Upper Eyelid Contour Changes After Müller's Muscle Conjunctiva Resection.

PURPOSE: To analyze the upper eyelid contour after Müller's muscle conjunctiva resection (MMCR) performed by four different surgeons. METHODS: Comparative cross-sectional analysis of the pre- and postoperative contours of a control group and four groups of upper lids (n = 88) of 65 patients who underwent MMCR at four international centers. The procedure employed was essentially the same as described by Putterman but performed with different instruments to entrap the posterior lamella. Multiple medial and lateral margin lid distances were measured on Bézier lines expressing the pre- and postoperative lid contours. RESULTS: Preoperatively, two groups had significant lateral and medial ptosis. After MMCR, the lateral segment of the lid's contour was corrected in all groups. In the two groups with more pronounced ptosis, the nasal lid contour was undercorrected. CONCLUSIONS: In MMCR, regardless of the instrument used to entrap the posterior lamella, the amount of medial tissue resection is essential to avoid postoperative nasal undercorrection. LEVEL OF EVIDENCE III: This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .

Rundle's Curve: A Useful Construct That Does Not Fit All.

PURPOSE: The purpose of this study was to analyze Rundle's original data and subsequent articles on Graves orbitopathy (GO) natural history. METHODS: Rundle's texts were analyzed qualitatively and quantitatively. Serial measurements were plotted and fitted with different functions. Subsequent articles in the English literature on the natural history of GO were also analyzed. RESULTS: Different functions such as simple linear regressions, parabolic, saturating exponential growth, and exponential decay functions were well fitted for Rundle's data on measurements of proptosis and supraduction along time. The few quantitative data of the same variables post-Rundle were also well-fitted with various functions. CONCLUSION: Rundle described in his articles, from 1945 to 1957, 2 phases of ocular changes in GO: dynamic and static. However, he did not mention the pathophysiology of these phases nor used the terms inflammatory and cicatricial. Actually, most of his observations and the subsequent data in the literature on proptosis and supraduction did not obey the biphasic pattern of the so-called Rundle's curve.

Position of the anterior ethmoidal foramen and trauma to the cranial base during transconjunctival medial orbital decompression: a systematic literature review.

PURPOSE: To review the literature on the location of the anterior ethmoidal foramen (AEF) and trauma during transconjunctival medial wall decompression. METHODS: A comprehensive literature search was conducted using the PubMed, Embase, and Scopus databases, combining the terms "olfactory fossa" and "fovea ethmoidalis" with "trauma," "cerebrospinal fluid leak," "pneumocephalus," "orbital decompression," and "anterior ethmoidal artery" (AEA). All cases of cranial base trauma during medial orbital decompression and the anatomical studies on the location of the AEF and the course of the AEA were reviewed. RESULTS: Ninety-four articles were identified, of which 37 were related to the AEF, 41 reported the course of the AEA, and 16 to reported cases of cranial base trauma. Out of these cases, 10 were related to transconjunctival medial orbital decompression, affecting 11 patients. Most AEFs are situated at the frontoethmoidal suture, but up to 38.15% of AEFs are located above the suture on the frontal bone. Most AEFs are adjacent to the roof of the ethmoidal sinus. The distance of the AEF to the cranial base increases in the presence of supraorbital ethmoidal cells (SOEC). CONCLUSIONS: The position of the AEF is variable and should not be considered a safe landmark for all patients.

Orbital and paranasal sinuses changes after neonatal orbital exenteration for massive teratoma management.

A neonate at 13 days of age underwent a lid and conjunctiva-sparing orbital exenteration for a massive right orbital teratoma. To reduce the degree of orbital contraction, the entire temporalis muscle was rotated into the orbit along with a dermis-fat graft. Sequential postoperative orbital imaging showed that hyperostosis developed in the orbital apex at the age of two months. Despite a significant expansion of the fat graft, by the age of 4 years, hyperostosis had progressed to the anterior portion of the orbit associated with over-pneumatization of the paranasal sinuses. This case demonstrates that the bony changes in the orbit after neonatal exenteration are complex and involve the development of the paranasal sinuses.

The effect of rim-off deep lateral orbital decompression on the lateral rectus shape and oculomotor balance.

