Corneal Neurotization for Neurotrophic Keratopathy: A Multicentre Experience.

PURPOSE: Corneal neurotization is an emerging technique that offers potential for visual rehabilitation in neurotrophic keratopathy. This study reports on a multicenter experience and outcomes for both direct and indirect methods of corneal neurotization. METHODS: Retrospective case series. Sixteen patients with neurotrophic keratopathy who underwent corneal neurotization across 5 centers in Australia and Israel were identified for inclusion. Corneal neurotization was performed via direct neurotization from the ipsilateral or contralateral supraorbital/supratrochlear nerve or by the use of an interpositional sural nerve graft. Change in corneal sensitivity (measured in millimeters by the Cochet-Bonnet aesthesiometer), visual acuity, and corneal health. RESULTS: Over a mean follow-up period of 31.3 months (range: 3 months-8 years), mean corneal sensitivity improved from 3.6 mm (range: 0-25 mm) to 25.3 mm (range: 0-57 mm). Visual acuity improved on average from 20/380 to 20/260. Twelve of 16 patients (75.0%) improved in at least 2 out of the 3 main outcome measures. Nine patients (56.3%) showed an improvement in visual acuity; 13 (81.3%) showed an improvement in average corneal sensitivity; and 11 (68.8%) showed an improvement in corneal health. There were no intraoperative or postoperative complications. CONCLUSIONS: Corneal neurotization is an emerging surgical treatment option for the management of neurotrophic keratopathy. With appropriate case selection, outcomes are favorable and complication rates are low, for a condition that is otherwise challenging to manage. Patients with severe neurotrophic keratopathy should be considered for this surgical treatment option.

Incidence and survival of uveal melanoma in Northern Ireland: how incomplete data can skew results in rare cancers.

BACKGROUND: The majority of Northern Irish uveal melanoma (UM) patients are diagnosed in Sheffield. This study aims to present incidence and survival outcomes for UM patients from Northern Ireland (NI). METHODS: Collaborative retrospective study between Sheffield and Northern Ireland Cancer Registry (NICR). For UM cases not on both databases, outcomes and survival rates (via Kaplan-Meier analysis) were compared. Anonymised NICR data were used to calculate whole-population incidence of UM for NI. RESULTS: In total, 161 patients from NI were diagnosed in Sheffield, 90 of which were not registered with NICR at the start of this study. Data-omissions were not consistent across patient groups, leading to significant differences between those patients registered and those not. Registered patients had an all-cause 5-year survival rate of only 68.9% compared to 92.5% of those not registered (p < 0.01) and were >17x more likely to have systemic metastases than those not registered (p < 0·001). Following rectification of data-omissions, the European age-standardised incidence rate of UM for NI was 8·6 per million. CONCLUSIONS: This study illustrates the impact of incomplete population-wide data, serving as a real-world lesson in case-identification bias. Rare cancers are at higher risk of omission due to systemic failures as the small numbers involved are not detected by system-wide validation procedures. Following this study, data-transfer agreements between England and NI were actioned, preventing future data-omissions. We present survival and incidence data for UM in NI for the first time, showing the incidence is amongst the highest in Europe, with good survival rates.

Paediatric fronto-orbital osteoblastoma: case report.

Osteoblastoma is a rare benign bone tumour. It represents 1% of all primary bone tumours. Common locations are the vertebral column and long bones of the extremities. Although well documented in craniofacial locations, only occasional case reports describe orbital involvement. The authors present a case of a fronto-orbital osteoblastoma which posed a significant diagnostic dilemma. Despite early local recurrence, the molecular findings were supportive of a benign lesion.

The use of frontalis sling in the management of variable ptosis secondary to congenital myasthenic syndrome.

Congenital myasthenic syndrome (CMS) describes a group of rare inherited disorders caused by impaired neuromuscular transmission at the motor endplate. Common ophthalmic manifestations associated with CMS include ptosis and ophthalmoplegia. A 19-year-old female presented with variable day-to-day ptosis secondary to CMS that was refractory to medical therapy. Bilateral silicone frontalis slings were used to stabilise the upper lid height and reduce fluctuation in severity of ptosis. Blepharoptosis surgery has been performed in patients with chronic myasthenia gravis (MG), but rarely in the setting of CMS. Blepharoptosis surgery in CMS patients with variable ptosis is difficult due to the risk of upsetting the original lid position and developing post-operative exposure keratopathy. Our case demonstrates that the frontalis sling procedure may be considered as an option in the management of variable blepharoptosis secondary to CMS.

