Congenital Nasolacrimal Duct Obstruction - Early Diagnosis and Graded Therapeutic Approach as Key Points for Successful Management.

PURPOSE: Epiphora in childhood is a frequent symptom that is typically associated with Congenital nasolacrimal duct obstruction (CNLDO). Nevertheless, inflammatory pathologies of the ocular surface as well as inside the eye, or even congenital glaucoma, must be considered in the differential diagnosis. METHODS: A comprehensive literature review concerning CNLDO was conducted. Different therapeutic steps are categorized and summarized in order to reflect the existing staged therapeutic concept. RESULTS: For CNLDO, a staged therapeutic concept is applicable, resulting in a cure rate of approximately 95% with only conservative or minimally invasive intervention. This concept includes five steps that encompass therapeutic interventions with increasing complexity. It includes conservative techniques, followed by probing and syringing, transcanalicular approaches without or with lacrimal intubation, and dacryocystorhinostomy which is the ultima ratio. CONCLUSION: To preserve the topographic anatomy as much as possible, therapeutic recommendations enable stepwise and individualized management of children with CNLDO.

Augenverätzungen.

Chemical burns of the ocular surface (CBOS) are emergencies of highest urgency. Therefore, an adequate emergency care is mandatory. Following a precise analysis of the initial damage, a staged therapeutic approach is used to prevent persistent impairment of the ocular surface. In the acute stage, the prevention of complications is targeted (symblepharon, conjunctival scarring, lacrimal stenosis, corneal ulceration, intraocular inflammation, elevated intraocular pressure, etc.). In later stages, if complications have developed, a secondary restoration of the ocular surface is focussed. Sometimes this requires several surgical interventions. Based on a review of international literature, this review highlights the pathophysiology according to different chemical agents, CBOS stages as well as main therapy strategies in early and advanced stages of CBOS. Acute treatment aims to lower inflammation, oxidative stress and tries to promote reepithelialisation. Besides conjunctival scarring, loss of goblet cells and corneal opacification a limbal stem cell insufficiency is the most harming complication. Several new techniques have been developed to recover the ocular surface with a sufficient and clear epithelial layer in order to avoid neovascularization of the cornea. The knowledge concerning the high risk potential for persistent visual impairment in CBOS patients and the ability for appropriate emergency care should be kept in every physician's mind dealing with CBOS.

Dacryolithogenesis or Dacryolithiasis-The Story So Far.

The term dacryoliths refers to the concretions found within the lacrimal system. When the term dacryoliths is unspecified, it usually refers to the noninfectious dacryoliths commonly isolated from the lacrimal sac and the nasolacrimal duct. More often, they are diagnosed incidentally during a dacryocystorhinostomy, and the reported incidence among all dacryocystorhinostomy surgeries is 5.7% to as high as 18%. Dacryolithiasis is a complex process occurring within the lacrimal system, and current evidence suggests a multifactorial etiology. The sequence of events can be summarized broadly into 4 stages: stage of susceptibility, stage of initiation/trigger, stage of development, and stage of maintenance. The triggering event is the breach of the lacrimal sac or nasolacrimal duct epithelium, resulting in microtrauma with blood leakage. The blood clots act as a nidus for subsequent sequential laying of mucopeptides, cellular debris present locally, debris washed from the ocular surface, and extraneous agents in tears. This process is aided by altered rheology and composition of the tear film. After the formation of dacryoliths, extracellular neutrophil traps usually form on the surface, which help to maintain the dacryoliths (which do not dissolve). This review highlights and discusses the possible sequence of events during dacryolithiasis.

Transcutaneous (External) Dacryocystorhinostomy with Reconstruction of the Ductus nasolacrimalis. / Transkutane (externe) Dakryozystorhinostomie mit Rekonstruktion des Ductus nasolacrimalis.

