PEMPix is the American Academy of Pediatrics Section on Emergency Medicine’s annual visual diagnosis competition. This year, in addition to the 10 finalists Maneesha Agarwal will be presenting at the National Conference and Exhibition we will be sharing four cases online in advance of the conference. This is the third of the four cases.

This case was submitted by
Dr. Niharika Goparaju
Pediatric Emergency Medicine Fellow at the University of Texas – Austin | Dell Children’s Medical Center

Instagram: @ngoparaj, @atxpemsquad, @dellmedschool, @cat_yee
Co-author: Catherine Yee, MD

The Case

A 12-year-old male with trisomy 21 presents with one week of right eye swelling and three days of clear eye drainage. Initially, the patient was self-inflicting trauma to the eye with his thumb. At the time, parents noted redness of the eye. Since then, patient has had progressive eyelid swelling leading to the inability to open the right eye altogether. Three days prior to presentation, the parents noted clear drainage from the right eye. The day prior to presentation, the parents flushed the right eye with water and applied castor oil to the eyelid.

The patient has had no fever, decreased appetite, emesis, cough, congestion, known foreign body in the eye, or chemical exposure. In addition to trisomy 21, the patient has a history of autism, intermittent alternating esotropia with horizontal nystagmus, constipation, and dysphagia. He’s had a prior strabismus surgery. He is completely unimmunized and on supplemental vitamins.

His exam is notable for features consistent with trisomy 21 and unremarkable other than his eye exam.

Left eye: Blinks to light, EOM intact, intraocular pressures unable to assess, visual fields unable to assess, no proptosis, cornea is clear, lens visualized, unable to assess IOP and visual fields.

Right eye: (exam limited by cooperation) mild upper and lower lid swelling, eye does not open spontaneously, cornea opacified – difficult to assess pupil, +conjunctival injection; negative fluorescein, unable to assess IOP and visual fields

Right Eye

Left Eye

What’s the Diagnosis?

A. Post traumatic cataract
B. Bacterial keratitis
C. Fuchs endothelial dystrophy
D. Open globe
E. Acute corneal hydrops

E. Acute corneal hydrops

The patient was seen by ophthalmology who recommended admission for medical management to prevent further self-inflicted trauma via clear plastic shields and initiate eye drops including atropine, prednisolone, and hypertonic saline. Once parents were comfortable administering drops at home, the patient was discharged a few days later with close out-patient ophthalmology follow-up. Eventually ophthalmic losartan was added with great improvement. It took two months for the patient to completely recover.

The term “hydrops” originates from the ancient Greek word “húdōr,” which means “water,” and is used in medicine to refer to any abnormal accumulation of serous fluid in a tissue or cavity. In the case of acute corneal hydrops, there is a break in the Descment membrane and endothelium, and aqueous humor influxes causing corneal edema. Conditions that weaken the cornea increase the risk of this condition and includes keratoconus (progressive thinning and cone-like steepening of the cornea. Patients with trisomy 21, atopic disease, learning disabilities, corneal anatomical variances, steep keratometry, and elevated intraocular pressure are also at increased risk of acute corneal hydrops. Eye rubbing also increases risk.

Symptoms of acute corneal hydrops includes sudden onset of decreased visual acuity, photophobia, pain, and a translucent milky-white appearance to the cornea. Diagnosis is made based on history and slit lamp findings. Medical management includes hypertonic saline drops to draw fluid from the epithelium, topical antibiotics to prevention secondary infection, antiglaucoma medications to reduce hydrodynamic forces, as well as cycloplegics, NSAIDs, and steroids to help with symptoms. Surgical treatment may also play a role and include intracameral gas injection, compression corneal sutures, and penetrating keratoplasty. While most cases spontaneously resolve in 2- 4 months, complications may include vision-debilitating scarring, infection, pseudocyst formation, and corneal perforation.

References

Mudgil T, Nagpal R, Goel S, Basu S. Acute Corneal Hydrops: Etiology, Risk Factors, and Management. In: Das S, ed. Keratoconus. Singapore: Springer; 2022. doi:10.1007/978-981-19-4262-4_12.

Barsam A, Petrushkin H, Brennan N, et al. Acute corneal hydrops in keratoconus: a national prospective study of incidence and management. Eye (Lond). 2015;29(4):469-474. doi:10.1038/eye.2014.333

Barsam A, Brennan N, Petrushkin H, et al. Case-control study of risk factors for acute corneal hydrops in keratoconus. Br J Ophthalmol. 2017;101(4):499-502. doi:10.1136/bjophthalmol-2015-308251

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Fan Gaskin JC, Patel DV, McGhee CN. Acute corneal hydrops in keratoconus – new perspectives. Am J Ophthalmol. 2014;157(5):921-928. doi:10.1016/j.ajo.2014.01.017

Maharana PK, Sharma N, Vajpayee RB. Acute corneal hydrops in keratoconus. Indian J Ophthalmol. 2013;61(8):461-464. doi:10.4103/0301-4738.116062

García de Oteyza G, Bregliano G, Sassot I, Quintana L, Rius C, García-Albisua AM. Primary surgical options for acute corneal hydrops: A review. Eur J Ophthalmol. 2021;112067212110378. doi:10.1177/11206721211037833

Ocular Trauma: Acute Evaluation, Cataract, Glaucoma. EyeWiki.https://eyewiki.org/Ocular_Trauma:_Acute_Evaluation,_Cataract,_Glaucoma. Accessed June 18, 2024.

Bacterial Keratitis. EyeWiki. https://eyewiki.aao.org/Bacterial_Keratitis#References. Published June 3, 2021. Accessed June 18, 2024.

Fuchs’ Endothelial Dystrophy. EyeWiki.https://eyewiki.aao.org/Fuchs%E2%80%99_Endothelial_Dystrophy#:~:text=Disease-,Fuchs’%20endothelial%20dystrophy%20is%20a%20non%2Dinflammatory%2C%20sporadic%20or,halos%2C%20and%20reduced%20visual%20acuity. Accessed June 18, 2024.

American Academy of Ophthalmology. Acute Anterior Uveitis. EyeWiki.https://eyewiki.aao.org/Acute_Anterior_Uveitis. Accessed June 20, 2024.

Wilson SE. Topical Losartan: Practical Guidance for Clinical Trials in the Prevention and Treatment of Corneal Scarring Fibrosis and Other Eye Diseases and Disorders. J Ocul Pharmacol Ther. 2023;39(3):191-206. doi:10.1089/jop.2022.0174