Primary Adnexal Signet-Ring Cell/Histiocytoid Carcinoma.

An 84-year-old man presented with a 2-year history of a progressive left-sided ptosis. Examination demonstrated a mechanical ptosis and concentric constriction of the palpebral aperture. CT imaging revealed demonstrated diffuse soft tissue infiltration of the upper and lower eyelids with extension into the anterior orbit. This case was diagnostically challenging because of a history of multiple other primary tumors. However, clinicoradiologic and histopathologic findings were consistent with a diagnosis of primary adnexal signet-ring cell/histiocytoid carcinoma. The patient underwent surgical excision but local recurrence was noted 2 months postoperatively.

Primary Nasocutaneous Fistulae in Granulomatosis With Polyangiitis: A Case Series and Literature Review.

PURPOSE: To report the presentation, investigation, management, and outcomes of primary nasocutaneous fistulae in granulomatosis with polyangiitis and review the literature of this complication. METHODS: Retrospective series of 5 patients with granulomatosis with polyangiitis and nasocutaneous fistulae and the medical and surgical management strategies employed. RESULTS: Two cases presented with fistulae as the primary symptom of their systemic disease. Systemic management of granulomatosis with polyangiitis is a primary concern and two cases had inadequate control at presentation such that surgical intervention was deferred. Of the other 3 cases, 2 were closed using a median forehead flap and the other with an orbicularis advancement flap. An endonasal approach was attempted in 1 case but was unsuccessful due to the friable nature of the nasal mucosal tissue. CONCLUSIONS: Nasocutaneous fistulae should alert the clinician to underlying granulomatosis with polyangiitis, which may be a presentation of this condition. Systemic management of disease is often a barrier to surgical management. Myocutaneous flaps may be the most reliable management option to achieve closure.

Neoadjuvant vismodegib in the management of locally advanced periocular basal cell carcinoma.

BACKGROUND/OBJECTIVES: Vismodegib, a hedgehog pathway inhibitor, has been used in the management of locally advanced basal cell carcinoma (BCC) not suitable for surgery or radiation therapy. We report our experience using neoadjuvant vismodegib for locally advanced periocular BCC, followed by surgical excision. Our aim was to assess the effect on the extent of surgical excision and histological response. SUBJECTS/METHODS: A retrospective case series of patients treated with neoadjuvant vismodegib, for the management of locally advanced periocular BCC prior to surgical excision, with intraoperative margin control. Patients were treated until a maximum clinical response was seen. The difference between the estimated surgical margins prior to vismodegib and the eventual margins used was compared. Fine (1 mm) vertical sections through the excised tumour were performed to assess the histological response and look for a multifocal tumour. RESULTS: Eight Caucasian patients had neoadjuvant treatment with vismodegib for a median duration of 6 months. Some clinical response was seen in all cases but was only partial in 6/8 patients. Histological evaluation of the excised specimen showed residual BCC in 6/8 cases and thus 2/8 showed complete histological regression. Two cases showed squamous differentiation. Side effects occurred in 7/8 patients all which resolved on cessation of therapy. The mean follow-up was 13.4 ± 5.2 months. CONCLUSIONS: Neoadjuvant treatment of periocular BCC showed a mixed clinical and histological response. Final surgical excision achieved clear margins in all patients with no recurrence at 13 months and a reduction in predicted defect size, but possible squamous differentiation in two cases.

In Vivo Morphology of Nasolacrimal Duct Opening into the Inferior Meatus.

PURPOSE: To study the in vivo morphology of nasolacrimal duct (NLD) openings into the inferior meatus. METHODS: Patients undergoing endoscopic dacryocystorhinostomy and lacrimal intubation had endoscopic real-time examination of the NLD opening. Morphology of NLD openings (size, shape, mucosal folds), and their location from the axilla of the inferior turbinate were assessed. RESULTS: Forty-three lacrimal systems of 39 adult patients (11 males, 28 females) with a mean age of 61 years were studied. Thirty-five patients had unilateral involvement, and 74% had anatomical NLD obstruction. Of 43 lacrimal systems, 41 had a visible NLD opening. The majority was sulci shaped (63%, 26/41) followed by fissure type (34%), and 2% had wide-open round morphology. The majority of the openings (93%) were oriented vertically, and 67% (14/21) of the sulci shaped openings continued into gutter like channels along the lateral nasal wall. Mucosal folds were present in 7% (3/41) of the openings along the edges. The mean distance between the opening and the axilla of the inferior turbinate was 3.7± 2.1 mm (median, 3.6; range, 0-7.2). CONCLUSION: Sulci shaped NLD openings were the most common morphology, and mucosal folds were less commonly observed compared with cadaveric studies.

