Enfermedad inflamatoria orbitaria idiopática / Orbital inflammatory disease

La enfermedad inflamatoria orbitaria idiopática (EIOI), comúnmente conocida como pseudotumor orbitario, es una enfermedad inflamatoria de etiología desconocida. Sus síntomas pueden ser muy variables tanto en intensidad, gravedad, formas de presentación o gravedad. Esta heterogeneidad ha condicionado que sea una entidad difícil de definir y clasificar. El pronóstico de la EIOI depende de su localización, presentación e histología. La EIOI suele responder favorablemente a los corticoides sistémicos, sin embargo, este hecho puede hacer que la entidad sea confundida con otras enfermedades que también tienen buena respuesta a corticoides, como la enfermedad relacionada con la IgG4 y las enfermedades linfoproliferativas. Esta controversia ha alzado una polémica entre autores que defienden la realización de biopsia previa al tratamiento en la mayoría de los casos, frente a otros que afirman que la biopsia debe indicarse en lesiones que no responden adecuadamente al tratamiento médico empírico. Si bien los corticoides se sitúan como los protagonistas de la EIOI, los efectos secundarios, las tasas de recidivas y la falta de respuesta de algunos subtipos han permitido el paso a agentes inmunosupresores e inmunomoduladores que ocupan un escalón fundamental en la terapia combinada o ahorradora de corticoides, junto con la radioterapia y la cirugía. El objetivo de esta revisión es actualizar la evidencia sobre el diagnóstico y tratamiento de la EIOI.

Orbital inflammatory disease (OID), commonly known as orbital pseudotumour, is an inflammatory disease of unknown cause. It has different forms of presentation and different degrees of severity. Its variable nature is the main cause for this disease to be misdiagnosed and misclassified. The prognosis of OID depends on the tissues affected and the histology. OID usually responds favourably to systemic steroid treatment. However, empiric steroids may mask other underlying diseases that respond well to this treatment as well, namely, IgG4-related disease or lymphoproliferative disorders. This fact has led to controversy among various authors as some recommend performing a biopsy in most of the cases, whereas others defend that this procedure should only be performed if the patient has not responded to empiric steroid treatment. Although steroids have been the mainstream treatment of OID, the side effects, relapse rates and lack of response in some cases have resulted in them being replaced by immunosuppressive and immunomodulator therapies that currently stand as a key steroid-sparing treatment option, in addition to radiotherapy and surgery. The aim of this review is to update the evidence on the diagnosis and treatment of OID.

Eye Protection for Patients With COVID-19 Undergoing Prolonged Prone-Position Ventilation.

Importance: Critically ill patients with coronavirus disease 2019 (COVID-19) who are unresponsive to maximum optimal ventilator settings may be in a prone position for at least 16 hours per day to improve oxygenation. This extended duration of prone positioning puts patients at risk of developing orbital compartment syndrome if direct pressure to the orbit and the globe occurs and concomitant protection of the eyes is not undertaken. Objective: To report 2 cases of orbital compartment syndrome, as well as optic disc edema and retinal hemorrhages, in the setting of prolonged prone positioning of patients in the intensive care unit during the COVID-19 pandemic. Design, Setting, and Participants: The cases took place from April 27, 2020, to May 4, 2020, at a COVID-19 intensive care unit of a tertiary care hospital. Four of 16 patients in the intensive care unit required prolonged prone-position ventilation. A bedside eye examination was performed on 4 selected patients due to the observed presence of substantial periorbital edema. Main Outcomes and Measures: Intraocular pressures and fundus findings of 4 patients with periorbital edema. Results: Two of 4 patients who were in the prone position for extended periods of time had bilateral fundoscopic findings of optic disc edema and retinal hemorrhages, possibly consistent with a papillophlebitis. Additionally, both patients had a substantial increase in intraocular pressure of 2- to 3-fold in the prone position compared with the supine position. Conclusions and Relevance: Prolonged prone positioning of patients with COVID-19 can be associated with elevated intraocular pressure from periorbital edema, direct compression on the eye, and increased orbital venous pressure. Orbital compartment syndrome can be avoided by the use of protective cushioning around the eyes and maintaining the patient's head position above heart level during prone positioning. Patients with COVID-19 may also develop papillophlebitis with optic disc edema and retinal hemorrhages, which may be associated with a hypercoagulable state caused by COVID-19. These observations suggest awareness for the possible presence of these ophthalmic findings while treating severely ill patients with COVID-19.

