605 e-Letters

  • Confounders to be addressed

    I read with great interest the article by Bae and collegues.1 In their retrospective study, the authors concluded that the presence of atypical epiretinal tissue (AET) in a full-thickness macular hole (FTMH) was related to poorer anatomical success and less visual recovery after surgery.
    I agree with the authors on the association of their OCT findings with the visual prognosis. I also agree with them that it is important to identify a good indicator of visual prognosis based on OCT findings. However, there are many confounders to be addressed in this study. For example, preoperative MH size with OCT has been known as a prognostic factor for postoperative visual outcome and anatomical success rate of MH surgery.2,3 A previous study also demonstrated that ERM prevalence increased with severity and size of the FTMH.4 In addition, preoperative visual acuity or preoperative photoreceptor integrity also seems to correlate with visual prognosis.
    Thus, their results should be supported by appropriate statistical analysis, that is, multivariate regression analyses. I hope that the authors will comment on the results of multivariate regression analyses to identify the most significant factor to predict visual prognosis after MH surgery.

    1. Bae K, Lee SM, Kang SW, et al. Atypical epiretinal tissue in full-thickness macular holes: pathogenic and prognostic significance. Br J Ophthalmol. 2018 (in press)
    2. Ullrich S, Haritoglou C, Gass...

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  • Re: Optical coherence tomography angiography identifies peripapillary microvascular dilation and focal non-perfusion in giant cell arteritis

    Dear Editor,
    We read with interest the paper by Gaier et al.1 The collection of 5 eyes affected by acute A-AION and analyzed with OCT-A is remarkable, due to the rare disease presentation. The main finding of the paper was that during the acute phase of A-AION, diffusely dilated superficial peripapillary capillaries were detectable on OCT-A. Interestingly, peripapillary capillary dilatation was also noted in the fellow eye. Unfortunately, the figures presented by the authors are too small to allow the reader to qualitatively appreciate the capillaries dilatation. More detailed images and a quantitative vessels analysis would have helped to document the microvascular changes.
    They hypothesized that the capillary dilatation may represent a form of luxury perfusion in the setting of short ciliary arterial compromise or a centrally mediated autoregolatory mechanisms in the setting of reduced perfusion of the optic nerve. These hypotheses are interesting, but it is important to differentiate the RNFL thickness increase from the capillary dilatation, as peripapillary capillary plexus density and RNFL thickness are highly correlated and fit well with a nonlinear stacked-layer model.2
    Moreover, the authors stated that OCT-A laminar analysis did not highlight the choroidal/choriocapillaris perfusion defects seen on FA. However, a recent study3 showed a tight correspondence between the choroidal perfusion defects visible on FA (and even better on indocyanine green...

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  • Letter to the Editor - XFS

    Dear Sir,

    I was most interested to read the review by Nazarali and co-authors to mark the centenary of the description of the exfoliation syndrome, XFS(1) sometimes called the pseudo-exfoliation syndrome (2). It is always interesting to see how our understanding increases incrementally with time and reviews such as these are important in helping shape further investigations.

    The linkage of environmental factors and XFS is important and as they say not well understood. Nazarali and co-authors might like to reflect on the findings in Australian Aboriginal people (3). Aboriginal people were found to have very high rates of XFS, being present in 16% of those aged 60 and above. The presence of XFS was related to total global radiation exposure and occupation. Most interestingly, XFS was not associated with high intraocular pressure or glaucoma. Surely this is an area where more research is required.

    1. Nazarali S, Damji F, Damji KF. What have we learned about exfoliation syndrome since its discovery by John Lindberg 100 years ago? Br J Ophthalmol 2018; doi:10.1136/bjophthalmol-2017-3111321
    2.Dvorak-Theobald G. Pseudo-exfoliation of the lens capsule - relation to “true” exfoliation of the lens capsule as reported in the literature and role in the production of glaucoma capsulocuticulare. Am J Ophthalmol 2018; doi.org/10.1016/j.ajo2108.02.018
    3. Taylor HR. Pseudoexfoliation, an environmental disease? Trans Ophthalmol Socs UK 1979; 99: 302-307...

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  • We must continue testing delivery models to create more efficient surgical care

    We were interested to see Roberts, et. al study [1] which explored whether a hub-and-spoke model using a femtosecond laser (FL) could increase the efficiency and reduce the cost of cataract surgery.

