| Two rare cases of the Pentalogy of Cantrell or its variants. | |
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MedLine Citation:
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PMID: 15515484 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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The Pentalogy of Cantrell (PC) is a rare association of defects involving the lower sternum, abdominal wall, diaphragm, pericardium and heart. We report two rare cases of the PC (variant form), showing fatal progression. Case 1 only survived two hours because of severe cardio-respiratory failure. Physical examination showed midline abdominal and thoracic defects, ectopic heart, pericardial defect, diaphragmatic defect, bilateral undescended testis, scoliosis, and adherence between left upper limb and trunk. In addition, the autopsy revealed diaphragmatic agenesia, intraabdominal testis, bilateral lung hypoplasia and lymphocytic meningitis. Case 2 only survived 15 minutes. In addition to the physical findings, including lower sternal defect, ectopic heart, epigastric omphalocele and scoliosis, the autopsy showed left diaphragmatic agenesia, pericardial agenesia, bilateral lung hypoplasia, deformed rib cage, anterior thoracic myeloschisis, adreno-hepatic fusion, left renal agenesia, meckel diverticulum and multiple accessory spleens. When comparing with other cases of PC, the concurrence of bilateral intraabdominal testis and lymphocytic meningitis in case 1, and adreno-hepatic fusion, anterior myeloschisis, meckel diverticulum, multiple accessory spleens, and renal agenesia in case 2 have not been described previously. |
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Authors:
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Adnan Aslan; Güngör Karagüzel; Ilke Unal; Nazif Aksoy; Mustafa Melikoglu |
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Publication Detail:
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Type: Case Reports; Journal Article; Research Support, Non-U.S. Gov't |
Journal Detail:
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Title: Acta medica Austriaca Volume: 31 ISSN: 0303-8173 ISO Abbreviation: Acta Med. Austriaca Publication Date: 2004 Aug |
Date Detail:
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Created Date: 2004-11-01 Completed Date: 2004-12-08 Revised Date: 2006-11-15 |
Medline Journal Info:
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Nlm Unique ID: 7501997 Medline TA: Acta Med Austriaca Country: Austria |
Other Details:
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Languages: eng Pagination: 85-7 Citation Subset: IM |
Affiliation:
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Department of Pediatric Surgery, Akdeniz University School of Medicine, Antalya, Turkey, adnanaslan@akdeniz.edu.tr |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Abdominal Muscles
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abnormalities* Abnormalities, Multiple / pathology* Autopsy Cesarean Section Diaphragm / abnormalities* Female Gestational Age Heart Defects, Congenital / pathology* Humans Infant, Newborn Infant, Premature Male Pregnancy |
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