Search

Two patients had persistent compressive dysthyroid optic neuropathy after decompression of the medial orbital wall and floor. In both cases, there was ~3 mm of unresected lamina papyracea anterior to the Annulus of Zinn, and removal of this residual bone led to resolution of the neuropathy. These illustrative cases suggest that, in some patients, even small amounts of residual crowding at the orbital apex can critically embarrass optic nerve perfusion, with resulting continued ischemic optic neuropathy.

The purpose of this study was to evaluate ophthalmic features and outcomes for patients who present with sinonasal mucoceles expanding into the orbit.Retrospective chart review for patients seen in a specialist orbital clinic over 25 years, with a review of demographics, clinical characteristics, imaging features, and outcomes after treatment.Sixty patients (38 males; 63%) presented at a mean age of 51 years (range 3-89). Symptom duration was extremely variable (1 week-15 years) with a mean of 14 months and median of 4 months-the commonest being periorbital swelling (33/62 orbits) or ache (20 orbits), proptosis (30 orbits), and diplopia (19/50 patients without visual impairment; 38%). Of mucoceles affecting orbital function, 60/62 (97%) were of frontal and/or ethmoid sinus origin, and probable predispositions included past trauma (12/62 orbits) or prior ipsilateral sinus surgery (14 orbits). Forty-two of the 59 (71%) patients who underwent sinus surgery had complete resolution of symptoms within 6 months. Of 10 orbits presenting with moderate to severe visual loss (Snellen 20/60 or worse), the acuity improved in 7/10 (70%) of these after sinus surgery. Although 12/62 (19%) of eyes presented with epiphora, this persisted after sinus surgery in 9 orbits, and areas of occult malignant change were found in 3/9 (33%).Sinus mucoceles expanding into the orbit can cause significant globe displacement, dysmotility, or visual impairment. Symptoms usually resolve within several months after functional sinus surgery, but where symptoms persist (particularly periorbital swelling or epiphora) this might indicate underlying secondary causes, such as occult malignancy.

To estimate the proportion of de novo lacrimal gland pleomorphic adenomas (PAs) and carcinomas expleomorphic adenomas (CEPAs), together with age at presentation and first symptom. Conjectural models of tumor growth are considered.Retrospective review of patients with orbital lobe PA or CEPA. The presenting age was examined for conformation to a Gaussian distribution and the cumulative distribution function derived for both tumor types. The risk of CEPA with age was estimated by logistic regression.About one-sixth (27/172; 16%) of these primary orbital lobe tumors were CEPAs, with 145 PAs (76/145 male; 52%) and 27 CEPAs (12/27 male; 44%). The mean presenting age for PAs was 48.3 years (median 47.7; range 11-84 years) and 57.7 years for CEPAs (median 61.2, range 27-91 years) ( p = 0.0062), and the standard deviations for each group are almost identical (16.3 for PAs, 15.9 for CEPAs; p = 0.92). Five (3.4%) PAs and 1 (3.7%) CEPA were asymptomatic: otherwise, the median symptom duration was 24 months for both PAs and CEPAs ( U test: p = 0.65). The odds of CEPA rises significantly with age, increasing 1.04-fold annually ( p = 0.0079).The almost identical measures of dispersion for the presenting ages of PA and CEPA suggests that, once malignant transformation occurs, there might be a relatively constant period before it is evident. CEPAs present about a decade after PAs, this unexpectedly later presentation for the malignancy possibly being explained by a gradual replacement of the PA by the newly arising carcinoma within the preceding benign tumor.

To evaluate the survival benefit of orbital exenteration in periocular malignancy, taking account of preoperative intent.Patients undergoing exenteration had retrospective chart review for demographics, clinical features, radiology, histology, and outcome. Based on systemic tumor status, the patient was either "Class I" (with absent or well-controlled systemic disease) or "Class II" (incurable active metastatic disease), and based on the extent of orbital disease and exenteration intent , was classed as either "Group A" (locally curative) or "Group B" (locally palliative).One hundred thirty-three patients (78 females; 59%) underwent exenteration at an average age of 61 years (median 64; range 5-91) for sebaceous, squamous and basal cell carcinomas, or for melanoma (22%, 19%, 11%, and 28%, respectively). There were 20% systemically incurable patients (26/133; Class II), and incurable local disease ("Group B") in 5% (5/107) of Class I and 15% (4/26) Class II patients. The overall survival (OS) was 88% at 12 months, 57% at 5 years, and 41% at 10 years, prognosis being worse with age more than 70 years ( p = 0.005), prior local radiotherapy ( p = 0.005) or positive resection margins ( p = 0.002). The mean OS for Type IA exenteration (145 months; 95% CI 122-168) was significantly different to 50 months for Type IB (95% CI 22-79; p = 0.02); likewise, OS for Type IIA procedures (31 months; 95% CI 11-51) was different to Type IIB (19 months; 95% CI 2-36) ( p = 0.001).Exenteration confers a significant survival with advanced periocular malignancies, even in patients with uncontrollable systemic disease, or where the local disease is deemed incurable.

