Vitreoretinal Surgery for Retinal Capillary Hemangiomas With Retinal Detachment

Retinal capillary hemangioma (RCH) is a kind of benign vascular neoplasm that occur sporadically or as the earliest manifestation of von Hippel-Lindau disease.^1,2 Pars plana vitrectomy (PPV) is reportedly indicated for treating large RCHs with severe complications, such as exudative retinal detachment, tractional retinal detachment, and vitreous hemorrhage.^3,4 However, such circumstances are often rare and surgery can be challenging. In this study, we shared our findings and experience with PPV for the management of severe RCHs.

MATERIALS AND METHODS

Eleven consecutive patients (12 eyes) with severe RCHs with RD who received PPV were enrolled in this retrospective study between 2013 and 2020. Patients were divided into laser photocoagulation (LC) and retinectomy groups. LC was applied for smaller RCH (<4 mm) without subretinal fluid (Group LC), and antivascular endothelial growth factor therapy was indicated when subretinal fluid occurred. For eyes with larger RCH (>4 mm), persistent RD (>6 mo) or longstanding vitreous hemorrhage, retinectomy was performed (Group R). The details of our study design and procedures are included in the Supplementary Digital Content 1, https://links.lww.com/APJO/A211.

RESULTS

The demographic and preoperative characteristics of 11 patients (12 eyes) were collected (Supplementary Digital Content 2, https://links.lww.com/APJO/A212). The preoperative findings, surgical procedures, and postoperative findings of the patients are shown in Table 1. The detailed results are included in the Supplementary Digital Content 3, https://links.lww.com/APJO/A213.

TABLE 1 - Preoperative Findings, Surgical Methods and Postoperative Findings of Patients With Severe Retinal Capillary Hemangioma Patients

Case	Preoperative Findings	Macular Involvement	Initial Surgery	Retinectomy-Group R or LC	Postoperative Complications	Relapse	Final retinal Status	Final VA logMAR	Follow-up, Months
1	RCH Glaucoma RD	—	23G PPV Laser Gas	LC	Cataract	−	Flat	2.0	66
2	RCH Vitreous hemorrhage	Subretinal fibrosis	23G PPV Localized R Laser SiO	R	RD	−	Flat	2.48	65
3	RCH RD	ERM Subretinal exudation	23G PPV Laser 90°R Laser SiO	R	—	−	Flat	0.4	63
4	RCH RD	ERM	23G PPV Cryo Laser SiO	LC	RD	−	Flat	2.0	48
5	RCH	Subretinal exudation	23G PPV Laser Localized R SiO	R	—	−	Flat	2.48	50
6	RCH RD	—	23G PPV Laser gas	LC	RD	+	Flat	1.0	97
7	RCH RD	ERM	23G PPV Localized R Laser SiO	R	—	+	RD	4.0	76
8	RCH	ERM	23G PPV Localized R Laser SiO	R	—	−	Flat	2.0	89
9	RCH RD	Subretinal exudation	23G PPV Laser Cataract surgery SiO	LC	—	−	Flat	1.0	74
10	RCH RD	—	23G PPV Laser Cryo SiO IVR	LC	Glaucoma	+	Flat with SiO	3.0	61
11	RCH RD	—	23G PPV Laser Cryo SiO IVR	LC	RD	+	Flat	2.48	60.5
12	RCH RD	ERM	23G PPV Laser IVR Gas	LC	—	−	Flat	0.0	8

Cryo indicates cryotherapy; ERD, exudative retinal detachment; ERM, epiretinal membrane; IVR, intravitreal ranibizumab injection; laser, laser photocoagulation; logMAR, logarithm of the minimum angle of resolution; PPV, pars plana vitrectomy; RCH, retinal capillary hemangioma; RD, retinal detachment; SiO, silicone oil; TRD, tractional retinal detachment; VA, visual acuity.

Retinal reattachment and RCH disappearance were considered an anatomical success. In addition, visual acuity (VA) improvement or stability was considered functional success. The anatomical success rate (Supplementary Digital Content 4, https://links.lww.com/APJO/A214, 5, https://links.lww.com/APJO/A215) was 66.7% after the first surgery, and at the final follow-up visit, 11 eyes (91.7%) remained stable, and the functional success was achieved in 10 out of 12 patients (83.3%). The analysis of clinicodemographic factors and final VA showed that the final VA was significantly correlated with preoperative VA (r=0.81, P<0.01).

DISCUSSION

RCH is a type of hemangioma that can occur sporadically or as an ocular hallmark of von Hippel-Lindau disease. A review of vitreoretinal surgery for RCHs in the past 10 years was performed (Supplementary Digital Content 6, https://links.lww.com/APJO/A216; Supplementary Digital Content 7, https://links.lww.com/APJO/A217).^3–6 Our study focused on the advanced stages of this rare disease. Anatomical success was accomplished for 11 eyes (91.7%) through PPV at the final visit. In addition, significant advances have been made in vitreoretinal instrumentation, technology, and adjuvants, such as the application of optical coherence tomography angiography and the development of new dyes. These improve surgical outcomes and reduce complications.^7,8 As a result, vitreoretinal surgeries are recommended, given their promising success rate for RCHs eyes with severe complications.

Endolaser photocoagulation, cryotherapy, or both was applied to treat RCH <3 mm in the previous study.⁴ In our study, RCH <4 mm was managed by laser photocoagulation, cryotherapy, or both. The anatomical and functional success rate (100% and 85.7%) in the LC group suggested that this therapy performed by an experienced surgeon could be considered as the optimal therapeutic approach for RCH <4 mm.

Localized retinectomy (≤90 degrees) was performed for 5 eyes to eradicate the tumor because of the tumor size or complications. Four of the 5 eyes (80%) had a better or stable VA at the last follow-up. More severe RCHs and complications may be associated with worse preoperative VA, requiring retinectomy. Analysis of clinicodemographic factors and final VA demonstrated that the final VA was strongly associated with preoperative VA (P<0.01). Accordingly, poor prognosis is determined the preoperative disease severity rather than the surgical approach. Earlier diagnosis and treatment are necessary for RCH patients. Besides, the Group R eyes were characterized by advanced complications, which made our study different from previous ones and unique.

CONCLUSIONS

In conclusion, this study demonstrated that the vitreoretinal surgical strategy has potential for challenging RCHs cases with severe complications. A better prognosis could be associated with better preoperative VA.