In Vivo Morphological Study of Common Canalicular Orifice and Its Impact on Lacrimal Probing.

PURPOSE: To study the in vivo morphology of common canalicular/lacrimal sac mucosal folds (CLS-MFs) and their relationship with probing findings. METHODS: Consecutive cases undergoing endoscopic dacryocystorhinostomy had endoscopic examination of the internal canalicular orifice (ICO). Details of CLS-MFs folds, probing findings, and outcomes of dacryocystorhinostomy were analyzed. RESULTS: Thirty-six lacrimal systems of 34 patients (mean age, 58 years; 7 males, 27 females) were examined. All 36 lacrimal systems had a single common canalicular orifice entering the lacrimal sac (100%). Overall, 61.1% (22/36) had visible CLS-MFs, out of which only 13.6% (3/22) were overhanging the ICO requiring manipulation of the probe to enter the lacrimal sac. The orientation of folds was superior 180° in 2, posterosuperior in 2, posterior in 6, inferior 180° in 6, and inferior 270° (excluding 10-2'o clock quadrant) in 6 systems. Two cases preoperatively labeled as having common canalicular obstruction, based on lacrimal syringing, had CLS-MFs impacting against the ICO without any anatomical obstruction. One of 5 cases with lacrimal sac mucocele had CLS-MFs located along the posterosuperior edge extending for 270°, which resulted in a soft stop on preoperative lacrimal syringing findings. Only 1 case had a true membrane (2.7%) overlying the ICO, which required excision. No differences in outcomes of dacryocystorhinostomy were observed between cases with CLS-MFs versus without any folds. CONCLUSION: CLS-MFs display a wide variation in terms of morphological location and extent along the ICO. They might be responsible for the false soft stop observed in some cases on preoperative syringing and do not require excision.

Pseudocystic appearance of an orbital carcinoid metastasis.

Carcinoid tumours are a low-grade neuroendocrine malignancy that infrequently metastisizes to orbital structures. The typical radiological appearance of carcinoid is a solid, well-circumscribed mass that enhances with contrast. We present a case of orbital carcinoid metastasis with pseudocystic radiological appearance and review of the literature.

Swinging inferior turbinate approach to the nasolacrimal duct.

Purpose: To report a case of a lacrimal sac tumour identified at the time of endoscopic dacryocystorhinostomy and describe a swinging inferior turbinate approach to the nasolacrimal duct (NLD) to facilitate an en-bloc excision of the lacrimal drainage apparatus.Methods: An 88-year-old male presented with a 6-month history of epiphora and mucocele. Endonasal DCR was performed for nasolacrimal duct obstruction (NLDO). Intraoperatively, a biopsy was performed of abnormal appearing lacrimal sac mucosa, following opening of the lacrimal sac. Subsequent definitive management was performed via a combined external and endoscopic approach using a swinging inferior turbinate approach to the NLD to facilitate an en-bloc excision of the lacrimal drainage apparatus, without removal of the bony medial wall of the maxillary sinus.Results: The excised lacrimal drainage showed insitu and invasive squamous cell carcinoma of the canaliculi and lacrimal sac with focal divergent neuroendocrine and sebaceous differentiation. There was no local tumour recurrence or metastatic spread at 3 months of follow-up.Conclusions: We describe a swinging inferior turbinate approach to the NLD to facilitate an en-bloc excision of the lacrimal drainage apparatus, without entering the maxillary sinus. We believe this modified technique is a useful option to consider in the management of tumours of the lacrimal drainage apparatus.

The Microbiome of the Nasolacrimal System and Its Role in Nasolacrimal Duct Obstruction.

