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Images in neonatal medicine
Bilateral chest wall mesenchymal hamartomas masquerading as fetal hydrops in utero
  1. Christopher Michael Faraday1,
  2. Clifford B D Mayes2,
  3. Daniel Hurrell3,
  4. Bharathi Rao4
  1. 1 Neonatal Intensive Care Unit, Royal Jubilee Maternity Hospital, Belfast, UK
  2. 2 Neonatal Intensive Care Unit, Belfast HSC Trust, Belfast, UK
  3. 3 Pathology Department, Alder Hey Children's Hospital, Liverpool, Merseyside, UK
  4. 4 Neonatal Intensive Care Unit, Royal Jubilee Maternity Service, Belfast, Belfast, UK
  1. Correspondence to Dr Christopher Michael Faraday, Royal Jubilee Maternity Service, Belfast BT12 6BA, UK; c.faraday{at}yahoo.co.uk

https://doi.org/10.1136/archdischild-2019-318649

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    A female infant born by spontaneous vaginal delivery at 35 weeks of gestation with expected fetal hydrops, due to presumed pleural effusions on antenatal ultrasound, presented with difficult and prolonged resuscitation. The neonatal team attended delivery due to the presumed hydrops, and the infant required immediate intubation as attempts were made to aspirate intrapleural fluid, though these were unsuccessful. On arrival to the neonatal unit at approximately 40 min of life, her pH was 6.87, and so intensive care was discontinued and the infant died at 45 min of life.

    Postmortem examination revealed bilateral mesenchymal hamartomas of the chest wall, which had resulted in lethal pulmonary hypoplasia (figure 1A); there were also chorioamnionitis and two-vessel funisitis. Externally, the infant was appropriately grown and displayed no dysmorphic features. Karyotype and microarray detected no abnormality.

    Figure 1
    Figure 1

    Neonatal chest wall hamartomas shown on postmortem, once the lungs were resected, occupying the left and right hemithorax (A) and appearing in the left hemithorax on chest radiograph, with chest wall deformation (B).

    Mesenchymal hamartomas are rare, benign tumours that develop during fetal life and may be asymptomatic1 but may also present in neonatal life with respiratory distress and marked deformity of the chest wall. They have distinct histopathological characteristics composed of normal skeletal elements that display a dysregulated propensity to proliferate, but no tendency to invade surrounding tissues.2 Radiologically, they appear as well-defined, partially ossified masses arising from the ribs (figure 1B). Treatment is most commonly by surgical excision because malignant transformation, though rare, has been described.3

    Although these benign tumours are rare, they should be considered in cases where there are abnormal intrathoracic inclusions, especially antenatally when the nature of such findings may inform further imaging and parental counselling.

    References

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    Footnotes

    • Contributors CMF reviewed the case notes and the literature and wrote the manuscript. DH performed and reported on the postmortem findings. CBDM was the lead consultant dealing with patient care and sought written consent from parents. BR supervised and proofread the manuscript.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Competing interests None declared.

    • Patient consent for publication Parental/guardian consent obtained.

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