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Sjögren's syndrome (SS) is a systemic autoimmune disease affecting the lacrimal and salivary glands. In up to one third of patients, SS may be complicated by potentially severe extra-glandular visceral involvement, which can be life-threatening. Diagnosis is often difficult based on a combination of clinical and biological evidence. The development of new imaging techniques can now help the clinician in his diagnostic approach. Long considered incurable, new medical and surgical treatments are being studied and are potentially promising. Paramedical management and regular physical activity are also essential and contribute to the improvement of patients' fatigue. The objective of this article is to review the main clinical manifestations as well as the diagnostic and therapeutic novelties developed in recent years.Le syndrome de Sjögren (SS) est une maladie autoimmune systémique touchant les glandes lacrymales et salivaires. Chez près d’un tiers des patients, il peut se compliquer d’atteintes extraglandulaires potentiellement sévères. Le diagnostic, reposant sur un faisceau d’arguments clinicobiologiques, est souvent difficile. Le développement des nouvelles techniques d’imagerie peut aujourd’hui aider le clinicien dans sa démarche diagnostique. Longtemps considéré comme incurable, de nouveaux traitements médicochirurgicaux sont en cours d’étude et potentiellement prometteurs. Une prise en charge paramédicale avec une activité physique régulière est également essentielle. L’objectif de cet article est de revoir les manifestations cliniques ainsi que les nouveautés diagnostiques et thérapeutiques développées ces dernières années.

IgG4-related disease is a rare multi-organic inflammatory disease that frequently involves the ENT and head and neck areas. In these cases, salivary gland and lacrimal gland involvement is very common and includes enlargement, infiltration, and formation of pseudotumours. Diagnosis of IgG4 related disease remains a challenge and relies on several clinical, serological, radiological and histopathological criteria to differentiate from other diseases with similar clinical presentation. Histology reveals IgG4 positive lymphoplasmocytic infiltrates, storiform fibrosis and obliterative phlebitis. Glucocorticoids are the first line of treatment and can be combined with other immunosuppressants. The prognosis is favorable if treatment is initiated early. Recurrences are common. Delay in diagnosis can have severe multi-organic consequences.Rare et encore peu connue, la maladie à immunoglobuline G4 (IgG4) est multiorganique et se manifeste fréquemment dans la sphère oto-rhino-laryngologique et cervico-faciale. Dans ces cas, les glandes lacrymales et salivaires sont les plus fréquemment touchées, présentant des tuméfactions, infiltrations et pseudotumeurs. Le diagnostic reste un défi ; il repose sur des critères cliniques, sérologiques, radiologiques et histopathologies qui visent à la distinguer des nombreuses maladies de présentations cliniques similaires. À l’histologie, on retrouve un infiltrat lymphoplasmocytaire riche en plasmocytes IgG4+, une fibrose storiforme et des phlébites oblitérantes. Les glucocorticoïdes sont la première ligne de traitement et peuvent être associés à d’autres immunosuppresseurs. Le pronostic est favorable si le traitement est initié rapidement. Les récidives sont courantes. Un retard diagnostique peut avoir des conséquences multiorganiques sévères.

To describe the anatomical landmarks of the retro-caruncular approach and its clinical applications based on a cadaveric study.A dissection of 8 orbits providing from 4 fresh cadavers was carried out at the anatomical laboratory of the University Hospital of Nice, France between October 2018 and January 2019.Main anatomical relationships encountered are anteriorly the Duverney-Horner muscle and the lacrimal sac, posteriorly the anterior and posterior ethmoidal arteries, superiorly the pulley of the superior oblique muscle, inferiorly the lacrimonasal duct and the tendon of the inferior oblique muscle. The retro-caruncular approach allows a safe surgical access behind the lacrimal sac and Duverney-Horner muscle. Many oculoplastic surgical procedures can be performed through this approach: dacryocystorhinostomy, medial orbital fractures repair, "médial" orbital "décompression", biopsy of medial and extraconal tumours, medial periosteal fixation in third-nerve palsy.The retro-caruncular approach is a safe procedure avoiding skin incision. It allows a wide surgical space even if it is reduced compared to a more conventional skin route. It requires a great anatomical knowledge and a longer surgical learning curve.

Lacrimal occlusion with punctal or canalicular plugs have been used to treat dry eye disease for more than 40 years. Indeed, punctal plugs constitute a safe and effective tool to retain the natural tear film and prolong the effect of tear substitutes. A wide variety of plugs is available, differing in their design, location (punctal versus canalicular) and their resorbability. There indications have increasingly broadened, and they are now one of the treatment options for numerous ocular surface diseases. Current research focuses on using punctal plugs for extended delivery of drugs to the ocular surface. This review addresses physiology of lacrimal drainage, available models of punctal plugs, their indications, practical details of prescribing and placing punctal and canalicular plugs, and possible complications.

