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

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.

scholarly journals Pentalogy of Cantrell: six new cases

2009 ◽  
Vol 150 (45) ◽  
pp. 2068-2073 ◽  
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.

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

2010 ◽  
Vol 21 (2) ◽  
pp. 235-237 ◽  
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.

scholarly journals Incomplete pentalogy of Cantrell: a case report

2018 ◽  
Vol 8 (1) ◽  
pp. 325
Author(s):  
Heera T. Shenoy ◽  
Prasanna Venugopal ◽  
Raghu S. ◽  
Remash K.
Keyword(s):  
Case Report ◽  
Abdominal Wall ◽  
Clinical Decision Making ◽  
Abdominal Wall Defect ◽  
Clinical Decision ◽  
Ectopia Cordis ◽  
Pentalogy Of Cantrell ◽  
Present Case Report ◽  
Cerebral Anomalies ◽  
Diaphragmatic Herniation

Pentalogy of Cantrell (PC) is a rare congenital anomaly characterized by a defect in the lower sternum, anterior diaphragm, and anterior abdominal wall; ectopia cordis; and congenital heart disease. Authors report a case of male foetus terminated at 20 weeks of gestation with an Incomplete (class 3) pentalogy of Cantrell presenting with gastroschisis, ectopia cordis and absence of lower sterna. Prognosis of pentalogy of Cantrell depends on severity of intra and extra cardiac defects, pulmonary hypoplasia, extent of abdominal wall defect, cerebral anomalies and diaphragmatic herniation. Full pentalogy of Cantrell is a severe and rare syndrome, but incomplete forms with combination of two or three defects are reported frequently similar to present case report.  Early diagnosis through obstetric ultrasound would help in improved informed clinical decision making on the part of the obstetrician and family.

scholarly journals First trimester diagnosis of body stalk anomaly complicated by ectopia cordis

2020 ◽  
Vol 48 (12) ◽  
pp. 030006052098021
Author(s):  
Yi Yang ◽  
Hong Wang ◽  
Zhenpeng Wang ◽  
Xuefeng Pan ◽  
Ying Chen
Keyword(s):  
Abdominal Wall ◽  
Umbilical Cord ◽  
Chromosomal Abnormalities ◽  
Abdominal Wall Defect ◽  
First Trimester ◽  
Nuchal Translucency ◽  
Ectopia Cordis ◽  
Wall Defect ◽  
Abdominal Organs ◽  
Increased Risk

Body stalk anomaly is a rare abnormality characterized by an abdominal wall defect with evisceration of abdominal organs, severe kyphoscoliosis, and a very short or absent umbilical cord. Ectopia cordis (EC) is a rare, lethal anomaly characterized by complete or partial malpositioning of the heart outside of the thorax. A 28-year-old healthy primigravida was referred to our department to undergo a nuchal translucency thickness scan at 12 weeks’ gestation. The scan revealed typical features of body stalk anomaly and EC. Given the lethal condition of the fetus, the patient opted for termination of the pregnancy. Body stalk anomalies, especially those complicated by EC, are universally lethal for the affected fetus. Selective termination should be recommended to avoid possible complications that can arise during pregnancy. Additionally, the future parents should be informed that because the condition is not associated with chromosomal abnormalities, there is no increased risk of recurrence.

scholarly journals Fetal Autopsy Showing Two Ventral Body Wall Defects: An Unusual Presentation

2020 ◽  
Author(s):  
Anupriya Kaur ◽  
Arun Prasad ◽  
Jessy Jayaraman Pushpaja ◽  
Kanchan Kapoora
Keyword(s):  
Abdominal Wall ◽  
Body Wall ◽  
Abdominal Wall Defect ◽  
Lower Lobe ◽  
The Body ◽  
Unusual Presentation ◽  
Thoracic Wall ◽  
Fetal Autopsy ◽  
Wall Defect ◽  
The Right

Thoracoschisis and gastroschisis are types of ventral body wall defects. Usually thoracoabdominoschisis presents as a continuous defect. Here the authors present the case of autopsy of 18 weeks old fetus with two well-separated ventral body wall defects. Thoracoschisis was a right lateral thoracic wall defect while gastroschisis, a midline infraumblical abdominal wall defect. Through the right lateral thoracic wall parts of two organs appeared to be protruding. On dissection, 8th and 9th ribs with the liver and the lower lobe of the right lung were herniating outside the body. The right lung was haemorrhagic. Various embryological theories on ventral body wall defects have been discussed in view of the present case.

scholarly journals Caesarean Scar Endometriosis May Require Abdominoplasty

2021 ◽  
Vol 14 ◽  
pp. 117954762110276
Author(s):  
Barczyński Bartłomiej ◽  
Sobstyl Małgorzata ◽  
Frąszczak Karolina ◽  
Sobstyl Anna
Keyword(s):  
Surgical Treatment ◽  
Abdominal Wall ◽  
Abdominal Wall Defect ◽  
Uterine Cavity ◽  
Tumour Resection ◽  
Wall Defect ◽  
Scar Endometriosis ◽  
Caesarean Scar ◽  
Hernia Prevention

