A propósito de un caso: Resolución quirúrgica en dos tiempos de  Pentalogía de Cantrell

BACKGROUND: Cantrell’s pentalogy includes the presence of five birth defects that represent a great challenge for surgeons. Abnormalities of the heart, pericardium, diaphragm, sternumand anterior abdominal wall are the main findings. Its incidence is low, however, it is essential to identify Cantrell´s pentalogy timely to adopt an adequate therapy for all specific defects, since it has high mortality. CASE REPORT: The patient was a full- term male newborn, with a history of abdominal wall defect compatible with an omphalocele detected by prenatal ultrasound. After the caesarean section, the abdominal wall defect was notable, the left liver lobe, intestines and heart emerged through it, the sternum also had a low fissure. The echocardiogram revealed a permeable oval foramen, mild tricuspid regurgitation, and severe pulmonary hypertension. EVOLUTION: Immediate surgical management was decided. Silo was placed, with progressive closure of the midline in 7 days. During the second surgical procedure, the diaphragmatic and pericardial defect was corrected with a bovine pericardial prosthesis. Despite the adequate evolution after surgery, at day 28 he presented with sudden cyanosis and didn’t respond to cardiopulmonary resuscitation and died. CONCLUSIONS: Cantrell’s Pentalogy is a rare disease, with peculiar clinical, anatomical and embryological characteristics, it represents a unique challenge for surgeons. Early diagnosis, as well as follow-up during pregnancy, planning a cesarean section in a high-level center and immediate surgical approach with a multidisciplinary team, are the key components in the management of patients with Cantrell’s Pentalogy. KEY WORDS: PENTALOGY OF CANTRELL, ECTOPIA CORDIS, UMBILICAL HERNIA. CONGENITAL.

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Pentalogy of Cantrell: six new cases

Orvosi Hetilap ◽

10.1556/oh.2009.28744 ◽

2009 ◽

Vol 150 (45) ◽

pp. 2068-2073 ◽

Cited By ~ 1

Author(s):

Barbara Pete ◽

Júlia Hajdú ◽

Ágnes Harmath ◽

Zsolt Csapó ◽

Csaba Papp ◽

...

Keyword(s):

Abdominal Wall ◽

Diaphragmatic Hernia ◽

Detection Rate ◽

Abdominal Wall Defect ◽

Ectopia Cordis ◽

Pentalogy Of Cantrell ◽

Wall Defect ◽

Prenatal Detection ◽

Cantrell's Pentalogy ◽

Prenatal Detection Rate

Cantrell’s pentalogy is a rare multiple malformation syndrome consisting of supraumbilical abdominal wall defect, sternal defect, pericardial defect, anterior diaphragmatic defect and heart malformation. Aims and methods: Prenatal ultrasound findings and malformations described during autopsy of the Cantrell’s pentalogy cases diagnosed between January 1991 and June 2009 in our institute were reviewed. A literature research was conducted to analyze the prevalence and prenatal detection rate of the five previously described malformations and ectopia cordis in the Cantrell’s pentalogy cases. Results: Six cases of Cantrell’s pentalogy were diagnosed during the study period in our department. Sonography detected multiple malformations in 3 cases, and isolated malformation in 3 cases. Analyzing the data of 49 Cantrell’s pentalogy cases altogether showed that, beside abdominal wall defect and ectopia cordis which had the highest prenatal detection rate (83% and 91% with a prevalence of 94% and 69%, respectively), sternal defect and anterior diaphragmatic hernia were also present in a large number of the cases (80% and 73% respectively). Conclusion: Sonographic identification of the sternal defect or diaphragmatic hernia may help to differentiate Cantrell’s pentalogy from malformations part of the syndrome but occurring as isolated defects.

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Managing a Colonoscopic Perforation in a Patient with No Abdominal Wall

Case Reports in Surgery ◽

10.1155/2018/7175381 ◽

2018 ◽

Vol 2018 ◽

pp. 1-4

Author(s):

Jayan George ◽

Michael Peirson ◽

Samuel Birks ◽

Paul Skinner

Keyword(s):

Abdominal Pain ◽

Incisional Hernia ◽

Plastic Surgery ◽

Abdominal Wall ◽

Abdominal Wall Defect ◽

Colonic Perforation ◽

Conservative Approach ◽

Wall Defect ◽

Surgical History ◽

History Of

We describe the case of a 37-year-old gentleman with Crohn’s disease and a complex surgical history including a giant incisional hernia with no abdominal wall. He presented on a Sunday to the general surgical on-call with a four-day history of generalised abdominal pain, nausea, and decreased stoma output following colonoscopy. After CT imaging, he was diagnosed with a large colonic perforation. Initially, he was worked up for theatre but following early senior input, a conservative approach with antibiotics was adopted. The patient improved significantly and is currently awaiting plastic surgery input for the management of his abdominal wall defect.

