Limb body wall complex – the history of the entity and presentation of our series of cases

Jacek Gulczyński; Małgorzata Świątkowska-Freund; Piotr Paluchowski; Blanka Hermann-Okoniewska; Ewa Iżycka-Świeszewska

doi:10.5114/pjp.2019.84460

eISSN: 2084-9869
ISSN: 1233-9687

Polish Journal of Pathology

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1/2019
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abstract:

Original paper

Limb body wall complex – the history of the entity and presentation of our series of cases

Jacek Gulczyński

¹

,

Małgorzata Świątkowska-Freund

²

,

Piotr Paluchowski

³

,

Blanka Hermann-Okoniewska

^{1, 4}

,

Ewa Iżycka-Świeszewska

^{1, 4}

Department of Pathology and Neuropathology, Medical University of Gdansk, Poland
Department of Obstetrics, Medical University of Gdansk, Poland
Department of History and Philosophy of Medical Sciences, Medical University of Gdansk, Poland
Department of Pathomorphology, Copernicus Hospital, Gdansk, Poland

Pol J Pathol 2019; 70 (1): 33-41

DOI: https://doi.org/10.5114/pjp.2019.84460

Online publish date: 2019/04/24

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We present an analysis of two first historically documented limb body wall complex (LBWC) cases and our own contemporary perinatal autopsy series of this rare complex. So far it was supposed that the first case of this complex was reported in 1685 by Paul Portal. Studying the Joachim Oelhaf’s autopsy report from 1613 with attached engraving showing the neonate with multiple birth defects led our research team to a conclusion that it was genuinely the first description of LBWC in the medical literature so far. We compared the Oelhaf’s case from 1613 and the Portal’s autopsy report from 1685 with our series of LBWC cases dissected in the Medical University of Gdansk between 1999 and 2011. Reviewing 1100 autopsy reports performed we encountered 9 cases of this unique complex. The analysis was supported by the literature review.

keywords:

Limb body wall complex – the history of the entity and presentation of our series of cases

Jacek Gulczyński 1 , Małgorzata Świątkowska-Freund 2 , Piotr Paluchowski 3 , Blanka Hermann-Okoniewska 1, 4 , Ewa Iżycka-Świeszewska 1, 4

LBWC, autopsy, teratology, birth-defects, amniotic band syndrome

Jacek Gulczyński

¹

,

Małgorzata Świątkowska-Freund

²

,

Piotr Paluchowski

³

,

Blanka Hermann-Okoniewska

^{1, 4}

,

Ewa Iżycka-Świeszewska

^{1, 4}