    Although the model was not cost-effective when compared to conventional phacoemulsification surgery, more efficient models should continue to be assessed. The Aravind Eye Care system uses an alternative hub-and-spoke model. Instead of separate operating theatres (OTs), the physician alternates between two beds in a single OT. This model, and the safe reuse of surgical supplies, results in phacoemulsification cataract surgery with excellent outcomes at 1/20th the cost and carbon emissions [2-4].

    Roberts, et. al recommend that the ideal number of OTs to maximise the utility of an FL in a hub-and-spoke model is four. However, they were not able to evaluate the effect of adding additional OTs to their model as they only had two OTs. We suggest that adopting the Aravind model to jump to the 1:4 model without further building work could significantly alter this paper’s conclusions. We would be interested to know if elements of the Aravind model, two beds one theatre, could be adopted in their setting.

    On average patients receiving FLACS spent 5.85±1.99 mins in the laser suite (LS), implying a potential throughput of between 8 and 15 cases per hour. We are interested to know the authors views on the the limits of the FL and what impact the adoption of bilateral...

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  • AML and metastatic risk after ophthalmic artery chemosurgery for retinoblastoma

    We would like to add some data to this interesting discussion about the impact of ophthalmic artery chemosurgery or intra-arterial chemotherapy (OAC) in the occurrence of secondary malignancies in children with retinoblastoma with germline mutations of the Rb1 gene.
    The discussion about the incidence of secondary leukemia is an important one. Secondary leukemias usually present early in the patient follow-up, usually around 2-3 years. Systemic chemotherapy has been implicated in their occurrence since, they were not identified as a common secondary malignancy in patients with retinoblastoma not receiving chemotherapy1. Their occurrence is low1, but as the case Dr Meadows and Lahey report are usually refractory to treatment and usually fatal. In our series of patients treated with systemic chemoreduction for conservative therapy from Argentina, secondary AML was the most common fatal secondary malignancy with a total of 2 out of 129 cases. We had a third case in a child receiving chemotherapy for extraocular disease, similarly to Dr Meadows and Lahey’s case2. In our current, yet unpublished results with ophthalmic artery chemosurgery, we had no case of secondary AML , in 71 consecutive cases with bilateral retinoblastoma treated with OAC with a median follow-up of 42 months. Secondary AML has been widely reported as a complication of systemic chemotherapy in pediatric oncology patients 3 including those receiving epipodophillotoxins 4and also alkylating agents5. Cum...

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  • Intravitreal methotrexate for vitreoretinal lymphoma has a very low local recurrence rate

    Dear Editor,
    We have read with interest the paper by Klimova et al. Some statements in the paper are confusing and may even mislead the readers.

    The authors claim in the survival section of the paper that: "Vitreoretinal lymphoma is a life-threatening disease, with a 5-year survival rate of 71% in our study". Vitreoretinal lymphoma (VRL) may affect vision, and in very advanced cases that we rarely see in recent years, may destroy the eye. However, VRL per se is not what that kills the patients, but the associated brain lymphoma or in some case the systemic lymphoma.

    According to the results in this study (and the title of the paper), "Combined (local and systemic) treatment in patients with PVRL showed favorable results in comparison with local therapy alone (p=0.695). However, the statistical significance was not reached". It is no wonder that they claim that combined treatment is better than local treatment when they have 60% relapses. However, no other study of intra-vitreal (IVit) Methotrexate showed such a high relapse rate. In our experience, the relapse rate is extremely low with IVit methotrexate alone. Actually, in summarizing our ten years results we had no recurrence of the intraocular disease (2) and summarizing now our 20-year experience with 113 eyes, we had only two cases of recurrences (unpublished data). It is difficult to explain the poor results of the authors’ patients, using either intravitreal methotrexate al...

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  • Outcomes of penetrating keratoplasty in congenital hereditary endothelial dystrophy

    Dear Editor,
    We have read with interest the recently published article by Al Arrayedh H, Collum L, Murphy CC (1). The authors concluded that a poor outcome was seen after PKP for CHED in Irish population, which arises from a combination of dense amblyopia and a high risk of graft failure in the long term. This is an important study which has a unique cohort of only autosomal recessive cases from a large Irish consanguineous family.
    We want to highlight some points in this article that were not clearly described .
    Author reported 2 previously diagnosed congenital glaucoma cases, which also affects the visual outcome of the surgery and may skew results of this study. But they had not mentioned clearly about this.
    In the figure 2 , the failed DSEK case also received regrafting twice but that was not shown in the legend.Also 32 eyes received penetrating keratoplasty as per the text but in that figure , 33 was mentioned.
    In some previous studies 12 years of age (2,3) has been mentioned as the demarcation for outcome of penetrating keratoplasty in congenital hereditary endothelial dystrophy . This was not analysed in this very important study (maybe because of small numbers) but it could have been a useful clinical hint for timing of surgery in these patients.


