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Virtual Pediatric Hospital: Correlapaedia - a Correlative Encyclopedia of Pediatric Imaging, Surgery, and Pathology: Case 25

Correlapaedia - a Correlative Encyclopedia of Pediatric Imaging, Surgery, and Pathology

Case 25

Michael P. D'Alessandro, M.D.,
Steven J. Fishman, M.D.,
Deborah E. Schofield, M.D.

Peer Review Status: Internally Peer Reviewed
Chief Complaint:
Newborn full term female with an anterior chest and abdominal midline defect.

Clinical History:
This newborn full term female was noted to have ascites on her prenatal ultrasound. During her vaginal delivery, the abdomen could not be delivered due to its large size. Delivery was finally accomplished after removing 200 cc of ascites from the baby by placing a needle through the abdominal wall and uterus of the mother.

Clinical Physical Exam:
Upon delivery the baby was noted to have a midline defect including a split anterior half of the sternum, an omphalocele and a thoracoabdominal ectopic cordis.

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Clinical Labs:

Clinical Differential Diagnosis:
Pentalogy of Cantrell

Imaging Findings:
A chest and abdominal film from the first day of life demonstrated the ectopia cordis and a paucity of bowel gas in the abdomen. Abdominal US from the second day of life (not provided) demonstrated massive ascites, and no hepatic lesions were seen. An echocardiogram noted the presence of double outlet right ventricle.

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Imaging Differential Diagnosis:
Ectopia cordis

Operative Findings:
On the second day of life the patient was taken to the operating room for closure of the midline defect. The midline defect extended from just below the umbilicus to halfway up the heart and was a few centimeters wide. The omphalocele sac was excised revealing the apex of the heart and the liver. The pericardium was intact. The diaphragm was intact. The omphalocele was closed by approximating the abdominal fascia. The heart was covered with skin.

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Pathological Findings:

Final Diagnosis:
Pentalogy of Cantrell

Follow-up and Prognosis:
The patient had an uncomplicated post-operative course. A chest CT was performed at 2 weeks of life and demonstrated a very hypoplastic appearing anterior chest wall and a stenosis of the left mainstem bronchus as the cause of the patient's left lung hyperinflation. This was confirmed by endoscopy 3 days later which showed this compression to be due to a pulsating vessel.

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Similar Cases:
Case 3, Case 19, Case 52


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