The dacryoscanner is the imaging technique of choice in case of persistent tearing. It allows the opacification of nasolacrimal system, and thus determines the site and the origin of obstruction. The site could be the lacrimal canaliculus, the lacrimal sac, or the nasolacrimal duct. The origin could be an idiopathic or a sequellar stenosis, a dacryolithiases, a bony malformation, or a tumor. This is a technique done by the radiologist under aseptic conditions, and easily tolerated by the patient. Many technical variants exist for an optimal opacification of the nasolacrimal system.

Dry eye disease (DED) can be evaluated clinically by the tear film break up time (BUT). There is a discrepancy between symptoms in relationship with dry eye disease and objectives measures of BUT. So we tried to evaluate the reliability of BUT in the diagnosis and treatment of this disease in black African people.We carried out a prospective study of 6 months where melanoderms adults presenting subjective symptoms of dry eye disease according a questionnaire of 7 items were included. Then the measure of BUT is performed after ocular instillation of fluorescein eyedrop. This measured is done by recording the time elapsed from the last complete palpebral blink to the appearance of the first dry spot. Patients with subjective symptoms and time of BUT lower than 10seconds were called concordants (suffering really of DED). The discrepant group was represented by a BUT upper than 10seconds despite the fact that these patients answered positively to the questionnaire.This survey included 234 patients and the average age was 49.1 years. Sex-ratio was 0.68 and we noticed predominance of BUT between 5 and 10seconds in 42.3% of cases. Subjective symptoms were strengthened clinically by a BUT lower to 10seconds in 149 patients. Therefore, the reliability of BUT as clinic tool for the diagnosis after symptoms in relationship with DED was 63.7%. In the group of concordants, women were more likely than men to experience symptoms of DED (P=0.0005). Age upper than 50 years and computer usage were risk factors of DED (P0.01).Dry eye disease is a multifactorial affection of tear and ocular surface frequently seen in female and people using computer and older than 50. The reliability of BUT near to 65% is a reason to perform this test in our daily practice.Tear BUT is a reliable clinical test which must be performed systematically to assess the diagnosis of DED in black African people.

To measure lacrimal secretion and to investigate dry eye symptoms in chronic hemodialysis patients.This was a cross-sectional descriptive study carried out in the hemodialysis center of Douala General Hospital from June to August 2016. The degree of symptomatology related to dry eye was assessed by the Ocular Surface Disease Index (OSDI) questionnaire. Lacrimal secretion was measured by the Schirmer test without anesthesia. We defined normal as wetting10mm and hyposecretion as a value≤10mm after 5minutes.Thirty-five patients were involved in the study, 16 men (45.7%) and 19 women (54.3%) for a total of 70 eyes examined. The mean age was 41.9 years, with an average duration of dialysis of 35.5 months±30.2. The symptoms were "normal" for 21 patients (60%), "minimal" for 5 (14.3%), "moderate" for 8 (22.9%) and "severe" for 1 (2.8%). Lacrimal hyposecretion was found in 19 eyes (27.1%). It was severe in 8 eyes. A correlation was found between diabetes, gender and hyposecretion.Hemodialysis patients are likely to develop lacrimal hyposecretion mostly because of comorbities. It is therefore important to systematically evaluate lacrimal secretion in these patients.

All the components of the ocular surface and the lacrimal system are affected by aging. Aging induces lacrimal gland fibrosis, Meibomian gland dysfunction, loss of corneal sensitivity, decreased corneal cell density, impairment of immune defences, increased local inflammation associated with hormonal changes, conjunctivochalasis, lid abnormalities, etc. Furthermore, homeostasis of the ocular surface may be altered by various age-related systemic comorbidities and iatrogenic interventions. Altogether, aging is considered the most predominant risk factor for dry eye disease. The increasing knowledge of the pathophysiology of aging of the ocular surface allows for refinement of the management of ocular surface disease in the elderly.