BACKGROUND: Dacryocystorhinostomy (DCR) is regarded as the gold standard for the treatment of chronic dacryocystitis (CDC). Different modifications of the surgical procedure have been developed over the years. METHODS: Patients with CDC due to postsaccal lacrimal stenosis and under treatment with DCR have been included in this retrospective study. Two groups of different surgical procedures were analysed: firstly DCR without reconstruction of the ductus nasolacrimalis (DNL, group 1) and secondly patients with reopening the DNL (group 2). Criteria for success were absence of permanent epiphora, absence of inflammation of the lacrimal sac (functional success), and no recurrent surgery with free patency of the lacrimal duct (anatomical success). This was obtained by questionnaire after a follow-up of at least 12 months. The impact of gender, ectasia of the lacrimal sac, dacryoliths, and prior lacrimal surgeries was analysed. RESULTS: Overall, 248 surgeries were enrolled in this trial. Mean age in group 1 was 68 years (range: 22 to 92 years) and gender ratio was 3.2 to 1 (female : male). In that group, 68 operations could be followed up. In group 2, 62 operations on patients of a mean age of 63 years (range: 24 to 89 years) and a gender ratio of 2.9 to 1 (female : male) were observed. Complete success occurred in 75.0% in group 1 and 75.8% in group 2. Recurrent operations were necessary in one case of group 2 (1.6%) and 4 cases of group 1 (5.9%). Gender (group 1 p = 1; group 2 p = 0.115; between groups p = 0.511), ectasia of the lacrimal sac (group 1 p = 0.877; group 2 p = 0.674; between groups p = 0.878), dacryolith (group 1 p = 1; group 2 p = 0.465; between groups p = 1), and prior lacrimal surgery (group 1 p = 0.092; group 2 p = 0.051; between groups p = 0.520) did not influence the success rates in each group or between groups. Significantly more dacryoliths were found during operations of group 2 (p = 0.010). CONCLUSION: Reconstruction of the DNL during DCR is a possible and easy modification, with a slightly better success rate in curing CDC. Intraoperatively, dacryoliths might not be apparent remain in the deeper parts of the lacrimal ducts. Therefore, these segments should be inspected during surgery.

Pressure-Lowering Effect of Interventional Fistula Occlusion in Secondary Glaucoma due to Orbital Draining Intracerebral Fistulae. / Drucksenkender Effekt der interventionellen Fistelokklusion bei Sekundärglaukom infolge orbital drainierender intrazerebraler Fisteln.

BACKGROUND: Carotid cavernous sinus fistulas (CSCF) are pathological connections of the internal and/or external carotid artery (and/or its branches) to the cavernous sinus. Ophthalmological symptoms and problems occur particularly when drainage is via the superior ophthalmic vein. MATERIAL AND METHODS: Seven eyes of six patients with a high-grade suspicion of CSCF were included in this retrospective monocentric study. Digital subtraction angiography (DSA) was performed in the included patients, where an interventional fistula closure was performed in the case of CSCF. Four of the six patients received a pre- and post-interventional day-night intraocular pressure profile. Furthermore, medical history, symptoms, visual acuity, slit lamp microscopic findings, and DSA findings were evaluated. RESULTS: The most common symptoms reported by patients were red eyes, diplopia, and exophthalmos. When the intraocular pressure (IOP) was measured, 83.33% of the patients showed increased values. The mean IOP in the day-night intraocular pressure profile in the affected eye before intervention was 23.5 (± 2.7) mmHg compared to 14.1 (± 2.3) mmHg in the healthy eye. A significant difference could thus be demonstrated in side comparison (p = 0.0047). The post-interventional measurement showed a mean IOP of 15.3 (± 1.0) mmHg in the affected eye and thus a significant difference to the pre-interventional measurement in the affected eye (p = 0.0018). Four of the six patients with CSCF were taking antiglaucomatous eye drops before the intervention, and two patients after the intervention. The number of antiglaucoma drugs used could also be reduced. CONCLUSION: Interventional fistula closure is an effective method for treating the secondary increase of IOP in CSCF. Successful closure of the fistula showed a significant reduction in IOP, which was not possible with the sole administration of antiglaucoma drugs. Radius-Maumenee syndrome should be considered as a differential diagnosis.

[Concrements of the lacrimal apparatus]. / Konkremente des Tränenapparates.

