{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,27]],"date-time":"2026-02-27T05:10:34Z","timestamp":1772169034519,"version":"3.50.1"},"update-to":[{"DOI":"10.12688\/f1000research.8246.1","type":"new_version","label":"New version","source":"publisher","updated":{"date-parts":[[2016,6,10]],"date-time":"2016-06-10T00:00:00Z","timestamp":1465516800000}}],"reference-count":2,"publisher":"F1000 Research Ltd","license":[{"start":{"date-parts":[[2016,6,10]],"date-time":"2016-06-10T00:00:00Z","timestamp":1465516800000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["f1000research.com"],"crossmark-restriction":false},"short-container-title":["F1000Res"],"abstract":"<ns4:p>Prune perineum syndrome (PPS) is a rare anomaly, with only two previous case reports, both dying in the perinatal period. We report the first case of PPS that reached childhood.<\/ns4:p>\n                  <ns4:p\/>\n                  <ns4:p>The patient presented with a hypoplastic genitalia and bilateral cryptorchidism. There was no evidence of an anal orifice. A significant prune-like mass was observed, extending from the perineum to both gluteal regions and to a cephalic mid-line bony prominence, with a 1cm central orifice that discharged urine. MRI confirmed the previous findings and revealed a right crossed ectopic kidney, intestinal malrotation, a hypoplastic infrarenal inferior vena cava and a hypoplastic right iliac artery. Endoscopic evaluation through the orifice revealed a cavity lined by urothelial mucosa, with a small communication to the anterior urethra in its anterior wall.<\/ns4:p>\n                  <ns4:p\/>\n                  <ns4:p>A staged reconstruction was planned, with a first-step urinary diversion through a continent abdominal reservoir associated to bilateral orchiopexy. He was discharged from the hospital three weeks later under intermittent catheterization. The next surgical step would be the resection of the perineal mass and its cavity associated to the removal of the prominent sacrococcygeal bones. Unfortunately, four months after the first surgery the patient developed an acute abdomen and was submitted to a laparotomy that revealed a necrotic ileal segment secondary to obstructive adherences. He developed severe malabsorption followed by septic shock, dying five weeks after the procedure.<\/ns4:p>\n                  <ns4:p\/>\n                  <ns4:p>Due to the lack of literature, there is no consensus for the management of these cases. The wish of the family for a better quality of life and social acceptance, compelled us to perform a urinary diversion, to be followed by a plastic and orthopedic reconstruction. Despite the successful initial result, the patient developed a late abdominal obstruction that was misdiagnosed, precipitating his untimely death five months after the first procedure.<\/ns4:p>","DOI":"10.12688\/f1000research.8246.2","type":"journal-article","created":{"date-parts":[[2016,6,10]],"date-time":"2016-06-10T06:23:08Z","timestamp":1465539788000},"page":"363","update-policy":"https:\/\/doi.org\/10.12688\/f1000research.crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["Case Report: Prune perineum syndrome: a rare case with an unfavourable outcome"],"prefix":"10.12688","volume":"5","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6636-7679","authenticated-orcid":false,"given":"Roberto I.","family":"Lopes","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Francisco T.","family":"D\u00e9nes","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gustavo B.","family":"Messi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marcos G.","family":"Machado","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"2560","published-online":{"date-parts":[[2016,6,10]]},"reference":[{"key":"ref-1","doi-asserted-by":"publisher","first-page":"233-236","DOI":"10.1002\/tera.1420200207","article-title":"Prune perineum.","volume":"20","author":"J Peeden","year":"1979","journal-title":"Teratology."},{"key":"ref-2","doi-asserted-by":"publisher","first-page":"145-148","DOI":"10.1002\/tera.1420280117","article-title":"Prune perineum syndrome: report of a second case.","volume":"28","author":"D Williams","year":"1983","journal-title":"Teratology."}],"container-title":["F1000Research"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/f1000research.com\/articles\/5-363\/v2\/xml","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/f1000research.com\/articles\/5-363\/v2\/pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/f1000research.com\/articles\/5-363\/v2\/iparadigms","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2018,12,5]],"date-time":"2018-12-05T13:01:31Z","timestamp":1544014891000},"score":1,"resource":{"primary":{"URL":"https:\/\/f1000research.com\/articles\/5-363\/v2"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2016,6,10]]},"references-count":2,"URL":"https:\/\/doi.org\/10.12688\/f1000research.8246.2","relation":{"has-review":[{"id-type":"doi","id":"10.5256\/f1000research.9672.r14293","asserted-by":"subject"},{"id-type":"doi","id":"10.5256\/f1000research.9672.r14291","asserted-by":"subject"},{"id-type":"doi","id":"10.5256\/f1000research.9672.r14291","asserted-by":"object"},{"id-type":"doi","id":"10.5256\/f1000research.9672.r14293","asserted-by":"object"}]},"ISSN":["2046-1402"],"issn-type":[{"value":"2046-1402","type":"electronic"}],"subject":[],"published":{"date-parts":[[2016,6,10]]},"assertion":[{"value":"Indexed","URL":"https:\/\/f1000research.com\/articles\/5-363\/v2#article-reports","order":0,"name":"referee-status","label":"Referee status","group":{"name":"current-referee-status","label":"Current Referee Status"}},{"value":"10.5256\/f1000research.9672.r14293, Dana Weiss, Division of Urology, Departments of Surgery, The Children's Hospital of Philadelphia, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA, 14 Jun 2016, version 2, indexed","URL":"https:\/\/f1000research.com\/articles\/5-363\/v2#referee-response-14293","order":0,"name":"referee-response-14293","label":"Referee Report","group":{"name":"article-reports","label":"Article Reports"}},{"value":"10.5256\/f1000research.9672.r14291, Rodrigo L.P. Romao, Division of Urology, Division of Pediatric General Surgery, Department of Surgery, IWK Health Centre, Dallhousie University, Halifax, NS, Canada, 01 Jul 2016, version 2, indexed","URL":"https:\/\/f1000research.com\/articles\/5-363\/v2#referee-response-14291","order":1,"name":"referee-response-14291","label":"Referee Report","group":{"name":"article-reports","label":"Article Reports"}},{"value":"10.5256\/f1000research.8868.r14064, Andrew J. Kirsch, Department of Pediatric Urology, Children's Healthcare of Atlanta, Emory University School of Medicine, Atlanta, GA, USA, 31 May 2016, version 1, indexed","URL":"https:\/\/f1000research.com\/articles\/5-363\/v1#referee-response-14064","order":2,"name":"referee-response-14064","label":"Referee Report","group":{"name":"article-reports","label":"Article Reports"}},{"value":"The author(s) declared that no grants were involved in supporting this work","order":3,"name":"grant-information","label":"Grant Information"},{"value":"This is an open access article distributed under the terms of the Creative Commons Attribution Licence, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.Data associated with the article are available under the terms of the Creative Commons Zero \"No rights reserved\" data waiver (CC0 1.0 Public domain dedication).","order":0,"name":"copyright-info","label":"Copyright"}]}}