Intraepithelial Sebaceous Gland Carcinoma: A Multicenter Retrospective Case Series.

PURPOSE: Intraepithelial sebaceous gland carcinoma is a rare form of sebaceous gland carcinoma, with 10 published case reports to date. The authors report the clinical, histological, and prognostic features of this rare carcinoma. METHODS: This is a multicenter retrospective case series of patients from 3 Australian sites. Information collected included patient demographics, clinical risk factors, initial and subsequent presentations, histology results, management, and outcomes. RESULTS: Twelve cases were identified. The average age of presentation was 72 years (range 52-92 years), with more females (n = 8) affected than males. The most common symptoms and signs were ocular irritation (n = 9) and eyelid mass (n = 8), with a predilection toward upper lid involvement. Five cases of sebaceous gland carcinoma were confirmed on initial histology. Initial management included wide local excision with margin control (n = 11), and primary topical mitomycin C (n = 1). Adjunctive conjunctival mapping biopsy was performed in 5 cases. Recurrence occurred in 7 cases, at an average of 31 months (range 7-83 months) after initial treatment. There were no cases of distant metastasis, however, 2 cases developed local invasion. Management of recurrences included exenteration (n = 2), further excisions alone (n = 3), and excision with adjuvant mitomycin C (n = 2). CONCLUSIONS: Clinicians and pathologists should have a high index of suspicion for primary intraepithelial sebaceous gland carcinoma on the upper eyelid. Close follow-up is recommended, given the high risk of local recurrence.

Orbital Myeloma and Plasmacytoma: An Australian Study.

PURPOSE: The purpose of this study was to provide Australian data on the clinical and radiological features and outcomes in patients with orbital plasmacytomas. METHODS: Multicentre retrospective review of orbital plasmacytoma and orbital involvement in multiple myeloma (MM) from 2005 to 2022 in Australia. RESULTS: Twenty-one participants were identified. The median age was 62 years (range 34-88 years), and 11 (52%) were females. Eighteen (84%) had a known diagnosis of MM prior to their orbital presentation, with all patients eventually being diagnosed with systemic MM. Thirteen (72%) were receiving active treatment for systemic myeloma on presentation, while 3 (17%) were in remission. All but 1 had unilateral orbital involvement (n = 20, 95%). Common presenting symptoms and signs were decreased visual acuity (n = 13, 62%), proptosis (n = 11, 52%), limited motility (n = 15, 71%), and optic neuropathy (n = 5, 24%). Radiologically, 15 (71%) involved the superotemporal orbit, 7 (33%) inferotemporal orbit, and 16 (76%) involved ≥1 extraocular muscle. Sixteen (76%) were biopsied and confirmed orbital plasmacytoma on histopathology. Treatment modalities included intravenous and oral steroids (n = 7, 33%), chemotherapy (n = 9, 43%), radiotherapy (n = 13, 62%), stem cell transplant (n = 3, 14%), and surgical debulking and decompression (n = 3, 14%). Mortality was high, with 15 (71%) having MM-related mortality. CONCLUSIONS: This is the largest cohort of Australian data on orbital plasmacytomas. Most patients have a diagnosis of systemic MM at presentation. It is crucial to recognize and treat these patients early due to a poor systemic prognosis.

Orbital cellulitis and osteomyelitis secondary to odontogenic infection with campylobacter rectus: a case report.

The authors report the first case of orbital osteomyelitis due to Campylobacter in a 50-year-old male on a background of poor dental health. Campylobacter rectus is a member of the human oral flora and is usually associated with periodontal disease. There are 16 reported cases of non-oral C. rectus invasive soft-tissue infections, of which only one reports of osteolytic changes. In our patient, it is hypothesised that contiguous spread of periodontal infection with C. rectus seeded infection to the orbit. C. rectus infection is a rare but significant pathogen that should be considered as the etiologic factor in a patient presenting with an orbital lesion and bony changes, particularly on a background of poor dentition.

Nasal turbinate mucosal graft for management of contracted anophthalmic socket.

