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Painless Presentation of Acute Hydrops in Keratoconus with Serial In-Vivo Imaging

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Painless Presentation of Acute Hydrops in Keratoconus with Serial In-Vivo Imaging Painless Presentation of Acute Hydrops in Keratoconus with Serial In-vivo Imaging Sara Berke-Silva, O.D. and Kimberly Reed, O.D. Nova Southeastern University Abstract Acute corneal hydrops is typically associated with acute pain, photophobia, and tearing at onset, but our patient denied these symptoms. The poster will present this case and review current diagnostic and therapeutic strategies, including controversies. I. Case History a. 21 year old Hispanic female b. The patient woke up 3 hours prior to examination with an acute onset of a ‘white spot’ and reduced vision OD. Denies pain, photophobia, tearing. c. After further questioning, the patient reported foreign body sensation OU that was unchanged from the normal amount she experiences without her contact lenses. d. Diagnosed with Keratoconus at age 15. Wore corneal RGPs until two years ago when semi-scleral lenses were employed with excellent visual outcomes. II. Pertinent Findings a. The patient was not photophobic and denied pain throughout the exam. Increased lacrimation was evident, unbeknownst to the patient. b. Slit lamp examination revealed significant corneal edema centrally with an absence of an anterior chamber reaction. Epithelial bullae were present. c. OCT confirmed posterior corneal compromise and significant corneal edema throughout all layers, with several bullae and microcysts in the epithelium. III. Differential Diagnosis a. Hydrops b. Corneal abrasion caused by contact lens wear c. Corneal edema caused by contact lens overwear d. Corneal infection causing ulceration e. Acute, severe corneal edema due to other cause of endothelial failure IV. Diagnosis and Discussion a. Acute corneal hydrops in the context of keratoconus has typically been associated with pain, photophobia, and tearing at onset (1,2,3), but our patient denied having these symptoms. She did experience the common complaint of severely decreased vision as well as a “white spot” on the eye. b. Judging from the appearance of the cornea, one is inclined to believe that acute corneal hydrops should be a painful condition. However, changes in the keratoconic cornea can help us understand how this condition can present without ocular discomfort. i. It has been shown (4) that nerve morphology found in keratoconic corneas is altered compared to normal corneas, and this translates to diminished corneal sensitivity. ii. Additionally, keratoconics wearing RGPs have even lower corneal sensitivity than keratoconics that do not wear these contact lenses (4). Our patient had worn RGPs for about three years which could have further decreased her corneal sensitivity. c. There are no good studies to date examining the symptomatology in acute corneal hydrops due to keratoconus. The absence of pain in this condition has been reported in the past (5,6,7), so it may not be as uncommon as conventionally thought. Further studies on the symptomatology of acute corneal hydrops may unmask the typical association (or cognitive bias) of pain with this condition. V. Treatment/Management a. Topical management was employed for our patient: Vigamox, Pred Forte and Muro 128 ung QID at initial visit. Cyclopentolate 1% was also instilled in-office as a precautionary measure in case the patient developed pain later as the condition progressed. Pain was not reported at any follow-up visit. b. Serial ocular coherence tomography (OCT) was used to monitor the condition. One study (8) performed OCT on hydrops patients and found three characteristic presentations: Decemet’s membrane (DM) detachment with no break, detachment with break and flat ends, and detachment with a break and rolled ends. i. Our patient showed an initial detachment with a break and rolled ends. ii. Our subsequent OCT images show increased stromal edema and epithelial bullae up until the 1 month follow-up where a significant decrease in stromal edema and bullae were observed. This correlated with the patient’s slightly improved vision at this time. iii. The same study also outlined two phases in the resolution of this condition. The detached DM must re-attach to the corneal stroma, and this is dependent on the depth of the initial detachment. Then, the endothelium must fill-in the gap created by the break, and this is dependent on the initial size of the break. 1. In our patient, re-attachment of the DM partially occurred slightly over two weeks after initial presentation. c. Intracamaral air bubble for acute corneal hydrops has been studied to speed up the resolution of the vision-impairing edema (1), but it does have its pitfalls including the necessity for a supine position for up to two weeks, cataract and pupil block glaucoma. The final best-corrected visual acuity was no different between this form of intervention and topical treatment in one recent study. d. Corneal collagen crosslinking has been shown to be an effective and safe way to halt the progression of keratoconus (9). This treatment uses topical riboflavin drops in conjunction with ultraviolet (UV) A radiation to increase the rigidity of the cornea, therefore halting the detrimental thinning and steepening of the corneal architecture. This promising treatment will hopefully make hydrops a dinosaur in the world of keratoconus-related complications, especially since it has been shown to be effective in the pediatric population. VI. Conclusion a. Corneal hydrops is an acute condition that is typically associated with pain, photophobia and tearing. A decrease in vision without these symptoms should not prompt the clinician to dismiss the possibility of corneal hydrops in a patient with keratoconus. Serial OCT is a helpful tool to monitor progression of this condition. References 1. Gaskin J, Patel D, McGhee C. Acute Corneal Hydrops in Keratoconus—New Perspectives. Am J Ophthalmol 2014;157:921–928. 2. Maharana PK, Sharma N, Vajpayee RB. Acute corneal hydrops in keratoconus. Indian J Ophthalmol 2013;61:461-4. 3. Grewal S, Laibson PR, Cohen EJ, et al. Acute hydrops in the corneal ectasias: associated factors and outcomes. Tr Am Ophthalmol Soc 1999; 97:187-203. 4. Leopoldo Spadea, Serena Salvatore, and Enzo Maria Vingolo, “Corneal Sensitivity in Keratoconus: A Review of the Literature,” The Scientific World Journal, vol. 2013, Article ID 683090, 7 pages, 2013. 5. Bilgin B, Unal B, Unal M, Doğan E, Cetinkaya A, Akyol M, Yücel I, Akar Y, Apaydın C, Ilhan D. Keratoconus presenting with bilateral simultaneous acute corneal hydrops. Contact Lens Anterior Eye 2013;36(2):98-100. 6. Boyd S, Gutierrez AM, McCulley JP. 2011. Atlas and Text of Corneal Pathology and Surgery. Jaypee Brothers Publishers. 7. Trattler W, Kaiser P, Friedman N. 2012. Review of Ophthalmology. Elsevier Health Sciences. 8. Basu S, Vaddavalli PK, Vemuganti GK, Ali MH, Murthy SI. Anterior segment optical coherence tomography features of acute corneal hydrops. Cornea 2012;31(5):479–485. 9. Sorkin N, Varssano D. Corneal Collagen Crosslinking: A Systematic Review. Ophthalmologica 2014;232:10–27. .
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