To evaluate the clinical features of developmental cysts of the orbit.Retrospective study of patients who had excision of cysts between 1992 and 2020.Three hundred and 58 patients (189 male; 53%) with orbital developmental cysts were identified, all being unilateral. Age at surgery varied from birth to 77 years (mean 17, median 18 years) and the average symptom duration was 5 years (median 18 months; range 1 day-50 years). The commonest presenting features were a peribulbar lump or upper lid swelling, followed by proptosis, pain, diplopia and reduced vision. Most patients (82%) had a palpable mass, with epidermoids, sebaceous dermoids and keratinised dermoids commonly affecting the superotemporal quadrant, and conjunctiva-containing cysts usually being biased to a medial location. Cysts were lined by keratinised epithelium with dermal structures (224/358; 63%), non-keratinised epithelium with dermal structures (69/358; 19%), epidermis without identifiable dermal structures (19/358 'epidermoids'; 5%), conjunctiva (12/358; 3%), respiratory epithelium (4/358; 1%), or mixed dermal and conjunctival epithelia (30/358 'dermo-conjunctival' cysts; 8%). Overall, two-thirds (242/358; 66%) had histological evidence of chronic intramural inflammation, and a half of cysts showed granuloma formation (178/358 cysts). Chronic inflammation was less common with conjunctival cysts (54%, 7/12 patients) and none showed granuloma formation.Developmental cysts of the orbit vary from the relatively common dermoid cysts to the extremely rare respiratory epithelial-lined cysts. Respiratory cysts, being deeper, may present late in life and cysts containing conjunctival epithelium tend to be less inflamed and typically favour the superonasal quadrant.

Chronic granulomatous inflammation occurs rarely alongside pleomorphic adenomas of the major salivary glands but would not appear to have been reported with lacrimal gland adenomas. We describe the clinical features, imaging and histopathology for 4 patients (3 female) who had granulomatous inflammation alongside lacrimal gland adenomas-the patients being with age 39, 44, 48, and 53 years at time of surgery. One patient had an asymptomatic lesion found on imaging, and the other 3 had symptoms for between 3 years and several decades. Conjecturally, this rare phenomenon might arise from an inflammatory response to leakage of secretions from the ductular components of the glands.

PURPOSE To examine the necessity for lateral osteotomy for intact removal of tumors within the orbital lobe of the lacrimal gland. METHODS Patients who, between 1992 and 2020, were scheduled for intact excision of well-defined masses within the orbital lobe of the lacrimal gland were identified from an orbital database. A retrospective review of the clinical records was performed and details of the tumor size obtained from histopathological reports. The first patients in the series had lateral osteotomy, whereas later, there was a shift toward osteotomy-free tumor excision through a skin-crease incision. The latter approach required isolation of the gland's orbital lobe on an intact sheet of periosteum, an anterior "flip" of the mass (out of the orbit, past the orbital rim) "hinged" at the isthmus between the orbital and palpebral lobes, and finally a division of the isthmus to release the intact mass. RESULTS Clinical details were available for 100 patients (50 male), with 56 tumors removed using lateral osteotomy and 44 without. The mean age at surgery was 50.3 years with osteotomy (median, 51.8; range, 19-85 years) and 51.1 years without (median, 50.3; range, 11-81 years; P = 0.81). There were no postoperative complications in either group. Although the range of tumor volumes was similar in the two groups (1.6-15.0 ml with osteotomy; 0.9-15.1 ml without), the average volume was somewhat greater in those having osteotomy (mean, 6.4 ml) as compared with those without (mean, 4.6 ml; P = 0.0016). Inadvertent rupture of the tumor occurred once in each group, with tumors of similar small size. CONCLUSIONS In many cases, large masses within the orbital lobe of the lacrimal gland can be safely removed intact, using the described flip technique without the need for lateral wall osteotomy.

Piecemeal excision of dermoid cysts carries the risk of implanting epithelial fragments into orbital fat, which is well recognized to continue secreting oily debris, inciting chronic, often granulomatous inflammation. The authors present the clinical and histological details for two patients with persistent lipogranulomatous inflammation for years after piecemeal excision of deep orbital dermoid cysts, in the absence of any residual epithelium. The importance of copious saline lavage - to 'float-out" and reduce microscopic lipid droplets - is also emphasised.

