Yoshiyuki Kitaguchi, Yasuhiro Takahashi & Hirohiko Kakizaki
To examine the predictability of exophthalmos reduction using preoperative computed tomography (CT) in deep lateral orbital wall decompression for Graves’ orbitopathy.
This was a retrospective, observational, case-control study conducted at a single institution. Forty-three patients (43 orbits) who were treated with deep lateral decompression with (27 patients) and without (16 patients) fat removal. Multivariate linear regression analyses were used to identify factors influencing exophthalmos reduction 3 months postoperatively. Variables investigated included age; smoking history; history of corticosteroid therapy and/or radiotherapy; preoperative diplopia; width, depth, and height of the greater wing of the sphenoid bone (trigone); and volume of fat removed. The influence of these parameters on the area of the orbital opening was also analyzed.
Trigone width and amount of fat removed were positively correlated with exophthalmos reduction (both, P < .050); none of the other variables exhibited significant correlations. The predictive equation for postoperative reduction in Hertel exophthalmometric values was 0.57 + 0.15 × trigone width (mm) + 1.1 × fat removed (cm3). Depth and height were positively correlated (both, P < .050) and width was negatively correlated (P = .0045) with orbital opening area.
Trigone width and amount of fat removed were positive predictors of exophthalmos reduction after deep lateral decompression. Trigone width was inversely associated with orbital width, and results showed that larger exophthalmos reductions could be achieved in narrower orbits. This CT-based prediction method will assist preoperative decision-making regarding additional fat removal and/or removal of another orbital wall.
David S Curragh & Dinesh Selva
Intraoperative handling and manipulation of orbital fat remains a challenge to orbital surgeons. We present a case series of endoscopic orbital fat decompression with medial orbital wall decompression for proptosis management in Grave’s orbitopathy, describing a technique for fat excision using a laryngeal skimmer blade, reporting clinical and surgical outcomes, and complications.
All patients who underwent endoscopic orbital fat decompression, with medial orbital wall decompression, for proptosis management in Grave’s orbitopathy between 2011 and 2018, under the care of a single surgeon, were included in this retrospective interventional case series.
Nineteen patients were included in this study. Using a laryngeal skimmer blade, orbital fat was excised endoscopically at the time of medial orbital wall decompression. The mean volume of orbital fat excised was 1.45 ± 0.63 ml and the mean reduction in proptosis was 4.5 ± 1.02 mm. There were no intraoperative complications.
In this study, we describe our experience of using a laryngeal skimmer blade as a method of excising orbital fat for orbital decompression in patients with proptosis secondary to Grave’s orbitopathy.
Kerstin Stähr, Anja Eckstein, Laura Holtmann, Anke Schlüter, Meaghan Dendy, Stephan Lang & Stefan Mattheis
Introduction: Different minimally invasive surgical approaches to the orbit allow individualized bone resection to reduce proptosis and decompress the optic nerve in patients with Graves’ orbitopathy (GO). This study aims to compare piezosurgery to an oscillating saw used to resect bone from the lateral orbital wall.
Methods: In a retrospective study, we analyzed balanced orbital decompressions performed on 174 patients (318 cases) with GO. An oscillating saw was used in 165 cases (saw group) and piezosurgery in 153 cases (piezo group). Peri- and postoperative complications, reduction of proptosis, new onset of diplopia and improvement of visual acuity in cases of pre-operative optic nerve compression were analyzed.
Results: We observed no significant differences in the surgical outcome between the two groups. Proptosis reduction was 4.6 mm in the saw group (p < 0.01) and 5.3 mm in the piezo group (p < 0.01). Intraoperative handling of the piezosurgery device was judged superior to the oscillating saw, due to soft tissue conservation and favourable cutting properties. Duration of the surgery did not differ between the groups. No serious adverse events were recorded in both groups.
Conclusion: The application of piezosurgery in orbital decompression is more suitable than an oscillation saw due to superior cutting properties such as less damage to surrounding soft tissue or a thinner cutting grove.
