Pentalogy Of Cantrell Research Articles

Sternal cleft: Anaesthetic management of a rare congenital anomaly.

Sir, A sternal cleft is a rare congenital anomaly resulting from the failure of fusion of lateral sternal bars during the 3rd month of intrauterine development.[1] It may be complete or incomplete (bifid type), depending on the degree of fusion of two lateral sternal bars, the latter being more common.[2,3] Associated anomalies, particularly cardiac anomalies, result in poor outcomes.[4,5] The surgical procedure is associated with critical changes in haemodynamics and cardiopulmonary mechanics, necessitating invasive monitoring, preparedness for cardiopulmonary bypass and resuscitation (CPR).[6] Here, we report anaesthetic management of a case of sternal cleft repair with a brief review of available literature. A full-term male baby, weighing 2.6 kg, was delivered by caesarean section. The attending paediatrician noted a longitudinal depression at the sternal region which was more evident during inspiration [Figure 1]. Pulsation of great vessels was easily seen through the defect which was covered by a thin layer of skin. Two parallel and completely separated sternal bars were palpated along the depression. There was no anterior abdominal wall defect, diastasis of recti, fascial haemangioma, atrophic skin or paradoxical movement of the thoracic cage. A provisional diagnosis of isolated sternal cleft was made, and the baby was evaluated for associated anomalies. Chest X-ray revealed normal lung fields with a wide gap between the medial ends of the clavicles [Figure 2]. Echocardiogram and blood investigations were normal. The child was posted for sternal cleft repair on the 3rd postpartum day. In the operating room, standard monitoring was attached. The pulse rate was 130/min, oxygen saturation 97% with oxygen (O2) supplementation by nasal cannula at 3L/min and respiratory rate was 34/min. The child was induced with inhalation of sevoflurane (2 to 8%) in O2 and two peripheral intravenous lines were secured. The injections of fentanyl (5μg) and atracurium (1.5mg) were administered, and trachea was intubated with 3.5mm uncuffed endotracheal tube. The right radial artery was cannulated for invasive blood pressure monitoring. Anaesthesia was maintained with isoflurane in O2 /air (40–60) mixture. The child was put on pressure controlled ventilation, and adequate tidal volume (VT) was achieved with peak inspiratory pressures (PIP) of 18 mmHg. A vertical midline incision from the suprasternal notch to xiphoid process was given by the surgeons, and margins of sternal bars were freshened, followed by closure of the defect. The blood loss was minimal. PIP and haemodynamics were observed for next 10 min before knotting the sutures. PIP of 20 mmHg was required to achieve the same VT with no significant changes in haemodynamics. On the 2nd postoperative day, the trachea was extubated, and the child was discharged home in stable condition on the 7th day. Figure 1 Obvious midline depression in the child Figure 2 Wide gap between the medial ends of the clavicles The reported incidence of sternal cleft varies across the institutes. Acastello et al.[7] noted an incidence of 0.15% of all chest deformities during a 25 years period observation. It is usually diagnosed at birth due to the presence of cosmetic deformity and is generally asymptomatic. Associated cardiac anomalies, defects in the anterior chest wall (ectopia cordis), or the presence of Cantrell's Pentalogy (defects involving the abdominal wall, sternum, diaphragm, pericardium and heart) indicate poor prognosis.[4,5] Surgical correction is required to restore bony protection to the vital organs and great blood vessels, to provide normal intra-thoracic pressure-volume relationships and to eliminate paradoxical movement of the thoracic viscera.[1,2,3] Surgery is preferred in the new born period as the thoracic cage is more compliant, and the closure of the chest wall can easily accommodate the thoracic viscera without compressing the underlying structures.[6] It presents distinct challenges to the anaesthesiologist due to the potential involvement of multiple organ systems and the risk of direct injury to the vital organs. Associated complex congenital heart disease or poor baseline cardiac functions warrant standby cardiopulmonary bypass.[4,5,6] Arterial and central venous pressure monitoring is useful, as is trans-oesophageal echocardiography (TEE). Intraoperative TEE offers real-time information on volume status and cardiac output. The repair may lead to compression on the intra-thoracic organs which may cause haemodynamic instability and decreased lung compliance necessitating invasive monitoring, and close observation of ventilatory parameters. Rapid blood loss, arrhythmias, cardiac dysfunction and pneumothorax are the possible complications.[2,3] Literature advises test closure for 5–10 min while observing the haemodynamics and cardiopulmonary interactions.[3] To conclude, repair of sternal cleft requires invasive haemodynamic monitoring, close observation of ventilatory parameters and preparedness for CPR. Elective postoperative mechanical ventilation with adequate analgesia helps in early recovery.

