A broad range of symptoms in allgrove syndrome: single center experience in Southeast Anatolia.

BACKGROUND: Allgrove syndrome (OMIM 231550) is a rare autosomal recessive disease characterized by non-CAH primary adrenal insufficiency (non-CAH PAI), alacrima, and achalasia. It is caused by mutations in the AAAS gene. The syndrome is also associated with variable progressive neurological impairment and dermatological abnormalities. METHODS AND RESULTS: We diagnosed 23 patients from 14 families with Allgrove syndrome, based on the presence of at least two characteristic symptoms, usually adrenal insufficiency and alacrima, between 2008 and 2018. A previously described nonsense variant of AAAS was detected in 19 patients from 12 families at homozygous state. Another novel homozygous mutation (c.394-397delCTGT) in AAAS was detected in four patients from two families. Presenting symptoms were alacrima (23/23; 100%), adrenal insufficiency (18/23; 78%), achalasia (13/23; 57%), short stature/growth retardation (16/23; 70%), hyperreflexia (15/23; 65%), palmoplantar hyperkeratosis (13/23; 57%), hyperpigmentation of the skin (10/23; 43%), hypoglycemia-induced convulsion (7/23; 30%), swallowing difficulty and vomiting (6/23; 26%). Serum DHEAS concentrations were low in all patients (23/23; 100%). CONCLUSIONS: Clinical symptoms vary even among patients carrying the same mutation. Triple A syndrome should be considered in the etiology of non-CAH PAI in Arab populations and in Southeast Turkey. Any child with non-CAH PAI should be evaluated for the presence of alacrima and/or achalasia or family history of alacrima and/or achalasia. Children with alacrima and/or achalasia should also be investigated for adrenal insufficiency. Definitive molecular diagnosis is essential for early diagnosis and management of adrenal insufficiency, neurological symptoms, and growth retardation in patients and early diagnosis of as yet asymptomatic cases in the family, together with genetic counseling.

Dacryops - A review.

Purpose: To review and summarize the epidemiology, clinical presentations, histopathologic characteristics and management of dacryops.Methods: A comprehensive PubMed, Embase and Google Scholar search of all articles written in English and non-English language articles with abstract translated to English on dacryops were reviewed. Data reviewed included epidemiology, etiology, pathogenesis, clinical presentations, histopathologic characteristics and management of dacryops.Results: Lacrimal ductal cysts or dacryops are classified based on locations: palpebral lobe cysts (simple dacryops); orbital lobe cysts; cysts of the accessory lacrimal glands of Krause and Wolfring; and cysts of ectopic (choristomatous) lacrimal glands. The exact etiology of dacryops remains unknown. Dacryops is usually asymptomatic but can present with varied symptoms depending on the type and associated complications. While complete excision of dacryops is generally recommended, marsupialization is also a safe and effective method for the treatment of dacryops in the palpebral lobe of lacrimal gland. Complete surgical excision via orbitotomy is required for deep orbital lesions.Conclusion: Dacryops is an uncommon benign cystic lacrimal gland tissue neoplasm. Dacryops can be classified into four types based on locations. Lesions could be either superficial that can be seen through the fornix or deep into the orbit. Observation is also an option for small or asymptomatic lesions. Surgery remains the mainstay of treatment for symptomatic cases, but approaches are different depending on the location of the lesions.

Clinicopathologic features of biopsied lacrimal gland masses in 95 Korean patients.