Dry eye disease is a multifactorial disease affecting the lacrimal functional unit and which has a significant impact on the quality of life of patients. This pathology works as a vicious circle at the ocular surface in which hyperosmolarity of the tear film plays a key role. This review intends to describe the different reported intracellular effects induced by hyperosmolarity in cells: alteration of cytoskeleton, cell cycle slowdown, adaptation mechanisms triggered as restoration of cell volume and accumulation of compatible osmolytes, the crucial role of the osmoprotectant factor Nuclear Factor of the Activated T cells-5 (NFAT5), apoptosis, as well as oxidative stress and inflammatory responses caused by this particular condition. Reported effects of hyperosmolarity in the experimental studies specific of dry eye disease concerning ocular surface cells will be described in parallel. Indeed, these data allow to understand a part of the pathophysiology of the disease, and specially the links between tear hyperosmolarity and inflammation of the ocular surface, the second key of the pathology phenomenon.

Lacrimal system injuries represent a significant part of ocular emergencies and mainly affect males of various ages including very young children. The most frequent presentations are canalicular laceration with a palpebral wound medial to the lacrimal punctum. The inferior canaliculus is the most commonly affected but bilateral injuries or injuries affecting both canaliculi can occur. The main causes are dog bites in children, scuffles in young adults and falls in elderlies. Antitetanic and antirabic measures have to be considered for open cases. The diagnosis is easily made by inspection and can be confirmed by probing. Other lesions can be associated and require proper identification, especially eyeball laceration requiring surgical repair within 6 hours. Otherwise, isolated canalicular wounds are to be repaired within 48 hours by an anastomotic suture with or without mono- or bi-canalicular silicone intubation. Other lacrimal tract injuries involving the lacrimal sac or the nasolacrimal duct are rare, commonly associated with blunt craniofacial trauma or iatrogenic after some surgical procedures.

Congenital dacryocystoceles are rare and often misunderstood pathologies. Their treatment varies and consists in simple follow-up, lacrimal catheterization or endoscopic surgical drainage, depending on medical teams. The aim of our study was to discuss the place of endoscopic drainage in the treatment of congenital dacryocystocele.We conducted a retrospective review on 18 cases of congenital dacryocystoceles taken in charge in a tertiary care center between 2009 and 2012.Thirteen newborns, including five bilateral cases, were taken in charge. The average age was 14.6 days. Six newborns presented with an acute dacryocystitis at the time of diagnosis. No respiratory complications were observed. Spontaneous drainage of the dacryocystocele was observed in 38.8% of the cases, occurring at 22 days of life on average. Endonasal endoscopic drainage was performed in 66.6% of the cases. No recurrence or complication was observed after surgery. After spontaneous drainage, one recurrence was observed. The mean follow-up period of these patients was 8.8 months.Spontaneous drainage is common. Conservative management may therefore be considered in absence of infection. In case of infection and/or persistence of dacryocystocele after 4 weeks of life, endonasal surgical drainage should be considered. Imaging of the facial structure should be performed before any surgical treatment.

Lacrimal cysts are rarely described and must be differentiated from diseases of the lacrimal gland. We present a rare case of lacrimal cyst developed from an accessory lacrimal gland.The management consisted of excision using a tarsoconjonctivale approach.Most cysts are diagnosed from the third decade, manifested by progressive exophthalmos. It is a benign disease. However, the differential diagnosis with malignancy is not excluded. Surgical resection is desirable if tumors become symptomatic.

La dacryoadénite tuberculeuse est une inflammation rare de la glande lacrymale causé par le bacille Mycobacterium tuberculosis. Elle pose un problème étiopathogénique et diagnostique. Nous rapportons dans cette observation le cas d'une dacryoadénite tuberculeuse bilatérale chez un jeune homme Marocain de 34 ans ayant présenté une tuberculose ganglionnaire et du cavum il y à 14 ans, ayant été confirmé par l'examen anatomopathologique. La tuberculose reste un diagnostic qui doit être toujours évoqué même dans les atteinte bilatérale surtout si il y a un antécédent personnel ou familiale positif de tuberculose. Le pronostic de cette affection est devenu favorable grâce au traitement antibacillaire précoce.

Epiphora is frequently related to stenosis of the lacrimal drainage pathways. In the evaluation of stenosis, dacryo-CT scan remains the gold standard, despite the need for radiation and catheterization of the lacrimal passages. Evaluation by high field 3T MRI compared to the gold standard in the morphological study of the lacrimal passages and quantification of the stenosis is attractive considering the lack of radiation and non-invasive nature of the technique.Twenty-four patients were included, including 9 with bilateral epiphora, representing 33 pathological drainage systems out of 48. Twenty-three drainage systems underwent subsequent surgery (18 patients, 5 patients bilaterally).The average diameter on CT scan images of the superior canaliculus was 0.70 mm (± 0.46), of the inferior canaliculus 0.69 (±0.42), the common canaliculus 0.68 (± 0.58), the lacrimal sac 4.32 (± 2.10), and the nasolacrimal duct 1.15 mm (±1.42). 3T dacryo-MRI overestimated the diameters by 0.35 to 1mm (up to 20 % of the lacrimal sac size), and the concordance between dacryo-CT scan and 3T dacryo-MRI was of average value (kappa 0.5, P0.05) concerning the diagnosis of stenosis. Furthermore, dacryo-CT scan demonstrated higher sensitivity (72.7 %) than 3T dacryo-MRI (42.4 %).The two techniques are not equivalent in the diagnosis of stenosis. An optimization of protocols and an evaluation on a larger cohort remain necessary before dacryo-CT scan can be replaced by dacryo-MRI in routine practice.

