Levinsen Mette, Kiilgaard Jens Folke, Thomsen Carsten, Heegaard Steffen, Nissen Kamilla Rothe
Department of Ophthalmology, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark.
Department of Radiology, Zealand University Hospital, Roskilde, Denmark.
Am J Ophthalmol Case Rep. 2021 Apr 16;22:101092. doi: 10.1016/j.ajoc.2021.101092. eCollection 2021 Jun.
Rhino-orbital-cerebral mucormycosis (ROCM) is a rare opportunistic infection with a high mortality despite relevant treatment.
A 3-year-old girl under treatment for acute lymphoblastic leukemia developed periorbital swelling, ophthalmoplegia and a necrotic palatal lesion during a period of neutropenia. Imaging revealed sinusitis, pre- and postseptal cellulitis. The disease later progressed to cerebral involvement and orbital apex syndrome with complete ophthalmoplegia, ptosis and loss of vision. The patient was treated with systemic antifungal therapy, hyperbaric oxygen and extensive surgery. This included orbital exenteration, skull base resection, cerebral debridement with placement of an Ommaya reservoir for intrathecal administrations of amphotericin B (AmB) and in addition endoscopic sinus surgery with local AmB installation. Chemotherapy was safely continued after resolution of the ROCM and the patient remains in complete remission after 5 years.
Patients with ROCM can be cured with aggressive multimodality treatment, including surgical intervention, even if in myelosuppression.
鼻眶脑型毛霉菌病(ROCM)是一种罕见的机会性感染,尽管进行了相关治疗,但其死亡率仍很高。
一名正在接受急性淋巴细胞白血病治疗的3岁女孩在中性粒细胞减少期出现眶周肿胀、眼肌麻痹和腭部坏死性病变。影像学检查显示鼻窦炎、眶隔前和眶隔后蜂窝织炎。疾病随后进展为脑部受累和眶尖综合征,伴有完全性眼肌麻痹、上睑下垂和视力丧失。患者接受了全身抗真菌治疗、高压氧治疗和广泛的手术治疗。这包括眶内容剜除术、颅底切除术、脑部清创术并放置Ommaya储液器以便鞘内注射两性霉素B(AmB),此外还进行了内镜鼻窦手术并局部应用AmB。ROCM缓解后安全地继续进行化疗,患者在5年后仍处于完全缓解状态。
即使处于骨髓抑制状态,ROCM患者通过包括手术干预在内的积极多模式治疗也可治愈。