in 1958 , cantrell et al . ( 1 ) described a syndrome which consists of the following : 1 ) a midline , supraumbilical abdominal wall defect ; 2 ) a defect of the lower sternum ; 3 ) a deficiency of the anterior diaphragm ; 4 ) a defect in the diaphragmatic pericardium ; 5 ) congenital intracardiac defects . several authors have described similar cases , and it is currently classified as an anterior body wall midline developmental anomaly ( 2 ) . the estimated prevalence varies from one in 65,000 to 200,000 births ( 2 , 3 ) . in this case report , we describe successful vaginal delivery of a pregnant woman who had been diagnosed with this syndrome in childhood . a 23-yr - old nulliparous , primigravid woman was referred to our maternal - fetal medicine unit at 18 weeks ' gestation . she had been previously seen at our institution in the pediatric clinic as a child of 6 yr , when she presented with two umbilici and an epigastric pulsatile mass present since birth . at that time , she complained of intermittent abdominal pain in the area between the two umbilici . on physical examination , the superior umbilicus was found to be a dimple in the epigastrium connected to the actual umbilicus inferiorly by a fibrous band . a 13 cm pulsatile chest plain radiograph showed situs solitus and slightly decreased pulmonary vascularity and abdominal plain radiograph was unremarkable . cardiac catheterization , angiography , and echocardiography were performed and the pulsatile mass was found to be a substernal apical diverticulum of the heart beneath the weakened abdominal wall . periodic follow - up studies over the next 6 yr , including chest radiography , ekg , and echocardiography showed no remarkable changes . at age 23 yr , she presented to our institution at 18 weeks ' gestation and was examined by echocardiography . the echocardiogram revealed left ventricular ejection fraction of 63% , suspected mild right ventricular outflow tract compression without a pressure gradient , and insignificant mild narrowing of left pulmonary artery origin . during prenatal evaluation , the patient did not complain of any significant symptoms except for a pulsatile sensation in the epigastrium and mild intermittent dyspnea . there were no abnormal obstetric findings , including antenatal routine laboratory results , midtrimester maternal serum screening test , and vaginal examination . on fetal ultrasonogram , an epigastric dimple and the umbilicus were connected by a fibrous band , and the substernal cardiac diverticular beating beneath the abdominal wall was similar as previously described ( fig . induction of labor with vaginal dinoprostone insertion and intravenous oxytocin was performed . during the intrapartum period , the 1-min and 5-min apgar score was 9 and 9 and there was no gross anomaly noted including the umbilical cord and umbilicus . postpartum uterine contraction was adequate , and she was discharged with the healthy baby without any significant hemorrhage or abdominal pain . previous case reports have described various conditions associated with cantrell 's pentalogy , but as far as we are aware , this is the first reported case of successful vaginal delivery of a pregnant woman diagnosed with this condition . all five anomalies must be present for diagnosis of cantrell 's pentalogy to be made , but the severity may be quite variable , ranging from subtle to lethal . prognosis depends on the severity of the cardiac and associated anomalies . in affected patients with complex heart anomalies who undergo surgery during the first days of life possible causes of death include cardiac rupture , tamponade , endocarditis , embolism , arrhythmia , and heart failure ( 3 , 4 ) . the etiology of cantrell 's pentalogy is unknown , but it has been postulated that a defect in the common mesodermal origin accounts for the association of the five anomalies . most of the reported cases are sporadic , but x - linked inheritance has been suggested in some families , and mutations in genes located on the x chromosome have been suspected ( 5 , 6 ) . it is possible that some intestinal loops pass the umbilical ring and herniate into the cord during embryonic development , and a defect in normal internalization of the physiological umbilical hernia results in a persistent herniation and abundant umbilical skin ( 7 ) . in fact , the care of pregnant woman with cantrell 's pentalogy has not been previously described . with close antenatal follow - up and intrapartum monitoring we observed the patient closely for any symptoms and signs of dyspnea , abnormal abdominal pain , or tachycardia . this case report demonstrates that vaginal delivery may be an option for women with cantrell 's pentalogy .
cantrell 's pentalogy is a rare condition that consists of defects involving the abdominal wall , lower sternum , anterior diaphragm , pericardium , and heart . in the literature to date , 
 pregnant women with cantrell 's pentalogy have not been discussed . 
 we performed successful vaginal delivery of a 23-yr - old nulliparous , primigravid woman who had been diagnosed with this condition . 
 diagnosis was based on cardiac catheterization , angiography , and echocardiogram , and abdominopelvic ct . 
 vaginal delivery may be an option for women with cantrell 's pentalogy and may be attempted with caution .