Levamisole-Adulterated Cocaine-Induced Mucous Membrane Pemphigoid: Case Reports and Literature Review.

PURPOSE: The aim of this study was to report 2 cases of levamisole-adulterated cocaine-induced mucous membrane pemphigoid. METHODS: This study is a review of case reports and literature. RESULTS: Two patients presented with bilateral severe purulent conjunctivitis, corneal ulceration, and rapidly progressive forniceal shortening. Both patients were active cocaine users. A complete blood analysis showed a positive antineutrophil cytoplasmic antibody immunofluorescence with a mixed perinuclear antineutrophil cytoplasmic antibody and cytoplasmic-staining antineutrophil cytoplasmic antibody pattern. Direct immunofluorescence examination of conjunctival tissue showed linear deposition of component 3 and immunoglobulins at the basal membrane. A diagnosis of levamisole-adulterated cocaine-induced mucous membrane pemphigoid was made. In case 1, this suspicion was confirmed by investigating remnants of cocaine on the patient's debit card using mass spectrometry, which contained traces of levamisole. In both cases, aggressive immunosuppressive therapy combining systemic corticosteroids and rituximab was able to control the disease. However, by the time these therapies were initiated, significant corneal injury had occurred requiring corneal grafts in both patients. CONCLUSIONS: Given the rising abuse of cocaine, it is important that ophthalmologists are made aware of its association with severe atypical cicatricial conjunctivitis. To the best of our knowledge, we present the first case proving the causal relationship between levamisole and ocular cicatricial pemphigoid.

Familial cases of a submicroscopic Xp22.2 deletion: genotype-phenotype correlation in microphthalmia with linear skin defects syndrome.

PURPOSE: Microphthalmia with linear skin defects syndrome (MLS or MIDAS, OMIM #309801) is a rare X-linked male-lethal disorder characterized by microphthalmia or other ocular anomalies and skin lesions limited to the face and neck. However, inter- and intrafamilial variability is high. Here we report a familial case of MLS. METHODS: A mother and daughter with MLS underwent a complete ophthalmological examination, and extensive imaging, including anterior segment pictures, corneal topography and keratometry, autofluorescence, infrared reflectance and red free images, as well as spectral-domain optical coherence tomography. The mother also underwent full-field flash electroretinography. In addition, high-resolution array comparative genomic hybridization analysis was performed in both as well as in the maternal grandparents of the proband. RESULTS: Microphthalmia and retinal abnormalities were noted in the proband and the mother, whereas only the mother presented with scars of the typical neonatal linear skin defects. Array comparative genomic hybridization analysis revealed a 185-220 kb deletion on chromosome band Xp22.2 including the entire HCCS gene. CONCLUSIONS: The identification of a deletion including HCCS led to the diagnosis of MLS in these patients. Retinal abnormalities can be part of the ocular manifestations of MLS.

Belgian Endothelial Surgical Transplant of the Cornea (BEST cornea) protocol: clinical and patient-reported outcomes of Ultra-Thin Descemet Stripping Automated Endothelial Keratoplasty (UT-DSAEK) versus Descemet Membrane Endothelial Keratoplasty (DMEK) - a multicentric, randomised, parallel group pragmatic trial in corneal endothelial decompensation.

OBJECTIVES: Corneal blindness is the third most frequent cause of blindness globally. Damage to the corneal endothelium is a leading indication for corneal transplantation, which is typically performed by lamellar endothelial keratoplasty. There are two conventional surgical techniques: Ultra-Thin Descemet Stripping Automated Endothelial Keratoplasty (UT-DSAEK) and Descemet Membrane Endothelial Keratoplasty (DMEK). The purpose of this study is to compare both techniques. METHODS AND ANALYSIS: The trial compares UT-DSAEK and DMEK in terms of clinical and patient reported outcomes using a pragmatic, parallel, multicentric, randomised controlled trial with 1:1 allocation with a sample size of 220 participants across 11 surgical centres. The primary outcome is the change in best-corrected visual acuity at 12 months. Secondary outcomes include corrected and uncorrected vision, refraction, proportion of high vision, quality of life (EQ-5D-5L and VFQ25), endothelial cell counts and corneal thickness at 3, 6 and 12 months follow-up appointments. Adverse events will also be compared 12 months postoperatively. ETHICS AND DISSEMINATION: The protocol was reviewed by ethical committees of 11 participating centres with the sponsor centre issuing the final definitive approval. The results will be disseminated at clinical conferences, by patient partner groups and open access in peer-reviewed journals. GOVERNANCE OF THE TRIAL: Both, trial management group and trial steering committee, are installed with representatives of all stakeholders involved including surgeons, corneal bankers, patients and external experts. TRIAL REGISTRATION NUMBER: NCT05436665.