Diagnosis of pentalogy of cantrell in the first trimester using transvaginal sonography and color Doppler.

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.

Ectopia cordis: a case report

Introduction: ectopia cordis is a rare congenital malformation, with an estimated incidence of 5 to 8 per million live births. It is defined as a malformation in which the heart is located in an extra-thoracic position. Ectopia cordis may occur as an isolated malformation or associated with other anomalies such as omphalocele, congenital heart disease or integrating Cantrell syndrome. The size and location of the defect influence the prognosis. Description: we report a case of a 24-year-old nulliparous woman, with no relevant family or personal history, in which the prenatal fetal ultrasound, performed at 21 weeks of gestation, revealed adefect of the anterior chest wall with exteriorization of the heart. Discussion: fetal echocardiography revealed a severe congenital heart disease. The parents decided to continue the pregnancy, after being duly informed by a multidisciplinary team. Delivery occurred at 37 weeks of gestation but the female newborn died one hour afterwards. Pathological examination confirmed the sonographic findings.

Retraction: Novel two-stage surgical treatment for Cantrell syndrome complicated by severe pulmonary hypertension: a case report

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. 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. Cantrell syndrome with pulmonary hypertension may respond well to this novel two-stage operation, which needs more verification via clinical practice.

Ectopia Cordis and Pentalogy of Cantrell: Report of Two Cases

Ectopia cordis (EC) is the presence of a live, beating heart outside the thorax and is one of the most unique congenital anomalies. EC has been a weak association noted with trisomy 18 and a few cases have been associated with other chromosomal abnormalities. Pentalogy of cantrell (PC) is a rare congenital syndrome of abdominal wall defect, lower sternal defect, diaphragmatic pericardial defect, anterior diaphragmatic defect, and intracardiac abnormalities. It has a rare frequency of about 1/100,000 births. The hallmark of this syndrome is an omphalocele associated with EC. Only a few patients with the full spectrum of the pentalogy have been described. Only very few patients survive attempts at surgical repair, the main causes of death being tachyarrhythmias, bradycardia, low blood pressure, rupture of the diverticulum, and heart failure. Early diagnosis is important about discuss the option of elective termination with the parents if intracardiac anomalies of incompatible with life are noted. We report two cases of PC diagnosed in early second trimester.

Pentalogy of Cantrell: first case reported in Saudi Arabia

Pentalogy of Cantrell (PC) is a rare congenital anomaly involving defects in the anterior diaphragm, supraumbilical abdominal wall, diaphragmatic pericardium, and lower sternum, and other congenital intracardiac abnormalities. Here, we report the case of a newborn infant who was born at 32 weeks of gestation and had all 5 features of PC, in addition to absent kidneys and a deformed left hand. Medical intervention would not be able to save the patient, so we allowed her to die in peace. We discuss here the etiology, prenatal diagnosis, and severity of and the mortality associated with this condition. To our knowledge, this was the first reported case of PC in Saudi Arabia.

Prenatal Diagnosis of Cantrell's Pentalogy Associated with Agenesis of Left Limb in a Twin Pregnancy.

Pentalogy of Cantrell is a rare malformation described in the literature. We report a case of pentalogy of Cantrell associated with left limb deficiency in a twin pregnancy. The fetus with multiple anomalies revealed kyphosis, ectopia cordis, and a large defect with protruding liver and bowel loops at 12 weeks and 3 days of gestational age on ultrasound scan. The other fetus was ultrasonographically normal. We diagnosed a case of pentalogy of Cantrell in a twin pregnancy after exclusion of limb body wall complex, body stalk anomaly, and amniotic band syndrome and after delivery of the fetuses. Macroscopic examinations were ectopia cordis, extrusion of the abdominal organs without membranes surrounding, and agenesis of the left limb.

Isolated asymptomatic short sternum in a healthy young girl.

Congenital sternal defects are rare deformities frequently associated with other anomalies of the chest wall and other organ systems. Although pectus excavatum, pectus carinatum, and cleft sternum can present as isolated deformity, in most cases they are associated with heart and inner organs anomalies and described as symptoms of syndromes like Marfan syndrome, Noonan syndrome, Poland anomaly, and Cantrell pentalogy. In contrast, the etiology of an isolated defect is not well understood. We observed a short sternum (dysmorphic manubrium, hypoplastic body, and complete absence of the xiphoid process) in a completely asymptomatic 13-year-old woman. A comprehensive instrumental exams panel was performed to exclude associated anomalies of the heart and of the other organ systems. The patient was completely asymptomatic and she did not need any medical or surgical treatment. To our knowledge, this is the first case of isolated short sternum reported in literature.