Concrements of the lacrimal apparatus, known as dacryoliths, can occur at different localizations and can cause a variety of symptoms. A common clinical sign is chronic inflammation, possibly exhibiting acute exacerbation. Based on a literature review and descriptive clinical cases with histopathological correlations, this contribution summarises the most important information concerning epidemiology, aetiopathogenesis, composition, histology, and therapy of lacrimal concrements. Furthermore, factors known to affect lacrimal lithogenesis are addressed. Concrements of the lacrimal gland cause a swelling at the lateral canthus. With only mild pain, this manifests as circumscribed conjunctival hyperaemia. Histologically, the gland tissue is characterised by acute-erosive to chronic inflammation. The concrements consist of amorphic material. Inflammatory infiltration is dominated by neutrophil granulocytes. Canalicular concrements are highly correlated with chronic canaliculitis. Besides epiphora, patients present with purulent discharge at the affected canaliculus. Actinomyces are frequently found inside these deposits and form drusen-like formations. The surrounding tissue reacts with plasma-cellular and granulocytic inflammation. Dacryoliths (concrements of the lacrimal sac) are associated with dacryocystitis, whereby acute and chronic types are common. Stones can be found in up to 18% of patients undergoing dacryocystorhinostomy or dacryoendoscopy. Preoperative diagnostic testing is challenging, as many lacrimal sac stones cannot be reliably visualised by diagnostic procedures. Recurring episodes of epiphora, mucopurulent discharge, and dacryocystitis are common indicators of dacryoliths. Lacrimal syringing is often possible and shows that total blockage is not present. Histology of the lacrimal mucosa reveals lymphocytic infiltration and submucosal fibrosis. The immediate vicinity of the dacryoliths shows acute inflammation. Therapy consists of stone extraction and improving lacrimal drainage, as the latter is recognised as the main risk factor for dacryolith formation.

Neutrophil Extracellular Traps Drive Dacryolithiasis.

Mucopeptide concretions, previously called dacryoliths, are macroscopic stones that commonly obstruct the lacrimal sac. The mechanism behind dacryolithiasis remains unclear; however, the involvement of various immune cells, including neutrophils, has been confirmed. These findings remain limited, and no information on neutrophil extracellular traps (NETs), essentially involved in the pathogenesis of other lithiases, is available yet. Here, we employ microcomputed tomography, magnetic resonance tomography, histochemistry, mass spectrometry, and enzyme activity analyses to investigate the role of neutrophils and NETs in dacryolithiasis. We classify mucopeptide concretions into three types, with respect to the quantity of cellular and acellular material, polysaccharides, and mucosubstances. We propose the role of neutrophils and NETs within the existing model of gradual formation and growth of mucopeptide concretions, with neutrophils contributing to the initial stages of dacryolithiasis, as they localized on the inner (older) parts of the tissue. As NETs localized on the outer (newer) parts of the tissue, we link their role to the late stages of dacryolithiasis, presumably maintaining the proinflammatory environment and preventing efficient clearance. An abundance of IgG on the surface indicates the involvement of the adaptive immune system later as well. These findings bring new perspectives on dacryolithiasis, in which the innate and adaptive immune system are essentially involved.

[Congenital anomalies in lacrimal drainage]. / Angeborene Fehlbildungen der ableitenden Tränenwege.

Congenital dacryostenosis is the most common reason for ophthalmic consultation in childhood. It is most often caused by persisting of Hasner's membrane. However, congenital malformations of the lacrimal drainage system can also occur in rare cases. In the area of the proximal lacrimal drainage system, supernumerary lacrimal puncta and canaliculi can arise as well as diverticula, fistula, and atresia. The distal lacrimal drainage system can be affected by fistulas, amniotoceles, and cysts. Association of lacrimal malformations with congenital systemic diseases is reported in about 10% of cases. Depending on the severity of the symptoms, surgical rehabilitation can be performed, and endoscopic procedures and modern lacrimal drainage intubation systems may be employed.

Concrements of the Lacrimal Apparatus. / Konkremente des Tränenapparates.