PURPOSE: To investigate the clinicopathologic features of lacrimal gland masses biopsied in a tertiary referral hospital in Korea. METHODS: Records from 95 Korean patients who underwent lacrimal gland mass biopsy were retrospectively reviewed. Data included demographics, clinical presentation, imaging findings, histopathologic diagnosis, and associated systemic disease. RESULTS: The median age was 52.0 years (range, 16-76 years), and 51 patients (53.7%) were female. Thirty-three patients (34.7%) had bilateral disease. The histopathologic diagnoses were as follows: chronic dacryoadenitis (52.6%, n = 50;29 non-specific and 21 immunoglobulin G4-related disease (IgG4-RD)), lymphoproliferative disease (25.5%, n = 24; 18 lymphoma and six lymphoid hyperplasia), benign epithelial tumour (13.7%, 13 pleomorphic adenoma), malignant epithelial tumour (3.2%, three adenoid cystic carcinoma), dacryops (3.2%, n = 3), solitary fibrous tumour (1.1%, n = 1), and xanthogranulomatous inflammation (1.1%, n = 1). Patients with chronic dacryoadenitis were significantly more likely to be younger (mean 47.5 years), have bilateral involvement (52.0%), and have a longer symptom period (mean 15.6 months) than those with lymphoproliferative disease (60.0 years, 25.0%, and 6.7 months, respectively; p < 0.05, each comparison). Patients with IgG4-related dacryoadenitis were significantly more likely to have bilateral involvement (85.7%) and have associated systemic involvement (52.4%) than those with non-specific dacryoadenitis (37.9 and 0%, respectively; p < 0.05, each comparison). Sixteen patients (16.8%) had associated systemic involvement: 11 with IgG4-RD and 5 with lymphoma. CONCLUSIONS: Chronic dacryoadenitis and lymphoproliferative disease were the most common causes of lacrimal gland masses in our cohort. Younger patients with bilateral involvement and a longer symptom period were more likely to have chronic dacryoadenitis than lymphoproliferative disease. Associated systemic involvement was not rare in patients with IgG4-RD or lymphoma. Our results suggest that biopsy of chronic lacrimal gland masses should be performed for proper evaluation and management.

Overview of benign and malignant lacrimal gland tumors.

PURPOSE OF REVIEW: The goal of this article is to review the key clinical and radiological features, treatment strategies, and prognosis of lacrimal gland tumors. RECENT FINDINGS: Debulking of the orbital lobe of the lacrimal gland may offer improved control rates in dacryoadenitis without compromise of tear film function. Contrary to previous belief, careful biopsy of the lacrimal gland prior to excision does not appear to increase the risk of recurrence in cases with suspected pleomorphic adenoma. Low-dose radiation (4 Gy) in two 2-Gy fractions appears to be effective and well tolerated in indolent non-Hodgkin lymphoma of the ocular adnexa with high local control rate. Eye-sparing surgery for adenoid cystic carcinoma (ACC) leaving minimal or no tumor residual in the orbit followed by adjuvant radiation therapy or chemoradiotherapy may provide good local control and long-term survival outcomes. Intra-arterial chemotherapy has been found to decrease recurrence and improve survival in ACC and can also be used as part of an eye-sparing treatment strategy. The development of targeted drugs may offer palliation for patients with unresectable or metastatic disease in lacrimal gland carcinoma. SUMMARY: This article offers an update on diagnosis, management, and prognosis of the major lacrimal gland lesions.

The Epidemiological, Clinical, and Histopathological Characteristics of Lacrimal Gland Biopsies in a Tertiary Care Center in Israel.

BACKGROUND: The distribution of pathology and clinical characteristics of lacrimal gland diseases are different in different areas of the world. OBJECTIVES: To evaluate the incidence rate, patient characteristics, and indications for surgical intervention of lacrimal gland lesions in a tertiary care center in Israel. METHODS: All biopsied or surgically removed lacrimal gland lesions at the Goldschleger Eye Institute from 2009 to 2015 were identified. The following data were collected: age, gender, indications for surgical intervention, diagnosis, treatment, and prognosis. RESULTS: We evaluated 28 lacrimal gland biopsies from 26 patients (11 men, 15 women). Mean age at biopsy was 47.5 years old. The most common presenting symptoms were: eyelid swollenness (57.14%), ptosis (32.14%), and proptosis (10.71%). All patients underwent computed tomography and magnetic resonance imaging. In 28 cases, infiltrations of the lacrimal gland were found. In nine cases infiltration of muscles or orbital extension were found. The most common pathologies were non-specified inflammation (44.82%), lymphoma (20.68%), and immunoglobulin G4-related disease (10.34%). The treatment was diverse according to the patient diagnosis. Prognosis of lacrimal gland disease was good; however, in five patients the systemic disease progressed. CONCLUSIONS: Lesions of the lacrimal gland comprise a wide variety of pathological findings that require different treatment strategies. Lacrimal gland biopsies enable physicians to precisely recognize the pathology; therefore, it is important to consider this surgical method in any patient with lesions in the lacrimal gland.

[A Retrospective Investigation of Lacrimation in Patients Treated with S-1].

