Pentalogy of Cantrell: two patients and a review to determine prognostic factors for optimal approach

Abstract

Two patients with incomplete pentalogy of Cantrell are described. The first was a girl with a large omphalocele with evisceration of the heart, liver and intestines with an intact sternum. Echocardiography showed profound intracardiac defects. The girl died 33 h after birth. The second patient was a female fetus with ectopia cordis (EC) without intracardiac anomalies; a large omphalocele with evisceration of the heart, stomach, spleen and liver; a hypoplastic sternum and rib cage; and a scoliosis. The pregnancy was terminated.

Concurrence of prenatally diagnosed Cantrell pentalogy and short and tight umbilical cord: A possible case of hexalogy

“…While there may be severe forms of the pentalogy, less severe forms may also be observed. incomplete expressions (5,6). In our case, our patient had all the findings described by Cantrell.…”

“…Some of the congenital anomalies observed together with the Cantrell syndrome are the cloacal exstrophy, genitourinary and vertebral anomalies, occipital encephalocele, horseshoe kidney, bilateral clubfeet, spina bifida, hydrocephaly, abnormal ears, trisomy 18, exencephaly, pulmonary hypoplasia, cystic hygroma, craniorachischisis, and cleft lip and palate. The most commonly observed congenital cardiac disorders in these patients are ventricular septal defects, followed by atrial septal defects, left ventricular diverticulitis, tetralogy of Fallot, double outlet right ventricle and pulmonary stenosis (7). Since the development of the PC and umbilical vein formation go hand in hand during the embryonic stage, Brochut et al (8) have suggested that their pathologies may also be interconnected.…”

Incomplete pentalogy of Cantrell: a

“…This report presents the case of a patient with ASD, who was also diagnosed with pentalogy of Cantrell, a rare multiple congenital malformation syndrome characterized by a combination of 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 [2]. Our patient demonstrated an incomplete form of pentalogy of Cantrell, which is very rare.…”

Pentalogy of Cantrell with Encephalocele and Cystic Hygroma

“…Özellikle kardiyak defektin ve pentalojiyi oluşturan diğer defektlerin derecesi prognozu belirlediğinden, ileri haftalarda fetal manyetik rezonans görüntüleme ile değerlen-dirme önerilmektedir. 12,13 Olgumuzda, erken gebelik haftasından dolayı fetüs, manyetik rezonans gö-rüntüleme ile değerlendirilmemiştir.…”