Correction of the lower eyelid malpositioning in the blepharophimosis-ptosis-epicanthus inversus syndrome.

PURPOSE: Blepharophimosis-ptosis-epicanthus inversus syndrome (BPES) is an autosomal dominant complex eyelid malformation. The authors aim to offer an explanation for the lower eyelid malformation and propose a novel surgical approach to correct it. METHODS: An observational and interventional case series of 10 consecutive, molecularly proven BPES patients who underwent surgical repair of the lower eyelid malformation. During surgery detailed anatomical examination and surgical repositioning of the medial canthal tendon was performed. All the patients were followed up regularly after the surgery and assessed for epiphora. RESULTS: All patients exhibited a marked asymmetry in the attachment of the lower and upper eyelid to the medial canthal tendon, with the lower eyelid being much less attached. This resulted in an abnormal downward concavity with a temporal ectropion and a temporally displaced lower eyelid. Consequently, the inferior punctum was displaced temporally. All patients underwent a novel surgical technique to remediate this, namely, inserting a 4.0 nylon suture between the tarsal plate of the lower eyelid and the medial canthal tendon during telecanthus surgery. This simple additional surgical step corrected not only the position of the lower eyelid but also its abnormal downward concavity, the temporal ectropion and the lateral displacement of the inferior punctum. None of the authors' patients had lasting epiphora. CONCLUSION: Lateral displacement of the inferior punctum is an important hallmark in the diagnosis of BPES. The authors demonstrate an anatomical explanation for the complex lower eyelid malformation and also propose a novel surgical technique to correct this. During surgical repair of the telecanthus and blepharophimosis, specific attention should be paid to reattachment of the lower eyelid to the medial canthal tendon. This understanding improves clinical diagnosis and surgical treatment of BPES patients.

Diagnostic patterns in keratoconus.

PURPOSE: To investigate the current patterns of diagnosis and referral in keratoconus. METHODS: A retrospective chart review was performed of patients who had recently been diagnosed with keratoconus and attended dedicated clinics at Antwerp University Hospital, Belgium and Maria Middelares General Hospital, Ghent, Belgium between June 2013 and February 2018. Exclusion criteria included longstanding keratoconus diagnosis, reduced cognitive capabilities and prior surgical procedures (corneal crosslinking, penetrating keratoplasty or any type of refractive surgery). RESULTS: Three-hundred and ninety-nine patients (722 eyes) were included in this study. The mean age was 24.7 ± 6.5 years and the average maximal keratometry was 51 ± 5.2 D for the better eye and 58.4 ± 9.6 D for the worse eye. Upon diagnosis, 233 eyes (32.2 %) and 51 eyes (7.1 %) had a thinnest pachymetry <450 and <400 µm, respectively. At 6-month follow-up, 58 % of patients had been fitted with specialty contact lenses. During follow-up, 199 eyes (27.6 %) underwent corneal crosslinking. One patient underwent corneal graft surgery of his worse eye due to contact lens intolerance and insufficient visual acuity. CONCLUSION: Despite advances in diagnostic tools, keratoconus is often diagnosed at a relatively late stage. Earlier detection of keratoconus would increase the overall clinical benefit of corneal crosslinking. Further research into screening strategies is required to develop cost-effective screening programs.

Mini-Scleral Lenses Improve Vision-Related Quality of Life in Keratoconus.

PURPOSE: To evaluate the effects of mini-scleral lenses on visual acuity (VA) and visual functioning in patients with keratoconus. METHODS: This prospective, interventional study examined the effects of mini-scleral lenses on VA and visual functioning in patients with keratoconus. Patients younger than 18 years or with a history of refractive surgery or corneal graft surgery were excluded. Patients were fitted with mini-Misa lenses, Senso mini-scleral lenses, or Zenlens mini-scleral lenses. Outcome measures were scleral lens-corrected VA and vision-related quality of life as assessed with the National Eye Institute Visual Functioning Questionnaire (NEI VFQ-39). RESULTS: Eighty-nine eyes of 50 keratoconus patients were included in the study. Median baseline logarithm of the minimal angle of resolution VA with habitual correction was 0.22 (range 0.02-1.04). Mini-scleral lens fitting resulted in a statistically significant visual improvement (median 0; P < 0.0001). At the 6-month follow-up, 11 patients (22%) had abandoned mini-scleral lens wear, primarily because of difficulties with lens handling (7 patients). Of the 39 patients with continued wear, 33 patients (84.6%) wore their lenses for a daily average of 12 hours. NEI-VFQ scoring in these patients showed significantly improved results for both visual functioning and socioemotional scales after scleral lens fitting (P < 0.0001). CONCLUSIONS: Mini-scleral lenses significantly improve VA and visual functioning on NEI-VFQ in patients with keratoconus. Difficulties with lens insertion and removal are the principal reason for lens dropout.

