Peripheral Neurectomy With Customized Nerve Reconstruction for Periorbital Neuropathic Pain: Initial Experience and Clinical Outcomes.

PURPOSE: To describe a novel, minimally invasive surgical technique to treat severe, intractable periorbital neuropathic pain. METHODS: A retrospective analysis of patients with severe, treatment-refractory periorbital pain who underwent transection of affected sensory trigeminal branches with nerve repair was performed. Collected data included etiology and duration of neuropathic pain, comorbidities, prior treatment history, surgical technique including site of transected sensory nerves and type of nerve repair, preoperative and postoperative pain scores as well as follow-up duration. Differences between preoperative and postoperative values were analyzed by the Wilcoxon signed-rank test. RESULTS: A total of 5 patients with severe periorbital neuropathic pain underwent transection of affected supraorbital, supratrochlear, infratrochlear, infraorbital, zygomaticotemporal, and zygomaticofacial nerves with customized nerve reconstruction. All 5 had improvement of periorbital pain after surgery, with 3 (60%) noting complete resolution of pain and 2 (40%) experiencing partial pain relief over a median follow-up period of 9 months (interquartile range [IQR], 6-19 months). Of the 3 patients who had complete resolution of pain, all reported continued pain relief. Median McGill pain scores significantly decreased from 8.4 (IQR, 8.2-10.0) preoperatively to 0.0 (IQR, 0.0-4.8; p < 0.001) postoperatively. All patients reported satisfaction with the surgical procedure and stated that they would undergo the procedure again if given the option. One patient with history of postherpetic neuralgia (PHN) had reactivation of herpes zoster at postoperative month 3, which was self-limited, without worsening of her neuropathic pain. Another patient with PHN required a staged procedure to achieve complete pain relief. CONCLUSION: Peripheral neurectomy with customized reconstruction of involved sensory nerves can successfully reduce and even eradicate periorbital neuropathic pain that was previously recalcitrant to combination pharmacotherapy and prior neurolysis procedures.

Clinical Outcomes and Patient Satisfaction After Corneal Neurotization.

PURPOSE: The aim of this study was to assess clinical outcomes of corneal neurotization (CN) and determine patient perception of postoperative results. METHODS: This was a retrospective study involving 29 eyes in 28 patients who underwent CN. Chart review data included demographic and clinical history; ophthalmic examination including visual acuity, ocular surface quality, and corneal sensation; surgical technique; and postoperative course. Subjective self-reported patient outcomes of surgical success were also assessed. Only eyes with at least 6 months of follow-up were included in the statistical analysis. RESULTS: A total of 24 eyes and 23 patients were included in statistical analyses. The median postoperative follow-up time was 12.2 months (interquartile range 10.9-18.5 mo). Twenty-three eyes (92%) achieved improvement in ocular surface quality. Eleven of 13 (85%) demonstrated healing of persistent epithelial defects at their last follow-up. Patients gained a median of 2.3 cm in Cochet-Bonnet esthesiometry measurements of sensation. No significant difference was found between preoperative and postoperative visual acuity. All 17 patients who provided self-assessment of their surgical outcome indicated they would undergo CN again if given the choice. Most of the patients reported that the postoperative pain was tolerable, with a median pain score of 3.0 on a 10-point scale (interquartile range 0.0-4.0). Sixteen patients (94%) reported full or partial return of skin sensation along the donor nerve distribution. CONCLUSIONS: CN provides improvement in corneal health and sensibility, with high patient satisfaction and minimal postoperative pain and morbidity.

Corneal neurotization for neurotrophic keratopathy: Review of surgical techniques and outcomes.

Neurotrophic keratopathy is a degenerative disease in which damage to the corneal nerves leads to corneal hypoesthesia. Injuries to neurotrophic corneas are notoriously difficult to treat and have traditionally been approached with supportive management. However, recent progress in the field of corneal neurotization has given new direction for addressing nerve loss directly by stimulating new nerve growth onto the cornea from nearby sensory nerves transferred to the perilimbal region. Herein, we review the surgical techniques utilized in corneal neurotization, including direct transfers and the use of nerve grafts. Considerations in surgical approach, as well as factors that influence prognosis and outcomes of the surgical intervention are also discussed.

Corneal Neurotization for Postherpetic Neurotrophic Keratopathy: Initial Experience and Clinical Outcomes.

