A Simple Step-by-Step Guide to Permanent Punctal Occlusion with the Ophthalmic Burr and Suture Technique.

The medical treatment of dry eye disease usually follows a step-wise approach to achieve clinical improvement, ranging from non-surgical interventions with intensive lubrication to permanent surgical punctal occlusion. While frequent lubrication is essential, the intense regime is often too burdensome and difficult to maintain at the required frequency. Punctal plugs are an invaluable alternative approach, but also have limitations, especially in conscious children, in whom inserting and re-inserting punctum plugs in clinic can be challenging. If a patient has permanent and severe dry eye disease and responded well to a trial of temporary punctal plugging, a permanent surgical solution should be considered next. Liu et al showed that a more successful, yet simple technique to achieve permanent occlusion is to combine de-epithelialising the punctum and ampulla with the immediate firm apposition of the de-epithelialised surface using a self-absorbable suture - with a success rate of 92% in a prospective study. This article demonstrates this technique step-by-step in an 8-year-old child with severe chronic dry eye disease following proton beam therapy for orbital rhabdomyosarcoma. She underwent this procedure with excellent results.

Successful Late Treatment of Orbital Infantile Haemangiomas in Two Teenagers with Topical Transcutaneous Timolol Maleate 0.5% Alone.

We previously described the first successful treatment of deep periocular and, subsequently, orbital infantile haemangiomas (IH) with topical transcutaneous timolol maleate 0.5% (TM 0.5%) alone as the first-line treatment in the acute proliferative phase in infancy. It is not known whether orbital IH with persistent proptosis in later years, untreated in infancy, would still respond to TM 0.5% treatment as well. To our best knowledge, we here present the first reports of the successful late treatment of persistent orbital IH with topical timolol maleate 0.5% applied to the skin overlying the orbital IH in two teenagers. Case 1 was an 11-year-old girl with proptosis measuring 4 mm before and 7 mm after Valsalva manoeuvre that diminished to 1 mm after topical treatment. Case 2 was a 10-year-old girl with 2 mm (before) and 4 mm proptosis (after Valsalva) that decreased to 0 mm proptosis with topical treatment. The time to resolution was 19 months (case 1) and 13 months (case 2). The treatment dose was 3 drops of TM 0.5% applied an average of 1.94 (case 1) and two times daily (case 2). In comparison to proptosis, resolution of periorbital swelling required less treatment time. There was no recurrence of either proptosis or periorbital swelling 1 year after stopping treatment in either case. The findings indicate that beta-blockers are effective not just in the proliferative but also in the late involutional phase in older children. No adverse clinical effects were noted throughout treatment in either case. The presence of increased proptosis with a Valsalva manoeuvre predicts a significant persistent vascular compartment in the orbital IH and may thus serve as a simple predictive clinical sign of likely treatment success. We recommend that a trial with this well-tolerated treatment form is considered in persistent cases of orbital IH, especially in the presence of enhanced proptosis with a Valsalva manoeuvre.

Ectopic vortex veins and varices in Donnai Barrow syndrome.

BACKGROUND: Donnai Barrow Syndrome (DBS) is a rare, multi-system autosomal recessively inherited disorder of relevance to ophthalmologists. To aim to describe the ocular phenotype using multimodal imaging for two cases of genetically confirmed DBS and compare against the published phenotype. MATERIALS AND METHODS: Retrospective case series of two unrelated patients with DBS and review of the literature. Both cases were referred to our tertiary unit for laser prophylaxis against retinal detachment. RESULTS: There was extreme high myopia greater than 20 dioptres without rhegmatogenous retinal detachment (RRD). Anterior segment features included iris transillumination and ciliary body hypoplasia. Posterior segment changes included previously described changes of optic nerve hypoplasia and a strikingly abnormal appearance of the fundus consisting of multiple bilateral giant posterior vortex veins (PVV). The mouse model shows a similar phenotype. CONCLUSIONS: Ectopic vortex veins of the choroid expand the phenotype of DBS and can be helpful in distinguishing the differential diagnosis of high myopia in children. Posterior vortex veins have been described in adult high myopia as acquired but our cases suggest that they could be congenital. Orbital manipulation and hypotony during surgery should be avoided to minimise complications. The evidence to recommend prophylactic laser retinopexy in these cases is inconclusive, and overall we recommend that conservative management should be considered using wide-angle retinal imaging in the clinic.

Adapted Bag-in-the-Lens Implantation Technique in Children with Congenital Ectopia Lentis.

