Tessier Number 9 Craniofacial Cleft Associated with Goldenhar Syndrome and Its Surgical Management: A Report of a Rare Case.

Purpose: To report surgical repair of a rare case of Tessier number 9 craniofacial cleft. Methods: Case report. Results: Tessier number 9 craniofacial cleft is the rarest cleft anomaly. This article reports a congenital eyelid coloboma in a 21-year-old woman that involved the lateral third of the left upper eyelid and extended to the lateral canthus, consistent with number 9 craniofacial cleft Tessier classification. The additional findings included a fibrotic band between the globe and the remnant of the upper lid, which caused a small-angle exotropia. There were also skin appendages in the preauricular area and the inner surface of the nasal columella consistent with Goldenhar syndrome. The eyelid coloboma was repaired by releasing the adhesions and using a composite graft of the hard palate to repair the posterior lamella. The anterior lamella was repaired by creating a skin advancement flap. The esthetic and functional outcomes were acceptable in the 2-year postoperative follow-up period. Conclusion: The composite hard palate graft can be used to repair posterior lamella defect in the case of Tessier number 9 craniofacial cleft.

Neuro-ophthalmic Manifestations of Coronavirus Disease 2019 and Its Vaccination: A Narrative Review.

Coronavirus disease 2019 (COVID-19) is a current pandemic caused by SARS-CoV-2 that has vastly affected the whole world. Although respiratory disease is the most common manifestation of COVID-19, the virus can affect multiple organs. Neurotropic aspects of the virus are increasingly unfolding, in so far as some respiratory failures are attributed to brainstem involvement. The neuro-ophthalmic manifestations of COVID-19 and the neuro-ophthalmic side effects of vaccination were reviewed. The major findings are that the SARS-CoV-2 infection commonly causes headaches and ocular pain. It can affect the afferent and efferent visual pathways by ischemic or inflammatory mechanisms. Optic nerve may be the origin of transient or permanent visual loss from papillophlebitis, idiopathic intracranial hypertension, or optic neuritis. Cerebrovascular strokes are not uncommon and may lead to cortical visual impairment or optic nerve infarction. SARS-CoV-2 may affect the pupillomotor pathways, resulting in tonic pupil (Adie's syndrome) or Horner's syndrome. Cranial neuropathies including third, fourth, sixth, and seventh nerve palsies have all been reported. Rhino-orbital mucormycosis superinfections in COVID-19 patients receiving steroids or other immunosuppressive therapies may result in unilateral or bilateral visual loss and ophthalmoplegia. Autoimmune conditions such as Guillain-Barré, Miller-Fisher syndrome, and ocular myasthenia have been reported.

Augmented fascia temporalis sling for paralytic ectropion of the lower lid.

PURPOSE: To report the effect of lower eyelid temporalis fascia sling combined with lateral canthoplasty and tarsorrhaphy for paralytic ectropion. METHODS: Prospective case series of 10 patients with lower lid paralytic ectropion who were treated with lower eyelid fascia temporalis sling and lateral canthoplasty in addition to lateral tarsorrhaphy as a single-session procedure. Additional medial tarsorrhaphy was applied if the medial lower lid apposition was not adequate at the end of the procedures. Eyelid configuration and function were compared before and after surgery. RESULTS: The mean age of patients was 65.8 ± 10 years. Mean marginal reflex distance 1 (MRD1) and MRD2 changed from 3.5 ± 1.4 and 8.6 ± 2.4 mm to 2.2 ± 1.4 and 5.3 ± 1.2 mm respectively (p = 0.001 and 0.006). Mean pre-operative lagophthalmos improved from 9.2 ± 4.9 to 3.4 ± 1.3 mm (p = 0.001). The mean follow-up was 28.9 ± 12.1 months. Three patients required additional medial tarsorrhaphy to address residual medial ectropion in the same session. CONCLUSION: Combination of lower lid fascia temporalis sling, lateral canthoplasty and tarsorrhaphy as a single-session procedure can effectively improve the functional and aesthetic complications of paralytic ectropion.

