Ilse Mombaerts

Orbital inflammation: Biopsy first

Orbital inflammation is a response of the immune system and not a diagnosis in itself. Exposing the underlying disease introduces a labyrynthine challenge owing to the broad array of possible causes ranging from infectious, structural, autoimmune, idiopathic to neoplastic origin. In this regard, and despite its unkown etiology, idiopathic orbital inflammation (IOI) intrinsically is a genuine diagnostic entity. Where clinical and radiological findings of an orbital inflammatory mass are inconclusive, pathological examination of the tissue biopsy-obtained by minimally invasive approach and local anesthesia-is advocated to work towards a diagnosis in a most timely and effective manner. A corticosteroid response can be observed in most orbital disorders with lymphocytic components, including IOI, and, accordingly, constitutes a paradoxical and weak tool to identify the diagnosis in orbital inflammation.

Surgical Debulking for Idiopathic Dacryoadenitis: A Diagnosis and a Cure

PURPOSE Idiopathic inflammatory tumor of the lacrimal gland, also called idiopathic dacryoadenitis, generally is treated with high-dose, long-term systemic corticosteroids, despite their limited success, high recurrence rate, and incidence of drug-induced side effects. This study describes the outcome of patients with idiopathic dacryoadenitis who were managed with surgical debulking. DESIGN Retrospective case series from 2 tertiary referral centers. PARTICIPANTS Forty-six patients (46 lacrimal glands). METHODS Review of the clinical records, radiologic scans, and histopathologic specimens, with additional immunoglobulin G4 immunostaining. MAIN OUTCOME MEASURES Clinical signs and symptoms at 2 months after the surgery and off medications. RESULTS Before referral, 41% (19 of 46) of the patients had received systemic high-dose corticosteroids, after which they all showed recurrence, of whom 26% (5 of 19) became dependent on corticosteroids. At referral, all patients underwent debulking surgery of the inflammatory lacrimal gland mass for diagnostic and therapeutic reasons. Additionally, intralesional or systemic low-dose corticosteroids were given during the operation or the first postoperative days in 54% (25 of 46) of the patients. At 2 months after the debulking surgery, a full clinical recovery was seen in 80% (37 of 46) of the patients. A recurrence occurred in 8% (3 of 37) of the patients 4 months and 2.2 and 4.6 years later. Surgical failure (20%; 9 of 46) was correlated with prior corticosteroid treatment (P = 0.002, Fisher exact test), but not with sclerosing inflammation present in 28% (13 of 46). The median follow-up time was 7.2 years (range, 0.7-18 years). CONCLUSIONS Debulking biopsy procedures for idiopathic dacryoadenitis, in addition to being diagnostic, may be therapeutic.

The Tunneled Forehead Flap in Medial Canthal and Eyelid Reconstruction

BACKGROUND The midline forehead flap is used in the reconstruction of large, deep defects of the medial canthal area and lower eyelid. Drawbacks are a cosmetically unfavorable skin bulge at the nasal bridge and obliteration of the natural medial canthal concavity, requiring correction in a second stage. OBJECTIVE We adopted a modification of the technique to avoid these drawbacks. METHODS We reviewed the medical records and photographs of patients who received the tunneled midline forehead flap procedure in the repair of medial canthal defects and in the anterior lamellar repair of eyelid defects. The forehead flap was elevated in the subdermal plane, and the pedicle was deepithelialized and transferred through a subgaleal tunnel from the pivot point of the flap into the primary defect. RESULTS Nine patients had defects of the medial canthal area, medial part of the eyelids, or both after surgical removal of malignant tumors. Follow‐up ranged from 5 months to 6.1 years (mean 2.1 years, median 11 months). In all cases, flap viability was maintained, globe protection was achieved, and the concave architecture of the medial canthus was preserved. CONCLUSION The tunneled midline forehead flap can be an advantageous single‐stage technique in medial canthal and medial eyelid repair. The authors have indicated no significant interest with commercial supporters.