A 67-year-old Caucasian male presented with severe contraction of socket lining 8 years after enucleation, dermis fat graft and successful ocular prosthesis fitting. Following two failed attempts at using amniotic membrane grafts to reform the socket lining, a total socket reconstruction was attempted using a novel nasal turbinate mucosal graft technique. This was performed in a staged fashion with lower fornix reconstruction followed by upper fornix reconstruction 3 months later. The patient was stable at 12 months review, with a satisfactory cosmetic outcome. Nasal turbinate mucosa was used as it was surgically accessible, provided natural socket lubrication due to its mucosal surface, and avoided oral mucosa and its associated morbidity. This case report suggests that nasal turbinate mucosa is a suitable autologous grafting material for total socket reconstruction in contracted anophthalmic sockets.

Clinical and Radiological Features of Intramuscular Orbital Amyloidosis: A Case Series and Literature Review.

PURPOSE: Orbital amyloidosis of the extraocular muscles (EOMs) is a rare condition, and its clinicoradiological features are not well elucidated. This study describes the characteristic clinical signs, MRI features, and potential treatment options. METHODS: Retrospective multicenter case series and literature review of EOM amyloidosis. RESULTS: Five cases were identified for inclusion. Common clinical findings were diplopia, ophthalmoplegia, and proptosis. Systemic amyloidosis was more likely to present with multiple muscle involvement, but no particular pattern was observed with localized disease. On MRI, amyloid deposition was characterized as a heterogeneous intramuscular mass with T2 hypointensity and post contrast enhancement. Management is dependent on the extent of disease and functional impairment; options include surgical debulking and radiation therapy. CONCLUSION: EOM amyloidosis is uncommon. The combination of clinical and radiologic findings described in this study should lead to its clinical suspicion.

Hughes flap in the management of lower lid retraction.

PURPOSE: We present a retrospective case series on the use of Hughes flap in managing acquired cicatricial lower lid retraction. METHODS: This was a multicentre, retrospective case series. Data was collected from medical records across different sites within Australia (Adelaide, Melbourne, and Sydney) and New Zealand (Hamilton). RESULTS: Fourteen patients were identified. The aetiology of cicatricial lower lid retraction included previous lid lesion excision and reconstruction, eyelid trauma, orbital fracture repair, orbital radiotherapy, and lateral canthal dystopia from previous lid surgeries. 4/14 (29%) cases had undergone other surgery to correct the retraction prior to the Hughes flap. Pre-operative lagophthalmos due to lower lid retraction was noted in 11/14 (79%) cases with a median 2 mm (range: 1-5 mm). Exposure keratopathy was present in 7/14 (50%) cases. There were no peri-operative complications during Hughes flap reconstruction. One patient had post-operative upper eyelid retraction that did not require any further intervention. One patient had persistent lagophthalmos and exposure keratopathy that is being managed conservatively. One patient had wound dehiscence and further lid retraction following flap division, which required further surgery. Median length of follow-up was 15 months (range: 0.5-84 months). At final review, improvement or resolution of symptoms was seen in 13/14 (93%) cases. CONCLUSIONS: A Hughes flap is an effective surgical technique for the management of cicatricial lower lid retraction.

A practical guide on the use of imiquimod cream to treat lentigo maligna.

Lentigo maligna (LM) is a common in situ melanoma subtype arising on chronically sun-damaged skin and mostly affects the head and neck region. Localisation in cosmetically sensitive areas, difficulty to obtain wide resection margins and advanced patient age/comorbidities have encouraged investigation of less invasive therapeutic strategies than surgery in managing complex cases of LM. Radiotherapy and imiquimod have emerged as alternative treatment options in this context. The treatment of LM with imiquimod cream can be challenging due to the nature of the disease including its often large size, variegated appearance, involvement of adnexal structures, poorly defined peripheral edge and frequent localisation close to sensitive structures such as the eyes and lips, and elderly patients with multiple comorbidities. Prolonged and unpredictable inflammatory reaction and side effects and compliance with a patient-delivered therapy can also be challenging. In the literature to date, studies evaluating the use of imiquimod to treat LM have utilised varying methodologies and provided short follow-up and these limitations have impaired the development of clear guidelines for dosage and management of side effects. Based on our multidisciplinary experience and review of the literature, we propose practical clinical strategies for the use of imiquimod for treating LM, detailing optimal administration procedures in various clinical scenarios and long-term management, with the aim of facilitating optimal patient outcomes.