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Pentalogy of Cantrell with double-outlet right ventricle: a case of surgical correction

Cardiology in the Young ◽

10.1017/s1047951110001770 ◽

2010 ◽

Vol 21 (2) ◽

pp. 235-237 ◽

Cited By ~ 4

Author(s):

Yoshio Ootaki ◽

James Strainic ◽

Ross M. Ungerleider

Keyword(s):

Cardiac Surgery ◽

Right Ventricle ◽

Abdominal Wall ◽

Abdominal Wall Defect ◽

Double Outlet Right Ventricle ◽

Total Correction ◽

Cardiac Defects ◽

Pentalogy Of Cantrell ◽

Wall Defect ◽

Defect Repair

AbstractPentalogy of Cantrell is characterised by a combination of severe defects in the middle of the chest including the sternum, diaphragm, heart, and abdominal wall. Mortality rate after cardiac surgery is usually high. We report a successful total correction of the cardiac defects in a case of Pentalogy of Cantrell with a double-outlet right ventricle prior to abdominal wall defect repair.

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Cantrell’s pentalogy: long-term survival and repair of a large abdominal wall defect

International Journal of Pregnancy & Child Birth ◽

10.15406/ipcb.2018.04.00101 ◽

2018 ◽

Vol 4 (3) ◽

Author(s):

Grecia Victoria Vivas Colmenares

Keyword(s):

Abdominal Wall ◽

Abdominal Wall Defect ◽

Term Survival ◽

Wall Defect ◽

Long Term Survival ◽

Cantrell's Pentalogy

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MAIN PRINCIPLES OF ETIOPATHOGENETIC TREATMENT OF NEWBORNS WITH GASTROSCHISIS

Neonatology surgery and perinatal medicine ◽

10.24061/2413-4260.xi.1.39.2021.1 ◽

2021 ◽

Vol 11 (1(39)) ◽

pp. 6-12

Author(s):

Olga Gorbatyuk ◽

K.T. Bertsun ◽

O.S. Rubina ◽

R.O. Gomon

Keyword(s):

Early Diagnosis ◽

Abdominal Wall ◽

Abdominal Wall Defect ◽

Anesthetic Management ◽

Surgical Correction ◽

Intraabdominal Pressure ◽

Preoperative Preparation ◽

Wall Defect ◽

Intraabdominal Hypertension ◽

High Level

Introduction. The paper focuses on the challenging issue of treatment of newborns with gastroschisis (GS) and a high level of intraabdominal hypertension in case of severe viscero-abdominal disproportion. The aim of the paper is to present the current state of the problem at the regional level based on our own clinical experience in observation and treatment of newborns with GS. Material and methods. The study is based on the analysis of examination and treatment of 29 newbornswith GS. The distribution of boys and girls was nearly the same: 16 (55.17%) boys and 13 (44.83%) girls. The number of premature babies was 23 (79.31%). The complex of diagnostic procedures included clinical laboratory examination, radiologic investigation (using contrast agents when required), ultrasound investigation and Doppler sonography, intraabdominal pressure measurement, histologic study of surgical specimens.Results. Prenatal information on the congenital anterior abdominal wall defect was obtained with the help of prenatal ultrasound. In most cases, fetal GS is diagnosed before 20 weeks of pregnancy. Prenatal ultrasound was performed in 13 (44.83%) pregnant women out of the researched group. Two false test results were obtained.Radiologic investigation of a child with GS is necessary to detect intestinal obstruction, necrosis or bowel perforation. Among patients under our study, two cases of ileal atresia were registered. The analysis of intraabdominal pressure indices in patients with GS showed a high level of intraabdominal hypertension in approximately 70% of cases. It proves that patients with GS have a high level of surgical and anesthetic risk. The main therapy measures include the following steps: appropriate preoperative preparation, anesthetic management, and the choice of appropriate perioperative techniques taking into account the level of intraabdominal hypertension and viscero-abdominal disproportion.Depending on the child's condition, type of GS and level of intraabdominal hypertension, there were twosurgical options:1. Primary radical surgery.2. Staged surgical treatment.Analyzing the results of the study, it may be noted that 8 newborns in total died after the surgery, which is 27.59%(it used to be 56-60% till 2005). The main causes of death were respiratory failure, neonatal sepsis and unfavorable premorbid conditions (very low gestational age, severe comorbidity, multiple congenital malformations).Conclusions. 1. Successful treatment of newborns with GS depends on the early diagnosis of the pathology, which must be prenatal, and elimination of contradictions in treatment tactics. 2. Before suturing the abdominal wall defect in newborns with GS, it is necessary to measure intraabdominal pressure, since intraabdominal hypertension leads to a significant deterioration in the mechanical properties of the lungs, hemodynamic abnormalities,oliguria, intestinal ischemia, decrease in organ perfusion, which must be taken into account when carrying out preoperative preparation and aesthetic management. 3. The level of intraabdominal hypertension in patients with GS,which is high in 68.96% of cases, must be an indication for choosing the method of surgical correction of the defect i.e.the refusal of radical plasty of the abdominal wall and the resort to staged intervention or other surgical techniquesthat involve an increase in the abdominal cavity volume. 4. The reduction in mortality in newborns with GS to 27.59%is possible due to the introduction of etiopathogenetic approaches to early diagnosis, preoperative management, anesthetic management and surgical correction of this pathology into the practice of neonatal surgery.

