Pentalogy of Cantrell.

Pentalogy of Cantrell is a constellation of five congenital defects that pose a unique challenge for surgeons. Defects of the heart, pericardium, diaphragm, sternum, and anterior abdominal wall are pathognomonic. Although the incidence is low, it is critical to identify it in a timely fashion in order to adequately address all aspects. Early diagnosis, supportive care, and strategic surgical planning with a multidisciplinary team are all key components in managing patients with Pentalogy of Cantrell. In this text we sought to explore the evolution of both the understanding and treatment for this complex entity and provide current recommendations to today's pediatric caregivers.

Incomplete Pentalogy of Cantrell--A Case Report.

Cantrell's Pentalogy is a rare congenital malformation consists of supraumbilical abdominal wall defect, defect in the lower part of sternum, agenesis of anterior portion of diaphragm, an absence of the diaphragmatic part of the pericardium and intracardiac malformation. This case report presents a female neonate, who was born at 40 weeks of gestation weighing 2400 gm and was admitted 4 hours after delivery with the complaints of something coming out from chest. On physical examination her vital signs were within normal limit, she had a systolic murmur on heart at lower left sternal area and there was a vascular structure present on the upper part of abdomen which was pulsatile and pulsation was synchronized with cardiac pulsation. On investigation chest X-ray lateral view showed absence of lower part of sternum, echocardiography findings were different in different institutes. Echocardiography findings at Mymensingh medical college hospital (MMCH) were large VSD (Ventriculo septal defect), ASD (Atrial septal defect) and rotated heart. On colour Doppler ultrasonogram at MMCH showed there were VSD, ASD, and a small epigastric swelling which was vascular and appears to be attached to the apex of the heart. On the other hand echocardiography findings of LAB AID hospital in Dhaka were Dextrocardia, complete AV (atrio ventricular) canal defect, almost common atrium, almost single ventricle, common AV valve, double outlet right ventricle (DORV), Cortriatriatum, mild A-V valve regurgitation and severe pulmonary hypertension (PAH). Echocardiography was also done at national heart foundation hospital in Dhaka. Findings were situs solitus, mesocardia to dextrocardia, DORV, large VSD, mild mitral inflow, mild TR (triuspid regurgitation), good LV (left ventricle) and RV (right ventricle) systolic function. Cardiologists at Dhaka in Bangladesh were suggested for surgery. Then the patient was consulted at Naryan Institute of Cardiac Science, Chennai in India. Here echocardiography findings were dextroversion/dextrocardia, DILV (Double inlet left ventricle), large inlet VSD with bidirectional shunt, mild TR, severe PAH with good ventricular function. Cardiologists in India were given comment about this patient. This patient was highly risky for surgery. They advised medical treatment and requested to review after one year. By taking medical treatment patient condition is well except failure to thrive and cyanosis develops during feeding and crying according to the statement of guardian of the patient. This case has 3 criterias among the five criteria of Cantrell's Pentalogy. So, it is incomplete Pentalogy of Cantrell.

Pentalogy of Cantrell: Case Report With Review of the Literature.

BACKGROUND: Pentalogy of Cantrell is a rare, congenital disorder characterized by lower sternal defects, diaphragmatic defect, pericardial defect, supraumbilical abdominal wall abnormalities, and/or intracardiac defects. The collective defects result from failure of either differentiation or migration of mesenchymal or mesodermal structures during the embryonic phase of development. Mortality of the disease complex is high, and treatment, when appropriate, revolves around surgical correction of the associated defects. PURPOSE: This article presents a case of pentalogy of Cantrell and examines the literature to report the most current evidence relative to the embryology and pathophysiology. In addition, the methods of pre- and postnatal diagnosis, management, and prognostic indicators are examined. METHODS/SEARCH STRATEGY: Case report was gathered from the medical records and is provided as it occurred. The literature was searched for evidence of best management strategies as well as care implications for families. FINDINGS/RESULTS: A female newborn was delivered at (Equation is included in full-text article.)weeks' gestation secondary to premature onset of labor. Prenatal ultrasonography identified an abdominal wall defect, diaphragmatic hernia, sternal defect, ventricular septal defect, and open neural tube defect. Examination immediately after delivery confirmed prenatal findings and a diagnosis of pentalogy of Cantrell was assigned. IMPLICATIONS FOR PRACTICE: Patients with the diagnosis of pentalogy of Cantrell should receive antenatal counseling relative to mortality and morbidity risks. An interprofessional approach in the immediate timeframe after delivery facilitates timely diagnostics and offers families prompt confirmation of antenatal findings. IMPLICATIONS FOR RESEARCH: Future research can focus on further elucidating genetic etiologies of pentalogy of Cantrell.

