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• W2032297227 abstract "Sir, Keratoglobus is a rare, bilateral clinical condition, which is characterized by a globular protrusion of the cornea and a generalized limbus-to- limbus corneal thinning. It causes severe visual impairment by inducing extreme myopia, irregular astigmatism and possibly also corneal scarring due to previous hydrops. It is variously managed (Hallermann 1975; Cameron et al. 1991; Burk & Joussen 2000; Jones & Kirkness 2001; Vajpayee et al. 2002), but an optimal surgical strategy has not been elaborated (Cameron 1993). Penetrating keratoplasty represents one of the first surgical approaches to have been tested, but it has since been renounced in favour of more complex procedures. However, the technical simplicity of penetrating keratoplasty is not a demerit and the procedure may yield good visual and tectonic outcomes. In the presented case report, we document a modification to penetrating keratoplasty which appears to avert some of the known side-effects of the technique and thus renders it a logical and realistic therapeutic choice for keratoglobus. A 33-year-old Afghan woman presented with bilateral forward corneal bulge and an associated visual impairment, which had become progressively worse during the 13 years prior to presentation. No previous eye disease or contact lens wear was reported and there was no evidence of systemic collagen disease or joint hyperextensibility. Visual acuity (VA) was 20/200 in the right eye, with a correction of + 2.00 D (sph)/− 4.00 D (cyl) at 160 °, and 20/400 in the left eye, with a correction of − 10.00 D (sph)/− 3.00 D (cyl) at 160 °. Slit-lamp examination revealed the typical appearance of keratoglobus with bilateral and symmetrical limbus-to-limbus corneal thinning (Fig. 1). As is typical, the cornea was most attenuated in the mid-inferior periphery. The cornea was clear, free of lipid deposits and had not undergone neovascularization. The horizontal corneal diameter was normal at 12 mm in both eyes. Cartographic maps of the corneal topography and thickness (Fig. 2) were produced by Orbscan scanning-slit topography/pachymetry (Orbtek Inc., Salt Lake City, Utah, USA). As visual function was not improved by spectacle correction, the patient elected to undergo corneal surgery in her left eye. A mid, full-thickness keratoplasty was performed uneventfully, using a 9-mm diameter hand trephine for the donor cornea and a 9.1-mm diameter trephine for the patient's eye. The latter trephine was centred not on the apex of the cornea but over its thinnest portion in the mid-inferior periphery (Fig. 3). One month after surgery, the refractive power of the cornea was − 1.00 D (sph)/+ 7.00 D (cyl) at 180 ° and VA was 20/50, both of which remained stable during the ensuing, uneventful 22 months. Local corticosteroids were prescribed for 8 months without complications. Central and peripheral thinning of the clear cornea with ectasia on slit-lamp examination (left eye). Note the marked irregularity of the illumination lamp reflex on the temporal cornea. Preoperative orbscan pachymetry of both eyes, showing bilateral symmetrical limbus-to-limbus corneal thinning. Simulated keratometry revealed the refractive power of the right cornea to be 63.8 D at 157 ° and 43.1 D at 67 ° and that of the left cornea to be 56.4 D at 74 ° and 46.4 D at 164 °. Orbscan, confirmed by ultrasound pachymetry, yielded central corneal thickness values in the right and left eyes of 462 µm and 426 µm, respectively. The thinnest areas of the cornea were located in the mid-inferior periphery, where its thickness was 334 µm in the right eye and 308 µm in the left. Immediate postoperative appearance showing the eccentric penetrating keratoplasty of intermediate size (9 mm in diameter), which was centred not on the apex of the cornea but over its thinnest portion in the mid-inferior periphery. In keratoglobus, a spectacle correction is made only if a useful visual acuity can be thereby achieved. Contact lenses are not considered as an alternative, owing to the grossly abnormal topography of the corneas and to the commonly held belief that these are at high risk of rupturing even when minimally traumatized. The surgical management of keratoglobus is challenging, owing to the extreme thinness of the entire cornea (Jones & Kirkness 2001). Various approaches have been tried, but an optimal technique has yet to be established (Hallermann 1975; Cameron et al. 1991; Cameron 1993; Burk & Joussen 2000; Jones & Kirkness 2001; Vajpayee et al. 2002). Penetrating keratoplasty may be the least difficult technique, but it is no longer recommended as a first option for keratoglobus (Alberth 1980; Jones & Kirkness 2001; Vajpayee et al. 2002). Suture fixation of a central penetrating keratoplasty may be complicated by the thinness of the mid- peripheral cornea, and the procedure yields a poor functional result owing to severe postoperative astigmatism, which is associated with the irregularity of the corneal periphery. Large- diameter penetrating keratoplasty (greater than 10 mm) or limbus-to- limbus epikeratoplasty are associated with high risks of secondary glaucoma, delayed re-epithelialization of the graft which becomes neovascularized owing to the frequent presence of a limbal stem cell defect, and graft rejection followed by graft failure (Jones & Kirkness 2001; Vajpayee et al. 2002). We therefore performed an eccentric penetrating keratoplasty of intermediate size (9 mm in diameter), which was centred over the thinnest portion of the cornea in order to maximize the thickness of the recipient bed for suturing. Using this simple manoeuvre, no severing of the peripheral recipient bed was experienced during suturing. Moreover, the chosen diameter permitted most of the diseased cornea to be removed without significantly heightening the risk of immune failure by preventing limbal contact of the graft. Finally, the graft did not jeopardize the trabecular meshwork. We therefore wish to advocate this simple mid-diameter, para-central penetrating keratoplasty, a single-stage procedure, for the surgical management of keratoglobus, the known longterm outcome of which adds it to the portfolio of the several other sound, but technically more demanding, surgical procedures available." @default.
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• W2032297227 date "2004-09-28" @default.
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• W2032297227 title "Keratoglobus surgery: penetrating keratoplasty redux" @default.
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• W2032297227 doi "https://doi.org/10.1111/j.1600-0420.2004.00271.x" @default.
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