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The modern methods of X-Ray based diagnostic in cases of ectopia cordis associated with pentalogy of Cantrell

Pediatrician (St Petersburg) ◽

10.17816/ped8492-98 ◽

2017 ◽

Vol 8 (4) ◽

pp. 92-98

Author(s):

Elena V. Kosovtsova ◽

Alexandr V. Pozdnyakov ◽

Nikolay G. Pilyugov ◽

Alexey B. Naumov ◽

Sergey P. Marchenko

Keyword(s):

Surgical Treatment ◽

Abdominal Wall ◽

Abdominal Wall Defect ◽

The Body ◽

Ectopia Cordis ◽

Pentalogy Of Cantrell ◽

Wall Defect ◽

X Ray ◽

Septal Defects ◽

Collateral Arteries

Pentalogy of Cantrell with ectopia cordis is an extremely rare and lethal congenital anomaly included congenital hearth disease (CHD), midline supraumbilical abdominal wall defect, defect of lower sternum part, deficiency of the anterior diaphragm, a defect in the diaphragmatic pericardium. The complexity of this syndrome is usually incompatible with life. Depending on the location of the protruding heart and on the extent of the body wall defect, ectopia cordis may be grouped into cervical, thoracic, thoracoabdominal, or abdominal types. The heart mostly uncovered, covered with a serous membrane less often, and covered with skin rare. Depending on combination defects pentalogy of Cantrell classified for 3 different classes by Toyama (classified in 1972): 1 class – complete syndrome (all five defects), 2 class – probable syndrome (included intracardial defect and ventral abdominal wall defect) 3 class – incomplete syndrome (with various combinations of defects present, including a sternal abnormality). Presented clinical case newborn pentalogy of Cantrell first Toyama class. We used X-Ray for firstly diagnostic, for visualization CHD was performed CT. For postnatal diagnostic CHD are used Echo and CT. CT allows graphically to see the type of CHD and predict a surgical treatment. The strategy of surgical treatment and further prognose are depend on combination of anomalies within the framework of pentalogy of Cantrell. By CT we determined atresia of the pulmonary artery with major aorto-pulmonary collateral arteries (MAPCA), ventricular and atrial septal defects.

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Pyloric Stenosis after Gastroschisis Closure

The American Surgeon ◽

10.1177/000313481307900925 ◽

2013 ◽

Vol 79 (9) ◽

pp. 896-896

Author(s):

Stephanie M. Streit ◽

Jennifer A Dixon ◽

André Hebra

Keyword(s):

Abdominal Surgery ◽

Abdominal Wall ◽

Pyloric Stenosis ◽

Abdominal Wall Defect ◽

Hypertrophic Pyloric Stenosis ◽

Wall Defect ◽

Birth History ◽

History Of ◽

Normal Gestation

Hypertrophic pyloric stenosis is a very common surgical problem in infants. It occurs most often in otherwise well babies with normal gestation and birth history. Rarely, pyloric stenosis has been described in babies with history of prior abdominal surgery. Below we discuss the management of hypertrophic pyloric stenosis in a child who remained hospitalized, recovering from repair of a congenital abdominal wall defect.

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Pentalogy of Cantrell: A Rare Multiple Congenital Malformation Syndrome

Journal of SAFOG with DVD ◽

10.5005/jp-journals-10006-1360 ◽

2015 ◽

Vol 7 (3) ◽

pp. 210-212

Author(s):

Balwinder Kaur ◽

Parneet Kaur ◽

Khushpreet Kaur ◽

Princy Mittal

Keyword(s):

Abdominal Wall ◽

Congenital Malformation ◽

South Asian ◽

Abdominal Wall Defect ◽

Cardiac Defects ◽

Pentalogy Of Cantrell ◽

Wall Defect ◽

Malformation Syndrome ◽

Congenital Cardiac Anomalies ◽

Congenital Malformation Syndrome

ABSTRACT Pentalogy of Cantrell is a rare multiple congenital malformation syndrome characterized by combination of five features: abdominal wall defect, defect of lower sternum, defect of diaphragmatic pericardium, defect of anterior diaphragm and congenital cardiac anomalies. These defects can be diagnosed as early as 1st trimester of pregnancy. The complexity of these anomalies, in particular the presence of cardiac defects, determines the management as well as prognosis. How to cite this article Kaur P, Kaur K, Kaur B, Mittal P. Pentalogy of Cantrell: A Rare Multiple Congenital Malformation Syndrome. J South Asian Feder Obst Gynae 2015;7(3):210-212.

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