PURPOSE: The purpose of the study was to measure the effect of rim-off deep lateral decompression for Graves orbitopathy on the lateral rectus muscle path and oculomotor balance. METHODS: Retrospective analysis of the medical records and pre- and postoperative computed tomography scans of 34 orbits of 23 patients who underwent deep lateral decompression alone. The oculomotor balance of these 23 patients was measured with the alternate cover test and prisms before and after surgery. Bezier functions were used to measure the postoperative path of the lateral rectus in all decompressed orbits. RESULTS: Deep lateral decompression induced a curvilinear deformation of the lateral rectus. There was no significant correlation between the position of the point of maximum muscle displacement and the size of the residual lateral wall. The changes in the lateral rectus path had no adverse effects on the oculomotor balance of the patients. CONCLUSIONS: The location of the curvilinear deformation of the lateral rectus does not depend on the residual segment of the lateral wall. The changes of the lateral rectus path have no deleterious effect on the oculomotor balance.

Monoclonal Antibodies for the Treatment of Graves Orbitopathy: Precision Medicine?

PURPOSE: To summarize the development, nomenclature, and rationale of the reported use of monoclonal antibodies (Mabs) in Graves Orbitopathy (GO) and to undertake a systematic review of the management of GO with Mabs. METHODS: The Pubmed and Embase databases and the Federal Brazilian searching site (Periódicos-CAPES) were screened. The authors searched all the keywords "monoclonal antibodies," "adalimumab," "belimumab," "infliximab," "rituximab," "teprotumumab," and "tocilizumab" combined with the terms "Graves Orbitopathy," "Graves eye disease" and "thyroid eye disease." All the articles published in English, French, and Spanish from 2000 to May 2022 were screened. Only publications with quantitative data on the activity of orbitopathy, proptosis, or both were included. RESULTS: Seventy-six articles of the 954 screened records met the inclusion criteria. Seven Mabs were described for treating GO. The three most reported Mabs were Rituximab, Tocilizumab, and Teprotumumab. Only eight randomized clinical trials compared the effect of these three Mabs and Belimumab with the effect of steroids or placebos. Adalimumab, Infliximab, and K1-70 only appeared in a few case series and case reports. Frequent mild-to-moderate and few major side effects occurred with the three most used Mabs. Relapse rates ranged from 7.4% for Tocilizumab to at least 29.4% for Teprotumumab. No randomized clinical trials compared Mabs head-to-head. CONCLUSION: Considering the lack of head-to-head comparisons between Mabs, the relapse rate, the possibility of severe collateral effects, and the cost of Mabs, it is not clear which Mab is the safest and most useful to treat GO.

Margin rotation for tarsal buckling following ptosis surgery.

PURPOSE: To assess the clinical characteristics of tarsal buckling after ptosis correction and its management with margin rotation techniques. METHODS: Multicenter retrospective review of ten patients who developed upper eyelid entropion following ptosis correction. In all cases the tarsal deformity was corrected with margin rotational procedures with either a lid crease anterior approach or a traditional posterior approach. Data collection included patient demographics, type of ptosis surgery, and photographic documentation of the affected eyelids. RESULTS: Entropion occurred after a variety of different ptosis surgery techniques, including frontalis sling, levator advancement and supramaximal levator resection. A horizontal tarsal fold was detected in all eyelids, being in the upper third of the tarsus in 70% and in the central tarsus in 20% of the cases. Tarsal buckling was corrected in all cases with rotational surgery, with nine cases being operated through an anterior lid crease approach and 1, through the traditional posterior approach. The most reported complication was minimal residual ptosis. CONCLUSION: Tarsal buckling following ptosis surgery is associated with folds located in the upper part of the tarsus. Margin rotation techniques are effective in restoring the natural position of the eyelid margin in these cases.

Implications of the olfactory fossa anatomy in intracranial complications of inferomedial orbital decompression.

Two patients with thyroid eye disease underwent inferomedial orbital decompression complicated by a cerebrospinal fluid (CSF) leak. One of the cases developed a postoperative pneumocephalus resolved with conservative measures. In the second case, the CSF leak was managed intraoperatively. In both patients, a computed tomography (CT) scan revealed a pronounced slope of the lateral lamella of the cribriform plate, forming an obtuse angle with its lateral bony extension. When this anatomical disposition of the olfactory fossa (OF) is present, the course of the anterior ethmoidal artery (AEA) is usually embedded in the skull base, and its foramen should not be taken as the upper limit of the transconjunctival ethmoidectomy. The shape and relative height of the olfactory fossa and fovea ethmoidalis, and the course of the AEA should always be assessed before transconjunctival medial decompressions.