Role of Optic Nerve Sheath Fenestration in Inflammatory Pachymeningitis Causing Visual Impairment.

Eye disease due to pachymeningitis caused by immunoglobulin G4-related disease (IgG4-RD) is a rare occurrence. Here, the authors report a unique case of a patient presenting with visual loss from raised intracerebral pressure from pachymeningitis most likely related to IgG4-RD. The patient was treated with acetazolamide and steroids, and an optic nerve sheath fenestration was performed to successfully save the patients vision.

Sleep phenotype in the Townes mouse model of sickle cell disease.

PURPOSE: Patients with sickle cell disease (SCD) regularly experience abnormal sleep, characterized by frequent arousals and reduced total sleep time. However, obstructive sleep apnea syndrome (OSAS) is a common comorbidity of SCD, making it unclear whether the disease per se is impacting sleep, or sleep disruption is secondary to the presence of OSAS. Thus, we assessed sleep, independent of OSAS, using a mouse model of SCD. METHODS: Sleep was compared between 10-to-12-week-old Townes knockout-transgenic mice with the sickle cell phenotype SS (n = 6) and Townes mice with sickle cell trait AS (n = 6; control). The mice underwent chronic polysomnographic electrode implantation (4EEG/2EMG) to assess sleep architecture. RESULTS: The SS mice had significantly lower hemoglobin concentration compared to control AS mice (7.3 ± 1.3 vs. 12.9 ± 1.7 g/dL; p < 0.01), consistent with the expected SCD phenotype. SS mice exhibited significantly decreased total NREM sleep time (45.0 ± 0.7 vs. 53.0 ± 1.3% 24 h sleep time; p < 0.01), but no change in total REM sleep time compared to the AS mice. The SS mice took longer to resume sleep after a wake period compared to the AS mice (3.2 ± 0.3 min vs. 1.9 ± 0.2 min; p < 0.05). Unexpectedly, SS mice experienced fewer arousals compared to AS mice (19.0 ± 0.9 vs. 23.3 ± 2.1 arousals/h of sleep; p = 0.031). CONCLUSIONS: The presence of decreased total NREM sleep associated with reduced arousals, in the absence of OSAS, suggests a distinctive underlying sleep phenotype in a mouse model of SCD.

Orbital Apex Syndrome in Pseudomonas Sinusitis After Functional Endoscopic Sinus Surgery.

Sinusitis complicated by orbital apex syndrome is rare. The majority of cases are due to mucormycosis in immunocompromised patients. The authors report a case of orbital apex syndrome secondary to Pseudomonas sinusitis. The patient was a 79-year-old Type 2 diabetic woman who underwent left-sided functional endoscopic sinus surgery. Three months postprocedure, she presented with a 3-week history of gradual vision loss and constant temporal headache on the ipsilateral side. Examination revealed light perception vision, a relative afferent pupillary defect, absent corneal reflex, and disc pallor. Imaging revealed opacification of the posterior ethmoidal air cells extending to involve the orbital apex. Urgent orbital and sinonasal decompression was performed. Intraoperative specimens grew Pseudomonas aeruginosa. The patient was treated with liposomal amphotericin B, posaconazole, and piperacillin/tazobactam. The patient recovered with no visual sequelae. Pseudomonas is an important mimicker to consider in sinusitis causing orbital apex syndrome, and it may occur following functional endoscopic sinus surgery.

Lower eyelid entropion repair with retractor mobilization and insertion onto the anterior surface of the tarsal plate.