Concrements of the lacrimal apparatus, known as dacryoliths, can occur at different localizations and can cause a variety of symptoms. A common clinical sign is chronic inflammation, possibly exhibiting acute exacerbation. Based on a literature review and descriptive clinical cases with histopathological correlations, this contribution summarises the most important information concerning epidemiology, aetiopathogenesis, composition, histology, and therapy of lacrimal concrements. Furthermore, factors known to affect lacrimal lithogenesis are addressed. Concrements of the lacrimal gland cause a swelling at the lateral canthus. With only mild pain, this manifests as circumscribed conjunctival hyperaemia. Histologically, the gland tissue is characterised by acute-erosive to chronic inflammation. The concrements consist of amorphic material. Inflammatory infiltration is dominated by neutrophil granulocytes. Canalicular concrements are highly correlated with chronic canaliculitis. Besides epiphora, patients present with purulent discharge at the affected canaliculus. Actinomyces are frequently found inside these deposits and form drusen-like formations. The surrounding tissue reacts with plasma-cellular and granulocytic inflammation. Dacryoliths (concrements of the lacrimal sac) are associated with dacryocystitis, whereby acute and chronic types are common. Stones can be found in up to 18% of patients undergoing dacryocystorhinostomy or dacryoendoscopy. Preoperative diagnostic testing is challenging, as many lacrimal sac stones cannot be reliably visualised by diagnostic procedures. Recurring episodes of epiphora, mucopurulent discharge, and dacryocystitis are common indicators of dacryoliths. Lacrimal syringing is often possible and shows that total blockage is not present. Histology of the lacrimal mucosa reveals lymphocytic infiltration and submucosal fibrosis. The immediate vicinity of the dacryoliths shows acute inflammation. Therapy consists of stone extraction and improving lacrimal drainage, as the latter is recognised as the main risk factor for dacryolith formation.

On the Current Care Situation and Treatment of Ocular Mucous Membrane Pemphigoid in Germany. / Zur Versorgungssituation und konservativen Therapie des okulären vernarbenden Schleimhautpemphigoids in Deutschland.

BACKGROUND: Ocular involvement in mucous membrane pemphigoid (MMP) is relatively rare, with a prevalence of 25 cases per million population, equating to approx. 2,100 patients throughout Germany. Diagnosis can be difficult - especially in cases of isolated ocular involvement - and treatment can be complex and lengthy. Immunosuppressants or immunomodulatory drugs are often used. Due to the complexity of diagnosis and treatment, MMP patients are usually referred to specialized centers. The aim of this project was to evaluate the current care situation of patients with ocular MMP in Germany. METHODS: A paper-based survey was designed and sent to all university eye clinics and other specialized centers in Germany in April 2020. The survey asked about the existence of a specialized outpatient service, the total annual number of patients with MMP, the annual number of newly diagnosed patients, any interdisciplinary collaboration for diagnostic or therapeutic purposes, as well as the local and systemic therapy used. RESULTS: Of a total of 44 clinics, 28 (64%) responded, reporting a total average of 27 ± 42 (0 - 200) patients and 3.6 ± 2.2 (0 - 10) new cases per year. This corresponds to a total of 741 patients. Only nine (32%) of the responding clinics offer specialized MMP clinics. 93% of the centers collaborate with the local dermatology department. 79% perform serological and histological diagnostics in-house. About half of the centers (n = 16) apply a standardized treatment regime. Systemic glucocorticoids (66.7%) are most commonly used, followed by mycophenolate mofetil and dapsone (57.1%), rituximab (33.3%), azathioprine and cyclophosphamide (28.6%), as well as methotrexate (19.0%). The least frequently used treatment is intravenous immunoglobulin (14.3%). CONCLUSION: This survey of German ophthalmology departments obtained data from about one third of the estimated total cohort of all patients with MMP in Germany. These are presumed to be exclusively patients with at least one ocular involvement. The complex care of these patients is usually provided in collaboration with a dermatologist and with the use of systemic anti-inflammatory medication. Currently, an ophthalmological MMP register is being established to better record the epidemiology and care situation of this rare disease in Germany and to improve it in the long term.

Congenital Anomalies in Lacrimal Drainage. / Angeborene Fehlbildungen der ableitenden Tränenwege.