Upper Eyelid Contour Changes After Orbital Decompression in Graves Orbitopathy.

PURPOSE: To evaluate the effect of orbital decompression on the upper eyelid contour. METHODS: A paired cross-sectional analysis of the upper eyelid contour was performed for 103 eyes of 66 patients who underwent orbital decompression. A control group of 26 normal subjects was also included. The eyelid contour of all participants were measured with Bézier lines adjusted to the eyelid contour and 9 midpupil eyelid margin (MPD) distances from a horizontal line bisecting the pupil. One central, corresponding to the margin reflex distance (MRD 1), and 8 equally distributed medially and laterally at 20% of the interval between the lines. Patients were classified as with flare if the height of the most lateral MPD relative to the MRD 1 was above the upper limit of the controls. RESULTS: Preoperatively 63 of the 103 contours were classified as flare + (F+). After decompression MRD1 showed a mean decrease of 0.4 mm and the location of the contour shifted 0.8 mm medially. These changes were not correlated with proptosis reduction. Orbital decompression decreased the lateral curvature of the contours especially for the F+ lids. In 40% of the F+ eyelids the flare sign disappeared after decompression. CONCLUSIONS: Orbital decompression affects the lateral eyelid contour and diminishes the amount of lateral eyelid retraction surgery necessary to correct the flare sign. In 40% of the patients, the eyelid contour is normalized with proptosis reduction only.

Plasma cell IgG4 positivity in orbital biopsies of non-IgG4-related conditions.

The IgG4-related disease (IgG4-RD) is a systemic condition defined as a fibro-inflammatory disorder, characterized by the occurrence of tumor-like lesions in multiple organs including the eye adnexa. The main diagnostic criterion is based on histopathological findings, especially on the IgG4+/IgG+ plasma cell ratio. In this article, we reviewed the literature of non-IgG4-RD orbital conditions with IgG4 positivity. There were 20 reports of inflammatory non-IgG4-RD orbital lesions and 14 reports of orbital lymphoid proliferations with significant IgG4 positivity. The role of plasma cells IgG4 in the pathogenesis of non-IgG4-RD is not clear. Considering the large spectrum of diseases caused by a variety of different etiopathogenic mechanisms, we think that the common denominator of IgG4+ in these conditions might be related to the peculiar properties of down regulation of immune response of the IgG4 and not to a specific link to IgG4-RD.

Congenital Opticmeningoceles.

Congenital optic nerve cystic-like malformations associated with normally developed globes are extremely rare. We describe 3 children who presented since birth with proptosis, and eye motility limitation. MRI showed in all cases that the intraorbital segment of the optic nerves was malformed with large cystic-like lesions in the intraconal segment of the orbit. In all cases, biopsies of the wall of the lesions were positive for glial fibrillary acidic protein. Since this protein is a neurobiomarker that exists only in astrocytes in the central nervous system, nonmyelinating Schwann cells of peripheral nerves, and enteric glial cells, we believe that these lesions represent true opticmeningoceles.

Frontalis Linkage Without Intraoperative Eyelid Elevation for the Management of Myopathic Ptosis.

PURPOSE: To report the effect of frontalis linkage without intraoperative eyelid elevation for the management of myopathic ptosis. METHODS: Retrospective analysis of 21 (42 eyelids) myopathic patients with bilateral ptosis who were operated between 1999 and 2017. All patients had orbicularis weakness and poor or absent Bell's phenomenon. Surgery consisted of using an autogenous fascia sling to link the tarsal plate to the frontalis muscle without any degree of intraoperative eyelid elevation. The main outcome measures were margin reflex distance, brow height and degree of brow excursion and degree of lagophthalmos, and exposure keratitis. RESULTS: After surgery, there were significant changes (p <0.0001) in both margin reflex distance and brow position. Mean margin reflex distance increased to 1.4 mm ± 1.34 DP and with full frontalis contraction, it reached 3.0 mm ± 1.73 DP, while mean brow position decreased 1.6 mm ± 1.59 SD, p < 0.0001. Postoperative lagophthalmos was not detected in 31 (74%) eyes. In the remaining 11 eyes (26%), lagophthalmos ranged from 1.2 to 5.2 mm (mean = 1.7 mm ± 0.74 DP). Mild inferior superficial keratitis was detected in 14 eyes (33.3%) of 7 patients only 3 of which had lagophthalmos. One patient needed additional surgery to correct unilateral eyelid retraction. Overall, 81.81% of the patients were pleased with the procedure. CONCLUSIONS: Myopathic ptosis can be alleviated with a minimal amount of lagophthalmos by just linking the tarsal plate to the frontalis muscle without lifting the eyelid margin intraoperatively.