Yoshiyuki Kitaguchi, Kentaro Ishihara, Kohji Nishida
Dysthyroid optic neuropathy (DON) is a sight-threatening complication of Graves’ orbitopathy. DON occurs in less than 5% of patients with Graves’ orbitopathy disease and is thought to require urgent treatment to rescue vision.1 High-dose intravenous glucocorticoids and orbital decompression are standard treatments for DON.2
Graves’ orbitopathy generally shows milder clinical presentation in euthyroidism than in hyperthyroidism.3 Thus, DON rarely develops in euthyroid patients,4, 5 and the treatment strategy for euthyroid optic neuropathy has not been established. Here we report a patient with spontaneously resolved euthyroid optic neuropathy who was not treated as described above.
Kyle T. Lewis, John R. Bullock, Ryan T. Drumright, Matthew J. Olsen & Alan D. Penman
The purpose is to evaluate the utility of optical coherence tomography (OCT) angiography in the evaluation of Graves’ orbitopathy (GO) and response to orbital decompression in patients with and without dysthyroid optic neuropathy (DON). This was a single-center, prospective case series in a cohort of 12 patients (24 orbits) with GO and ±DON, (6 orbits) who underwent bilateral orbital decompression. All patients underwent pre- and postoperative OCT angiography of the peripapillary area. Vessel density indices were calculated in a 4.5 mm × 4.5 mm ellipsoid centered on the optic disk using split-spectrum amplitude decorrelation angiography algorithm, producing the vessel density measurements. Mean change in vessel density indices was compared between pre- and postoperative sessions and between patients with and without DON. Patient 1, a 34-year-old male with GO and unilateral DON OD, showed a significant reduction in blood vessel density indices oculus dexter (OD) (DON eye) after decompression while a more modest reduction was found oculus sinister (OS) with the greatest change noted intrapapillary. Patient 2, a 50-year-old male with DON OU, showed worsening neuropathy following decompression OD that was confirmed by angiographic density indices. Patient 3, a 55-year-female with DON, showed a reduction in blood vessel density OD and increased density OS. Patients without DON showed overall less impressive changes in indices as compared to those with DON. Using OCT angiography, response to surgical treatment in GO orbits, more so in orbits with DON, can be demonstrated and quantified using vessel density indices with reproducibility.
Tooley, Andrea A.; Salomao, Diva R.; Bradley, Elizabeth A.; Garrity, James A.
Purpose: The authors aimed to determine key features of IgG4-related ophthalmic disease (IgG4-ROD) and Graves orbitopathy (GO) to aid in diagnosis.
Methods: The authors retrospectively identified ophthalmology patients seen between June 2009 and November 2013 with clinical overlap of GO and IgG4-ROD. Patient findings were reviewed to characterize the 2 conditions.
Results: Among 8 patients (7 male and 1 female), the mean age was 45.8 years. Time between diagnoses of GO and IgG4-ROD ranged from 1 month to 8 years. Imaging showed enlarged extraocular muscles in all patients. Enlarged infraorbital nerves were seen in 4 patients. Tissue biopsy showed CD20+ lymphocytes with a large proportion of IgG4 plasma cells in 7 of 8 orbital specimens. Six patients had a ratio of IgG4:IgG cells >40%.
Discussion: No pathognomonic clinical findings for GO or IgG4-ROD have been reported, but some key features can help distinguish the conditions. GO is likely if findings include increased thyrotropin receptor antibodies, lid retraction/lid lag, and enlarged extraocular muscles with typical tendon-sparing morphology. Findings suggestive of IgG4-ROD include history of asthma and progressive orbital disease in patients with previous diagnosis of GO, disproportionately large lateral rectus muscle, and enlarged infraorbital nerves. Increased serum IgG4 level and biopsy showing >10 IgG4+ plasma cells/high-power field and IgG4:IgG ratio >40% will support the diagnosis of IgG4-ROD.
Conclusions: GO and IgG4-ROD are complicated inflammatory processes affecting the orbit and present diagnostic challenges. The authors recommend biopsy for patients who do not follow the usual clinical course of GO or have clinical characteristics of IgG4-ROD.
Jonathan C. P. Roos & Rachna Murthy
Rapamycin (alternatively known as sirolimus) is a macrolide immunosuppressant commonly used for organ transplantation. It acts both on lymphocytes through the mechanistic target of rapamycin (mTOR) pathway to reduce their sensitivity to interleukin-2 (IL-2) and, importantly, also has anti-fibrotic properties by acting on myofibroblasts. The latter have been implicated in the pathogenesis of thyroid eye disease (TED).
