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Ectopia cordis (EC) is a rare malformation that occurs as an isolated lesion or as part of the pentalogy of Cantrell which is characterized by midline closure defects. This was first described by Haller et al. in 1706. EC is seen with a frequency of 5.5-7.9 per 1 million births. It has five types that are: cervical, cervico-thoracic, thoracic, thoraco-abdominal, and abdominal. Its differentiation from the pentalogy of Cantrell, first described by Cantrell in 1958, must be done well. Thoracic type has the worst prognosis and due to this poor prognosis in the postnatal period, termination may be offered to these patients as an option. In this paper, a case of an isolated thoracic-type complete EC detected in the prenatal ultrasonography of a pregnant woman referred to our clinic at the 18 weeks and 3 days of gestation is presented., (© 2020. Società Italiana di Ultrasonologia in Medicina e Biologia (SIUMB).)

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., Competing Interests: None declared., (Thieme. All rights reserved.)

We present a 31 gestational weeks' premature baby whose fetal echocardiogram showed ectopia cordis, single ventricle and severe pulmonary stenosis. At 31 gestational weeks, an emergency caesarean section was performed, and his birth weight was 1756 g, SpO2 was 80% on 100% O2. Epicardial echocardiogram showed double inlet right ventricle, severe valvular pulmonary stenosis and no ductus arteriosus. The risk of surgery was very high, so we decided to perform balloon valvuloplasty by direct puncture of the heart. We punctured the apex of the ventricle using a 16-gauge needle under echo guidance, advanced the guidewire to the pulmonary artery and performed balloon valvuloplasty. Soon after the procedure, the cyanosis improved dramatically. This is the first report of a transcatheter procedure performed by direct puncture of the heart for ectopia cordis with complex congenital heart disease.

Surgical correction of Cantrell syndrome is often associated with an extremely high mortality rate due to the possibility of wound infection or the severity of cardiac anomalies. We report a case of Norwood operation and repositioning of the heart successfully performed 1 day after the birth of a neonate with pentalogy of Cantrell. The patient had double-outlet right ventricle, subaortic stenosis, aortic valve stenosis, hypoplastic aortic arch, and coarctation of the aorta. The patient underwent the Glenn operation at the age of 1 year and is now waiting for the Fontan operation., (Copyright © 2020 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Case Description: A 1-year-old spayed female domestic shorthair cat was evaluated for a sternal defect and ventral abdominal wall hernia., Clinical Findings: The cat appeared healthy. Palpation revealed a sternal defect, and the heart could be observed beating underneath the skin at the caudoventral aspect of the thorax. A 3-cm-diameter freely movable mass, consistent with a hernia, was also palpated at the cranioventral aspect of the abdomen. Thoracic radiographic and CT images revealed a sternal cleft, cranial midline abdominal wall hernia, and peritoneopericardial diaphragmatic hernia (PPDH)., Treatment and Outcome: Thoracotomy and celiotomy were performed. The sternal cleft was repaired with a porcine small intestinal submucosa graft, titanium contourable mesh plate, and interrupted 25-gauge cerclage wires. A diaphragmatic herniorrhaphy was used to correct the PPDH. Thoracic radiographs were obtained immediately after surgery to confirm repair of the sternal cleft, abdominal wall hernia, and PPDH and at 1 and 3 months after surgery to assess the surgical implants, which had not migrated and were intact with only mild bending at the cranial and caudal margins of the mesh plate. At both recheck examinations, the cat appeared healthy with no complications reported by the owner., Clinical Relevance: A novel surgical technique was used to successfully repair a large sternal cleft in an adult cat with no postoperative complications reported. This technique may be useful for the treatment of sternal clefts in other cats. This was the first report to describe an adult cat with congenital defects consistent with incomplete pentalogy of Cantrell.

