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 Table of Contents  
ORIGINAL ARTICLE
Year : 2019  |  Volume : 57  |  Issue : 2  |  Page : 128-131

Outcome of early and late vitrectomy for retinal detachment associated with von Hippel–Lindau-related retinal capillary hemangioma


Department of Vitreo-Retinal Services, Aravind Eye Hospital and Postgraduate Institute of Ophthalmology, Madurai, Tamil Nadu, India

Date of Web Publication10-Sep-2019

Correspondence Address:
Dr. Karthik Kumar
Department of Vitreo-Retinal Services, Aravind Eye Hospital and Postgraduate Institute of Ophthalmology, Madurai, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/tjosr.tjosr_24_19

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  Abstract 


Aim: The aim of this study was to report the outcome of early and late vitrectomy for retinal detachment-associated with von Hippel–Lindau (VHL)-related retinal capillary hemangioma (RCH). Materials and Methods: Records of patients diagnosed with VHL-related RCH, from 2016 to 2018, were retrospectively analyzed for patients' age, gender, family history, systemic disorders, previous treatment taken, clinical characteristics of RCH, associated complications, timing of surgery, and surgical outcome. The treatment of RCH depends on its location, size, and clinical expressions. Results: Twenty-one eyes of 15 patients with a median age of 40 years (range, 22–65 years) were seen. Nine patients had central nervous system hemangioblastomas, eight had associated renal complications (four – renal cell carcinoma and four – multiple renal cysts), four had pancreatic cysts, while one had testicular cyst and pheochromocytoma each. Four eyes presented with no perception of light. Eight eyes presented with retinal detachment (RD). Four eyes underwent a combination treatment, four underwent cryotherapy, five underwent laser, and two underwent transpupillary thermotherapy, while four underwent intravitreal bevacizumab. Of eight eyes with tractional RD (TRD), four presented with no perception of light (NPL) . The other four eyes underwent surgery. Of the eyes that underwent surgery, two underwent early vitrectomy for extramacular TRD, whereas the other two underwent surgery in late stage. The two eyes that underwent early vitrectomy gained best-corrected visual acuity (BCVA) of 20/20, and the retina was attached in single surgery. The other two eyes that underwent surgery at an advanced stage had to undergo multiple surgeries and gained BCVA <10/200. Conclusion: Early vitrectomy is a safe and effective modality of treatment for VHL-related RCH complicated with TRD.

Keywords: Tractional retinal Detachment, vitrectomy, von hippel lindau


How to cite this article:
Kumar K, Kohli P, Babu N, Jena S, Ramasamy K. Outcome of early and late vitrectomy for retinal detachment associated with von Hippel–Lindau-related retinal capillary hemangioma. TNOA J Ophthalmic Sci Res 2019;57:128-31

How to cite this URL:
Kumar K, Kohli P, Babu N, Jena S, Ramasamy K. Outcome of early and late vitrectomy for retinal detachment associated with von Hippel–Lindau-related retinal capillary hemangioma. TNOA J Ophthalmic Sci Res [serial online] 2019 [cited 2019 Nov 18];57:128-31. Available from: http://www.tnoajosr.com/text.asp?2019/57/2/128/266383




  Introduction Top


Retinal capillary hemangiomas (RCHs) are orange–red circumscribed, round vascular tumors supplied by a pair of prominent vessels. They are mostly located in the peripheral retina and rarely in the juxtapapillary retina. They can occur either as isolated vascular abnormalities or as a part of von Hippel–Lindau (VHL) syndrome. Isolated RCH is usually stable and not associated with vision-threatening complications and hence can be safely observed. However, RCH associated with VHL is usually progressive and complicated by either exudative retinal detachment (ExRD) due to tumor exudation or tractional RD (TRD) due to glial proliferation around the tumor. Observational studies have shown that 20%–25% of patients were blind in at least one eye at the time of presentation. Hence, VHL-related RCH warrants treatment.[1],[2],[3],[4],[5],[6]

VHL is a rare syndrome with an incidence of 1 in 36,000–40,000 population. It is an autosomal dominant syndrome arising due to germline mutation in the VHL gene. It is a multisystem disorder with a predilection for the central nervous system (CNS) and retina.

The manifestations include CNS hemangioblastomas, renal cell carcinoma (RCC), pheochromocytoma, and pancreatic neuroendocrine tumors. RCH is seen in 49%–62% of VHL patients and may even be the first manifestation of the disease.[1],[2],[3],[4],[5],[6]

The treatment of RCH depends on its location, size, and clinical expressions. Laser photocoagulation and cryotherapy remain the treatment of choice for small, extrapapillary RCH. However, larger lesions generally do not respond well to laser or cryotherapy. Several treatments have been tried for the treatment of more threatening lesions. These include photodynamic therapy, intravitreal antivascular endothelial growth factor, radiotherapy, and vitreoretinal surgery.[1],[2],[3],[4],[5],[6],[7],[8],[9],[10],[11],[12],[13],[14],[15],[16],[17],[18],[19],[20],[21],[22]

This study was conducted to analyze the surgical outcomes of vitreoretinal surgery in eyes with TRD caused due to VHL-associated RCH.


