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Two cases of maternal antenatal splenic rupture and hypotension associated with Moebius syndrome and cerebral palsy in offspring

Further evidence for a utero placental vascular aetiology for the Moebius syndrome and some cases of cerebral palsy

  • Medical Genetic
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Abstract

Abstract

We wish to report two cases of congenital abnormality after antenatal car accidents resulting in ruptured spleen and severe hypotension in the mothers at 8 and 14 weeks gestation. The first case had the classical Moebius syndrome with 6th and 7th cranial nerve palsy with abnormal brain stem evoked responses, presumably due to hypoxic/ischaemic brain stem damage and the second case had severe retardation and hypertonic cerebral palsy which at post mortem was found to be due to old hypoxic/ischaemic lesions to the caudate nucleus putamen and striatum.

Conclusion

The cases described provide evidence that severe maternal hypotension during pregnancy can be associated with lesions to the midbrain and brain stem of offspring. The mechanism is probably uteroplacental insufficiency, and extrapolation from these two unusual cases would support utero-placental insufficiency as a cause of Moebius syndrome and limb deficiency after chorionic villus sampling.

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Authors and Affiliations

  1. Department of Clinical Genetics, The New Children's Hospital, Parramatta, PO Box 3515, 2124, Sydney, NSW, Australia

    A. H. Lipson

  2. Centre for Medical Genetics, Institut et de Genetique, B-6280, Loverval, Belgium

    Y. Gillerot

  3. Department of Neurology, Centre Hospitalier Universitaire Tivoli, B-7011, La Louviere, Belgium

    S. Giurgea

  4. Department of Neuropathology, Mater Misericordiae Hospital, QLD 4104, South Brisbane, Australia

    A. E. G. Tannenberg

Authors
  1. A. H. Lipson
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  2. Y. Gillerot
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  3. A. E. G. Tannenberg
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  4. S. Giurgea
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Lipson, A.H., Gillerot, Y., Tannenberg, A.E.G. et al. Two cases of maternal antenatal splenic rupture and hypotension associated with Moebius syndrome and cerebral palsy in offspring. Eur J Pediatr 155, 800–804 (1996). https://doi.org/10.1007/BF02002911

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  • DOI: https://doi.org/10.1007/BF02002911

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