Orbital cellulitis secondary to giant sino-orbital osteoma: A case report.

BACKGROUND: Although osteoma is a common benign tumor of the paranasal sinuses, its orbital extension is not common. Secondary orbital cellulitis has rarely been reported in association with sino-orbital osteoma. CASE: A 30-year-old woman presented with left side proptosis, orbital pain and inflammation. Orbital CT scan showed a well-defined giant osteoma in the superonasal part of the left orbit originating from the left ethmoidal sinus associated with opacity of the ipsilateral ethmoidal sinus and infiltration of orbital soft tissue. After treatment by systemic antibiotics, osteoma was resected with combined external and endoscopic surgery and the patient recovered uneventfully. CONCLUSION: Sino-orbital osteoma may manifest primarily as orbital cellulitis and needs early surgical intervention.

Choroidal Thickness in Acute Non-arteritic Anterior Ischemic Optic Neuropathy.

PURPOSE: To compare the choroidal thickness in eyes with acute non-arteritic anterior ischemic optic neuropathy (NAION) with healthy contralateral eye and normal controls. METHODS: Thirty-eight eyes with NAION, thirty-eight unaffected fellow eyes, and seventy-four eyes from 37 healthy, age- and sex-matched subjects were included in this prospective comparative case-control study. Choroidal thickness was measured by enhanced depth imaging (EDI) of spectral domain optical coherence tomography (SD-OCT). Peripapillary choroidal thickness (PCT) was measured at 1000 and 1500 µ m from Bruch's membrane opening (BMO). Subfoveal choroidal thickness (SFCT) was measured in central subfoveal area, and 500 microns apart in temporal and nasal sides. Choroidal thickness among NAION eyes, uninvolved fellow eyes, and control eyes were compared. RESULTS: The mean of PCT at 1000 µ m was significantly thicker in NAION and fellow eyes compared to control eyes (169.7 ± 47, 154.4 ± 42.1, and 127.7 ± 49.9 µ m, respectively, P < 0.001 and P = 0.42). The mean PCT at 1500 µ m was also significantly thicker in NAION and fellow eyes compared to control eyes (178.6 ± 52.8, 162.6 ± 46.1, and 135.1 ± 59 µ m, respectively, P = 0.007 and P = 0.048). The mean PCT at 1000 and 1500 µ m was significantly greater in NAION compared to fellow eyes (P = 0.027 and P = 0.035, respectively). The mean of SFCT was significantly thicker in NAION compared to control eyes (P = 0.032); however, there was no significant difference between uninvolved fellow and control eyes (P = 0.248). CONCLUSION: Thicker choroidal thickness in acute NAION and uninvolved fellow eyes compared to normal eyes suggests a primary choroidal role in NAION pathophysiology.

Slanted versus Augmented Recession for Horizontal Strabismus.

PURPOSE: To compare the surgical outcomes of slanted versus augmented recession in patients with horizontal strabismus. METHODS: In this randomized clinical trial, a total of 100 esotropic (ET) and exotropic (XT) patients with a high AC/A ratio which was defined as a difference of ≥ 10 prism diopters (pd) between the distance and near deviations were included if the patients had a distance deviation ≥ 15 pd. Patients were randomly assigned into the slanted (n = 26 in ET and n = 24 in XT group) and augmented recession groups (n = 25 in ET and n = 25 in XT group). In the slanted group, recession was performed on the superior and inferior poles of the muscle based on the distance and near deviations, respectively, while in the augmented recession group, the muscles were recessed 1.00 or 1.50 mm more than the standard amount according to the distance and near difference between 10 and 20 pd or > 20 pd, respectively. RESULTS: The mean age was 9.8 ± 9.6 years and 63% were female. There was a significant postoperative reduction of difference in convergence excess in ET cases compared to patients who underwent the augmented recession procedure (12.65 ± 6.16 vs 8.64 ± 6.1 pd, P = 0.014). Among our XT groups, there was no significant difference in postoperative reduction in the XT angle in the slanted group compared with the augmented group (P > 0.05). CONCLUSION: Slanted recession is recommended in convergence excess ET patients. In XT patients, either slanted or augmented recession may be chosen according to the priority and experience of the surgeon.

Glaucoma in Ectropion Uveae Syndrome: A Case Report and Literature Review.

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.

Lateral Rectus-Medial Rectus Union: A New Surgical Technique for Treatment of Complete Third Nerve Palsy.

PURPOSE: To describe a new surgical technique for the treatment of complete third nerve palsy. METHODS: This study included patients with chronic complete third nerve palsy. The lateral rectus muscle was split, followed by disinsertion of the superior and inferior halves, which were passed between the sclera and superior and inferior rectus muscles, respectively. Then the medial rectus muscle was sutured as posteriorly as possible from its insertion and cut. Next, the distal stump of the medial rectus muscle was split into two halves and united with the superior and inferior halves of the lateral rectus muscle. Finally, the proximal portion of the medial rectus muscle was sutured back to its original insertion. In cases with hypotropia and a functional superior oblique muscle, superior oblique tenectomy was also performed. Success was defined as postoperative horizontal deviation of 10 prism diopters (PD) or less and vertical deviation of 5 PD or less. RESULTS: Ten patients with a mean age of 32.4 ± 18.4 years had surgery using this technique; 2 of them had a history of strabismus surgery. Mean exotropia was 84 ± 14.9 PD, which reduced to 6.5 ± 8.2 PD. Mean vertical deviation was 16.5 ± 10 PD, which reduced to 2.5 ± 3.5 PD. Mean follow-up was 13.2 ± 7.9 months, and the success rate was 70% and 90% for horizontal and vertical deviations, respectively. CONCLUSIONS: The lateral rectus-medial rectus union technique is an effective and long-lasting procedure to overcome large-angle exotropia associated with complete third nerve palsy. This method can also be employed as a reoperation procedure. [J Pediatr Ophthalmol Strabismus. 2019;56(1):10-18.].

Acute Transient Sixth Nerve Palsy in Chediak-Higashi Syndrome.

Neurologic disorders in Chediak-Higashi syndrome are usually late presentations and also may manifest long after bone marrow stem cell transplantation. To the authors' knowledge, transient neurological deficit has not been reported yet. They describe a 6-year-old boy with Chediak-Higashi syndrome in the accelerated phase who presented with transient sixth nerve palsy. [J Pediatr Ophthalmol Strabismus. 2018;55:e22-e25].

Effect of Cycloplegia on Corneal Biometrics and Refractive State.

PURPOSE: To determine changes in refractive state and corneal parameters after cycloplegia with cyclopentolate hydrochloride 1% using a dual Scheimpflug imaging system. METHODS: In this prospective cross-sectional study patients aged 10 to 40 years who were referred for optometric evaluation enrolled and underwent autorefraction and corneal imaging with the Galilei dual Scheimpflug system before and 30 minutes after twice instillation of medication. Changes in refraction and astigmatism were investigated. Corneal biometrics including anterior and posterior corneal curvatures, total corneal power and corneal pachymetry were compared before and after cycloplegia. RESULTS: Two hundred and twelve eyes of 106 subjects with mean age of 28 ± 5 years including 201 myopic and 11 hyperopic eyes were evaluated. Mean spherical equivalent refractive error before cycloplegia was -3.4 ± 2.6 D. A mean hyperopic shift of 0.4 ± 0.5 D occurred after cycloplegia (P < 0.001). The astigmatism power did not significantly change (P = 0.8), however, 26.8% of eyes with significant astigmatism experienced a change of more than 5 degrees in the axis of astigmatism. Changes in posterior corneal curvature were scant but statistically significant (P = 0.001). Moreover, corneal thickness was slightly increased in the central and paracentral regions (P < 0.001 and P < 0.001, respectively). CONCLUSION: Cycloplegia causes a hyperopic shift and astigmatism axis changes, along with an increase in central and paracentral corneal thickness and change in posterior corneal curvature. The effects of cycloplegia on refraction and corneal biometrics should be considered before cataract and refractive surgeries.

The relationship between abduction deficit and reoperation among patients with infantile esotropia.

