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.

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.

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.

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.

High nasolacrimal sac-duct junction anatomical variation - retrospective review of dacryocystography images.

PURPOSE: In assessing epiphora, dacryocystography (DCG) is often performed to determine the presence of any structural anomaly of the nasolacrimal drainage apparatus. We describe an anatomical variation of the nasolacrimal duct, termed high sac-duct junction (HSDJ) morphology, that is seen on DCG, which could lead to more difficult, and possibly unsuccessful, intubation of the nasolacrimal duct (NLD). METHODS: This was a multi-center, retrospective, observational case series. Seven hundred and thirty-four DCGs were included in this study, of which 45 were pediatric images. DCG images underwent a blinded review by an oculoplastic surgeon for the presence of HSDJ morphology. The direct extension of the NLD from the inferior border of the lacrimal sac is commonly regarded as a normal morphology. HSDJ was defined as NLD originating from the inferior aspect of the medial wall of the lacrimal sac. RESULTS: HSDJ was seen in 28/689 (4.1%) adult scans and 7/45 (15.6%) pediatric scans. In total, there were 35/734 (4.8%) DCGs demonstrating lacrimal sacs with HSDJ. CONCLUSIONS: We report the prevalence of an anatomical variant of the lacrimal sac and NLD, observed on DCG, in a population of patients with epiphora, where the NLD originates from the inferior aspect of the medial wall of the lacrimal sac. Knowledge of this anatomic variant may have management implications for patients with epiphora as it may make nasolacrimal intubation more technically difficult. Further studies are needed to correlate this anatomic variation with symptoms and management outcomes.

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.

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.

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.

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.

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.

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.

Interobserver agreement on interpretation of conventional dacryocystography and dacryoscintigraphy findings: A retrospective single-centre study.

IMPORTANCE: This study assesses the interobserver agreement on dacryocystography (DCG) and dacryoscintigraphy (DSG) findings. BACKGROUND: There are no standard grading criteria to guide the interpretation of conventional DCG and DSG findings and therefore there may be a degree of subjectivity. This study evaluates the level of interobserver agreement in the interpretation of DCG and DSG findings. DESIGN: A retrospective observational study at the Royal Adelaide Hospital. PARTICIPANTS: A total of 165 patients who presented with epiphora with 276 DCGs and 290 DSGs performed were included in this study. METHODS: DCG and DSG images were obtained, anonymized, randomized and interpreted by three independent oculoplastic surgeons. Standard grading criteria were set for both DCG and DSG images. Data from all observers were analysed for interobserver agreement using Kappa (κ) statistics, generated using a variation of Cohen's kappa for multiple observers. MAIN OUTCOME MEASURES: Level of interobserver agreement (κ values) in the grading of DCG and DSG findings. RESULTS: There was an overall moderate interobserver agreement for DCG findings (κ = 0.55), with the highest agreement on interpreting canalicular obstruction (κ = 0.80), followed by proximal nasolacrimal duct obstruction (κ = 0.67) and normal patency (κ = 0.63). There was an overall fair interobserver agreement for DSG findings (κ = 0.36), with the best being moderate agreement (κ = 0.42-0.50) for interpreting pre-sac delay and post-sac proximal delay. CONCLUSIONS AND RELEVANCE: DCG offers good reliability in interpreting patent and obstructed systems. On the other hand, DSG has poor agreement and highlights some of the limitations in the ability to guide epiphora management.

Time-resolved three-dimensional technique for dynamic magnetic resonance dacryocystography.