The intraoperative use of polydioxanone foil to reduce the risk of sino-orbital fistula formation in orbital exenteration.

BACKGROUND: Orbital exenteration is a radical surgical procedure resulting in deformity. It involves removal of the globe, optic nerve, extra-ocular muscles, orbital fat, lacrimal gland, and peri-osteum. Sino-orbital fistula (SOF) formation is a common documented post-operative complication, usually connecting the orbit and the ethmoid sinus. SOFs can cause leaks of serous fluid, and act as an entry site for pathogens into the orbit leading to socket infection and breakdown. METHODS: This retrospective study analyzed exenterations performed over a 22-year period (1993-2015) at the National Ocular Oncology Service Centre for Scotland. PDS is a crystalline, biodegradable polyether-ester that is strong with good shape-memory and flexibility. Orbital exenterations with and without the use of PDS foil were compared in terms of SOF formation. RESULTS: A total of 30 exenterations were performed during the study period. A total of 29 were analyzed. Choroidal malignant melanoma was the most common indication for performing orbital exenteration (n = 7, 24.14%). The most common post-operative complications seen were SOF (n = 8, 27.59%). A total of 8 out 21 (38.10%) cases not using PDS developed SOFs. By contrast, none of nine patients receiving PDS plates developed SOFs (p = 0.0332). CONCLUSIONS: This is the first study to compare SOF rate in patients undergoing exenteration with and without the use of PDS foil. PDS foil is a safe material, which has effectively reduced the incidence of SOF formation.

The use of nasal packing post rhinoplasty: does it increase periorbital ecchymosis? A prospective study.

BACKGROUND: Periorbital edema and ecchymosis following rhinoplasty is disturbing for both the patients and their surgeons. The study aim was to determine whether nasal packing after lateral osteotomies in rhinoplasty surgery increases the risk of periorbital ecchymosis post-operatively. METHODS: This was a prospective self-controlled single-blinded study. Seventy four patients who underwent rhinoplasty with bilateral lateral osteotomies by a single surgeon were enrolled in the study. Nasal cavity packing for one side was done while the other side was left unpacked. Periorbital ecchymosis was evaluated by the operating surgeon and a separate surgeon who is unaware of the packing side separately on the first, fourth and seventh day post-operatively. A 4-grade scale was utilized to assess the ecchymosis with grade 4 being the most severe. RESULTS: Nasal packing was found to significantly increase the severity and duration of periorbital ecchymosis post rhinoplasty. While no difference was observed between the packed and unpacked sides on the first post-operative day, significant difference was noted on the 4th day (mean score 2.36 and 1.15 for the packed and unpacked sides, respectively) and on the 7th day after surgery in favor of the unpacked side (score 1.24 and 0.61 for the packed and unpacked sides, respectively). CONCLUSION: We advise against the routine use of nasal packing in rhinoplasty unless necessary as it contributes to worsen the periorbital ecchymosis from lateral osteotomies and thereby increases the patients' "down time" after surgery.

Sphenoid sinus dehiscence as a risk for visual consequences in an immunocompromised patient.

Isolated sphenoid sinus disease is a rare entity with severe and potentially life threatening sequela. Because of the proximity of the sinus to the orbit, anatomical defects within the surrounding bony structures can facilitate communication with orbital content, predisposing the patient to substantial visual consequences. We report a case of a 51-year-old immunocompromised male who presented with headache and gradual unilateral decreases in vision. Computed tomography revealed opacification of the left sphenoid sinus accompanied by unusual bony dehiscence of the proximal optic canal. Early recognition and treatment of sphenoid sinusitis requires urgent surgical intervention with delay of treatment potentially leading to irreversible blindness or other devastating consequences. Bony dehiscence of the sphenoid sinus overlying the optic nerve has only been found in 4% of cadavers. It is associated with increased risk of orbital complications and predicts a poor prognosis. Immediate intervention is particularly important in immunocompromised individuals who are at greater risk of these severe complications.