Pentalogy of Cantrell with ectopia cordis totalis, total anomalous pulmonary venous connection, and tetralogy of Fallot: a case report and review of the literature.

Pentalogy of Cantrell is a rare condition with a varied expression and a high mortality. We present a patient with the classic pentad (type 1), but with a previously undescribed constellation of cardiac manifestations including ectopia cordis totalis, total anomalous pulmonary venous return, and tetralogy of Fallot. This case reminds us of the challenges associated with the management of various forms of this condition. We discuss the prenatal diagnosis, genetic basis, postnatal evaluation, and management of this entity.

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.

Corrección de onfalocele gigante en un paciente con pentalogía de Cantrell / Giant omphalocele correction in a patient with pentalogy of Cantrell

La pentalogía de Cantrell es una rara malformación congénita caracterizada por un amplio defecto de la región inferior del esternón, el diafragma y la región anterior abdominal. Se caracteriza por la migración de órganos y su pronóstico depende de la presencia de malformaciones cardiacas. Presentamos el caso de un varón de 18 años sometido a corrección programada de un onfalocele gigante. Se empleó monitorización invasiva y del gasto cardiaco para evitar (como había sucedido en dos intervenciones previas) episodios de inestabilidad por aumento de la presión intraabdominal y disminución del retorno venoso. El pronóstico depende de múltiples factores; lo más importante es el buen control hemodinámico y respiratorio. A pesar de la monitorización avanzada, la mortalidad es muy alta(AU)

Pentalogy of Cantrell is a rare congenital malformation characterised by a large defect in the lower region of the sternum, diaphragm, and lower abdominal region. It is also characterised by the migration of organs, and its prognosis depends on the presence of cardiac malformations. We present the case of an 18 year-old male subjected to a scheduled giant omphalocele correction. Invasive monitoring, including cardiac output, was used to avoid episodes of instability due to the increase in abdominal pressure and the increase in venous return (as had happened on two previous operations). The prognosis depends on multiple factors, with good haemodynamic and respiratory control being the most important. Mortality is high despite the advances in monitoring(AU)

[Clinic diagnosis and treatment of patients with Cantrell syndrome].

OBJECTIVE: To analyze the diagnostic feature, treatment and prognosis of patients with Cantrell syndrome. METHODS: Clinical manifestation, diagnosis, operation and follow-up data of 5 patients with Cantrell syndrome were summarized in this retrospective analysis. RESULTS: The age of the 5 patients was 7 days-76 years, definite diagnosis was made in 3 cases and 2 cases presented feature of incomplete Cantrell syndrome. Three patients with full Cantrell syndrome were correctly diagnosed before operation and confirmed by operation. One patient with incomplete Cantrell syndrome (two-vessel stenosis) received bypass surgery. Another asymptomatic patient with incomplete Cantrell syndrome (apical diverticulum of the left ventricle) does not need operation and is under observation. During follow-up, 1 patient died at 60 months after operation and the remaining 4 patients are alive and well. CONCLUSIONS: With the development of modern imaging technology, it becomes easy to make correct diagnose Cantrell syndrome before operation. Prognosis is fine post timely operation and related intervention.