Femtosecond Laser-Assisted Tuck-In Penetrating Keratoplasty for Advanced Keratoglobus with Endothelial Damage
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CASE REPORT Femtosecond Laser-Assisted Tuck-In Penetrating Keratoplasty for Advanced Keratoglobus With Endothelial Damage Jorge L. Alió del Barrio, MD, PhD,*† Olena Al-Shymali, MD,*‡ and Jorge L. Alió, MD, PhD, FEBOphth*† thinning, especially peripherally, in which the normal stromal Purpose: To describe the outcomes of femtosecond laser-assisted thickness is frequently reduced to less than one-fifth.1 KTG tuck-in penetrating keratoplasty as a single-step surgical procedure often presents at birth and results in the globular protrusion of for visual and anatomical rehabilitation of patients with severe the cornea, leading to severe loss of vision as a result of extreme keratoglobus (KTG) and endothelial damage. myopia, irregular astigmatism, and scarring.2 Severe complica- Methods: tions frequently appear as acute corneal hydrops and corneal Two eyes of a 7-year-old patient with bilateral severe 1–3 KTG and previous corneal hydrops were operated. Assisted by the perforations occurring spontaneously or after minimal trauma. KTG has been described in both congenital and femtosecond laser, both donor and recipient corneas were prepared. 2 An 8.5-mm full-thickness donor tissue with a peripheral partial- acquired forms. The former has been associated with blue thickness rim of 1.25 mm was sutured into an 8.5-mm recipient bed sclera syndrome, osteogenesis imperfecta, Leber congenital amaurosis, and connective tissue disorders, such as Ehlers– with a previously dissected intralamellar peripheral pocket up to the 2,4,5 limbus. The graft was secured with 16 interrupted 10-0 nylon sutures Danlos or Marfan syndrome. Acquired KTG has been and the peripheral donor rim tucked into the host stromal pocket. associated with vernal keratoconjunctivitis and chronic mar- ginal blepharitis due to frequent eye rubbing, thyroid eye Results: Six months after surgery, both grafts remained healthy and disease, and idiopathic orbital inflammation.6 clear. One eye developed mild postoperative ocular hypertension. No KTG management is challenging and is not standardized. intraoperative or other postoperative complications were observed. Conservative therapies, such as spectacles and contact lenses for Corrected distance visual acuity was 20/50 in both eyes, with the correction of myopia and astigmatism, are not always complete functional rehabilitation of the patient. Restoration of the possible in noncollaborative children and may provoke a risk peripheral corneal thickness was observed in the pachymetric map. of corneal perforation through trauma. Corneal repair after Conclusions: perforation in such eyes is very complex and involves a high risk Femtosecond laser-assisted tuck-in penetrating of final eye loss requiring evisceration or enucleation.1 Thus, keratoplasty can provide excellent anatomical and functional reha- prevention is critical in patients with severe KTG. In the absence bilitation of patients with severe KTG and endothelial damage, of a definitive standard procedure, different lamellar keratoplasty through a single-step surgical procedure and a single donor cornea and epikeratoplasty techniques have been described to provide per eye. The femtosecond laser permits accurate dissection of these structural support to these extremely weakened corneas, already thin corneas without inadvertent perforation risk. although in cases of previous hydrops and endothelial damage, Key Words: keratoglobus, tuck-in penetrating keratoplasty, corneal a 2-step procedure with a second central corneal graft will be 7 transplant, femtosecond laser required. In such cases, standard penetrating keratoplasty (PKP) should be avoided because it is technically demanding, and it is (Cornea 2017;36:1145–1149) often unsuccessful because of the severe host peripheral stromal thinning and the high rejection risk of these almost limbus-to- limbus grafts in young recipients. eratoglobus (KTG) is a rare noninflammatory bilateral In this article, we report the case of a child with bilateral Kcorneoscleral ectasia characterized by generalized corneal advanced KTG and previous bilateral corneal hydrops in which successful visual and anatomical rehabilitation was Received for publication April 12, 2017; revision received May 11, 2017; achieved by femtosecond laser-assisted tuck-in PKP as accepted May 19, 2017. Published online ahead of print July 6, 2017. a single-step surgical procedure. From the *Cornea, Cataract and Refractive Surgery Unit, Vissum Corporación, Alicante, Spain; †Division of Ophthalmology, School of Medicine, Universidad Miguel Hernández, Alicante, Spain; and ‡Research and Development Department, Vissum Corporation, Alicante, Spain. MATERIALS AND METHODS The authors