Bilateral Congenital Iris Sphincter Agenesis Diagnosed After Massive Bleeding Episode During Repair of Aneurysmal Dilation of Patent Ductus Arteriosus: A Case Report.

Tomohiro Yamamoto, M. Schmidt-Niemann, E. Schindler
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Abstract

We report a rare case of an infant with both an aneurysmal dilation of the patent ductus arteriosus (PDA) and bilateral congenital iris sphincter agenesis. Her mydriasis without pupillary light reflex was first noted after a massive intraoperative bleeding episode during the PDA ligation. The assumption that the mydriasis was a sign of cerebral ischemia led to additional examinations and intensive medical therapies that in retrospect were unnecessary. This is the first reported case of combined aneurysmal dilation of a PDA and congenital iris sphincter agenesis in the anesthesia literature.
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动脉导管未闭动脉瘤性扩张术中大出血后诊断双侧先天性虹膜括约肌发育不全一例。
我们报告一例罕见的婴儿动脉瘤性扩张的动脉导管未闭(PDA)和双侧先天性虹膜括约肌发育不全。她在PDA结扎术中大量出血后首次发现无瞳孔光反射的瞳孔散瞳。臆断瞳孔是脑缺血的征兆,导致了额外的检查和密集的药物治疗,回想起来是不必要的。这是麻醉文献中首次报道的PDA合并动脉瘤扩张和先天性虹膜括约肌发育不全的病例。
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