Pentalogy of Cantrell is a congenital anomaly associated with defects in the abdominal wall, sternum, diaphragm, and diaphragmatic pericardium formation, in addition to the development of cardiac abnormalities. It is a rare disease with an estimated incidence of one case for every 65,000 births, being more common in males (60% of cases). It has a reserved prognosis with mortality around 63%, and a maximum of 9 months survival after surgery. There are few case reports addressing the pentalogy of Cantrell, which is justified by the rarity of this pathology. In this report our objective was to describe a surgical case of a female patient and make some anesthetic considerations about this rare congenital malformation., (Copyright © 2018 Sociedade Brasileira de Anestesiologia. Publicado por Elsevier Editora Ltda. All rights reserved.)

Background: Repairing body wall defects is a critical step in the treatment of some congenital deformities, and this procedure may need the help from plastic surgeons. Although there are many articles about congenital deformities, body wall defects of these malformations are rarely studied as independent targets., Methods: In this article, the authors present an LDT classification for congenital body wall defects according to the position of the defects, the tissue layers involved, and the surgical urgency, each of which is represented by letters L, D, and T, respectively. That is, the defects in different areas (L), full-thickness (D 1 ), or partial (D 0A , D 0B ) defects, defects needing instant repair (T 2 ), semi-elective repair (T 1 ), or elective repair (T 0 ). Based on this classification system, the authors have performed body wall reconstruction on two pairs of thoraco-omphalopagus twins, one pair of ischiopagus tetrapus twins, and an infant and an adult, both of whom were diagnosed with pentalogy of Cantrell associated with ectopia cordis., Results: Except for one pair of thoraco-omphalopagus twins who died after emergency separation, all the other patients survived. Another pair of thoraco-omphalopagus twins suffered from wound dehiscence and partial flap necrosis, respectively, after surgery. An expanded polytetrafluoroethylene mesh in one sister of the ischiopagus twins was removed because of infection., Conclusions: LDT classification not only can help doctors categorise different congenital body wall defects rapidly and easily, but can also guide the reconstruction of these defects. It may have clinical value to plastic surgeons to some extent., (Copyright © 2017 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.)

A 27-year-old woman, gravida 2, para 0, presented with an incomplete Pentalogy of Cantrell with an omphalocele, diaphragmatic hernia, and a pericardial defect at 32 weeks' gestation. A large pericardial effusion compressed the lungs and had led to a reduced lung growth with an observed-to-expected total lung volume of 28% as measured by MRI. The effusion disappeared completely after the insertion of a pericardio-amniotic shunt at 33 weeks. After birth, the newborn showed no signs of pulmonary hypoplasia and underwent a surgical correction of the defect. Protracted wound healing and a difficult withdrawal from opioids complicated the neonatal period. The child was discharged on postnatal day 105 in good condition. This case demonstrates that in case of Pentalogy of Cantrell with large pericardial effusion, the perinatal outcome might be improved by pericardio-amniotic shunting., (© 2017 S. Karger AG, Basel.)

Cantrell syndrome includes the defects of the heart, pericardium, diaphragm, abdominal wall, and sternum. The operative mortality is usually high. We report here a one-stage surgical correction in a case of Cantrell syndrome with left ventricular diverticulum.

Background: The purpose of this study was to evaluate our experience with pentalogy of Cantrell and the various embryologic variants., Materials and Methods: Patient charts and diagnostic imaging studies of all fetuses evaluated at Texas Children's Fetal Center for pentalogy of Cantrell between April 2004 and June 2014 were reviewed retrospectively. Data collected from patient charts included demographic information, clinical presentation, fetal and postnatal imaging findings, operative treatment, pathologic evaluation, and outcomes., Results: There were 10 patients who presented with embryologic variants of pentalogy of Cantrell over a 6-y period. Two cases displayed the full range of embryologic defects observed, and eight cases exhibited variants of the classic pentalogy. Sternal and pericardial defects were each present in 40% of patients. Additional anomalies present included pulmonary hypoplasia, pulmonary artery stenosis, and chromosomal abnormalities. Four patients presented with diaphragmatic defects but no defect in the pericardium, and one patient presented with a defective pericardium but no associated diaphragmatic defect, suggesting highly specific losses of somatic mesoderm during embryologic development. One patient was lost to follow-up, and a second patient underwent termination of pregnancy. Five of the remaining eight patients survived, one of which had the full range of embryologic defects and now attends preschool but requires speech and occupational therapy. The remaining surviving patients have developed without serious sequelae., Conclusions: This report highlights the spectrum of anomalies observed in the pentalogy of Cantrell and demonstrates that these fetuses can survive but with substantial morbidity., (Copyright © 2015 Elsevier Inc. All rights reserved.)

