Sunah Kang, Ji Won Seo, Chan Joo Ahn, Bita Esmaeli, Ho-Seok Sa
Aim To calculate a regression formula for intraoperative lagophthalmos to determine the amount of correction in levator resection for mild to moderate congenital ptosis.
Methods This retrospective study included 38 eyelids from 28 consecutive children with congenital ptosis with levator function of 4 mm or better who showed satisfactory surgical outcomes defined as postoperative margin reflex distance-1 (MRD1) ≥3 mm in each eye and difference in MRD1 ≤1 mm between eyes at 6 months after levator resection. We investigated whether the degree of intraoperative lagophthalmos measured by calliper correlated with the preoperative values of MRD1, levator function and age. A stepwise multiple regression analysis was performed with intraoperative lagophthalmos as the dependent variable.
Results The mean intraoperative lagophthalmos was 7.4±0.9 mm (range, 6–10 mm). The intraoperative lagophthalmos was found to have a statistically significant negative correlation with preoperative MRD1 (r2 =0.55, p<0.0001) and levator function (r2 =0.53, p<0.0001), respectively. A stepwise multiple regression analysis resulted in the following regression formula: Intraoperative lagophthalmos=9.08 – 0.48×Preoperative MRD1 – 0.26×Levator function (r2 =0.60, p<0.0001).
Conclusion Intraoperative lagophthalmos in patients with satisfactory surgical outcome correlated negatively with both preoperative MRD1 and levator function and accounting for both variables resulted in a stronger correlation than either variable alone. Surgeons would be able to calculate the amount of surgical correction using this formula of intraoperative lagophthalmos, which could lead to a satisfactory surgical outcome in levator resection for congenital ptosis.
Oded Sagiv, Priyadharsini Nagarajan, Renata Ferrarotto, Thomas J Kandl, Sudip D Thakar, Bonnie S Glisson, Mehmet Altan, Bita Esmaeli
Background/Aims Locally advanced (T4 per American Joint Committee on Cancer (AJCC) 8th edition) periocular basal cell carcinoma (BCC) can lead to loss of the eye. We report the neoadjuvant use of vismodegib followed by surgery in patients with such lesions with eye preservation as primary goal.
Methods This retrospective interventional study includes all patients with a T4 periocular BCC (per 8th edition AJCC for eyelid carcinoma) treated by the senior author between 2013 and 2017 with neoadjuvant vismodegib prior to definitive surgery.
Results Eight patients had a T4 tumour. Six patients presented with recurrent disease. Indications for neoadjuvant treatment were an unresectable tumour in one patient, an attempt to avoid an orbital exenteration in six patients and an attempt to avoid disfiguring facial surgery in one patient. Patients were treated for a median of 14 months (range: 4–36 months). All patients underwent an eye-sparing surgery following neoadjuvant vismodegib and all final surgical margins were negative for tumour. Five patients had a complete response to vismodegib with no microscopic residual BCC found during surgery; three patients had a significant partial response with residual tumour found on pathology. At last follow-up, a mean of 18 (range: 6–43) months after surgery, all patients were off-vismodegib and alive without evidence of disease.
Conclusions Neoadjuvant vismodegib for locally advanced (T4) periocular BCC enabled an eye-sparing surgery in all patients in our cohort. Prolonged treatment was well tolerated by most patients. Over half of patients achieved a complete response with no residual microscopic disease. Careful long-term follow-up is needed to confirm long-term disease-free survival.
Sameh E Soliman, Cynthia VandenHoven, Leslie D Mackeen, Brenda L Gallie
Background/aims To assess tumour control, vision and anatomical visual potential in eyes with perifoveal retinoblastoma treated by sequential photocoagulation from the antifoveal tumour edge inwards, avoiding treatment near the fovea. Patients were monitored for tumour control, foveal and perifoveal anatomy at each treatment session by optical coherence tomography (OCT) and treated for amblyopia when the other eye had better vision.
Methods Eyes with perifoveal retinoblastoma treated between 1 January 2011 and 31 May 2017 with laser therapy after chemotherapy for juxtafoveal (fovea clear of tumour but <3000 µm from tumour edge) or foveolar retinoblastoma (tumour underlying fovea) were retrospectively reviewed for tumour control without recurrence, anatomical success (foveal pit preservation and/or restoration with ≥500 µm perifoveal retina free of tumour and scar) and functional success (acceptable (>0.1 decimal) or good (>0.3 decimal) visual acuity (VA)).
Results Twenty-two eyes (14 juxtafoveal, 8 foveolar tumours) of 20 patients (19 bilateral, 1 familial and 11 females) were included. No juxtafoveal tumour had tumour recurrence, and 13/14 patients showed foveal pit preservation with ≥500 µm of perifoveal retina tumour free. Foveolar tumours had significant worse anatomical outcomes: failure to restore foveal pit or perifoveal retina (8/8, p=0.001) and tumour recurrences (5/8, p=0.001). Functional success with acceptable VA was achieved in 12/14 juxtafoveal and 5/8 foveal tumours eyes (p=0.01). Amblyopia therapy data were insufficient to evaluate impact on VA.
Conclusions Anatomical visual potential and functional vision were better in juxtafoveal than foveolar retinoblastoma treated with foveal-sparing laser photocoagulation guided by OCT. The role of amblyopia therapy requires a prospective study.
Christina Stathopoulos, Alexandre Moulin, Marie-Claire Gaillard, Maja Beck-Popovic, Francesco Puccinelli, Francis L Munier
Background/aims To report conservative therapy in diffuse infiltrating retinoblastoma (DIR) and describe specific optic coherence tomography (OCT) features of the tumour.
Methods Retrospective review of all DIR cases treated conservatively between 1998 and 2012.
Results Three patients (three eyes) were included, cases 1 and 3 with previous enucleation of the contralateral eye and case 2 with unilateral retinoblastoma referred after prior pars plana vitrectomy with silicone oil. Mean age at diagnosis was 7 years (range 14 months–14 years). Globe and vision preservation (Snellen visual acuity of 12.5/10) was achieved in case 3 with a recurrence-free follow-up of 33 months after first-line thermotherapy followed by salvage intra-arterial chemotherapy (IAC) plus focal treatments. Cases 1 and 2 were enucleated for progressive disease, case 1 after first-line intravenous chemotherapy (IVC) consolidated by focal therapies and salvage treatments given over 8 years of partial remission and case 2 after IAC, brachytherapy and intracameral chemotherapy. Neither showed any high-risk histopathological features, and no adjuvant chemotherapy was necessary. Both patients are alive without metastasis (mean follow-up of >10 years). Pathognomonic features of the tumour were revealed by OCT in all cases, showing infiltration of the ganglion cell layer and horizontal growth over the inner plexiform layer. Complete restoration of the retinal microanatomy was documented after retraction of the tumour following IVC in case 2 and IAC in case 3.
Conclusion This is the first report of successful conservative management in DIR. OCT enabled diagnosis, delimitation of the tumour margins and monitoring of the treatment response in this context.