Lacrimation is among the typical adverse drug reactions associated with S-1 treatment. However, lacrimation frequencies differ between reports, and a clear consensus regarding reaction times, risk factors, and symptomatic treatment for lacrimation is lacking. We retrospectively investigated the reaction times, risk factors, and outcomes of symptomatic treatment for lacrimation in 202 patients treated with S-1. The median estimated creatinine clearance noted upon initiation of cancer treatment was 75.8mL/min. The median of the relative treatment intensity was 87.1%, while the incidence of lacrimation was 26.7%. The median cumulative dose of S-1 before the onset of lacrimation was 23,520 mg in all patients, and 5,050 mg in those who developed lacrimation. Of the patients who developed lacrimation, 40.7% developed this symptom within 2 months after starting S-1 treatment. There were no apparent risk factors. The most frequently employed symptomatic treatment was a physiological saline ophthalmic solution provided as a hospital preparation. After treatment with this ophthalmic solution, 29.4% of the affected patients showed improvement and 70.6% showed no change; none however, experienced worsening of symptoms. These results suggest that clinicians should assess the presence of lacrimation after starting treatment with S-1. Symptomatic treatment with an ophthalmic solution that does not have a tear retention capacity may be useful in patients who have developed lacrimation.

Excessive watering eyes in gastric cancer patients receiving S-1 chemotherapy.

BACKGROUND: Watering eyes is a common late adverse event associated with S-1 chemotherapy; however, the frequency and predictive factors are not known. METHODS: This study included 304 consecutive gastric cancer patients treated with adjuvant S-1 monotherapy for 1 year at Shizuoka Cancer Center. We retrospectively evaluated the frequency of watering eyes, and explored other nonhematological adverse events during the first course of S-1 monotherapy which could become predictive factors for watering eyes. RESULTS: The severest grade of watering eyes during S-1 monotherapy was grade 2 in 41 patients (13.5 %) and grade 3 in 36 patients (11.8 %). The median time to onset of grade 2 and grade 3 watering eyes was 82 days (range 6-344 days) and 249 days (range 84-653 days), respectively, and the median cumulative S-1 dose at the onset of grade 2 and grade 3 watering eyes was 4174 mg/m(2) (range 491-16,095 mg/m(2)) and 10,243 mg/m(2) (range 4943-16,341 mg/m(2)), respectively. Multivariate analysis showed that anorexia (odds ratio 2.37, P = 0.008), oral mucositis (odds ratio 3.86, P = 0.0003), skin hyperpigmentation (odds ratio 3.84, P = 0.0001), and rash (odds ratio 3.76, P = 0.01) observed during the first course were significantly associated with watering eyes. CONCLUSION: The risk of watering eyes was higher in patients who also had anorexia, oral mucositis, skin hyperpigmentation, or rash during first course of S-1 monotherapy than in those without them.

[The Interdisciplinary Conference on Lacrimal System Disorders of ophthalmic and ENT surgeons at the University Hospital Halle : An analysis of present patient material]. / Die interdisziplinäre Tränenwegskonferenz von Ophthalmo- und Rhinochirurgen am Universitätsklinikum Halle (Saale) : Eine Auswertung des bisherigen Krankenguts.

BACKGROUND AND AIM: The lacrimal system (LS) crosses regions of ocular adnexa, the nose, and the paranasal sinuses. Surgery of disorders requires a targeted ophthalmologic and otolaryngologic examination. Since 2013, an Interdisciplinary Conference on Lacrimal System Disorders (ICLSD) has existed at the University Hospital Halle. First results of our experiences with this cooperation between ophthalmic and ENT surgeons are presented. METHODS: In a retrospective study at the Department of Ophthalmology, University Hospital Halle, all patients aged 22-80 years (n = 43, 10 with bilateral manifestation; mean age 65.1 years) having had a consultation in ICLSD between February 2013 and May 2015 were analyzed. Thereby, 53 LS were included in the descriptive evaluation. RESULTS: The main relevant pathology in terms of ophthalmologic manifestation was chronic dacryocystitis and its complications (n = 42), whereas nasal septum deviation (n = 11) and chronic rhinosinusitis (n = 10) were the most frequent otorhinolaryngologic pathologies. Prior to consultation, ENT (n = 34) and/or ophthalmic surgery (n = 40) had been performed. During ICLSD, an individualized therapeutic regime for each patient was developed. Surgery was required in 43 out of 53 LS. Of these, 32 were mainly ophthalmic, 11 mainly ENT interventions. At the time of evaluation (median 3 months; range 1 to 24 months), 29 out of 38 patients (76.3 %) were free of complaints. CONCLUSION: Since 2013, competences of ophthalmic and ENT surgeons have been integrated in ICLSD for advanced diagnosis and therapy of lacrimal disorders at the University Hospital Halle. Encouraging functional results are shown in this study, as most patients have had a long and complicated history of lacrimal system disorders. ICLSD enables interdisciplinary patient care.