The aim of this study was to determine the incidence and recovery rate for pupillary abnormalities after excision of orbital cavernous venous malformations (OCVMs).Retrospective case-note and imaging review for patients with OCVMs, with an estimation of odds ratios (ORs) in relation to the position of the mass and surgical approach.The 287 included patients (176 female; 61%) presented at a mean age of 46.5 years: 243/287 (85%) OCVMs were intraconal, 253/287 (88%) located in the posterior two-thirds of the orbit, and 29/287 (11%) wedged tightly in the apex. Pupillary changes-varying from minor sectoral paresis (only evident on slit-lamp examination) to a marked mydriasis-were noted in 21% (60/287) of patients and were strongly associated with removal of intraconal (60/243 [25%]; p0.001), apical (14/29 [48%]; OR, 4.3; p0.001), inferior or inferotemporal (41/105 [39%]; OR, 5.5; p0.001) masses. Lateral approaches-either with (23/129; 18%) or without (30/71; 42%) osteotomy-were associated with 88% (53/60) of all abnormalities (OR, 4.1; p0.001). Where known, the abnormalities improved in 43/50 (86%), although 15/43 (35%) had a persistent tonic pupil.Pupillary abnormalities after excision of OCVMs can occur in up to a quarter of intraconal lesions-particularly those located inferotemporally or inferiorly-and in about a half of apical lesions. Lateral or inferolateral orbital approaches that disrupt the inferior intraconal fat appear to be associated with a higher risk. Most changes resolve or improve markedly, although a third of those affected may have a persistent tonic pupil.

AIM To describe the characteristics of patients presenting with congenital orbital cysts containing epithelia of both cutaneous phenotype-that is, epidermis with or without keratin, and at least one dermal structure (sweat gland or pilosebaceous unit)-and conjunctival phenotype (with goblet cells); these cysts with mixed lining are termed "dermo-conjunctival" cysts. PATIENTS AND METHODS Review of clinical records for patients having dermo-conjunctival cysts that were treated between 1997 and 2017; patients with cysts of solely conjunctival or solely cutaneous origin were omitted. Data recorded included gender, age at presentation, laterality, orbital location, ophthalmic features, surgical and radiological data, and light microscopic findings. RESULTS Of 241 patients with congenital orbital cysts, 22 (9%) contained both cutaneous and conjunctival epithelium; unlike the relatively common congenital cysts lined with solely cutaneous epithelia, these dermo-conjunctival cysts typically occupied the superonasal or nasal quadrants of the orbit (p < 0.000001). Fifteen (68%) of the 22 patients were male, and the group presented at a median age of 22 years (range 8-51 years), with symptoms for a median duration of 5 years (range 1 month-33 years). Fourteen (64%) had noted an orbital mass, 3 (14%) had inflammatory pain, and 1/22 (4%) had reduced acuity. Globe displacement was axial in 7 patients (32%) and nonaxial in 13 (59%), and ocular motility was restricted in 4/22 (18%). Of 17 patients with imaging, 9 (53%) had bone expansion, and the cyst was intimately related to the trochlea in 10 (59%). By definition, all cysts contained conjunctival epithelium with goblet cells: hair shafts were present in 13/22 (59%) specimens, sebaceous units in 18 (82%), sweat glands in 6 (27%), and keratinized epithelium was present in 8 (36%). Fourteen (63%) of cysts had mild chronic inflammation within the cyst wall, and granulomas were present in 8 (36%). CONCLUSION Congenital dermo-conjunctival orbital cysts are rare and favor a medial location-this suggesting an origin from sequestered caruncular tissues, the only postseptal source of both dermal and conjunctival structures. Unlike pure cutaneous cysts that typically present as superficial masses in childhood, dermo-conjunctival cysts are often postseptal and present in adults.

To report the characteristics and long-term outcomes of patients presenting with carcinoma ex-pleomorphic adenoma (CXPA) or malignant mixed tumor of the lacrimal gland.A retrospective case-note review for patients with CXPA, seen at Moorfields Eye Hospital between 1985 and 2018, was performed for demographics, presentation, imaging, histopathology, management, and outcome.Twenty-six patients (11 male; 42%) presented at a mean age of 46 years (median 46.4; range 24-81), with average symptom(s) duration of 67 months (median 24 months; range 6 weeks-53 years). The commonest symptoms were proptosis (13 patients; 50%), orbital ache or pain (11/26, 42%), having noted a lump in the upper eyelid (8/26, 31%), and diplopia (7/26, 27%). About 90% of patients had nonaxial globe displacement, over a half had a palpable mass and 3-quarters had reduced motility. Adenocarcinoma was the commonest malignant component of the tumor, occurring in 16 (62%) of the patients, followed by adenoid cystic carcinoma in 5 patients (19%); 10/26 (28%) tumors showed significant invasion on histological examination. A quarter of the patients had tumor-related death at an average of 2.8 years after diagnosis (median 2.5; range 1.5-4.4 years), and the overall probability of survival for the whole cohort was 63% at both 5 years and 10 years after treatment. The prognosis for CXPA was significantly worse with a history of prior incompletely excised pleomorphic adenoma (p0.001), a markedly invasive malignant component (p = 0.001), evidence of perineural invasion (p = 0.042), and an American Joint Committee on Cancer tumor size of T3 or greater (p = 0.024).Intact excision of CXPAs that do not display histological evidence of extension beyond the tumor pseudocapsule have a good outcome, similar to that for intact excision of benign pleomorphic adenomas. In contrast, our patients with markedly invasive CXPA had a tendency to local recurrence or distant metastasis, and tumor-related death occurred in about two-thirds.