Combination of absorbable mesh and demineralized bone matrix in orbital wall fracture for preventing herniation of orbit.

After restoration of orbit wall fracture, preventing sequelae is important. An absorbable mesh is commonly used in orbit wall fracture, yet it has limitation due to orbit sagging when bony defect is larger than the moderate size (1 × 1 cm2). In this study, the authors present a satisfactory result in treating orbit wall fracture larger than the moderate size with a combination of absorbable mesh and demineralized bone matrix.From 2009 to 2012, 63 patients with bony defect larger than the moderate size, who were treated with a combination of absorbable mesh and demineralized bone matrix, were reviewed retrospectively. The site of bony defect, size, and applied amount of demineralized bone matrix were reviewed, and a 2-year follow-up was done. Facial computed tomography scans were checked preoperative, immediate postoperative, and 2-year postoperative.Among the 63 patients, there were 52 men and 11 women. Mean age was 33.3 years. The most common cause was blunt blow (35 cases); mean defect size was 13.36 × 12.82 mm2 in inferior wall fracture and 20.69 × 14.41 mm2 in medial wall fracture. There was no complication except for 3 cases of infraorbital nerve hypoesthesia. A 2-year follow-up computed tomography showed that the surgical site preserved bony formation without herniation. In treating moderate-sized bony defect in orbit wall fracture, absorbable mesh and demineralized bone matrix can maintain structural stability through good bony formation even after degradation of absorbable mesh.

Serial sub-conjunctival 5-Fluorouracil for early recurrent anophthalmic contracted socket.

BACKGROUND: To evaluate the effects and side-effects of serial sub-conjunctival injections of 5-fluorouracil (5-FU) in early postoperative period for recurrent anophthalmic contracted socket. METHODS: Retrospective comparative case series at tertiary eye care centre including fifteen adult patients with features of postoperative recurrent socket contraction after buccal mucosal graft. Group A comprised eight patients treated with 10 mg weekly sub-conjunctival injection of 5-FU in the fornices. Group B comprised seven patients taken as control. Main outcome measures were: superior fornix depth (SFD), inferior fornix depth (IFD), and socket volume (SV) at 6 months follow up. Patients were re-evaluated clinically for recurrence at last follow-up. RESULTS: The mean ± SD values in group A versus group B were as follows: 10 ± 1.6 mm vs 5.1 ± 0.9 mm (p < 0.005) for SFD, 6.7 ± 1.5 mm vs 3.5 ± 0.5 mm (p = 0.02) for IFD; and 2 ± 0.55 ml versus 0.27 ± 0.06 ml (p = 0.005) for SV at 6 months. There was significant improvement in depth of fornices and volume of socket in seven patients in group A. One patient in group A did not benefit from 5-FU treatment. The beneficial effects of 5-FU were observed when first injection was given within 4 weeks after socket reconstruction. There was no recurrence (in six cases) and no side-effects seen in group A at final follow-up of 18.2 (12-24) months. CONCLUSIONS: Weekly injections of 5-FU are effective for stopping the progression of recurrent contracted socket following primary reconstructive surgery. It provides early rehabilitation, and avoids repetitive surgery.

Antifibrotic effects of quercetin in primary orbital fibroblasts and orbital fat tissue cultures of Graves' orbitopathy.

