Ophthalmic Presentation and Outcome for Sinonasal Mucoceles.

PURPOSE: The purpose of this study was to evaluate ophthalmic features and outcomes for patients who present with sinonasal mucoceles expanding into the orbit. PATIENTS AND METHODS: 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. RESULTS: 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%). CONCLUSION: 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.

Dupilumab-associated ectropion and punctal stenosis treated with tacrolimus ointment (0.03%) in a 15-year-old girl.

A 15-year-old Asian girl with severe atopic dermatitis was referred for dupilumab-associated blepharoconjunctivitis. Medical history was significant for severe atopic dermatitis. She was started on prednisolone acetate 1% ophthalmic suspension three times daily, and dupilumab injections were withheld after the initial visit. The patient was noted to have right lower eyelid ectropion, cicatricial occlusion, and severe punctal stenosis 6 weeks later. She was started on 0.03% tacrolimus ointment to the eyelid margin. Resolution of ectropion and restoration of punctal patency with residual stenosis were observed 4 weeks later. This is the first reported adolescent case of dupilumab-associated ectropion and punctal stenosis successfully treated with topical tacrolimus ointment.

Transconjunctival botulinum toxin injection into the lacrimal gland in crocodile tears syndrome.

Purpose: To determine the efficacy and safety of botulinum toxin injection into the lacrimal gland as a symptomatic treatment of crocodile tear syndrome (CTS). Methods: Our study included six patients of unilateral gustatory hyper lacrimation following either an episode of facial paralysis or post trauma or any related surgery that posed a risk of damaging the facial nerve. Detailed history regarding previous trauma, duration of facial paralysis, previous significant surgery, and duration of steroid use following facial paralysis was noted. Schirmer's test was done at baseline and 3 months follow-up. Patients' consent was taken prior to treatment with botulinum toxin injection in the lacrimal gland. Repeat injection of 5 U botulinum toxin (type A) was administered into the lacrimal gland of all patients transconjunctivally within an interval of 1 week. All patients were followed up with Schirmer's test at 6 weeks and 3 months. Any complications during treatment were recorded. Results: All six patients showed complete or partial disappearance of reflex lacrimation while chewing following botulinum injection measured by a significant reduction in Schirmer's value. When comparing Schirmer test values before (27.8 ± 3.58 mm) and after (11.6 ± 2.28 mm) BTX-A injection, the differences observed (P = 0.002) were statistically significant (P < 0.05). Only two patients developed mild transitory ptosis. No other complications were noted. Conclusion: Transconjunctival botulinum toxin injection into the lacrimal gland is an effective and safe method to decrease reflex lacrimation during eating or chewing in CTS or gustatory hyper-lacrimation syndrome.

Upper Eyelid Retraction in Graves' Ophthalmopathy: Our Surgical Experience on 153 Cases of Full-Thickness Anterior Blepharotomy with Mullerectomy.

BACKGROUND: We present our experience on 153 cases of full-thickness anterior blepharotomy with mullerectomy (FTABM) in the treatment of upper eyelid retraction (UER) related to Graves' ophthalmopathy (GO). METHODS: We included all the patients who underwent a graded FTABM between 1st January 2015 and 30th June 2020 for UER GO-related. The analysis included: pre-/post-operative conjunctival symptoms, epiphora, GO-Quality of Life Questionnaire (QoL), lagophthalmos, marginal reflex distance (MRD-1) index, eyelid symmetry within 1mm. The statistical analysis was designed to detect postoperative improvement in objective and subjective clinical features. Outcomes were analysed through Chi-squared test for dichotomous variables and through Wilcoxon-Mann-Whitney test for continuous variables. RESULTS: Of the 111 patients, 42 underwent a bilateral procedure, while 69 a monolateral. Conjunctival symptoms were reported in 32% of cases before surgery and in 12.4% after FTABM (p < 0.001). Epiphora was complained by 29.6% of patients preoperatively and in 12.4% postoperatively (p < 0.001). Preoperative lagophthalmos was found in 12.4% (mean value of 0.34 ± 0.76 mm) of eyelids, and in 2.6% (mean value 0.05 ± 0.19 mm) eyelids (p = not significant) postoperatively. Pre-operative GO-QoL was 24.9 ± 4.4 mm; while post-operative GO-QoL was 35.3 ± 5.5 mm (p < 0.001). The MRD-1 varied from 10.12 ± 2.1 mm preoperatively, to 4.3 ± 0.6 mm (p < 0.001) after surgery. Asymmetric palpebral fissure was noted in 94 (84.7%) patients before surgery and in 7 (6.3%) after the procedure (p < 0.001). CONCLUSION: FTABM is an effective procedure to treat UER GO-related. The technique manages to prevent complications of UER and determine good aesthetics. LEVEL OF EVIDENCE IV: This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 . Level IV, therapeutic study.