Epidemiology of Oculoplastic and Reconstructive Surgeries Performed by a Single Specialist With 15 Years' Experience at a Tertiary Center.

PURPOSE: To evaluate the prevalence of oculoplastic surgeries performed in a tertiary hospital in South Korea and to highlight the proper choice of surgery according to disease. METHOD: Retrospective chart review was conducted over a 12-month period (March 2013 to February 2014) on all of the patients who underwent oculoplastic and reconstructive surgery by a single oculoplastic specialist with 15 years' experience. RESULTS: A total of 656 patients were enrolled in the current study. Of them, 355 patients (54.1%) had eyelid surgery, 151 patients (23.0%) had orbital surgery, and 150 patients (22.8%) had lacrimal surgery. Orbital surgery was more commonly performed in men (122 male patients versus 29 female patients, P = 0.00 by Pearson chi-squared test) and was also more common in younger patients than in other surgery groups (56.3 years in eyelid surgery versus 39.3 years in orbital surgery versus 46.9 years in lacrimal surgery, P = 0.001 by Pearson chi-squared test). Blepharoplasty was the most common eyelid surgery, contributing 24.2% of total eyelid surgeries, followed by lid mass excision (68 patients, 19.1%), Hotz operation (59 patients, 16.6%), and levator advancement (47 patients, 13.2%). Among orbital surgeries, reconstruction of orbital wall fractures was the most common, and was performed in 89 patients (58.9%) of total orbital surgeries, followed by orbital tumor debulking surgery (25 patients, 16.5%) and orbital decompression for dysthyroid optic neuropathy (19 patients, 12.5%). Endoscopic endonasal dacryocystorhinostomy (DCR) was the most common lacrimal surgery (84 patients), occurring in 56% of total lacrimal surgeries, followed by Monoka tube intubation (16 patients, 10.6%) for congenital nasolacrimal duct obstruction and conjunctivo-DCR using Jones tube (12 patients, 8%). CONCLUSIONS: Eyelid-related conditions, especially cosmetic blepharoplasty, were among the most commonly encountered surgeries in the Oculoplastics Department of a tertiary care hospital in South Korea. Blowout fracture repair and endoscopic DCR were the most common in orbital and lacrimal surgery. The authors hope that the current survey will contribute to the surgical training of ophthalmology residents and, on a larger scale, health care policies.

The frequency of granulomatous lacrimal gland inflammation as a cause of lacrimal gland enlargement in patients without a diagnosis of systemic sarcoidosis.

PURPOSE: To determine the rates of orbital sarcoidosis in patients with clinical lacrimal gland enlargement and no history of sarcoidosis. METHODS: This was a retrospective study of patients from the Orbital Surgery Service at Wills Eye Institute who underwent lacrimal gland incisional biopsy from 1994-2009 for lacrimal gland enlargement. Patients were excluded if they had a history of sarcoidosis, other autoimmunities, or lacrimal gland enlargement thought to be malignant in etiology. Subject charts were reviewed for pathology results, as well as clinical and demographic data. Rates of sarcoidosis in a patient population presenting with isolated lacrimal gland enlargement were thereby analyzed. RESULTS: 75 patients fulfilling study criteria had lacrimal gland biopsies. 67 (89)% had benign lacrimal gland pathology and 8 (11)% had malignant lacrimal gland pathology. Sarcoidosis was found histologically in 15 (20%). The most frequent pathologic finding was non-specific chronic non-granulomatous inflammation, seen in 32 (43%) studies specimens. Age and gender were not statistically different between sarcoid positive and sarcoid negative patients. Patients with a positive biopsy were more likely to be African American. 26 (35%) of the studied patients had ACE levels obtained. The average ACE level was statistically greater in patients with sarcoidosis-positive biopsies than in those with negative biopsies. DISCUSSION: In patients with clinical lacrimal gland enlargement, incisional biopsy led to a diagnosis of sarcoidosis in 20% of patients. An elevated ACE level may help predict which patients have a greater diagnostic yield with biopsy.

