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= Disease Entity = == Disease == Brown-McLean Syndrome (BMS) describes a condition in which the peripheral (2-3mm) inferior cornea become edematous and progresses circumferentially, sparing the central cornea in most cases.<ref name="brown1">Brown, S., and McLean, J. Peripheral corneal edema after cataract extraction a new clinical entity. Tr Am Acad Ophth Otol. 1969; 79:465. </ref> <ref name="brown2">Brown, S. Peripheral Corneal Edema After Cataract Extraction. AJO. 1970; 70: 326-328. </ref> First described in 1969<ref name="brown1"/> as “peripheral corneal edema after cataract extraction,” it was later renamed to Brown-McLean syndrome.<ref name="charlin3">Charlin, R. Peripheral corneal edema after cataract extraction. Am J Ophthalmol. 1985; 99:298-303. </ref> == Etiology == Although it was initially believed to be associated with an underlying endothelial dystrophy, further supported by a possible genetic predisposition as some cases of relatives presenting with this condition have been identified,<ref name="brown2"/><ref name="gothard4">Gothard T., Hardten D., Lane S., et al. Clinical findings in Brown-McClean Syndrome. Am J Ophthalmol. 1993; 115:729-737. </ref> <ref name="vote5">Vote, B., Grupcheva, C., Ormonde, S., McGhee C. In vivo confocal microstructural analysis and surgical management of Brown McLean syndrome associated with spontaneous crystalline lens luxation. J Cataract Refract Surg. 2003; 29: 614-618. </ref> no causative dystrophy or gene has been identified.<ref name="charlin3"/><ref name="girard6">Girard, L. Peripheral corneal edema after cataract extraction. Am J Of Ophthalmology. 1985; 100: 353. </ref> <ref name="charlin7">Charlin, R. Peripheral corneal edema after cataract extraction:Reply. Am J Of Ophthalmology. 1985; 100: 354. </ref> == Pathophysiology == While its pathophysiology is still unknown, it was initially described in aphakic patients classically after intracapsular lens extraction.<ref name="brown1"/> <ref name="brown2"/> Most patients were elderly although patients as young as 12 years old have been reported.<ref name="almousa8">Almousa, R., Johns, S., Gibson, R. Atypical clinical presentations of Brown-McLean syndrome. Eye. 2007; 21: 249-250. </ref> <ref name="tourkmani9">Tourkmani, A., Martinez, J., Berrones, D., et al. Brown-McLean Syndrome in a Pediatric Patient. Case Reports in Ophthalmology. 2015; 6:139-142. </ref> It typically occurs several years after surgery, averaging 6-16 years post-operative.<ref name="parejaesteban10">Pareja-Esteban, J., Montes, M., Perez-Rico, C., et al. Brown McLean Syndrome after insertion of an anterior chamber intraocular lens: description of one case. Arch Soc Esp Oftalmol. 2007; 82: 315-318. </ref> Some speculations that a genetic predisposition for endothelial disease coupled with endothelial trauma have been proposed.<ref name="charlin3"/> Other surgeries have been associated with BMS including include PK,<ref name="sugar11">Sugar, A. Brown McLean syndrome occurring in a corneal graft. Cornea. 1997; 16: 493-494. </ref> anterior chamber intraocular lens (ACIOL),<ref name="parejaesteban10"/><ref name="diazllopis12">Diaz-Llopis, M., Garcia-Delpech, S., Salom, D., Udaondo, P. Brown McLean syndrome and refractive phakic anterior chamber intraocular lenses. Arch Soc Esp Ofthalmol. 2007; 82: 737-740. </ref> phacoemulsification,<ref name="mallikarjun13">Mallikarjun, M., Kavitha, v., Rajashekar, J., et al. Brown-McLean Syndrome after phacoemulsification. Indian Journal of Ophthalmology. 2019; 67: 1710-1711. </ref> and pars plane vitrectomy with lensectomy.<ref name="gothard4"/> Interestingly, endothelial trauma is not necessary to induce BMS. Ultrasound biomicroscopy does not always reveal the presence of iridocorneal apposition<ref name="sugar11"/> and the presence of an iridectomy is not always reported as protective.<ref name="suwan14">Suwan, Y., Teekhasaenee, C., Lekhanont, K., Supakontanasan, W. Brown-McLean syndrome: the role of iridodenesis. Clinical Ophthalmology. 