IMPORTANCE: This study introduces a new technique in the assessment of nasolacrimal drainage with magnetic resonance imaging. BACKGROUND: To describe a new dynamic magnetic resonance dacryocystography (MRDCG) protocol and report normative findings on asymptomatic lacrimal drainage systems. DESIGN: A prospective observational study at the Royal Adelaide Hospital. PARTICIPANTS: Seventeen patients with unilateral epiphora were prospectively recruited. METHODS: Patients underwent dynamic MRDCG. The time-resolved angiography with interleaved stochastic trajectories (TWIST) sequence was used to visualise contrast flow through the lacrimal drainage system. MAIN OUTCOME MEASURES: Tear transit times and anatomical dimensions were measured. RESULTS: Dynamic MRDCG with TWIST sequence reliably demonstrated contrast flow. Tear transit times and anatomical dimensions were comparable to previously reported values in other imaging modalities. CONCLUSIONS AND RELEVANCE: Dynamic MRDCG provides good structural and temporal resolution to the assessment of lacrimal outflow obstructions. The physiological nature of this modality allows potential assessment for functional obstruction and future research on tear flow assessment.

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.

Endoscopic Orbital Decompression by Oculoplastic Surgeons for Proptosis in Thyroid Eye Disease.

PURPOSE: Orbital decompression is an established surgical treatment option for a range of orbital conditions. Traditionally, Ear, Nose and Throat surgeons have adopted the endoscopic route while ophthalmologists operate via an external approach. The authors report the outcomes of endonasal decompression performed by oculoplastic surgeons experienced in endonasal techniques. METHODS: This was a retrospective case series of patients who underwent endoscopic orbital decompression for proptosis secondary to thyroid eye disease across 2 hospital sites between January 2011 and July 2018. Inclusion criteria were patients who had endoscopic decompression for proptosis in inactive thyroid eye disease or active disease without dysthyroid optic neuropathy. Information collected includes patient demographics, diagnosis, surgical details, preoperative and postoperative clinical findings (including, visual acuity, color vision, exophthalmometry readings, palpebral aperture, intraocular pressure, ocular motility, diplopia, and visual field), complications, and further treatment. RESULTS: There were 70 cases of endoscopic decompression. The majority of patients had endoscopic medial and posterior medial wall/floor decompression (44.3%; 31/70 cases). Visual acuity remained stable in 98.6% (69/70). There was an average reduction in proptosis of 3.5 ± 1.2 mm (standard deviation [SD]) in the endoscopic medial wall only group, 3.9 ± 0.9 mm (SD) in endoscopic medial wall and posterior medial portion of the floor group, and 7.6 ± 2.1 mm (SD) in the 3-wall decompression group. Motility improved in 11.4% (8/70) and worsened in 2.9% (2/70). There were no significant intraoperative or postoperative complications associated with endoscopic surgery. CONCLUSIONS: Oculoplastic surgeons experienced in endonasal techniques can perform endoscopic orbital decompression with outcomes comparable to the literature.The authors report the outcomes of a series of endonasal orbital decompression carried out by oculoplastic surgeons.

Lacrimal Gland Orbital Lobe Cysts Associated With Lymphoid Hyperplasia or Mucosa-Associated Lymphoid Tissue Lymphoma in Patients With Chronic Autoimmune Disease.

Large cysts in the orbital lobe of the lacrimal gland are rare and are associated with Sjögren syndrome and B-cell mucosa-associated lymphoid tissue lymphoma. The authors describe 4 new cases of large orbital lobe lacrimal gland cysts. The first 2 patients, both with Sjögren syndrome, had unilateral cysts associated with chronic inflammation. Mucosa-associated lymphoid tissue lymphoma was also identified in the cyst wall of the second case and could not be completely excluded in the first case. The third patient, with a history of rheumatoid arthritis, had bilateral cysts, again associated with mucosa-associated lymphoid tissue lymphoma. The fourth patient, with no history of systemic disease, had a unilateral cyst associated with reactive lymphoid hyperplasia. Finally, the authors report the long-term outcomes of 3 previously reported cases.

Prelacrimal approach for nasolacrimal duct excision in the management of lacrimal system tumours.