Complete Pentalogy of Cantrell: A case report and a review of literature

Pentalogy of Cantrell is a rare congenital malformation syndrome involving specific combination of ventral midline defects with ectopia cordis', as the most severe malformation. The complete form consists of five anatomical defects. We report a case of a one hour old female neonate delivered to a 27 year old primipara who presented with the complete form of the syndrome. She had surgery on the 4 th day of life, however died 36 hours 1 It is also known as Cantrell-Haller-Ravitch syndrome named after the initial authors. 2 The cardinal features of the syndrome were anterior abdominal defect (omphalocele) in association with ectopia cordis (EC) (evisceration of the heart). Toyama (1972) subsequently described the full spectrum of the Cantrell's syndrome to consist of ectopia cordis, a defect in the diaphragmatic pericardium, a deficiency of the anterior diaphragm, lower sternal defect, a midline supraumbilical abdominal wall defects, various structural congenital intracardial anomalies. 3 The precise cause of Pentalogy of Cantrell has remained elusive. One hypothesis that has enjoyed wide acceptance is that it results from a developmental failure of a segment of the lateral mesoderm at about the 14 th to 18 th days of intrauterine life, with subsequent failure in the development of the transverse septum of the diaphragm and the failure in the ventromedial migration of the paired mesodermal folds of the upper abdomen.

A Case of Cantrell's Syndrome with Left Ventricular Diverticulum in a Neonate

A Case of Cantrell's Syndrome with Left Ventricular Diverticulum in a Neonate

Cantrell Syndrome: A Rare Case Report

Cantrell syndrome is a rare syndrome of congenital defects involving the abdominal wall, sternum, diaphragm, pericardium and the heart. The spectrum of anomalies varies widely. Less than 160 cases have been described in the world literature. We reported a premature infant, with the syndrome. The case with a rare congenital malformation consisting of a pentad of findings: ectopia cordis and absent pericardium and a midline supraumbilical wall defect, evisceration of the intestines and liver, and short sternum. We presented this case because of its rarity and discuss the pathologic findings.

Ectopia cordis

We report on a preterm male newborn with complete ectopia cordis associated with Cantrell's syndrome. The neonate had an ectopia cordis involving defects of the lower sternum, supraumbilical abdominal wall, anterior portion of the diaphragm, and diaphragmatic portion of the pericardium associated with complex congenital heart defects. The infant died shortly after birth. We discuss this case and review the literature.

Pentalogy of Cantrell associated with exencephaly and spinal dysraphism: antenatal ultrasonographic diagnosis. Case report

The pentalogy of Cantrell, a rare syndrome, consists of the defects in the anterior diaphragm, diaphragmatic pericardium, lower sternum and supraumbilical abdominal wall, along with congenital cardiac abnormalities. Till date, only few patients with full spectrum of this syndrome have been reported with only 2 cases showing associated exencephaly and spinal dysraphism. We report extremely rare association of complete pentalogy of Cantrell syndrome with exencephaly and spinal dysraphism on antenatal sonogram and autopsy in a 18 weeks fetus of a 19 year-old primi gravida female.

Fontan operation for the Cantrell syndrome using a clamshell incision

A median sternotomy could be difficult for a child with ectopia cordis and complex congenital cardiac anomalies. We report a patient with ectopia cordis, functionally single ventricle and bilateral superior vena cava, who underwent a staged Fontan procedure through a clamshell incision and the sternothoracotomy approach.

Discordant anencephaly and Cantrell syndrome in monozygotic twins conceived by ICSI and IVF–ET

Discordant anencephaly and Cantrell syndrome in monozygotic twins conceived by ICSI and IVF–ET

Spectrum of Cantrell’s Pentalogy: Case Series From a Single Tertiary Care Center and Review of the Literature

The pentalogy of Cantrell (PC) was first described in 1958. It includes five anomalies: a deficiency of the anterior diaphragm, a midline supraumbilical abdominal wall defect, a defect in the diaphragmatic pericardium, various congenital intracardiac abnormalities, and a defect of the lower sternum. Five patients showing the PC spectrum are reported. The report presents the prenatal diagnosis, the postnatal course, and the patients' outcome at a tertiary care center from June 2001 to May 2012. A literature review and the management plan for this group of patients also are discussed. All patient data were obtained via electronic medical records retrospectively after approval by the institutional review board at the home institution. The patients in the study were three males and two females. For all of the patients, a prenatal diagnosis had been determined. The mean gestational age at delivery was 36.6 weeks. One patient had associated cranial and spine malformations. All the patients had associated congenital heart disease but a normal karyotype. Four of the five patients died in the first year of life. The ages at death ranged from 0 to 259 days (mean, 46.2 ± 51.8 days). The patients who did not survive had withdrawal of care due to increased morbidity, associated complications, or parental wishes. The pentalogy of Cantrell is a wide spectrum of associations. Patients with the complete PC together with complex congenital heart disease or extracardiac malformations may have a poor prognosis. Incomplete PC cases may have a better outcome based on associated anomalies. Prenatal counseling plays a very important role in the decision-making process for the families and has a significant impact on the postnatal management. A multidisciplinary team approach is essential for successful postpartum outcomes.

