pentalogy of Cantrell


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pen·tal·o·gy of Can·trell

(kan-trel'),
a congenital defect involving a cleft lower sternum, an anterior diaphragmatic defect, absence of the parietal pericardium, a connected or separate omphalocele, and a major cardiac anomaly, most often tetralogy of Fallot and left ventricular diverticulum.

pentalogy of Cantrell

[pen·tal′ə·jē kan·trel]
Etymology: Gk, pente, five + logos, word; James R. Cantrell, American physician, 20th century
a cleft in the inferior part of the sternum associated with midline abdominal defects such as omphalocele and defective pericardium and diaphragm with communication between the pericardial and peritoneal cavities and with cardiac anomalies such as ventricular septal defect or, less often, atrial septal defect, tetralogy of Fallot, or left ventricular diverticulum.
References in periodicals archive ?
Pentalogy of Cantrell (POC) is a congenital rare disorder characterized by several birth defects in the chest and abdominal area.
The condition encompasses variably sized 'simple' abdominal wall defects with no associated abnormalities, to those associated with a spectrum of congenitally acquired abnormalities that include BeckwithWiedemann syndrome, 'omphalocoele, extrophy, imperforate anus, spinal' (OEIS) complex and the pentalogy of Cantrell.
Emma's daughter, Milly, was born with a very rare condition called Pentalogy of Cantrell - a condition characterised by a combination of defects involving the diaphragm, abdominal wall, pericardium (the thin membrane that lines the heart), lower sternum and the heart.
sup][3],[5] Some syndromes associated with exomphalos are nonchromosomal disorders such as Beckwith–Wiedemann syndrome, pentalogy of Cantrell, Charge syndrome, Marshall–Smith syndrome, Goltz syndrome, fetal valproate syndrome, and chromosomal genetic disorders such as trisomies 13, 14, 15, 18, or 21.
Pentalogy of Cantrell can be caused by vascular dysplasia, as well as mechanical teratogens, genetic mutations or a viral infection in the first trimester of pregnancy [4].
Keywords: Pentalogy of Cantrell, Ectopia cordis, midline developmental anomalies
Pentalogy of Cantrell and amniotic bands: A case report and review of the literature
Se hizo busqueda en las bases de datos Medline via PubMed, Ovid, Hinari y Fabumed, utilizando las palabras clave: abdominal wall defects; omphalocele, gastrosquisis, diapragmatic hernia; OEIS complex, limb-body-wall complex; pentalogy of Cantrell.
A predominantly cephalic defect results in a constellation of anomalies known as pentalogy of Cantrell.
If EC associated with deficiency of anterior diaphragm, midline supraumbilical abdominal wall defect, defect in diaphragmatic pericardium, various congenital intracardiac abnormalities, and defect of lower sternum, called pentalogy of Cantrell (PC) with an estimated incidence of 1/65.
Key Words: omphalocele, non-invasive ventilation, continuous positive airway pressure, Pentalogy of Cantrell, tracheostomy, paediatrics