Systemic Diffuse Large B-Cell Lymphoma Presenting as Bilateral Orbital Vasculopathy.

The authors report a case of a 58-year-old man with bilateral proptosis and signs of orbital inflammation without any associated systemic findings. MRI showed diffuse orbital infiltration. An intraconal orbital biopsy revealed polyclonal lymphoplasmacytic infiltration and non-necrotizing lymphoid small-vessel vasculopathy. The orbital symptoms resolved following a 2-week course of oral prednisone. Three months later, the patient experienced abdominal pain and weight loss. Ultrasonographic investigation showed hepatosplenomegaly, a retroperitoneal mass, and inguinal lymphadenopathy. A lymph node biopsy revealed a diffuse large B-cell lymphoma. The patient underwent chemotherapy but died 6 months later with metastatic disease. This case suggests that diffuse orbital inflammation with nonspecific vasculitis may be an early paraneoplastic finding associated with diffuse large B-cell lymphoma.

Frontalis-Orbicularis Muscle Advancement for Correction of Upper Eyelid Ptosis: A Systematic Literature Review.

PURPOSE: To review the level of standardization of frontalis-orbicularis muscle advancement to correct severe blepharoptosis and the degree of scientific evidence supporting the procedure as a useful modality of blepharoptosis repair. METHODS: The authors searched the Medline, Lilacs, and Scopus databases for all articles in English, Spanish, and French that used as keywords the terms "frontalis muscle flap," "orbicularis muscle flap," and "ptosis." Data retrieved included authorship specialty, geographic region where the surgeries were performed, characteristics of the samples reported, type and dimensions of the flaps used, time of follow-up, rate of undercorrection, and complications. RESULTS: Thirty-eight articles were retrieved and analyzed. Most studies originated from Asian countries, especially China, Taiwan, and Korea. Many variations of the procedure were encountered, including location of incisions and frontalis flap design. There were 23 case series with more than 10 patients. None compared the procedure to conventional frontalis suspension surgery. The samples were not homogeneous, including patients with different type of ptosis, variable degrees of levator function, and using distinct methods of evaluating eyelid position. Undercorrection rates ranged from 1.8% to 38% with a median value of 12.2%. The rate of complications (eyelid crease abnormalities, entropion, hematoma, and supraorbital nerve injury) was low. CONCLUSIONS: The direct frontalis-orbicularis muscle advancement has been judged positively in all reports analyzed. However, the level of standardization of the surgery is low, and the reported series are not homogeneous. Further studies are needed to better evaluate this operation.

Interlamellar Autogenous Tarsal Graft for the Correction of Lower Eyelid Trichiasis Associated With Eyelid Margin Thinning.

PURPOSE: To evaluate the use of autogenous tarsal graft for surgical correction of lower eyelid trichiasis associated with eyelid thinning. METHODS: A total of 23 eyelids of 19 consecutive patients with trichiasis were operated with a variant of the Van Millingen procedure. The surgery consisted of interposing a 3-mm high fragment of autogenous ipsilateral upper eyelid tarsus corresponding to the trichiasis segment between the pretarsal anterior lamella and the lower tarsal plate. Preoperative assessment included quantification of eyelid thickness on the midpoint of the segment with trichiasis, biomicroscopy of the eyelid margin and location of the trichiasis. All patients were examined at 1, 3, 6, and 12 months after surgery. Eyelid thickness was measured at 1 month and 12 months of follow up. RESULTS: Preoperatively the mean thickness of the eyelid margin was 0.99 mm ± 0.06 standard error. One month postoperatively eyelid thickness increased to 2.08 ± 0.28 standard error, decreasing over time to reach 1. 48 mm ± 0.18 standard error 1 year after surgery, a value that did not differ from that of the control group. Small granulomas were diagnosed only in the early phases of the postoperative period. Trichiasis recurrence was seen in just 1 eyelid. Isolated abnormal lashes lateral or medial to the graft were present in 5 eyelids at the end of the study. CONCLUSIONS: Autogenous tarsal grafts placed parallel to the lower tarsus are a good option both to correct misdirected eyelashes and to restore normal lower eyelid thickness.