  Materials and Methods Top


This was a retrospective study conducted at Aravind Eye Hospital, Madurai, India. The study conformed to the tenets of the Declaration of Helsinki. The records of patients diagnosed with VHL between 2016 and 2018 were searched with the help of search words “von Hippel–Lindau” and “retinal hemangioblastoma” in the hospital's electronic medical record. The records were analyzed for patients' age, gender, family history, systemic disorders, previous treatment taken, clinical characteristics of RCH, associated complications, timing of surgery, and surgical outcome.

The diagnosis of VHL was made on the basis of a combination of careful questioning of the past medical history, family history, physical examination, radiologic screening, and fundus examination. Criteria required for the diagnosis of VHL were as follows: (1) a positive family history and a CNS hemangioblastoma (including RCH), pheochromocytoma, or clear cell renal carcinoma or (2) ≥2 CNS hemangioblastomas or 1 CNS hemangioblastoma along with a visceral tumor (with the exception of epididymal and renal cysts) in the absence of family history. Patients with retinal hemangiomas not satisfying the clinical criteria were excluded from the study.

The patients underwent treatment as per the presenting conditions. The peripheral RCH was treated with triple-freeze cryotherapy, the more posterior ones were treated with laser, while the peripapillary RCH was treated with transpupillary thermotherapy (TTT). The associated macular edema was treated with intravitreal bevacizumab (IVB) injection. The patients presenting or progressing to TRD underwent surgery. Some eyes underwent a combination of treatment.

Surgical Techniques

All eyes underwent 23-gauge pars plana vitrectomy. Core vitrectomy was followed by posterior hyaloid detachment. Triamcinolone was used to stain the vitreous in order to ensure complete vitreous removal. Perfluorocarbon liquid was injected over the posterior pole to stabilize the retina, and preretinal membranes were meticulously dissected. Bimanual technique was used whenever necessary. The blood vessels supplying the RCH were localized and endodiathermized to decrease the vascularity of the tumor. The lesion was treated with endolaser therapy. Careful vitreous base shaving was done, and inner limiting membrane was peeled for around 2 disc diameter around the fovea. Fluid–air exchange was done, and peripheral 360° endolaser photocoagulation was applied. Finally, 1000 centistoke silicone oil was injected for tamponade, and the sclerotomies were closed with 6.0 vicryl suture. None of the patients underwent scleral buckling procedure.

All the patients were advised prone position for the first 2 weeks. All patients were routinely examined on postoperative day 1, 2 weeks, and then, monthly until the disease stabilized. Visual acuity measurements and intraocular pressure were measured at every visit.


  Results Top


A total of 15 patients with VHL were seen, of which 7 were male and 8 were female. The median age of the presentation was 40 years (range, 22–65 years). Unilateral RCH was present in nine patients, whereas six patients had bilateral presentation. Hence, a total of 21 eyes were affected.

Of 15 patients, nine (60.0%) patients had CNS hemangioblastomas and one had undergone craniectomy. Eight (53.3%) patients had associated renal complications. While four patients had RCC, four had multiple renal cysts. One of the patients with RCC had tumor metastasis and subsequently died. Four (26.7%) patients had pancreatic cysts, whereas one (6.7%) patient had testicular cyst and pheochromocytoma each. The patient with pheochromocytoma also died due to CNS bleed owing to high blood pressure.

Of 21 eyes, 4 eyes (19.0%) presented with no perception of light. Eight (38.1%) eyes presented with RD. One patient with unilateral RCH had already undergone RD surgery elsewhere and presented with phthisis bulbi. The patients underwent a combination of treatment as per the presenting conditions. Four eyes (19.0%) underwent a combination treatment. Four (19.0%) eyes underwent cryotherapy [Figure 1], five eyes (23.8%) underwent laser, two (9.6%) eyes underwent TTT, while four (19.0%) eyes underwent IVB. Eight (38.1%) eyes presented with TRD, of which four presented with NLP (including the one which had undergone surgery elsewhere) and the other four eyes underwent surgery.
Figure 1: Ultrawide image of a patient with exudation secondary to peripheral retinal capillary hemangioma managed with cryotherapy and intravitreal bevacizumab

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Of the four eyes that underwent surgery, two underwent early vitrectomy for extramacular TRD [Figure 2]a, whereas the other two underwent surgery in late stage. The two eyes that underwent early vitrectomy gained best-corrected visual acuity (BCVA) of 20/20, and the retina was attached in single surgery [Figure 2]b. The other two eyes that underwent surgery at an advanced stage had to undergo multiple surgeries and gained BCVA <10/200.
Figure 2: (a) Fundus image of a patient with multiple extramacular tractional retinal detachment, and (b) ultrawide image after surgery showing reattached retina and scared retinal capillary hemangioma

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Intractable bleeding was not noted in any of the cases. There was no recurrence of RCH in any of the cases. None of the cases progressed to neovascular glaucoma. None of the eyes needed any form of retreatment for RCH.