A 5 mo old male German shepherd dog weighing 15.5 kg was presented with an abdominal wall hernia and exercise intolerance. Physical examination showed a grade II/VI systolic heart murmur and an area of cutaneous atrophy overlying a midline supraumbilical wall defect. Thoracic radiography, computed tomography, and ultrasound examination revealed a congenital caudal sternal cleft, a supraumbilical diastasis rectus, and a patent ductus arteriosus. Exploratory surgery confirmed defects of the pars sternalis of the diaphragm and caudoventral pericardium and a persistent left cranial vena cava. Those findings were compatible with Cantrell's pentalogy. Surgical treatment included ligation of the patent ductus arteriosus through the sternal cleft, diaphragmatic reconstruction with paracostal extension of the diaphragmatic defect, pericardial and linea alba appositional reconstruction, and primary approximation of the sternal halves. Growth and exercise activity were normal 10 mo after surgery. The discovery of a midline cranial abdominal wall, pericardial, diaphragmatic, or sternal defect should prompt a thorough examination to rule out any possible associated syndrome. Cantrell's pentalogy presents various degrees of expression and is rare in dogs. Management involves early surgical repair of congenital anomalies to protect the visceral structures. The prognosis in dogs with mild forms of the syndrome is encouraging.

Pentalogy of Cantrell (PoC) is a rare congenital midline defect. We present a case and its treatment of PoC with complete ectopia cordis and congenital heart disease. Postnatally the congenital heart defect was surgically corrected and the ectopic heart was covered by musculous mobilized flap. Due to cephalic orientation of the heart and limited intrathoracic space, replacement of the heart into the thoracic cavity was initially not performed. After 11 years of follow up our patient now is without relevant limitations solely wearing a thoracic shelter. This case elucidates the complexity of further management. The potential risk of disastrous hemodynamic compromise by intrathoracic shift is to compare with the limited safety of the ectopic heart., (© 2014 Japanese Teratology Society.)

The ideal treatment for pentalogy of Cantrell is neo-sternum reconstruction by using autologous tissues. Although treatment timing varies depending on the degree of deformity and patient's condition, the principle is performing the procedure at the earliest, to prevent blunt or piercing trauma to the heart. However, the challenge is performing the procedure on a neonate, because feasibility of the procedure is affected by the size of the defect, and limitations in utilizable autologous tissues. We used a small biocompatible patch (Permacol) and lower costal cartilage to perform curative neo-sternum reconstruction, which is a simple and safe treatment method.

A 14-month-old girl with pentalogy of Cantrell, a very rare congenital syndrome characterized by an epigastric omphalocele and malformations of the heart, sternum, pericardium, and diaphragm, underwent echocardiography and multidetector computed tomography before surgical repair of these deformities was attempted. These tests revealed multiple cardiovascular and noncardiovascular abnormalities. After surgery, the patient's cardiovascular status was stable. Although studies have shown that echocardiography, multidetector computed tomography, and magnetic resonance imaging may each play a role in the diagnosis and management of this condition, there are few data available to support the use of one imaging modality over another.

Pentalogy of Cantrell is a very rare condition with very high mortality. We present an adult survivor with a classic pentad who underwent sequential surgical repairs as a neonate, child, and young adult. He required home mechanical ventilation for the first two years of life and subsequently needed noninvasive nocturnal ventilation as an adult., (© The Author(s) 2014.)

