Debunking the Puzzle of Eyelid Apraxia: The Muscle of Riolan Hypothesis.

PURPOSE: Apraxia of eyelid opening (AEO) has been defined by the presence of an intermittent nonparalytic bilateral loss of the volitional ability to open the eyes or to maintain the eyelids in a sustained elevated position. It is not known whether the condition represents an apraxia, a dystonia, or a freezing phenomenon, and several different nomenclatorial terms have been suggested for this condition including the so-called AEO (scAEO), blepahrocolysis, focal eyelid dystonia, and so on. The primary goal of this review is to attempt to clarify the pathogenetic mechanisms underlying scAEO as a clinical phenomenon. This review also addresses the issue of whether scAEO is part of the spectrum of blepharospasm (BSP) which includes BSP, dystonic blinks and other dystonic eyelid conditions, or whether it is a separate phenomenologically heterogeneous disease with clinical features that merely overlap with BSP. METHODS: A literature review was conducted in PubMed, MEDLINE, PubMed Central (PMC), NCBI Bookshelf, and Embase for several related keywords including the terms "apraxia of eyelid opening," "pretarsal blepharospasm," "blepharocolysis," "eyelid freezing," "eyelid akinesia," "levator inhibition," "blepharospasm-plus," as well as "blepharospasm." The clinical findings in patients with scAEO who fulfilled the classic diagnostic criteria of the disease that were originally set by Lepore and Duvoisin were included, while patients with isolated blepharospasm or dystonic blinks (DB) were excluded. In addition, electromyographic (EMG) studies in patients with scAEO were reviewed in detail with special emphasis on studies that performed synchronous EMG recordings both from the levator muscle (LPS) and the pretarsal orbicularis oculi muscle (OO). RESULTS: The apraxia designation is clearly a misnomer. Although scAEO behaves clinically as a hypotonic freezing phenomenon, it also shares several cardinal features with focal dystonias. The authors broadly categorized the EMG data into 3 different patterns. The first pattern (n = 26/94 [27.6%]) was predominantly associated with involuntary discharges in the OO muscle and has been termed pretarsal blepharospasm (ptBSP). The commonest pattern was pattern no. 2 (n = 53/94 [56.38%]), which was characterized by involuntary discharges in the OO muscle, together with a disturbed reciprocal innervation of the antagonist levator muscle and is dubbed disturbed reciprocal innervation (DRI). This EMG pattern is difficult to discern from the first pattern. Pattern no. 3 (n = 15/94 [15.9%]) is characterized by an isolated levator palpebrae inhibition (ILPI). This levator silence was observed alone without EMG evidence of contractions in the pretarsal orbicularis or a disturbed reciprocal relation of both muscles. CONCLUSION: EMG evidence shows that the great majority (84%) of patients show a dystonic pattern, whereas ILPI (16%) does not fit the dystonic spectrum. The authors propose that a spasmodic contraction of the muscle of Riolan may be the etiological basis for levator inhibition in patients with ILPI. If this is true, all the 3 EMG patterns observed in scAEO patients (ptBSP, DRI, and ILPI) would represent an atypical form of BSP. The authors suggest coining the terms Riolan muscle BSP ( rmBSP ) for ILPI, and the term atypical focal eyelid dystonia ( AFED ) instead of the term scAEO, as both terms holistically encompass both the clinical and EMG data and concur with the authors' theorem.

Orbital Vascular Anomalies: A Nomenclatorial, Etiological, and Nosologic Conundrum.

