Cataract surgery and a challenging postoperative course in a child with Blau syndrome: a case report

Blau syndrome is a rare NOD2-associated autoinflammatory disorder characterized by granulomatous arthritis, dermatitis, and uveitis. Ocular involvement may be severe in pediatric patients. Detailed reports on cataract surgery and postoperative management in children with Blau syndrome remain limited.

A 7-year-4-month-old Chinese girl with genetically confirmed Blau syndrome presented with decreased vision in both eyes. Her articular manifestations were well controlled under systemic treatment, but she developed severe bilateral ocular involvement, including chronic uveitic changes, complicated cataracts, and vitreous opacities. Sequential cataract surgery with in-the-bag intraocular lens implantation, posterior capsulotomy, and anterior vitrectomy was performed in both eyes. Although visual acuity initially improved, she later developed severe postoperative inflammation, fibrocellular proliferation on the intraocular lens surface, pupillary block, iris bombe, and secondary glaucoma in the right eye. Treatment required intensified anti-inflammatory and immunomodulatory therapy and peripheral iridectomy. Short-term follow-up showed improvement in intraocular pressure and visual acuity.

In Blau syndrome, ocular inflammation may progress despite good control of articular manifestations. Lifelong ophthalmic surveillance is essential, even when systemic disease appears well controlled. In children requiring cataract surgery, careful timing, optimized perioperative systemic and topical anti-inflammatory treatment, and close postoperative monitoring are important to reduce postoperative inflammatory complications.