Pentalogy Of Cantrell Research Articles

A case report of ectopia cordis and omphalocele

A rare congenital defect in fusion of the anterior chest wall resulting in an extrathoracic location of the heart. Cantrell's pentalogy is a congenital anomaly resulting from embryologic development defect and consists of the following: A deficiency of the anterior diaphragm, a midline supraumbilical abdominal wall defect, a defect in the diaphragmatic pericardium, congenital intracardiac abnormalities, and a defect of the lower sternum. Here we report a rare case of ectopic cordis with omphalocele.

Pentalogy of Cantrell with Complete Ectopia Cordis in a Fetus with Asplenia

Cantrell's pentalogy (CP) is a rare, mainly sporadic spectrum of congenital midline thoracoabdominal defects that includes sternal anomalies, ventral diaphragmatic hernia, partial absence of the pericardium, supraumbilical abdominal wall defects, and congenital heart malformations. The approximate incidence is 1 in 100 000, with a 2∶1 male predominance. A 25-year-old pregnant woman was referred to the Prenatal Diagnosis Unit of the University Hospital of Padua for multiple congenital malformations at 21 weeks of gestation. A level 2 ultrasound scan was performed and confirmed the presence of multiple anomalies compatible with the diagnosis of CP associated with complete ectopia cordis. Fetal autopsy furthermore revealed asplenia, which usually presents as part of the heterotaxia spectrum. To our knowledge, an association of CP and complete ectopia cordis with asplenia has never been reported so far.

Isolated Complete Cleft

Cleft sternum is a rare congenital anomaly due to failure of fusion of the sternal bone. The etiology of this defect is not well understood. The anomaly is more common in females and may be associated with Cantrell's pentalogy. It can be partial or complete. An 8-year-old female child presented with complete defect in the sternum which was confirmed with a chest radiograph and computed tomography. Only 24 cases of isolated complete cleft sternum have been reported so far. The treatment ranges from primary closure of the defect to the use of various autologous and prosthetic grafts. Our patient was treated with primary closure of the defect.

Left Ventricle Diverticulum with Partial Cantrell's Syndrome.

Cantrell syndrome is a very rare congenital disease associating five features: a midline, upper abdominal wall disorder, lower sternal abnormality, anterior diaphragmatic defect, diaphragmatic pericardial abnormality, and congenital abnormalities of the heart. In this paper, we report a case of partial Cantrell's syndrome with left ventricular diverticulum, triatrial situs solitus, ventricular septal defect, dextrorotation of the heart, an anterior pericardial diaphragmatic defect, and a midline supraumbilical abdominal wall defect with umbilical hernia. The 5-month-old patient underwent a successful cardiac surgical procedure. A PTFE membrane was placed on the apex of the heart to facilitate reopening of the patient's chest. Postoperative course was uneventful. The patient was discharged with good clinical condition and with a normal cardiac function.

Prenatal diagnosis of Cantrell Pentalogy in first trimester screening: case report and review of literature

Pentalogy of Cantrell is a heterogeneous and rare thoraco-abdominal wall closure defect with the estimated prevalence of 1/65.000 to 1/200.000 births. Supraumbilical midline wall defect (generally omphalocele), deficiency of the anterior diaphragm and diaphragmatic peritoneum, defect of the lower sternum and several intracardiac defects are the components of Cantrell pentalogy. Etiology is unknown but a defect on the lateral mesoderm during the early stage of pregnancy is the most accepted hypothesis. Nowadays both 2- dimensional (2D) and 3-dimensional (3D) sonography are commonly used in diagnosis. In our case, a fetus with 11 weeks of gestation was reported as Cantrell pentalogy during first trimester screening. Additionally, unilateral limb defect and lumbar lordoscoliosis were detected through 3D sonography. Pregnancy was terminated according to parental desire. Karyotype was 46 XY. Early diagnosis is feasible in the first trimester if ectopia cordis and omphalocele exist. Additionally, development in ultrasound technology provides us with better visualization and early diagnosis. Prognosis seems to be poor in patients with complete Cantrell syndrome and patients with associated anomalies. Termination is the choice of treatment. Early diagnosis gives us a chance to reduce maternal morbidity and mortality related to termination.

Pentalogy or Hexalogy of Cantrell?

