Lid-Related Keratopathy in Stevens-Johnson Syndrome: Natural Course and Impact of Therapeutic Interventions in Children and Adults.

PURPOSE: To compare the long-term visual outcomes of different management strategies in children and adults with Stevens-Johnson Syndrome (SJS)-induced chronic lid-related keratopathy. DESIGN: Retrospective comparative case series. METHODS: This study included 705 eyes of 401 patients (81 children and 320 adults) with SJS who presented with chronic lid-related keratopathy between 1990 and 2015. Affected eyes received either conservative therapy [topical medications (n = 363)] or definitive management (n = 342) that included mucous membrane grafting (MMG), prosthetic replacement of the ocular surface ecosystem (PROSE) contact lenses, or both. The primary outcome measure was change in best corrected visual acuity (BCVA) over time. The secondary outcome measure was the odds of developing corneal ulceration or perforation in the first year. RESULTS: The treatment subgroups were comparable at baseline in terms of BCVA and previous management (P > .10). Over 10 years, children and adults who received conservative therapy lost at least 5 lines of median BCVA and carried a 3 times higher risk of developing corneal ulceration in the first year. Conversely, definitive therapy provided significant benefit by improving median BCVA (P < .0001). In children, MMG was more effective than PROSE (P = .009), whereas PROSE was more effective than MMG in adults (P = .028). However, the combination of MMG followed by PROSE provided the best results in both children and adults (P < .036). CONCLUSIONS: Both MMG and PROSE changed the natural course and helped in preserving and improving vision in eyes with SJS-induced lid-related keratopathy. Regardless of age, those who received both MMG and PROSE had the best long-term visual outcomes.

Chronic Ocular Sequelae of Stevens-Johnson Syndrome in Children: Long-term Impact of Appropriate Therapy on Natural History of Disease.

PURPOSE: To describe the long-term ocular and visual morbidity in children with chronic sequelae of Stevens-Johnson syndrome (SJS) and visual outcomes of various management strategies. DESIGN: Retrospective comparative case series. METHODS: This study included 568 eyes of 284 children with SJS who presented between 1990 and 2015. Affected eyes received either conservative therapy (n = 440) or definitive management (n = 128), including lid margin mucous membrane grafting (MMG), prosthetic replacement of the ocular surface ecosystem (PROSE) contact lenses, allogeneic limbal transplantation, or keratoprosthesis using an algorithmic approach based on the severity of dryness and cause and extent of corneal damage. The primary outcome measure was best-corrected visual acuity (BCVA). RESULTS: Two thirds of patients presented more than a year after acute SJS, 99% without prior amniotic membrane grafting, with low vision or blindness in 60% of eyes. Children 8 years or younger in age had significantly worse ocular and visual morbidity (P ≤ .037). At 5 years of follow-up, definitive therapy significantly altered the natural history of the disease by improving BCVA and preventing the development or progression of keratopathy, as compared to conservative therapy (P ≤ .002). In eyes with lid-related keratopathy, MMG was significantly more effective than PROSE, although both were significantly better than conservative therapy and the combination of MMG followed by PROSE provided the best results (P < .0001). CONCLUSION: Children receiving suboptimal care during acute SJS presented later with severe ocular and visual morbidity. Timely therapy, particularly with PROSE and MMG in eyes with lid-related keratopathy, changed the natural course and helped in preserving and improving vision.

Pharmacological therapy for amblyopia.

Amblyopia is the most common cause of preventable blindness in children and young adults. Most of the amblyopic visual loss is reversible if detected and treated at appropriate time. It affects 1.0 to 5.0% of the general population. Various treatment modalities have been tried like refractive correction, patching (both full time and part time), penalization and pharmacological therapy. Refractive correction alone improves visual acuity in one third of patients with anisometropic amblyopia. Various drugs have also been tried of which carbidopa & levodopa have been popular. Most of these agents are still in experimental stage, though levodopa-carbidopa combination therapy has been widely studied in human amblyopes with good outcomes. Levodopa therapy may be considered in cases with residual amblyopia, although occlusion therapy remains the initial treatment choice. Regression of effect after stoppage of therapy remains a concern. Further studies are therefore needed to evaluate the full efficacy and side effect profile of these agents.