Purpose: To summarize the experience and feasibility of radical one-stage surgical repair of pentalogy of Cantrell in eight cases in a single tertiary care center., Patients and Methods: From August, 2007 to June, 2011, eight patients with pentalogy of Cantrell were diagnosed. One of them had undergone intracardiac operation 2 years ago, leaving a large thoracoabdominal defect with most of the heart outside the chest. After correction of cardiac malformations, patients were individually treated with different operative procedures to restore their cardiac position and anatomy and repair their thoracoabdominal and diaphragmatic defects., Results: Surgical procedures in all the patients were uneventful. Seven cases underwent one-stage complete correction. The only patient candidate for second-stage operation underwent extended thoracoplasty because of inadequate accommodation for the heart. Diaphragmatic defects were repaired with a PROCEED patch in two cases and closed directly in the remainder. The right sixth and seventh ribs were grafted for chest reconstruction in two cases and the right seventh rib in one case. Chest wall clefts were closed directly in the remaining five cases. A left ventricular diverticulum was removed in one of the two patients. An interventricular residual shunt (0.5cm) was observed in one case and treated by transcatheter closure on the fifth day after operation. One case with double outlet right ventricle suffered from low cardiac output syndrome during the postoperative period and recovered after medical treatment. The early and midterm clinical outcomes were satisfactory without death or serious complications during the follow-up period of 24-72 months., Conclusion: The diversity and complexity of POC result in a variable clinical course, with different clinical manifestations which require unique surgical procedures. Innovative multidisciplinary management strategies are necessary to achieve better outcomes. The key points in surgical treatment are: a) to obtain satisfactory correction of the cardiac malformation, b) adequate space for return of the heart to the mediastinum, and c) a thoracoabdominal wall repair which protects the heart from excessive compression or distortion. If treated correctly, most patients with less complex cardiac defects who survive the newborn period can be successfully treated via a one-stage operation., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Pentalogy of Cantrell with ectopia cordis is a rare congenital anomaly, first described in 1958 by Cantrell, has a reported incidence of around 5-10 cases per one million live births with wide variety of clinical presentations. We are reporting a child with ectopia cordis along with cleft lower sternum, upper abdominal wall defect, ectopic umbilicus and diaphragmatic defect. Echocardiography in first month of life revealed a restrictive perimembranous ventricular septal defect and a small patent Foramen Ovale, both closed spontaneously in infancy. CT angiography at 10 months of age revealed a defect in the thoracic and abdominal walls along with herniation of left ventricular apex into epigastrium. The two ventriculi formed a tail that looked like a crocodile. This patient underwent surgical correction at our institution at 14 months of age and recovered well with no residual issue.

The pentalogy of Cantrell is a rare and fatal malformation. A left ventricular diverticulum is a common finding of this pentalogy. The present report summarizes our experience with three patients who suffered from pentalogy of Cantrell. All three cases had a left ventricular diverticulum. The intracardiac malformations and the left ventricular diverticulum were corrected simultaneously. All patients recovered uneventfully. One-stage surgical correction could improve short-term outcomes in selected patients with pentalogy of Cantrell., (© 2014 Wiley Periodicals, Inc.)

Introduction: Cantrell syndrome is a rare syndrome of congenital defects, which can be complicated by severe pulmonary hypertension and left ventricular diverticulum; it has proved difficult to treat in clinical practice., Case Presentation: A 6-month-old Han Chinese baby girl weighing 3.5 kg was diagnosed, using ultrasonography and radiography, as having Cantrell syndrome complicated by severe pulmonary hypertension. For safety, we divided management into two stages. For the first stage, we dealt with the left ventricular diverticulum and pulmonary hypertension. Three months later, we performed diorthosis for an intracardiac malformation., Conclusions: Cantrell syndrome with pulmonary hypertension may respond well to this novel two-stage operation, which needs more verification via clinical practice.

