{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,8]],"date-time":"2026-09-08T10:36:51Z","timestamp":1788863811559,"version":"build-2803163510"},"reference-count":17,"publisher":"Georg Thieme Verlag KG","issue":"01","license":[{"start":{"date-parts":[[2020,12,15]],"date-time":"2020-12-15T00:00:00Z","timestamp":1607990400000},"content-version":"vor","delay-in-days":349,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["European J Pediatr Surg Rep"],"published-print":{"date-parts":[[2020,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Venous malformations represent a major sector of vascular anomalies. Most cases are asymptomatic or subclinical; however, large extensive lesions can cause severe disability and sometimes mortality. In this report, we present a successful case of sirolimus treatment in managing an extensive venous malformation in the pelvis of a 21-month-old boy who presented with life-threatening complications. With a history dating since the day 2 of life, the patient suffered from chronic bleeding due to scrotal skin ulcerations, in addition to recurrent attacks of severe bleeding per rectum necessitating hospital admission and blood transfusion (three attacks since the age of 7 months). Pelvic magnetic resonance image showed the typical findings of extensive venous malformation involving the pelvis, perineum, scrotum, and extending to the gluteal region. The lesion was seen totally encasing the anorectum with marked thickening of their walls almost occluding their lumen.<\/jats:p><jats:p>Oral sirolimus (2\u2009mg\/m2) was started with a target blood trough level of 5 to 10\u2009ng\/mL. Over a follow-up period of 5 months, there was obvious clinical improvement that included healing of skin lesions (scrotal ulcer) with complete re-epithelialization, absence of bleeding per rectum with improvement of constipation, and rise of hemoglobin level from 7.5 to 11.5\u2009g\/dL.<\/jats:p>","DOI":"10.1055\/s-0040-1716895","type":"journal-article","created":{"date-parts":[[2020,12,15]],"date-time":"2020-12-15T23:55:31Z","timestamp":1608076531000},"page":"e90-e94","source":"Crossref","is-referenced-by-count":9,"title":["Sirolimus: A Rescue Drug to Control Complications of Extensive Venous Malformation"],"prefix":"10.1055","volume":"08","author":[{"given":"Mohamed Aly","family":"Abdelbaky","sequence":"additional","affiliation":[{"name":"Department of Pediatric Surgery, Ain Shams University Faculty of Medicine, Cairo, Egypt"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Iman Ahmed","family":"Ragab","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Ain Shams University, Faculty of Medicine, Cairo, Egypt"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amr AbdelHamid","family":"AbouZeid","sequence":"additional","affiliation":[{"name":"Department of Pediatric Surgery, Ain Shams University Faculty of Medicine, Cairo, Egypt"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shaimaa Abdelsattar","family":"Mohammad","sequence":"additional","affiliation":[{"name":"Department of Radiodiagnosis, Ain Shams University Faculty of Medicine, Cairo, Egypt"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mohamed Moussa","family":"Dahab","sequence":"additional","affiliation":[{"name":"Department of Pediatric Surgery, Ain Shams University Faculty of Medicine, Cairo, Egypt"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mohammed","family":"Elsherbeny","sequence":"additional","affiliation":[{"name":"Department of Pediatric Surgery, Ain Shams University Faculty of Medicine, Cairo, Egypt"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hatem Abdelkader","family":"Safaan","sequence":"additional","affiliation":[{"name":"Department of Pediatric Surgery, Ain Shams University Faculty of Medicine, Cairo, Egypt"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"194","published-online":{"date-parts":[[2020,12,15]]},"reference":[{"key":"ref1","first-page":"171","volume-title":"Venous malformations","author":"R A Bly","year":"2018"},{"issue":"02","key":"ref2","doi-asserted-by":"crossref","first-page":"45","DOI":"10.1053\/j.tvir.2018.03.001","article-title":"Treatment of venous malformations: the data, where we are, and how it is done","volume":"21","author":"A N Hage","year":"2018","journal-title":"Tech Vasc Interv Radiol"},{"issue":"05","key":"ref3","doi-asserted-by":"crossref","first-page":"317","DOI":"10.1053\/j.sempedsurg.2017.09.007","article-title":"Late sequelae and long-term outcomes of vascular 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Part 2: low-flow lesions","volume":"61","author":"L Flors","year":"2019","journal-title":"Radiologia (Madr)"},{"key":"ref11","first-page":"314","article-title":"Vascular anomalies","volume":"36","author":"V J Gokani","year":"2018","journal-title":"Surgery"},{"issue":"01","key":"ref12","first-page":"86","article-title":"Sirolimus in the treatment of vascular anomalies","volume":"27","author":"P Triana","year":"2017","journal-title":"Eur J Pediatr Surg"},{"issue":"01","key":"ref13","doi-asserted-by":"crossref","first-page":"318","DOI":"10.1016\/j.jvs.2019.06.217","article-title":"Efficacy and safety of sirolimus in the treatment of vascular anomalies: a systematic review","volume":"71","author":"C Freixo","year":"2020","journal-title":"J Vasc Surg"},{"key":"ref14","first-page":"161","volume-title":"Head and neck lymphatic Malformation diagnosis and treatment","author":"J A Perkins","year":"2018"},{"issue":"11","key":"ref15","doi-asserted-by":"crossref","first-page":"1037","DOI":"10.1161\/CIRCULATIONAHA.116.026886","article-title":"Germline loss-of-function mutations in EPHB4 cause a second form of capillary malformation-arteriovenous malformation (CM-AVM2) deregulating RAS-MAPK signaling","volume":"136","author":"M Amyere","year":"2017","journal-title":"Circulation"},{"issue":"02","key":"ref16","doi-asserted-by":"crossref","first-page":"e20153257","DOI":"10.1542\/peds.2015-3257","article-title":"Efficacy and safety of sirolimus in the treatment of complicated vascular anomalies","volume":"137","author":"D M Adams","year":"2016","journal-title":"Pediatrics"},{"issue":"01","key":"ref17","doi-asserted-by":"crossref","first-page":"141","DOI":"10.1186\/s13023-019-1118-1","article-title":"The impact of sirolimus therapy on lesion size, clinical symptoms, and quality of life of patients with lymphatic anomalies","volume":"14","author":"M Ozeki","year":"2019","journal-title":"Orphanet J Rare Dis"}],"container-title":["European Journal of Pediatric Surgery Reports"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/www.thieme-connect.de\/products\/ejournals\/pdf\/10.1055\/s-0040-1716895.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2020,12,15]],"date-time":"2020-12-15T23:55:51Z","timestamp":1608076551000},"score":1,"resource":{"primary":{"URL":"http:\/\/www.thieme-connect.de\/DOI\/DOI?10.1055\/s-0040-1716895"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,1]]},"references-count":17,"journal-issue":{"issue":"01","published-online":{"date-parts":[[2020,1,28]]},"published-print":{"date-parts":[[2020,1]]}},"URL":"https:\/\/doi.org\/10.1055\/s-0040-1716895","relation":{},"ISSN":["2194-7619","2194-7627"],"issn-type":[{"value":"2194-7619","type":"print"},{"value":"2194-7627","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,1]]}}}