Incomplete pentalogy of Cantrell: single stage repair

Usha, Manoharan; Mahimaiha, Jayaranganath; Shivanna, Devananda Nijagal

doi:10.1136/heartjnl-2011-301510

Cited by 5 publications

(2 citation statements)

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“…Both multi-stage as well as single stage surgical repairs have been described and adopted in complete and incomplete syndromes of Pentalogy of Cantrell (20)(21)(22)(23)(24)(25). The basic principle in unstable patients is to focus on saving the child's life and thus a multi-stage operation may be preferable.…”

Section: Initial Management and Surgical Repairmentioning

confidence: 99%

Pentalogy of Cantrell

Williams

Marayati

Beierle

2019

Seminars in Pediatric Surgery

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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 of this defect is small, 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.

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Section: Initial Management and Surgical Repairmentioning

confidence: 99%

Pentalogy of Cantrell

Williams

Marayati

Beierle

2019

Seminars in Pediatric Surgery

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“…Milder forms of the abnormality exist; class 2 disease comprises four defects, including intracardiac and ventral wall abnormalities, and class 3 comprises a sternal defect and various degrees of expression of the other anomalies (52,62). Patients with less severe forms of the disease have a better prognosis and may undergo single or staged surgical repair, depending on the constellation of findings (63)(64)(65)(66).…”

Section: Pentalogy Of Cantrellmentioning

confidence: 99%

Complex Abdominal Wall Defects: Appearances at Prenatal Imaging

Pakdaman

Woodward²,

Kennedy³

2015

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Abdominal wall defects are a complex group of anomalies, and many are incorrectly diagnosed. Evaluation of the defect relative to the umbilical cord insertion site is fundamentally important in differentiating among the various malformations. The two most common abdominal wall defects are gastroschisis, in which the defect is on the right side of the normally inserting cord and free-floating bowel loops are present, and omphalocele, in which the cord inserts on a membrane-covered midline defect. Omphalocele may also form a portion of a more complex defect that may remain undiagnosed without thorough evaluation. In cloacal exstrophy, the defect extends inferiorly and the bowel loops extrude between the two bladder halves. In pentalogy of Cantrell, the defect extends superiorly and is typically associated with ectopia cordis. Bladder exstrophy is a lower abdominal defect in which the hallmark finding is absence of a fluid-filled bladder. The cord insertion site is normal to low but does not form part of the defect. Both body stalk anomaly and abdominoschisis due to amniotic bands cause severe malformations, often involving extrusion of solid organs and the bowel. Although these two entities have many overlapping features, body stalk anomaly may be recognized on the basis of absence of a free-floating umbilical cord. With use of an algorithmic approach beginning with discovery of the location of the defect, a more precise diagnosis can be determined that may directly affect pre- and postnatal management decisions.

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

2013

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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.

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Incomplete pentalogy of Cantrell: single stage repair

Cited by 5 publications

References 1 publication

Pentalogy of Cantrell

Pentalogy of Cantrell

Complex Abdominal Wall Defects: Appearances at Prenatal Imaging

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

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