Correlapaedia - a Correlative Encyclopedia of Pediatric Imaging, Surgery, and Pathology
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.
Clinical Differential Diagnosis:
Pentalogy of Cantrell
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.
Imaging Differential Diagnosis:
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.
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.
Images 6 and 7
Case 3, Case 19, Case 52
Follow us on Twitter @pedseducation and @pedsimaging and Facebook @pedsimaging and Instagram @pedsimaging
Please send us comments by filling out our Comment Form.
All contents copyright © 1992-2016 Donna M. D'Alessandro, M.D. and Michael P. D'Alessandro, M.D. and the authors. All rights reserved.
"Virtual Pediatric Hospital", the Virtual Pediatric Hospital logo, and "A digital library of pediatric information" are all Trademarks of Donna M. D'Alessandro, M.D. and Michael P. D'Alessandro, M.D.
Virtual Pediatric Hospital is funded in whole by Donna M. D'Alessandro, M.D. and Michael P. D'Alessandro, M.D. Advertising is not accepted.
Your personal information remains confidential and is not sold, leased, or given to any third party be they reliable or not.
The information contained in Virtual Pediatric Hospital is not a substitute for the medical care and advice of your physician. There may be variations in treatment that your physician may recommend based on individual facts and circumstances.