    1. AlArrayedh H, Collum L, Murphy CC. Outcomes of penetrating keratoplasty in congenital hereditary endothelial dystrophy. Br J Ophthalmol. 2018 Jan;102(1):19-25

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  • Response to AML after OAC

    Dear Sir,

    We are writing this letter in response to a “letter to the editor” from Drs. Leahey and Meadows about our recently published paper “Second primary malignancies in retinoblastoma patients treated with intra-arterial chemotherapy: the first 10 years”.1 There are a number of incorrect statements in their letter and we hope this letter will clarify those errors made by them.

    1) Drs. Leahey and Meadows state that…”the author’s report is profoundly misleading with regard to the risk of SPMs following OAC”. To prove that they then state that the median follow-up range is 2.5 years with a maximum follow-up of 12 years. How is that “profoundly misleading”? In the same paragraph they then state that patients failing OAC required radiation therapy and suggest that all of these second neoplasms are a consequence of radiation. That is not true. In our paper (page 273, Table 3) we documented that not one of the children who developed a second tumor had received radiation. How could radiation be the cause of a second tumor if the patients never received radiation? Dr. Leahey and Meadows also wrote that…”patients failing OAC require radiation”. That’s not true. On page 272 we stated that…”patients who received external beam radiation prior to presentation at our clinic were excluded from analysis”. None of the patients in this series received radiation after OAC and no patient in our center has received radiation in the past 10 years. We do not dispute that radia...

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  • Planning an extra-ocular muscle biopsy: a note of caution.

    We read the excellent paper ‘Review of extraocular muscle biopsies and utility of biopsy in extraocular muscle enlargement’ by Eade et al.1 with great interest. The authors reviewed the pathology in extraocular muscle biopsies performed over a 25-year period and reported the clinical and radiological features that might distinguish between benign and malignant diseases. As the authors note, it is imperative for the orbital surgeon to consider a muscle biopsy when the diagnosis is in doubt. With this in mind we would like to highlight two relevant cases of simulated extraocular muscle enlargement seen radiologically due to deviated ocular position rather than a pathological process related to the muscle itself. In both cases this confused the clinical picture and nearly resulted in needless surgery.

    In case 1, a 42-year-old woman was referred to the oculoplastic clinic with diplopia, reduced vision in the right eye associated with retro-bulbar pain and facial paraesthesia. On examination, there was evidence of a right esotropia with a reduction of abduction (consistent with a 6th cranial nerve palsy) associated with reduced sensation involving the V1 and V2 distribution. Optic nerve function was normal. Investigations revealed an elevated serum IgG subclass 4 (1.18 g/L) and normal serum ACE. The MRI report confirmed increased girth of the right medial rectus muscle in conjunction with enlargement and pathological enhancement of right cavernous sinus extending into...

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  • Acute Myeloid Leukemia following OAC

    Having read the article of secondary primary malignancies (SPM) in patients treated with ophthalmic artery chemosurgery (OAC) (1), we wish to update Dr. Habib’s report. A patient with unilateral Group D heritable retinoblastoma (RB) treated by their team with 5 OAC treatments in 2012 through 2013 developed acute myeloid leukemia (AML) on 4/26/2017 and died 3 months later.
    Following an intraocular recurrence after OAC and subsequent enucleation the patient’s care was transferred to our institution. Despite adjuvant chemotherapy he developed widespread bone and marrow metastases 8 months after enucleation. He then underwent intensive chemotherapy and an autologous stem cell transplant. Twenty eight months later he developed AML with a fms-like tyrosine kinase 3 (FLT3) mutation. Melphalan administered during all OAC treatments and the drugs administered following that failure must be implicated in this outcome (2). By failing to eradicate retinoblastoma, metastatic disease ensued requiring further treatment and subsequently, AML.
    The author’s report is profoundly misleading with regard to the risk of SPMs following OAC. Although the maximum period of follow-up is 12 years, the median is 2.5 years. Patients failing OAC required radiation therapy and are known to be 3 times more likely to develop additional cancers (3). We await that follow-up.
    The goal of RB care is cure. This patient’s course illustrates the danger of failing to prevent metastatic disease wi...

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