Chronic Granulomatous Dacryoadenitis Associated With Pleomorphic Adenoma.

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.

Congenital alacrima.

The congenital absence of tear production or alacrima is a distinctively unusual clinical sign that harbors a wide variety of etiologies. While alacrima can be only isolated to the lacrimal system, it is more often associated with progressive multisystem involvement from underlying genetic disorders. Recognizing the subtle ocular signs in these diseases will promote a timely diagnosis and management before potential life-threatening consequences occur. Hence, the current article will review the ophthalmological findings, systemic manifestations, genetic associations, and differential diagnosis of congenital alacrima.

Reduced Tearing With Stable Quality of Life After Vidian Neurectomy: A Prospective Controlled Trial.

OBJECTIVES/HYPOTHESIS: Although vidian neurectomy (VN) is associated with decreased lacrimation, its impact on dry eye quality-of-life is not well-defined. Endoscopic endonasal transpterygoid approaches (EETA) may require vidian nerve sacrifice. STUDY DESIGN: A prospective cohort trial. METHODS: A prospective trial evaluating VN during EETA on lacrimation by phenol red thread testing and dry eye severity by the five-item Dry Eye Questionnaire (DEQ-5) was performed. Preservation of the contralateral vidian nerve allowed comparison between the eye subjected to VN and the control eye postoperatively. RESULTS: Twenty-one subjects were enrolled with no preoperative difference in lacrimation between eyes (P = .617) and overall mild dry eye severity. Although the control eye had no difference in lacrimation pre- and postoperatively, decreased tearing was noted in the VN eye at 1 month (20.8 mm vs. 15.8 mm, P = .015) and at 3 months (23.2 mm vs. 15.8 mm, P = .0051) postoperatively. Overall, no difference was noted in the DEQ-5 score for dry eye severity between the pre- and postoperative measures. However, six patients were noted to have moderate to severe dry eye severity postoperatively and five of these six had decreased lacrimation (<20 mm) preoperatively. Patients with decreased tearing preoperatively demonstrated significantly worse postoperative DEQ-5 scores when compared to patients with normal tearing (P < .0056). CONCLUSIONS: VN during EETA results in decreased tearing but is not associated with increased dry eye severity overall. However, patients with decreased tearing preoperatively are at risk for increased dry eye severity and should be counseled for this risk. LEVEL OF EVIDENCE: 2 Laryngoscope, 131:1487-1491, 2021.

[Clinical and pathological analysis of 64 patients with primary neoplasms of the lacrimal drainage system].