Repeatability of the Pentacam HR in Various Grades of Keratoconus.

PURPOSE: To evaluate the repeatability of an extensive number of relevant indices with the Pentacam HR in keratoconus of varying severity and normal eyes. DESIGN: Reliability analysis. METHODS: This study was performed at Antwerp University Hospital, Belgium, and enrolled 20 healthy volunteers (20 eyes) and 69 patients (69 eyes) with keratoconus. Three consecutive measurements were performed by the same operator with Pentacam HR in keratoconus and normal eyes. Exclusion criteria included past ocular surgery, recent rigid contact lens wear, and corneal scarring. The keratoconus group was subdivided according to the Belin/Ambrosio total deviation value: subclinical, mild, and moderate. The within-subject standard deviation and repeatability limit were computed for repeatability assessment. The tolerance index (TI) was calculated to compare across parameters with different measurement scales. For the sample size included, TI > 0.36 signified statistical significance at the 0.05 level. RESULTS: Repeatability in subclinical keratoconus did not differ significantly from controls (P > .05), except for wavefront aberrations. In mild keratoconus, 11 of 18 (61.1%) anterior corneal, 7 of 14 (50%) posterior corneal, 2 of 5 (40%) pachymetry, 7 of 11 (63.6%) combined, and 1 of 6 (16.7%) densitometry parameters showed significantly worse repeatability compared to controls (TI > 0.36). Repeatability of most parameters worsened in moderate disease. In particular, maximal keratometry and anterior astigmatism showed significantly worse repeatability in moderate keratoconus. CONCLUSIONS: Measurement variability of Pentacam HR is of clinical relevance when assessing for progression of keratoconus. We provide reference repeatability values and scale independent analysis of relevant corneal parameters in keratoconus of varying degrees.

European eye bank association.

BACKGROUND: The European Eye Bank Association (EEBA) is a technical-scientific organization for eye banks. Founded in 1989 with the simple objective of sharing information on eye banking, the Association is today the leading pan-national association in Europe dedicated to the advancement of eye banking and an authoritative reference point for eye banks which work according to quality standards. METHODS: The Association establishes and maintains an agreed set of medical and technical standards, promotes the collection of data on eye bank activities and processes, provides opportunities for the discussion of all aspects of eye banking practice, including eye donor selection and procurement, relevant research and development, education and training in eye banking, and maintains linkage with national and international corneal transplant communities and relevant bodies. RESULTS: The recent introduction of a more structured and focused committee, a permanent secretariat, the development of a website has enabled the Association to establish closer links and collaborative activities with key regulatory bodies and to provide a more constant exchange of clinical, scientific and technical ideas and best practice with fellow professionals by means of its annual meetings, the EEBA directory and website, and a regular newsletter. CONCLUSION: The EEBA is a scientific organization committed to defining minimum standards and to encouraging eye banks to maintain the highest possible standards for quality and safety. Through its annual meetings, and the collection and exchange of detailed information from member eye banks, the Association can rightly claim to speak with a confident and representative voice on eye banking in Europe.

Ectopic meningioma anterior to the lacrimal gland fossa.

A 66-year-old man reported a slowly growing tumor on the lateral edge of his left upper eyelid. This lesion was hard but movable on palpation. A neoplasm of the lacrimal gland was suspected. CT showed a highly calcified lesion at the left upper eyelid. Resection of the tumor was performed, which was located just behind the orbital septum and in front of the lacrimal gland. Anatomopathologic investigation of the excised specimen with immunohistochemistry revealed a benign meningioma of a meningotheliomatous type, containing multiple bone elements. An ectopic orbital meningioma is rare, and this is the first case of a unique lateral localization of this lesion. Therefore, it should be included in the differential diagnosis of a lacrimal gland tumor.

