Ocular complications after organ and bone marrow transplantation in children.

BACKGROUND: Organ and bone marrow transplantation commonly are performed in children. Ocular complications usually are described as secondary to post-transplantation medications. The complication rate is unknown. METHODS: A retrospective chart review was performed of 93 children who were younger than 18 years of age and had transplantation surgery from 1989 to 2004. The rate and type of ocular complications, including those requiring ocular surgery, were analyzed. Medications and visual loss associated with adverse effects also were studied. RESULTS: Of the 93 patients, 74 patients met the entry criteria. Sixty-one patients had at least 1 year of follow-up, and the longest follow-up duration was 14 years. The 1-year post-transplantation complication rate was 16.0% (95% confidence interval 6.8-24.4%). Adverse effects included cytomegalovirus (CMV) retinitis, cataract, graft-versus-host disease, lymphoproliferative disorder, persistent strabismus, and transient visual loss. Four patients underwent eye surgery, including lensectomy for cataract, tarsorrhaphy for corneal ulcer, and iris biopsy. They had surgery 0.9 to 4.7 years after transplantation. Most patients were taking prednisone and cyclosporine when their complication was diagnosed. One patient's visual acuity deteriorated to no light perception in one eye and 20/250 in the other eye secondary to CMV retinitis. Most patients had a final visual acuity > or =20/40. CONCLUSION: Transplantation surgery in children produces a significant risk of ocular impairment. The 1-year complication rate was 16.0%. Eye surgery may be required within the first few years after transplantation. Although most patients maintained a final visual acuity of 20/40 or better, one patient became bilaterally legally blind.     

Ocular complications of bone marrow transplantation.

Forty-one patients who had undergone bone marrow transplantation were examined. Nineteen (63%) of the 30 who had received fractionated total body irradiation (TBI) had cataracts in comparison with only 1 (9%) of the 11 non-irradiated patients. No significant differences in steroid therapy were demonstrated between these groups of patients. Dry eyes were observed in eight irradiated patients, seven of whom had preceding graft-versus-host disease. Superficial punctate staining of the bulbar conjunctiva was observed in 25 (83%) of the irradiated group and, in milder form, in five (45%) of the non-irradiated group. Our data suggest that the incidence of cataract following fractionated TBI is influenced not only by the total dose of radiation but also by its rate of administration (defined by midline tissue dose rate and fractionation schedule). In addition dry eyes and conjunctival staining may be exacerbated by TBI, though other aspects of conditioning may also be implicated.     

Ocular complications of pediatric bone marrow transplantation

ObjectiveTo investigate the ocular complications in pediatric bone marrow transplantation (BMT) patients.DesignCross-sectional study.ParticipantsA total of 29 pediatric BMT patients were studied.TestingComprehensive ophthalmic check-up, including best-corrected visual acuity, intraocular pressure (IOP), Schirmer’s test, tear breakup time, and slit-lamp and fundus examinations, was performed.Main outcome measuresTear film instability and its related complications, IOP, cataract, and fundus lesions were measured.ResultsThe mean age of patients was 9.1 years (range, 1.5–15 years). The mean post-BMT duration was 20.2 months (range, 3–54 months). Fifteen patients (51.7%) had tear abnormalities. Subconjunctival fibrosis was detected in two patients (6.9%). Dry and scaly skin of the eyelids was seen in one patient (3.4%). Lens opacities were found in 2 (33.3%) of 6 irradiated patients and 2 (8.7%) of 23 nonirradiated patients. Two patients (6.9%) had fundus changes, one with unilateral epiretinal membrane and the other with bilateral multiple discrete chorioretinal hypopigmented lesions in the middle to peripheral part of the retina. The overall complication rates for the anterior and posterior segments were 75.8% and 6.9%, respectively.ConclusionOcular manifestations of BMT in children are not uncommon. The most common anterior segment problem is tear dysfunction. Posterior segment complications are less common but do exist. High rate of cataract formation is reported, and this probably is the most important long-term “amblyogenic” problem in these immature eyes. Awareness and management of these problems with routine eye examination and early intervention are recommended.     

Cytomegalovirus retinitis in children following bone marrow transplantation

The authors describe three children, aged one, 13 and 24 months, who developed cytomegalovirus (CMV) retinitis seven to 18 months following allogeneic BMT. The underlying disease in two patients was severe combined immunodeficiency disease (SCID) and acute myeloid leukemia (AML) M5 in the third. All three patients developed chronic graft-versus-host-disease (GVHD) and received massive immunosuppressive therapy. The CMV retinitis was treated with ganciclovir. Clinical improvement was observed in the two SCID patients. The AML patient whose acute inflammatory retinitis was controlled, nevertheless developed optic atrophy in both eyes and VEP and ERG responses disappeared.