Objective: To analyze the pathological classification and age distribution of primary neoplasms of the lacrimal drainage system. Methods: Retrospective case series study. A total of 64 patients (65 eyes) were diagnosed with primary neoplasms of the lacrimal drainage system and received surgery at Tianjin Eye Hospital from January 2006 to December 2016. All the clinical data of the patients were analyzed, including gender, diseased eye, age, clinical manifestations, composition of benign and malignant masses, and prognosis. The histopathological composition and age distribution of patients with primary lacrimal mass, lacrimal duct mass and lacrimal sac mass were analyzed according to the different diseased sites. Results: Twenty-three patients (24 eyes) were male, and 41 patients (41 eyes) were female. The right eye was involved in 36 patients, the left eye in 27 patients, and both eyes in one patient. The age at diagnosis ranged from 12 to 78 years old [mean, (46±4) years]. The course of disease was (13.1±4.2) months, ranging from 1 month to 7 years. The chief complaint was tear discharge in 43 patients, tumor in 19 patients, and abscess discharge in 2 patients. There were 29 patients with angular displacement and 21 patients with swelling pain. There were 51 patients with benign lesions, 4 with borderline lesions, and 9 with malignantlesions. These neoplasms consisted of primary peripunctal neoplasms in 17 patients, primary canalicular neoplasms in 2 patients, and primary lacrimal sac neoplasms in 45 patients. All primary peripunctal neoplasms (17 cases) were benign, among which nevi (10 cases) occupied the first place. All primary canalicular neoplasms (2 cases) were benign, there were 1 case of epidermoid cysts and 1 case of degenerative disease. Among the primary benign lacrimal sac masses (32 cases), mucous epithelial cysts (9 cases), dermoid cysts (6 cases), and epidermoid cysts (6 cases) occupied the first three places. Among the primary borderlin lacrimal sac masses (4 cases), there were 2 cases of giant cell tumor of soft tissue, 1 case of solitary fibrous tumor, and 1 case of inflammatory myofibroblastic tumor. The primary malignant mass of lacrimal sac (9 cases) was dominated by squamous cell carcinoma (3 cases). In terms of age distribution, the patients with primary peripunctal mass were mainly in the group of 40-59 years old (14 cases). The primary benign mass of lacrimal sac mainly occurred in the group of less than 40 years old (15 cases) and the group of 40-59 years old (11 cases). The patients with primary lacrimal sac borderline and malignant masses were all in the groups of over 40 years old. A total of 49 patients were followed up for 27 months to 16 years. The average follow-up time was (57.2±3.8) months. Lacrimal sac transitional cell carcinoma relapsed 7 months after surgery in one patient, and lacrimal sac melanoma relapsed 1 year after surgery in one patient. The patients did not relapse in 24 months and 38 months after surgery respectively. There were no recurrence of other cases. Conclusions: Primary peripunctal neoplasms are mostly characterized with benign lesions, among which nevi are most common. Mucous epithelial cysts, epidermoid cysts, and dermoid cysts are the major benign lacrimal sac neoplasms. Squamous cell carcinomas are the most common malignant lacrimal sac neoplasms. The malignant tumor of lacrimal sac often occurs in the middle-aged and elderly patients. (Chin J Ophthalmol, 2020, 56: 364-369).

[Retrospective investigation of spontaneous bloody tears: a report of 27 cases].