Symmetry of Upper Eyelid Contour After Unilateral Blepharoptosis Repair With a Single-strip Frontalis Suspension Technique.

PURPOSE: To analyze the upper eyelid contour of patients with unilateral congenital ptosis who underwent single-strip frontalis suspension. METHODS: The authors compared the upper eyelid shape of the right and left eyes of 10 patients who underwent unilateral frontalis suspension with a single strip of autogenous fascia. At a mean postoperative time of 10.1 ± 4.01 months, the image J software was used to measure the ratio between the nasal and temporal areas of the upper half of the palpebral fissure. The midpupil upper eyelid distance (MRD1) was also measured on the photos with the same software. The nonparametric Wilcoxon signed-rank test was used to compare the data. RESULTS: Postoperative MRD1 ranged from 2.5 to 4.7 mm (median = 3.8) on the affected side. The MRD1 for nonoperated eyelid ranged from 1.8 to 5.0 mm (median = 3.5). On the operated side, the temporal areas ranged from 50.3 to 85.7 mm (median 65.2) and nasal areas ranged from 41.5 to 72.3 (the median was 60.1). In the contralateral, nonoperated palpebral fissures, the temporal areas ranged from 42.7 to 94.3 mm (median = 54.5) and the nasal areas ranged from 36.8 to 86.1 mm (median 52.3). The T/N ratio distributions were almost identical between groups, ranging from 0.9 to 1.2 (median = 1.1) in the operated eyes and from 0.9 to 1.3 (median = 1.1) in the fellow eyes. CONCLUSIONS: In autogenous fascia frontalis suspension procedures, the upper eyelid contour of the ptotic eyelids can be adequately normalized with a single area of traction on the tarsal plate.

Lacrimal Gland Involvement in Blepharophimosis-Ptosis-Epicanthus Inversus Syndrome.

PURPOSE: To describe the involvement of the lacrimal gland (LG) in blepharophimosis-ptosis-epicanthus inversus syndrome (BPES). DESIGN: Observational, cross-sectional study. PARTICIPANTS: Twenty-one patients with BPES (10 female, 11 male) aged on average 15 years (range, 2-39 years), from 3 Brazilian medical centers and 1 Portuguese medical center. METHODS: Patients had their ocular surface evaluated with slit-lamp biomicroscopy, and tear production quantified with the Schirmer test I. The LG volumes were measured on computed tomography (CT) scans in the BPES sample and in a group of age-matched subjects imaged for nonorbital diseases. Sixteen patients were screened for mutations in the FOXL2 gene. MAIN OUTCOME MEASURES: Lacrimal meniscus height, Schirmer test I, presence of superficial punctate keratopathy (SPK), LG volume, and molecular analysis of the FOXL2 gene. RESULTS: Absence of LG was detected bilaterally in 9 patients (42.8%) and unilaterally in 2 patients (9.5%). When considering only patients with measurable LG, the median volume was 0.22 cm3 in the right eye (range, 0.06-0.36 cm3) and 0.24 cm3 in the left eye (range, 0.08-0.34 cm3). These values were significantly lower than those for the age-matched controls (median = 0.54 right eye and 0.53 left eye; P < 0.05). There was a significant association between deficiency of tear production and LG volume reduction and agenesis. Molecular analysis of the FOXL2 gene revealed the presence of 8 distinct mutations, 4 of them novel ones. A significant reduction of LG size or agenesis was associated with mutations affecting protein size (due to underlying changes in the stop codon location) or the DNA-binding forkhead domain (Fisher exact test, P = 0.021). In 3 probands, the underlying genetic defect was not found. CONCLUSIONS: This is the first study reporting LG volumes in BPES, describing a significant number of patients with LG agenesis. The association between alacrima and BPES is not incidental, and a thorough evaluation of tear production is recommended especially if ptosis surgery is planned.