We describe a simple technique for chest wall reconstruction in pentalogy of Cantrell using split-thickness cartilage grafts. This technique provides a robust reconstruction, gives immediate and excellent cosmetic results, allows for skeletal and tissue growth, and avoids the use of synthetic material., (© 2012 Wiley Periodicals, Inc.)

Purpose: Children with a history of a congenital abdominal wall defect (AWD) occasionally require additional abdominal surgery later in life, after their defect is closed. In an effort to evaluate surgical history and assess the feasibility of laparoscopic surgery in this select patient population, a retrospective review was conducted looking at all patients treated with congenital AWD at a tertiary-care facility., Patients and Methods: Patients admitted between January 1, 2000 and January 1, 2011 with AWD were included in the study. Abdominal surgical procedures subsequent to closure of the defect were compared for indication, technique, length of surgery, estimated blood loss, and postoperative length of stay., Results: During the stated time period, there were 139 admissions with AWD (67 girls and 72 boys). Thirty percent of the children (n=43) underwent subsequent abdominal surgery after the closure of their defect for a total of 65 procedures. Seventy-four percent of these procedures (n=48) were performed by an open technique, and 26% were laparoscopic (n=17). Because of dense adhesive disease, 2 cases (12%) were converted from laparoscopic to open. There were no complications as a result of laparoscopic surgery in any of the patients., Conclusions: This study evaluates the surgical history of patients with AWDs after their defect is closed. The results of this study suggest that in many situations, laparoscopic surgery is feasible and can be safely conducted on children with a history of congenital AWD. Based on these results, a history of congenital AWD should not be considered a contraindication for laparoscopy.

Ectopia cordis is a rare congenital malformation in which the heart is located partially or totally outside the thoracic cavity. It comprises 0.1% of congenital heart diseases. The authors present a case of a male baby born at term by emergency caesarean section due to prolonged fetal bradycardia, who was noted to have a large pulsating mass in the thoracoabdominal area. In view of lower thoracolumbar abdominal defect, ectopic placement of the umbilicus, deficiency of the diaphragmatic pericardium, deficiency of anterior diaphragm and intracardiac abnormalities, a diagnosis of ectopia cordis-Pentalogy of Cantrell was made. He was transferred to a tertiary centre and required oxygen supplement initially. He was sent home after 1 week, on propanolol, with weekly oxygen saturation checks. He is awaiting further surgical intervention pending the required weight gain.

Cantrell et al. described a syndrome with five anomalies characterised by defects of the abdominal wall, lower sternum, anterior diaphragm, diaphragmatic pericardium, and heart. Because most of the children who survived could not have the thoracoabdominal wall adequately reconstructed at the initial operation, ventral herniation is often the result and they have to live with the danger of direct trauma to the unprotected heart. It therefore becomes important protect the unguarded heart while improving the appearance of the thoracoabdominal region. The reconstruction of the lower sternum has rarely been reported. We describe four such patients, three of whom had a thoracoabdominoplasty and umbilicoplasty using autologous rib cartilage and rectus muscles. All these children had solidly reconstructed sternums, and their abdominal appearances are excellent.

We present a case of a 10-month-old male infant with thoracoabdominal ectopia cordis, as part of Cantrell pentad, repaired using a poly-L-lactic-polyglycolic acid plate, a resorbable plating system widely used in craniomaxillofacial reconstruction. This is the first reported case of sternal reconstruction using a poly-L-lactic-polyglycolic acid plate. The repair was successfully carried out without cardiopulmonary compromise and good aesthetic outcome was achieved., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Pentalogy of Cantrell is a rare congenital anomaly characterized by a combination of severe defects in the middle of the chest and abdomen including intracardiac defects. Survival rate after cardiac surgery is extremely low. We present a successful staged complete repair of an omphalocele, a ventricular septal defect and a sternal defect in a case of pentalogy of Cantrell., (© 2012 Wiley Periodicals, Inc.)