PURPOSE: To assess early outcomes of corneal neurotization for postherpetic neurotrophic keratopathy (NK). METHODS: Retrospective analysis of patients who underwent corneal neurotization for postherpetic NK by a single experienced oculoplastic surgeon was performed. Collected data included stage and etiology of NK as well as comorbidities, prior treatment history, neurotization technique, donor nerve site, preoperative and postoperative examination findings (i.e., ocular surface quality, corneal clarity, corneal sensation by Cochet-Bonnet esthesiometry, and visual acuity [VA]), and follow-up duration. Differences between preoperative and postoperative values were analyzed by Wilcoxon signed-rank test. RESULTS: Of 23 adult patients who underwent corneal neurotization, 3 (13%) had history of herpes simplex keratitis and/or endotheliitis, and 4 (17%) had history of herpes zoster ophthalmicus. One patient with herpes zoster ophthalmicus was excluded due to inadequate follow-up duration. Of the 6 patients included in the study, 3 (50%) had Mackie stage 1 disease, 1 (17%) had stage 2, and 2 (33%) had stage 3 with impending perforation, but all had markedly diminished corneal sensation, with a median denervation time of 11.8 months (interquartile range [IQR] 9.4-29.2 months). Following neurotization, median corneal sensation improved significantly from 1.6 cm (interquartile range 0.0-1.9 cm) to 3.6 cm (IQR 3.0-5.6 cm, p = 0.028), with 1 patient achieving full sensation by postoperative month 5. All patients with a persistent epithelial defect preoperatively showed complete corneal healing by their last follow-up visit. VA also improved postoperatively in all patients (p = 0.028). Median follow-up duration was 11.3 months (interquartile range 9.6-17.9 months). CONCLUSIONS: Corneal neurotization can successfully reinnervate corneas previously devitalized by herpetic disease and halt the progressive nature of postherpetic NK. If utilized appropriately and early in the disease process, neurotization may reduce morbidity and maximize visual potential in postherpetic NK.

Prostaglandin-Associated Periorbitopathy in Children and Young Adults with Glaucoma.

OBJECTIVE: To evaluate for relative palpebral and orbital changes after long-term unilateral exposure to prostaglandin analogues (PGAs) in patients with childhood glaucoma. DESIGN: Prospective cross-sectional cohort study. PARTICIPANTS: A total of 29 patients with history of childhood glaucoma, who were treated unilaterally with PGAs for at least 12 months. METHODS: Based on 4 standardized clinical photographs (en face with eyes open, right and left side views with eyes open, and en face with eyes closed), 3 masked expert graders each independently selected the eye they perceived to have received unilateral PGA treatment by physical appearance alone and graded the following features relative to the other eye: (1) ocular (e.g., conjunctival hyperemia, iris heterochromia, and buphthalmos), (2) palpebral (e.g., eyelash trichomegaly, eyelash hypertrichosis, eyelid erythema, eyelid edema, eyelid hyperpigmentation, high upper eyelid crease, upper eyelid ptosis, upper and/or lower eyelid retraction, and eyelid skin atrophy with presence of telangiectasias), and (3) periorbital (e.g., superior sulcus hollowing, proptosis, enophthalmos, hypoglobus, and hyperglobus). An interrater reliability analysis was performed using the Fleiss kappa (κ) statistic to determine consistency among raters. MAIN OUTCOME MEASURES: Frequencies of each feature of prostaglandin-associated periorbitopathy (PAP); group consensus; interrater reliability of selected PGA-treatment laterality. RESULTS: Median unilateral PGA exposure time was 31.7 months (interquartile range: 18.8-44.3 months). Eyelash trichomegaly and hypertrichosis (n = 22, 76%), high upper eyelid crease (n = 20, 69%), upper eyelid ptosis (n = 14, 52%), and superior sulcus hollowing (n = 15, 52%) were the most frequently observed features of PAP in PGA-treated eyes compared with untreated fellow eyes. Most of these changes were mild, but 20% to 30% of patients exhibited moderate eyelash and/or eyelid changes. One patient had severe PAP after long-term unilateral PGA exposure. Group consensus with correctly selected laterality was achieved in all patients. The inter-rater reliability was excellent (κ = 0.815, P < 0.001, 95% confidence interval [0.605, 1.000]). CONCLUSIONS: Mild-to-moderate changes in the ocular adnexa can develop in children and young adults with long-term PGA exposure. Patients and their families should be educated on the possibility of PAP, especially when initiating monocular PGA therapy.

Clinical and Morphologic Outcomes of Minimally Invasive Direct Corneal Neurotization.

