Brown McLean Syndrome

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 by Daniel Anderson, MD on April 28, 2020.


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.[1] [2] First described in 1969[1] as “peripheral corneal edema after cataract extraction,” it was later renamed to Brown-McLean syndrome.[3]

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,[2][4] [5] no causative dystrophy or gene has been identified.[3][6] [7]

Pathophysiology

While its pathophysiology is still unknown, it was initially described in aphakic patients classically after intracapsular lens extraction.[1] [2] Most patients were elderly although patients as young as 12 years old have been reported.[8] [9] It typically occurs several years after surgery, averaging 6-16 years post-operative.[10] Some speculations that a genetic predisposition for endothelial disease coupled with endothelial trauma have been proposed.[3] Other surgeries have been associated with BMS including include PK,[11] anterior chamber intraocular lens (ACIOL),[10][12] phacoemulsification,[13] and pars plane vitrectomy with lensectomy.[4]

Interestingly, endothelial trauma is not necessary to induce BMS. Ultrasound biomicroscopy does not always reveal the presence of iridocorneal apposition[11] and the presence of an iridectomy is not always reported as protective.[14] [15] Non-surgical cases of BMS include patient who ha ve had lens subluxation,[2] [5]spontaneous lens resorption,[2][3] endothelitis,[16] keratoconus,[17] angle closure glaucoma[4] or myotonic dystrophy.[18]

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.[4][19]

Physical examination

Slit lamp examination often reveals peripheral inferior corneal edema initially, involving the limbus.[20] It generally spares the superior cornea[3][14] although rare case reports describe the involvement of superior peripheral corneal edema at the onset.[8][11]

Iridodenesis is often present, believed to cause intermittent endothelial trauma and the characteristic deposition of brown/orange endopigment in BMS.[2] [3] Iris atrophy underlying the areas of corneal edema have also been described.[1] [2] The central cornea tends to have few if any guttae.[4] [8] While transient central corneal swelling associated with elevated rises in intraocular pressure has been noted in these patients,[1] [2] [4] persistent central edema occurs rarely and in severe cases.[11] Gonioscopy appears wide and deep with variable pigmentation of the trabecular meshwork.[2] [5] 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.[11] High myopia is sometimes associated with BMS, with prevalence ranging from 40-61% in some cases.[3][14]

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.[12] [16][21] [22] [23]


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.[5][24] 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.[5]

On electron microcopy, the peripheral corneas of BMS showed abnormal posterior collagen in Descemet’s with destroyed endothelial cells.[21] Scanning electron microcopy can show a distinct line between normal and diseased endothelium.[21] Anterior segment optical coherence tomography (AS-OCT) shows thickened peripheral cornea.[22] Scheimpflug imaging shows thickened peripheral cornea with normal thickness central cornea.[13][22]

Management

Medical therapy

Most cases of BMS are responsive to hypertonic saline and topical steroids.[10] [16] [17] Patients can also trial contact lenses, which has been shown to be well tolerated despite the peripheral corneal edema.[25]

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.[23] 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. [19][26]

In the cases of ACIOL-associated BMS, treatment often includes removal of the ACIOL for resolution of the corneal edema.[12] In untreated cases where central corneal decompensation occurs, a corneal transplant may be necessary.[2][11][12]

Complications

Ruptured bullae from BMS can result in infectious corneal ulcerations.[4][27]

