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AuthorZvizdic, Zlatan
AuthorSefic-Pasic, Irmina
AuthorMesic, Amira
AuthorTerzic, Sabina
AuthorVranic, Semir
Available date2021-04-13T06:33:18Z
Publication Date2021-04-01
Publication NameMedicine
Identifierhttp://dx.doi.org/10.1097/MD.0000000000025470
CitationZvizdic, Zlatan MD, PhDa; Sefic-Pasic, Irmina MD, PhDb; Mesic, Amira MDc; Terzic, Sabina MD, PhDd; Vranic, Semir MD, PhDe,f,∗ The complete spectrum of pentalogy of Cantrell in one of a set of dizygotic twins, Medicine: April 09, 2021 - Volume 100 - Issue 14 - p e25470 doi: 10.1097/MD.0000000000025470
ISSN0025-7974
URIhttp://hdl.handle.net/10576/18219
AbstractPentalogy 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. We report a case of complete POC affecting only the first-born of a set of premature dizygotic twins. 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. The patient underwent surgical intervention at 18 days of age. Despite adequate and appropriate postoperative treatment, the baby rapidly deteriorated and died 72 hours after surgery. 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.
SponsorQatar National Library
Languageen
PublisherLippincott, Williams & Wilkins
Subjectpediatric pathology
Congenital syndromes
Pentalogy of Cantrell
TitleThe complete spectrum of pentalogy of Cantrell in one of a set of dizygotic twins: A case report of a rare congenital anomaly.
TypeArticle
Issue Number14
Volume Number100
ESSN1536-5964
dc.accessType Open Access


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