Non-Immune Hydrops

 

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Fluid in two body cavities or one cavity plus oedema at birth. The prognosis depends on associated prematurity, the underlying cause, the severity of any associated pulmonary hypoplasia and the severity of the ongoing post-natal fluid accumulation (with problems of infection and malnutrition). Mortality is still up to 70%. 1

Resuscitation

Resuscitation and stabilisation is often difficult. It may be necessary to drain pleural effusions in the delivery room, at the same time as resuscitating the baby. Find out about the size of pleural effusions and severity of the hydrops from obstetric staff before delivery. It is important to prepare equipment before delivery 2-3.

Click here to see images of hydropic infants.

Associations with Non-Immune Hydrops

Cardiovascular
  • SVT
  • Heart block
  • Truncus arteriosus
  • Coxsackie myocarditis
  • Hypoplastic Left Heart Syndrome
  • Endocardial fibroelastosis
  • VSD/AV canal
  • Premature closure of foramen ovale
  • Premature closure of PDA
  • Tumours (rhabdomyomas)
  • Arterial calcification
  • Cardiomyopathy (e.g. carnitine deficiency)
  • AV malformations
  • Any cause of heart failure
Chromosomal
  • Trisomy 21
  • Triploidy
  • 45XO (Turner's)
  • Many others reported
Dysmorphic Syndromes
 
Neurological
  • Encephalocoele
  • Agenesis of the corpus callosum
  • Tuberous sclerosis
  • Vein of Galen aneurysm
  • Arthrogryposis
Gastrointestinal
  • Jejunal atresia
  • Midgut volvolus
  • Meconium peritonitis
  • Hepatitic fibrosis
  • Hepatic vascular malformations
  • Familial cirrhosis and portal hypertension
Genitourinary
  • Congenital nephrotic syndrome
  • Urethral obstruction and renal dysplasia
  • Polycystic kidneys
  • Renal vein obstruction
  • Vaginal and uterine abnormalities
Haematological
  • Twin-twin transfusion
  • Rhesus isoimmunisation
  • Feto-maternal haemorrhage
  • α-thalassaemia (homozygous)
  • Fetal anaemia or blood loss
  • G6PD deficiency
  • Pyruvate kinase deficiency
Lymphatic
  • Chylothorax
  • Congenital lymphangectasia
  • Cystic hygroma of neck
  • Noonan's Syndrome
Infective
  • Parvovirus
  • CMV
  • Toxoplasma
  • Syphilis
  • Leptospirosis
  • Chagas Disease
  • Congenital hepatitis
  • Rubella
  • Herpes simplex
  • Varicella
Respiratory
  • Diaphragmatic hernia
  • Cystic adenomatoid malformation
  • Hamartoma
  • Tracheo-oesophageal fistula
  • Atresia of right main bronchus
  • Sequestration
  • Pulmonary lymphangiectasia
  • Mediastinal teratoma
Other
  • Retroperitoneal fibrosis
Skeletal
  • Osteogenesis imperfecta
  • Asphyxiating thoracic dystrophy
  • Thanatophoric dwarfism
  • Achondrogenesis
  • Hyperphosphatasia
  • Saldino-Noonan dwarfism

Tumours

  • Teratoma
  • Neuroblastoma
  • Haemangioma
Placental/Umbilical
  • True knot
  • UV thrombosis
  • Placental chorioangioma
  • UA aneurysm
Maternal
  • Diabetes
  • Preeclampsia
  • Drugs (i.e. indomethacin)
Metabolic
  • Gaucher's Disease
  • GM1 gangliosidosis
  • Hurler's Syndrome (MP 1H)
  • Morquio (MP IVb)
  • MP type VII
  • Mucolipidosis type I and II
  • Sialic acid storage disease
  • Galactosialidosis

This list is not comprehensive! Also it does not give an idea of how common conditions may be.

Investigations

Many of these may have been done antenatally. Particular clinical findings may indicate other investigations for aetiology. Target investigations at clinical features. Collect cord blood EDTA and clotted samples. Up to 50% of non-immune hydrops remain unexplained after full investigation.

Anaemia
  • Evidence of fetal anaemia.
  • Maternal blood group and antibodies.
  • Baby blood group and Coombs.
  • Early haemoglobin/PCV.
  • Maternal Kleihauer
Biochemistry
  • Liver function including albumin/protein
  • Renal function.
Cardiac rhythm in utero
  • Evidence from ultrasound scans and CTGs.
  • Post-natal ECG + monitoring.
Fluid examination
  • Protein and albumin
  • Cell cytology (commonly finding marked lymphocytosis).
  • Triglyceride levels after feeding started.
Placenta
  • Macroscopic examination, histology and Toxoplasma PCR.
Ultrasound
  • Head, heart, chest, abdomen.
X-rays
  • Chest, abdomen and long bones (skeletal abnormalities and congenital infection).
  • Further CT/MRI as indicated by clinical course and other results.
Infections
  • Maternal or baby evidence of infections listed above.
Chromosomes  
Hb electrophoresis  
Metabolic testing
  • Use family history as a guide. 
  • Look for features of possible conditions before launching into investigations for specific conditions.

Infective causes

Parvovirus
  • Fetal anaemia that may have recovered
  • PCR, IgG and IgM titres
  • Send baby/cord serum.
CMV
  • Urine culture/PCR.
  • Serum for CMV PCR.
Toxoplasma
  • Maternal and baby blood, placenta and amniotic fluid PCR
  • Baby/cord IgM.
Syphilis
  • Maternal serology (VDRL)
  • Baby/cord serology.
Congenital hepatitis
  • Maternal hepatitis B serology
  • Baby LFTs and liver US.
Rubella
  • Maternal serology before pregnancy
  • Urine ± CSF PCR.
  • WBC for rubella PCR
  • Serum IgM.
Herpes simplex
  • WBC PCR.
Varicella
  • Other features of congenital varicella.

Look for supportive evidence with long bone X-rays, cerebral US/CT and ophthalmic exam.

References

1 Fraser SH. Non-immune hydrops: no longer an automatic death sentence. Australia and NZ Perinatal Society. Perth 1997.
2 Stephenson T et al. Diagnosis and management of non-immune hydrops. Arch Dis Child 1994; 70: F151-4
3 Jones DC. Diagnosis and management of nonimmune hydrops. P452-61