Inverted papilloma arising primarily from the lacrimal sac.

A 24-year-old female was referred with epiphora and a non-tender swelling within the right lacrimal fossa, present for 6 months. There were no previous episodes of dacryocystitis. Computed tomography showed a homogenous mass within the lacrimal fossa without contrast enhancement. Pathology following resection of the whole lacimal sac revealed the presence of an inverted papilloma (IP) with a sharp transition between the papilloma and the normal lacrimal duct epithelium. In situ hybridisation was positive for low risk HPV subtypes. An IP is a benign but infiltrative epithelial neoplasm with malignant potential characterised by a high recurrence rate. Majority of cases arise from the nasal wall and paranasal sinuses. In this case report, we demonstrate an IP confined to the lacrimal sac with clear-cut pathology proof, which implicates the lacrimal sac as a primary site of origin.

The Outcome of Scleral Lens Fitting for Keratoconus With Resolved Corneal Hydrops.

PURPOSE: To investigate the success and failure of scleral lens fitting in patients with keratoconus with resolved hydrops. METHODS: Retrospective case series of patients with keratoconus who attended the Ophthalmology Department at Antwerp University Hospital, Belgium, and developed acute hydrops between January 1, 2015, and June 30, 2018. The exclusion criteria included amblyopia, mental retardation, and insufficient follow-up. RESULTS: Of the 25 patients with keratoconus with acute hydrops, 14 patients (16 eyes) were included in this study. Median age was 31 years (range 19-52 years). Before hydrops, the median maximum keratometry value of affected eyes was 81.3 D (range 66.3-108.3 D). Acute hydrops was managed medically in 5 eyes (31.3%), by observation in 5 eyes (31.3%), by application of bandage contact lens in 1 eye (6.3%), and compression corneal sutures in 5 eyes (31.3%). Contact lens fitting after hydrops was not necessary for 2 patients (2 eyes; 12.5%) because of excellent vision in the fellow eye. Of the remaining patients, 11 (68.8%) were successfully fitted with mini-scleral lenses, with a median visual acuity of 0.7 (0.5-0.9; decimal Snellen). Three patients (3 eyes; 18.8%) proceeded to graft surgery because of limited visual acuity with scleral lenses (n = 2) or persistent edema (n = 1). CONCLUSIONS: Scleral lenses are effective for providing satisfactory visual acuity after corneal hydrops in keratoconus. A scleral lens trial is advised before listing patients for graft surgery.

Cutting and pasting corneas: combination of a corneal allograft with relocation of a crescent of autologous corneal tissue in therapeutic penetrating keratoplasty.

PURPOSE: A therapeutic penetrating keratoplasty (TPKP) technique that achieves satisfactory visual rehabilitation with a minimal risk of rejection is described. METHODS: A patient presented with a central corneal perforation in the left eye extending to the temporal limbus. We performed a novel surgical procedure, in which a corneal allograft was combined with relocation of a crescent of autologous corneal tissue. RESULTS: The TPKP placed the optical zone of the donor cornea over the center of the entrance pupil. The chances of an immunologic rejection were minimized by intercalating a crescent of autologous tissue between the allograft and the limbal vessels. CONCLUSION: The combination of an allograft with a crescent of autologous corneal tissue minimizes the disadvantages associated with eccentric or oversized trephination.

A multilevel analysis of factors influencing the flow efficiency of the cataract surgery process in hospitals.

PURPOSE: To detect factors contributing to variation in cataract surgery processes. METHODS: A multilevel study was conducted to compare the process of cataract surgery between hospitals in Belgium. The main data were collected through non-participative observations and time measurements in four hospitals. Surgeons (n = 16) performing cataract surgery in the selected region and their patients (n = 274) undergoing cataract surgery were observed. Flow efficiency is measured in the operating room (OR) as time for preparation, surgery, exit and turnover. RESULTS: Flow efficiency in the OR can be negatively influenced by the severity of the cataract [+2.778 (1.139) min in preparation time (p < 0.05); +4.616 (1.786) min in surgery time when severe cataract (p < 0.05)] and the presence of special-cause variation [+2.832 (1.893) min preparation time (p < 0.05); +2.503 (1.277) min surgery time (p < 0.05); +1.181 (0.350) min exit time (p ≤ 0.001)]. Administering topical analgesia instead of peribulbar [+13.548 (4.436) min preparation time (p ≤ 0.001)], retrobulbar [+3.856 (1.548) min surgery time (p ≤ 0.05)] or general analgesia [+5.617 (2.536) min surgery time (p < 0.05); +5.175 (0.817) min exit time (p ≤ 0.001)] enhances flow efficiency. The experience of surgeons (>15 years) impacts flow efficiency [+12.838 (5.922) min surgery time when low experience]. The volume of cataracts performed annually per surgeon did not have a significant impact on flow efficiency. The use of specialized scrub nurses [-7.146 (3.099) min preparation time (p ≤ 0.05); -2.116 (0.586) min turnover time (p ≤ 0.05)] and the eye clinic design [-1.742 (0.686) min exit time (p < 0.05); 2.296 (1.034) min turnover time (p ≤ 0.05)] benefit flow efficiency. CONCLUSION: Controllable and uncontrollable factors with clinical and organizational causes influencing flow efficiency in the cataract process were found. These factors can be taken into account in the management of the healthcare process.