Pentalogy of Cantrell (PC) is a rare congenital syndrome involving the abdominal wall, sternum, diaphragm, pericardium, and heart. The embryonic period in which PC develops coincides with that of umbilical cord (UC) formation. The aim of the following study was to address the question of whether PC is associated with UC pathologies. Four cases, prenatally identified between 2002 and 2008, were enrolled in this study. Umbilical cord pathologies defined as single umbilical artery, short cord, or UC with atypical coiling pattern were retrospectively assessed on stored ultrasound images and from autopsy reports. The literature regarding PC and UC pathologies was reviewed. Three singleton pregnancies and 1 monoamniotic twin pregnancy with twin reversed arterial perfusion sequence were reviewed. All had a normal karyotype. Three showed the classical PC stigmata, with ectopia cordis. One fetus had no ectopia cordis; this case had a normal UC, whereas all others fetuses showed a short UC with atypical coiling pattern. Of 26 publications dealing with PC, the UC was described in only 8 cases, 7 of which were abnormal. There seems to be a strong correlation between the PC and UC abnormalities, in particular in cases with ectopia cordis. We speculate that the insult leading to the classical malformations of PC and UC abnormalities is the same or the sequence of malformations itself may alter the early fetoplacental blood flow and therefore the normal development of the UC angioarchitecture.

Craniorachischisis With A Variant Of Pentalogy Of Cantrell, With Lung Extrophy

A case of cranioraschischisis including incomplete pentalogy of Cantrell (PC) is described. The female fetus had a large omphalocele with evisceration of the heart, left lung, liver, stomach, and intestines accompanying anencephaly, cervical, thoracal lumbar, spina bifida. The fetus had ectopia cordis and diaphragmatic agenesia with an intact sternum. We present a case of a neonate with the stigmata for PC with the exception of a sternal defect. A literature review is also included. Sonographers should check for ventral and dorsal anomalies with PC because they may occur simultaneously.

Complete Cleft Sternum Associated With Clavicular Hypoplasia and Pectus Excavatum

A sternal cleft is a rare congenital and developmental disorder. In general, this disorder is associated with skeletal and extraskeletal abnormalities (eg, Cantrell syndrome), but isolated sternal clefts have also been reported. A sternal cleft with pectus excavatum is a rare congenital abnormality. We present a complete sternal cleft with right-sided clavicular hypoplasia and pectus excavatum in a 31-year-old female patient.

Cantrell syndrome with complex cardiac malformations: a case report

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.

Pentalogía de Cantrell en el primer gemelo de un embarazo gemelar monocigótico: presentación de un caso y revisión de la literatura

Introducción: la Pentalogía de Cantrell (PC) se compone de cinco anomalías: defecto superior de pared abdominal, defecto esternal inferior, defecto diafragmático anterior, defecto pericárdico diafragmático y anormalidades del corazón. Cerca de 250 casos han sido reportados, solo 6 involucran gemelos, y de estos 4 describen discordancia de esta anomalía.Objetivo: realizar la presentación de un caso de PC asociado a embarazo gemelar y la revisión de la literatura de esta asociación con énfasis en el diagnóstico prenatal y la etiología.Materiales y métodos: se presenta un caso de Pentalogía de Cantrell que afecta solo al primer gemelo de un embarazo gemelar monocigótico, se realizó una búsqueda de la información en las bases de datos SciELO y PubMed con la terminología MeSH "Pentalogy of Cantrell", "twin" y en español "Pentalogía de Cantrell" y "gemelar".Conclusión: la PC puede ser sospechada ecográficamente por ectopia cordis y defecto toracoabdominal. La presentación de un caso en un embarazo gemelar es una asociación poco frecuente.

Late diagnosis of incomplete Cantrell's syndrome on CT scan

MOTS CLES Syndrome de Cantrell ; Cardiopathie congenitale ; Scanner cardiaque A 45-year-old man with chronic myeloid leukaemia was referred for cardiac evaluation before allogeneic bone-marrow transplantation. His medical history included isolated right ventricle (RV) dilatation discovered 10 years earlier, with episodes of right heart failure induced by severe postchemotherapy anaemia. The electrocardiogram showed first-degree atrioventricular block, complete right bundle branch block and left atrial enlargement (Fig. 1). Chest radiographs showed cardiomegaly and large bowel images in the area anterior to the heart (Fig. 2). Transthoracic echocardiography showed isolated RV enlargement (53mm) with normal ejection fraction. Left ventricle (LV) dimension and ejection fraction were normal. The RV/LV diameter ratio was 1.2. No atrial or ventricular septal defects or pericardial abnormality was seen. Systolic pulmonary pressure estimation was 45mmHg. Pulmonary-to-systemic blood flow ratio (Qp/Qs) was 2.1 with no visible left-to-right shunt. Right heart catheterization showed normal pulmonary artery occlusion pressure (5mmHg), normal mean pulmonary pressure (18mmHg) and no sign of restrictive cardiomyopathy. Oximetric study demonstrated an oxygen saturation of 66% in the superior and inferior vena cava, 84% in the right atrium, 80% in the RV and 77% in the pulmonary artery. Qp/Qs was 1.6. A computed tomography scan showed the association of transdiaphragmatic hernia, partial apical agenesis of the pericardium, and sinus venosus atrial septal defect with abnormal pulmonary venous return (Fig. 3).