To compare autonomic nervous system cardiovascular tests (ANSCVT) and lacrymal and or salivary secretion tests (LSST).One hundred and twenty-four patients (57 females, 67 males, mean age 45) with lower urinary tract symptoms (overactive bladder syndrome or voiding dysfunction) without neurogenic or urological causes, were included. Urodynamic was performed in all the patients with LSST (saliva flowmetry-sialometry [SFS], candy weight-loss test [CWT], Schirmer test [SchT]) and ANSCVT (ratio 30:15, orthostatic hypotension analysis, deep breath test [DBT], Valsalva maneuver [VM], cold pressor test [CPT] and hand grip test [HGT]).In eight cases, ANSCVT were altered (four in OAB syndrome, four in voiding dysfunction). No correlation was found between positive ANSCVT and LSST (P=0.72 for CWT, P=1 for SFS, P=0.1 for SchT). In contrary, there is a significant correlation between SchT and CWT (P=0.049), but not between SchT and SFS (P=0.69) or SFS vs CWT (P=0.06).In this series LSST were not sufficient to track down autonomic nervous system alterations and ANSCVT always necessary. Autonomic dysfunctions were infrequent in OAB syndrome and "idiopathic" voiding dysfunctions (6.5%).

Myoepithelial carcinomas are unusual tumors most often located in salivary glands. It is very rarely located in lacrimal glands; only 5 cases have been reported. We report a sixth case.An 88-year-old male patient presented with diplopia, painless right sided exophthalmia, as well as eyeball deviation due to a tumor located at upper external quadrant of the orbit. A biopsy initially suggested a sarcoma. The pathological analysis of the biopsy allowed diagnosing a myoepithelial carcinoma of the lacrimal gland. Despite the monoblock resection of the tumor, a recurrence was observed 3 months after removal. The patient died 8 months after the initial surgery.This case illustrates the clinical and pathological characteristics of a myoepithelial carcinoma. This tumor has a high grade of malignancy, and is very rarely described in lacrimal glands. The morphological diagnosis of this tumor is difficult with a problematic differential diagnosis with fusiform cells sarcomas (leiomyosarcoma, undifferentiated sarcoma), and epithelial-myoepithelial carcinoma.

Hydrocystoma is a benign tumor whose development involves the sudoriferous eccrine or apocrine glands. We report the case of a giant apocrine hydrocystoma in a 70-year-old female diabetic patient. The tumor's natural progression over 10 years and its volume caused significant functional and aesthetic damage. This article discusses the radiological, clinical, anatomopathological, and therapeutic aspects of this tumor. The case is original in the tumor's location, size, and its double visual and lachrymal functional involvement as well as its aesthetic damage.

The ideal approach to orbital medial wall fractures remains controversial. Only the coronal approach exposes the medial wall completely. The transcaruncular approach enables a clear and wide view of the medial wall without any visible scar compared to usual transcutaneous techniques. This approach can be combined with a transconjunctival approach, thus providing an extended exposure of the whole medial wall and the orbital floor.The conjunctival incision is performed with a needle electrocautery in the groove between the plica semilunaris and the caruncle. It is extended laterally within the inferior conjunctival fornix for 20mm to the lateral palpebral commissure. The sub-conjunctival dissection to the posterior lacrimal crest is performed with Stevens scissors through an avascular plane along the medial wall behind Horner's muscle. The periosteum is incised behind the posterior lacrimal crest. The sub-periosteal periorbital dissection is continued medially thus exposing the insertion of the inferior oblique muscle which is coagulated and cut over the inferior orbital rim. Reconstruction is performed after exposing the complete fracture after freeing the periorbital tissue. The periorbita is sutured with interrupted 5-0 Vicryl stitches. The inferior conjunctiva and the caruncle are closed with a continuous 6-0 Maxon suture.

The radiation dose received by the eye depends on the pathology, tumour location (ocular globe, orbit, neighboring structures) and the radiation technique. The major complication is the complete loss of vision, which is often multifactorial. This article, based on a literature review, describes the radiation effects and the tolerance doses for all eye structures: cornea, lens, retina, optic nerves, orbit and ocular adnexa.