have no funding or conflicts of interest to disclose. Reprints: Jorge L. Alió, MD, PhD, FEBOphth, Avda de Denia s/n, Vissum, Case Report Instituto Oftalmologico de Alicante, 03016 Alicante, Spain (e-mail: [email protected]). A 7-year-old boy was referred to our clinic presenting Copyright © 2017 Wolters Kluwer Health, Inc. All rights reserved. bilateral KTG with severe and recalcitrant pain and Cornea Volume 36, Number 9, September 2017 www.corneajrnl.com | 1145 Copyright Ó 2017 Wolters Kluwer Health, Inc. Unauthorized reproduction of this article is prohibited. Alió del Barrio et al Cornea Volume 36, Number 9, September 2017 photophobia for the last 2 months. Slit-lamp examination and N400mm, S 402 mm, and I 302 mm. In an attempt to reattach visual function test were impossible to be performed because the DM, air was injected into the anterior chamber of the left of a complete lack of cooperation. Therefore, a full examina- eye (Fig. 1B). Bilateral tuck-in PKP under general anesthesia tion was performed under general anesthesia: the right eye was scheduled with a 1-month gap in between. showed severe 360-degree corneal thinning up to the limbus with severe stromal scarring of the temporal half of the cornea affecting the visual axis. An underlying severe Descemet Surgical Technique membrane (DM) tear was observed without acute stromal edema. The intraocular structures were intact; the intraocular Donor Tissue Preparation pressure (IOP) was 6 mm Hg; ultrasound pachymetry values The 60-kHz IntraLase iFS femtosecond laser (AMO were as follows: central (C) 370 mm, temporal (T) 358 mm, Inc, Irvine, CA) was used to create an 8.5-mm-diameter and nasal (N) 270 mm, superior (S) 260 mm, and inferior (I) 310-mm-deep anterior side cut continued with a 300-mm-deep 315 mm. Examination revealed that the left eye was similar to ring lamellar cut up to a diameter of 9.5 mm (8.4–9.5 mm). the right eye, but with a giant central DM tear, which appeared Then, after the ring lamellar cut, the cornea was peripherally detached and retracted within the central 6 mm of the cornea manually dissected using a crescent blade up to the limbus. (Fig. 1A–B), together with overlying moderate corneal edema. Afterward, an 11-mm trephine was used for full-thickness Severe scarring affected the whole stromal surface (Fig. 1A); trephination from the endothelial side, subsequently removing IOP: 5 mm Hg; ultrasound pachymetry: C 330 mm, T 350 mm, the loose superficial rim of the peripheral cornea, finally FIGURE 1. Left eye preoperative external anterior segment photo- graphs before (A) and after (B) in- tracameral air injection. Note the severe diffuse stromal scarring as a consequence of giant DM split and detachment; slit-lamp postoperative anterior segment images 6 months after transplantation in the right (C) and left eyes (D), presenting the latest a fixed pupil as a consequence of Urrets–Zavalia syndrome. Observe the complete transparency of both penetrating grafts and the peripheral partial-thickness donor rim tucked within the host cornea; topography sagittal curvature of the right (E) and left eyes (F) 6 months after surgery shows a high degree of regular astigmatism. 1146 | www.corneajrnl.com Copyright © 2017 Wolters Kluwer Health, Inc. All rights reserved. Copyright Ó 2017 Wolters Kluwer Health, Inc. Unauthorized reproduction of this article is prohibited. Cornea Volume 36, Number 9, September 2017 Femtosecond Laser-Assisted Tuck-In PKP FIGURE 2. Femtosecond laser-as- sisted tuck-in PKP surgical diagram: (A) final appearance of the donor cornea; (B) final appearance of the peripheral recipient stromal pocket; (C) the thread passes through the central 8.5-mm portion of the donor cornea with partial-thickness bite and the anterior portion of the re- cipient’s stromal pocket; (D) the peripheral circumferential donor rim remains tucked into the recipient’s stromal pocket, enhancing the peripheral corneal thickness. obtaining a “top hat-shaped” donor cornea with a central with oral acetazolamide and complete topical hypotensive 8.5-mm full-thickness sector and a 1.25-mm peripheral therapy, but leaving thereafter mild ocular hypertension that partial-thickness rim (Fig. 2A). required long-term topical hypotensive treatment (Fig. 1D). No other postoperative complications or rejection signs were Recipient Preparation recorded. The patient experienced a great visual function The recipient cornea was prepared under sedation in the improvement and recovered a normal academic activity at same manner as the donor with the laser, except for an anterior school. The patient was able to cooperate well throughout the side-cut depth of 160 mm and a ring lamellar cut depth of postoperative ophthalmic examinations. At the sixth-month 150 mm (the recipient side-cut depth was estimated as half of follow-up visit, both corneal grafts were healthy: