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Purpose To compare muller muscle conjunctival resection (MMCR) and external levator advancement (ELA) in terms of objective and subjective symmetry in unilateral ptosis. Methods The patients who underwent unilateral MMCR (group1, n = 25) and ELA (group2, n = 25) were reviewed retrospectively. With the written algorithm, margin reflex distance-1 (MRD1), localization of the contour peak, and the percentage of overlapping curvatures (POC) of both upper eyelid curves were calculated. Semi-automated measurements were compared with the manual measurements. The correlation between objective and subjective symmetry was evaluated. Results Preoperative levator functions were similar in both groups (13.93 ± 3.25 mm and 13.3 ± 2.86 mm, respectively). We found strong correlations between manual and semi-automated measurements (ICC: 0.942, 95% CI = 0.924–0.956, p Conclusions Besides subjective symmetry, MMCR and ELA are equally effective in ensuring symmetry objectively, as measured with MRD1 and contour peak symmetry and POC in unilateral ptosis. POC may be used as a criterion of symmetry in the evaluation of ptosis.

Background To investigate the clinical characteristics of children with congenital ptosis, with particular attention given to the incidence of anisometropia, and the difference in axial length (AL) between the right and left eyes. Methods The medical charts of 55 patients with congenital ptosis at Niigata University Medical and Dental Hospital were retrospectively analyzed. Clinical characteristics, including age, cycloplegic refraction, AL, and the presence of amblyopia and its causes were analyzed. Results Age at the initial visit was 16 ± 20 (mean ± standard deviation, the same applies below) months. Of the 49 patients whose cycloplegic refraction was measured, hyperopic anisometropia, defined as ≥ one-diopter difference in spherical equivalent (SE), was observed in 1/11, 9/27 and 5/11 patients with bilateral, right, and left ptosis, respectively. Among 14/38 patients with hyperopic anisometropia involving unilateral ptosis, 13 demonstrated a larger SE in the ptotic eye than in the non-ptotic eye. The inter-eye difference in AL (AL of the ptotic eye minus that of the non-ptotic eye) in six patients with unilateral ptosis and hyperopic anisometropia ipsilateral to the ptotic eye (-0.29 ± 0.40 mm) was significantly smaller than that in three patients with unilateral ptosis and no hyperopic anisometropia (0.38 ± 0.29 mm). Conclusions At our institute, children with congenital ptosis had a high incidence of hyperopic anisometropia ipsilateral to the ptotic eye. Furthermore, this condition was associated with a shorter axial length. These results indicate that refractive correction for hyperopic anisometropia is important for proper visual development in children with congenital ptosis.

SUMMARY Patients with aponeurotic blepharoptosis who are treated with external levator advancement may experience complications such as upper eyelid height asymmetry and often require revision surgery. The authors assessed the efficacy of Muller muscle-conjunctival resection in cases with upper eyelid height asymmetry following bilateral external levator advancement. The authors used retrospective analysis of 11 cases between September of 2016 and October of 2018 with eyelid asymmetry following bilateral external levator advancement. Following a positive phenylephrine test, these patients underwent unilateral Muller muscle-conjunctival resection revision surgery to treat the undercorrected eyelid. Preoperative and postoperative marginal reflex distance 1, symmetry outcomes, and clinical outcomes of patients were evaluated after the Muller muscle-conjunctival resection. The average patient age was 54.81 ± 3.95 years (range, 37 to 69 years; median, 56 years); seven patients (63.6 percent) were women. Hering dependency was seen in all patients before the external levator advancement. A total of three patients underwent bilateral external levator advancement simultaneously, and eight patients underwent bilateral external levator advancement sequentially. The phenylephrine test was positive in all patients before the Muller muscle-conjunctival resection. Symmetry outcomes were assessed after the revision surgery as perfect (

Objective Kallmann syndrome (KS) may be accompanied by anosmia or hyposmia and midline defects. We present an overweight 16-year-old boy with a lack of puberty, anosmia, congenital right eye ptosis, and normal intellectual function. Methods Testicular ultrasonography was performed. Whole-exome sequencing was performed on peripheral blood specimens. Genetic results were confirmed by Sanger sequencing. Anosmia was evaluated quantitatively using the Korean version of the Sniffin' stick test II. Results Our patient presented with a complaint of lack of body hair growth and small penile size with no remarkable medical history. He was the second son of third-degree consanguineous healthy parents. Physical examination revealed pubertal Tanner stage I. Congenital right eye ptosis and obesity were noted. Anosmia was confirmed. The laboratory evaluation revealed a low serum level of testosterone, follicle-stimulating hormone, and luteinizing hormone. An X-linked recessive homozygous mutation, c.628_629 del (p.1210fs∗) in exon 5 of the ANOS1 gene was revealed and was also found in the patient's uncle and great uncle on the mother's side. Conclusion To date, approximately 28 ANOS1 mutations producing KS phenotypes have been described. However, to the best of our knowledge, this particular X-linked recessive mutation has not been previously reported in KS. Furthermore, ptosis is a rare finding in KS literature. Identification of these cases increases awareness of the phenotypic heterogeneity in novel forms of KS, thereby expediting early definitive treatment, which may prevent the development of further complications.

Background Ptosis can be a manifestation of a more serious situation. Hence, the analysis of the complaint and the search for etiology are crucial in such cases. Ptosis has many causes; some of them lead to unilateral ptosis while others cause bilateral ptosis. For instance, myasthenia gravis is a cause of bilateral ptosis while oculomotor nerve palsy induces unilateral disease. Proper evaluation of the patient and identification of the cause are important to achieve accurate management and good prognosis. Case presentation A 47-year-old male patient attended the ER complaining of dropping the right eye lid of 2 days’ duration. There was no associated pain or diplopia. On examination, the extraocular muscles’ (EOM) motility was intact, normal pupil and corneal reflexes, and there was swelling of the upper eyelid. Ophthalmological examination revealed normal anterior and posterior chambers as well as the vitreous and retina. The patient had a previous history of traumatic intracranial hemorrhage that was resolved without surgical intervention. He also had diabetes mellitus and hypertension. The patient was transferred to the MRI unit to perform MRI study of the brain and orbit with MRA and IV contrast administration. MRI findings confirmed the diagnosis of LPS myositis, and the patient received medical treatment and improved. Conclusion Proper radiological diagnosis leads to accurate management and achieves rapid recovery and optimal patient care.

Purpose The aim of this study was to compare the accuracy and clinical outcome of closed versus open-sky approach of Muller muscle–conjunctival resection for correction of mild and moderate ptosis with good levator function and positive phenylephrine test. Patients and methods A prospective comparative nonrandomized interventional study was conducted on patients attending the Outpatient Clinic of the Ophthalmology Department in Al Azhar University Hospital (Damietta branch) during the period from April 2018 to August 2019. The study was conducted on 50 eyelids of 45 patients with mild to moderate ptosis with good levator function and positive phenylephrine test. The patients were treated with either a closed or open-sky approach of Muller muscle–conjunctival resection, with 25 eyelids being treated by each approach. A total of 40 cases had unilateral ptosis and five cases were bilateral. All cases were congenital except one case that was a Pancoast tumor causing preganglionic Horner’s syndrome. The eyelid elevation, marginal reflex distance 1, lid contour, dry eye, and symmetry between both eyelids were the main outcome measures. Results There were significant differences between the two groups regarding the postoperative marginal reflex distance 1 (3.64±0.44 mm in the open-sky approach vs. 3.18±0.53 mm in the closed approach, P=0.002). There was significantly more correction of the degree of ptosis in the open-sky approach. Undercorrection was reported in 4% of the cases in the open-sky approach versus 28% in the closed approach, whereas temporal flare was reported in only 4% of the cases in the open-sky approach (P=0.0047). There was a significant reduction in the postoperative tear film in the patients treated with the closed approach in comparison with those treated with open-sky approach (P=0.007). Conclusion The open-sky approach offered advantages over the closed approach, including the opportunity for adjustment of the lid height and reducing the risk of dry eye.

Xia-Gibbs syndrome (XGS) is a very rare genetic condition. The clinical spectrum is very broad and variable. The phenotype and evolution in a Congolese boy with XGS have been reported. At 6 years he had speech delay, drooling, marked hyperactivity, attention deficit, aggressive behavior, and intellectual disability. Dysmorphological evaluation revealed strabismus, mild unilateral ptosis, uplifted ear lobes, flat philtrum, thin upper lip vermillion, high arched palate, and flat feet. Patient-only whole exome sequencing identified a known pathogenic frameshift variant in the AHDC1 gene [NM_001029882.3(AHDC1):c.1122dupC;(p.Gly375ArgfsTer3)]. The clinical follow-up revealed the deterioration of his fine motor skills and significant cerebellar phenotype including tremor, pes cavus, and gait instability at the age of 12 years. This patient was compared with three previously reported patients with the same variant but did not identify a consistent pattern in the evolution of symptoms with age.

Background: Cysticercosis is a parasitic infection caused by Cysticercus cellulosae, the larval form of cestode, Taenia solium. It is a preventable cause of blindness endemic in many developing countries especially where there is poor hygiene and sanitation. Humans become infected by ingestion of contaminated food and water. The most preferred locations are central nervous system (CNS), eyes, heart, bladder, muscles and subcutaneous tissues. Ocular cysticercosis can be extraocular or intraocular and may show different clinical presentations. We report a case of ocular cysticercosis which presented with unilateral ptosis and mild pain. Case: A young male patient presented with mild swelling and drooping of right upper eyelid for 2 weeks associated with mild pain and redness on first 3 days. Ocular examination revealed ptosis and mild swelling on temporal side of right upper eyelid. The swelling was soft in consistency with no tenderness. Levator function was good with normal Bell’s phenomenon and ocular motility was in full in all cardinal gazes. The computed tomography imaging of the orbit showed bulky right superior rectus muscle with ring enhancing hypodense lesion suggestive of right eye myocysticercosis involving superior rectus muscle. The patient was treated with oral albendazole and oral prednisolone for 4 weeks and a significant improvement of ptosis was seen at 1 month follow up visit. Conclusion: Extraocular muscle cysticercosis should be highly suspicious in young patients with unilateral acquired blepharoptosis. Radiological investigations play a vital role in diagnosis and a complete resolution can be achieved with standard medical therapy alone. Keywords: Cysticercosis, Myocysticercosis, Orbital cysticercosis, Ptosis.

BackgroundHorner’s syndrome (HS) is characterized by unilateral ptosis, ipsilateral miosis with normally reactive pupil, and in some cases, ipsilateral facial anhidrosis.Case presentationWe report an adult male presenting with ptosis. Neurological examination revealed ptosis and miosis in the right eye. Anhidrosis was not observed, and the patient did not complain about it. Laboratory tests were within normal limits. He had searched a general practitioner because of purulent nasal discharge, nasal obstruction, and anosmia. He was diagnosed with acute rhinosinusitis. Symptomatic treatment was started. The subject had full recovery of the nasal symptoms, but his right upper eyelid was drooping progressively. In the follow-up, after the third month, the patient was recovering from ptosis and miosis. In 1 year, the ptosis was minor with less than 1 mm and miosis was no more observed.DiscussionA few cases of HS secondary to a sinus infection have been reported in the literature. To the authors’ knowledge, there are only two case reports of individuals who developed sinusitis and Horner’s syndrome. The present case is the first to present isolated HS features. Our report suggests that sinusitis should be listed as a probable cause of isolated Horner’s syndrome. Moreover, patients presenting with this presentation probably have a good prognosis.

Myasthenia gravis and Graves’ disease are known to co-exist in adults, yet there have only been a small number of paediatric cases reported. We report a 5 year old female who was diagnosed with ocular myasthenia gravis after presenting with unilateral ptosis and subsequently found also to have Graves’ disease. She was treated successfully with pyridostigmine, corticosteroids and carbimazole without symptom recurrence or progression to generalised myasthenia gravis. The aetiology of the coexistence is not fully understood, nor is the relationship between the two disorders’ presentation and treatment. We discuss the variation in clinical presentation of myasthenia gravis between populations and when associated with autoimmune thyroid disease, potential HLA-related genetic susceptibility and the varying approaches to treatment of the co-existent disorders.

BACKGROUND Frontalis flap advancement is an alternative means of congenital ptosis repair from frontalis suspension utilizing autologous fascia or allogenic implants. Variations in technique, including flap division, location and number of skin incisions, and dissection planes, are described in the literature. MATERIALS AND METHODS A retrospective case series of patients with congenital myogenic ptosis treated with simplified, minimal dissection frontalis flap advancement involving a single upper eyelid crease incision with dissection in the preseptal and subcutaneous planes without division to mobilize the frontalis flap. Inclusion criteria: pediatric patients age

Highlights • Distant silicone oil migration may occur following retina surgery. • Silicone oil in periocular tissue results in granulomatous reaction. • It may resemble a periocular xanthelasma-like skin lesion. • Tissue diagnosis is essential for confirmation in such cases., Introduction Silicone oil has been used for many years in retinal surgeries for retinal detachment. One of its reported complications is oil migration to the periorbital area, resulting in granulomatous reaction. Presentation of case A 56-year-old lady, with history of retinal detachment that was repaired by vitrectomy, silicone oil removal and epi-retinal membrane peeling, presented to us with unilateral ptosis and a skin lesion that resembled xanthelasma. Discussion Histopathology of this lesion showed silicone oil infiltrating the surrounding connective tissue and fat with absence of foamy histiocytes. Conclusion We are reporting a case of silicone oil migration with pseudo-xanthelasma lesion. This has been reported only twice to the best of our knowledge in the English-written literature.

A 51-year-old woman with a history of acute myeloid leukemia (AML) in remission and no past ocular history presented emergently at the University of Kansas Hospital with binocular diplopia and right-sided ptosis of 3 days’ duration. The patient also complained of fatigue. She originally attributed her symptoms to allergies, stating that she had also recently experienced mild right-sided eye discharge and sinus pressure. Past medical history was significant for mild hypertension and AML with myelodysplasia-related features that was treated about 6 months prior with haploidentical peripheral stem-cell transplantation. The patient was a former smoker (40 pack-years) and had a family history of glaucoma (sister). She had recently finished a regimen of fluconazole for prophylaxis after stem-cell transplantation and was also taking acyclovir, letermovir, dapsone, and tacrolimus.

Background Guillain-Barré syndrome is an acute inflammatory polyradiculoneuropathy. Nearly half of patients with Guillain-Barré syndrome have cranial nerve involvement. However, isolated bilateral ptosis without ophthalmoplegia is a rare manifestation, and isolated unilateral ptosis without ophthalmoplegia in Guillain-Barré syndrome has not previously been reported in the literature. Furthermore, only few cases of Guillain-Barré syndrome with cranial nerve enhancement visualized by gadolinium-enhanced magnetic resonance imaging have previously been reported. We describe the first reported case of unilateral ptosis without ophthalmoplegia in Guillain-Barré syndrome and associated multiple cranial nerve enhancement seen by gadolinium-enhanced magnetic resonance imaging. Case presentation Our patient was a 55-year-old Sinhalese man who was admitted to a tertiary care hospital in Sri Lanka with acute-onset progressive weakness in the lower limbs followed by the upper limbs. He had bilateral symmetrical flaccid quadriparesis with absent reflexes and flexor plantar response. Left-sided isolated partial ptosis without associated ophthalmoplegia was noted with normal pupils. The patient’s neurological examination was otherwise normal. A nerve conduction study showed a severe demyelinating type of polyneuropathy. No decremental response to repetitive nerve stimulation was observed, and the result of a single-muscle-fiber electromyogram was negative. A diagnosis of Guillain-Barré syndrome was made, and the patient was treated with intravenous immunoglobulin. His condition gradually deteriorated over the next few days, and he became quadriplegic despite the completion of immunoglobulin therapy. Later he developed multiple cranial nerve palsies, including bi-lateral lower motor neuron type facial nerve palsy, and he required mechanical ventilation. By this time, he had complete left-sided ptosis with a normal right eye. He never developed ophthalmoplegia or ataxia. Magnetic resonance imaging of the brain showed contrast enhancement in the intracranial part of multiple cranial nerve roots and basal leptomeninges. He gradually improved with plasmaparesis, and ptosis was the first to improve. Conclusions Even though Guillain-Barré syndrome was recognized a century ago, there are still many unanswered questions about it and its florid presentation. Large-scale studies are needed for better understanding of its pathophysiology and prototypes and to find answers for still-unanswered questions. The clinician must have a high index of suspicion and be familiar with mimics and prototypes to diagnose Guillain-Barré syndrome accurately without delay.

Purpose: To report a case of advanced childhood glaucoma secondary to congenital ectropion uveae (CEU). Case Report: The patient was a seven-year-old boy with unilateral glaucoma secondary to CEU and facial asymmetry, mild unilateral ptosis, and proptosis in the left eye. The intraocular pressure (IOP) was 28 mmHg and cup-to-disc ratio was 0.8 in the left eye. After starting glaucoma medication, IOP decreased to 21 mmHg. In view of the uncontrolled IOP with medication and high cup-to-disc ratio and increased axial length of the left eye, mitomycin-C (MMC)-augmented trabeculectomy was planned. Despite sub-tenon MMC injection and bleb needling, the bleb failed after six months, and we had to perform a shunt procedure to control the IOP. Conclusion: Although CEU is rare, ophthalmologists should be familiar with this syndrome because of the high frequency of glaucoma and its challenging management during childhood.

Objective: Our aim was to analyze the success rates of patients who underwent levator aponeurosis or frontal suspension with silicone tube surgery due to blepharoptosis according to the elevator function (LF) and to compare our results with the literature. Material and Methods: We included twenty-five eyes of 47 patients who had levator aponeurosis or frontal suspension with silicone tube surgery in this study. The patients were grouped as good, moderate, and poor according to their LF. Good and moderate patients had levator aponeurosis while poor patients had frontal suspension with silicon tube surgery. The follow-up period after surgery was 2 to 36 months. The results were evaluated with margin reflex distance (MRD) which greater than 2 mm was considered as successful, between 1 and 2 mm was satisfactory, and less than 1 mm was unsuccessful. Also, patients required revision surgery was considered as unsuccessful. Results: Twenty-nine (61.7%) men and 18 (38.3%) women with a mean age of 35.16 years (range = 0-84 years) were included in this retrospective study. Thirteen cases (27.7%) had bilateral, and 34 cases had unilateral ptosis(72.4%). Eight of the 13 patients with bilateral ptosis had bilateral and 5 of them had unilateral surgery. Blepharoptosis was due to congenital (60.0%), aponeurotic (19%), traumatic (1.8%), Horner's syndrome (1.8%) and myotonic dystrophy (1.8%). The preoperative mean MRD value was 0.56 ± 0.85 mm (0-3 mm). The levator function (LF) was poor in 18 eyes (32.7%), moderate in 9 eyes (16.4%) and good in 28 eyes (50.9%). Thirty-seven patients (67.3%) underwent levator aponeurosis, and 18 patients had frontal suspension with silicon tube surgery. In our postoperative controls, 33 patients were considered as successful. Three patients were considered as satisfactory, and one patient was considered unsuccessful. Discussion: Levator aponeurosis and frontal suspension with silicone tube surgeries are both effective surgical methods to treat ptosis. Postoperative success is positively affected by determining the surgery method, according to LF. Our results showed that elevator aponeurosis surgery is more satisfying in good and moderate cases, and frontal suspension with silicone tube is best in poor cases.

BACKGROUND This study aims to evaluate the effect of a modified approach on severe congenital ptosis treatment. METHODS Through anterior approach, Muller muscle was preserved, meanwhile the upper tarsus was suspended to combined fascia sheath (CFS) and levator muscle (LM) complex. The main outcome measures included marginal reflex distance1 (MRD1), palpebral fissure height (PFH), MRD1 regression, MRD1 improvement, and patient self-satisfaction. RESULTS There were 70 patients (90 eyelids) with severe congenital ptosis received treatment of modified operation, including 20 bilateral and 50 unilateral ptosis. The average LF was 2.53 ± 1.06 mm. The preoperative MRD1 and PFH was -0.06 ± 0.76 mm and 4.25 ± 0.85 mm, respectively. The follow-up was at least 6 months with average of 12.67 ± 4.92 months. The immediate postoperative MRD1 and PFH average was 4.52 ± 0.39 mm and 9.24 ± 0.26, respectively. The last follow-up MRD1 and PFH average was 2.43 ± 0.57 mm and 7.16 ± 0.69 mm, respectively, which was improved significantly (P < 0.01). There were variety degrees of MRD1 regression especially in the first month after operation, and the last follow-up MRD1 regression was 2.09 ± 0.67 mm. The lagophthalmos was obvious immediately after operation and regularly released after 6 months. Objective curative effect assessment showed 78 (86.7%) satisfactory eyes, 4 (4.4%) improved eyes, and 8 (8.9%) noneffective eyes. Two noneffective eyes required reoperation of frontalis suspension. Self-satisfaction rate was 93.3%. Parameters analysis showed that LF had positive correlation with last follow-up MRD1 and negative correlation with MRD1 improvement (P < 0.01). Five eyes had levator shorten history, which was associated remarkably with CFS + LM curative effect (P < 0.01). Mean swelling time was 0.53 ± 0.41 months, and no complications were observed until the last visit. CONCLUSIONS This modified method gives powerful correction and vivid eyelid contour, featured with simple surgical procedures, few complications, and satisfactory effect, which is worth to wide application.

PURPOSE: To report case of isolated orbital cysticercosis METHODS: Patients with drooping of eyelid were referred to our hospital. Patients were evaluated and investigated to find the cause of ptosis. RESULTS: Two patients with acute unilateral ptosis were referred to our hospital. Patients were investigated, blood test, stool tests and MRI were done to rule out the causes. In both the patients ptosis was due to involvement of LPS muscle by cysticercus larva. All the patients gave history of excessive consumption of cabbage and pork. The mainstay of treatment included oral albendazole (15 mg/kg/day) and oral prednisolone (1mg/kg/day) for 4 weeks. After 4 weeks, oral albendazole was stopped and oral prednisolone was slowly tapered over the next one month. The effect of above treatment was seen after few days with regression of swelling and improvement of signs and symptoms. CONCLUSION: Orbital cysticercus stands as one of the major causes of ptosis in the lower socioeconomic status people who are consuming mainly unhygienic leafy vegetables such as cabbage, burgers and undercooked pork. Therefore, these patients should be vigorously evaluated on scans (MRI and CT) so that early diagnosis can be made and treatment can be given to prevent the permanent visual loss or dissemination to brain.

Introduction Isolated Sphenoid Sinusitis and sinus lesions is a rare entity accounting for just 1-3% of all Sinus afflictions. Most have occurred in men between the ages of 30 and 40 years. Case Report A case of right sphenoid sinus mucocele is reported in a male patient aged 68 years, with size of the lesion (35 x 34 mm) detected by CT & MRI scans. The patient presented with a 3 weeks history of unilateral ptosis, diplopia, and photophobia. He also complained of bilateral nasal obstruction, nasal stuffiness, and a mucoid nasal discharge. Endoscopic decompression of the right sphenoid sinus was performed, and approximately 160 ml of thick, sterile mucoid secretion was aspirated. Despite the size of the mucocele, no significant destruction of the sphenoid walls was evident. Postoperatively within 15 days the patient's symptoms improved significantly. Conclusion The Nasal Endoscope has revolutionised sphenoid sinus mucocele treatment. An adequate sphenoidotomy and drainage give excellent results.

Objectives To describe an extremely rare case of sporadic hemangioblastoma (HB) within the cavernous sinus and Meckel's cave with extension to the cerebellopontine angle (CPA) cistern. Methods A 73-year-old male presented with hearing loss, unilateral ptosis, and facial numbness. Results The imaging showed a complex cystic-solid mass centered at the left cavernous sinus and Meckel's cave with extension to the CPA cistern. Patient underwent retrosigmoid craniectomy for partial resection of the CPA angle component of the mass. Surgical pathology confirmed the diagnosis of HB and patient was scheduled for subsequent radiotherapy of the residual mass. Conclusions We present an exceptional case of supratentorial HB without associated von Hippel–Lindau (VHL) disease, which was predominantly located in the cavernous sinus and Meckel's cave and led to multiple cranial nerve symptoms. We describe imaging characteristics and radiologic–pathologic correlation of this atypically located HB, which can be difficult to consider in the differential diagnosis presurgically.

We present a case of a woman with a swollen upper eyelid in ptosis, conjunctival nodules, and chemosis present for a couple of weeks. She did not respond to therapy for hordeolum or allergy. A biopsy followed by histopathological examination showed a large infiltration of eosinophilic granulocytes and flame figures, so the diagnosis of Wells syndrome was made. Oral methylprednisolone in high dose was necessary, and tapering was slow because of frequent bouts of relapse. After 3 months, the swelling and hard feeling of the eyelid disappeared, but a slight ptosis remained. Eyelid swelling and eosinophilia recurred 18 months after resolution of the first episode.

Due to its rarity and the limited literature, the clinicopathological characteristics of peripheral nerve involvement in immunoglobulin G4 (IgG4)-related disease are unknown. We present two cases of IgG4-related disease, accompanied by peripheral neuropathy, presenting as unilateral ptosis (case 1) and sclerosing cholangitis (case 2), respectively. In both cases, sural nerve biopsy indicated vasculitis as the underlying pathophysiology; the peripheral neuropathy was refractory to corticosteroid therapy. In contrast to the previously proposed pathomechanism of IgG4-related neuropathy (direct lymphoplasmacytic infiltration), the pathological findings in our cases suggest that vasculitis occurs secondary to systemic autoimmune conditions.

Background Syphilis is a sexually transmittable infectious disease caused by the spirochaete Treponema pallidum, which classically causes symptoms in three stages. Case presentation A previously healthy male in his thirties was admitted to our department after suffering from diplopia and unilateral ptosis for two days. Clinical examination revealed a left-sided oculomotor palsy with an ipsilateral ptosis. Supplementary cerebral computer tomography with angiography and magnetic resonance imaging was normal except for lymphadenopathy in the neck and mediastinum. As the patient was homosexual, we chose to test for HIV. The doctor on duty, being from Belarus where syphilis was recognised as endemic during her studies, also screened for syphilis. The HIV test was negative, while the syphilis test was positive. Lumbar puncture showed moderate pleocytosis and positive serology for syphilis, confirming the diagnosis of neurosyphilis. The patient recovered fully after a course of penicillin. Interpretation Even though syphilis remains uncommon in Norway, the incidence has been increasing in recent decades, especially in male homosexual communities. Patients may subsequently present with a diversity of symptoms.

A 3-month-old child was referred because of jaundice and altered coagulation not corrected by vitamin K and plasma.

Vaccinations for shingles are recommended for most adults over 60 years of age and are typically well tolerated. The present case describes acute onset of unilateral ptosis, proptosis, and orbital myositis developing within days after administration of shingles vaccination. The episode resolved to baseline after 1 week of treatment with steroids. To the authors' knowledge, this is first reported case of orbital inflammation following shingles vaccination. Given the temporal relationship and rapid response to treatment, this may represent an autoimmune reaction to the shingles vaccine.

We describe herein a case with left-side ptosis induced by pure midbrain infarction in a 49-year-old woman. She also presented with diplopia and right-side cerebellar ataxia. MRI demonstrated new ischemic stroke of the left ventral paramedian midbrain. In this case, ischemia of the left oculomotor fascicles caused the left-side ptosis and diplopia, and ischemia of the left decussation of the superior cerebellar peduncle caused the right-side cerebellar ataxia. These symptoms resulted from inner superior medial mesencephalic branch infraction. This case offers an educational example that can be explained by models proposed in the past and requires knowledge of neuroanatomy and cerebrovasculature.

Introduction. Distal arthrogryposis type 5D (DA5D) is an autosomal recessive disease. The clinical symptoms include contractures of the joints of limbs, especially camptodactyly of the hands and/or feet, unilateral ptosis, a round-shaped face, arched eyebrows, and micrognathia, without ophthalmoplegia. ECEL1 is a DA5D causative gene that encodes a membrane-bound metalloprotease. ECEL1 plays important roles in the final axonal arborization of motor nerves in limb skeletal muscles and neuromuscular junction formation during prenatal development. Methods. A DA5D family with webbing of the elbows and fingers was recruited. We performed whole-exome sequencing (WES) and filtered mutations by disease-causing genes of arthrogryposis multiplex congenita (AMC). Mutational analysis and cosegregation confirmation were then performed. Results. We identified novel compound heterozygous mutations of ECEL1 (NM_004826: c.69C>A, p.C23∗ and c.1810G>A, p.G604R) in the proband. Conclusions. We detected causative mutations in a DA5D family, expanding the spectrum of known ECEL1 mutations and contributing to the clinical diagnosis of DA5D.

Purpose: To evaluate the results after frontalis sling surgery in congenital unilateral ptosis. Methods: This was a prospective study which included 27 eyes of patients with congenital unilateral ptosis and fair to poor levator action (

Purpose: To highlight the presentation and management of a patient with eyelid, orbital and nasopharyngeal silicone oil migration through a glaucoma drainage implant presenting as pseudo-xanthelasma and ptosis. Observations: A 68-year male presented with unilateral ptosis and presumed xanthelasma. He had a history of glaucoma drainage implant surgery, pseudophakia, and multiple retinal detachment repairs with silicone oil. During ptosis repair it was discovered that his presumed xanthelasma was in fact an eyelid silicone granuloma. Additional work up revealed silicone infiltration of the eyelids, orbits, and nasopharynx, resulting from emulsified silicone oil leakage through his glaucoma valve implant. Conclusions and Importance: Silicone oil may emulsify with time, with potential egress via a glaucoma filtration device. Clinicians should be alert for eyelid, orbital and sinonasal findings that may indicate occult migration. Keywords: Silicone oil, Xanthelasma, Orbit, Foreign body granuloma, Baerveldt, Glaucoma drainage implant

Horner syndrome was identified in 25 of 30 777 avian admissions to Currumbin Wildlife Hospital during 2010-2016. Unilateral ptosis and erection of facial feathers were distinct findings on physical examination and consistent across 9 species. Affected birds were biased toward adults (64%) suffering traumatic injuries (88%). Concurrent injuries requiring treatment were present in 38% of cases, and 76% had additional neurologic deficits. Prognosis for release was poor, with an overall success rate of 32%. Resolution of clinical signs increased to 44% with higher doses of meloxicam and required an average hospitalization of 22 days (range, 3-78 days). Further investigation of the underlying causes of Horner syndrome in birds to provide treatment and prognostic guidelines is warranted.

Background To present the outcomes of the tarsal switch procedure using an anterior approach to correct severe ptosis with poor levator muscle function ( Methods This retrospective case series included 11 patients with severe neurogenic or acquired myogenic palpebral ptosis. All patients underwent the tarsal switch procedure through an anterior approach from 2012 to 2015. Margin reflex distance (MRD1 and MRD2) and the palpebral fissure were evaluated preoperatively and postoperatively. Data were compared using the Wilcoxon signed-rank test. P-values Results Surgery was performed on 18 eyelids (11 patients). The median age at surgery was 57 years (range, 29-86 years). Four patients had unilateral ptosis and seven had bilateral ptosis. Nine patients had myogenic ptosis and two had neurogenic ptosis. Postoperatively, the chin-up position improved in all patients. The MRD1 increased statistically significantly, from 0 mm preoperatively to 1.0 mm postoperatively (P=0.001). The MRD2 decreased statistically significantly, from 4.5 mm preoperatively to 3.0 mm postoperatively (P=0.001). The palpebral fissure did not change (4.0 mm preoperatively to 4.0 mm postoperatively) (P=0.13). Conclusions The tarsal switch procedure through an anterior approach is an effective alternative for correcting severe ptosis, especially neurogenic or acquired myogenic ptosis. This procedure can be performed with minimal risk of ocular surface exposure and provides stable outcomes.

Unilateral ptosis may be seen in focal seizures and should be actively sought for during video-EEG (scalp and intracranial) evaluations. Further studies are needed to confirm its lateralizing value, but it was contralateral to seizure onset in this case.

Cornelia de Lange syndrome (CdLS) is a rare multisystemic disorder that is characterized by mental retardation, prenatal and postnatal growth retardation, limb anomalies, and distinctive facial features, which include arched eyebrows that often meet in the middle (synophrys), long eyelashes, low-set ears, small and widely spaced teeth, and a small and upturned nose. Ophthalmic manifestations include long eyelashes, nasolacrimal duct obstruction, myopia, ptosis, and strabismus. There has been no report of surgical treatment for esotropia and unilateral ptosis in patients with CdLS in Korea. I report a patient with CdLS who underwent surgical treatment for esotropia and unilateral ptosis with a good surgical outcome.

The Severe Acute Respiratory Syndrome Coronavirus-2 (SARS-CoV-2), the virus that causes coronavirus disease 2019 (COVID-19), has spread as a global pandemic with significant morbidity and mortality. As the prevalence of COVID-19 has risen, so has the diversity of its clinical presentation. SARS-CoV-2 is considered to have neuroinvasive and neurotropic qualities that can lead to central and peripheral nervous system manifestations. We describe a 65-year-old woman who developed new-onset unilateral ptosis and mitosis following a diagnosis of COVID-19. To our knowledge, this is the first reported case describing transient Horner syndrome in association with COVID-19.

The purpose of this study was to demonstrate accompanying clinical features besides lid droop in congenital ptosis and to determine the effect of upgaze on lower eyelid position in patients with congenital ptosis.Pediatric patients with congenital ptosis who visited our clinic over a 1-month period in 2014 were recruited for this study. Clinical photographs and measurements were taken prospectively in primary gaze and upgaze. Measurements taken from photographs and clinical records include inferior scleral show (ISS), vertical lower lid height (LL), and canthal tilt angle (CTA).There were 35 patients with congenital ptosis. There was more ISS in unilateral ptosis eyes than in the normal side in both primary gaze (p 0.05) and upgaze (p 0.01), with corresponding increase in ISS in upgaze (p 0.05). There was also more ISS in unilateral ptosis eyes than in bilateral ptosis eyes in primary gaze (p 0.05) and upgaze (p 0.01), with accompanying increase in ISS in upgaze (p 0.01). There was less elevation of the lower lid in unilateral ptosis eyes (0.5 ± 0.7 mm) than in the contralateral normal eyes (0.8 ± 0.7 mm) in upgaze, but this was not statistically significant (p = 0.07). CTA was lower in ptosis eyes than in normal eyes in primary gaze and upgaze (p 0.01 and p 0.05, respectively).ISS was larger in ptosis eyes and upgaze appears to increase ISS. Canthal tilt is lower in congenital ptosis eyes than in normal eyes in primary gaze and upgaze.