The complete spectrum of pentalogy of Cantrell in one of a set of dizygotic twins: A case report of a rare congenital anomaly
We report a case of complete POC affecting only the first-born of a set of premature dizygotic twins.
Diagnosis:
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.
Interventions:
The patient underwent surgical intervention at 18 days of age.
Outcomes:
Despite adequate and appropriate postoperative treatment, the baby rapidly deteriorated and died 72 hours after surgery.
Lessons:
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.
Source: Medicine - Category: Internal Medicine Tags: Research Article: Clinical Case Report Source Type: research
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