A survey of the histopathology of lacrimal gland lesions in a tertiary referral centre.

PURPOSE: To describe the histopathological distribution of biopsied lacrimal gland lesions in a tertiary referral centre in Singapore. METHODS: This was a retrospective chart review. Clinical records of patients who underwent lacrimal gland biopsy at a tertiary referral centre in Singapore between 2000 to 2010, were reviewed. Data collated included patient demographics, clinical presentation, association with systemic disease and histopathological diagnosis. RESULTS: Sixty-nine patients were studied. Median age of presentation was 50 years. Forty patients (58%) were female and the majority (84%, n = 29) were Chinese. The mean follow-up duration was 35.0 ± 34.5 months. 30.4% (n = 21) of the patients had bilateral disease. Chronic dacryoadenitis (46%, n = 32) was the most common histopathological diagnosis, followed by lymphoproliferative disorders (38%, n = 26) and pleomorphic adenoma (10%, n = 7). The diagnoses in four other patients included adenoid cystic carcinoma, lacrimal gland hypertrophy, lacrimal duct cyst and orbital vascular malformation. CONCLUSION: Chronic dacryoadenitis and lymphoproliferative disorders are the two commonest causes of lacrimal gland lesions in our series. Although many cases remain non-specific, about 60% have a specific inflammation that may be associated with a systemic disease. As one third of our patients with lymphoproliferative disease of the lacrimal gland had an associated systemic lymphoma, patients with such lesions should be referred for investigation of possible systemic lymphoma. The results of our study can aid in providing a more targeted approach to patient management.

[Safety of the preoperative evaluation of pleomorphic adenoma: a retrospective analysis]. / Sicherheit der präoperativen Evaluation des pleomorphen Adenoms der Tränendrüse: eine retrospektive Analyse.

BACKGROUND: The main problem of the management of suspected pleomorphic adenoma of the lacrimal gland is the safety of the preoperative diagnosis without biopsy. The current clinical standard (en bloc resection if suspected) has been questioned in this regard in recent publications. In this context we have analysed all consecutive lesions of the lacrimal gland region in our department for 5 years and evaluated pre-and intraoperative clinical and histological findings in regard to the safety of the preoperative diagnosis pleomorphic adenoma. METHODS: Retrospective analyses were performed for 93 consecutive cases of lesions of the lacrimal gland, which were resected or biopsied in the Department of Ophthalmology Charité CVK 2005 - 2009, so that a histological assessment was available. Clinical symptoms, radiological signs, histological diagnosis, therapeutic approach and results were documented. RESULTS: 65 patients had a dacryoadenitis (70 %), 5 were diagnosed with sarcoidosis (5 %), in another 5a pseudotumour of the orbit was diagnosed (5 %). Pleomorphic adenomas were found in 4 patients (4 %), 10 patients had a lymphoma (11 %), 7 of these 10 had an extranodal marginal zone lymphoma (EMZL), an adenocarcinoma was diagnosed in one patient (1 %), two patients had adenocystic carcinoma of the lacrimal gland (2 %) and another patient had a malignant mixed tumour as a consequence of an incomplete resection of pleomorphic adenoma (1 %). 8 patients were treated by an en bloc resection following the preoperative diagnosis pleomorphic adenoma. CONCLUSIONS: The majority of lesions in the lacrimal gland are inflammatory processes. The preoperative evaluation of the diagnosis pleomorphic adenoma had a sensitivity of 100 % and a specificity of 50 %.

Epidemiology of oculoplastic conditions: the Singapore experience.

