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.

Orbital apex anatomy: relationship between the optic foramen and anterior face of sphenoid sinus - a radiological study.

BACKGROUND/OBJECTIVES: This study aims to identify radiologically the position of the optic foramen in relation to the anterior face of the sphenoid sinus, to aid surgeons in their planning for orbital decompression. METHODS: CT scans of 100 orbits from 50 adult patients without any abnormality were assessed. Primary outcome measures included: position and measurement of the distance from the optic foramen to the anterior face of the sphenoid sinus. Secondary outcomes included: medial orbital wall length, distance from the optic foramen and the anterior face of the sphenoid sinus to the carotid prominence in the sphenoid sinus, and the thickness of bone anterior to the optic foramen. RESULTS: The mean location of the optic foramen was just posterior to the position of the anterior face of sphenoid sinus, with an average distance of +0.4 +/- 3.5 mm. In 54% of orbits the optic foramen was positioned posterior to the anterior face of the sphenoid sinus. The finding was symmetrical in 80% of patients. CONCLUSIONS: Our study identifies that the optic foramen lies posterior to the anterior face of sphenoid sinus in approximately half of cases. The position may be asymmetric in 20% of individuals.

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.

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.

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.

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 fat decompression for the management of proptosis in Grave's orbitopathy using a laryngeal skimmer blade.

BACKGROUNDS/OBJECTIVES: Intraoperative handling and manipulation of orbital fat remains a challenge to orbital surgeons. We present a case series of endoscopic orbital fat decompression with medial orbital wall decompression for proptosis management in Grave's orbitopathy, describing a technique for fat excision using a laryngeal skimmer blade, reporting clinical and surgical outcomes, and complications. SUBJECTS/METHODS: All patients who underwent endoscopic orbital fat decompression, with medial orbital wall decompression, for proptosis management in Grave's orbitopathy between 2011 and 2018, under the care of a single surgeon, were included in this retrospective interventional case series. RESULTS: Nineteen patients were included in this study. Using a laryngeal skimmer blade, orbital fat was excised endoscopically at the time of medial orbital wall decompression. The mean volume of orbital fat excised was 1.45 ± 0.63 ml and the mean reduction in proptosis was 4.5 ± 1.02 mm. There were no intraoperative complications. CONCLUSIONS: In this study, we describe our experience of using a laryngeal skimmer blade as a method of excising orbital fat for orbital decompression in patients with proptosis secondary to Grave's orbitopathy.

Augmented endoscopic orbital apex decompression in dysthyroid optic neuropathy.

PURPOSE: To describe a modified technique of endoscopic orbital decompression for dysthyroid optic neuropathy nonresponsive to pulsed corticosteroids. METHODS: Retrospective, interventional single centre case series included 17 consecutive patients with dysthyroid optic neuropathy (DON) who were refractory to pulse corticosteroids. Removal of the posteromedial floor and the orbital process of palatine bone (OPPB) was performed in addition to the endoscopic transethmoidal medial orbital wall decompression (ETMOWD), to achieve maximal orbital apex decompression. Main outcome measures were change in visual acuity (VA), color vision, degree of proptosis reduction, incidence of new-onset diplopia, and any complications. RESULTS: Seventeen eyes (100%) had a statistically significant improvement in VA from 1.0 ± 0.44 LogMAR to 0.0 ± 0.15, with an average improvement of 0.41 ± 0.30 LogMAR (p 0.05, paired t-test). Fourteen out of 16 eyes had a complete improvement in color vision and two eyes had partial recovery. Afferent pupillary defect (76.5%) resolved in all cases. Five out of 10 cases with preoperative visual field defects demonstrated no residual field defects following surgery. The range of proptosis reduction was 0-5 mm (mean 2.7 ± 1.3 mm). No patients with diplopia (12/17) had worsening or developed new-onset diplopia following surgery. CONCLUSION: Combined removal of the posterior medial floor including the OPPB with ETMOWD may be a viable alternative in the surgical management of DON.