AIM: To determine the relationship between abduction deficit and reoperation among patients with infantile esotropia (IET). METHODS: The records of 216 patients (432 eyes) with IET who underwent surgery, from 2010 to 2015 were studied. Patients with IET whose deviation appeared before 6mo of age and had stable preoperative deviation in two examinations with at least 2wk apart and a minimum 3mo postoperative follow up were included. Cases with early onset accommodative esotropia, congenital cataract, retinopathy of prematurity (ROP), manifest nystagmus, fundus lesions, neurologic and ophthalmic anomalies, 6th nerve palsy and Duane's syndrome were excluded. Preoperative abduction deficit was considered from -1 to -3 grading scale. Three months after surgery, children were classified into no-need reoperation [deviation≤15 prism diopters (PD)], and need-reoperation groups (deviation>15 PD). RESULTS: In this retrospective study, 117 female and 99 male patients with the mean surgical age of 4.7±6.4y were included. Reoperation rate was 33.3% and 16.0% in IET patients with and without abduction deficit, respectively in patients who had a history of late surgery. Abduction deficit increased the odds of reoperation by 82% [OR=1.82, 95% confidence interval (CI) =1.05 to 3.19, P=0.003] in patients who had a history of late surgery (>2 years old, P=0.021). Abduction deficit was improved significantly after operation (P<0.001). CONCLUSION: Based on our results, abduction deficit can be considered as a risk factor of reoperation in IET patients who are operated at the age of more than 2y.

Graded versus ungraded inferior oblique anterior transposition in patients with asymmetric dissociated vertical deviation.

PURPOSE: To report the surgical outcomes of graded versus ungraded inferior oblique anterior transposition (IOAT) in treatment of patients with asymmetric dissociated vertical deviation (DVD) and bilateral inferior oblique overaction (IOOA). METHODS: A total of 74 eyes of 37 patients with asymmetric DVD (interocular difference of ≥5Δ) and bilateral IOOA of > +1 were included in this randomized clinical trial. In the ungraded group (n = 18), both inferior oblique muscles were sutured at the inferior rectus level; in the graded group (n = 19), the inferior oblique muscles of eyes with more DVD were sutured at the level of the inferior rectus and inferior oblique muscles of eyes with less DVD were sutured 2 mm posterior to the level of the inferior rectus muscle. RESULTS: DVD was significantly reduced in each group (P < 0.001 for both). Although the postoperative mean difference of asymmetry of DVD was less in the ungraded group compared to the graded group (1.2 ± 1.9 vs 3.2 ± 1.2 [P = 0.001]), the absolute amounts of reduction of DVD asymmetry were similar (4.3 ± 2.3 vs 4.4 ± 3.1 [P = 0.78]). IOOA and V patterns were also reduced postoperatively. CONCLUSIONS: Each method of IOAT was effective in reducing DVD, asymmetry, IOOA, and V patterns.

Inferior Oblique Overaction: Anterior Transposition Versus Myectomy.

PURPOSE: To compare the efficacy of inferior oblique myectomy and anterior transposition for correcting inferior oblique overaction (IOOA). METHODS: This retrospective study was conducted on 56 patients with IOOA who had either myectomy or anterior transposition of the inferior oblique muscle from 2010 to 2015. The authors compared preoperative and postoperative inferior oblique muscle function grading (-4 to +4) as the main outcome measure and vertical and horizontal deviation, dissociated vertical deviation (DVD), and A- and V-pattern between the two surgical groups as secondary outcomes. RESULTS: A total of 99 eyes of 56 patients with a mean age of 5.9 ± 6.5 years were included (47 eyes in the myectomy group and 52 eyes in the anterior transposition group). There were no differences in preoperative best corrected visual acuity, amblyopia, spherical equivalent, and primary versus secondary IOOA between the two groups. Both surgical procedures were effective in reducing IOOA and satisfactory results were similar between the two groups: 61.7% and 67.3% in the myectomy and anterior transposition groups, respectively (P = .56). After adjustment for the preoperative DVD, there was no statistically significant difference between the two groups postoperatively. The preoperative hypertropia was 6 to 14 and 6 to 18 prism diopters (PD) in the myectomy and anterior transposition groups, respectively. After surgery, no patient had a vertical deviation greater than 5 PD. CONCLUSIONS: Both the inferior oblique myectomy and anterior transposition procedures are effective in reducing IOOA with similar satisfactory results. DVD and hypertropia were also corrected similarly by these two surgical procedures. [J Pediatr Ophthalmol Strabismus. 2017;54(4):232-237.].