In this chapter, we report the results of orbital tumor management in a few neurosurgical departments and compare it to a Paris neurosurgical department that has developed a close relation with an ophthalmological department. These departments' activity is quite low, treating mainly sphenoorbital meningiomas. Other tumor groups are unequally and sporadically managed.

Epithelial tumors of the lacrimal gland account for 50% of the expansive lesions of the lacrimal fossa. Pleomorphic adenomas have a good prognosis if surgery achieves en bloc removal distant from the tumoral capsule. Nevertheless, despite more severe treatment, the average survival remains limited for adenocarcinomas and cylindromas.

Knowledge of orbital tumor classification is essential because it is the basis of an optimal therapeutic strategy. These tumors comprise the sphenoorbital meningiomas, the optic nerve sheath meningiomas, the optic nerve gliomas, the schwannomas, the histiocytic tumors, the metastases, the lacrimal gland tumors, the mesenchymal tumors of the soft tissues, the primary orbital wall tumors, the tumors arising from the nasal and paranasal sinuses, the hematopoietic tumors, the vascular tumors, and the congenital tumors.

CT dacryocystography, performed under sterile technique and following local anesthesia, is well tolerated by patients. It is the imaging technique of choice in patients with persistent tearing following clinical examination with non-invasive procedures when patency of the lacrimal drainage system remains uncertain, when a dacryolith or tumor is suspected or following unsuccessful dacryocystorhinostomy. Two techniques are available: instillation or direct cannulation, either initially or after non-visualization of the nasolacrimal system after contrast instillation. Non-specific idiopathic stenosis at the mid portion of the nasolacrimal duct is the most frequent etiology in patients over 50 years old; dacryoliths are present in 5 to 20% of cases of nasolacrimal duct stenosis. CT dacryocystography also allows evaluation of surrounding structures (medicolegal) to identify variants and anomalies when surgery is contemplated to avoid potential complications.

This clinical case report describes the clinical findings and diagnosis of lacrimal gland adenocarcinoma that developed 20 years after external beam radiotherapy in the treatment of bilateral retinoblastoma. Visual acuity, slit lamp biomicroscopy, fundus color photography, nuclear magnetic resonance, lateral orbitotomy and histological analysis are described.

We report the case of a 21-year-old patient who presented with epiphora in the left eye and a bilateral ocular motility problem. The clinical examination revealed bilateral moderate abduction limitation (impairment of the VIth cranial nerve), facial diplegia with amimic face (impairment of the VIIth cranial nerve), punctal agenesis of the left eye, absence of the left pectoralis major muscle, left breast aplasia, and hypoplasia of the left upper limb and hand. Neither the lacrimal puncta nor the canaliculi could be found during surgical exploration of left lacrimal system using operating microscope. Based on these findings, the patient was diagnosed with Poland-Möbius syndrome associated with punctal and canalicular agenesis. Poland-Möbius syndrome is a rare entity characterized by the association of two different syndromes: Poland syndrome and Möbius syndrome. Several abnormalities in association with this syndrome have been published. However, punctal and canalicular agenesis is not among these reported abnormalities.

The authors report the different palpebral and lacrimal wounds found in children and their treatment. Early microscopic surgery is important for good prognosis. The eyelids must be thoroughly examined searching for a marginal injury, superior palpebral levator muscle resection, and lacrimal system injury after ocular injuries have been investigated. The treatment is microscopic surgery of the eyelid and the canaliculus with most often a monocanalicular intubation. The authors present two clinical studies conducted in the Reims Hospital that show the frequency of this traumatology and the main characteristics. In children, most injuries come from dog bites with canalicular laceration in the left lower eyelid. In palpebral injuries without canalicular section, the injury was most often located on the right lower eyelid. Preliminary treatment of childhood palpebral and lacrimal injuries is necessary with microscopic surgery. It is important to repair the canaliculus with or without canalicular intubation and the eyelid margin.

MALT lymphoma of the lacrimal gland is rare. Orbital involvement is usually located on the anterior orbit. We report the case of a 65-year-old man consulting for an eyelid tumor located on the right upper and lateral lid, which clinically recalled lacrimal gland involvement. The gland biopsy and the immunohistochemical analysis concluded in the diagnosis of B and MALT lymphoma. The assessment of the ganglion and extra-ganglion enlargement of these lymphomas is of capital importance in treatment. A multidisciplinary analysis, especially oncological, is important so that the best therapeutic plan can be adopted.