Pentalogy of Cantrell

Williams, Arthur Robin; Marayati, Raoud; Beierle, Elizabeth A.

doi:10.1053/j.sempedsurg.2019.04.006

Cited by 39 publications

(34 citation statements)

References 41 publications

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“…Survival is related to the severity and complexity of heart malformations and EC location. [ 14 ] Late mortality is caused by complications of heart dysfunction, infections, or adhesive small bowel obstruction. [ 14 , 15 ] Due to the patient's preoperative unstable status and the heart's postoperative inability to adjust to the intrathoracic conditions, our patient died shortly after surgery.…”

Section: Discussionmentioning

confidence: 99%

The complete spectrum of pentalogy of Cantrell in one of a set of dizygotic twins

Zvizdić¹,

Sefić-Pašić²,

Mesic³

et al. 2021

Medicine

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Rationale: Pentalogy of Cantrell (POC) is an extremely rare syndrome with an estimated incidence of 1:65,000 to 200,000 live births. Its complete form includes a midline epigastric abdominal wall defect, defects affecting the lower sternum, anterior diaphragm, diaphragmatic pericardium, and various intracardiac defects. Patient concerns: We report a case of complete POC affecting only the first-born of a set of premature dizygotic twins. Diagnosis: A giant omphalocele with an eviscerated liver and bowel on prenatal, obstetric ultrasonography at 24 gestational weeks was observed. At birth, physical examination confirmed a massive (10 × 8 cm) epigastric omphalocele in which a significant part of the liver was seen. A postnatal echocardiogram revealed the presence of an ostium secundum atrial septal defect, perimembranous ventricular septal defect, and moderate pulmonary stenosis. X-ray showed an abnormal intrathoracic positioned stomach, which was confirmed with a plain x-ray of the upper intestinal tract with hydrosoluble contrast. Computed tomography (CT) scan revealed the sternum's absence and a close connection between the pericardial sac and the stomach wall. Interventions: The patient underwent surgical intervention at 18 days of age. Outcomes: Despite adequate and appropriate postoperative treatment, the baby rapidly deteriorated and died 72 hours after surgery. Lessons: POC is a complex, high-mortality syndrome whose management requires a multidisciplinary approach and meticulous planning. Despite all efforts, POC carries a poor prognosis, particularly in patients affected by its complete form.

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Section: Discussionmentioning

confidence: 99%

The complete spectrum of pentalogy of Cantrell in one of a set of dizygotic twins

Zvizdić¹,

Sefić-Pašić²,

Mesic³

et al. 2021

Medicine

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“…In this study, the two reported cases died, albeit for causes unrelated to the surgical procedure. Consequently, early defect closure has recently been considered a factor that increases the risk of death 21 .…”

Section: Discussionmentioning

confidence: 99%

Mortality in neonates with giant omphalocele subjected to a surgical technique in Barranquilla, Colombia from 1994 to 2019

Barrios-Sanjuanelo

Abelló-Munarriz

Cardona‐Arias

2021

Sci Rep

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No studies of the efficacy and safety of surgical techniques for the primary closure of giant omphalocele have been performed in Colombia. To determine the mortality rate and factors associated with mortality in neonates with giant omphalocele subjected to the surgical technique of early closure with a surgical silo described by Abello in Barranquilla, Colombia from 1994 to 2019. Retrospective cohort study of 30 neonates diagnosed with giant omphalocele and subjected to early closure of the defect. Medical history data were collected, information bias was controlled for, and descriptive statistical analysis was performed using Fisher’s exact test and the Mann–Whitney U test in SPSS 25.0. Of the patients in the cohort, 36.7% presented technique-related complications, 56.7% developed sepsis, 23.3% had low birth weight, 26.7% were preterm births, 43.3% had other malformations, 26.7% had congenital heart defects, and 13.3% presented pulmonary hypertension. The mean hospital stay was 26 days. The mortality rate was 16.7%; it was significantly higher among patients with other malformations, congenital heart defects, pentalogy of Cantrell and pulmonary hypertension. The Abello technique for the treatment of giant omphalocele showed a high neonatal survival rate and a low rate of procedure-related complications. The main factors associated with the death of neonatal patients were the presence of other malformations, congenital heart defect, pentalogy of Cantrell and pulmonary hypertension.

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“…Introducción J ames R. Cantrell definió esta pentalogía en 1958, las anormalidades incluyen: un defecto de la pared abdominal supraumbilical, la parte inferior del esternón, agenesia de la porción anterior del diafragma, ausencia de la cara diafragmática del pericardio y malformaciones cardiacas [1], [2]. La incidencia se estima de 1 en 200.000 nacimientos [3].…”

Section: Introductionunclassified

Bloqueo caudal más anestesia general balanceada en un paciente con pentalogía de Cantrell, presentación de un caso clínico

Olivera¹,

Llave²,

Perilla³

et al. 2021

Rev. chil. anest.

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The pentalogy of Cantrell is a disorder characterized by congenital abnormalities in the abdominal wall, lower sternum, anterior diaphragm, diaphragmatic pericardium, and cardiac anomalies. It is a rare disease with 250 cases registered around the world. The anesthetic implications will require a specialized management given the ventilatory mechanics and cardiac function which are compromised by the disease in the newborn. We present the case of a female patient with pentalogy of Cantrell without prenatal diagnosis, who had an operative procedure to correct patent ductus arteriosus and abdominal mesh placement under balanced general anesthesia with sevoflurane and fentanyl plus caudal block. This case is reported to provide our experience in the anesthetic management of this type of patients.

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Pentalogy of Cantrell

Cited by 39 publications

References 41 publications

The complete spectrum of pentalogy of Cantrell in one of a set of dizygotic twins

The complete spectrum of pentalogy of Cantrell in one of a set of dizygotic twins

Mortality in neonates with giant omphalocele subjected to a surgical technique in Barranquilla, Colombia from 1994 to 2019

Bloqueo caudal más anestesia general balanceada en un paciente con pentalogía de Cantrell, presentación de un caso clínico

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