Cantrell syndrome is a rare condition of congenital defects of 5 developmentally associated structures: the abdominal wall, sternum, diaphragm, pericardium, and heart. Few patients survive, and even fewer survive with good outcomes of quality of life. We present a case with the pentalogy and profound cardiac malformations. Despite repeated life-threatening conditions, the boy survived to have his first operation at 5 months old. The operation reduced the heart within the thoracoabdominal area, repaired the abdominal wall defect, and released multiple stenoses of the heart and great vessels. The patient recovered uneventfully. At 4.5 years old, he is healthy and developed normally., (Copyright © 2011 Elsevier Inc. All rights reserved.)

We report the MDCT findings of a 17-month-old girl with Cantrell's pentalogy, a rare congenital disease characterized by several defects in the ventral thoracoabdominal wall including ectopia cordis, and, in this patient, associated with tetralogy of Fallot. This case provides an example of the utility of a wide volume in coverage and high-pitch MDCT scan in the evaluation of complex cardiovascular anatomy in infants with congenital heart disease without the need of an ECG-gating acquisition.

Pentalogy of Cantrell is characterised by a combination of severe defects in the middle of the chest including the sternum, diaphragm, heart, and abdominal wall. Mortality rate after cardiac surgery is usually high. We report a successful total correction of the cardiac defects in a case of Pentalogy of Cantrell with a double-outlet right ventricle prior to abdominal wall defect repair.

We present a case of ectopia cordis with a complex single-ventricle congenital heart defect in association with pentalogy of Cantrell. Management by a skilled multidisciplinary team was critical for patient survival. Early fetal diagnosis and the use of advanced imaging techniques allowed adequate time for planning and identified critical anatomic details. Preserving the heart's natural covering, performing cardiac surgery without cardiopulmonary bypass, and using catheter intervention decreased the risk to the patient. Complete coverage of the defect was achieved using skin generated with tissue expanders. This case illustrates the importance of collaboration when caring for infants with critical, high-risk disease and highlights the increased potential for survival with complex ectopia cordis in the current medical era.

Pentalogy of Cantrell is 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. This congenital defects could be diagnosed as early as in the first trimester of pregnancy. The complexity of this anomaly especially coexistent heart defects, determines the way and order of surgical treatment and commonly is a prognostic factor. A case of male newborn with prenatal diagnosed omphalocele and diaphragmatic hernia is reported in the present work. The child was operated within two hours after birth. Intraoperativelly a significant defect of diaphragmatic pericardium and heart surrounded by a small bowel were discovered. The defect of diaphragm was sutured and a drainage of left pericardial cavity was put in place. Afterwards, the abdominal wall defect was sutured after transferring organs into a peritoneal cavity. Six days after the procedure cardiac sonography was performed and it indicated the presence of hemodynamically insignificant congenital intracardiac defect--atrial septal defect. Up-to-date psychomotor development of the boy currently five months old, is correct. Prenatal diagnosis of the complexity of congenital defects and possibility of fetus condition monitoring allows to plan precisely the time and way of the delivery as well as to decide the treatment method of the newborn. Due to the absence of hemodynamic disorders and in spite of the present cardiac defect, an early surgical treatment of our patient was possible.

Pentalogy of Cantrell is a rare congenital anomaly involving deficiency of the following structures: anterior diaphragm, supraumbilical abdominal wall, diaphragmatic pericardium, lower sternum, and associated congenital intracardiac abnormality. We describe a 3-month-old child with this syndrome having left ventricular diverticulum along with omphalocele who presented to us with a pulsating mass in the epigastrium. The defect was evaluated and defined by computed tomography scan. A team of pediatric, cardiac, and plastic surgeons successfully repaired the defects. This case report discusses the review of literature along with management options and concludes that there must be an emphasis on early repair of left ventricular diverticulum to prevent complications. Antenatal ultrasound can also detect the anomaly, and early postnatal diagnosis of the syndrome, followed by immediate surgical repair, can prevent lethal complications.