PURPOSE: To describe clinical outcomes of a minimally invasive technique for direct corneal neurotization to treat neurotrophic keratopathy. METHODS: All cases of corneal neurotization for neurotrophic keratopathy performed by a single surgeon using minimally invasive direct corneal neurotization were reviewed. The supraorbital donor nerve was directly transferred to the cornea through an upper eyelid crease incision using either a combination of endoscopic and direct visualization or direct visualization alone. Detailed ocular and adnexal examinations as well as Cochet-Bonnet esthesiometry of the affected cornea were performed. Corneal histopathology and in vivo confocal microscopy after minimally invasive direct corneal neurotization were reviewed in one patient who underwent simultaneous penetrating keratoplasty. RESULTS: Five consecutive cases in 4 patients were included, with a mean follow up of 15.8 months (range: 11-23 months). Average denervation time was 17.8 months (range: 6-24 months). Baseline corneal conditions were Mackie stage 1 (20%), Mackie stage 2 (40%), and Mackie stage 3 (40%). All patients demonstrated improvements in corneal sensibility and appearance postoperatively. All patients demonstrated stable or improved visual acuity. No patients developed persistent epithelial defects postoperatively, and all achieved return of tactile skin sensation in the donor nerve sensory distribution. In vivo confocal microscopy after minimally invasive direct corneal neurotization and simultaneous penetrating keratoplasty demonstrated regeneration of corneal nerves. Complications included an asymptomatic small bony excrescence lateral to the supraorbital notch in one patient and cataract progression in the patient who underwent penetrating keratoplasty. CONCLUSIONS: Minimally invasive direct corneal neurotization is a safe and effective treatment of neurotrophic keratopathy.

Corneal Neurotization: A Review of Pathophysiology and Outcomes.

PURPOSE: The objective of this study is to provide a systematic review of the clinical outcomes of corneal neurotization and present the pathophysiology of corneal wound healing, neurotrophic keratopathy, and corneal neurotization. METHODS: A literature review of published articles and meeting abstracts between December 2008 and February 2019 in the English language with the terms "corneal neurotization," "corneal neurotisation," "corneal reinnervation," and "neurotrophic keratopathy" was performed. Reported clinical data before and after corneal neurotization, and surgical techniques, were collected and analyzed. RESULTS: A total of 54 eyes that underwent corneal neurotization were identified. Final Logarithm of the Minimum Angle of Resolution (logMAR) best-corrected visual acuity improved to 0.85 (standard deviation [SD] = 0.65) from 1.25 (SD = 0.71) with a mean improvement of 0.41 (SD = 0.55; p < 0.0001). Central corneal sensation measured using Cochet-Bonnet esthesiometer improved from 2.18 mm (SD = 0.4) to 40.10 mm (SD = 18.66) with a mean filament length change of 38.00 mm (SD = 18.95; p < 0.0001). The median time to the reported maximal sensation return was 8 months (interquartile range 6-10). The most common reported limitation to visual recovery was corneal scarring (31.5%). Children (ages 0-17 years) as compared with adults (ages 18-82 years) had significantly greater final central corneal sensation esthesiometry readings, central corneal sensation return, and improvement in the logMAR best-corrected visual acuity (p < 0.011). CONCLUSIONS: Neurotrophic keratopathy disturbs the homeostatic balance of trophic factors and trigeminal nerve reflexes needed to support ocular surface health and corneal healing. Corneal neurotization can significantly improve corneal sensation and visual acuity and should be considered for the treatment of refractory neurotrophic keratopathy, especially in pediatric populations.

CO2 Laser Lash Tilt Technique for the Treatment of Mild to Moderate Lash Ptosis and Augmentation of Upper Eyelid Blepharoplasty Results.