PURPOSE: To evaluate a modified surgical technique for the correction of lower eyelid involutional entropion in terms of recurrence rate and relief of symptoms. METHOD: A prospective series of 67 consecutive eyelids in 55 patients who underwent lower eyelid entropion repair using a modified surgical approach not previously published in the literature to the authors' knowledge. The surgical technique, via a skin crease incision, involves disinsertion of the lower eyelid retractors from the tarsus and conjunctiva and suturing the retractors onto the anterior surface of the tarsal plate. Standard excision of redundant lower lid skin and orbicularis muscle was also performed as well as lateral canthal tendon repair. All procedures were performed by a single surgeon (B. A. O'donnell). This study adhered to the principles of the Declaration of Helsinki. RESULTS: There were 67 eyelids with a minimum of 12 months follow-up: of these only 1 patient (1%) required revision surgery (average follow-up 24 months). Four other eyelids had persistent or recurrent irritation (6%) not suggestive of, or secondary to, recurrence. Seven eyelids (10%) had undergone previous operative repair with no further recurrence following their most recent surgery. CONCLUSION: Disinsertion and reattachment of the retractors to the anterior tarsal plate in lower eyelid entropion repair is an effective surgical technique to improve lower eyelid stability. Although minimum follow-up is 12 months and average follow-up 24 months, longer follow-up would further evaluate this technique.

Non-Exenteration Management of Sino-Orbital Fungal Disease.

PURPOSE: To describe the non-exenteration management of sino-orbital fungal infection, a life-threatening condition for which orbital exenteration is generally considered a first-line treatment. METHODS: A retrospective case series is presented of 7 orbits in 6 consecutive patients admitted and treated at 2 major metropolitan tertiary teaching hospitals in Sydney, New South Wales, Australia. RESULTS: Seven orbits in 6 consecutive patients with sino-orbital fungal infection were treated conservatively with surgical debridement and intravenous antifungal agents. Four patients were immunosuppressed and the other 2 patients were otherwise healthy. All presented with pain, proptosis, or loss of vision. Causative organisms found were Mucormycoses, Aspergillus, and Scedosporium prolificans. Exenteration was avoided in all patients as part of their planned management and 5 patients, including 1 with bilateral disease, survived their disease without exenteration. Medical treatment included intravenous liposomal amphotericin B or voriconazole. A single immunosuppressed patient deteriorated and as a last resort, exenteration was performed, but this made no difference to his clinical course and in retrospect could have been avoided as he died of multiple cerebral metastases diagnosed shortly after his deterioration. CONCLUSION: The authors recommend that patients with sino-orbital fungal disease preferably be treated conservatively, without orbital exenteration.

Periocular Squamous Cell Carcinoma: TNM Staging and Recurrence.

PURPOSE: To analyze the TNM stage, management, and recurrence rates of patients with histologically confirmed squamous cell carcinoma (SCC) of the eyelid. DESIGN: Retrospective case series from 3 Australian centers. PARTICIPANTS: A total of 254 cases of eyelid SCC from 254 patients (median age, 73 years; range, 28-102 years; 159 were male). METHODS: Tumors were staged according to The American Joint Committee on Cancer 7th edition TNM criteria for eyelid carcinoma. MAIN OUTCOME MEASURES: Outcomes and recurrence rates according to TNM stage at presentation. RESULTS: A total of 25 cases (9.8%) were recurrent tumors. TNM classifications were as follows: T1N0M0, 74 patients (29.1%); T2aN0M0, 92 patients (36.2%); T2bN0M0, 50 patients (19.7%); T3aN0M0, 31 patients (12.2%); T3bN0M0, 5 patients (2.0%); T2bN0M1, 1 patient (0.4%); and T3bN1M1, 1 patient (0.4%). Perineural invasion (PNI) was present histologically in 8.3% of cases. Treatment modalities included Mohs microsurgery (31.1%), wide local excision (WLE) with paraffin section control (21.7%), WLE with frozen-section control (19.3%), and excision without margin control (24.4%). Three cases did not receive treatment. Median follow-up was 40 months (range, <1-132 months). Local recurrence occurred in 17 treated patients (6.8%). The recurrence rate was 5.3% (12/226 patients) for primary tumors and 20% (5/25 patients) for recurrent tumors (P = 0.019). Four patients (1.6%) died of their disease during follow-up. Higher T stage was significantly associated with both PNI (P = 0.035) and local recurrence (P < 0.001). We could not identify a T-stage threshold below which there was no risk of recurrence, as evidenced by 3 T1 primary tumors that recurred. CONCLUSIONS: Higher T stage was significantly associated with local recurrence, and recurrent tumors had a 4-fold increased risk of further recurrence compared with primary tumors. Therefore, it may be reasonable to consider sentinel lymph node biopsy or close nodal surveillance and follow-up for patients with recurrent or high T-stage tumors. Of note, we could not identify a T-stage threshold below which there was no risk of recurrences; therefore, clinicians should be aware of the potential for low T-stage tumors to recur.