Objective: To summarize clinical experience on the clinical feature, etiology and treatment of patients with spontaneous bloody tears as the initial symptom. Methods: Retrospective series of case studies. The clinical data and follow-up data of 27 cases of bloody tears as the first symptom in Lacrimal Center of Ophthalmology, the Third Medical Center of Chinese PLA General Hospital from June 2015 to December 2018 were reviewed. The clinical feature, specific cause, diagnosis, treatment and prognosis of these cases were evaluated. Results: A total of 27 cases were collected in this study. The patients were 10 males (37.0%) and 17 females (63.0%), including 21 adults (≥ 18 years old, 77.8%) and 6 minors (<18 years old, 22.2%). There were 22 monocular cases (81.5%) and 5 binocular cases (18.5%). Five cases (18.5%) were bleeding from the eye and other parts of the body, and 22 cases (81.5%) were bleeding only from the eye. There were 19 cases (70.4%) with hematic epiphora and secretions from the punctum, 3 cases (11.1%) with blood-stained tears, and 7 cases (25.9%) with blood-like tears. With regard to etiology, 6 cases (22.2%) were combined with systemic lesions, one of which was granulomatosis with polyangiitis and five of which (<18 years old) were idiopathic bloody tears. Twenty-one cases (77.8%) were local lesions, including 18 cases only involving the lacrimal system, 2 cases only involving the ocular surface, and 1 case involving both the lacrimal system and the ocular surface. Among the 21 cases with local lesions, 5 cases were induced by foreign body, 6 cases were induced by simple inflammation, and 10 cases were induced by tumor including 1 case with conjunctival benign tumor and 9 cases with tumor of the lacrimal system (5 with malignant tumor and 4 with benign tumor). Patients with idiopathic bloody tears received psychological and medical treatment, and interictal discharge was lengthened. One case of granulomatosis with polyangiitis was treated by trans-nasal endoscopic dacryocystorhinostomy. With the recurrence of granulomatosis and polyangiitis, bloody tears recurred after surgery. One patient with conjunctival hemangioma was untreated. Lesions in the lacrimal duct system were removed and dacryocystorhinostomy was performed. In this study, 2 patients (1 with small cell neuroendocrine carcinoma and 1 with adenoid cystic adenocarcinoma) died and the other had a good prognosis. Conclusions: Among the cases of bloody tears, adults and local lesions are more common. Most of the lesions are located in the lacrimal system and are tumors. The main treatment is to remove the lesions, and if necessary, to expand the resection and reconstruct the lacrimal duct. Idiopathic bloody tears occur in minors, who are gave psychotherapy and necessary medical treatment. (Chin J Ophthalmol, 2020, 56: 53-58).

Nasolacrimal duct obstruction secondary to lacrimal sac involvement by sebaceous carcinoma.

Sebaceous carcinoma (SC) is the third most common eyelid malignancy in Australia, and is potentially fatal. It usually presents as a nodule or diffuse eyelid thickening, and is commonly misdiagnosed. We describe a case of SC with lacrimal sac involvement, presenting with clinical features of nasolacrimal duct obstruction. At the time of endoscopic dacryocystorhinostomy (DCR), nasal endoscopy revealed a polypoid mass of the opened lacrimal sac. Biopsy of the mass showed poorly differentiated SC. The lacrimal drainage apparatus was later excised via a combined external and endoscopic approach. Conjunctival map biopsies showed extensive intraepithelial disease, which was treated with topical mitomycin C. At three-month follow-up, there was no evidence of residual disease on nasal endoscopy or repeat conjunctival biopsy.

Isolated Langerhans Cell Histiocytosis of the Lacrimal Gland in Conjunction With Mucosa-Associated Lymphoid Tissue Lymphoma and Elevated IgG4 Plasma Cells.

Langerhans cell histiocytosis (LCH) is a clonal neoplastic proliferation of Langerhans-type cells. Orbital LCH is infrequent, typically manifesting as an isolated lytic bony lesion with an adjacent soft tissue mass in a child. Isolated lacrimal gland involvement by LCH is extremely rare, with only 2 previously reported cases. The authors describe a 37-year-old woman with a 6-month history of painless right upper eyelid swelling and diffuse right lacrimal gland enlargement without bony changes on computed tomography scan. Excisional biopsy of the lacrimal gland demonstrated concurrent LCH, extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue, and increased IgG4-expressing plasma cells. Work-up was negative for systemic hematolymphoid malignancy and IgG4-related disease. This case illustrates the association between LCH, mucosa-associated lymphoid tissue lymphoma, and elevated IgG4 plasma cells in the lacrimal gland, and we review the emerging theories proposed to explain this phenomenon.

A Child with Weight Loss and Alacrimation: Triple A Syndrome.