This article provides the reader with an outline of the anatomy and physiology of the lacrimal system and illustrates how a variety of pathologies within this system can result in the development of a watery eye. It describes the role of the nurse consultant in the diagnosis and management of patients with watery eye in the lacrimal clinic, and how the training programme for the development of such skills was implemented. Following training, within the realms of an established pilot study, the nurse consultant began to implement her skills in a newly established nurse-led lacrimal clinic. Patients seen in the lacrimal clinic would previously have been assessed and managed by a doctor. To confirm the accuracy of this approach, an audit was undertaken comparing the nurse-led diagnosis and management plans with that of an oculoplastic doctor. In addition, patient waiting times in the clinic and patient satisfaction were assessed, as important indicators of quality of care.

To compare the characteristics for symptomatic and asymptomatic orbital cavernous venous malformations (OCVMs).A retrospective case-note review for patients with OCVMs was performed for demographics and presenting features.Four hundred and fourteen patients (258 female; 62%) presented at a mean age of 49.4 years and all were unilateral. Ninety-two (22%) asymptomatic OCVMs were found a decade later than 322 symptomatic patients (57.8 vs. 47.0 years; p0.001). Patients with symptomatic OCVMs had an average symptom duration of 3.3 years with the commonest being altered vision (57%), proptosis (55%), orbital ache (21%), and diplopia (13%). Visual obscurations were reported by 11% of symptomatic patients, whereas 30% had a hyperopic shift.Mean LogMAR was 0.39 for symptomatic OCVMs and 0.1 for asymptomatic ( p0.001), and a relative afferent pupillary defect was detected in 38% symptomatic and 7% asymptomatic patients ( p0.001). Mean relative exophthalmos was 3.9 mm in symptomatic patients and 1.5 mm in asymptomatic ( p0.001), but there was no correlation between age and exophthalmos. Symptomatic lesions had higher rates of nonaxial displacement (30% vs. 7%; p0.00001), more palpable masses (17% vs. 2%; p = 0.0001), and more impairment of motility (29% vs. 10%; p = 0.0024). Fundal abnormalities were noted in 63% of patients with symptomatic OCVMs, as compared to 26% of asymptomatic ( p0.0001).Symptomatic OCVMs may present with reduced visual function, either due to the globe or optic nerve compression, or proptosis. Asymptomatic (presumed) OCVMs- representing 22% of all OCVMs-are usually found a decade later than symptomatic lesions, and a few may have only mild functional impairment.

PURPOSE To evaluate periorbital volume changes, using quantitative photogrammetric stereoimaging, after bony orbital decompression for thyroid eye disease (TED) and compare this to changes in Hertel exophthalmometry. Tissue volumes of the upper and lower eyelids were also assessed independently. DESIGN Prospective, nonrandomized, nonconsecutive series of patients. SUBJECTS Adults with inactive TED who had elected to undergo bony orbital decompression at Moorfields Eye Hospital between 2015 and 2017. METHODS With their eyes gently closed, patients with inactive TED underwent imaging using the VECTRA M3 system (Canfields Imaging, Fairfield, NJ), both before and at least 3 months after orbital decompression. Proptosis was assessed by Hertel exophthalmometry. Using the manufacturer's software, changes in periorbital volumes between the preoperative and postoperative images were calculated for the upper and lower eyelids. The Pearson product-moment correlation coefficient was used to assess the linear relationship between changes in periorbital volume and exophthalmometry. MAIN OUTCOME MEASURES Change in stereoimaging volumes of the upper and lower eyelids, and clinical proptosis as measured with Hertel exophthalmometry. RESULTS Thirty-three patients (11 males; 33%) underwent lateral decompression (39 orbits in 26 patients), lateral decompression with complete ethmoidectomy (3 orbits in 3 patients), combined decompression of lateral wall, medial wall, and medial half of floor (10 orbits in 6 patients), or bilateral lateral, medial, and complete floor decompression (1 patient). The corresponding average volumetric changes were 1.74 ml (median 1.64; range 0.39-3.73 ml), 3.38 ml (median 3.38, range 1.89-4.88 ml), 4.05 ml (median 3.53, range 1.72-6.43 ml), and 4.52 ml (range 4.36-4.68 ml), respectively. Similarly, the average reduction in proptosis was 3.6 mm (median 3; range 1.5-7 mm), 5.3 mm (median 5; range 5-6 mm), 7.4 mm (7.5; range 6-9 mm), and 9 mm (range 8-10 mm). Periorbital volume changes were related to the reduction in exophthalmometry (r = 0.713, p < 0.0001), and reduction of lower eyelid volume did not increase further with more than two-wall decompression. CONCLUSION Graded orbital decompression reduces both proptosis and the upper and lower eyelid tissue volumes, the eyelid changes being likely to influence decisions about future restorative eyelid surgery. The significant changes in eyelid profiles underlines the established principle of thyroid periocular rehabilitation, namely decompression, then strabismus surgery, and finally eyelid surgery.