PURPOSE: Vascular anomalies are a heterogeneous group of disorders that frequently present in the periorbital region. They encompass 2 broad entities: vascular tumors, which possess a proliferative endothelium, and vascular malformations, which are basically localized defects of vascular morphogenesis. The primary goal of this review was to address inaccurate or controversial terminology in the oculoplastic literature concerning orbital and periorbital vascular anomalies and to categorize these lesions in an abridged and simplified hierarchical list that adheres as much as possible to the most recent (2018) iteration for the classification of vascular lesions proposed by the International Society for the Study of Vascular Anomalies (ISSVA). The secondary goal of this review was to review and update information regarding the genetic underpinnings of vascular anomalies and the downstream signaling pathways that are subsequently affected as a result of these genetic errors. METHODS: A literature review was conducted in PubMed, MEDLINE, PubMed Central, National Center for Biotechnology Information Bookshelf, and Embase for several related keywords including "vascular anomalies, vascular malformations, vascular tumors, and cavernous venous malformation," both with and without adding the keywords "eyelid," "orbital," and "periorbital." In addition, a detailed search was conducted for controversial or obsolete keywords like "cavernous hemangioma," "lymphangioma," and "varices," again in their systemic and orbital/periorbital context. RESULTS: Crucial issues in the 2018 ISSVA classification regarding the proper categorization of orbital vascular anomalies, particularly venous lesions, were critically evaluated and revised, and a regional, simplified, and abridged modification of the ISSVA 2018 classification was proposed. CONCLUSIONS: Interdisciplinary and intradisciplinary dialogue concerning orbital vascular anomalies is seriously compromised due to the lack of a unanimous agreement on terminology and the absence of a unified classification concept system. The authors recommend that oculoplastic surgeons adopt ISSVA terminology whenever technically possible and scientifically sound. However, they also propose modifying the ISSVA 2018 classification specifically to adapt to the peculiarities of vascular anomalies in the periorbital region. At present, the simplified classification proposed here is a preliminary first step towards managing patients with orbital vascular anomalies with greater diagnostic and therapeutic precision, until such time in the future when the entire genetic makeup of orbital vascular anomalies is more completely elucidated. Optimistically, this could pave the way for a more robust classification and the ultimate therapeutic cure.

Perioperative Prophylactic Antibiotics in 1,250 Orbital Surgeries.

PURPOSE: Intravenous antibiotic prophylaxis is used for many clean-contaminated surgeries or clean surgeries with an implant, but its value for clean orbital surgery has not been determined. This study investigated infection risks and adverse effects related to antibiotics in patients undergoing orbital surgery. METHODS: A prospective, nonrandomized comparative case series of all patients undergoing orbital surgery with participating surgeons between October 1, 2013, and March 1, 2015. Types of surgery, antibiotic regimens, corticosteroid use, antibiotic side effects, and surgical site infections (SSIs) were entered into an electronic database and subsequently analyzed. Cases in which patients received postoperative oral antibiotics were analyzed separately. RESULTS: Of 1,250 consecutive orbital surgeries, 1,225 met inclusion criteria. A total of 1208 patients were included in the primary analysis: 603 received no antibiotic prophylaxis (group A), and 605 received a single dose of intravenous antibiotic (group B). Five patients (0.42%) developed an SSI, 3 in group A and 2 in group B. The difference in SSI rates was not statistically significant between the 2 groups (p = 0.66). Antibiotic prophylaxis, alloplastic implants, paranasal sinus entry, and corticosteroid use were not associated with differences in SSI rates. All SSIs resolved on a single course of oral antibiotics; an implant was removed in 1 case. There were no complications associated with a single dose of intravenous prophylaxis. However, 12% of 17 patients (group C) who received 1 week of oral postoperative prophylactic antibiotics developed antibiotic-related complications (diarrhea, renal injury), yielding a number needed to harm of 8.5. CONCLUSIONS: In this large series, antibiotic prophylaxis does not appear to have reduced the already low incidence of SSI following orbital surgery. Given the detriments of systemic antibiotics, the rarity of infections related to orbital surgery, and the efficacy of treating such infections should they occur, patients undergoing orbital surgery should be educated to the early symptoms of postoperative infection and followed closely, but do not routinely require perioperative antibiotics.

An unusual case of idiopathic calcinosis of the eyelid.

Idiopathic subepidermal calcinosis, also known as idiopathic calcinosis cutis, is a rare condition, which typically presents as a small, hard, subepithelial mass in the absence of systemic disease and trauma. We report on an atypical case of idiopathic calcinosis, which appeared in an otherwise healthy 61-year-old female along the tarsus of her right upper eyelid. The purpose of this case report is to demonstrate idiopathic calcinosis in an unusual location that is deep to the dermis and to discuss the vast differential diagnosis of this entity.

Embryologic and Fetal Development of the Human Orbit.

PURPOSE: To review the recent data about orbital development and sort out the controversies from the very early stages during embryonic life till final maturation of the orbit late in fetal life, and to appreciate the morphogenesis of all the definitive structures in the orbit in a methodical and timely fashion. METHODS: The authors extensively review major studies detailing every aspect of human embryologic and fetal orbital morphogenesis including the development of extraocular muscles, orbital fat, vessels, nerves, and the supportive connective tissue framework as well as bone. These interdisciplinary studies span almost a century and a half, and include some significant controversial opposing points of view which the authors hopefully sort out. The authors also highlight a few of the most noteworthy molecular biologic studies regarding the multiple and interacting signaling pathways involved in regulating normal orbital morphogenesis. RESULTS: Orbital morphogenesis involves a successive series of subtle yet tightly regulated morphogenetic events that could only be explained through the chronological narrative used by the authors. The processes that trigger and contribute to the formation of the orbits are complex and seem to be intricately regulated by multifaceted interactions and bidirectional cross-talk between a multitude of cellular building raw materials including the developing optic vesicles, neuroectoderm, cranial neural crest cells and mesoderm. CONCLUSIONS: Development of the orbit is a collective enterprise necessitating interactions between, as well as contributions from different cell populations both within and beyond the realm of the orbit. A basic understanding of the processes underlying orbital ontogenesis is a crucial first step toward establishing a genetic basis or an embryologic link with orbital disease.