Transcatheter right ventricular outflow tract stent implantation in a child with pentalogy of cantrell, double outlet right ventricle, and severe pulmonary stenosis

We described a 5-week-old boy with dextrocardia, double outlet right ventricle, and severe pulmonary stenosis with pentalogy of Cantrell. There was a defect within the sternum, diaphragm, and a prominent left ventricular diverticulum. Hypercyanotic spells developed with desaturation to 50%. Two Vision coronary stents were implanted in the right ventricular outflow tract with a significant improvement in oxygen saturation to 85%. This represents the first report of transcatheter pulmonary stent implantation as a form of palliation in a child with pentalogy of Cantrell.

Prenatal diagnosis of pentalogy of Cantrell using three-dimensional ultrasound

In 1958, Cantrell et al [1] described a syndrome called the pentalogy of Cantrell (POC), consisting of deficiency of the anterior diaphragm, midline supraumbilical abdominal wall defect, defect in the diaphragmatic pericardium, congenital intracardiac abnormalities, and defect of the lower sternum. Although prenatal detection of POC has been reported previously, to date no prenatal illustration of POC by threedimensional (3D) ultrasound (US) has been described [2]. The application of 3D US in malformations, especially in the evaluation of small parts, appears to assist in prenatal diagnosis and to be clinically useful. The multiplanar planes proved most helpful in delineating the exact nature and anatomic level of the defect [3]. Here, we reported two cases of POC with different presentation diagnosed using two dimensional (2D) and three dimensional (3D) ultrasound (US). Case 1: A 19-year-old woman, gravida 1, para 0, was transferred to our hospital because of fetal congenital anomaly at 15 weeks of gestation. US revealed a crownerump length of 87.3 mm and the fetal heart outside the thorax by Doppler US. Omphalocele and marked spinal curvature were also observed (Fig. 1). After discussion with the patient and family, they decided on termination of the pregnancy. The thoracoabdominal ectopia cordis, left cleft sternum and abdominal wall defect, omphalocele, scoliosis, umbilical cord cyst, and polydactyly of the left hand were grossly observed in the arbutus (Fig. 2). The histopathologic examination at autopsy demonstrated the previously mentioned findings and left diaphragm deficiency with internal organs exposure. Microscopically, the internal organs were well developed. Moreover, the abortus revealed normal female karyotype. Case 2: A 32-year-old woman, gravida 1, para 0, was also transferred to our hospital for fetal abdominal wall defect at 13

Pentalogy of Cantrell diagnosed in the first trimester of pregnancy

Introduction: Pentalogy of cantrell is a rare syndrome of unknown etiology characterized by multiple structural congenital anomalies. case report: We aimed to present an early diagnosed case of pentalogy of cantrell accompanied by craniorachischisis. conclusion: Pentalogy of cantrell can be diagnosed with a careful ultrasonography in the first trimester. Potential side effects of pregnancy termination to the mother can be reduced by the early diagnose of the disease.

Syndromic exomphalos in Ile-Ife Nigeria: Management challenges

Background: Between 50% and 70% of children with exomphalos have other associated anomalies, which may involve the heart, abdominal viscera and urogenital organs resulting in high morbidity and mortality. The aim of this study is to review the management of patients with syndromic exomphalos in our center and to highlight the management challenges. Materials and Methods: We retrospectively reviewed the records of patients with pentalogy of cantrell, cloacal exstrophy and Beckwith-Wiedemann syndrome who presented to our unit between January 1997 and December 2007. Results: There were a total of 10 patients. Pentalogy of cantrell was the diagnosis in three patients while four of them had cloacal exstrophy and another three patients had Beckwith-Wiedemann syndrome. Their ages at presentation ranged between 2 h and 6 weeks, with a median of 27 h. Their birth weights ranged between 1.6 kg and 4.95 kg, median 2.45 kg. 5 patients (50%) had a low birth weight ( Conclusion: The management of congenital ventral body wall defects is challenging in resource poor settings like ours, due to lack of appropriate facilities and relevant manpower.