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CC BY-NC 4.0 · Arch Plast Surg 2015; 42(04): 391-398
DOI: 10.5999/aps.2015.42.4.391
Review Article

A Review of Macrodystrophia Lipomatosa: Revisitation

Theddeus OH Prasetyono
Division of Plastic Surgery, Department of Surgery, Cipto Mangunkusumo Hospital/Faculty of Medicine, University of Indonesia, Jakarta, Indonesia
,
Enjeline Hanafi  
Division of Plastic Surgery, Department of Surgery, Cipto Mangunkusumo Hospital/Faculty of Medicine, University of Indonesia, Jakarta, Indonesia
,
Windi Astriana  
Division of Plastic Surgery, Department of Surgery, Cipto Mangunkusumo Hospital/Faculty of Medicine, University of Indonesia, Jakarta, Indonesia
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Macrodystrophia lipomatosa (MDL) is a rare congenital non-hereditary disorder that has significant impact on patient morbidity. This study provides a comprehensive review of the natural history, diagnosis, management, and outcomes of the disorder. A literature search in PubMed was conducted to identify cases of MDL from January 1950 to 14 February 2014. After ruling out articles without information related to the management of the disorder, a summary of 32 studies was performed. An additional three cases from the authors are also presented. Based on 57 journal articles and three additional cases from the authors, around 108 cases of MDL were reviewed. Most patients were males who were admitted to a treatment clinic in the first four years of life. The lower extremities were more frequently affected, with unilateral presentation being most common. They commonly underwent a single-staged surgical procedure with follow-up periods ranging from more than one year up to 21 years. Out of 43 cases that underwent surgical procedures, 13 reported no complications, and there were seven cases of esthetic satisfaction and 15 cases of significant functional improvement. Depending on the severity of a patient's condition, the use of non-invasive diagnostic tools should be carefully considered. Surgery might be a better choice of management than observation, taking into account possible future complications in the absence of surgery and the beneficial outcomes of surgical procedures.

Keywords

Congenital limb deformities - Fingers - Lower extremity - Upper extremity

This article contains Appendices 1-4.


Appendix 1. The summary of age, gender, and anatomical location of the inclusive articles

Appendix 2. The summary of length of follow-up, diagnostic tools, management, complications, and outcomes of the inclusive articles

Appendix 3. Digital presentation of Macrodystrophia lipomatosa in upper extremity

Appendix 4. Digital presentation of macrodystrophia lipomatosa in lower extremity

APPENDIX REFERENCES



Publikationsverlauf

Eingereicht: 11. Dezember 2014

Angenommen: 23. April 2015

Artikel online veröffentlicht:
05. Mai 2022

© 2015. The Korean Society of Plastic and Reconstructive Surgeons. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonCommercial License, permitting unrestricted noncommercial use, distribution, and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes. (https://creativecommons.org/licenses/by-nc/4.0/)

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