Anatomic Considerations in Thyroid Eye Disease.

PURPOSE: To review and summarize the clinical findings in thyroid eye disease (TED) related to the unique anatomical structures of the eyelids and orbit. METHODS: A PubMed search was performed searching for anatomical relationships between eyelid and orbital anatomy and the clinical findings of TED. RESULTS: The major clinical findings associated with TED are varied. They typically involve both the eyelid and the orbit. In the eyelid, usual findings include upper eyelid retraction, contour abnormalities, eyelid edema, prolapsed orbital fat, conjunctival injection and chemosis, caruncular edema, and meibomian gland dysfunction. The exact causes of these changes remain a matter of controversy, but numerous hypotheses have been proposed, most with limited experimental support. In the orbit, inflammation and congestion characterize the acute active phase, with tissue expansion and fibrosis persisting into the chronic inactive phase. All of these findings result, at least in part, from the unique anatomy of the eyelids and orbit and their interaction with the immunologic processes underlying TED. Here we review these major characteristics of TED, with special reference to their anatomic relationships. CONCLUSIONS: The major findings characterizing TED are related to unique anatomic features in the eyelid, including Müller muscle, the levator palpebrae superioris muscle, and suspensory ligaments of the conjunctiva. In the orbit, Graves-related remolding results from the special physiologic features of the extraocular muscles, the structure of the orbital walls and their relations to adjacent paranasal sinuses, and the reaction of orbital fat to immunologic challenges.

Seminoma Metastatic to the Orbit.

PURPOSE: Seminomas are solid tumors in young men, but which rarely metastasize to the orbit. The authors review the known literature on seminoma metastatic to the orbit, and describe an additional case in a 33-year-old man. METHODS: A literature search was performed on the MEDLINE database using keywords "seminoma," "testicular germ-cell tumors," "testicular cancer," "testicular neoplasm," "orbital metastasis," and "germ-cell neoplasms." RESULTS: Malignant neoplasms of the testis account for only 1% of cancers in men. None-the-less, testicular germ cell seminoma is the most common solid tumor found in young men between the ages of 15 and 39. Only seven previous cases have been mentioned in the literature. The pathogenesis remains unclear although genetic, environmental, and maternal factors may play a role. The number of cases is too few to determine the best treatment options, but surgical excision and adjunctive orbital radiotherapy appear to be most appropriate. CONCLUSIONS: Although metastases to the orbit are rare, seminoma should be considered in the differential diagnosis of all young men with proptosis.

The Hook and Release Technique During Enucleation Surgery.

PURPOSE: To describe the results and potential benefit of direct muscle release from the globe during enucleation surgery without identifying sutures in the rectus muscle insertion sites, a technique referred to as the hook and release technique. METHODS: Single center, retrospective chart review of patients who underwent enucleation with direct removal of the rectus muscles without identifying sutures in their insertion sites between January 2011 and September 2015 was carried out. The inclusion criteria were primary enucleation without previous strabismus surgery, retinal detachment surgery, or orbital surgery that entered the fibrous connective tissue framework. Forty charts of enucleated patients that had direct release of their extraocular muscles without identifying sutures before releasing them from the globe were identified and reviewed. The primary outcome measure was intraoperative or immediate postoperative complications. This retrospective chart review was performed with research ethics board approval and in compliance with the Declaration of Helsinki. RESULTS: Data show that following the hook and release technique, the rectus muscles were easily located and reconnected to the orbital implant wrap. The oblique muscles were not reattached. In each of the 40 patients, the 4 rectus muscles were easily located by gently applying traction anteriorly at the conjunctiva/Tenons' edge using double-pronged skin hooks. There was no instance of a lost or slipped muscle following the hook and release technique. CONCLUSION: The hook and release technique is a simple and efficient method to remove the 4 rectus muscles from the globe and still easily locate them. They are not "lost" and do not "slip out of position" but held in place by the orbital connective tissue framework and the extraocular muscle pulley system. This technique has been very helpful teaching resident staff how to do enucleation surgery as it avoids the more time consuming placement of double-armed locking sutures through the rectus muscle insertions and the potential risk of globe penetration while the muscles remain attached to the eye. If the surgeon desires to attach the muscles to the orbital implant, then sutures are passed after the eye is removed, thus eliminating the worry of globe penetration and avoiding accidentally cutting preplaced extraocular muscle sutures during the remaining enucleation procedure.