PURPOSE: We investigated the effects of quercetin on fibrotic markers and matrix metalloproteinases (MMPs) in primary cells and whole orbital tissues from Graves' orbitopathy (GO). METHODS: Orbital fat tissues were harvested from GO for primary cell and tissue cultures during orbital fat decompression. To determine noncytotoxic dose and time of quercetin treatment, 3-(4,5-dimethyl-thiazol-2-yl)-2,5-diphenyl-tetrazolium bromide (MTT) assay and LDH release assay were performed. The effects of quercetin on fibrosis were evaluated according to a scratch wound closure assay, and Western blotting for expression of fibronectin, collagen Iα, α-smooth muscle actin with or without TGF-ß stimulation, and MMP-2, -7, -9, and tissue inhibitor of metalloproteinase-1 with or without IL-1ß stimulation. The gelatinolytic activities of MMP-2 and MMP-9 were measured using gelatin zymography. In tissue cultures, MMP secretion and MMP and collagen Iα mRNA levels were determined by enzyme-linked immunosorbent assays and reverse transcription-polymerase chain reaction (RT-PCR), respectively. RESULTS: Quercetin significantly inhibited cell migration at nontoxic concentrations. In primary cells, quercetin dose-dependently downregulated expression of TGF-ß-stimulated fibronectin and collagen Iα, and IL-1ß-enhanced MMP-2 and MMP-9. However, without IL-1ß stimulation, 10-50 µM of quercetin increased MMP-2 expression and activity, but dose-dependently suppressed MMP-9 expression and activity. In tissue cultures, quercetin dose-dependently inhibited MMP-2 and -9 activity and secretion, but 30 and 50 µM of quercetin increased tissue MMP-2 mRNA. MMP-9 and collagen Iα mRNA levels were dose-dependently suppressed. CONCLUSIONS: Quercetin inhibited fibrotic markers and affected MMP-2 and MMP-9 activities in primary cell and orbital fat tissue cultures from GO at nontoxic concentrations. Our results support the potential use of quercetin for active inflammation and treatment or prevention of chronic fibrosis in GO.

Blepharoplasty complications.

There are three major areas of concern for a patient who has complications following a cosmetic blepharoplasty: cosmetic issues, functional issues, and uncomfortable/symptomatic issues or a combination of the above. An emphasis will be placed on avoiding cosmetic eyelid complications as well as the medical and surgical management of complications.

Complications in periocular rejuvenation.

Thorough preoperative evaluation with meticulous surgical planning to achieve facial aesthetic balance between the forehead, eyelids, and midface is imperative to avoid or decrease potential functional and/or cosmetic complications in cosmetic periocular surgery. Before performing surgery, the physician should be aware of the patient's history of dry eyes, previous facial trauma, previous injection of Botox Cosmetic, history of previous laser-assisted in situ keratomileusis, and past facial surgery. A full evaluation should be performed on the upper eyelid/brow region to assess for the presence of brow ptosis, brow/eyelid asymmetry, dermatochalasis/pseudodermatochalasis, eyelid ptosis, and deep superior sulcus. On the lower eyelid/cheek examination, special attention should be directed to the diagnosis of underlying negative vector, dry eyes, prominent eyes, lower lid retraction, ectropion, lateral canthal dystopia, lower eyelid laxity, scleral show, and lagophthalmos, with a rejuvenation goal that focuses on obtaining a youthful fullness through repositioning and reinforcing efforts to avoid the negative effects of hollowness. Intraoperative and postoperative medical and surgical management of cosmetic periocular surgery complications focus on decreasing the risk of postoperative ptosis, lagophthalmos, lid retraction, and lid asymmetry, with special attention to limiting the risk of visual loss secondary to orbital hemorrhage.

Prevention and management of orbital hematoma.

Orbital hematoma is an uncommon but serious complication of sinus surgery. Appropriate perioperative attention may minimize risk, but early diagnosis and appropriate management are crucial to preventing vision loss. The surgeon, postoperative care staff, and patient must be aware of the signs and symptoms. Immediate examination must be performed, including pupil size, symmetry, and reactivity, visual acuity, and measurement of intraocular pressure. Immediate consultation from an ophthalmologist or oculoplastic surgeon is mandatory if the diagnosis is suspected. The surgeon and ophthalmologist should follow an algorithm of advancing therapies based on serial examination, ranging from observation only to immediate surgical intervention.

Steri-strips as an aid to intra-operative monitoring of the eye during endoscopic sinus surgery.