Series of Extranodal Natural Killer/T-cell Lymphoma, Nasal Type, with Periorbital Involvement

Abstract Purpose: To describe the clinical presentation, diagnosis and treatment of periorbital extranodal natural killer/T-cell lymphoma, nasal type. Methods: Case series of three patients with periorbital involvement of extranodal natural killer/T-cell lymphoma, nasal type, of whom clinical data, orbital imaging and immunohistochemical analysis were collected. For the purpose of this study, all histopathological and immunohistochemical slides were re-examined. Results: All patients presented with painless eyelid swelling and a history of sinonasal disease, of whom one with bilateral panuveitis, not responding to systemic antibiotics. Extraocular muscle involvement was present in 2 cases upon presentation and in 1 case later on. Initial paranasal and orbital biopsies were negative in 2 patients, with only the second orbital biopsy leading to the diagnosis. Natural killer/T-cell and cytotoxic markers were present in all cases, as well as Epstein–Barr virus encoded RNA in situ hybridization. The patients died respectively 5, 9 and 35 months from diagnosis despite treatment with chemotherapy and radiotherapy. Conclusion: Extranodal natural killer/T-cell lymphoma, nasal type, should be suspected in a painless periorbital cellulitis with chronic sinusitis, not responding to conventional therapy. A high index of suspicion is necessary in biopsies showing angiodestruction and necrosis. Epstein-Barr virus encoded RNA in situ hybridization and expert hematopathologist consultation is necessary to decrease the delay in diagnosis.

Computer-aided design and three-dimensional printing in the manufacturing of an ocular prosthesis

Restoring the facial appearance in acquired anophthalmos entails precision fitting of a customised ocular prosthesis. The fabrication is an artisanal process, and is primarily based on an impression of the anophthalmic cavity. This is usually achieved by the impression-moulding method, which, however, may introduce errors, resulting in a poorly fitted prosthesis. We developed a new method in the manufacturing of a customised ocular prosthesis based on computer-aided design and computer-aided manufacturing. An ocular prosthesis, based on a three-dimensional (3D) printed impression-free mould of the anophthalmic cavity, was successfully fitted in a 68-year-old male. To the best of our knowledge, this is the first case of a customised ocular prosthesis designed with the aid of 3D printing. Trial registration number B322201628407, Pre-results.

Direct caroticocavernous fistula and traumatic dissection of the ipsilateral internal carotid artery: endovascular treatment.

Abstract After severe craniocerebral trauma a 14-year-old boy developed progressive exophthalmos with venous congestion and chemosis, due to a direct caroticocavernous fistula. Angiography revealed traumatic occlusion of the ipsilateral internal carotid artery and absence of the inferior petrosal sinus. After failure of an approach via the anterior and posterior communicating arteries, the cavernous sinus was successfully catheterised through the occluded internal carotid artery, and embolisation performed with coils.

Consensus on Diagnostic Criteria of Idiopathic Orbital Inflammation Using a Modified Delphi Approach

Importance Current practice to diagnose idiopathic orbital inflammation (IOI) is inconsistent, leading to frequent misdiagnosis of other orbital entities, including cancer. By specifying criteria, diagnosis of orbital inflammation will be improved. Objective To define a set of criteria specific for the diagnosis of IOI. Design, Setting, and Participants A 3-round modified Delphi process with an expert panel was conducted from June 8, 2015, to January 25, 2016. Fifty-three orbital scientist experts, identified through membership in the Orbital Society, were invited to participate in on online survey and they scored, using 5-point Likert scales, items that are eligible as diagnostic criteria from the literature and from personal experience. The items were clustered around the anatomic subtypes of IOI: idiopathic dacryoadenitis and idiopathic orbital fat inflammation (2 nonmyositic IOIs), and idiopathic orbital myositis (myositic IOI). Items with dissensus were rescored in the second round, and all items with consensus (median, ≥4; interquartile range, ⩽1) were ranked by importance in the third round. Main Outcomes and Measures Consensus on items to be included in the criteria. Results Of the 53 experts invited to participate, a multinational panel of 35 (66%) individuals with a mean (SD) years of experience of 31 (11) years were included. Consensus was achieved on 7 of 14 clinical and radiologic items and 5 of 7 pathologic items related to diagnosis of nonmyositic IOI, and 11 of 14 clinical and radiologic items and 1 of 5 pathologic items for myositic IOI. There was agreement among panelists to focus on surgical tissue biopsy results in the diagnosis of nonmyositic IOI and on a trial with systemic corticosteroids in myositic IOI. Panelists agreed that a maximum number of 30 IgG4-positive plasma cells per high-power field in the orbital tissue is compatible with the diagnosis of IOI. Conclusions and Relevance An international panel of experts endorsed consensus diagnostic criteria of IOI. These criteria define a level of exclusion suggested for diagnosis and include tissue biopsy for lesions not confined to the extraocular muscles. This consensus is a step toward developing guidelines for the management of IOI, which needs to be followed by validation studies of the criteria.