PURPOSE: To assess the safety, efficacy, and patient satisfaction of a newly described technique for the treatment of mild to moderate lash ptosis performed as augmentation to upper eyelid blepharoplasty. METHODS: Patient medical records for 27 consecutive cases of upper eyelid blepharoplasty between January 2016 and June 2017 and 19 consecutive cases of upper eyelid blepharoplasty with the laser lash tilt procedure between July 2016 and January 2017 performed by the senior author were retrospectively reviewed. Lash position in preoperative and postoperative photographs was graded in a randomized masked fashion on a 4-point scale by 5 oculoplastic surgeons. RESULTS: There was a significant improvement in mean lash position scores for the control group (0.52 ± 0.34, p < 0.001) with mean lash score improving from 1.14 ± 0.46 before surgery to 0.62 ± 0.46 after blepharoplasty alone. There was also significant improvement in mean lash position scores for the laser lash group (0.98 ± 0.52, p < 0.01) with mean lash score improving from 1.66 ± 0.62 before surgery to 0.68 ± 0.50 after blepharoplasty with laser lash treatment. The improvement in lash position seen in the laser lash group was significantly greater than the improvement seen in the control group (p = 0.001). There were no complications and all patients were satisfied with their results. CONCLUSIONS: Compared with upper eyelid blepharoplasty alone, the addition of the CO2 laser lash tilt technique provides a significant improvement in the upward tilt of the eyelashes as they emerge from the eyelid margin.

Idiopathic orbital inflammation with bone destruction and extension into the paranasal sinuses.

Idiopathic orbital inflammation developed in the right orbit of a woman in her mid-thirties, causing tearing, photophobia, diplopia, altered depth perception, proptosis, and pain on eye movements. Computed tomography disclosed a mass involving the intraconal and extraconal nasal right orbit, extending to the orbital apex with anterior displacement of the globe, effacement of the medial rectus muscle, portions of the fat plane, and the superior oblique muscle, and bone destruction with extension of the mass through the orbital floor into the superior maxillary sinus and through the lamina papyracea into the ethmoid sinus. Orbital biopsy disclosed dense fibrous connective tissue with numerous lymphocytes and macrophages. Immunohistochemical stains supported a diagnosis of idiopathic inflammatory pseudotumor involving the orbit and sinus mucosa. Treatment with a prednisone taper and a retrobulbar injection of triamcinolone acetonide have relieved her symptoms and diminished her proptosis. This patient highlights the rare potential of idiopathic orbital inflammation to erode though bone into adjacent cranial structures.

Minimally Invasive Corneal Neurotization With Acellular Nerve Allograft: Surgical Technique and Clinical Outcomes.

PURPOSE: To describe a minimally invasive surgical technique and its clinical outcomes with the use of acellular nerve allograft to re-establish corneal sensibility in patients with neurotrophic keratopathy. METHODS: Acellular nerve allograft was coapted to an intact supraorbital, supratrochlear, or infraorbital nerve and transferred to the affected eye. Donor nerve pedicles were isolated through a transpalpebral or transconjunctival approach. Retrospective evaluation of preoperative and postoperative corneal sensibility, ocular surface, and best-corrected visual acuity was performed in all patients. Mean follow-up period was 6 months (range: 3-10 months). RESULTS: Corneal neurotization with acellular nerve allograft was successfully performed in 7 patients with restoration of corneal sensibility and corneal epithelial integrity. In vivo confocal microscopy demonstrated increased nerve density in corneal stroma at 4 months after surgery. CONCLUSIONS: The use of acellular nerve allograft allows for a minimally invasive approach to successful corneal neurotization.

Adenocarcinoma of Pigmented Ciliary Epithelium in a Child With Aicardi Syndrome and Congenital Microphthalmia With Cyst.

A 5-year-old girl with Aicardi syndrome and microphthalmia with cyst of the OD presented with progressive enlargement of the cyst causing pain. Microophthalmia with inferior cyst (35 × 25 × 12 mm) was noted at birth, and Aicardi syndrome was diagnosed at 10 months by the presence of the classic triad of callosal agenesis, infantile spasms, and chorioretinal lacunae. She underwent enucleation with cyst resection, and subsequent reconstruction with a dermis fat graft. Histopathologic study revealed adenocarcinoma of the pigmented ciliary epithelium. Full-body metastatic workup was negative. Adenocarcinoma of the pigmented ciliary epithelium is an extremely rare eye tumor with only 4 documented cases in the literature, none arising in a microophthalmic eye with cyst. Aicardi syndrome is also a rare disease that has been associated with increased incidence of malignancy and ocular abnormalities, but has never been described in association with microophthalmia with cyst or with adenocarcinoma of the pigmented ciliary epithelium. Herein, the authors present a review of the case and relevant literature.

Endoscopic Corneal Neurotization: Cadaver Feasibility Study.

PURPOSE: The authors describe a cadaver feasibility study investigating a minimally invasive technique for corneal neurotization with the supraorbital nerve harvested endoscopically. METHODS: A cadaver study was performed to investigate the technical feasibility of corneal neurotization via endoscopic supraorbital nerve transfer to the corneoscleral limbus. RESULTS: Endoscopic corneal neurotization was successfully performed on each cadaveric hemiface. CONCLUSION: The use of an endoscope allows for a minimally invasive approach to corneal neurotization with the supraorbital nerve.

