{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,8]],"date-time":"2026-09-08T10:26:47Z","timestamp":1788863207435,"version":"build-2803163510"},"reference-count":0,"publisher":"Georg Thieme Verlag KG","issue":"06","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Eur J Pediatr Surg"],"published-print":{"date-parts":[[2019,12]]},"abstract":"<jats:p>\n            Background\u2003Diffuse esophageal leiomyomatosis (DEL) is a rare disorder characterized by benign hypertrophy of esophageal smooth muscle cells. No rigorous summary of available evidence on how to best manage these patients exists.<\/jats:p><jats:p>\n            Objective\u2003To define the clinical features and outcomes of pediatric patients with DEL.<\/jats:p><jats:p>\n            Materials and Methods\u2003A systematic literature search of the PubMed and Cochrane databases was performed with respect to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement (end-of-search date: October 6, 2018). The algorithm: \u201cesophageal leiomyomatosis AND (children OR pediatric*)\u201d was implemented.<\/jats:p><jats:p>\n            Results\u2003Thirty-five studies including a total of 58 patients were analyzed. The female:male ratio was 1.45:1. Mean patient age was 8.54\u2009\u00b1\u20094.67 years. The most common disease manifestations were dysphagia and gastrointestinal symptoms (90.0%, 95% confidence interval [CI]: 78.2\u201396.1), followed by failure to thrive (57.9%, 95% CI: 36.2\u201376.9) and pulmonary symptoms (56.4%, 95% CI: 41.0\u201370.7). Alport syndrome (AS) was seen in 57.7% (95% CI: 44.2\u201370.1) of the patients. The most commonly implemented procedure was esophagectomy (85.2%; n\u2009=\u200946\/54; 95% CI: 73.1\u201392.6) with gastric transposition (37.8%; n\u2009=\u200917\/45; 95% CI: 25.1\u201352.4). Postoperative complications developed in 33.3% (n\u2009=\u200915\/45; 95% CI: 21.3\u201348) of the patients. All-cause mortality was 7.0% (95% CI: 2.3\u201317.2) and disease-specific mortality was 3.5% (95% CI: 0.3\u201312.6).<\/jats:p><jats:p>\n            Conclusion\u2003DEL is an uncommon condition that typically occurs in the setting of AS. Esophagectomy with gastric transposition is the mainstay of treatment. Although complications develop in one-third of the patients, mortality rates are low.<\/jats:p>","DOI":"10.1055\/s-0038-1676507","type":"journal-article","created":{"date-parts":[[2018,12,21]],"date-time":"2018-12-21T23:50:51Z","timestamp":1545436251000},"page":"487-494","source":"Crossref","is-referenced-by-count":9,"title":["Diffuse Esophageal Leiomyomatosis in Pediatric Patients: A Systematic Review and Quality of Evidence Assessment"],"prefix":"10.1055","volume":"29","author":[{"given":"Ioannis A.","family":"Ziogas","sequence":"additional","affiliation":[{"name":"Pediatric Surgery Working Group, Society of Junior Doctors, Athens, Greece"},{"name":"School of Medicine, Aristotle University of Thessaloniki, Thessaloniki, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Konstantinos S.","family":"Mylonas","sequence":"additional","affiliation":[{"name":"Pediatric Surgery Working Group, Society of Junior Doctors, Athens, Greece"},{"name":"Laboratory of Experimental Surgery and Surgical Research, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Georgios","family":"Tsoulfas","sequence":"additional","affiliation":[{"name":"1st Department of Surgery, Papageorgiou University General Hospital, School of Medicine, Aristotle University of Thessaloniki, Thessaloniki, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eleftherios","family":"Spartalis","sequence":"additional","affiliation":[{"name":"Laboratory of Experimental Surgery and Surgical Research, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nikolaos","family":"Zavras","sequence":"additional","affiliation":[{"name":"Department of Pediatric Surgery, Attikon University General Hospital, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nikolaos","family":"Nikiteas","sequence":"additional","affiliation":[{"name":"Laboratory of Experimental Surgery and Surgical Research, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dimitrios","family":"Schizas","sequence":"additional","affiliation":[{"name":"1st Department of Surgery, Laikon University General Hospital, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"194","published-online":{"date-parts":[[2018,12,21]]},"container-title":["European Journal of Pediatric Surgery"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/www.thieme-connect.de\/products\/ejournals\/pdf\/10.1055\/s-0038-1676507.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2019,11,22]],"date-time":"2019-11-22T23:47:03Z","timestamp":1574466423000},"score":1,"resource":{"primary":{"URL":"http:\/\/www.thieme-connect.de\/DOI\/DOI?10.1055\/s-0038-1676507"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,12,21]]},"references-count":0,"journal-issue":{"issue":"06","published-online":{"date-parts":[[2019,11,22]]},"published-print":{"date-parts":[[2019,12]]}},"URL":"https:\/\/doi.org\/10.1055\/s-0038-1676507","relation":{},"ISSN":["0939-7248","1439-359X"],"issn-type":[{"value":"0939-7248","type":"print"},{"value":"1439-359X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2018,12,21]]}}}