Triple A syndrome or Allgrove's syndrome is a rare autosomal recessive disorder usually manifested with three main clinical features, i.e. achalasia, alacrimation and adrenal inadequacy. Sometimes, it presents with polyneuropathy and neurological complications. Here, we report a case of a 7-year girl presenting with features of weight loss who was diagnosed with adrenal insufficiency at the age of 7 years while achalasia was diagnosed at the age of 3 years. First manifestation was achalasia and at that time, alacrimation was also defected. A 7-year XX female child presented at Endocrine Clinic of Armed Forces Institute of Pathology (AFIP) with hyperpigmentation, easy fatigue and weight loss. She had one sibling with same complaints and one brother died at the age of 3 years because of adrenal insufficiency. Her laboratory investigations revealed low cortisol level and high ACTH level, with inadequate response as well as short synacthen test (dynamic function test). This is a first case of Allgrove's syndrome reported in a tertiary hospital setting of Pakistan. Allgrove's syndrome should be considered in patients who report with adrenal insufficiency.

Orodental Findings in Patients with Lacrimo-Auriculo-Dento-Digital Syndrome.

Lacrimo-auriculo-dento-digital syndrome (LADD) is a rare autosomal dominant disorder arising from heterozygous mutations in the genes encoding fibroblast growth factor receptors two and three and the gene encoding the fibroblast growth factor 10. The characteristics associated with LADD are mainly related with hypoplasia or aplasia of lacrimal and salivary ducts, low cup-shaped ears, sensorineural or conductive hearing loss, abnormalities of teeth, and anomalies of the hands and feet. The purpose of this paper is to describe a 13-year-old female patient with a history of a blocked tear duct, mild hearing loss, congenitally missing teeth, tauro- dontism, and malformation of the fingers who was referred for a dental evaluation. She was diagnosed with LADD syndrome based on her clinical picture. (J Dent Child 2019;86(1):53-60)
Received August 16, 2018; Last Revision November 8, 2018; Accepted November 9, 2018.

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

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

A case of fatal osteolytic hypercalcemia complicated with IgG4-related ophthalmic disease leading to renal failure.

A 40-year-old male was hospitalized with renal impairment and severe hypercalcemia. His concentration of serum IgG4 was high, but serum whole PTH, 1-25(OH)2 vitamin D3 and PTHrP were not elevated. Computed tomography showed swelling of the bilateral lacrimal glands and systemic lymphadenopathy. The histological findings of lacrimal gland biopsy fulfilled the diagnostic criteria of IgG4-related ophthalmic disease (IgG4ROD). Bone scintigraphy showed increased ectopic uptake in the stomach, heart, lungs, and kidneys. He died on day 16 of admission, although the therapies for hypercalcemia were continued. Autopsy results showed an increase of osteoclasts in the bone marrow and metastatic calcification in multiple organs, and excluded from the differential diagnosis other disorders which present lymph-node swelling and hypercalcemia such as cancer, lymphoma, Castleman's disease, and sarcoidosis. He was given a diagnosis of IgG4ROD with osteolytic hypercalcemia.

Idiopathic edematous punctal stenosis with chronic epiphora: preponderance in young women.

PURPOSE: To describe a previously unreported phenomenon of idiopathic edematous punctal stenosis (IEPS) with chronic epiphora, presenting almost exclusively in young female patients. METHODS: A review of patients who presented with chronic epiphora and edematous punctal stenosis of unknown cause (IEPS) at the outpatient clinic of Soroka Medical Center between August 2011 and August 2015. Associated findings from clinical examination were recorded. RESULTS: A total of 32 patients with IEPS were documented. There were 30 female and 2 male patients. Average age at diagnosis was 37.6 ± 13.4 years, range 19-63 years, median 35 years. Temporary alleviation of symptoms was reported in subjects treated with topical steroids (43.8%), tacrolimus ointment (15.6%), and matrix metalloproteinase inhibitors (3.1%). No improvement in symptoms was reported in 37.5% of patients. Symptoms were bilateral in 78.1% of the patients. Spontaneous resolution was achieved in only 6.3% (2/32). CONCLUSIONS: IEPS accompanied by chronic epiphora has not been characterized to date. Our data show a clear predominance of females, most of them in their fertile years.