Endometriosis is defined as an ectopic presence of endometrium-like tissue outside uterine cavity, which most commonly involves intraperitoneal organs. However, one of the less frequent forms of the disease is abdominal wall endometriosis usually developing in surgical scars following obstetric and gynaecological surgeries involving uterine cavity entering, that is, caesarean section, myomectomy or hysterectomy. In this case report we present a case of a patient with extensive caesarean scar endometriosis, who required complex surgical management. Successful surgical treatment involved not only radical tumour resection and application of mesh in postoperative hernia prevention but also adequate wound closure ensuring satisfactory cosmetic results, which was most challenging. The abdominal wall defect could not be sutured by traditional technique, thus polypropylene mesh was used and partial abdominoplasty was performed. The wound healed without complication and 24-month follow-up showed no evidence of local recurrence and satisfactory cosmetic result. In case of extensive endometrial abdominal wall tumours surgical treatment may involve application of advanced plastic surgery techniques, like abdominoplasty or skin/musculocutaneous flaps transposition.

Surgical treatment of inguinal eventration in argentine terrestrial turtle (Chelonoidis chilensis) – case report

2020 ◽  
Vol XXV (144) ◽  
pp. 46-50
Author(s):  
Juan Carlos Troiano ◽  
Diego Federico Blanco ◽  
Matias Eugenio Sclocco ◽  
Francisco Antonio Bava
Keyword(s):  
Case Report ◽  
Surgical Treatment ◽  
Abdominal Wall ◽  
Abdominal Wall Defect ◽  
Coelomic Cavity ◽  
Intact Skin ◽  
Wall Defect ◽  
Posterior Limb ◽  
Left Posterior ◽  
The Right

Eventration is a separation of the musculoaponeurotic layers of the abdominal wall resulting in protrusion of abdominal contents, and characterized by the absence of the hernial sac and intact skin. We describe a case of chronic left sided eventration associated with deformity of the right femoral fossa in a 30-year-old female Argentine land tortoise. The animal was referred for evaluation of a soft and non-painful deformity of the left lumbar fossa and third degree lameness of the left posterior limb. Eventration of one bladder lobe and oviduct loops was diagnosed by ultrasonography. We describe the anesthetic protocol and the surgical technique used in the treatment with approach to the coelomic cavity through the prefemoral fossa, as well as the resolution of the abdominal wall defect.

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

2020 ◽  
Vol 12 (3) ◽  
pp. 227-230
Author(s):  
Andrea Karina Muñoz Mora ◽  
María Soledad Ordóñez Velecela ◽  
Luis Enrique Marcano Sanz ◽  
Miurkys Endis Miranda
Keyword(s):  
Abdominal Wall ◽  
Abdominal Wall Defect ◽  
Unique Challenge ◽  
Left Liver Lobe ◽  
Pentalogy Of Cantrell ◽  
Wall Defect ◽  
Mortality Case ◽  
History Of ◽  
Cantrell's Pentalogy ◽  
High Level

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.

scholarly journals Original method of the frontal abdominal wall defect reconstruction using the principle of the reconstructive-restorative surgery

2021 ◽  
Vol 11 (1) ◽  
pp. 372-378
Author(s):  
I. P. Khomenko ◽  
E. V. Tsema ◽  
V. Yu. Shapovalov ◽  
S. V. Tertyshny ◽  
P. P. Yermuraki
Keyword(s):  
Surgical Treatment ◽  
Abdominal Wall ◽  
Anterior Abdominal Wall ◽  
Abdominal Wall Defect ◽  
Infrared Camera ◽  
Patient Treatment ◽  
Wall Defect ◽  
Surgical Infection ◽  
Defect Reconstruction ◽  
Small Intestinal

The author srepresent the original clinical data devoting to the further progress in reconstructive surgery. The methodology of the staged anterior abdominal wall defect reconstruction using the Keystone method was used firstly.The aim of the work is to demonstrate the peculiarity of preoperative preparation of patients with the anterior abdominal wall wounds complicated by small intestinal fistulas.The article uses the clinical materials of the concrete patient treatment in the Department of surgical infection of the South Region Military Medical Clinical Centre (Odessa, Ukraine) using the principle of the reconstructive-restorative surgery.The clinical case of the successful surgical treatment of anterior abdominal wall eventration, resulting from numerous laparotomies with pronounced lateralization of the wound, has shown that the solution to this problem should be based on the principles of reconstructive surgery.Preoperative modelling of full-layer flaps using a combined technique (infrared camera FLIR and portable Doppler SONO Trax) reduces the risk of necrosis of the movable flap and accelerates the process of closing of the defect of the anterior abdominal wall. Staged (“step-by-step”) surgical treatment of the postoperative wound of the anterior abdominal wall by reconstruction via to the Keystone method showed a positive and rapid result of solving such a difficult problem as eventration complicated by small intestinal fistula.Restoration of the integrity of the anterior abdominal wall should be considered from the standpoint of reconstructive-restorative features

scholarly journals Pentalogy of Cantrell: A Rare Multiple Congenital Malformation Syndrome

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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