Pentalogy of Cantrell is a rare congenital anomaly characterized by a combination of severe defects in the middle of the chest and abdomen including intracardiac defects. Survival rate after cardiac surgery is extremely low. We present a successful staged complete repair of an omphalocele, a ventricular septal defect and a sternal defect in a case of pentalogy of Cantrell.
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Successful Surgical Repair of Complete Pentalogy of Cantrell.
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Pentalogy of Cantrell (PC) presents a distinctive challenge for clinicians and surgeons. In this case report, we have discussed the presentation, management, and literature review of a case of PC in a 17-month-old female child. The child was successfully managed with single-stage operation by a multidisciplinary team without any postoperative complications.
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Pentalogy of Cantrell is a rare congenital syndrome characterized by defects in the abdominal wall, sternum, diaphragm, and heart. A severe manifestation of this syndrome is ectopia cordis, where the heart is located partially or entirely outside the chest cavity. Gastroschisis involves a defect in the abdominal wall, where the intestines protrude outside the abdomen without a protective membrane.
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