  Discussion Top


RCH associated with VHL are usually progressive and are complicated by exudation and RD. The disease can be severe enough to cause loss of total vision, phthisis bulbi, and neovascular glaucoma leading to painful blind eye, which needs to be enucleated.[1],[2],[3],[4],[5],[6] Vitrectomy has been propounded as the treatment of choice for such cases.[7],[8],[9],[10],[11],[12],[13],[14],[15],[16],[17],[18],[19],[20],[21],[22] However, the timing of surgery remains controversial. Most of the surgeons advocate vitrectomy as the last resort for advanced cases.[8],[9],[10],[11],[12],[13],[14],[15],[16],[17],[18],[19],[20],[21],[22] However, in 2017, van Overdam et al. proposed that early vitrectomy can be an effective approach for management of patients with large RCH.[7]

Toy et al. in their longitudinal study of 249 patients with VHL showed that those developing only peripheral involvement maintained relatively stable mean visual acuity. They proposed that peripheral RCHs were nonexudative in nature and were easily amenable to ablative treatment.[3] We also had a similar finding as 13 eyes have peripheral RCH, which were treated successfully, and eyes maintained good vision.

Peyman et al. first documented a case of RCH excision in 1983.[19] McDonald et al. first showed that vitrectomy for epiretinal membranes and TRD associated with RCH can lead to good visual gain.[18] Farah et al. reported a decrease in size of RCH after ligation of transretinal feeder vessel as treatment. However, new feeder vessels later appeared.[20]

Gaudric et al. reported the long-term results of 23 eyes which underwent surgery for severe cases of RCH. They performed vitrectomy with or without retinectomy and RCH resection. They concluded that surgery is a useful tool in salvaging vision in eyes that develop exudative and TRD due to large multiple RCHs. In their subanalysis, they observed that the eyes that underwent radical procedures such as retinectomy and RCH resection had a higher incidence of proliferative vitreoretinopathy (PVR) and neovascular glaucoma.[8] Similarly, Krzystolik et al. also reported and evaluated the long-term results of 24 eyes which underwent surgery for severe cases of RCH. They concluded that surgery in advanced VHL eye disease can improve or preserve visual function. However, ocular VHL disease seemed to have an accelerated postoperative course in cases where retinotomy was performed.[12] Avci et al. reported promising anatomical and visual outcomes after resection of RCH in VHL eyes complicated with RD in 12 eyes.[13] Karacorlu et al. reported vitrectomy, endodiathermy of vessels, and endoresection of RCH as an effective treatment option for complex RCH. They concluded in their retrospective study of 12 eyes that high anatomical success and visual outcome were achieved with minimal need for retreatment.[14] Schlesinger et al. reported favorable outcomes after tying off the feeder artery, followed by total excision of RCH in three patients.[21] Liang et al. reported successful results in four cases after ablation of vessels and excision of RCHs.[22] van Overdam et al. performed vitrectomy, vessel diathermy, and RCH excision on four patients with large RCH (i.e., >5 mm) without any complication. In their small series, they found that early treatment was effective and resulted in lesser complications.[7] Few case reports highlight the successful treatment of such cases after combining vitrectomy with photodynamic therapy (PDT) or IVB.[11],[16],[17]

In our small series, we found that an early vitrectomy in case of RCH can lead to a better visual and anatomical outcome. The eye surgery was undertaken early in the course, and RD could be reattached easily, resulting in a better visual outcome. On the contrary, the eyes where surgery was done late in course underwent a more complicated surgery, required more than one surgery for the retina to get reattached, and resulted in worse outcome. As the draining vessels were cauterized carefully, uncontrollable bleeding was not noted in any case with no case of recurrence of RCH.

We in our short series observed that early vitrectomy is a safe and effective modality of treatment for VHL-related RCH complicated with TRD. The earlier the surgery is done, the better the outcome. The limitations of the study were the lack of genetic testing, small sample size, and short duration of follow-up.

There is no standard surgical treatment for RCH due to VHL. Various combinations such as retinectomy, RCH endoresection, and IVB have been proposed with varying results. The utilization of cerclage, like in any other disease with exudation, is controversial. Long-term randomized control trials are required to investigate the optimal treatment modality for complex RCH. However, rarity of the disease makes such a trial difficult to perform.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

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