Squamous Cell Carcinoma of the Caruncle.

Squamous cell carcinoma (SCC) of the lacrimal caruncle is a rare entity. The authors report the management and outcomes of 3 cases of caruncle SCC. Case 1 underwent wide margin surgical excision with adjuvant topical chemotherapy for a poorly differentiated SCC. He later developed regional lymph node metastasis and required modified radical neck dissection. Case 2 underwent wide margin surgical excision with cryotherapy and adjuvant topical chemotherapy for an invasive moderately differentiated SCC. She later developed a recurrence and underwent orbital exenteration. Case 3 was a moderately differentiated SCC treated with wide margin excision alone and had no recurrence during 5-year follow up. Careful surveillance of caruncle SCC is required, given the observed propensity for local recurrence and/or regional metastasis.

The natural history of orbital cavernous hemangiomas.

PURPOSE: Orbital cavernous hemangiomas (OCH) often present as incidentally found asymptomatic lesions, and it has been reported that such lesions do not grow. The authors' own experience has been different. They describe the natural history of OCH in a cohort with asymptomatic incidentally found lesions and compare them with patients with symptomatic OCH to identify any characteristics that might predict those which enlarge and produce symptoms. METHODS: This was a retrospective comparative case series from 3 tertiary referral orbital centers in Australia. There were 104 patients with OCH, 31 of whom presented with incidentally found lesions. For patients with incidental lesions, only those with a minimum follow-up period of 12 months were included. Outcome measures included demographic features, clinical features at presentation and during follow up, anatomical location of the OCH, change in maximal linear dimensions of the OCH over time, follow-up period, and requirement for treatment in the follow-up period. RESULTS: There were 104 patients included in the study. Thirty-one had an asymptomatic, incidental OCH on imaging performed for other indications. Seventy-nine patients had treatment, and 11 of these had presented with an incidental, asymptomatic OCH that enlarged and produced symptoms or new clinical findings. In the 20 other patients with incidentally found OCH, there was no or minimal change in the follow-up period of 1.2 to 20 years (mean 5.8 years, standard deviation [SD] 4.6 years). None of the factors analyzed in the study were able to predict those patients with an incidental OCH that enlarged and required treatment. Patients with incidental lesions that did not grow over several years did not change over much longer periods of time. When considering all patients with OCH, both incidentally diagnosed and symptomatic, there were 4 factors that predicted whether someone with an OCH would require treatment. These were maximal linear dimension, male gender, extraconal location (increased risk of requiring treatment), and being an incidental finding (decreased risk of requiring treatment). CONCLUSIONS: OCH may present as an incidental finding. A significant number will grow over time and require treatment. The authors could not identify any factor that would predict those lesions that enlarge over time and require treatment. If an incidental OCH does not change over several years, it is very unlikely to do so in longer periods of follow up.

The anatomical location and laterality of orbital cavernous haemangiomas.

PURPOSE: To determine the anatomical location and laterality of orbital cavernous haemangiomas (OCH). DESIGN: Retrospective case series. METHODS: The records of 104 patients with OCH were analyzed. MAIN OUTCOME MEASURES: The anatomical location of each OCH defined by the location of a point at the centre of the lesion, and its laterality. RESULTS: There were 104 patients included in the study. No patient had more than one lesion. Sixteen (15.4%) were located in the anterior third of the orbit, 74 (71.2%) were in the middle third, and 14 (13.5%) in the posterior third. In the middle third, 10 of 74 (13.5%) were extraconal and 64 intraconal (86.5%), with 30 of 64 (46.9%) middle third intraconal lesions lying lateral to the optic nerve. Of 104 lesions, 56 (53.8%) were left sided, showing a trend towards a predilection for the left side (p = 0.065). If data from other published series which included data on laterality is added to our own data and analysed, 270 of 468 (57.7%) OCH occurred in the left orbit (p < 0.005). CONCLUSIONS: OCH may occur at almost any location within the orbit. The commonest location is the middle third of the orbit, in the intraconal space lateral to the optic nerve. This may reflect an origin of these lesions from the arterial side of the circulation, as there are more small arteries in the intraconal space lateral to the optic nerve than in other locations. A predilection for the left orbit remains unexplained.

