Background: Keratoconus is a progressive, non-inflammatory corneal ectasia that often escapes diagnosis until complications arise. Acute corneal hydrops, a rare vision-threatening complication, results from a rupture in Descemet’s membrane, leading to sudden stromal edema. Long-term outcomes can depend on effective patient education, especially in individuals experiencing communication challenges or developmental delays. Early identification, appropriate management, and patient education are essential in reducing morbidity and preventing recurrence or progression.

Case Presentation: A previously healthy young adult with developmental delay presented with a 24-hour history of severe left eye pain, corneal cloudiness, photophobia, and sudden vision loss. Visual acuity tested at count-fingers in the left eye (OS) and 20/40 in the right (OD); intraocular pressures were normal bilaterally. Slit lamp examination revealed marked corneal edema, bullae, and central thinning with a deep anterior chamber OS. The right eye showed subtle inferocentral thinning without edema. Optical Coherence Tomography demonstrated extreme corneal swelling (central corneal thickness 2295 μm) and irregular keratometric rings OS, with early inferior steepening OD, which confirmed bilateral keratoconus with acute corneal hydrops OS. The patient was initiated on sodium chloride drops and ointment in both eyes, Cyclopentolate daily OS for pain, and artificial tears. At follow-up, the patient’s mother revealed a long-standing history of chronic eye rubbing due to allergic itching, which the patient had previously been unable to communicate. Olopatadine drops was prescribed for allergy control and strict eye-rubbing precautions were discussed. At one-week follow-up, pain and edema began to resolve and vision improved to 20/50 OS. The patient was informed that a preemptive corneal cross-linking procedure may benefit progression of currently mild keratoconus OD.

Discussion: This case illustrates the importance of recognizing acute hydrops as an initial manifestation of keratoconus, thorough history-taking, and patient advocacy. In individuals with communication challenges, risk factors for keratoconus such as chronic eye rubbing may be overlooked. In this case, caregiver insight was essential in identifying allergies and eye rubbing as the root cause of the patient’s presentation, and allowed for targeted intervention. By initiating allergy control and behavioral counseling, progression of keratoconus was prevented in both eyes. This case highlights the value of early patient education and caregiver engagement, especially in vulnerable populations, to modify the trajectory of keratoconus and better preserve vision.