review of etiology and pathophysiology.
    Bellini C1, Hennekam RC.
  4. Up to date Non-immune hydrops fetalis – (references 1-5)
  5. Acta Obstetricia Et Gynecologica Scandinavica [Acta Obstet Gynecol Scand] 2001 Aug; Vol. 80 (8), pp. 726-30.
  6. Am J Perinatol. 2007 Jan;24(1):33-8. Epub 2006 Dec 27. Prognostic factors and clinical features in liveborn neonates with hydrops fetalis. Huang HR1, Tsay PK, Chiang MC, Lien R, Chou YH.
  7. Obstet Gynecol. 1995 Apr;85(4):578-82. Non-immune hydrops after 20 weeks' gestation: review of 10 years' experience with suggestions for management. McCoy MC1, Katz VL, Gould N, Kuller JA.
  8. Hum Pathol. 1995 Nov;26(11):1252-9. Immunohistochemical distribution of surfactant apoproteins in hypoplastic lungs of nonimmunologic hydrops fetalis. Toki N1, Sueishi K, Minamitani M, Maeda H, Nakano H, Suzuki Y.
  9. National Institute for Health and Care Excellence. NICE guideline CG 149 published August 2012.

References  and Evidence levels:
A. Meta-analyses, randomised controlled trials/systematic reviews of RCTs
B. Robust experimental or observational studies
C. Expert consensus.
D. Leeds consensus. (where no national guidance exists or there is wide disagreement with a level C recommendation or where national guidance documents contradict each other)

Approved By

Trust Clinical Guidelines Group

Document history

LHP version 1.0

Related information

Not supplied

Equity and Diversity

The Leeds Teaching Hospitals NHS Trust is committed to ensuring that the way that we provide services and the way we recruit and treat staff reflects individual needs, promotes equality and does not discriminate unfairly against any particular individual or group.