A variant of pentalogy of cantrell in a live birth

Pentalogy of Cantrell which usually comprises of anomalies of the ectodermal and mesodermal tissues is a very rare congenital condition which in the extreme of cases is incompatible with life. In this report a variant of the condition in a live newborn baby who presented to the University of Nigeria Teaching Hospital with abnormalities of the heart, urinary bladder and sternum is discussed. The aim of the report is to create an awareness and high index of suspicion amongst health professionals.

Ectopia cordis with tetralogy of Fallot in an infant with pentalogy of Cantrell: high-pitch MDCT exam

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.

Heart with a trunk: form fruste of Cantrell's Syndrome

A newborn presented with a prominent pulsatile supraumbilical tumour which was initially diagnosed as umbilical hernia by the caring paediatrician. On referral, clinical examination showed a subcutaneous pulse-synchronous pulsatile mass with diastasis of m. rectus abdominis ( Panel A ). Echocardiography demonstrated situs solitus with levocardia and a secundum atrial septal defect. The apex of the left ventricle (LV) continued into a funnel-like diverticulum running caudally and …

Cantrell syndrome

Cantrell syndrome

Extrathoracic heart in northern Cameroon: a case report

Sternal clefts, ectopia cordis, and Cantrell's pentalogy continue to be very rare congenital anomalies in pediatric surgery. The prenatal diagnosis is easily made with ultrasound by visualizing the heart outside the thoracic cavity. Ectopia cordis is frequently associated with other congenital defects involving multiple organ systems. We report a case of ectopia cordis with successful surgical correction on a 7 months old child from northern Cameroon.

Successful Vaginal Delivery of a Pregnant Woman 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.

Pentalogy of cantrell: A report of three cases

Pentalogy of Cantrell is a rare upper midline syndrome that may present in association with anomalies outside the torso. The pentad — the supraumbilical body wall defect, sternal defect, deficiency of the anterior diaphragm, defect of the diaphragmatic pericardium, and the intracardiac anomalies — was first described by Cantrell et al., in 1958. The defect is said to be more common in males, and survival is dependent on the cardiac malformations and on the degree of completeness of the syndrome. We report three cases of Cantrell's pentalogy managed in our unit. Two of the patients were females and one a male. All were seen at peripheral health centers before being referred to us. Age at presentation for the girls was 18 hours and 36 hours, respectively, the boy presented at the age of six weeks. All of their parents were unschooled manual workers. All patients presented with a defect in the supraumbilical body wall, bifid sternum, and a visible cardiac impulse. We were unable to do echocardiography to rule out intracardiac anomalies in the three patients. The thin membranous covering of the epigastrium in the female patients was managed conservatively. Both female patients were discharged against medical advice as requested by their parents, due to financial constraints. The male patient was lost to follow up after two clinic visits. A multidisciplinary approach to the management of this syndrome is recommended.

Pentalogy of Cantrell vs. Limb Body Wall Complex: Differential Diagnosis of a Severe Malformation in Early Pregnancy

The pentalogy of Cantrell (PC) and the limb body wall complex (LBWC) are both rare syndromes of midline defects which are usually clearly distinguishable.

Surgical treatment of three cases of Cantrell's syndrome

This ten-year retrospective study was designed to examine the morbidity and mortality of three cases of Cantrell's syndrome between 1998 and 2008. The three patients showed different degrees of Cantrell's pentalogy including abdominal ectopia cordis, thoracic-abdominal ectopia cordis and left ventricular diverticulum. Of the three, the 5-month-old boy suffering from complicated congenital heart disease with abdominal ectopia cordis received a successful single stage repair and reconstruction of the abdominal wall. The 33-week-old premature girl with thoracic-abdominal ectopia cordis underwent two stage correction of tetraology of Fallot. The 4-year-old girl underwent ectomy of left ventricular diverticulum and thoracoabdominal wall repair. Twenty-four to thirty-five months follow-up were satisfactory. We hold that two-stage repair are technically feasible for Cantrell's syndrome, especially for those with complex congenital heart diseases. Post-operative ventilatory support and multiple post-operative care should be prolonged. Malnutrition, infection and arrhythmia are central problems in medical care and surgery should be considered if there was progressive heart failure or hemodynamic instability.