AIMS To assess whether orbital lobe and palpebral adenomas of the lacrimal gland are biologically similar, based upon a comparison of the age at presentation and at first symptom. The size of the excised tumor in the 2 groups is also examined and considered in relation to age at surgery. METHODS Review of clinical details and histopathology reports for patients with biopsy-proven lacrimal gland pleomorphic adenomas, under the care of Moorfields Eye Hospital. The age at presentation and at earliest symptoms were calculated for each patient. Where available, estimates of tumor volume were obtained from the 3 orthogonal measurements taken during histopathological examination. RESULTS One hundred eighty-seven patients (100 male patients; 53%) presented at an average age of 48 years (median, 46.8; range, 11-88): the average age for 144 patients with orbital lobe tumors (mean, 48 years; median, 47.8; range, 11-88) was similar to that for 43 patients with palpebral tumors (mean, 46 years; median, 46.8; range, 24-81) (p = 0.45). The average age at first symptom(s) in the 2 groups is similar (orbital 45 years, palpebral 44 years; p = 0.63). In contrast, there is a very highly significant difference in estimated tumor volumes, with the mean for orbital lobe pleomorphic adenomas being 5.4 ml (median, 4.9 ml; range, 1.5-13.6) and that for palpebral being 0.91 ml (median, 0.7 5 ml; range 0.11-3.61) (p = 1.42 × 10-25). CONCLUSIONS As expected, excised palpebral adenomas-readily palpable in the eyelid-are very much smaller than orbital lobe tumors, that tend to be impalpable. If pleomorphic adenomas in the 2 locations were biologically identical, they should arise at the same time and have similar growth rates-which would mean that orbital pleomorphic adenomas ([PAs] being, on average, 6 times the size of palpebral PAs) should present several decades later. The age at presentation and age at first symptom are, however, almost identical in the 2 groups. These observations provide incontrovertible evidence that, in their biological behavior, palpebral and orbital PAs are distinct entities-either arising at different ages (with orbital tumors arising much earlier in life) or having different growth rates (palpebral PAs being slower).

Purpose Well-differentiated neuroendocrine or carcinoid tumors are found most commonly in the gastrointestinal tract. When metastatic to the orbit, they tend to have a propensity for the extraocular muscles. The purpose of this study was to better understand the diversity in presentation of orbital carcinoid disease and to determine predictors for survival. Methods In this observational cross-sectional cohort study, data from 8 tertiary orbital practices were compiled. Demographic, clinical, pathologic, American Joint Committee on Cancer stage and grade, imaging, and management data were extracted for all the patients. Descriptive statistics were calculated. Subgroups were compared utilizing analysis of variance analyses and Kaplan-Meier curves. Time to progression and disease-specific and overall mortality were calculated. Comparisons were performed for the following a priori pairs: unknown versus known primary tumor, single versus multiple extraocular muscle involvement, unilateral versus bilateral orbital disease, extraocular muscle versus other orbital involvement, and excisional versus incisional surgery. Results A total of 28 patients with carcinoid tumors of the orbit were identified. Of these, 57.1% of patients were female, the mean age at diagnosis of the primary tumor was 58.8 years and the mean age at diagnosis of orbital disease was 62.6 years. At primary presentation, all patients were American Joint Committee on Cancer stage III or IV and 21.4% demonstrated carcinoid syndrome. Muscle involvement was noted in 78.6% of patients, and of these, 72% were noted to have single muscle disease. Eight patients had no primary tumor identified; 3 of these 8 demonstrated disseminated disease at the time of diagnosis. The overall 5-year survival rate was 81.8% from diagnosis of primary tumor and 50% from diagnosis of orbital disease. Subgroup analysis revealed that patients with unilateral orbital disease when compared with bilateral orbital disease had a longer progression-free survival and time to death from all causes (p = 0.025). Patients with disease localized to the orbit at presentation had longer time to death than those with disseminated disease. Treatment with surgery, radiation, or octreotide did not appear to affect survival. Patients managed with systemic chemotherapy had a shorter time of survival than the rest of the group. All other subgroup comparisons were not found to be statistically significant. Conclusions Neuroendocrine tumors of the orbit represent a wide spectrum of disease, with some cases being part of disseminated disease, while others being localized presentations. This heterogeneity may be responsible for the slightly higher overall survival in these patients than others with metastatic carcinoid tumors in other locations.

