Zhang L, Yan S, Pan L, Wu SF. Progressive disfiguring facial masses with pupillary axis obstruction from Morbihan syndrome: A case report. World J Clin Cases 2021; 9(24): 7163-7168 [PMID: 34540973 DOI: 10.12998/wjcc.v9.i24.7163]
Corresponding Author of This Article
Lei Pan, MD, Attending Doctor, Surgeon, Department of Plastic and Reconstructive Surgery, Plastic and Reconstructive Surgery Center, Zhejiang Provincial People's Hospital, People's Hospital of Hangzhou Medical College, No. 158 Shangtang Road, Hangzhou 310014, Zhejiang Province, China. prs_dr_panlei@163.com
Research Domain of This Article
Dermatology
Article-Type of This Article
Case Report
Open-Access Policy of This Article
This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
World J Clin Cases. Aug 26, 2021; 9(24): 7163-7168 Published online Aug 26, 2021. doi: 10.12998/wjcc.v9.i24.7163
Progressive disfiguring facial masses with pupillary axis obstruction from Morbihan syndrome: A case report
Lei Zhang, Sheng Yan, Lei Pan, Su-Fan Wu
Lei Zhang, Sheng Yan, Su-Fan Wu, Department of Plastic and Reconstructive Surgery, Plastic and Reconstructive Surgery Center, Zhejiang Provincial People’s Hospital, Affiliated Hospital of Hangzhou Medical College, Hangzhou 310014, Zhejiang Province, China
Lei Pan, Department of Plastic and Reconstructive Surgery, Plastic and Reconstructive Surgery Center, Zhejiang Provincial People's Hospital, People's Hospital of Hangzhou Medical College, Hangzhou 310014, Zhejiang Province, China
Author contributions: Zhang L collected the data and drafted the manuscript; Yan S participated in the operation; Pan L designed the research and revised the paper; Wu SF helped to check the language; all authors read and approved the final manuscript.
Informed consent statement: Written informed consent was obtained from the patient for publication of this case report.
Conflict-of-interest statement: The authors declare that they have no conflicts of interest regarding this work.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Lei Pan, MD, Attending Doctor, Surgeon, Department of Plastic and Reconstructive Surgery, Plastic and Reconstructive Surgery Center, Zhejiang Provincial People's Hospital, People's Hospital of Hangzhou Medical College, No. 158 Shangtang Road, Hangzhou 310014, Zhejiang Province, China. prs_dr_panlei@163.com
Received: January 5, 2021 Peer-review started: January 5, 2021 First decision: April 29, 2021 Revised: May 31, 2021 Accepted: July 9, 2021 Article in press: July 9, 2021 Published online: August 26, 2021 Processing time: 230 Days and 17.3 Hours
Abstract
BACKGROUND
Morbihan syndrome is a rare illness, which presents with recurrent pitting edema on the upper two thirds of the face causing facial contour deformities, and Morbihan syndrome obstructing the pupillary axis with ptosis was seldomly reported.
CASE SUMMARY
A 59-year-old woman presented with a 15-year history of facial swelling that progressively invaded bilaterally from the inner canthus and eyelids outwards. Imaging examination indicated that the bilateral periorbital and the left temporal soft tissues had swelling without cranium invasion. Histopathological analysis showed a large amount of lymphocyte infiltration, and immunohistochemistry showed positive expression of CD68 in clear-cut granulomas and D2-40 in the lymphatic endothelium. Finally, the clinical diagnosis of Morbihan syndrome was confirmed.
CONCLUSION
Morbihan syndrome is an infrequent and refractory disease, which is characteristic with recurrent woody facial edema on the upper two thirds of the face. Solid facial edema is persistent and non-pitting, causing facial contour deformities and even vision field impairment. The diagnosis of Morbihan syndrome depends on clinical features, imaging information, and pathology. Blepharoplasty is optional to improve the visual field for these patients with severe pupillary axis obstruction.
Core Tip: Morbihan syndrome is a rare and refractory condition that is characterized by persistent edema on the upper two thirds of the face. We report a Morbihan syndrome patient with progressive disfiguring facial masses to cause pupillary axis obstruction. The features and prognosis of this disease are carefully described to help clinical physicians know this illness.