PURPOSE: To study the prevalence of eyelid, lacrimal, orbital conditions in an outpatient and surgical environment in a tertiary care hospital in Singapore. METHODS: An 8-month study was conducted in the Oculoplastics Department at the National University Health System (NUHS). Demographic data, diagnosis details and surgical records were compared. RESULTS: Of 623 patients seen over 906 visits, prevalence was as follows: eyelid (60.3%), orbital (20.6%), lacrimal (16.3%), dysthyroid exophthalmos (6.0%). Sixty-two (10.0%) patients had more than one condition. The most common conditions encountered included blepharoptosis, lacrimal obstruction, and orbital deformities (including fractures). During the same period, distribution of surgical procedures was as follows: eyelid (77.4%), lacrimal (13.1%) and orbit (9.5%). Interestingly, epiblepharon correction comprised 9.5% of the clinical/surgical cases. CONCLUSIONS: Although eyelid related conditions are most common, lacrimal and orbital pathologies cause significant morbidity. Oculoplastics specialists and ophthalmologists should consider placing more emphasis on surgical training specific to such conditions. We also propose separate classifications for epiblepharon and thyroid eye disease in the International Classification of Diseases (ICD) as they are unique clinical problems in their own right. A postoperative status will also help efficiently classify patients as such patients are at risk for related complications subsequently. An examination of healthcare budget allocation for such conditions is also suggested.

Prevalence of lacrimal gland prolapse in the functional blepharoplasty population.

UNLABELLED: Traditional functional blepharoplasty has focused on the excision of variable amounts of skin, muscle, and fat, with little attention given to the finding of the lacrimal gland prolapse (LGP). The reported incidence of LGP found on clinical examination, on patients of all ages presenting for blepharoplasty, is 15%. The author's experience with the procedure in the older age group (older than 60), is that this percentage is much lower than that found at surgery. To verify whether this is correct, the author evaluated lacrimal gland position intraoperatively in a group of patients undergoing functional upper blepharoplasty. In those patients who had LGP, the author routinely repositioned the gland and evaluated surgical outcomes. METHODS: The author evaluated the presence of an intraoperatively displaced lacrimal gland in his functional blepharoplasty population over a 2.5-year period (2008-2010). Patients with a history of previous eyelid surgery, trauma, or who had concurrent ptosis or other eyelid malpositions were excluded from the study. When present, the degree of prolapse was graded as mild (0-2 mm), moderate (3-5 mm), or severe (6 mm or more). The author arbitrarily suture-repositioned the gland in all patients with 4 mm or more of prolapse. In cases with less prolapse, light cautery to the tip of the gland capsule and surrounding soft tissue allowed adequate repositioning without suture fixation. Pertinent patient demographics and postoperative complications were documented. Patients were seen consistently to 6 months after surgery with an average follow up of 12 months. RESULTS: Fifty-seven patients were included in the study. Thirty-four patients (60%) had some degree of LGP. Of these, 8 patients (24%) had mild prolapse, 23 patients (67%) had moderate prolapse, and 3 patients (9%) had severe prolapse. Nineteen patients (56%) with LGP had the gland suture-repositioned, and 15 patients (44%) received cautery to retro-place the gland. In one patient (3%), transient dry-eye symptoms developed after surgery. Otherwise, there were few benign and self-limiting postoperative complications, consisting of prolonged upper eyelid swelling and transient mild pain. CONCLUSION: Lacrimal gland prolapse is a common finding during functional upper blepharoplasty surgery and appears to be a normal involutional periorbital aging change. Most cases are moderate in degree (as defined in the article), and not associated with specific preoperative symptoms or complaints, except lateral hooding with concomitant visual field deficit. Repositioning the gland intraoperatively is generally complication free and typically not associated with increased morbidity or healing time. The clinical significance of LGP in the elderly undergoing blepharoplasty surgery is unknown and requires further study.

Endoscopic dacryocystorhinostomy among the old and oldest-old populations - A case control study.

OBJECTIVE: Despite increased risks of nasolacrimal duct obstruction (NLDO) with age, and the continuous growth of the old population proportion, data on endoscopic dacryocystorhinostomy (eDCR) among the old is lacking. This study aims to evaluate long-term eDCR efficacy and safety in the old and oldest-old population. METHODS: A retrospective case-control study of patients aged 80 ≤ (oldest-old) and 65-79 (old) compared with younger controls who underwent eDCR, between 2002 and 2017. Pre-, intra- and postoperative factors were collected using an integrated hospital-community system. Success rates were analyzed and measured at the first visit following surgery (immediate success), and after five years. Demographics, comorbidities, complications rates, and outcomes were compared between the groups. RESULTS: The study groups included 52 oldest-old patients (mean age 83.4 ± 3.6), 127 old patients (72.3 ± 4.14) and 142 control patients (57.8 ± 18.0). The immediate and success rates were 94.2%, 93.7% and 90.8% and five-year success rates were 80.0%, 76.6% and 80% among oldest-old, old and controls, respectively. No significant differences in success rates were found, even despite higher comorbidity rates among the study's group (96 and 92.8% vs. 63.2%, among oldest-old, old and controls respectively, p <0.001). Intra- and postoperative complications rates were low in all groups. CONCLUSIONS: Among older population, including oldest-old and old, eDCR safety and long-term outcomes are comparable with younger patients, suggesting that eDCR should be offered to NLDO patients, regardless of age.