Congenital dacryostenosis is the most common reason for ophthalmic consultation in childhood. It is most often caused by persisting of Hasner's membrane. However, congenital malformations of the lacrimal drainage system can also occur in rare cases. In the area of the proximal lacrimal drainage system, supernumerary lacrimal puncta and canaliculi can arise as well as diverticula, fistula, and atresia. The distal lacrimal drainage system can be affected by fistulas, amniotoceles, and cysts. Association of lacrimal malformations with congenital systemic diseases is reported in about 10% of cases. Depending on the severity of the symptoms, surgical rehabilitation can be performed, and endoscopic procedures and modern lacrimal drainage intubation systems may be employed.

[Deep periocular trauma with affection of the lacrimal ducts during Caesarean section]. / Tiefes periokuläres Trauma mit Tränenwegsbeteiligung durch Kaiserschnittentbindung.

Periocular injuries during a caesarean section are extremely rare. The case report of a five hour old newborn addresses the trauma management concerning diagnostics, therapy, and post-operative care of a deep periocular soft tissue injury. The pattern of injury consisted of full thickness wounds of the medial and lateral lid margins, opening of the superior conjunctival fornix, and penetration of Tenon's capsule. The reconstruction was performed layer by layer while an autostable monocanaliculonasal lacrimal intubation was inserted.

Rare Diseases of the Orbit. / Seltene Erkrankungen der Orbita.

This article provides an overview of rare orbital diseases. Congenital malformations, inflammatory diseases, benign and malignant neoplasias are described. Although it represents a relatively small area of the body the orbit contains multiple different tissues. Therefore, a great variety of diseases can be found within the orbital space. That is the reason, why both the completeness and the level of detail in the description of particular diseases must be somewhat limited. Nevertheless, clinical manifestations, important aspects of diagnosis, treatment strategies, and, when specific data are available, the prognosis are described. The authors tried to highlight the most characteristic aspects of the different diseases to describe their relevant aspects in spite of the brevity of the subsections.

[Primary Reconstruction of Lacrimal Ducts after Trauma - Bicanaliculonasal Intubation Using Autostable Tubes]. / Primäre Rekonstruktion der ableitenden Tränenwege nach Trauma ­ bikanalikulonasale Schienung mit autostabilen Silikonverweilsonden.

Trauma of the medial canthal region can affect all parts of the lacrimal ducts. Depending on the patient's age, there may be different mechanisms of injury. Surgical reconstruction using two autostable ("self-retaining") monocanaliculonasal lacrimal tubes is demonstrated by two case reports. Here, a 6 years old girl attacked by a dog (Rottweiler) showed severe periocular soft tissue damage with deep involvement of the lacrimal ducts. The child's inferior canaliculus was lacerated. Furthermore, the common canaliculus was dissected from the lacrimal sac. Deep soft tissue damage consisted of blunt and sharp injuries down to the cheek and nose. After lacrimal intubation with two autostable monocanaliculonasal tubes, the soft tissue was reconstructed by direct canalicular suturing and stepwise closure of each layer. One of the lacrimal tubes was fixated at the lid by additional sutures, in order to avoid stent prolapse. Beside slight medial depression of inferior lid, there were no signs of functional disturbance of the lacrimal ducts. The follow-up was 18 months. The second case report presents a 43 years old man. He accidentally cut himself with a butcher's knife. He suffered a deep wound of his medial canthal region. The patient presented with proximal (located near the lacrimal sac) laceration of both canaliculi. The medial canthal ligament as well as the orbicularis muscle had been cut. Using two autostable monocanaliculonasal lacrimal tubes, the lacrimal ducts were stented and reconstructed with sutures. During the follow-up of 6 months, no epiphora or mucopurulent lacrimal discharge occurred. In lacrimal trauma, different parts of the lacrimal ducts may be affected at the same time. Identification of the injured structures with respect to the kind of injury, localisation and the extent of damage is crucial. Stepwise wound closure in respect to the topographic anatomy is mandatory. Here, lid and lacrimal ducts must be respected as one functional unit. Simultaneous use of autostable monocanaliculonasal lacrimal tubes in both canaliculi can be applied for trauma reconstruction of the lacrimal ducts in children and adults.