Aims To report the characteristics and management for patients with palpebral pleomorphic adenoma of the lacrimal gland. Methods The records for patients with palpebral pleomorphic adenoma seen at Moorfields Eye Hospital between 1978 and 2019 were reviewed for demographics, presentation, imaging, histopathology, management, and outcome. Results Forty-three patients (25 male; 58%) with palpebral pleomorphic adenoma were identified, presenting at a mean age of 46 years (median 46.4 years; range 24-81 years) with symptoms for an average of 31 months (median 18.0 months; range 1 month to 17 years). The commonest symptom was a painless upper lid lump (37 patients; 86%), although 4 patients (9%) had a localized ache, and 1 (2%) had episodic epiphora. A moderately-mobile, firm round mass (like a "chick-pea") was readily palpable in 39/43 (91%) patients, and significant ptosis and upper lid swelling was noted in 6 cases (14%). Imaging was reviewed in 35 patients, 28 (80%) having a well-defined homogeneous mass in front of the orbital rim, on the anterior pole of the lacrimal gland. With the exception of 8 tumors removed from the actual palpebral lobe using a conjunctival incision, all others were excised intact through an upper eyelid skin-crease incision. Histopathology confirmed an intact pseudocapsule in 39/43 (91%) and 10 (23%) had a mild chronic lymphocytic inflammation within the tumor and/or neighboring lacrimal gland. There were no tumor recurrences at an average follow-up of 40 months (median 9.1; range 2 weeks to 230 years). Conclusion Most palpebral pleomorphic adenomas are discovered as an upper lid lump and a few first become manifest as upper lid swelling with ptosis. To avoid inadvertent incisional biopsy, they should be recognized preoperatively-especially as most are readily palpable as a hard lump superotemporally, often freely mobile in the suborbicularis plane (anterior to the tarsus and main lacrimal gland). Excision, generally easiest through a transcutaneous route, was judged intact in all cases and confirmed in 91% of histological reports, with no recurrent tumors.

AIM: To describe patients with sarcoid-like granulomatous orbitopathy (SLGO), the rate of known and subsequent systemic sarcoidosis, and the treatment and outcome for the condition. PATIENTS AND METHODS: Retrospective review of patients with SLGO presenting between 1990 and 2018, excluding solely lid or lacrimal drainage disease. RESULTS: Sixty-one patients (45 female; 74%) were identified, 54 having biopsy-proven sarcoidosis (47 orbital, 24 prior extra-orbital), and half were Afro-Caribbean. The average age at presentation was 45.3 years (range 18–78 years), with the commonest symptoms being swelling, pain and diplopia; of clinical signs, most patients (87%) had eyelid swelling, reduced motility (25%), and disease-related visual impairment (10%). Localized dacryoadenitis was present in 49/61 (21/49 bilateral) patients, and more diffuse disease in 28/61 (8/28 bilateral). Systemic involvement was found in 23 (62%) of the 37 first presenting with orbital disease. Twenty-three (38%) patients were observed and two-thirds received oral corticosteroids, with 53/61 (87%) patients having subjective and objective clinical improvement. The average follow-up was 53.4 months (range 1–315 months) and clinical recurrence occurred in 12 (20%) patients at 26.5 months (range 1–115 months) after first diagnosis. CONCLUSIONS: Systemic sarcoidosis may be discovered in about two-thirds of patients presenting with SLGO (that tends to present with inflammatory features), but the treatment response appears similar in patients with known sarcoidosis and those with newly-diagnosed systemic disease after orbital presentation. With long-term follow-up, a third of patients have spontaneous regression of orbital disease, but 20% have recurrence after reducing or stopping systemic immunosuppression.

Purpose Although it is a commonly held belief that stretching of the optic nerve is important in dysthyroid optic neuropathy (DON), it is probably mainly due to microvascular ischemia arising from high pressure at the orbital apex. If stretch is important in DON, one would expect longer optic nerves and reduced optic nerve compliance in these patients. Methods Retrospective comparative case series for 75 patients with DON, and for age- and sex-matched control patients with thyroid eye disease (but no DON). Masked assessments of medial wall length, lateral wall length, optic nerve limits (ONLs), globe dimension, and globe projection were made from orbital CT images, and, from these measurements, various derivatives were estimated. The optic nerve shape was also judged as "straight," "mildly curved," or "markedly curved." Estimates of optic nerve compliance were compared in the 2 groups and examined particularly to look for evidence of reduced compliance in orbits with DON. Results Paired right-left comparisons suggested a small systematic bias in the estimates of orbital dimensions, possibly due to software performance; this does not complicate the study, as only ipsilateral orbits were compared in the 2 groups. In 115 paired orbits, there was no difference in the proportions with various optic nerve configuration (p = 1.0), but the mean ONL in DON was 32.9 mm, compared with 31.5 mm in thyroid eye disease without DON (p = 0.011). In 5 patients with bilateral thyroid eye disease but unilateral DON, when corrected for side-to-side bias, the ONL on the DON side was greater than the non-DON side (DON, 36.7 mm; non-DON, 34.7 mm; p = 0.066). Compliance estimates for the 115 paired orbits are almost identical (p = 0.99). Conclusions The almost identical compliance estimates for DON and non-DON orbits suggest that ONL elongation in DON, albeit significant, is not extending optic nerve to its elastic limits and is, therefore, unlikely to be causing metabolic stress to the optic nerve. The 1.4 mm extra ONL in DON orbits probably just represents more retrobulbar tissues-with more advanced disease-and optic nerve stretch is probably not significant in the pathogenesis of DON.

