Surgical release of the chest wall skin and fascia for sclerodermatous graft versus host disease causing restrictive lung disease: A case report

Mitchell Nash; Kim Cartwright; Rebecca Nguyen; Peter Middleton; Peter Maitz

doi:10.1016/j.ijscr.2024.109455

Surgical release of the chest wall skin and fascia for sclerodermatous graft versus host disease causing restrictive lung disease: A case report

Int J Surg Case Rep. 2024 Apr:117:109455. doi: 10.1016/j.ijscr.2024.109455. Epub 2024 Mar 7.

Authors

Mitchell Nash¹, Kim Cartwright², Rebecca Nguyen³, Peter Middleton⁴, Peter Maitz⁵

Affiliations

¹ Burns Unit, Concord Repatriation General Hospital, Concord, NSW 2137, Australia. Electronic address: mitchellnash@gmail.com.
² Department of Haematology, Wollongong Hospital, Wollongong, NSW 2500, Australia.
³ Department of Respiratory Medicine, Liverpool Hospital, Liverpool, NSW 2170, Australia; South West Sydney Clinical School, Faculty of Medicine, University of New South Wales, Liverpool, NSW 2170, Australia.
⁴ Respiratory & Sleep Medicine, Westmead Clinical School, Westmead Hospital, Westmead, NSW 2145, Australia; Sydney University, Camperdown, NSW 2006, Australia.
⁵ Burns Unit, Concord Repatriation General Hospital, Concord, NSW 2137, Australia; Sydney University, Camperdown, NSW 2006, Australia.

Abstract

Introduction: Graft versus host disease (GVHD) remains a significant source of morbidity and mortality in the setting of allogeneic stem cell transplantation. Skin involvement is reported to be as high as 70-95 % in this group with GVHD and the severity of the involvement varies widely. Surgical management of complications of severe cutaneous GVHD is uncommon and is rarely mentioned as a treatment option.

Case presentation: We present a case of severe sclerodermatous skin changes restricting chest expansion and exercise tolerance to the point of limiting basic activities of daily life. A 54-year-old male presents with severe restrictive lung disease from sclerodermatous graft versus host disease (GVHD) after stem cell transplant for Chronic Myeloid Leukaemia (CML). He experienced limited symptomatic relief from maximal medical therapy and photochemotherapy, and subsequently underwent a skin release and split skin grafting of his chest and abdomen in an effort to improve exercise tolerance and quality of life.

Clinical discussion: Despite an initial improvement in functioning, the patient's spirometry and lung function continued to decline with time, possibly suggesting that he did not gain a sustained benefit from surgical release of his cutaneous GVHD.

Conclusion: While delineating between disease progression and surgical outcome is difficult in this case, the patient would argue that by delaying or reducing further decline in function, the surgical release procedures led to improved quality of life in subsequent years. However further research is required to establish a clear role for surgery in the treatment of refractory cutaneous GVHD.

Keywords: Case report; Escharotomy; GVHD; Restrictive lung disease.

Publication types

Case Reports