Hematol Oncol Clin North Am. 2022 Dec;36(6):1201-1215. doi: 10.1016/j.hoc.2022.08.010.
Microvascular occlusions caused by sickle cell disease (SCD) can affect all ocular and orbital structures. Sickle cell retinopathy (SCR) is the most common ophthalmic manifestation of SCD. Fortunately, most individuals with SCR are visually asymptomatic. Vision loss in SCD most commonly occurs as a consequence of proliferative sickle cell retinopathy (PSR), in which pathologic retinal neovascularization occurs. To prevent significant vision loss and blindness, which can occur from complications of PSR, regular retinopathy surveillance screening examinations and consistent follow-up with a retina specialist are recommended. Scatter laser photocoagulation is the current gold-standard treatment to prevent vision threatening progression of PSR. Patients with sickle cell disease should have regular checkups with their dental care provider. Patients should be educated on the importance of proper dental care, a healthy diet, and the need for early intervention if they suspect any dental problems or are having dental pain. If any dental procedures that involve surgery or sedation are planned, it is critical to consult with the hematologist before the procedure is started. Prophylactic antibiotics may have to be prescribed before invasive dental procedures, such as extractions or periodontal surgery but is best determined by discussions between the dental care provider and the hematologist. Osteonecrosis is a highly prevalent skeletal complication of sickle cell disease that affects all genotypes. Risk factors for osteonecrosis include older age, HbSS genotype with concomitant alpha-thalassemia trait, frequent vaso-occlusive episodes, history of acute chest syndrome, elevated body mass index, and low white blood cell counts. Osteonecrosis causes progressive joint damage and associates with chronic pain, frequent acute care visits, and overall poor health-related quality of life. Current consensus guidelines recommend analgesics, physical therapy, and early consideration of joint arthroplasty in sickle cell-related osteonecrosis, although surgery may be deferred until late adolescence after growth plates have fused.
镰状细胞病 (SCD) 引起的微血管闭塞可影响所有眼部和眶部结构。镰状细胞性视网膜病变 (SCR) 是 SCD 最常见的眼部表现。幸运的是，大多数 SCR 患者没有视力症状。SCD 中的视力丧失最常见于增生性镰状细胞性视网膜病变 (PSR)，其中会发生病理性视网膜新生血管形成。为了防止 PSR 的并发症导致的显著视力丧失和失明，建议定期进行视网膜病变监测筛查检查，并与视网膜专家保持一致的随访。散射激光光凝是预防 PSR 威胁视力进展的当前金标准治疗方法。镰状细胞病患者应定期接受牙科保健提供者的检查。应向患者宣传适当的牙科保健、健康饮食的重要性，如果怀疑有任何牙科问题或有牙痛，需要早期干预。如果计划进行任何涉及手术或镇静的牙科程序，在开始该程序之前，必须咨询血液科医生。在进行侵入性牙科程序（如拔牙或牙周手术）之前，可能需要开预防性抗生素，但最好由牙科保健提供者和血液科医生之间的讨论来确定。骨坏死是镰状细胞病的一种高发骨骼并发症，影响所有基因型。骨坏死的危险因素包括年龄较大、伴有α-地中海贫血特征的 HbSS 基因型、频繁的血管阻塞发作、急性胸部综合征史、身体质量指数升高和白细胞计数低。骨坏死会导致进行性关节损伤，并与慢性疼痛、频繁的急性护理就诊和整体较差的健康相关生活质量相关。目前的共识指南建议在镰状细胞相关骨坏死中使用镇痛药、物理疗法和早期考虑关节置换术，尽管手术可能会延迟到生长板融合后的青春期后期。