Endoscopic Denker's Approach for the Treatment of Extensive Sinonasal Tumors: Our Experience.

OBJECTIVES: Surgical management of large sinonasal tumors, spreading to the pterygopalatine, and infratemporal fossae have always been a challenge for the ENT surgeon and the multidisciplinary team. METHODS: Endoscopic Denker's approach allows complete exposure of the anterior, inferior, and lateral recesses of the maxillary sinus, without necessitating a gingivobuccal or a transseptal incision. Moreover, endoscopic Denker's approach facilitates direct, straight entry to the pterygopalatine and infratemporal fossae. RESULTS: Twenty-two patients with extensive sinonasal pathology underwent resection via an endoscopic Denker's approach at our institution, with the majority of them being diagnosed with JNA and inverted papilloma. Denker's procedure facilitated wide exposure of the surgical field and total tumor resection, without significant perioperative complications and good disease-free results in follow up. CONCLUSIONS: Our experience is in accordance with the literature, where this approach seems to be superior to alternative options for accessing the pterygopalatine and infratemporal fossae. We believe that adequate expertise in endoscopic sinonasal surgery is necessary prior to embarking on this technique, but in general, it represents a valuable tool in the surgeon's armamentarium. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:1458-1462, 2021.

Ocular adnexal lymphoma in Denmark: a nationwide study of 387 cases from 1980 to 2017.

BACKGROUND: Nationwide studies of ocular adnexal lymphoma (OAL) are very rare in the literature, and knowledge on incidence, subtype distribution and long-term survival data is limited. This is the largest national study of OAL to date. This study sought to find information on incidence, changes in incidence, clinical findings, distribution of subtypes, survival and prognostic factors. METHODS: Patients diagnosed with OAL from January 1, 1980 to December 31, 2017 were identified in Danish registers, and clinical as well as survival data were collected. The data were analysed with Kaplan-Meier plots and log-rank test. RESULTS: 387 patients were included in the study. The major lymphoma subtypes were extranodal marginal-zone B cell lymphoma (EMZL) (55%), diffuse large B cell lymphoma (DLBCL) (13%), mantle cell lymphoma (MCL) (11%) and follicular lymphoma (FL) (10%). OAL is a disease of the elderly (median age 69 years). The incidence of lymphoma of the ocular adnexal region has increased significantly throughout the time period of the study (Pearson correlation coefficient, r=0.65; P<0.001). In the period 1980-1984, the incidence was 0.086 per 100 000, which increased to 0.307 per 100 000 in the period 2013-2017. Low-grade, low-stage primary lymphomas were treated with radiotherapy, whereas patients with high-stage, high-grade and/or relapsed disease were treated with chemotherapy. Low-grade subtypes EMZL (89%) and FL (56%) had better 10-year disease-specific survival than the high-grade lymphomas DLBCL (38%) and MCL (31%)(p<0.001). CONCLUSION: OAL is increasing in incidence in the Danish population for unknown reasons. However, the prognosis for most OAL is favourable, as highlighted in this national long-term study.

Congenital dacryocystocele: prenatal MRI findings.

