The complete spectrum of pentalogy of Cantrell in one of a set of dizygotic twins
<h3>Rationale</h3><p dir="ltr">Pentalogy of Cantrell (POC) is an extremely rare syndrome with an estimated incidence of 1:65,000 to 200,000 live births. Its complete form includes a midline epigastric abdominal wall defect, defects affecting the lower sternum, anterior di...
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2021
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| author | Zlatan Zvizdic (9212371) |
| author2 | Irmina Sefic-Pasic (17563257) Amira Mesic (16515279) Sabina Terzic (20402234) Semir Vranic (3353012) |
| author2_role | author author author author |
| author_facet | Zlatan Zvizdic (9212371) Irmina Sefic-Pasic (17563257) Amira Mesic (16515279) Sabina Terzic (20402234) Semir Vranic (3353012) |
| author_role | author |
| dc.creator.none.fl_str_mv | Zlatan Zvizdic (9212371) Irmina Sefic-Pasic (17563257) Amira Mesic (16515279) Sabina Terzic (20402234) Semir Vranic (3353012) |
| dc.date.none.fl_str_mv | 2021-04-09T03:00:00Z |
| dc.identifier.none.fl_str_mv | 10.1097/md.0000000000025470 |
| dc.relation.none.fl_str_mv | https://figshare.com/articles/journal_contribution/The_complete_spectrum_of_pentalogy_of_Cantrell_in_one_of_a_set_of_dizygotic_twins/27991595 |
| dc.rights.none.fl_str_mv | CC BY 4.0 info:eu-repo/semantics/openAccess |
| dc.subject.none.fl_str_mv | Biological sciences Genetics Biomedical and clinical sciences Pharmacology and pharmaceutical sciences congenital anomalies diagnosis pentalogy of Cantrell treatment twins |
| dc.title.none.fl_str_mv | The complete spectrum of pentalogy of Cantrell in one of a set of dizygotic twins |
| dc.type.none.fl_str_mv | Text Journal contribution info:eu-repo/semantics/publishedVersion text contribution to journal |
| description | <h3>Rationale</h3><p dir="ltr">Pentalogy of Cantrell (POC) is an extremely rare syndrome with an estimated incidence of 1:65,000 to 200,000 live births. Its complete form includes a midline epigastric abdominal wall defect, defects affecting the lower sternum, anterior diaphragm, diaphragmatic pericardium, and various intracardiac defects.</p><h3>Patient concerns</h3><p dir="ltr">We report a case of complete POC affecting only the first-born of a set of premature dizygotic twins.</p><h3>Diagnosis</h3><p dir="ltr">A giant omphalocele with an eviscerated liver and bowel on prenatal, obstetric ultrasonography at 24 gestational weeks was observed. At birth, physical examination confirmed a massive (10 × 8 cm) epigastric omphalocele in which a significant part of the liver was seen. A postnatal echocardiogram revealed the presence of an ostium secundum atrial septal defect, perimembranous ventricular septal defect, and moderate pulmonary stenosis. X-ray showed an abnormal intrathoracic positioned stomach, which was confirmed with a plain x-ray of the upper intestinal tract with hydrosoluble contrast. Computed tomography (CT) scan revealed the sternum's absence and a close connection between the pericardial sac and the stomach wall.</p><h3>Interventions</h3><p dir="ltr">The patient underwent surgical intervention at 18 days of age.</p><h3>Outcomes</h3><p dir="ltr">Despite adequate and appropriate postoperative treatment, the baby rapidly deteriorated and died 72 hours after surgery.</p><h3>Lessons</h3><p dir="ltr">POC is a complex, high-mortality syndrome whose management requires a multidisciplinary approach and meticulous planning. Despite all efforts, POC carries a poor prognosis, particularly in patients affected by its complete form.</p><h2>Other Information</h2><p dir="ltr">Published in: Medicine<br>License: <a href="http://creativecommons.org/licenses/by/4.0" target="_blank">http://creativecommons.org/licenses/by/4.0</a><br>See article on publisher's website: <a href="https://dx.doi.org/10.1097/md.0000000000025470" target="_blank">https://dx.doi.org/10.1097/md.0000000000025470</a></p> |
| eu_rights_str_mv | openAccess |
| id | Manara2_131e80c74a7080e5df2649e2558e7a09 |
| identifier_str_mv | 10.1097/md.0000000000025470 |
| network_acronym_str | Manara2 |
| network_name_str | Manara2 |
| oai_identifier_str | oai:figshare.com:article/27991595 |
