Eur J Pediatr Surg
DOI: 10.1055/s-0038-1676507
Review Article
Georg Thieme Verlag KG Stuttgart · New York

Diffuse Esophageal Leiomyomatosis in Pediatric Patients: A Systematic Review and Quality of Evidence Assessment

Ioannis A. Ziogas*
1  Pediatric Surgery Working Group, Society of Junior Doctors, Athens, Greece
2  School of Medicine, Aristotle University of Thessaloniki, Thessaloniki, Greece
,
Konstantinos S. Mylonas*
1  Pediatric Surgery Working Group, Society of Junior Doctors, Athens, Greece
3  Laboratory of Experimental Surgery and Surgical Research, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece
,
Georgios Tsoulfas
4  1st Department of Surgery, Papageorgiou University General Hospital, School of Medicine, Aristotle University of Thessaloniki, Thessaloniki, Greece
,
Eleftherios Spartalis
3  Laboratory of Experimental Surgery and Surgical Research, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece
,
Nikolaos Zavras
5  Department of Pediatric Surgery, Attikon University General Hospital, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece
,
Nikolaos Nikiteas
3  Laboratory of Experimental Surgery and Surgical Research, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece
,
Dimitrios Schizas
6  1st Department of Surgery, Laikon University General Hospital, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece
› Author Affiliations
Funding None.
Further Information

Publication History

17 August 2018

31 October 2018

Publication Date:
21 December 2018 (eFirst)

Abstract

Background Diffuse 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.

Objective To define the clinical features and outcomes of pediatric patients with DEL.

Materials and Methods A 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: “esophageal leiomyomatosis AND (children OR pediatric*)” was implemented.

Results Thirty-five studies including a total of 58 patients were analyzed. The female:male ratio was 1.45:1. Mean patient age was 8.54 ± 4.67 years. The most common disease manifestations were dysphagia and gastrointestinal symptoms (90.0%, 95% confidence interval [CI]: 78.2–96.1), followed by failure to thrive (57.9%, 95% CI: 36.2–76.9) and pulmonary symptoms (56.4%, 95% CI: 41.0–70.7). Alport syndrome (AS) was seen in 57.7% (95% CI: 44.2–70.1) of the patients. The most commonly implemented procedure was esophagectomy (85.2%; n = 46/54; 95% CI: 73.1–92.6) with gastric transposition (37.8%; n = 17/45; 95% CI: 25.1–52.4). Postoperative complications developed in 33.3% (n = 15/45; 95% CI: 21.3–48) of the patients. All-cause mortality was 7.0% (95% CI: 2.3–17.2) and disease-specific mortality was 3.5% (95% CI: 0.3–12.6).

Conclusion DEL 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.

Authors' Contribution

Study concept and design, and critical revision: Mylonas, Schizas, Ziogas, Tsoulfas, Spartalis, Zavras, and Nikiteas. Data acquisition, analysis and data interpretation, and drafting of the article: Ziogas and Mylonas. Supervision: Mylonas and Nikiteas.


* Ioannis A. Ziogas and Konstantinos S. Mylonas share first co-authorship.


Supplementary Material