BACKGROUND: Congenital dacryocystocele can be diagnosed prenatally by imaging. Prenatal MRI is increasingly utilized for fetal diagnosis. OBJECTIVE: To present the radiological and clinical features of seven fetuses with congenital dacryocystocele diagnosed with prenatal MRI. MATERIALS AND METHODS: The institutional database of 1,028 consecutive prenatal MR examinations performed during a period of 4 years was reviewed retrospectively. The cases of congenital dacryocystocele were identified by reading the report of each MRI study. RESULTS: The incidence of dacryocystocele diagnosed with prenatal MRI was 0.7% (n = 7/1,028). The dacryocystocele was bilateral in three fetuses. Mean gestational age at the time of diagnosis was 31 weeks. The indication for prenatal MRI was the presence or the suspicion of central nervous system abnormality in six fetuses and diaphragmatic hernia in one. Dacryocystocele was associated with an intranasal cyst in six of ten eyes. Prenatal sonography revealed dacryocystocele in only two of seven fetuses. Of eight eyes with postnatal follow-up, four did not have any lacrimal symptoms. CONCLUSION: Prenatal MRI can delineate congenital dacryocystocele more clearly and in a more detailed fashion than ultrasonography. Presence of dacryocystocele was symptomatic in only 50% of our patients, supporting that prenatal diagnosis of dacryocystocele might follow a benign course.

Ocular alignment, media, and eyelid disorders in Down syndrome.

Background: Determining the age and gender distribution of ocular disorders in Down syndrome patients aged 10 to 30 years.Methods: In this study, 226 of 250 invited patients through special needs schools, the National Down Syndrome Society, and relevant nonprofit organizations were included. In Noor Eye Hospital, the patients underwent a complete eye examination by a general ophthalmologist and suspect cases were reexamined by a sub-specialist. Examinations included ocular alignment, conjunctiva, eyelid, lacrimal system, cornea, iris, and lens assessment.Results: Mean age of participants was 16.05 ± 4.82 years and 53.0% were male. The most common ocular abnormalities were blepharitis (81.9%, 95% CI:78.0 to 85.3), lens opacity (37.8%, 95% CI:33.3 to 42.3), strabismus (23.4%, 95% CI:19.5 to 27.4; 21.2% esotropia, 0.9% exotropia, and 1.8% dissociated vertical deviation), floppy eyelid (19.9%, 95% CI:16.3 to 23.9), posterior embryotoxon (17.7%, 95% CI:14.2 to 21.2) and nystagmus (11.7%, 95% CI:8.9 to 15.0). Based on independent sample t test, the prevalence of nystagmus (P = .041) and congenital lens opacity  (P<0.001) significantly increased with age. There was no significant inter-gender difference in the prevalence of any of the studied disorders by chi-square test.Conclusion: In young patients with Down syndrome, the prevalence of ocular pathologies appears to be high and increase with aging. It can be resulted from the cumulative prevalence of undiagnosed or untreated cases. Findings of the study can be a reliable reference for health policy in terms of screening for eye disease and addressing eye care needs.

Lymphoma of the Lacrimal Gland - An International Multicenter Retrospective Study.

PURPOSE: To characterize the clinical features of subtype-specific lacrimal gland lymphoma and their effect on patient survival. DESIGN: Multicenter retrospective interventional case series. METHODS: Patient data were collected from 6 international eye cancer centers from January 1, 1980, through December 31, 2017. All patients with histologically verified primary or secondary lymphoma of the lacrimal gland were included. Primary endpoints were overall survival (OS) and disease-specific survival (DSS). RESULTS: A total of 260 patients with lacrimal gland lymphoma were identified. The median age was 58 years and 52% of patients were men. Non-Hodgkin B-cell lymphomas constituted 99% (n = 258) and T-cell lymphomas constituted 1% (n = 2). The most frequent lymphoma subtypes were extranodal marginal zone B-cell lymphoma (EMZL) (n = 177, 68%), follicular lymphoma (FL) (n = 26, 10%), diffuse large B-cell lymphoma (DLBCL) (n = 25, 10%), and mantle cell lymphoma (MCL) (n = 17, 7%). Low-grade lymphomas (EMZL and FL) were most commonly treated with external beam radiotherapy (EBRT), whereas high-grade lymphomas (DLBCL and MCL) were treated with chemotherapy in combination with rituximab and/or EBRT. The prognosis was relatively good with a 5-year OS and DSS of 73.8% and 87.5%, respectively. Lymphoma subtype was a statistically significant predictor for DSS, with EMZL (5-year DSS: 93.4%) having the best prognosis and DLBCL (5-year DSS: 52.6%) having the poorest. CONCLUSIONS: This is the largest reported collection of data of subtype-specific lacrimal gland lymphoma. The subtype distribution of lacrimal gland lymphoma resembles that of the ocular adnexa. Prognosis is good and the histologic subtype is a significant predictor for disease-specific survival.