congenital hereditary endothelial dystrophy ( ched ) is a corneal dystrophy characterized by bilateral diffuse clouding of the corneas , causing impaired vision . depending on the mode of inheritance , it manifests either in infancy or young childhood with a cloudy cornea , light sensitivity , tearing , and sometimes nystagmus.1 ched is primarily a disease of the corneal endothelium , but it has traditionally been treated with penetrating keratoplasty.2 targeted corneal endothelium transplantation techniques , such as descemet s stripping automated endothelial keratoplasty ( dsaek ) , have become a standard of care for the treatment of corneal endothelial dysfunction in adults,3 but few reports exist describing its use in the pediatric age group.47 in this report , the authors describe a case of dsaek being performed in a child with ched . a 10-year - old boy presented to the corneal and refractive surgery unit as a referral from a local ophthalmologist due to poor vision and hazy corneas since infancy . the father and both of the child s siblings were affected by the same condition . a diagnosis of ched was made on the basis of clinical findings and the positive family history . the corneas of both eyes were diffusely edematous ( figure 1 ) , with central corneal thicknesses of 921 m and 891 m in the right and left eyes respectively , measured with ultrasonic pachymetry . intraocular pressures were 15 mmhg in the right eye and 18 mmhg in the left eye . axial lengths of the eyes were slightly shorter than normal , being 22.53 mm and 22.49 mm , as confirmed on a - scan ultrasound biometry . the patient was scheduled for dsaek on the right eye , to be performed under general anesthesia . to assist in graft sizing , the eye was measured with a pair of calipers at the beginning of the procedure and was found to be 10.5 mm . the endothelial graft was prepared using a moria cb microkeratome head ( 350 m blade depth ) and a moria altk artificial ( ac ) anterior chamber ( moria surgical , doylestown , pa ) . the thickness of the donor cornea was 673 m , as measured by ultrasonic pachymetry . after the microkeratome pass , the posterior lamella tissue was punched from the endothelial side using a disposable trephine , 8 mm in diameter . the donor tissue was then covered with optisol solution ( chiron ophthalmics , irvine , ca ) until use . with the surgeon sitting temporally , the central corneal epithelium was debrided to improve visualization of the ac ( figure 2a ) . a 1-mm side port was created at the 6 oclock position , through which an ac maintainer was inserted and the irrigating fluid turned on . a 2.75-mm temporal clear corneal incision was created , through which descemet s membrane ( dm ) scouring was performed at the required diameter using a reverse sinskey hook ( figure 2b ) . the ac maintainer was then turned off , and trypan blue dye was injected into the ac . the ac maintainer was then turned back on to wash the dye out of the ac . a 90 descemet s stripper was then used to strip dm from the overlying corneal stroma ( figure 2c ) . a peripheral iridectomy was performed at the 11 oclock position using a vitrector ( figure 2d ) . a 4.5-mm sized nasal clear corneal incision was created to prepare for insertion of the tip of the busin glide ( moria surgical).8,9 the donor disk was transferred to the busin glide with the endothelial surface oriented upwards . the central area of the endothelium was coated with viscoelastic , taking care not to let viscoelastic reach the stromal side of the graft . with the surgeon sitting superiorly , the loaded glide was inverted and positioned at the entrance of the nasal clear corneal incision . busin forceps were then passed through the temporal incision across the ac to grasp the donor disk at the glide opening . the donor disk was pulled into the ac ( figure 2e ) and allowed to unfold spontaneously under continuous irrigation from the ac maintainer ( figure 2f ) . an air bubble was injected under the graft and the ac maintainer was turned off ( figure 2 g ) . the epithelial surface was then gently massaged using a blunt , bent , irrigating cannula centered on the disk ( figure 2h ) . after centration was achieved , a large air bubble was injected into the ac to keep the disc adherent to the overlying stroma for around 15 minutes ( figure 3 ) . at the conclusion of the procedure , some air was exchanged with balanced salt saline ( bss ) , reducing the bubble size to approximately 2/3rd of the ac volume . postoperatively , the patient was positioned in a face - up position for the first 3 to 4 days . alcon , fort worth , tx ) and 1% prednisolone acetate suspension ( pred forte , allergan , irvine , ca ) administered hourly for the first week . best spectacle corrected visual acuity ( bscva ) at 3 months was 20/400 . at 1 year , the patient had bscva of 20/100 with a refraction of + 4  0.5  180 , and was able to play soccer at school with this vision . the corneal stroma , although it became clearer , did not become totally transparent , with moderate residual stromal haze being present even at 1 year while the central corneal thickness was 658 m ( figure 4a  postoperative appearance at 2 weeks ; figure 4b  appearance at 1 month postoperatively ; figure 4c  postoperative appearance at 3 months ; figure 4d  postoperative appearance at 1 year ) . conventional penetrating keratoplasty has a higher risk of graft failure and poorer visual prognosis than that of adult keratoplasty . children are harder to examine , their eyes have low scleral rigidity , and produce more of a fibrin reaction . during surgery , positive vitreous pressure may be experienced . there is an increased risk of infection and rejection due to sutures loosening frequently , necessitating their early replacement or removal . pkp wounds are more prone to traumatic rupture in children than in adults.10 targeted endothelial keratoplasty now forms an accepted standard of care for corneal endothelial dysfunction in adults.3 the benefits that endothelial transplantation procedures such as dsaek have over conventional pkp include faster visual rehabilitation , fewer or no sutures , and a structurally stronger globe . these benefits are especially desirable in the pediatric age group . to date , however , reports of dsaek being performed in children are few in number , with varying degrees of success , with some being successful and others being converted to pkp . while performing dsaek in both cases , we found that the ac view was poor due to the corneal edema . debriding the corneal epithelium did little to improve visibility during the procedure . movement inside the eye was made difficult by the shallow ac and phakic state of the eye ( figure 1 ) . pineda et al , attempted to perform dsaek on an eye with ched , but the procedure was converted to a pkp due to difficulty in detaching descemet s membrane and poor clarity of the cornea.4 in contrast , the case the authors performed was successfully completed in spite of an inability to remove dm . recipient interface can predispose to graft failure in dsaek.11 as the photos show , the time taken for the corneal stroma to clear after surgery was prolonged ( figure 4a  d ) . the corneal stroma retained some anterior haze for up to 1 year after surgery ( figure 5a  appearance of corneal stroma at 3 months ; figure 5b  appearance of corneal stroma at 6 months ; figure 5c  appearance of corneal stroma at 1 year ) . this suboptimal outcome may have been due to the persistent stromal haze or the coexistent amblyopia due to hyperopia . had surgery been performed in a younger patient , there might have been less corneal haze and more potential for treating the amblyopia . other recent reports describe dsaek being performed for ched in babies as young as 6 months12 and neonates.13 these cases could potentially reach better levels of vision as there is sufficient time to treat any associated amblyopia . no examinations under anesthesia were required in this case , as the child was cooperative enough to have his sutures removed at the slit lamp . this is in contrast to pediatric pkp , where multiple hospital admissions are usually needed during the postoperative course . moreover , as the final refraction showed in this child , the procedure was refractively neutral , due to the absence of sutures . in conclusion , dsaek can be used successfully in the treatment of ched in children , as an alternative to pkp . dsaek allows for a more rapid visual rehabilitation of patients and fewer subsequent postoperative hospital admissions for treatment .