Prophylactic Antibiotics for Enucleation and Evisceration: A Retrospective Study and Systematic Literature Review.

PURPOSE: To report surgical site infection (SSI) rates of eviscerations and enucleations with implants performed without perioperative intravenous (IV) antibiotics or postoperative oral antibiotics, and to give SSI prevention recommendations. METHODS: A single-center retrospective chart review was performed after obtaining institutional review board approval. Charts were found by Current Procedural Terminology codes. Demographics, surgical indication, procedure, implant, antibiotic use, and postoperative course were recorded. SSIs occurring within 30 days after surgery were reviewed and postoperative infection rates were determined. RESULTS: Four hundred eighty-one cases from January 1999 to December 2015 were analyzed. There were 102 eviscerations with implants, 314 enucleations with implants, 23 enucleations without implants, 23 implant exchanges, 15 implants placed secondarily after enucleation, and 4 implant removals. Seventy cases (14.6%) were given perioperative IV antibiotics, and in this group one periorbital infection occurred unrelated to orbital surgery (1.4%). Of the 411 cases (85.4%) not given perioperative IV antibiotics, 1 of 87 eviscerations with implants developed an SSI (1.1%), 2 of 273 enucleations with implants developed SSIs (0.7%), and none of the 13 enucleations without implants developed SSIs. CONCLUSIONS: To our knowledge, this is the first published case series reporting SSI rates of enucleations and eviscerations with implants performed without perioperative IV antibiotics or postoperative oral antibiotics. With infection rates comparing favorably to other case series where antibiotics were given, the routine use of perioperative IV antibiotics and postoperative oral antibiotics for enucleations and eviscerations may not be indicated.

Efficacy and Safety of Low-Dose Iodine Plaque Brachytherapy for Juxtapapillary Choroidal Melanoma.

PURPOSE: To evaluate low- vs high-dose plaque brachytherapy for juxtapapillary choroidal melanoma. DESIGN: Retrospective interventional case series. METHODS: Setting: Single institution. STUDY POPULATION: Forty-seven patients with juxtapapillary choroidal melanoma. INTERVENTION: Iodine-125 plaque brachytherapy. Eyes were divided into apex low-dose (LD) and high-dose (HD) groups (≤ or > median apex dose 84.35 Gy). Main outcome measures were time to distant failure, local failure, death, enucleation, radiation retinopathy, optic neuropathy, and best-corrected visual acuity (BCVA). RESULTS: Freedom from distant failure rates were 96% and 95% in apex LD and HD groups at 5 years and 77% and 95% at 10 years, respectively (P = .84). Freedom from local failure rates were 90% in the apex LD group vs 89% in the HD group at 5 and 10 years (P = .96). Apex LD and HD groups did not differ for time to death or enucleation. Five- and 10-year freedom from radiation retinopathy and optic neuropathy rates were higher in the apex LD than HD group. Loss of ≥3 BCVA lines, final BCVA 20/40 or better, and final BCVA 20/200 or worse were more favorable in the 5 mm LD compared to HD group. Visual acuity outcomes did not differ between apex LD and HD groups. CONCLUSIONS: Low-dose iodine-125 plaque brachytherapy (67.5-81 Gy at tumor apex) provides safe and effective tumor control for juxtapapillary choroidal melanoma and may be associated with reduced radiation toxicity. Larger trials are needed to determine the optimal therapeutic dose for juxtapapillary choroidal melanoma.

Subepidermal Calcinosis in the Ocular Adnexa: A Systematic Review.