To describe successful treatment and reversal of extraocular muscle fibrosis in TED with sirolimus.
Case report and literature review with clinic-pathological correlation.
A patient with Graves’ orbitopathy (GO) developed significant ocular motility restriction, which was unresponsive to steroids and conventional immunosuppression. Unlike these prior treatments, rapamycin therapy improved the diplopia and fields of binocular single vision over a period of months. There were no adverse effects directly attributable to the treatment.
With its low renal toxicity and ability to specifically target the underlying fibrotic pathways in GO, rapamycin may prove a useful adjunct to standard immunosuppressive regimes. We encourage further reporting of case series or the instigation of controlled trial.
Mario Salvi & Danila Covelli
B cells have multiple actions on different phases of an immune reaction, mainly resulting in B and T cell-interaction (help), production of cytokines, regulation of dendritic cells and downregulation of regulatory B cells. The effectiveness of B cell depletion therapy is probably due to blockade of the antigen-presenting function of B cells, occurring very early in the setting of autoimmune reactions. B cells undergo a maturation process from stem cells during which the CD 20 antigen, which is the target of rituximab (RTX), is expressed from the stage of pre-B cells to mature and memory B cells, but not on plasma cells. During the maturation process, the cytokine B cell stimulating factor (BAFF) induces maturation of B cells and expansion of clones to produce plasma cells and eventually antibodies. The effect of RTX in GO is rather rapid, with significant improvement of the disease already 4–6 weeks after the first RTX infusion. Based on the evidence of significant lymphocytic infiltration in the orbits of patients with active GO, it is reasonable to postulate that RTX may cause depletion of B cells and block their antigen-presenting cell mechanism. Since it has been reported that serum BAFF concentrations are elevated in hyperthyroid GD patients and that BAFF is expressed on the thyrocytes of patients with either autoimmune disease or nodular goiter, the hypothesis that belimumab, an anti-BAFF monoclonal antibody, may be effective in patients with active GO his currently being tested in a randomized controlled trial.
Jun Soo Byun, Jeong Kyu Lee
To assess the relationships between eyelid position and levator palpebrae superioris (LPS)-superior rectus (SR) complex and inferior rectus (IR) muscle volume in patients with Graves’ orbitopathy (GO) with unilateral upper eyelid retraction.
This was a cross-sectional observational study of 48 patients with GO with unilateral upper eyelid retraction. To measure muscle volume, computerized tomography scans were performed, and 3D images were analyzed. Digital photographs were taken, and vertical eyelid height was measured using computed eyelid analysis software. The measured muscle volumes and eyelid heights were assessed, and correlation analysis was performed. To verify the parameters that are predictive for the presence of upper eyelid retraction, receiver operating characteristic curves were analyzed, and logistic regression was performed.
The volume of the LPS/SR muscle complex in the eyes with upper eyelid retraction was increased in 41 eyes (85.4%). The mean volume of the LPS/SR complex was 0.92 ± 0.40 cm3 in the eyes with upper eyelid retraction and 0.72 ± 0.27 cm3 in the contralateral eyes (p < 0.0001). While there was no correlation between LPS/SR complex volume and margin reflex distance1 (MRD1, the vertical distance between the center of the pupil to the center of the upper eyelid margin) (R = 0.024, p = 0.869), MRD1 and MRD2 (the vertical distance between the center of the pupil and the center of the lower eyelid margin) in the retracted eyes were negatively correlated (R = − 0.441, p = 0.002). In patients with upper eyelid retraction without increased LPS/SR complex volume, IR volume and MRD2 of the contralateral eye were 0.48 ± 0.10 cm3 and 5.92 ± 0.45 mm, respectively. In the retracted eye, they were 0.37 ± 0.17 cm3 and 5.32 ± 0.59 mm, respectively (p = 0.018, and 0.028). Regression models incorporating LPS/SR complex volume, MRD1, and lid lag could predict the presence of upper eyelid retraction with an accuracy of 92.5%.
In patients with GO, increased LPS/SR complex volume in the retracted eye and increased IR volume of the contralateral eye were both associated with unilateral upper eyelid retraction. The combination of LPS/SR complex volume, MRD1, and lid lag can be used as a reliable index of upper eyelid retraction in patients with GO.