Endoscopic Corneal Neurotization: Technique and Initial Experience.

PURPOSE: The authors describe a minimally invasive surgical technique to re-establish corneal sensibility in a patient with neurotrophic keratopathy with the supraorbital nerve harvested endoscopically. METHODS: Pedicled contralateral supraorbital nerve was harvested endoscopically through small eyelid crease and scalp incisions and transferred to the affected eye. RESULTS: Endoscopic corneal neurotization was successfully performed with restoration of corneal sensibility and corneal epithelial integrity. CONCLUSIONS: The use of an endoscope allows for a minimally invasive approach to successful corneal neurotization with the supraorbital nerve.

Perioperative antibiotics for prevention of acute endophthalmitis after cataract surgery.

BACKGROUND: Endophthalmitis is a severe inflammation of the anterior or posterior (or both) chambers of the eye that may be sterile or associated with infection. It is a potentially vision-threatening complication of cataract surgery. Prophylactic measures for endophthalmitis are targeted against various sources of infection. OBJECTIVES: To evaluate the effects of perioperative antibiotic prophylaxis for endophthalmitis following cataract surgery compared with no prophylaxis or other form of prophylaxis. SEARCH METHODS: We searched CENTRAL (which contains the Cochrane Eyes and Vision Trials Register) (2016, Issue 12), Ovid MEDLINE, Epub Ahead of Print, In-Process & Other Non-Indexed Citations, Ovid MEDLINE Daily (January 1946 to December 2016), Embase (January 1980 to December 2016), Latin American and Caribbean Health Sciences Literature Database (LILACS) (1982 to December 2016),the ISRCTN registry (www.isrctn.com/editAdvancedSearch), ClinicalTrials.gov (www.clinicaltrials.gov), and the World Health Organization (WHO) International Clinical Trials Registry Platform (ICTRP) (www.who.int/ictrp/search/en). We used no date or language restrictions in the electronic searches for trials. We last searched the electronic databases on 6 December 2016. We also searched for additional studies that cited any included trials using the Science Citation Index. SELECTION CRITERIA: We included randomized controlled trials that enrolled adults undergoing cataract surgery (any method and incision type) for lens opacities due to any origin. We included trials that evaluated preoperative antibiotics, intraoperative (intracameral, subconjunctival or systemic), or postoperative antibiotic prophylaxis for acute endophthalmitis. We excluded studies that evaluated antiseptic preoperative preparations using agents such as povidone iodine or antibiotics for treating acute endophthalmitis after cataract surgery. DATA COLLECTION AND ANALYSIS: Two review authors independently reviewed abstracts and full-text articles for eligibility, assessed the risk of bias for each included study, and abstracted data. MAIN RESULTS: Five studies met the inclusion criteria for this review, including 101,005 adults and 132 endophthalmitis cases. While the sample size was very large, the heterogeneity of the study designs and modes of antibiotic delivery made it impossible to conduct a formal meta-analysis. Interventions investigated included the utility of adding vancomycin and gentamycin to the irrigating solution compared with standard balanced saline solution irrigation alone, use of intracameral cefuroxime with or without topical levofloxacin perioperatively, periocular penicillin injections and topical chloramphenicol-sulfadimidine drops compared with topical antibiotics alone, and mode of antibiotic delivery (subconjunctival versus retrobulbar injections; fixed versus separate instillation of gatifloxacin and prednisolone). The risk of bias among studies was low to unclear due to information not being reported. We identified one ongoing study.Two studies compared any antibiotic with no antibiotic. One study, which compared irrigation with antibiotics in balanced salt solution (BSS) versus BSS alone, was not sufficiently powered to detect differences in endophthalmitis between groups (very low-certainty evidence). One study found reduced risk of endophthalmitis when combining intracameral cefuroxime and topical levofloxacin (risk ratio (RR) 0.14, 95% confidence interval (CI) 0.03 to 0.63; 8106 participants; high-certainty evidence) or using intracameral cefuroxime alone (RR 0.21, CI 0.06 to 0.74; 8110 participants; high-certainty evidence) compared with placebo, and an uncertain effect when using topical levofloxacin alone compared with placebo (RR 0.72, CI 0.32 to 1.61; 8103 participants; moderate-certainty evidence).Two studies found reduced risk of endophthalmitis when combining antibiotic injections during surgery and topical antibiotics compared with topical antibiotics alone (risk ratio (RR) 0.33, 95% confidence interval (CI) 0.12 to 0.92 (periocular penicillin and topical chloramphenicol-sulfadimidine; 6618 participants; moderate-certainty evidence); and RR 0.20, 95% CI 0.04 to 0.91 (intracameral cefuroxime and topical levofloxacin; 8101 participants; high-certainty evidence)).One study, which compared fixed versus separate instillation of gatifloxacin and prednisolone, was not sufficiently powered to detect differences in endophthalmitis between groups (very low-certainty evidence). Another study found no evidence of a difference in endophthalmitis when comparing subconjunctival versus retrobulbar antibiotic injections (RR 0.85, 95% CI 0.55 to 1.32; 77,015 participants; moderate-certainty evidence).Two studies reported any visual acuity outcome; one study, which compared fixed versus separate instillation of gatifloxacin and prednisolone, reported only that mean visual acuity was the same for both groups at 20 days postoperation. In the other study, the difference in the proportion of eyes with final visual acuity greater than 20/40 following endophthalmitis between groups receiving intracameral cefuroxime with or without topical levofloxacin compared with no intracameral cefuroxime was uncertain (RR 0.69, 95% CI 0.22 to 2.11; 29 participants; moderate-certainty evidence).Only one study reported adverse events (1 of 129 eyes had pupillary membrane in front of the intraocular lens and 8 eyes showed posterior capsule opacity). No study reported outcomes related to quality of life or economic outcomes. AUTHORS' CONCLUSIONS: Multiple measures for preventing endophthalmitis following cataract surgery have been studied. High-certainty evidence shows that injection with cefuroxime with or without topical levofloxacin lowers the chance of endophthalmitis after surgery, and there is moderate-certainty evidence to suggest that using antibiotic eye drops in addition to antibiotic injection probably lowers the chance of endophthalmitis compared with using injections or eye drops alone. Clinical trials with rare outcomes require very large sample sizes and are quite costly to conduct; thus, it is unlikely that many additional clinical trials will be conducted to evaluate currently available prophylaxis. Practitioners should rely on current evidence to make informed decisions regarding prophylaxis choices.

