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Pentalogy of Cantrell (PC) is a rare congenital anomaly involving defects in the anterior diaphragm, supraumbilical abdominal wall, diaphragmatic pericardium, and lower sternum, and other congenital intracardiac abnormalities. Here, we report the case of a newborn infant who was born at 32 weeks of gestation and had all 5 features of PC, in addition to absent kidneys and a deformed left hand. Medical intervention would not be able to save the patient, so we allowed her to die in peace. We discuss here the etiology, prenatal diagnosis, and severity of and the mortality associated with this condition. To our knowledge, this was the first reported case of PC in Saudi Arabia.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6074926 | PMC |
http://dx.doi.org/10.5144/0256-4947.2014.75 | DOI Listing |
BMJ Case Rep
March 2025
Department of Pediatric Surgery, All India Institute of Medical Sciences, Vijaypur, Jammu, Jammu, Jammu and Kashmir, India
A term neonate (TN) of 2 days, delivered by lower segment caesarean section in a private nursing home of a rural area, was referred to the outpatient department of paediatric surgery with a chief concern of large abdominal swelling and mild respiratory distress. The TN was diagnosed with giant omphalocele and a right diaphragmatic hernia.Intraoperatively, the omphalocele sac was found to contain liver, stomach, spleen and small intestine, with the latter herniating through a right diaphragmatic defect.
View Article and Find Full Text PDFJ Clin Ultrasound
February 2025
Department of Ultrasound Diagnosis, Chengxian Maternity and Child-Care Hospital, Longnan, Gansu Province, People's Republic of China.
Pentalogy of Cantrell (POC) is rare in prenatal diagnosis. The diagnosis of POC can be made antenatally through the use of ultrasonography, although this is challenging in cases where the defects are minor. We have reported a case diagnosis of incomplete POC through sections of the four-chamber view and the left ventricular outflow tract view.
View Article and Find Full Text PDFRadiol Case Rep
April 2025
University of Cape Coast, College of Health and Allied Sciences, Department of Imaging Technology & Sonography, Cape Coast, 1920382, Ghana.
This report presents a case of a male neonate diagnosed with a complete (Class I) PC characterized by a midline anterior wall defect with herniation of the heart and abdominal organs (ectopia cordis and omphalocele), along with diaphragmatic, sternal, and ventral abdominal wall defects consistent with PC. Additional anomalies included alobar holoprosencephaly, spina bifida, and clubfoot. The neonate was delivered via preterm cesarean section due to poor prognosis associated with this PC and other structural anomalies that were detected.
View Article and Find Full Text PDFJ Indian Assoc Pediatr Surg
November 2024
Consultant Paediatric Cardiologist, Medicover Woman and Child Hospitals, Madhapur, Hyderabad, Telangana, India.
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.
View Article and Find Full Text PDFCureus
September 2024
Department of Obstetrics and Gynecology, South Central Regional Medical Center, Laurel, USA.
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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