scholarly journals Incomplete pentalogy of Cantrell: a case report

Author(s):  
Heera T. Shenoy ◽  
Prasanna Venugopal ◽  
Raghu S. ◽  
Remash K.

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.

2017 ◽  
Vol 8 (4) ◽  
pp. 92-98
Author(s):  
Elena V. Kosovtsova ◽  
Alexandr V. Pozdnyakov ◽  
Nikolay G. Pilyugov ◽  
Alexey B. Naumov ◽  
Sergey P. Marchenko

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.


2009 ◽  
Vol 150 (45) ◽  
pp. 2068-2073 ◽  
Author(s):  
Barbara Pete ◽  
Júlia Hajdú ◽  
Ágnes Harmath ◽  
Zsolt Csapó ◽  
Csaba Papp ◽  
...  

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.


Microsurgery ◽  
2018 ◽  
Vol 39 (2) ◽  
pp. 174-177 ◽  
Author(s):  
Annika Senghaas ◽  
Thomas Kremer ◽  
Volker J. Schmidt ◽  
Leila Harhaus ◽  
Christoph Hirche ◽  
...  

Microsurgery ◽  
2017 ◽  
Vol 39 (1) ◽  
pp. 85-90 ◽  
Author(s):  
Dong Yeon Kim ◽  
Junho Lee ◽  
Jeong Tae Kim ◽  
Hye Kyung Chang ◽  
Suk-Ho Moon

2019 ◽  
Vol 6 (38) ◽  
pp. 2603-2605
Author(s):  
Ritu Saloi ◽  
Roonmoni Deka ◽  
Santanu Kumar Sarma

2021 ◽  
Vol 10 (8) ◽  
pp. 550-554
Author(s):  
Rajiv Dharampal Bhola ◽  
Sweta Gajanan Kale Pisulkar ◽  
Surekha Anil Dubey Godbole ◽  
Hetal Satish Purohit ◽  
Anjali Bhoyar Borle

Combined intra and extra oral defects can be stated as those facial defects which have an intraoral communicating route. Midfacial defects are aptly classified into 2 major categories by Marunick et al. 1 as midline midfacial defects in which the nose and / or upper lip defects are included; and the second major group was lateral defects in which the cheek and orbital defects are categorized. However, defects which include combinations of the above-mentioned defects are in existence. Midfacial defects which are acquired, present themselves often with severe disfigurement of structures and hence show impaired function. It is a meticulous task to rehabilitate the defects which are caused as a result of cancerous lesion resection as they are huge. Such post resection lesions frequently are rehabilitated by a facial prosthesis to maintain function as well as the appearance in the normal form. In adjunction to the facial prosthesis, an intraoral prosthesis which constitutes of an obturator is also required to regain the natural speech and pattern of swallowing. Fabrication of such facial prosthesis not only requires the artistic capability but also excellent clinical decision making of the prosthodontist. Mode of retention of the combined prosthesis should also be kept in mind while fabricating as it is also a difficult task to retain them because of the size and weight of the same. Moreover the prosthesis should also be secured in its place with these aids which can also prove as a challenge. This case report states rehabilitating a large surgically resected midfacial defect with the assistance of a “3-piece prosthesis” which constitutes a sectional intraoral obturator along with maxillary and mandibular extraoral facial prosthesis.


2021 ◽  
Vol 108 (Supplement_6) ◽  
Author(s):  
S Robertson ◽  
G Lazaraviciute ◽  
L Kerr ◽  
D Hendry

Abstract We present a case report of an unexpected pathology in addition to a rare complication in a 62-year-old female following an anterior exenteration and an abdominal wall reconstruction. Initial biopsies characterised the malignancy as a potential urachal adenocarcinoma, but the final pathology revealed a solitary endometrial adenocarcinoma metastasis from endometrial cancer 6 years previously. Due to the nature of the tumour, an abdominal wall resection was required, and reconstruction involved a pedicled thigh flap. Post-operatively the small bowel herniated under the flap and this resulted in true mechanical small bowel obstruction. The loop of herniated bowel is visible on CT imaging in the area of the proximal thigh. The patient returned to theatre for an emergency laparotomy and the abdominal wall defect was closed instead with a surgical implant derived from animal tissue. The patient made an excellent recovery, was discharged home, and continues to do well. This patient is an example of excelling in adversity.


2021 ◽  
Vol 8 (2) ◽  
pp. 275-278
Author(s):  
Suriya Desikan ◽  
Sendhil Coumary A ◽  
Syed Habeebullah

Pentalogy of Cantrell is a rare structural disorder. It is a combination of congenital defects in the midline comprising of supraumbilical abdominal wall defect, defect in the lower sternum, deficient anterior diaphragm, defect in diaphragmatic pericardium and intracardiac defects. It is proposed to result from an insult occurring as early as 14 to 17 day of embryonic life. It involves the ventral midline due to disruption of midline mesenchyme. Etio-pathogenesis is still largely unknown. The insult could be environmental or disruption of a single gene, either autosomal or X linked. Very rarely Cantrell’s syndrome is associated with neural tube disorders. Here, we report one such rare case of Pentalogy of Cantrell.


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