The incidence of cytomegalovirus retinitis in our pediatric population of bone marrow transplant (BMT) recipients in the last three years was higher than expected: 3/85 (3.5%). Alertness to the possibility of intraocular complications is advocated. Early detection of CMV retinitis and intensive treatment with ganciclovir can save vision. It is therefore suggested to perform ocular examinations as part of the routine follow-up of BMT patients, especially in children with profound immune deficiency.     

Cytomegalovirus retinitis in children following bone marrow transplantation

The authors describe three children, aged one, 13 and 24 months, who developed cytomegalovirus (CMV) retinitis seven to 18 months following allogeneic BMT. The underlying disease in two patients was severe combined immunodeficiency disease (SCID) and acute myeloid leukemia (AML) M5 in the third. All three patients developed chronic graft-versus-host-disease (GVHD) and received massive immunosuppressive therapy. The CMV retinitis was treated with ganciclovir. Clinical improvement was observed in the two SCID patients. The AML patient whose acute inflammatory retinitis was controlled, nevertheless developed optic atrophy in both eyes and VEP and ERG responses disappeared. The incidence of cytomegalovirus retinitis in our pediatric population of bone marrow transplant (BMT) recipients in the last three years was higher than expected: 3/85 (3.5%). Alertness to the possibility of intraocular complications is advocated. Early detection of CMV retinitis and intensive treatment with ganciclovir can save vision. It is therefore suggested to perform ocular examinations as part of the routine follow-up of BMT patients, especially in children with profound immune deficiency.     

Ocular manifestations of graft versus host disease following bone marrow transplantation

The ocular manifestations of Graft Versus Host Disease (GVHD) include keratoconjunctivitis sicca, cicatricial lagophthalmos, sterile conjunctivitis, corneal epithelial defects, corneal ulceration and melting. These manifestations are more frequent in patients with chronic GVHD than in patients with acute GVHD. The more severe ocular complications are associated with severe systemic chronic GVHD and poorer survival. Recent improvements in the systemic management of these patients have led to the more frequent recognition of the ocular problems. The high prevalence of ocular involvement and potentially severe ocular problems in GVHD patients necessitate close ophthalmic monitoring.     

Phacoemulsification in patients after allogeneic bone marrow transplantation

OBJECTIVE: To study the outcomes of phacoemulsification in allogeneic bone marrow transplant (allo-BMT) recipients. DESIGN: Retrospective, noncomparative, interventional case series. METHODS: Retrospective study of 34 eyes of 19 consecutive patients who had visually significant cataracts after allo-BMT and subsequently underwent phacoemulsification. MAIN OUTCOME MEASURES: Best-corrected vision at the last follow-up visit and development of postoperative complications. RESULTS: Surgery was done at a mean interval of 37 months after BMT, and the mean postoperative follow-up was 13 months. Twenty-one eyes (62%) had subnormal Schirmer I scores as a result of graft-versus-host disease (GVHD) involving the lacrimal gland. Of these, 71% (15 eyes) additionally had significant ocular surface epitheliopathy because of conjunctival GVHD. Frequent lubrication (95%), punctal occlusion (76%), topical steroids (33%), and other topical immunosuppressive therapies (14%) were used to manage GVHD-induced ocular surface disease before cataract surgery. Twelve patients (63%) also received systemic steroids and immunosuppressives. Patients proceeded to surgery only after their ocular surface disease was well controlled. Early postoperative complications included intraocular pressure elevation (three eyes), worsening of dry eye syndrome (two eyes), and corneal thinning (one eye). Posterior capsular opacification (PCO) requiring laser capsulotomy occurred in 44% of eyes. In eyes with preoperative conjunctival GVHD, 47% had recurrence with cessation of immunosuppressive therapy after surgery. Visual acuity at last follow-up visit was 20/30 or better in 33 (97%) eyes. CONCLUSIONS: Phacoemulsification is an effective procedure in restoring vision in patients who have cataracts develop after BMT. However, coexisting ocular disease must be recognized and aggressively treated both before and after surgery to ensure good visual outcomes.     

Eye changes following bone marrow transplantation

Blood diseases such as acute leukemia can now be cured by bone marrow transplants. Complications such as a chronic host-versus-graft reaction lead to typical conjunctival changes, as described for Sj?gren's syndrome and ocular pemphigoid. A patient with typical eye complications is described and the probable immunological course discussed.     