References

  1. 1.0 1.1 1.2 1.3 1.4 Brown, S., and McLean, J. Peripheral corneal edema after cataract extraction a new clinical entity. Tr Am Acad Ophth Otol. 1969; 79:465.
  2. 2.0 2.1 2.2 2.3 2.4 2.5 2.6 2.7 2.8 2.9 Brown, S. Peripheral Corneal Edema After Cataract Extraction. AJO. 1970; 70: 326-328.
  3. 3.0 3.1 3.2 3.3 3.4 3.5 3.6 Charlin, R. Peripheral corneal edema after cataract extraction. Am J Ophthalmol. 1985; 99:298-303.
  4. 4.0 4.1 4.2 4.3 4.4 4.5 4.6 Gothard T., Hardten D., Lane S., et al. Clinical findings in Brown-McClean Syndrome. Am J Ophthalmol. 1993; 115:729-737.
  5. 5.0 5.1 5.2 5.3 5.4 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.
  6. Girard, L. Peripheral corneal edema after cataract extraction. Am J Of Ophthalmology. 1985; 100: 353.
  7. Charlin, R. Peripheral corneal edema after cataract extraction:Reply. Am J Of Ophthalmology. 1985; 100: 354.
  8. 8.0 8.1 8.2 Almousa, R., Johns, S., Gibson, R. Atypical clinical presentations of Brown-McLean syndrome. Eye. 2007; 21: 249-250.
  9. Tourkmani, A., Martinez, J., Berrones, D., et al. Brown-McLean Syndrome in a Pediatric Patient. Case Reports in Ophthalmology. 2015; 6:139-142.
  10. 10.0 10.1 10.2 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.
  11. 11.0 11.1 11.2 11.3 11.4 11.5 Sugar, A. Brown McLean syndrome occurring in a corneal graft. Cornea. 1997; 16: 493-494.
  12. 12.0 12.1 12.2 12.3 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.
  13. 13.0 13.1 Mallikarjun, M., Kavitha, v., Rajashekar, J., et al. Brown-McLean Syndrome after phacoemulsification. Indian Journal of Ophthalmology. 2019; 67: 1710-1711.
  14. 14.0 14.1 14.2 Suwan, Y., Teekhasaenee, C., Lekhanont, K., Supakontanasan, W. Brown-McLean syndrome: the role of iridodenesis. Clinical Ophthalmology. 2016; 10: 672-677.
  15. Lim, J., Lam, S., Sugar, J. Brown-McLean syndrome. Arch Ophthalmol. 1991; 109:22-23.
  16. 16.0 16.1 16.2 Hara, T., Hara, T. Brown McLean Syndrome associated with corneal endothelitis in a pseudophakic eye. J Cataract Refract Surg. 1993; 19:780-786.
  17. 17.0 17.1 Jamil, A., Rahman, F., Mirza, K., Igbal, W. Brown-McLean syndrome with keratoconus. J Coll Physicians Surg Pak. 2012; 22: 179-181.
  18. 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.
  19. 19.0 19.1 Martins, E., Alvarenga, L., Sousa, L., et al. Anterior stromal puncture in Brown-McLean Syndrome. J Cataract Refract Surg. 2004;30:1575-1577.
  20. Tanaka, M., Matsuda, M., Manabe R. Brown McLean syndrome- a report of three cases. Folia Ophthalmol Jpn. 1989; 40: 2618-2622.
  21. 21.0 21.1 21.2 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.
  22. 22.0 22.1 22.2 Kam, K., Jhansi, V., Young, A. Brown McLean Syndrome. BMJ Case Report. 2013; dot: 10.1136/bcr-2013-201280.
  23. 23.0 23.1 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.
  24. 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.
  25. Tuft, S., Kerr, M., Sherrard, E., Buckley, R. Peripheral Corneal Edema following cataract extraction (Brown-McLean Syndrome). Eye. 1992; 6: 502-505.
  26. Katsev, D., Kincaid, M., Fouraker, B., et al. Recurrent corneal erosion: pathology of corneal punchure. Cornea. 1991; 10: 418-423.
  27. Moreno-Montanes, J., Heras, J., Rodriguez, R. Varibilidad en la presentación del síndrome de Brown-McLean. Arch Soc Esp Ofthalmol. 2005; 79: 299-302.
  1. Mohebbi, M., Shadravan, M., Pour, E., et al. Brown-McLean syndrome in a patient with Hallermann-Streiff Syndrome. KJO. 2016; 30: 76-77.
  2. Vogel, M., Petrosyan, T., Chin, B., et al. Brown-McLean syndrome. Optometry. 2011; 82: 485-488. </ref>
  3. Sun, B., He, Y., Zhong, P., Wang, R. [Peripheral corneal edema after cataract extraction]. Zhonghua Yan Ke Za Zhi. 1998; 34: 31-33.
  4. Charlin, R., Quintano, R. Edema corneal periferico post facoeresis. Sindrome de Brown McLean. Arch Chilenos Ofthalmol. 1980; 37:17.
  5. 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.