Role of natural killer cells in the rejection process of corneal allografts in rats.

BACKGROUND: The exact mechanism of human corneal allograft rejection, which is the major cause of corneal transplant failure, remains unclear. We investigated the role of natural killer (NK) cells in rat corneal allograft rejection by examining the aqueous humor (AH) cell infiltrate on different postoperative days. METHODS: Flow cytometric analysis was performed on the AH and submandibular draining lymph node (DLN) cells before transplantation and at different time points thereafter. In addition, we performed functional cytotoxicity assays with cells present in the AH during corneal rejection. RESULTS: We demonstrated a gradual increase in the absolute cell number of different hematopoietic subpopulations in the AH after allogeneic cornea transplantation. CD3CD4 cells, mainly monocytes and macrophages, were the predominant subpopulation 2 days after transplantation, followed by a successive relative increase of CD4 T cells, CD8 T cells, CD161 T cells, and NK cells. NK and CD161 T cells were present at a 10- to 15-fold higher percentage than in the DLN, suggestive of local expansion of these cells. A higher percentage of NK cells were CD8-negative compared with DLN NK cells. AH cells specifically lysed allogeneic cells, and this cytotoxicity was mainly attributable to NK cells but not to CD4 or CD8 T lymphocytes. CONCLUSION: These results confirm the crucial role of CD4 cells in the allogeneic corneal graft rejection process and implicate NK cells as possible mediators of the rejection.

Factors influencing the decline in endothelial cell density after corneal allograft rejection.

PURPOSE: Corneal allograft rejection is one of the major causes of transplant failure. The purpose of the current study was to examine the decline in endothelial cell density (ECD) in patients experiencing allograft rejection, by comparing this decline with the normal evolution in patients who undergo penetrating keratoplasty (PKP) and to identify possible factors predictive of this endothelial cell loss after corneal allograft rejection. METHODS: In a case-control study of 45 corneas that underwent corneal allograft rejection, specular microscopy photographs taken within the shortest time preceding the onset of rejection and after the resolution of the rejection were analyzed. RESULTS: The observed percentage loss of ECD in 21 (47%) corneas was not significantly greater than expected. A second group of 13 (29%) corneas showed a decline in ECD that was significantly greater than expected. Finally there were 11 corneas (24%) in which endothelial cells were no longer observable. The only two risk factors that reached statistical significance after multiple logistic regression analysis were a delay in diagnosis (a delay of >1 day yielded an odds ratio of 10.40; P=0.02) and a recipient age of more than 60 years (odds ratio, 6.95; P=0.04). CONCLUSIONS: Corneal allograft rejection does not necessarily cause a higher than expected endothelial cell loss; almost half of the patients in this study showed a decline in ECD that is comparable to the decline in patients who undergo PKP and have an uneventful follow-up. The most important variable influencing the extent of endothelial cells loss is a delay in diagnosis and treatment.

Confirmation of ofloxacin precipitation in corneal deposits by microbore liquid chromatography-quadrupole time-of-flight tandem mass spectrometry.

We investigated the corneal precipitate of a 6-year-old boy with vernal keratoconjunctivitis (VKC), treated with topical ofloxacin 0.3% eyedrops. Because of the extremely small sample amount (corneal scraping), a very sensitive and specific method was needed with the possibility of an unambiguous identification of ofloxacin, supposed to be present in the precipitate. In this respect, tandem Q-TOF mass spectrometry combined with micro LC (1 mm I.D.) was chosen. Confirmation of the presence of ofloxacin in the deposit was obtained by means of the characteristic product ion spectrum produced by CID. This clearly indicated that the precipitate, removed by corneal scraping from the 6-year-old boy with VKC, contained ofloxacin.