Clinically Recognizing Enlarged Extraocular Muscles from Lymphoid Origin

retro-mode images create a pseudo 3-dimensional image of the deeper retinal tissues by highlighting objects by blocking the reflected light from the other side. Our 56-year-old patient with primary open-angle glaucoma underwent trabeculectomy with mitomycin C 2 weeks earlier. His IOP was 4 mmHg at the time the first photograph was taken. This novel photograph shows the wrinkled retinal pigment epithelium (RPE), and this finding was confirmed by 3-dimensional optical coherence tomography (OCT; Fig 2, available at www.aaojournal.org). The wrinkled RPE was seen as a pseudo 3-dimensional image made by shadows created by blocking and correcting the infrared laser scattering. The IOP increased to 11 mmHg within 2 weeks, decreasing the retinal edema and macula folds, which was confirmed by OCT images. The retro-mode pictures showed the detailed RPE wrinkles and these wrinkles were gradually decreased after the return of the IOP to a normal range. By using these retro-mode images, we could directly evaluate the recovery of the RPE damage. Retro-mode images have been previously used to identify diabetic macular edema, drusen in age-related macular degeneration, retinoschisis in highly myopic eyes, and central serous chorioretinopathy. Our findings suggest that the retromode images may also be a useful tool for evaluating the recurrent in RPE wrinkling diseases like Vogt-Koyanagi-Harada disease and posterior scleritis.

Diagnosis of enlarged extraocular muscles: when and how to biopsy

Purpose of review To review current knowledge regarding diagnosis of nonthyroid orbital disorders with extraocular muscle enlargement. Recent findings Recent publications have focused on immunoglobulin G4-related disease as a possible cause of enlarged extraocular muscles, on patterns of strabismus that raise a clinical suspicion of intramuscular lymphoma, and on surgical techniques to access the muscles for tissue biopsy. Summary With enlarged extraocular muscles, features to distinguish between competing diagnostic possibilities are based on imaging in the context of history and clinical signs. Infraorbital nerve enlargement in the presence of muscle enlargement strongly favours a diagnosis of immunoglobulin G4-related disease and reactive lymphoid hyperplasia. As our understanding of minimally invasive orbital surgery evolves, the diagnostic focus is shifting toward earlier identification through muscle biopsy.

The survival of an angled extended Jones’ tube

Aim To analyse the long-term outcome of lacrimal bypass surgery with an angled extended Jones’ tube. Design Retrospective, non-comparative, non-interventional case series. Methods Seventy-one patients who underwent transcaruncular insertion of an angled extended Jones’ tube between 1995 and 2005 were followed up over a median period of 8.7 years (range 11 days–17 years). Main outcome measures Patency and anatomic position of the tube, type and time lapse of the complications and subjective relief of epiphora. Results Overall, complications occurred in 39% of the tubes (35/90) at a median of 25 days (range 3 days–6 years) after the insertion. The major complications, that is, tube displacement or obstruction, were observed in 31% (28/90), with an incidence rate of 8% per year. The minor complications, that is, conjunctival overgrowth or granuloma at the lateral end, were encountered in 8% (seven of 90). The 10-year tube survival rate was 60%, with complete relief of epiphora in 88% of the patients. Conclusions The long-term outcome with the angled extended Jones’ tube inserted transcaruncularly remains relatively good, with most of the complications occurring in the early postoperative period.