Chronic ocular surface disease after allogeneic bone marrow transplantation

Graft-versus-host disease (GVHD) is a common complication of allogeneic bone marrow transplantation (allo-BMT). Ocular surface disease (OSD) is one of the most common manifestations of chronic ocular GVHD, yet little is known about it. In this article, we review the available literature on this condition and present results from our study of the manifestations of OSD in the chronic phase (>3 months duration) post allo-BMT. Our study consisted of a retrospective chart review of 62 allo-BMT patients with chronic OSD evaluated at our center between 1995 and 2002. The clinical features, systemic associations, treatment, and status of OSD at the last follow-up are presented and discussed in the context of other reports of OSD in GVHD.     

Ocular findings after bone marrow transplantation in a pediatric population.

PURPOSE: With the increasing use of bone marrow transplantation (BMT) in children to treat diseases of the hematopoietic system, ocular complications have been recognized in greater numbers. The authors performed a retrospective study to determine the prevalence and types of ocular abnormalities in a pediatric population following BMT. DESIGN: Retrospective noncomparative study. PARTICIPANTS: One hundred and four consecutive pediatric patients with aplastic anemia and various hematologic malignancies who required BMT. INTERVENTION: Bone marrow transplantation. MAIN OUTCOME MEASURES: Visual acuity and slit-lamp biomicroscopic and funduscopic examinations. RESULTS: Ocular changes developed in 51% of patients. The most frequent findings included dry eye syndrome (12.5%), cataract (23.0%), and posterior segment complications (13.5%). A final visual acuity of 20/40 or better was achieved in 95.7% of eyes. CONCLUSIONS: Pediatric patients can develop severe and potentially vision-threatening complications following BMT. Despite the high incidence of anterior and posterior segment abnormalities, ocular symptoms were generally mild and the majority of patients retained excellent visual function. Factors associated with ocular complications included the underlying disease, total body irradiation, systemic chemotherapy, graft-versus-host disease, and immunosuppression. Familiarity with these risk factors and potential complications in pediatric patients is important for successful treatment.     

Irradiation cataract in children after bone marrow transplantation

Chemotherapy and radiation produce a dose-dependent anti-leukemic effect. Combined chemoradiotherapy and bone marrow transplantation (BMT) were given in our clinic to treat children with acute leukemias. Total body irradiation of 10 Gy in a single dose was used. One long-term side effect of this treatment was the development of subcapsular cataract; this was seen in all nine long-term survivors of the 17 children with acute lymphoblastic (ALL) or acute myelogenous (AML) leukemia who were treated as described above. One year after marrow transplantation, all the eyes studied had visual acuity of 20/20 and an optically clear lens. Three years later, 60% of the eyes had visual acuity of less than 20/40 and all had posterior, subcapsular cataracts. The cataract in all cases was quite uniform, consisting of opacities in the posterior subcapsular region. Cataract formation was treatment-related and seemed to correlate only to the type of total body irradiation. We concluded that the cataracts seen in the present study were a late complication of allogeneic BMT and were specifically due to the single-dose total body irradiation.     

Retinal complications of bone marrow and solid organ transplantation

PURPOSE OF REVIEW: A review is presented of the current literature on retinal complications of bone marrow and solid organ transplantation. RECENT FINDINGS: Retinal complications of bone marrow and solid organ transplantation include microvascular retinopathy, infection, and hemorrhagic findings. Other clinical observations include central serous chorioretinopathy, bilateral optic disc edema, and cyclosporine-related retinal toxicity. The cause of these clinical findings is likely to be multifactorial, resulting from the combined effects of cyclosporine, total body irradiation, infections, high-dose chemotherapy, and recurrent malignancies. SUMMARY: Understanding of these clinical entities of the posterior segment is important in minimizing the potentially sight-threatening complications from bone marrow and solid organ transplantation.     

Ocular changes in the mucopolysaccharidoses after bone marrow transplantation. A preliminary report

Metabolic correction and physiologic response were evaluated after bone marrow transplantation in mucopolysaccharidosis. Eleven patients were prospectively evaluated to determine the effect of bone marrow transplantation on the progressive ocular manifestations of these disorders. Follow-up of 0.6 to 2.8 years after successful donor stem cell engraftment showed that some patients had slow clearing of the corneal clouding, reduction of intracytoplasmic inclusions in the conjunctiva, resolution of optic nerve edema, and stabilized or improved retinal function as determined by electroretinography. These preliminary results suggest that early bone marrow transplantation may alter some of the progressive ophthalmic characteristics of the mucopolysaccharidoses. Long-term follow-up is necessary to determine if these early alterations in the ocular features are predictive of a prolonged functional improvement in the visual status.