BACKGROUND: Subluxation of the crystalline lens in childhood confronts the surgeon with a dilemma: to operate or to wait and see. Surgery is usually not performed when the subluxation is still limited. However, postponing the surgery increases the surgical difficulty as the capsular bag becomes more difficult to use as a means of support for the intraocular lens (IOL). A large number of children already present a pronounced subluxation at first presentation. In this paper, we describe a technique to optimise centration and fixation of the bag-in-the-lens (BIL) IOL in children younger than 7 years of age with congenital ectopia lentis. METHODS: Between October 2019 and December 2020, we performed lens extraction using a combination of bean-shaped segments to support the BIL IOL and a 6 - 0 polypropylene loop fixated at the sclera, following the Yamane technique, for the purpose of centration. We used this technique for seven eyes of four patients. The patients were between 2 and 6 years old; 3 boys and 1 girl. A definite diagnosis of Marfan syndrome was made for two children; for the other two, there was no proven underlying pathology. The luxation was upwards in all cases. The degree of luxation was severe in all eyes. The preoperative refraction values showed high astigmatism values for all eyes, ranging from 6.5 to 11.25 dioptres. Three out of the four patients were myopic, ranging from - 1.5 to - 9 dioptres. RESULTS: The surgery could be performed without major complications in all eyes. Good centration was obtained, which remained stable in the postoperative period. Refraction improved with greatly diminished degrees of astigmatism (ranging from 0.25 to 3 dioptres) and myopia (spheres ranging from - 2 to + 1.75 dioptres). CONCLUSION: Our novel technique incorporated the BIL technique with the addition of bean-shaped segments and a polypropylene 6/0 suture fixated at the sclera. In this way, we were able to obtain good centration and stability of the implanted IOL, as well as a good refractive outcome in all cases.

Topical Timolol for Infantile Haemangioma of the Orbit.

Infantile haemangiomas (IHs) are the most common benign tumours of the eyelid and orbits in infancy. Beta-blockers, in the form of oral propranolol, have become first-line treatment in severe cases with functionally significant or disfiguring IH. However, adverse drug reactions of oral propranolol in infants are reported in 1 in 11 and serious or potentially life-threatening systemic side effects in 1 in 38, including dyspnoea, hypotension, hyperkalaemia, hypoglycaemia, and cyanosis, therefore requiring careful and close monitoring during the course of systemic treatment. More recently, two large meta-analyses have shown topical beta-blockers, such as timolol maleate 0.5%, to be as effective as oral propranolol in superficial IH, but with no or significantly fewer adverse effects, and have advocated that topical beta-blockers replace oral propranolol as the first-line treatment of superficial IH. We have previously reported the therapeutic response of deep periocular IH to primary topical timolol maleate 0.5% monotherapy. Here we also describe the first successful treatments of large orbital IHs with primary topical timolol maleate 0.5% monotherapy in four infants, resulting in immediate cessation of progression and rapid clinical improvement or resolution in all cases. No adverse effects and no recurrence during long-term follow-up of up to 2.5 years after cessation were seen in any of the patients treated with topical timolol maleate 0.5%.

Safe and Effective Treatment of Intracranial Infantile Hemangiomas with Beta-Blockers.

Infantile hemangiomas are common benign vascular tumors but are rarely found in an intracranial location. Our literature review identified 41 reported cases. There is no general consensus on management of these rare lesions and until recently, treatment was limited to surgery or pharmacological management with steroids or interferon. Although beta-blockers have been widely prescribed in the treatment of cutaneous infantile hemangiomas since 2008, their use in the treatment of intracranial infantile hemangiomas has been minimal. We present a case of infantile hemangioma affecting the right orbit, associated with intracranial extension, causing intermittent right facial nerve palsy. The patient achieved an excellent outcome following combined treatment with oral propranolol and topical timolol maleate 0.5%, with complete regression of the lesion by 4 months. We conclude that beta-blockers are a safe and effective treatment of intracranial infantile hemangiomas and can be employed as first-line management of these lesions.

Central Retinal Artery Occlusion Following Prone Transcranial Surgery for Craniosynostosis and Discussion of Risk Factors.

Vision loss is a known rare complication of prone positioning during surgery. Vision loss following prone surgery is most commonly attributed to direct pressure on the eye but can also be caused by central retinal artery occlusion (CRAO) in the absence of pressure on the eye. Central retinal artery occlusion has not been previously described following prone transcranial surgery for craniosynostosis. We present two cases of monocular CRAO following prone calvarial expansion. A multidisciplinary root cause analysis suggested that raised intracranial pressure and intraoperative tranexamic acid may have been risk factors for the development of CRAO in these cases as no conventional risk factors for CRAO following prone surgery were present. Because of this, we retrospectively reviewed all prone transcranial procedures performed at the Oxford Craniofacial Unit for the presence of raised intracranial pressure and intraoperative tranexamic acid use. A total of 662 prone procedures have been performed between 1994 and March, 2019. Tranexamic acid has been used routinely in all transcranial procedures since 2012 and in the last 311 consecutive prone cases. Fifty-one (7.7%) prone procedures were performed for raised intracranial pressure, and tranexamic acid was used in the 33 most recent of these. Since the implementation of standard intraoperative administration of tranexamic acid there have been 2 cases of CRAO following prone surgery. The overall incidence of CRAO was 0.3% but was 6% in the context of raised intracranial pressure and tranexamic acid use. Prone positioning raised intracranial pressure and tranexamic acid use together may represent a potent combination of risk factors for CRAO.