Separate Primary Melanomas of the Bulbar Conjunctiva and Eyelid Skin: Clinical Implications of Multiple Primary Melanomas.

PURPOSE: We report a patient with previous in situ melanoma of the forehead skin who was referred for treatment of a bulbar conjunctival melanoma and a separate superficially invasive melanoma of the eyelid skin, and we offer a review of the biological and clinical implications of patients who have multiple primary melanomas. METHODS: This article offers a clinicopathological correlation with a review of the relevant literature. RESULTS: An 80-year-old white man was referred for evaluation of a suspicious conjunctival tumor and a lower-eyelid lesion. Excisional biopsies revealed that both were primary melanomas arising within in situ disease. Over the span of 25 years, the patient had three separate foci of in situ melanoma, two of which spawned invasive melanoma. CONCLUSION: Separate melanomas arising from the bulbar conjunctiva and eyelid skin have rarely been reported. Multiple primary melanomas of the skin, however, are not uncommon. Based on studies of persons with multiple cutaneous melanomas, the prognosis is best predicted by the tumor with the greatest depth of invasion. Patients with multiple melanomas should be examined for dysplastic nevi, additional cutaneous melanomas, and screened periodically for future lesions. Ongoing studies enrolling patients with multiple primary melanomas are attempting to generate insights into low-penetrance susceptibility genes.

Melanoma-Associated Spongiform Scleropathy in Oculodermal Melanocytosis with Primary Orbital Melanoma.

PURPOSE: To describe spongiform scleropathy in a patient with oculodermal melanosis and without evidence of uveal melanoma. METHODS: Clinical-pathological correlation conducted in compliance with HIPPA (Health Insurance Privacy and Portability Act) regulations. RESULTS: Melanoma-associated spongiform scleropathy was an incidental finding in an 87-year-old woman with oculodermal melanocytosis treated for primary orbital melanoma. All previously reported cases of this scleropathy have been associated with uveal melanoma. CONCLUSIONS: The mechanism of scleral degeneration in melanoma-associated spongiform scleropathy is unknown, and its clinical and prognostic significance is speculative. This is the first case of a so-called melanoma-associated spongiform scleropathy reported in an eye without uveal melanoma.