縫合プルスルー法を用いたデスメットストリッピング自動内皮角化術で管理したピアソン骨髄膵症候群児の内皮機能障害

Endothelial dysfunction in a child with Pearson marrow-pancreas syndrome managed with Descemet stripping automated endothelial keratoplasty using a suture pull-through technique.

• Raghav Vadhul
• Caroline S Halbach
• Raymond G Areaux
• Susan Berry
• Joshua H Hou

抄録

重度の進行性羞明症を呈するピアソン骨髄膵臓症候群の既往歴を持つ4歳の少女が、両側のびまん性角膜肥厚と末梢性色素網膜症を呈していることが判明した。彼女は両眼に修正縫合プルスルー法を用いたデスメット・ストリッピング自動内皮角膜形成術（DSAEK）を受けた。術後、白内障形成やグラフト剥離は認められず、角膜は薄くなり、羞明も劇的に改善した。

A 4-year-old girl with a history of Pearson marrow-pancreas syndrome presenting with severe, progressive photophobia was found to have bilateral, diffuse corneal thickening and peripheral pigmentary retinopathy. She underwent Descemet stripping automated endothelial keratoplasty (DSAEK) surgery in both eyes using a modified suture pull-through technique. Postoperatively there was no evidence of cataract formation or graft detachment; her corneas thinned, and her photophobia improved dramatically.

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