PURPOSE: Acquired nasolacrimal duct obstruction (NLDO) is a common problem leading to epiphora, the pathophysiology of which remains unclear. Culture-based studies have found Staphylococcal species to be the most prevalent organisms, reported in 47% to 73% of patients with NLDO. Recently, culture-independent molecular methods of have allowed more comprehensive detailing of local microbiota. This study aims to evaluate the sinonasal and lacrimal microbiome of patients undergoing dacryocystorhinostomy for NLDO using 16S-amplicon sequencing. METHODS: Guarded intraoperative swabs were taken from the middle meatus (MM), inferior meatus, and the opened lacrimal sac of 14 NLDO patients undergoing dacryocystorhinostomy and from the inferior meatus and MM on the contralateral unaffected side. MM swabs from 12 control patients were compared with NLDO patients. RESULTS: Comparing microbiota at lacrimal sac to MM and inferior meatus sites reveals that the lacrimal sac microbiome is dominated by Staphylococci (36.3%) and Corynebacterium (35.8%). No significant genus differential abundance between the 3 sites, and between the ipsilateral and contralateral sinonasal swabs, and no convincing evidence of reduced alpha diversity in all comparisons. There was a statistically significant lower relative abundance of Corynebacterium (37.6% vs. 65.1%; p = 0.035) in the MM of NLDO patients compared with controls. CONCLUSIONS: The lacrimal sac microbiome in acquired NLDO is similar to the sinonasal microbiome. The relative abundance of Corynebacterium was reduced compared with controls. These findings suggest that an altered sinonasal microbiome may be associated with NLDO, either as a consequence or a risk factor, and merits future research.The authors have demonstrated a decreased relative abundance of Corynebacterium at the middle meatus of patients with ipsilateral nasolacrimal duct obstruction (NLDO), compared with controls, and that the lacrimal sac microbiome is similar to the sinonasal microbiome. An altered microbial state may, therefore, be associated with NLDO, either as a consequence or a risk factor, and merits future research.

Nasolacrimal duct obstruction secondary to lacrimal sac involvement by sebaceous carcinoma.

Sebaceous carcinoma (SC) is the third most common eyelid malignancy in Australia, and is potentially fatal. It usually presents as a nodule or diffuse eyelid thickening, and is commonly misdiagnosed. We describe a case of SC with lacrimal sac involvement, presenting with clinical features of nasolacrimal duct obstruction. At the time of endoscopic dacryocystorhinostomy (DCR), nasal endoscopy revealed a polypoid mass of the opened lacrimal sac. Biopsy of the mass showed poorly differentiated SC. The lacrimal drainage apparatus was later excised via a combined external and endoscopic approach. Conjunctival map biopsies showed extensive intraepithelial disease, which was treated with topical mitomycin C. At three-month follow-up, there was no evidence of residual disease on nasal endoscopy or repeat conjunctival biopsy.

Endoscopic orbital decompression for proptosis in non-thyroid eye disease.

PURPOSE: Orbital decompression is an established surgical treatment option for a range of orbital conditions. We report the outcomes of endonasal decompression to recess the globe for conditions and pathologies other than thyroid eye disease. METHODS: This was a retrospective case series of patients who underwent endoscopic orbital decompression for proptosis secondary to non-thyroid eye disease orbital pathologies. The procedures were carried out by oculoplastic surgeons across two hospital sites between January 2011 and July 2018. Information collected includes patient demographics, diagnosis, surgical details, pre- and postoperative clinical findings (including visual acuity, exophthalmometry readings, intraocular pressure, ocular motility and diplopia), complications and further treatment. RESULTS: There were seven cases of endoscopic decompression, each due to a different pathology. The reasons for decompression were proptosis secondary to optic nerve sheath meningioma (1); sphenoid wing meningioma (1); idiopathic myositis (1); axial myopia (1); chronic third nerve palsy (1); to protuberant keratoprosthesis (1); and Crouzon syndrome with corneal exposure (1). Visual acuity remained stable or improved in all patients postoperatively. There was an average reduction in proptosis of 3.5 ± 1.4 mm (standard deviation - SD). Ocular motility remained stable in 100% (7/7). There were no intraoperative or postoperative complications, including no new cases of postoperative diplopia. CONCLUSIONS: Endoscopic orbital decompression can be performed for patients with proptosis associated with a large globe, facial dysplasia or medial and infero-medial orbital lesions.

Cerebrospinal Fluid Leak From the Orbital Roof During Orbital Exenteration.

Cerebrospinal fluid leaks are rarely encountered by orbital surgeons, but can occur during dacryocystorhinostomy, orbital decompression, orbital exenteration, and enucleation. The authors present a case of cerebrospinal fluid leak from an injury to the orbital roof during orbital exenteration with the use of monopolar cautery. This was repaired with multilayer closure. Surgeons performing orbital exenteration must be aware of the possibility of this complication when using monopolar electrocautery to transect the orbital contents at the apex during an exenteration.Injury to the orbital roof with monopolar cautery during orbital exenteration can result in cerebrospinal fluid leak, and orbital surgeons must be aware of the possibility of its occurrence.