The use of the paramedian forehead flap alone or in combination with other techniques in the reconstruction of periocular defects and orbital exenterations.

PURPOSE: The paramedian forehead flap (PMFF) is a reconstructive option for large eyelid defects and orbital exenterations. We report a series of cases where PMFF reconstruction was carried out at various institutions in Australia. METHODS: This study was a multi-centre, retrospective, non-comparative case series investigating the clinical outcomes of the PMFF for reconstructing periocular defects and orbital exenterations. RESULTS: This case series describes twenty-seven patients (Female = 15, Male = 12), operated between 1991 to 2019, with a median age of 81 years (range: 45-93 years). Defect locations involved combinations of the medial canthus (16/27, 59.3%), upper eyelids (7/27, 25.9%), lower eyelid (4/27, 14.8%), both upper and lower eyelids (5/27, 18.5%), and orbital (7/27, 25.9%). There were no cases of flap necrosis. Minor post-operative complications were observed in ten patients with the most common being lagophthalmos. Median duration of follow-up was 17months (Range: 2months- 23years). CONCLUSIONS: The PMFF is a versatile reconstructive tool for a range of periocular defects and orbital exenterations with minor post-operative complications.

Globe-sparing surgery for medial canthal Basal cell carcinoma with anterior orbital invasion.

PURPOSE: To describe a case series of patients with anterior orbital invasion by medial canthal basal cell carcinoma (BCC) managed with non-exenterating surgery. DESIGN: International, multicenter, retrospective, noncomparative, consecutive case series. PARTICIPANTS: Twenty patients identified from the individual institutions' databases with histologically confirmed orbital invasion by periocular BCC. METHODS: Examination of charts, relevant imaging, and histopathologic data. MAIN OUTCOME MEASURES: Demographics; clinical characteristics and radiologic features; histopathologic features; surgical techniques for excision, reconstruction, and subsequent procedures; complications; visual acuity; and recurrence. RESULTS: Twenty patients were identified. Twelve of 20 patients (60%) had recurrent BCCs, with 1 patient having had prior radiotherapy for previously incomplete excision. Eighteen of 20 patients (90%) had a palpable mass, 16 of 20 patients (80%) had clinical involvement of the nasolacrimal system, and 1 of 20 patients (5%) had limited extraocular movements. Preoperative radiologic evidence of orbital invasion was found in 10 of 20 patients (50%). Histologic evidence of orbital invasion was present in every patient, the subtypes being infiltrative (9/20, 45%), nodular (4/20, 20%), micronodular (2/20, 10%), multifocal (1/20, 5%), and mixed (4/20, 20%); extratumoral perineural invasion was present in 1 patient (5%). Final margins were clear in 18 of 20 patients (90%), positive in 1 of 20 patients (5%), and unclear in 1 of 20 patients (5%). Reconstruction was by direct closure in 1 patient and by a variety of standard oculoplastic flaps and grafts in 19 of 20 patients (95%). Twelve of 20 patients (60%) had postoperative extraocular muscle movement restriction, and 15 of 20 patients (75%) had epiphora. Subsequent revision procedures were needed in 12 of 20 patients (60%), including insertion of a lacrimal bypass tube and revision of medial canthal position. At a mean follow-up of 38 months, 18 of 20 patients (90%) were still alive (2 deaths due to other causes) with 1 recurrence (exenterated). Postoperative visual acuity was within 2 Snellen lines of preoperative visual acuity in 17 of 20 patients (85%). CONCLUSIONS: With careful planning and margin control, conservative surgery in this highly selected group proved possible with a low rate of disease recurrence, albeit with a relatively short follow-up. Postoperative complications, such as epiphora and ophthalmoplegia, were largely expected; most patients underwent subsequent revision procedures to address these and other complications. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.