Purpose: To report a case of a lacrimal sac tumour and describe a prelacrimal approach to the maxillary sinus to excise the nasolacrimal duct in its management. To our knowledge, this approach has not been adapted to remove the nasolacrimal duct for the management of pathological processes involving the nasolacrimal system. Methods: A 58-year-old female patient presented with a 6-month history of epiphora. A lacrimal sac mass was identified, and a biopsy revealed squamous cell carcinoma. Surgical excision was performed via a combined external and endoscopic prelacrimal approach. Results: A prelacrimal approach to the maxillary sinus to excise the nasolacrimal duct in combination with an external approach facilitated an en-bloc excision of the nasolacrimal apparatus. This allowed preservation on the entire inferior turbinate following reconstitution of the lateral nasal wall at the completion of the procedure. Conclusion: The endoscopic endonasal prelacrimal approach to the maxillary sinus is a useful method to approach and excise the nasolacrimal duct in the management of nasolacrimal pathology.

Primary replacement for the management of exposed orbital implant.

Purpose: We present a series of primary orbital implant replacement for cases of implant exposure to describe our experience of this one-staged surgical approach. Methods: This study reports on a one-stage technique which involved the removal of the exposed implant or dermis fat graft (DFG) and insertion of a secondary (replacement) in the same procedure, with a variety of materials, including autologous tissue. Re-exposure in a socket where a DFG was placed was defined as a new defect in the newly epithelialized conjunctiva or dehiscence of the dermis-conjunctiva junction. All cases of primary replacement for the management of exposed orbital implant, porous and non-porous, were included, even when there were clinical signs suggestive of infection. The primary outcome was the rate of re-exposure, requiring additional surgical procedures. Infection following primary replacement was a secondary outcome. Results: Seventy-eight patients had primary replacement for the management of an exposed orbital implant. 6.4% had re-exposure at a mean follow-up of 49.7 months (9.1% for ball implants and 4.5% for DFG). The rate of exposure was higher in those with prior signs of infection than those without (8% vs. 3.6%). Re-exposure occurred in 4.5% of cases with DFG implantation, 4.3% of cases with non-porous implants and in 20% of cases with porous implants. Conclusion: Primary replacement for management of exposed orbital implant, porous and non-porous, has a high rate of successful outcome even in cases with presumed or confirmed infection.

Case report: a case of diffuse unilateral subacute neuroretinitis (DUSN) in a child.

BACKGROUND: Diffuse unilateral subacute neuroretinitis (DUSN) is a rare cause of posterior uveitis in the United Kingdom. It typically presents unilaterally in children and young adults but rarely bilateral cases have been reported. It is also rare to have multiple worms in the same eye causing the clinical picture. In this article, we present a challenging case of DUSN in a young girl unresponsive to conventional treatments suggesting the possibility of multiple worms being present in the same eye. CASE PRESENTATION: An 8-year-old girl presented with a 2-month history of headaches. On occasions the headaches were associated with redness and watering of her left eye. She denied any visual loss or visual symptoms. Her visual acuity was reduced to 6/30 in her left eye. Fundal examination revealed a unilateral chorioretinitis. Investigation did not reveal a specific cause for the chorioretinitis. Over 15 months her visual acuity improved to 6/9 but the fundal appearance changed and a diagnosis of DUSN was made. She was treated with focal laser, systemic anti-helminthic and immunosuppressive treatments but continued to develop new, active areas of chorioretinitis, raising the possibility of multiple worms in the sub-retinal space. There is also a concern as to other central nervous system (CNS) involvement given her significant and ongoing headaches. CONCLUSION: We present a challenging case of DUSN in a young girl; a condition that remains rare in the UK. She was unresponsive to both focal laser and systemic anti-helminthic and immunosuppressive treatments suggesting the possibility of multiple worms being present in the sub-retinal space. This case highlights the difficulties often encountered in the treatment of DUSN, even when a worm can be identified. Her visual prognosis is poor as there was ongoing recurrence of active chorioretinitis.