Sir,
Deep anterior lamellar keratoplasty (DALK) using the Anwar’s big bubble technique has been used successfully to treat the cloudy corneas in Hurler’s and Martoux–Lamy syndromes.1, 2 We herein report a case of Hurler’s syndrome with intradescemetic air entrapment during DALK in a child.
Case report
A 10-year-old boy with Hurler’s syndrome presented with diminished vision and bilateral cloudy corneas. The BSCVA was 6/36 OD and 6/24 OS. AS-OCT of both eyes revealed hyper reflectivity throughout corneal stroma suggestive of stromal deposits. DALK was performed under general anesthesia in his right eye using Anwar’s technique (using a bent sharp 30-gauge needle with bevel facing downwards); however, excessive corneal haze precluded the assessment of single bubble formation during surgery. Donor cornea from which descemet’s membrane had been scraped with merocel after staining with 0.06% trypan blue was sutured to the recipient’s bare descemet’s membrane with 10-0 nylon sutures.
On the first postoperative day, severe corneal edema was present with an immobile air bubble in the center of the cornea (Figure 1a). AS-OCT revealed splitting of the descemet’s membrane and trapped air bubble in the intradescemetic space (Figure 1b). Topical 1% prednisolone acetate, 0.3% moxifloxacin, and 5% hypertonic saline drops four times a day were started. There was gradual resorption of the air bubble by third day with reapposition of the descemet’s layers (Figure 1c) and resolution of stromal edema (Figure 1d). His visual acuity at 3 months improved to 6/9 with -2.5Dcyl@140° and endothelial cell count was 2263/mm2.
Comment
Touboul et al3 recently reported occurrence of intradescemetic air bubble post DALK in an adult patient with corneal opacity. There exists potential space between the striated and unstriated layers of the Descemet’s membrane due to its development in three major processes.4 It is this space that gives way during various phases of air injection during DALK. Deposition of mucopolysaccharides in corneal stroma with poor visibility of air bubble in our case probably facilitated intradescemetic cleavage with air entrapment. Awareness of this complication and performing gentle air–fluid exchange at the end of procedure can avoid persistent double or triple chamber and resultant failure of the procedure.
References
Anwar M, Teichmann KD . Big-bubble technique to bare Descemet’s membrane in anterior lamellar keratoplasty. J Cataract Refract Surg 2002; 28: 398–403.
Mohsen RK, Mohammadali J, Ahmad S, Medi E, Farid K . Deep anterior lamellar keratoplasty for Maroteaux-Lamy syndrome. Cornea 2010; 29 (12): 1459–1461.
Touboul D, Binder PS, Colin J . Intradescemetic air bubble trapping during deep anterior lamellar keratoplasty. Cornea 2012; 31: 191–193.
Murphy C, Alvarado J, Juster R . Prenatal and postnatal growth of the human Descemet’s membrane. Invest Ophthalmol Vis Sci 1984; 25: 1402–1415.
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Arora, R., Gupta, D., Jain, P. et al. Intradescemetic air trap post deep anterior lamellar keratoplasty in a child with mucopolysaccharidosis. Eye 28, 495–496 (2014). https://doi.org/10.1038/eye.2014.5
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DOI: https://doi.org/10.1038/eye.2014.5