Although uncommon, ocular complications of endoscopic sinus surgery have the potential to cause considerable morbidity. Direct intra-operative monitoring of the eye may alert the surgeon to potential complications at an early stage. We describe the use of Steri-strips during endoscopic sinus surgery as an aid to monitoring for ocular complications during surgery. This is a cheap, simple and effective method of intra-operative monitoring.

Steroids in rhinoplasty: a survey of current UK otolaryngologists' practice.

The use of steroids during rhinoplasty to reduce post-operative periorbital oedema and ecchymosis has been advocated. A number of randomized controlled trials have demonstrated the benefit of steroids in rhinoplasty. The aim of this study was to determine current UK practice in the use of steroids during rhinoplasty performed by otolaryngologists. A postal survey of consultant otolaryngologists in the UK was conducted. We received 203 responses, with 115 consultants performing 12 or more rhinoplasties per year. Only 28 consultants (24 per cent) used steroids routinely in patients undergoing rhinoplasty and of these 11 used a protocol, although this was unpublished. Dexamethasone was the most common steroid used (82 per cent), being administered as a single intravenous dose of 8 mg in the majority of cases (54 per cent). There was no correlation between the use of steroids and the number of rhinoplasties performed by individual consultants. Despite the evidence supporting the use of steroids to reduce post-operative sequelae following rhinoplasty, only a minority of consultants in the UK appear to use them as part of their practice.

Does creating a subperiosteal tunnel influence the periorbital edema and ecchymosis in rhinoplasty?

PURPOSE: The study goal was to determine whether creating a subperiosteal tunnel before lateral osteotomy had an effect on postoperative periorbital edema, ecchymosis, and subconjunctival ecchymosis. PATIENTS AND METHODS: Eighteen consecutive patients who underwent septorhinoplasty were included in the study. In all patients lateral osteotomies were carried out bilaterally, after creating a subperiosteal tunnel on a randomly chosen side and without creating a subperiosteal tunnel on the other side. The patients were seen on the second postoperative day, and a different surgeon who was unaware of the side with the periosteal tunnel determined the side of the face with more edema and ecchymosis. Subconjunctival ecchymosis was evaluated and recorded, as well. RESULTS: Creating subperiosteal tunnels before lateral osteotomy statistically increased periorbital ecchymosis. Although there was no statistically significant difference, creating subperiosteal tunnels also increased development and severity of subconjunctival ecchymosis and edema. CONCLUSIONS: We suggest performing lateral osteotomy without creating subperiosteal tunnels.

[Surgery of craniofacial deformities]. / Chirurgie kraniofazialer Fehlbildungen.

Most craniofacial abnormalities are craniosynostoses due to premature fusion of one or more craniofacial sutures. The typical cranial deformities result from growth inhibition perpendicular to the affected sutures. Functional impairment is caused either directly by the pathological growth pattern or indirectly by the increased intracranial pressure. Craniosynostosis is frequently accompanied by dysmorphia or dystopia of the orbits, resulting in possible functional problems. The indications for operation are to increase the intracranial volume and to correct aesthetic impairments. The planning of the surgical procedures has to consider the orbital problems. This review describes the different disease patterns of premature craniosynostosis and specific surgical approaches for their correction.

Reversal of optic canal stenosis in osteopetrosis after bone marrow transplant.

PURPOSE: To describe a patient with infantile osteopetrosis and optic atrophy secondary to optic canal stenosis who demonstrated optic canal enlargement after bone marrow transplant. METHODS: Case report. A 3-month-old infant with infantile "malignant" osteopetrosis underwent ophthalmic examination, including visual evoked potentials, electroretinogram, and computed tomography (CT). Bone marrow transplant was performed at 8 months of age. RESULTS: Examination revealed visual loss and optic atrophy, left eye greater than right eye, secondary to optic canal stenosis. Flash visual evoked potentials revealed a normal waveform in both eyes with increased latency in the left eye. Electroretinogram was normal in both eyes. CT after bone marrow transplant showed enlargement of the optic canals. Vision remains stable 43 months after bone marrow transplant. CONCLUSIONS: Bone marrow transplant in infantile osteopetrosis may be followed by reversal of optic canal stenosis and preservation of vision.