PURPOSE: To review the clinical and histopathologic findings associated with subepidermal calcinosis of the eyelids. METHODS: A systematic review of the literature on subepidermal calcinosis of the eyelids was performed. Cases included were idiopathic in nature and met the histologic criteria for subepidermal calcinosis with calcium deposits in the dermis of the skin. RESULTS: Twenty-one publications presenting 53 cases of subepidermal calcinosis involving the eyelids were published between 1970 and 2016. Males were affected more than females (67% vs. 33%), and 89% of patients were 21 years of age or younger. A total of 63% were non-Caucasian. Most cases involved a single lesion (82%), and lesions were most frequently located on the upper eyelid (63%). In 81% of cases, the lesion was less than 5 mm in diameter. When reported, the treatment of choice was complete surgical excision. CONCLUSIONS: Subepidermal calcinosis should be considered in the differential diagnosis of idiopathic lesions on the eyelid, particularly in young males with no history of systemic disease or laboratory abnormalities. These nodules usually present as painless, small, firm, mobile solitary cutaneous lesions with a predilection for the upper eyelid. Diagnosis is confirmed by histopathology, and treatment is with surgical excision.

Localizing the Lost Rectus Muscle Using the Connective Tissue Framework: Revisiting the Tunnel Technique.

PURPOSE: To describe a technique for localizing a lost rectus muscle during strabismus or retinal surgery or following trauma. METHODS: In this single center, retrospective chart review, 5 patients were identified between January 2012 and June 2016 with a lost rectus muscle; 3 during strabismus surgery and 2 post trauma. The inclusion criteria included a lost rectus muscle during strabismus surgery, or a disinserted/lacerated rectus muscle following ocular/orbital trauma. The primary outcome measure was successful reattachment of the rectus muscle. RESULTS: The lost rectus muscle was identified in each patient and reattached to the globe by gently applying traction anteriorly at the conjunctiva/Tenon edge using double-pronged skin hooks and following the path of the rectus muscle through its Tenon capsule tunnel where it remained attached by suspensory ligaments. There was no instance where orbital fat was obscuring or blocking the view of the lost rectus muscles. There were no other complications associated with the procedure. CONCLUSIONS: The authors describe a simple and effective method in 5 patients to localize a lost rectus muscle based on knowledge of the orbital connective tissue framework.

Piezosurgery in External Dacryocystorhinostomy.

BACKGROUND: Dacryocystorhinostomy (DCR) can be performed via an external or endoscopic approach. The use of ultrasonic or piezosurgery has been well described for endoscopic DCRs but is lacking for external DCRs. This study presents a case series of external DCRs performed using piezosurgery evaluating results and complications. METHODS: Prospective, consecutive case series of patients undergoing primary external DCR for lacrimal drainage insufficiency. A standard external DCR technique was used using 1 of 2 piezosurgery systems for all bone incision. All patients received silicone intubation to the lacrimal system. Surgical outcome was measured in terms of patient-reported epiphora as follows: 1) complete resolution, 2) improvement >50%, 3) improvement <50%, and 4) No improvement. Intra and postoperative complications were also recorded. RESULTS: Fifty-two patients, 14 male and 38 female, were included in the study, with 2 patients having bilateral surgery. The average age of the patients was 55.8 years. The average length of follow up was 221 days. Surgical outcomes showed 72% of patients with complete resolution of epiphora and 21% with >50% improvement. There were 4 patients (7%) who had <50% improvement. There was 1 (2%) intraoperative complication and 2 (4%) postoperative complications recorded. CONCLUSIONS: Piezourgery is a viable modality for performing external DCRs. The lack of surgical complications shows a potential for decreased soft tissues damage. The surgical success rate based on patient-reported epiphora is similar to those published for mechanical external DCRs. This modality may benefit the novice surgeon in the reduction of soft and mucosal tissue damage.

Embryologic and Fetal Development of the Human Eyelid.

PURPOSE: To review the recent data about eyelid morphogenesis, and outline a timeline for eyelid development from the very early stages during embryonic life till final maturation of the eyelid late in fetal life. METHODS: The authors extensively review major studies detailing human embryologic and fetal eyelid morphogenesis. These studies span almost a century and include some more recent cadaver studies. Numerous studies in the murine model have helped to better understand the molecular signals that govern eyelid embryogenesis. The authors summarize the current findings in molecular biology, and highlight the most significant studies in mice regarding the multiple and interacting signaling pathways involved in regulating normal eyelid morphogenesis. RESULTS: Eyelid morphogenesis involves a succession of subtle yet strictly regulated morphogenetic episodes of tissue folding, proliferation, contraction, and even migration, which may occur simultaneously or in succession. CONCLUSIONS: Understanding the extraordinary process of building eyelid tissue in embryonic life, and deciphering its underlying signaling machinery has far reaching clinical implications beyond understanding the developmental abnormalities involving the eyelids, and may pave the way for achieving scar-reducing therapies in adult mammalian wounds, or control the spread of malignancies.