| publishDate | 2021 |
| repository.mail.fl_str_mv | |
| repository.name.fl_str_mv | |
| repository_id_str | |
| rights_invalid_str_mv | CC BY 4.0 |
| spelling | The complete spectrum of pentalogy of Cantrell in one of a set of dizygotic twinsZlatan Zvizdic (9212371)Irmina Sefic-Pasic (17563257)Amira Mesic (16515279)Sabina Terzic (20402234)Semir Vranic (3353012)Biological sciencesGeneticsBiomedical and clinical sciencesPharmacology and pharmaceutical sciencescongenital anomaliesdiagnosispentalogy of Cantrelltreatmenttwins<h3>Rationale</h3><p dir="ltr">Pentalogy of Cantrell (POC) is an extremely rare syndrome with an estimated incidence of 1:65,000 to 200,000 live births. Its complete form includes a midline epigastric abdominal wall defect, defects affecting the lower sternum, anterior diaphragm, diaphragmatic pericardium, and various intracardiac defects.</p><h3>Patient concerns</h3><p dir="ltr">We report a case of complete POC affecting only the first-born of a set of premature dizygotic twins.</p><h3>Diagnosis</h3><p dir="ltr">A giant omphalocele with an eviscerated liver and bowel on prenatal, obstetric ultrasonography at 24 gestational weeks was observed. At birth, physical examination confirmed a massive (10 × 8 cm) epigastric omphalocele in which a significant part of the liver was seen. A postnatal echocardiogram revealed the presence of an ostium secundum atrial septal defect, perimembranous ventricular septal defect, and moderate pulmonary stenosis. X-ray showed an abnormal intrathoracic positioned stomach, which was confirmed with a plain x-ray of the upper intestinal tract with hydrosoluble contrast. Computed tomography (CT) scan revealed the sternum's absence and a close connection between the pericardial sac and the stomach wall.</p><h3>Interventions</h3><p dir="ltr">The patient underwent surgical intervention at 18 days of age.</p><h3>Outcomes</h3><p dir="ltr">Despite adequate and appropriate postoperative treatment, the baby rapidly deteriorated and died 72 hours after surgery.</p><h3>Lessons</h3><p dir="ltr">POC is a complex, high-mortality syndrome whose management requires a multidisciplinary approach and meticulous planning. Despite all efforts, POC carries a poor prognosis, particularly in patients affected by its complete form.</p><h2>Other Information</h2><p dir="ltr">Published in: Medicine<br>License: <a href="http://creativecommons.org/licenses/by/4.0" target="_blank">http://creativecommons.org/licenses/by/4.0</a><br>See article on publisher's website: <a href="https://dx.doi.org/10.1097/md.0000000000025470" target="_blank">https://dx.doi.org/10.1097/md.0000000000025470</a></p>2021-04-09T03:00:00ZTextJournal contributioninfo:eu-repo/semantics/publishedVersiontextcontribution to journal10.1097/md.0000000000025470https://figshare.com/articles/journal_contribution/The_complete_spectrum_of_pentalogy_of_Cantrell_in_one_of_a_set_of_dizygotic_twins/27991595CC BY 4.0info:eu-repo/semantics/openAccessoai:figshare.com:article/279915952021-04-09T03:00:00Z |
| spellingShingle | The complete spectrum of pentalogy of Cantrell in one of a set of dizygotic twins Zlatan Zvizdic (9212371) Biological sciences Genetics Biomedical and clinical sciences Pharmacology and pharmaceutical sciences congenital anomalies diagnosis pentalogy of Cantrell treatment twins |
| status_str | publishedVersion |
| title | The complete spectrum of pentalogy of Cantrell in one of a set of dizygotic twins |
| title_full | The complete spectrum of pentalogy of Cantrell in one of a set of dizygotic twins |
| title_fullStr | The complete spectrum of pentalogy of Cantrell in one of a set of dizygotic twins |
| title_full_unstemmed | The complete spectrum of pentalogy of Cantrell in one of a set of dizygotic twins |
| title_short | The complete spectrum of pentalogy of Cantrell in one of a set of dizygotic twins |
| title_sort | The complete spectrum of pentalogy of Cantrell in one of a set of dizygotic twins |
| topic | Biological sciences Genetics Biomedical and clinical sciences Pharmacology and pharmaceutical sciences congenital anomalies diagnosis pentalogy of Cantrell treatment twins |