VP15.11: Pentalogy of Cantrell: a rare, not‐to‐be‐missed pathology in the first trimester

Pentalogy of Cantrell (POC) is a rare condition characterized by complete or partial expression of 5 types of congenital birth defects originating in (1) the lower sternum, (2) the anterior diaphragm, (3) the diaphragmatic pericardium, (4) the midline/ventral supraumbilical abdominal wall region, and (5) the heart. POC can be diagnosed antenatally by means of ultrasonography (US), the task being difficult if defects are minor. Advances in the field of US and the introduction of the first morphology trimester as state-of-the-art pregnancy monitoring, facilitate the early diagnosis of this condition. We performed a systematic review on 67 reported cases of POC diagnosed in the first trimester of pregnancy (published from January 1980 to July 2019). The aim of our systematic review was twofold: to assess the main US findings in the first trimester of pregnancy and to increase awareness of early diagnostic possibilities. Our study showed that POC can be diagnosed in the first trimesterof pregnancy based on key US findings such as the association between omphalocele and ectopia cordis. When these two anomalies are present, increased nuchal translucency can also be considered a marker of POC in the first trimester.

Download Full-text

Pentalogy of Cantrell diagnosed in the first trimester of pregnancy

International Journal of Case Reports and Images ◽

10.5348/ijcri-2014-03-476-cr-7 ◽

2013 ◽

Vol 5 (3) ◽

pp. 215 ◽

Cited By ~ 1

Author(s):

Yasemin Cekmez ◽

Tülay Tos ◽

Zehra Yilmaz ◽

Nilay Pişkinpaşa ◽

Tuncay Küçüközkan

Keyword(s):

First Trimester ◽

Pentalogy Of Cantrell ◽

First Trimester Of Pregnancy

Download Full-text

Pentalogy of Cantrell: Diagnosis in the first trimester

Journal of Obstetrics and Gynaecology ◽

10.1080/01443610500335795 ◽

2005 ◽

Vol 25 (8) ◽

pp. 812-813 ◽

Cited By ~ 9

Author(s):

P. Sarkar ◽

J. Bastin ◽

D. Katoch ◽

A. Pal

Keyword(s):

First Trimester ◽

Pentalogy Of Cantrell

Download Full-text

Pentalogy of Cantrell: An extremely rare congenital anomaly diagnosis in the first trimester screening

Archives of Cardiovascular Diseases Supplements ◽

10.1016/j.acvdsp.2021.06.100 ◽

2021 ◽

Vol 13 (4) ◽

pp. 332-333

Author(s):

Fathi Mraihi ◽

Amal Badri ◽

Jihene Basli ◽

Dalenda Chelli

Keyword(s):

Congenital Anomaly ◽

First Trimester ◽

First Trimester Screening ◽

Pentalogy Of Cantrell ◽

Rare Congenital Anomaly ◽

Trimester Screening

Download Full-text

Pentalogy of Cantrell: A Report of One Case at the Yaounde Central Hospital in Cameroon

Donald School Journal of Ultrasound in Obstetrics & Gynecology ◽

10.5005/jp-journals-10009-1264 ◽

2012 ◽

Vol 6 (4) ◽

pp. 412-414

Author(s):

Jeanne Hortence Fouedjio ◽

Florent Fouelifack Ymele ◽

Bruno Kenfack ◽

Mandana Mehta ◽

Robinson Enow Mbu

Keyword(s):

Obstet Gynecol ◽

Abdominal Wall Defect ◽

First Trimester ◽

Central Hospital ◽

Pentalogy Of Cantrell ◽

Wall Defect ◽

Malformation Syndrome ◽

Congenital Cardiac Anomalies ◽

First Trimester Of Pregnancy ◽

Congenital Malformation Syndrome

ABSTRACT Pentalogy of Cantrell is a rare multiple congenital malformation syndrome characterized by a combination of five features: A midline supraumbilical abdominal wall defect; a defect of the lower sternum; a defect of the diaphragmatic pericardium; deficiency of the anterior diaphragm and congenital cardiac anomalies. These defects can be diagnosed as early as the first trimester of pregnancy. The complexity of these anomalies, in particular the presence of any cardiac defects, determines the management as well as the prognosis. We report a case of pentalogy of Cantrell diagnosed by ultrasound at 32 weeks of gestational age, the fetus died 3 hours after delivery. How to cite this article Ymele FF, Fouedjio JH, Kenfack B, Mehta M, Mbu RE. Pentalogy of Cantrell: A Report of One Case at the Yaounde Central Hospital in Cameroon. Donald School J Ultrasound Obstet Gynecol 2012;6(4):412-414.

Download Full-text

Prenatal Diagnosis of Pentalogy of Cantrell With Craniorachischisis by Three-Dimensionalultrasonography in the First Trimester

Taiwanese Journal of Obstetrics and Gynecology ◽

10.1016/s1028-4559(09)60315-6 ◽

2009 ◽

Vol 48 (3) ◽

pp. 317-318 ◽

Cited By ~ 4

Author(s):

Susumu Murata ◽

Masahiko Nakata ◽

Masahiro Sumie ◽

Masakazu Mastsuabra ◽

Norihiro Sugino

Keyword(s):

Prenatal Diagnosis ◽

First Trimester ◽

Pentalogy Of Cantrell

Download Full-text

Diagnosis of Pentalogy of Cantrell in the First Trimester Using Transvaginal Sonography and Color Doppler

Case Reports in Obstetrics and Gynecology ◽

10.1155/2015/179298 ◽

2015 ◽

Vol 2015 ◽

pp. 1-3 ◽

Cited By ~ 5

Author(s):

Ayşe Figen Türkçapar ◽

Ayla Sargın Oruc ◽

Aysegül Öksüzoglu ◽

Nuri Danışman

Keyword(s):

Abdominal Wall Defect ◽

Color Doppler ◽

First Trimester ◽

Cystic Hygroma ◽

Doppler Imaging ◽

Transvaginal Sonography ◽

Pentalogy Of Cantrell ◽

Complementary Role ◽

Intracardiac Defect

We report the prenatal diagnosis of Cantrell syndrome in the first trimester. During a routine transabdominal ultrasonographic examination, a midline supraumbilical abdominal wall defect including herniated liver and ectopia cordis with a large omphalocele containing the intestines and cystic hygroma was incidentally identified at the 12th week of gestation. A transvaginal sonography examination revealed a severe lumbosacral scoliosis in addition to the inability to visualize the abdominal aorta which was indicative of a severe intracardiac defect. The parents opted for pregnancy to be terminated. In this case report, we discuss the complementary role of transvaginal sonography and Doppler imaging in the diagnosis of Cantrell syndrome in early pregnancy.

Download Full-text