Cluster Headache Associated with Secondary Unilateral Blepharospasm: A Case Report and Review of the Literature.

PURPOSE: Cluster headache is one of the most serious types of headache that is accompanied by autonomic parasympathetic symptoms. Its association with hemifacial spasm in the same side had been rarely reported. The aim of this report is describing a case with this association and treatment strategies. CASE REPORT: Here we report a 37-year-old female with cluster headache associated with secondary unilateral blepharospasm that was successfully treated with combination therapy including botulinum toxin injection. CONCLUSION: Hemifacial spasm associated with cluster headache needs special attention and can be treated successfully.

Surgical results of consecutive exotropia.

PURPOSE: To determine the success rates of different surgical procedures and the risk factors of surgical failure among patients with consecutive exotropia. METHODS: Forty patients with exotropia were observed at least 6 weeks after their esotropia surgery. Surgical planning was based on the medial rectus muscle function. Lateral rectus weakening in patients with normal medial rectus function, medial rectus strengthening for patients with limited medial rectus function (-1 to -3), and combined procedure occurred when the correction of each type of surgery was less than the amount of deviation. Postoperatively, patients were divided into success (8 prism diopters [PD] or less) or failure (8 PD or greater) groups. The follow-up period was at least 3 months. RESULTS: The mean preoperative exotropia was 29 ± 13 PD, which was reduced to 7 ± 7 PD postoperatively (P < .001). Successful results were achieved in 31 patients (77.5%), 17 (81%) in lateral rectus weakening, 10 (83%) in medial rectus strengthening, and 4 (57%) in the combined procedure group. More preoperative exodeviation was observed in the failure group compared to the success group (P = .015).The mean dose response was 2.27 ± 0.92 PD/mm in the lateral rectus weakening, 4.25 ± 2.27 PD/mm in the medial rectus strengthening, and 2.31 ± 0.66 PD/mm in the combined procedure groups. CONCLUSION: If the choice of surgical planning is based on medial rectus function and the amount of exodeviation, satisfactory alignment would be achieved in the majority of patients with consecutive exotropia. The preoperative amount of exodeviation was the only risk factor of surgical failure in the study.

Sublingual versus vaginal misoprostol for the management of missed abortion.

AIM: To evaluate the efficacy of two routes of misoprostol administration (sublingual and vaginal) for the treatment of missed abortion. METHODS: Two hundred and twenty women with confirmed missed abortion who received 400 microg/6 h misoprostol either sublingually or vaginally, were included in this randomized control trial. All women were admitted to hospital for follow-up care for 2 days. If the pregnancy was not completely evacuated during this time, the patient underwent immediate surgical completion. Efficacy was defined as the percentage of women discharged from the study without the need for surgical intervention. RESULTS: The effectiveness was high in the sublingual group and statistically different (sublingual 84.5%, vaginal 46.4% P = 0.000 RR = 0.54 95%CI = 0.442-0.681). The groups differed in terms of complications like bleeding (88.2% vs 65.5%), pain (85.5% vs 56.4%), diarrhea (69.1% vs 36.4%) and fever (23.6% vs 13.3%) in the sublingual group versus the vaginal group, but the mean time to expulsion was shorter (9.68 h SD = 5.51 95%CI = 8.61-10.57) in the sublingual group than the vaginal group (16.64 h SD = 14.01 95%CI = 13.8-19.48), P = 0.000. Women in the sublingual group were highly satisfied with the method. CONCLUSION: Sublingual misoprostol for the medical management of missed abortion is more effective and more acceptable than the vaginal route. However, it showed more adverse effects.