Purpose To compare serum angiotensin-converting enzyme (sACE) titers in patients with newly diagnosed sarcoid-like dacryoadenitis and nongranulomatous dacryoadenitis. Study design Retrospective comparative case series. Patients and methods Clinical details and presenting sACE titers were reviewed for patients with newly diagnosed sarcoid-like dacryoadenitis (SLD) and nongranulomatous dacryoadenitis (NGD). All sACE titers were expressed as a percentage of the upper end of normal range ("normalized"). Using Student t test, sACE titers for patients with unilateral SLD were compared with unilateral NGD and the sensitivity and specificity of sACE as a predictor for SLD calculated; this was repeated for patients with bilateral dacryoadenitis. The proportion of patients with raised sACE were compared in each group (Fisher exact test), and the mean sACE titers compared for these selected patients. Results For unilateral SLD the mean sACE was 97%, compared with 57% in NGD (p=0.0010), and the sensitivity of sACE titer was 42%, specificity 92%, positive predictive value 75%, and negative predictive value 73%. Area under curve from receiver operating characteristic analysis for unilateral dacryoadenitis was 0.78 (p=0.0040). In contrast, there was no significant difference for bilateral disease (SLD 148%, NGD 82%; p=0.33), and somewhat lower positive predictive value (54%), negative predictive value (71%) and area under curve (0.71; p=0.070). Although not significantly so with bilateral disease, the proportion of patients with raised sACE was consistently greater in SLD as compared to NGD-being highest with bilateral SLD (7/12 patients; 58%) and lowest in unilateral NGD (2/26; 8%). Seventeen of the 27 patients with SLD were later found to have systemic involvement: where systemic disease was present, the mean sACE levels were significantly different in unilateral (102%) and bilateral dacryoadenitis (209%; p=0.029) disease, whereas there was no difference in the absence of systemic involvement (sACE with unilateral dacryoadenitis = 86%, bilateral = 75%; p=0.72). Conclusions Normalized serum titers of ACE are minimally predictive for SLD, whether unilateral or bilateral, with some false negatives and false positives in all groups. However, with unilateral dacryoadenitis, an sACE of >125% maximum range appears absolutely predictive for SLD, and >150% maximum range for bilateral disease.

To examine the necessity for lateral osteotomy for intact removal of tumors within the orbital lobe of the lacrimal gland.Patients who, between 1992 and 2020, were scheduled for intact excision of well-defined masses within the orbital lobe of the lacrimal gland were identified from an orbital database. A retrospective review of the clinical records was performed and details of the tumor size obtained from histopathological reports. The first patients in the series had lateral osteotomy, whereas later, there was a shift toward osteotomy-free tumor excision through a skin-crease incision. The latter approach required isolation of the gland's orbital lobe on an intact sheet of periosteum, an anterior "flip" of the mass (out of the orbit, past the orbital rim) "hinged" at the isthmus between the orbital and palpebral lobes, and finally a division of the isthmus to release the intact mass.Clinical details were available for 100 patients (50 male), with 56 tumors removed using lateral osteotomy and 44 without. The mean age at surgery was 50.3 years with osteotomy (median, 51.8; range, 19-85 years) and 51.1 years without (median, 50.3; range, 11-81 years; P = 0.81). There were no postoperative complications in either group. Although the range of tumor volumes was similar in the two groups (1.6-15.0 ml with osteotomy; 0.9-15.1 ml without), the average volume was somewhat greater in those having osteotomy (mean, 6.4 ml) as compared with those without (mean, 4.6 ml; P = 0.0016). Inadvertent rupture of the tumor occurred once in each group, with tumors of similar small size.In many cases, large masses within the orbital lobe of the lacrimal gland can be safely removed intact, using the described flip technique without the need for lateral wall osteotomy.

PURPOSE To evaluate the clinical presentation, anatomical location, and histological features of congenital conjunctival cysts of the orbit. The location and the histological features of inflammation in these patients were compared with those for 293 orbital dermoid cysts. PATIENTS AND METHODS Retrospective review of the clinical details, imaging, and histopathology for patients who had excision of conjunctival cysts from their orbit between 1992 and 2020; patients with a history of trauma or surgery were omitted. RESULTS Twelve patients (7 male; 58%) with congenital conjunctival cysts were identified, the patients presenting at an average age of 16 years (median 26; range 1-61) with a symptoms for a mean duration of 20 months (median 24; range 6-36). The commonest symptoms were peribulbar lump (6/12 patients; 50%), and eyelid swelling and blepharoptosis (6/12 patients; 50%). An orbitaxl mass was palpable in 10 patients (83%), 3 patients (25%) had mild proptosis (1-3 mm), and the cysts were most commonly located superiorly (6/12 patients; 50%) or superonasally (3/12; 25%) in the anterior half of the orbit. Imaging was performed in 7 cases, this showing an intimate relation to the common sheath of the superior rectus/levator complex in 3 patients (25%) and to the trochlea in 1 (8%). All cysts were excised completely, and no patient had postoperative complications or recurrence. Chronic mild and nonspecific inflammation was evident within the cyst wall in 7 cases (54%), but-unlike 55% of the 293 dermoid cysts-none showed granuloma formation. CONCLUSION Congenital conjunctival cysts are rare and usually present with a palpable mass in the upper eyelid sulcus. A significant proportion of these cysts have an intimate relationship with the trochlea, or the superior rectus, levator palpebrae or superior oblique muscles and, to minimize the risk of postoperative diplopia or ptosis, particular care must be exercised during surgery.