Therapeutic penetrating keratoplasty: clinical outcome and evolution of endothelial cell density.

PURPOSE: To identify the influence of the underlying etiology on the outcome of therapeutic penetrating keratoplasty (PKP) in the management of corneal perforation, impending perforation or refractory corneal disease. METHODS: A retrospective observational case series with 28 patients (28 eyes) was performed. Patients were divided in two subgroups, based on the underlying ocular disease leading to the therapeutic PKP. Group I consisted of 15 patients (54%) with an infectious etiology, the other 13 patients (46%) with a noninfectious condition belonged to group II. Mean follow up was 23.2 months (median 21 months). RESULTS: An enucleation could be avoided in all but two cases. In group I, 12 of 15 (80%) grafts remained clear, compared with only 3 of 13 (23%) in group II (p = 0.0004). Visual acuity (VA) amelioration was achieved in 20 of 28 patients (71%). In group I the postoperative VA was significantly better than preoperatively (p = 0.002); however, in group II the improvement was only borderline significant (p = 0.05). Overall five out of ten (50%) patients with a therapeutic PKP in group I had an endothelial cell density >or= 1900 cells/mm2 at the time of their last specular microscopy. CONCLUSION: Our study demonstrates the importance of underlying etiology on the outcome of therapeutic PKP. Graft survival and postoperative visual acuity varied widely depending on the underlying ocular disease, and it was clear that therapeutic PKP has a much better prognosis in infectious conditions when compared with noninfectious conditions. We believe that these good results can justify the use of therapeutic PKP in selected cases of infectious keratitis.

Corneal manifestations and in vivo confocal microscopy of Gaucher disease.

PURPOSE: To report corneal abnormalities and confocal microscopy findings in a patient with a variant of Gaucher disease (GD). METHODS: Case report with slit-lamp photography, confocal microscopy, and molecular analysis of the glucocerebrosidase gene. RESULTS: Ophthalmic evaluation in a 57-year-old white patient demonstrated corneal opacities scattered throughout the cornea. Confocal microscopy revealed a completely distorted stromal architecture. The anterior part showed keratocytes with an abnormal morphology intermingled with minute white dots. In the posterior part, normal keratocytes were virtually absent and replaced by hyperreflective rod-like structures. Analysis of the glucocerebrosidase gene disclosed a heterozygous F216Y/L444P mutation. The patient's old records revealed that these corneal abnormalities were already present at the age of 16 years, almost 15 years before the diagnosis of GD was made. His 2 siblings known with the same disorder and mutations also showed abnormal visual acuity and increased central corneal thickness. The confocal microscopy demonstrated some subclinical abnormalities, but otherwise normal corneas. CONCLUSIONS: Our patient had an unusual mutation responsible for his GD. Although corneal opacities are virtually unknown in GD, except in the D409H homozygous cardiovascular subtype, this patient had marked corneal stromal abnormalities.

Confocal microscopy of corneal crystals in a patient with hereditary tyrosinemia type I, treated with NTBC.

PURPOSE: To describe the confocal microscopic findings in a patient with hereditary tyrosinemia type I (HT-I) treated with 2-(2-nitro-4-trifluoromethylbenzoyl)-1,3-cyclohexanedione (NTBC) who developed corneal crystals. METHODS: In this case study, we describe the confocal microscopic findings in a boy, who was diagnosed with HT-I at the age of 4 months. At 16 years of age, he developed painful corneal lesions in both eyes. On slit-lamp examination, whorl-like branching epithelial corneal lesions were found, staining faintly with fluorescein. His NTBC treatment was stopped and reintroduced at a lower dose after 1 month. The lesions clearly regressed, leaving only mild residual epithelial scarring, without fluorescein staining and without pain. Confocal microscopy was performed in the acute painful stage and in the asymptomatic convalescent stage 5 months later. RESULTS: Confocal microscopy confirmed the presence of slender birefringent spiky crystals in the very superficial corneal epithelium. In the asymptomatic convalescent phase, the crystals clearly persisted on confocal microscopy, although they were barely visible on slit-lamp examination. CONCLUSIONS: This is the first in vivo demonstration by confocal microscopy of corneal crystals present in a patient with proven type I tyrosinemia, under NTBC treatment.