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Prune-Belly syndrome, a rare case presentation in neonatology: about one case in Yaounde, Cameroon

The Prune-Belly syndrome (PBS) is a rare pathology predominating in male infants, classically manifesting with the triad including aplasia of the abdominal wall muscles, dilatation of the urinary tract, and testicular abnormalities. We report and discuss the case of a full-term male newborn, in whom...

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Autores principales: Ngwanou, Dany Hermann, Ngantchet, Emmanuel, Moyo, Georges Pius Kamsu
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The African Field Epidemiology Network 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7406465/
https://www.ncbi.nlm.nih.gov/pubmed/32821313
http://dx.doi.org/10.11604/pamj.2020.36.102.24062
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author Ngwanou, Dany Hermann
Ngantchet, Emmanuel
Moyo, Georges Pius Kamsu
author_facet Ngwanou, Dany Hermann
Ngantchet, Emmanuel
Moyo, Georges Pius Kamsu
author_sort Ngwanou, Dany Hermann
collection PubMed
description The Prune-Belly syndrome (PBS) is a rare pathology predominating in male infants, classically manifesting with the triad including aplasia of the abdominal wall muscles, dilatation of the urinary tract, and testicular abnormalities. We report and discuss the case of a full-term male newborn, in whom clinical examination at birth revealed abdominal wall muscle hypoplasia, cryptorchidism, urinary tract dilatation and renal failure. The diagnosis was made based on physical assessment, abdominal ultra-sonographic imaging, and blood sampling of urea and creatinine. For such cases, the recommended surgical management usually consists in a sequential surgical intervention including urinary tract reconstruction, abdominoplasty, and orchidopexy. However, these could not be practiced in due time in our patient, who died on the seventh day of life because of kidney failure. The prognosis of infants with Prune-Belly syndrome may be improved by quality antenatal follow-up, to enable the early diagnosis and preparation for prompt surgical intervention.
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spelling pubmed-74064652020-08-19 Prune-Belly syndrome, a rare case presentation in neonatology: about one case in Yaounde, Cameroon Ngwanou, Dany Hermann Ngantchet, Emmanuel Moyo, Georges Pius Kamsu Pan Afr Med J Case Report The Prune-Belly syndrome (PBS) is a rare pathology predominating in male infants, classically manifesting with the triad including aplasia of the abdominal wall muscles, dilatation of the urinary tract, and testicular abnormalities. We report and discuss the case of a full-term male newborn, in whom clinical examination at birth revealed abdominal wall muscle hypoplasia, cryptorchidism, urinary tract dilatation and renal failure. The diagnosis was made based on physical assessment, abdominal ultra-sonographic imaging, and blood sampling of urea and creatinine. For such cases, the recommended surgical management usually consists in a sequential surgical intervention including urinary tract reconstruction, abdominoplasty, and orchidopexy. However, these could not be practiced in due time in our patient, who died on the seventh day of life because of kidney failure. The prognosis of infants with Prune-Belly syndrome may be improved by quality antenatal follow-up, to enable the early diagnosis and preparation for prompt surgical intervention. The African Field Epidemiology Network 2020-06-17 /pmc/articles/PMC7406465/ /pubmed/32821313 http://dx.doi.org/10.11604/pamj.2020.36.102.24062 Text en © Dany Hermann Ngwanou et al. https://creativecommons.org/licenses/by/4.0 The Pan African Medical Journal (ISSN: 1937-8688). This is an Open Access article distributed under the terms of the Creative Commons Attribution International 4.0 License (https://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Ngwanou, Dany Hermann
Ngantchet, Emmanuel
Moyo, Georges Pius Kamsu
Prune-Belly syndrome, a rare case presentation in neonatology: about one case in Yaounde, Cameroon
title Prune-Belly syndrome, a rare case presentation in neonatology: about one case in Yaounde, Cameroon
title_full Prune-Belly syndrome, a rare case presentation in neonatology: about one case in Yaounde, Cameroon
title_fullStr Prune-Belly syndrome, a rare case presentation in neonatology: about one case in Yaounde, Cameroon
title_full_unstemmed Prune-Belly syndrome, a rare case presentation in neonatology: about one case in Yaounde, Cameroon
title_short Prune-Belly syndrome, a rare case presentation in neonatology: about one case in Yaounde, Cameroon
title_sort prune-belly syndrome, a rare case presentation in neonatology: about one case in yaounde, cameroon
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7406465/
https://www.ncbi.nlm.nih.gov/pubmed/32821313
http://dx.doi.org/10.11604/pamj.2020.36.102.24062
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