Congenital cranial dysinnervation disorders.

The European Neuromuscular Centre (ENMC) derived the term Congenital Cranial Dysinnervation Disorders in 2002 at an international workshop for a group of congenital neuromuscular diseases. CCDDs are congenital, non-progressive ophthalmoplegia with restriction of globe movement in one or more fields of gaze. This group of sporadic and familial strabismus syndromes was initially referred to as the 'congenital fibrosis syndromes' because it was assumed that the primary pathologic process starts in the muscles of eye motility. Over the last few decades, evidence has accumulated to support that the primary pathologic process of these disorders is neuropathic rather than myopathic. This is believed that for normal development of extra ocular muscles and for preservation of muscle fiber anatomy, normal intra-uterine development of the innervation to these muscles is essential. Congenital dysinnervation to these EOMs can lead to abnormal muscle structure depending upon the stage and the extent of such innervational defects. Over last few years new genes responsible for CCDD have been identified, permitting a better understanding of associated phenotypes, which can further lead to better classification of these disorders. Introduction of high-resolution MRI has led to detailed study of cranial nerves courses and muscles supplied by them. Thus, due to better understanding of pathophysiology and genetics of CCDDs, various treatment modalities can be developed to ensure good ocular alignment and better quality of life for patients suffering from the same.

Impact of Superior Oblique Transposition on Primary Position Deviation, a Pattern and Intorsion in Third Nerve Palsy.

PURPOSE: To evaluate the role of superior oblique transposition on primary position alignment, A pattern, and intorsion in third nerve palsy. METHODS: Ten patients with isolated, unilateral third nerve palsy were included in this prospective study. The patients were treated by conventional surgery on horizontal recti together with superior oblique transposition by Scott's procedure in the paretic eye. RESULTS: Pre-operative primary horizontal deviation was 60-80 PD (mean 70.00±7.45 PD). Pre-operative primary hypotropia was 15-22 PD (mean 18.80±2.48 PD). Mean A-pattern was 17.80±2.65 PD. All patients included in the study had some degree of objective torsion as measured by Guyton's method. One patient had grade I objective intorsion, 2 had grade II, 6 had grade III, and 1 had grade IV objective intorsion. Primary position horizontal alignment (up to ±8 PD) was achieved in 9 patients. Primary position vertical alignment (up to ±8 PD) was achieved in all 10 patients. Only 2 of 10 patients had A-pattern of 10 PD, in the remaining 8 patients it was eliminated (P<0.05). Postoperatively, 5 patients had no objective intorsion and 5 had grade I intorsion, and none of the patients had hypertropia or paradoxical eye movements. CONCLUSION: Superior oblique transposition by Scott's procedure along with conventional surgery on horizontal recti in third nerve palsy results in better horizontal and vertical alignment, and improves A-pattern and intorsion, thus leading to better binocular interaction.

Surgical management of third nerve palsy.

Third nerve paralysis has been known to be associated with a wide spectrum of presentation and other associated factors such as the presence of ptosis, pupillary involvement, amblyopia, aberrant regeneration, poor bell's phenomenon, superior oblique (SO) overaction, and lateral rectus (LR) contracture. Correction of strabismus due to third nerve palsy can be complex as four out of the six extraocular muscles are involved and therefore should be approached differently. Third nerve palsy can be congenital or acquired. The common causes of isolated third nerve palsy in children are congenital (43%), trauma (20%), inflammation (13%), aneurysm (7%), and ophthalmoplegic migraine. Whereas, in adult population, common etiologies are vasculopathic disorders (diabetes mellitus, hypertension), aneurysm, and trauma. Treatment can be both nonsurgical and surgical. As nonsurgical modalities are not of much help, surgery remains the main-stay of treatment. Surgical strategies are different for complete and partial third nerve palsy. Surgery for complete third nerve palsy may involve supra-maximal recession - resection of the recti. This may be combined with SO transposition and augmented by surgery on the other eye. For partial third nerve, palsy surgery is determined according to nature and extent of involvement of extraocular muscles.