Severe congenital bilateral corneal ulceration due to Wolf-Hirschhorn syndrome: a case-report and review of the ophthalmic literature.

A newborn boy with genetically confirmed Wolf-Hirschhorn syndrome presented with severe bilateral corneal ulceration that required emergency surgical tarsorrhaphies and permanent lower punctal occlusion. The patient healed completely, with no recurrence over 18 months of follow-up.

Early and Late Histological and Ultrastructural Findings in Resected Infantile Capillary Hemangiomas Following Treatment with Topical Beta-Blocker Timolol Maleate 0.5.

BACKGROUND: Infantile capillary hemangiomas (IHs) affect approximately 4-5% of infants. The systemic nonselective ß-adrenergic antagonist, propranolol, has become the standard first-line treatment for severe IHs. The topical ß-antagonist, timolol maleate, has also demonstrated efficacy and safety in treating superficial and some deep capillary hemangiomas. Despite their therapeutic success and prevalent use, the mechanism of action of ß-adrenergic antagonists in the treatment of IHs is not well understood. METHODS: Histopathological and electron microscopic evaluation of two periocular IHs excised at 1 week and 24 months following topical timolol treatment was performed. RESULTS: Distinct morphological differences were observed between spontaneously regressed and ß-antagonist-treated IHs. The former was characterized by diffuse collagen deposition and interstitial fibrosis, while the latter showed organized concentric collagen IV deposition within obliterated vessel lumen, suggestive of waves of endothelial cell apoptosis, leaving behind layers of basement membrane deposits as a stress response. CONCLUSIONS: Based on these observations, we hypothesize that, apart from their well-known cardiac and vasodilatory effects, ß-antagonists could induce endothelial cell apoptosis in IH leading to endovascular occlusion and we present supporting evidence to explain why this response might be specific to hypoxic tissue.

Topical timolol maleate 0.5% solution for the management of deep periocular infantile hemangiomas.

This retrospective, consecutive, clinical case series examined the use of topical timolol in the treatment of 5 children with deep, periocular infantile hemangiomas that caused astigmatism, change in head posture, or ptosis. All patients were treated with timolol maleate solution 0.5% twice daily until the lesions had regressed. All 5 children showed regression of the lesion and improvement in amblyogenic risk factors within 2 weeks.

Review of topical beta blockers as treatment for infantile hemangiomas.

The treatment of infantile hemangiomas changed from the use of oral corticosteroids to oral propranolol on the serendipitous discovery of propanolol's clinical effectiveness in 2008. Since then, clinicians have begun to use topical beta blockers--in particular, timolol maleate 0.5% gel forming solution--with good effect. Topical beta blockers are now used for lesions with both deep and superficial components and those that are amblyogenic. When initiated in the proliferative phase of the lesion, the effectiveness of the treatment can be seen within days. There is no consensus on dosing, treatment bioavailability, or clinical assessment of lesions, but these are topics for future research.

A simple technique for temporary eyelid closure in severe exposure keratopathy.

We describe a novel and simple technique for temporary lid closure in severe bilateral corneal exposure keratopathy, where a rapid method for corneal protection is required. The aim is to create a stable dressing base for secure closure of the eyelids that also allows instillation of medication and examination of the eye.

Retropupillary Artisan intraocular lens implantation in very young children with aphakia following penetrating eye injuries.

Visual rehabilitation of children with penetrating eye injuries often carries a poor prognosis. When capsular support is insufficient, implantation of the aphakic Artisan (Ophtec, The Netherlands) iris-claw intraocular lens (IOL) in front of the iris is an accepted alternative to anterior chamber or scleral-fixated implantation. With anterior chamber lens implantation, however, there is concern about long-term endothelial loss. Posterior iris (or retropupillary) fixation of an Artisan IOL theoretically reduces this risk. We present three consecutive cases of penetrating eye injury resulting in aphakia successfully repaired by retropupillary implantation of the Artisan IOL. The patients achieved best-corrected visual acuities of 20/25 to 20/30 (0.10-0.15 logarithm of the minimum angle of resolution) at 8, 13, and 22 months' follow-up, respectively.