Purpose To report the surgical outcomes of Descemet's stripping endothelial keratoplasty (DSEK) in cases of congenital hereditary endothelial dystrophy (CHED) cohort and compare it with penetrating keratoplasty (PK) in a paired-eye setting.

Methods Ours is a retrospective, comparative, consecutive and interventional clinical case series. All patients less than 14 years of age who underwent PK in one eye and DSEK in the contralateral eye at a single centre from January 2006 and February 2011 were analysed. Main outcome measures were graft clarity, visual outcome and complications.

Results The mean age of the patients was 6.6±2.19 years at the time of presentation. The outcomes of two surgeries were compared with 1 year of follow-up at the corresponding follow time. At 1 year, all grafts were clear. There was no significant difference in the spherical component of the refraction; the astigmatism was significantly lower after EK. The refraction stabilised in patients with EK as early as 3 months, while it continued to change up to 1 year after PK. Complications included graft dislocation in two cases of DSEK, which were managed by rebubbling, and a graft dehiscence in one case of PK, which was managed by resuturing. The final visual acuity improved in all the patients.

Conclusions Endothelial keratoplasty is a viable option to conventional PK in cases of CHED. It offers an advantage of early visual stabilisation compared with PK.