Experience in the Treatment of Pentalogy of Cantrell with Artificial Materials in a Single Clinical Center

Objective To summarize experience in the treatment of pentalogy of Cantrell (POC) in our hospital and explore the effect of artificial materials in repairing sternal defects. Materials and Methods A retrospective analysis was performed on treatment of five children with POC treated by using the Gore-Tex patch and titanium mesh in the Department of Cardio-Thoracic Surgery, Children's Hospital of Chongqing Medical University, from January 2010 to January 2019. Results The concurrent conditions included double outlet of right ventricle (n = 2), ventricular septal defect (VSD) and atrial septal defect (ASD) (n = 1), VSD and ASD and patent ductus arteriosus (n = 1), and VSD and left ventricular diverticulum (n = 1) in five cases with POC. Color Doppler echocardiography and computed tomography (CT) + three-dimensional (3D) reconstruction of the thorax and abdomen were performed preoperatively. The cardiac malformation was corrected according to color Doppler echocardiography, and a Gore-Tex patch was used to repair the pericardial defect. Titanium mesh was made according to CT 3D reconstruction with a 3D printing mold to repair sternal defects. All patients underwent a one-stage operation, all hearts were eventually repositioned, no deaths occurred after the operation, and follow-up was performed for 6 months to 2 years. The patients recovered well, and the exterior thorax was normal. Conclusion The diagnosis of POC is not difficult. The priority of surgical treatment for POC is to obtain satisfactory corrections of cardiac malformation. The repair of the pericardial defect with the Gore-Tex patch and the sternal defect with the titanium mesh can make the heart return to the mediastinum, reduce the pressure on the heart, reduce the surgical trauma, reduce the difficulty of repairing the sternal defect, and optimally restore the exterior thorax.

Incomplete pentalogy of Cantrell: a case report and review

BACKGROUND: The pentalogy of Cantrell is a rare congenital malformation characterized by five component defects in the anterior abdominal wall, lower sternum, anterior diaphragm, and diaphragmatic pericardium and congenital heart disease. The occurrence of the five features is quite rare. The pentalogy of Cantrell is classified as complete, partial, and incomplete. Few studies have described the successful treatment of neonates with the pentalogy of Cantrell, with even fewer publications about an incomplete defect. CASE REPORT: We report the successful surgical treatment of a newborn boy with an incomplete pentalogy of Cantrell. In this patient, the diaphragmatic hernia was eliminated at the first stage, and a temporary container for umbilical cord hernia was made by suturing the silastic sac to the edges of the defect in the anterior abdominal wall for subsequent gradual immersion of the hernia contents into the abdominal cavity. These steps made it possible to reduce intra-abdominal and, accordingly, intrathoracic pressures, provide favorable conditions for the healing of the diaphragm, and thus stabilize the childs respiratory and cardiovascular systems. Subsequently, the cardiovascular system was examined under more favorable conditions and intracardiac defects were excluded. Moreover, the proposed technique made it possible to safely perform the second stage of surgical correction, i.e., radical plasty of the anterior abdominal wall on day 14 of life with full restoration of the normal anatomical and physiological relationships, by which time the diaphragm and mediastinum had taken their correct topographic position. The literature review provides data from 32 sources. CONCLUSION: The pentalogy of Cantrell is a severe congenital malformation with a high risk of poor outcomes. Reporting of all possible variants of the pentalogy of Cantrell (complete, partial, or incomplete), regardless of the outcome, is important for the accumulation of experience in treating such patients, which by focusing on the clinical situation and the combination of various defects in the pentalogy of Cantrell will improve the strategy and prognosis for this defect.

A rare autopsy case of pentalogy of Cantrell with tetralogy of Fallot and olfactory bulb agenesis

Introduction/Objective Pentalogy of Cantrell is an uncommon congenital disorder characterized by sternal, diaphragmatic, pericardial, intracardiac and supraumbilical abdominal or chest wall defects. It is usually associated with other abnormalities and thus poses a therapeutic challenge, with a high mortality rate despite early diagnosis. Methods/Case Report This is an autopsy case of a 1 day old, former 37-week-old male infant with a prenatal diagnosis of incomplete pentalogy of Cantrell including omphalocele, ectopia cordis, pleural effusions, cardiac anomalies and polyhydramnios who was delivered via elective cesarean section. Maternal history included tobacco use during pregnancy, multiple psychiatric disorders, and a family history of an unbalanced translocation. The infant required intubation, ventilation, and chest tube insertion at birth due to respiratory distress. He also developed pneumoperitoneum which had to be decompressed. Within 24 hours the infant passed, despite advanced care. On autopsy, external examination showed central and peripheral cyanosis and a large omphalocele containing the heart, parts of the liver and intestines. Initial internal examination revealed a hypoplastic left lung, anterior diaphragmatic defect and absence of the pericardium. Organ dissection post-fixation showed an overriding aorta, pulmonic stenosis, subaortic valve ventricular septal defect and right ventricular hypertrophy, all components of tetralogy of Fallot. On brain examination, the olfactory groove and bulb were both absent. Examination of the placenta and 3 vessel cord revealed no abnormalities. Chromosomal microarray analysis on cord blood was normal. Conclusion The spectrum of defects seen in this disease complex has been postulated to be due to failure of differentiation or migration of mesenchymal structures during embryonic development but ultimately, this entity is not well understood. Identifying various disease associations through autopsies can help to establish possible etiologies, and options for therapeutic interventions or screening.

Prenatal ultrasound diagnosis of anterior abdominal wall defects in sub Saharan Africa; simple but often missed

Background: Congenital anterior abdominal wall defects (AAWD) is a spectrum of abdominal wall defects that includes omphalocele, gastroschisis, bladder exstrophy, cloacal exstrophy, prune belly syndrome and pentalogy of Cantrell. Early Prenatal diagnosis of AAWD provides opportunity for abnormal karyotypes screening and planned delivery in a specialized centre. Ultrasound can detect these defects during pregnancy. This study aims to evaluate the detection rate of AAWD during routine obstetric ultrasonography in our region.Methods: A retrospective study of all patients that presented with AAWD to our centre from January 2008 to July 2020. Data included patient’s age, sex, birth weight, diagnosis, resuscitation time, outcome, maternal age, parity and antenatal ultrasound scan (USS) records. Antenatal USS before 12 weeks only, were excluded. Data analysed using excel.Results: Of the 140 with AAWD, 84.29% had omphalocele, 10% gastroschisis, 2.14% prune belly syndrome and 0.71% each with bladder exstrophy, cloacal exstrophy and pentalogy of Cantrell. There were 123 booked pregnancies. Majority (112) had antenatal care elsewhere while 11 attended our Centre. Ultrasonography of 108 pregnancies scanned at12 weeks or beyond, had 4 confirmed prenatal diagnosis of AAWD. All done in our centre. Mean gestational age at diagnosis was 24weeks. Outcome was rupture1 (25%) and 25% mortality (prenatally diagnosed) and 51.92% mortality for patients with missed diagnosis.Conclusions: Our obstetric ultrasound detection rate of AAWD is very low. There is a need for improvement in training to improve perinatal care of these defects.