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Antenatal diagnosis of intracranial haemorrhage and porencephalic cyst
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  1. T Williams1,
  2. A G Wilkinson2,
  3. J Kandasamy3,
  4. S Cooper1,
  5. J P Boardman4
  1. 1Simpson Centre for Reproductive Health, Royal Infirmary of Edinburgh, Edinburgh, UK
  2. 2Department of Radiology, Royal Hospital for Sick Children, Edinburgh, UK
  3. 3Department of Neurosurgery, Royal Hospital for Sick Children, Edinburgh, UK
  4. 4University of Edinburgh / MRC Centre for Reproductive Health, Edinburgh, UK
  1. Correspondence to Dr T Williams, thomascwilliams83{at}googlemail.com

https://doi.org/10.1136/bcr-2014-209130

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    Description

    A 27-year-old woman was referred at 37 weeks’ gestation for confirmation of malpresentation. Incidental hydrocephalus was found on ultrasound. Fetal MRI confirmed dilation of the lateral and third ventricles and an extensive mixed signal intensity cystic structure in the left hemisphere (7×6×4 cm) with evidence of blood products and restricted diffusion (figure 1A). The maternal platelet count was 114×109/L, and maternal antibody testing for neonatal alloimmune thrombocytopaenia (NAIT) was positive (anti-HPA (human platelet-specific antigen) 1a alloantibodies, maternal genotype HPA 1bb, paternal genotype HPA 1ab).

    Figure 1
    Figure 1

    (A) Antenatal MRI. (B) Postnatal MRI day 1.

    Subsequently a live female infant was delivered by caesarean section. At birth the infant had widened sutures and a petechial rash. The platelet count was 4×109/L and the infant received two HPA1a-negative platelet transfusions shortly after birth. MRI the next day confirmed posthaemorrhagic ventricular dilation and a large haemorrhagic parenchymal infarction with porencephalic cyst (figure 1B, arrow). She required a cystoventriculoperitoneal shunt at 4 weeks.

    NAIT is an IgG mediated disorder that occurs after maternal exposure to incompatible fetal paternally derived platelet antigens; it can occur in first pregnancies.1 Outcomes for NAIT with intracranial haemorrhage (ICH) are poor: registry data suggest that only 12% of children with ICH secondary to NAIT survive without significant neurodisability.2 Detailed antenatal imaging and testing for NAIT when fetal intracranial haemorrhage is suspected enables planned early treatment with matched platelets if the diagnosis is confirmed, which minimises the duration of postnatal profound thrombocytopenia and risk of further haemorrhage.

    References

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      . Neonatal alloimmune thrombocytopenia: pathogenesis, diagnosis and management. Br J Haematol 2013;161:3–14. doi:10.1111/bjh.12235
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      1. Tiller H,
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      . Fetal intracranial haemorrhages caused by fetal and neonatal alloimmune thrombocytopenia: an observational cohort study of 43 cases from an international multicentre registry. BMJ Open 2013;3: pii:e002490. doi:10.1136/bmjopen-2012-002490
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    Footnotes

    • Contributors TW devised idea for article, selected appropriate images and drafted the text. GW selected appropriate images for article and contributed to text. JK revised the text for important intellectual content. SC revised the text for important intellectual content. JPB devised idea for article, selected appropriate images and drafted the text. All authors approved the final submitted version.

    • Competing interests None.

    • Patient consent Obtained.

    • Provenance and peer review Not commissioned; externally peer reviewed.