2016; 10: 672-677. </ref> <ref name="lim15">Lim, J., Lam, S., Sugar, J. Brown-McLean syndrome. Arch Ophthalmol. 1991; 109:22-23. </ref> Non-surgical cases of BMS include patient who ha ve had lens subluxation,<ref name="brown2"/> <ref name="vote5"/>spontaneous lens resorption,<ref name="brown2"/><ref name="charlin3"/> endothelitis,<ref name="hara16">Hara, T., Hara, T. Brown McLean Syndrome associated with corneal endothelitis in a pseudophakic eye. J Cataract Refract Surg. 1993; 19:780-786. </ref> keratoconus,<ref name="jamil17">Jamil, A., Rahman, F., Mirza, K., Igbal, W. Brown-McLean syndrome with keratoconus. J Coll Physicians Surg Pak. 2012; 22: 179-181. </ref> angle closure glaucoma<ref name="gothard4"/> or myotonic dystrophy.<ref name="rutzen18">Rutzen, A., Deen, A., Epstein, A., et al. Cataract surgery in a patient with Brown-McLean syndrome. J Cataract Refract Surg. 2001; 27: 1335-1337. </ref> = Diagnosis = == History == As mentioned above, a prior history of intracapsular or extra capsular lens extraction is often present but other intraocular surgeries as well as non-surgical ocular conditions can also be seen with BMS. == Symptoms == Although usually asymptomatic, when present, patients often complain of foreign body sensation and photophobia.<ref name="gothard4"/><ref name="martins19">Martins, E., Alvarenga, L., Sousa, L., et al. Anterior stromal puncture in Brown-McLean Syndrome. J Cataract Refract Surg. 2004;30:1575-1577. </ref> == Physical examination == Slit lamp examination often reveals peripheral inferior corneal edema initially, involving the limbus.<ref name="tanaka20">Tanaka, M., Matsuda, M., Manabe R. Brown McLean syndrome- a report of three cases. Folia Ophthalmol Jpn. 1989; 40: 2618-2622. </ref> It generally spares the superior cornea<ref name="charlin3"/><ref name="suwan14"/> although rare case reports describe the involvement of superior peripheral corneal edema at the onset.<ref name="almousa8"/><ref name="sugar11"/> Iridodenesis is often present, believed to cause intermittent endothelial trauma and the characteristic deposition of brown/orange endopigment in BMS.<ref name="brown2"/> <ref name="charlin3"/> Iris atrophy underlying the areas of corneal edema have also been described.<ref name="brown1"/> <ref name="brown2"/> The central cornea tends to have few if any guttae.<ref name="gothard4"/> <ref name="almousa8"/> While transient central corneal swelling associated with elevated rises in intraocular pressure has been noted in these patients,<ref name="brown1"/> <ref name="brown2"/> <ref name="gothard4"/> persistent central edema occurs rarely and in severe cases.<ref name="sugar11"/> Gonioscopy appears wide and deep with variable pigmentation of the trabecular meshwork.<ref name="brown2"/> <ref name="vote5"/> In the setting of a transplanted cornea with BMS, pigment and edema was found to start more centrally in the donor graft with a peripheral zone of no pigment prior to the graft host junction.<ref name="sugar11"/> High myopia is sometimes associated with BMS, with prevalence ranging from 40-61% in some cases.<ref name="charlin3"/><ref name="suwan14"/> == Diagnostic procedures == Specular microscopy studies reveal that central corneal endothelial count and morphology remain normal; the peripheral corneal endothelium often have decreased cell count and morphology although can be normal as well. endothelial count often decreases and morphology alters although can also be normal.<ref name="diazllopis12"/> <ref name="hara16"/><ref name="reed21">Reed, J., Cain, L., Weaver, R., Oberfeld S. Clinical and pathologic findings of aphasic peripheral corneal edema: Brown-McLean syndrome. Cornea. 1992; 11: 577-583. </ref> <ref name="kam22">Kam, K., Jhansi, V., Young, A. Brown McLean Syndrome. BMJ Case Report. 2013; dot: 10.1136/bcr-2013-201280. </ref> <ref name="mai23">Mai, H., Hamilton, D. Annular Amniotic Membrane Transplantation as a Host Incorporated Graft in the Management of Brown McLean Syndrome. Cornea. 