Graft versus host disease: a pediatric perspective

ABSTRACT

Introduction: Allogeneic hematopoietic stem cell transplantation (HSCT) is an established treatment of myeloid and lymphoid malignancies, solid tumors and a number of metabolic conditions in children. An important treatment complication is graft-versus-host disease (GVHD) – a multiorgan condition with significant morbidity and mortality post-HSCT including ocular complications.

Areas covered: In this article, we reviewed the literature on pediatric ocular GVHD, including its incidence and risk factors, clinical features, current diagnostic criteria and clinical grading, treatment strategies and disease prognosis.

Expert opinion: Even though less frequent than in adults, ocular GVHD remains a common complication in children after HSCT. Clinical features of pediatric ocular GVHD include keratoconjunctivitis sicca, pseudomembranous and cicatricial conjunctivitis, keratopathy, lid deformities, and meibomian gland dysfunction. Diagnosing and monitoring ocular GVHD in children are challenging as current criteria are poorly adapted for the pediatric cohort. Treatment strategies of pediatric ocular GVHD focus on controlling inflammation and optimizing the tear film to protect and maintain the ocular surface.

KEYWORDS:

• Graft-versus-host disease
• hematopoietic stem cell transplantation
• HSCT
• bone marrow transplantation
• BMT
• keratoconjunctivitis sicca
• dry eye
• children
• pediatric

Article highlights

• A significant proportion of children would develop ocular manifestations of GVHD after allogeneic HSCT treatment.

• Ocular manifestations of GVHD in children are similar to those in adults, and mainly consist of keratoconjunctivitis sicca and ocular surface inflammation.

• Children seldom report symptoms of ocular dryness, which makes diagnosis and monitoring of ocular GVHD challenging. Baseline examination and frequent assessments subsequent to HSCT are therefore vital in the early detection of ocular GVHD.

• Treatment of ocular GVHD in children aims to reduce inflammation, optimize the ocular surface and tear film, and protect and maintain the ocular surface.

• Options to optimize the ocular surface and tear film include frequent usage of topical lubricants, lid hygiene, and punctal occlusion.

• Ocular surface inflammation is mostly managed by topical corticosteroids initially, followed by topical immunosuppressants such as cyclosporine and tacrolimus, although the latter lacks evidence in children.

• Scleral contact lenses provide a physical barrier as well as a tear reservoir that protects and maintains the ocular surface. Use of autologous serum eyedrops delivers growth factors that help to promote ocular surface healing.

• Surgical treatments include tarsorrhaphy, amniotic membrane transplant, and in rare occasions limbal stem cell and/or corneal transplantation.

• Cataract, although unrelated to the disease process, is commonly encountered in children with HSCT and ocular GVHD, and surgical removal is important in restoring vision in these children.

Declaration of interest

K Mireskandari and A Ali are consultants for Santen Inc. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Additional information

Funding

This paper was not funded.