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A Rare Case of Polysplenia Syndrome Associated with Severe Cardiac Malformations and Congenital Alveolar Dysplasia in a One-Month-Old Infant: A Complete Macroscopic and Histopathologic Study
Polysplenia syndrome represents a type of left atrial isomerism characterized by multiple small spleens, often associated with cardiac malformations and with situs ambiguus of the abdominal organs. The case presented is of a one-month-old male infant, weighing approximately 3000 g, born at the Count...
Autores principales: | , , , , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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MDPI
2022
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Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9144318/ https://www.ncbi.nlm.nih.gov/pubmed/35621846 http://dx.doi.org/10.3390/jcdd9050135 |
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author | Mohor, Cosmin Ioan Fleaca, Sorin Radu Oprinca Muja, Alexandra Oprinca, George Calin Roman, Mihai Dan Chicea, Radu Boicean, Adrian Gheorghe Dura, Horatiu Tanasescu, Ciprian Ion, Nicolas Catalin Ionut Faur, Mihai Bacila, Ciprian Ionut Batar, Florina Mohor, Calin Ilie |
author_facet | Mohor, Cosmin Ioan Fleaca, Sorin Radu Oprinca Muja, Alexandra Oprinca, George Calin Roman, Mihai Dan Chicea, Radu Boicean, Adrian Gheorghe Dura, Horatiu Tanasescu, Ciprian Ion, Nicolas Catalin Ionut Faur, Mihai Bacila, Ciprian Ionut Batar, Florina Mohor, Calin Ilie |
author_sort | Mohor, Cosmin Ioan |
collection | PubMed |
description | Polysplenia syndrome represents a type of left atrial isomerism characterized by multiple small spleens, often associated with cardiac malformations and with situs ambiguus of the abdominal organs. The case presented is of a one-month-old male infant, weighing approximately 3000 g, born at the County Clinical Emergency Hospital of Sibiu, who was hospitalized from birth until death. The patient suffered cardio-respiratory arrest due to severe hypoxia and septicemia on the background of a series of complex cardiac malformations associated with congenital abdominal organ anomalies. Examination of the body revealed a common atrium with complete atrioventricular canal defect, left ventricular hypertrophy, right ventricle hypoplasia, truncus arteriosus, superior vena cava duplication, bilobation of the lungs, situs ambiguous of the abdominal organs with right-sided stomach, a midline liver, gall bladder agenesis, multiple right-sided spleens and complete inversion of the intestines and pancreas. Histopathology concluded that the patient suffered cardiac lesions consistent with infantile lactic acidosis, as well as pulmonary modifications suggesting congenital alveolar dysplasia and altered hepatic architecture compatible with fibrosis. |
format | Online Article Text |
id | pubmed-9144318 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-91443182022-05-29 A Rare Case of Polysplenia Syndrome Associated with Severe Cardiac Malformations and Congenital Alveolar Dysplasia in a One-Month-Old Infant: A Complete Macroscopic and Histopathologic Study Mohor, Cosmin Ioan Fleaca, Sorin Radu Oprinca Muja, Alexandra Oprinca, George Calin Roman, Mihai Dan Chicea, Radu Boicean, Adrian Gheorghe Dura, Horatiu Tanasescu, Ciprian Ion, Nicolas Catalin Ionut Faur, Mihai Bacila, Ciprian Ionut Batar, Florina Mohor, Calin Ilie J Cardiovasc Dev Dis Case Report Polysplenia syndrome represents a type of left atrial isomerism characterized by multiple small spleens, often associated with cardiac malformations and with situs ambiguus of the abdominal organs. The case presented is of a one-month-old male infant, weighing approximately 3000 g, born at the County Clinical Emergency Hospital of Sibiu, who was hospitalized from birth until death. The patient suffered cardio-respiratory arrest due to severe hypoxia and septicemia on the background of a series of complex cardiac malformations associated with congenital abdominal organ anomalies. Examination of the body revealed a common atrium with complete atrioventricular canal defect, left ventricular hypertrophy, right ventricle hypoplasia, truncus arteriosus, superior vena cava duplication, bilobation of the lungs, situs ambiguous of the abdominal organs with right-sided stomach, a midline liver, gall bladder agenesis, multiple right-sided spleens and complete inversion of the intestines and pancreas. Histopathology concluded that the patient suffered cardiac lesions consistent with infantile lactic acidosis, as well as pulmonary modifications suggesting congenital alveolar dysplasia and altered hepatic architecture compatible with fibrosis. MDPI 2022-04-27 /pmc/articles/PMC9144318/ /pubmed/35621846 http://dx.doi.org/10.3390/jcdd9050135 Text en © 2022 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Case Report Mohor, Cosmin Ioan Fleaca, Sorin Radu Oprinca Muja, Alexandra Oprinca, George Calin Roman, Mihai Dan Chicea, Radu Boicean, Adrian Gheorghe Dura, Horatiu Tanasescu, Ciprian Ion, Nicolas Catalin Ionut Faur, Mihai Bacila, Ciprian Ionut Batar, Florina Mohor, Calin Ilie A Rare Case of Polysplenia Syndrome Associated with Severe Cardiac Malformations and Congenital Alveolar Dysplasia in a One-Month-Old Infant: A Complete Macroscopic and Histopathologic Study |
title | A Rare Case of Polysplenia Syndrome Associated with Severe Cardiac Malformations and Congenital Alveolar Dysplasia in a One-Month-Old Infant: A Complete Macroscopic and Histopathologic Study |
title_full | A Rare Case of Polysplenia Syndrome Associated with Severe Cardiac Malformations and Congenital Alveolar Dysplasia in a One-Month-Old Infant: A Complete Macroscopic and Histopathologic Study |
title_fullStr | A Rare Case of Polysplenia Syndrome Associated with Severe Cardiac Malformations and Congenital Alveolar Dysplasia in a One-Month-Old Infant: A Complete Macroscopic and Histopathologic Study |
title_full_unstemmed | A Rare Case of Polysplenia Syndrome Associated with Severe Cardiac Malformations and Congenital Alveolar Dysplasia in a One-Month-Old Infant: A Complete Macroscopic and Histopathologic Study |
title_short | A Rare Case of Polysplenia Syndrome Associated with Severe Cardiac Malformations and Congenital Alveolar Dysplasia in a One-Month-Old Infant: A Complete Macroscopic and Histopathologic Study |
title_sort | rare case of polysplenia syndrome associated with severe cardiac malformations and congenital alveolar dysplasia in a one-month-old infant: a complete macroscopic and histopathologic study |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9144318/ https://www.ncbi.nlm.nih.gov/pubmed/35621846 http://dx.doi.org/10.3390/jcdd9050135 |
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