2013; 32: 714-715. </ref> Similarly, in vivo confocal microscopy of the peripheral cornea generally shows enlarged corneal nerves, and fibrosis of the Bowman’s layer as well as irregularly shaped and sized basal epithelium but can also be normal in some cases.<ref name="vote5"/><ref name="lim24">Lim, L., Tarafdar, S., Collins, C., et al. Corneal endothelium in Brown McLean Syndrome: In-vivo Confocal Microscopy Finding. Seminars in Ophthalmology. 2012; 27: 6-7. </ref> In vivo confocal microscopy of the central cornea generally normal endothelium but did reveal grouped large keratocytes with prominent nuclei in the posterior stroma as well as thick corneal nerves.<ref name="vote5"/> On electron microcopy, the peripheral corneas of BMS showed abnormal posterior collagen in Descemet’s with destroyed endothelial cells.<ref name="reed21"/> Scanning electron microcopy can show a distinct line between normal and diseased endothelium.<ref name="reed21"/> Anterior segment optical coherence tomography (AS-OCT) shows thickened peripheral cornea.<ref name="kam22"/> Scheimpflug imaging shows thickened peripheral cornea with normal thickness central cornea.<ref name="mallikarjun13"/><ref name="kam22"/> = Management = == Medical therapy == Most cases of BMS are responsive to hypertonic saline and topical steroids.<ref name="parejaesteban10"/> <ref name="hara16"/> <ref name="jamil17"/> Patients can also trial contact lenses, which has been shown to be well tolerated despite the peripheral corneal edema.<ref name="tuft25">Tuft, S., Kerr, M., Sherrard, E., Buckley, R. Peripheral Corneal Edema following cataract extraction (Brown-McLean Syndrome). Eye. 1992; 6: 502-505. </ref> == Surgical therapy == For refractory or symptomatic cases, surgical management may be warranted. In recurring cases of epithelial bullae secondary to BMS, an annular amniotic membrane transplant using two trephines of different diameters placed basement membrane side up has been shown to be successful while maintaining central visual acuity.<ref name="mai23"/> Anterior stromal puncture with 23-G or 25-G needle can be used as well to induce collagen expression thereby improving epithelial cell adhesion and subepithelial fibrosis, both of which improve blockade of fluid penetration into the corneal epithelium. <ref name="martins19"/><ref name="katsev26">Katsev, D., Kincaid, M., Fouraker, B., et al. Recurrent corneal erosion: pathology of corneal punchure. Cornea. 1991; 10: 418-423. </ref> In the cases of ACIOL-associated BMS, treatment often includes removal of the ACIOL for resolution of the corneal edema.<ref name="diazllopis12"/> In untreated cases where central corneal decompensation occurs, a corneal transplant may be necessary.<ref name="brown2"/><ref name="sugar11"/><ref name="diazllopis12"/> == Complications == Ruptured bullae from BMS can result in infectious corneal ulcerations.<ref name="gothard4"/><ref name="morenomontanes27"> Moreno-Montanes, J., Heras, J., Rodriguez, R. Varibilidad en la presentación del síndrome de Brown-McLean. Arch Soc Esp Ofthalmol. 2005; 79: 299-302. </ref> = References = <references /> <ol> <li value="28">Mohebbi, M., Shadravan, M., Pour, E., et al. Brown-McLean syndrome in a patient with Hallermann-Streiff Syndrome. KJO. 2016; 30: 76-77. </li> <li>Vogel, M., Petrosyan, T., Chin, B., et al. Brown-McLean syndrome. Optometry. 2011; 82: 485-488. </ref> <li>Sun, B., He, Y., Zhong, P., Wang, R. [Peripheral corneal edema after cataract extraction]. Zhonghua Yan Ke Za Zhi. 1998; 34: 31-33. </li> <li>Charlin, R., Quintano, R. Edema corneal periferico post facoeresis. Sindrome de Brown McLean. Arch Chilenos Ofthalmol. 1980; 37:17. </li> <li>Jaffe, N. Corneal Edeman. In Jaffe, N., Jaffe, M., and Jaffe, G (eds). Cataract surgery and its complications, ed. 5. St. Louis, C.V. Mosby, 1990. p. 429. </li> </li></ol>
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