Salivary gland pleomorphic adenomas not uncommonly show extensive sclerosis ("dense hyalinization," "coagulative necrosis," or "mummification"), which arise spontaneously or after prior surgery, and this change is considered a high-risk factor for malignant transformation of benign salivary adenomas. While minor hyalinization is common in lacrimal gland adenomas, massive sclerosis-where almost all (90% or more) of the tumor is replaced by an amorphous hyaline material-is extremely rare. Four patients (2 males) are described in whom, despite not having an acute inflammatory episode, their lacrimal gland tumor showed marked sclerotic necrosis within the majority of either the benign or malignant parts of the tumor. Three tumors had evidence of malignant change, 2 to adenocarcinoma, and 1 to mucoepidermoid carcinoma, but none of the malignant areas showed perineural or endovascular invasion. Extensive sclerosis in association with a lacrimal gland pleomorphic adenoma (LGPA) is rare (4/110 of the cases) and-as with salivary adenomas-appears to be associated with a high chance of associated malignant change. Both the surgeon and the histopathologist should regard extensive sclerotic necrosis as a harbinger for malignant change in association with benign pleomorphic adenomas of the lacrimal gland.

To describe the evolution of a surgical technique for the correction of large-angle incomitant exodeviations.Retrospective review of an interventional case series from 2005 to 2019 in a single centre, with analysis of surgical procedure, prism diopter (PD) deviations and complications.Thirty-one patients underwent surgery at an average age of 42 years (range 4-75 years) for minimal medial rectus function, mostly from oculomotor nerve palsy (23/31; 74%). The mean pre-operative exodeviation was 75 PD (range 30-200PD). Sixteen patients (52%) had undergone previous strabismus surgery. Thirty-eight operations were performed in which the medial rectus insertion was anchored to the periosteum of the posterior lacrimal crest via a retrocaruncular transconjunctival approach. The ipsilateral lateral rectus (LR) was disinserted and fixed to lateral orbital tissue in 29/38 (76%) operations, injected with botulinum toxin in 5, recessed in 2 and had already undergone maximal LR recession in 2. In all but the first 8 operations, temporary limbal sutures were passed through the eyelids to maximally adduct the globe post-operatively. At last follow-up (mean 24 months; range 2-130), the mean reduction in exodeviation was 49PD (range 10-80) and overall residual deviation was 26PD (range 80PD base-in to 14PD base-out). The 5 LR toxin procedures had a mean reduction of 22PD (range 10-40). Seven patients had persistent diplopia, one a transient corneal erosion and one caruncle suture exposure 4 years after surgery.Large-angle exodeviations can be markedly improved by bi-rectus fixation. This approach is both safe and effective and can be performed in complex patients with multiple previous procedures.

Aims To describe patients with periocular solitary extramedullary plasmacytoma (SEMP) and multiple myeloma (MM), together with an estimate of the risk of progression from SEMP to MM. Patients and methods A retrospective case-note review for patients seen between 1978 and 2020, examining demographics, presentation, imaging, pathology, management, and outcome. Results Twenty patients (10 male; 50%) presented at a mean age of 60.9 years, with an average symptom duration of 4.5 months. Ten (50%) patients had known systemic myeloma at ophthalmic presentation (the MM group) and, on average, they presented one decade earlier than those with occult MM discovered after orbital biopsy (p = 0.06); the majority (9/15; 60%) of patients with MM were female, whereas there was a male bias (4/5; 80%) with SEMP (p = 0.30). Most tumors (15/20; 75%) were within the anterior part of the orbit, especially superolaterally (16 patients; 80%), and the soft-tissue mass often appeared to "explode" from the frontal bone or greater wing of the sphenoid (16/20; 80%). Full treatment details were known for 19 patients: 6 (32%) had solely orbital radiotherapy, 4 (21%) chemotherapy, 6 (32%) combined chemoradiation, and 3 (16%) had combined chemoradiation with stem-cell transplant (Table 3). After an average follow up of 58 months, 1/5 (20%) patients with SEMP and 11/15 (73%) with MM had tumor-related death. The overall survival probability for all 20 patients with periocular plasmacytoma was 34% at 5 and 10 years, with MM patients having a worse outlook (27% 5-year, and 18% 10-year survival) compared with SEMP (53% survival at 5 and 10 years) (p = 0.18). None of the 5 patients with SEMP progressed to systemic MM over an average follow up of 9.1 years. Conclusions Although 50% patients with periocular plasmacytoma appear to have a SEMP at ophthalmic presentation, a half of these patients were found to have occult MM within 6 months of biopsy. Of those without systemic disease around the time of biopsy, none developed MM over an average follow up of more than 9 years.