Long-term Efficacy of Mitomycin C Augmented Trabeculectomy in a Mixed Pediatric Glaucoma Cohort.

PRECIS: This paper reports a retrospective consecutive case series investigating the efficacy of Mitomycin C-augmented trabeculectomy in the treatment of primary and secondary pediatric glaucoma in a mixed etiology, multiethnic cohort of patients. PURPOSE: To evaluate the long-term efficacy and safety of Mitomycin C-augmented trabeculectomy in a mixed, tertiary-referral, pediatric glaucoma cohort. METHODS: Retrospective consecutive review of all children (37 eyes) undergoing Mitomycin C-augmented trabeculectomy by a single surgeon between 2008 and 2016. Seventeen eyes (45.9%) had primary congenital glaucoma, and 20 eyes (54.1%) had secondary glaucoma. The median age at surgery was 11 months (range, 2 to 146). The mean follow-up was 69.2±4.7 months (range, 3.5 to 107.9). RESULTS: Overall, trabeculectomy was successful in 80.6% of eyes at 12 months, 60.5% at 3 years, and 57.5% at 5 years. 45.9% cases (17 eyes) required further laser or surgery for uncontrolled intraocular pressure (IOP) and were therefore deemed as failures. The time to failure ranged from 0.4 to 65.1 months (mean, 22.2±5.1 mo). The proportion of children achieving visual acuity of 1.0 LogMAR equivalent or better increased from 43.2% preoperatively to 63.6% at 1 year and 68% at 5 years. The mean IOP reduced from 24.85±0.88 mm Hg preoperatively to 15.14±0.94 mm Hg at 3 months (39% reduction) and 17.42±1.08 mm Hg at 5 years (30% reduction). IOP-lowering medication requirement reduced from 4.14±0.20 agents preoperatively to 0.84±0.22 at 3 months (80% reduction) and 1.78±0.36 at 5 years (57% reduction). There were no sight-threatening complications such as hyphaema, bleb leak, chronic hypotony, endophthalmitis, retinal detachment, or loss of light perception. CONCLUSIONS: This study provides valuable evidence that Mitomycin C-augmented trabeculectomy is safe and effective as a treatment of primary or secondary pediatric glaucoma, with particularly encouraging results in cases of secondary glaucoma. Trabeculectomy offers the potential for delaying or avoiding glaucoma drainage device surgery in a significant proportion of children.

Routine Orthoptic-led Paediatric Fundus Digital Imaging: Benefits to Patients and Healthcare System.

AIMS: Dilated fundus examinations are a vital, yet time-consuming and sometimes distressing part of paediatric ophthalmology examinations. Limited resources, personnel and time can result in prolonged waiting time and increase risk from delayed diagnosis and treatment. Using a Nikon D80 TopCon TRC-NW6S non-mydriatic fundus camera (TopCon (GB) Ltd, Newbury), we aimed to demonstrate the safety and efficacy of orthoptic-led fundus digital imaging and the potential time and cost benefits to the healthcare system. METHODS: We conducted a retrospective review of all digital fundus images taken over a six month period in 2012 (n = 616, age range 2.1-16.5 years, mean age 8.7 years). RESULTS: Overall success rate for paediatric fundus digital imaging was 97%. Successful images were achieved in 87% of patients without the need for pupil dilation. Images were taken for a variety of clinical reasons. 45% of patients were discharged immediately, many with copies of photographs to facilitate follow-up with community optometrists. CONCLUSIONS: Orthoptic-led fundus digital imaging is an innovative, speedy, safe and efficient method of documenting fundal appearance, enabling serial documentation of stability/progression of ocular disease. It allows adequate examination of routine patients, freeing up time within busy clinics. Paediatric fundus digital imaging brings a potential positive cost benefit to healthcare systems under pressure, and facilitates skill development for allied health professionals.

Coats disease in a 3-week-old boy.

Coats disease is a rare, idiopathic retinal vasculopathy that predominantly affects males in the first decade of life. We report the case of a 3-week-old boy who presented with atypical rapidly progressing disease suggestive of Coats exudative vasculopathy. The eye developed retinal fibrosis and phthisis bulbi within 4 weeks. Retinoblastoma could not be ruled out, although histopathology after enucleation revealed no retinoblastoma. To our knowledge, this is the youngest case of Coats disease to be reported in the literature. This case highlights the wide variation in the clinical presentation of Coats disease and the difficulty in differentiating it from diffuse infiltrative retinoblastoma.

Long-term retinoblastoma follow-up with or without general anaesthesia.

BACKGROUND: Children with treated retinoblastoma undergo frequent examinations to monitor for recurrent or new tumours. Examinations under anaesthesia allow a more complete examination in younger children, however they are stressful for the family, subject the child to medical risk and consume resources. The risk of recurrent or new tumours declines with age and it is common practice to examine older children without general anaesthesia. There are no studies on the safety and cost effectiveness of this practice, or guidelines on when examination without anaesthesia (EWA) can be safely commenced. PROCEDURE: Retrospective case note review of 128 sequential patients treated for retinoblastoma in a national referral centre over 10 years. RESULTS: Following exclusions, 113 eyes of 84 children were analysed. The mean age at diagnosis was 20 months (range birth to 71 months). There were 55 unilateral and 29 bilateral cases. The mean follow-up was 77.7 months (range 12-178 months). EWA was commenced at a mean age of 53 months (range 12-98 months). The age of conversion to EWA was largely dependent on child cooperation and disease activity. Tumour activity was detected on EWA in one child at the age of 86 months, 9 months after the last active treatment and treated successfully. CONCLUSIONS: Examination without general anaesthesia does not appear to expose children to an increased risk of undetected tumour growth. This study highlights the important factors to be considered when deciding a safe time to commence EWA.

Fibrous tumor of the superior oblique tendon in Proteus syndrome.

Proteus syndrome is a disorder of patchy, or mosaic, postnatal overgrowth. Mosaic somatic mutation of the AKT1 gene has been identified in over 90% of individuals meeting the diagnostic criteria for Proteus syndrome. Onset occurs in infancy and can involve any tissue of the body. The connective tissue and bone, skin, central nervous system, and the eye are commonly involved. Epibulbar tumors, strabismus, and posterior segment involvement have previously been reported. However, there have not been any reports of lesions affecting the extraocular tendons. We report a case of Proteus syndrome patient presenting with vertical strabismus secondary to a fibrous tumor within the superior oblique tendon. The tumor was successfully excised with complete resolution of the strabismus.

"Triple-TEN" in the treatment of acute ocular complications from toxic epidermal necrolysis.

PURPOSE: Toxic epidermal necrolysis (TEN) is a devastating form of Stevens-Johnson syndrome (SJS) with acute and chronic ocular complications. We present a novel aggressive combination strategy, termed "Triple-TEN", for the management of acute ocular TEN designed to minimize the risk of chronic, blinding sequelae. METHODS: Two patients with life-threatening TEN accompanied by severe ocular surface defects and fulminant symblephara formation underwent "Triple-TEN" management of their acute ocular disease under aseptic techniques in the critical care setting, after failed treatment with intensive topical therapy and surgical division of symblephara. The Triple-TEN protocol comprises (1) subconjunctival triamcinolone (Kenalog 20 mg) administered into each of the fornices to curb the local inflammatory response without compromising systemic immunity, (2) placement of amniotic membrane tissue mounted on a polycarbonate skirt (ProKera) over the corneal and limbal regions to facilitate reepithelialization of the ocular surface, and (3) insertion of a steeply curved acrylic scleral shell spacer (Technovent, SC21) to vault the lids away from the globe providing a barrier to symblephara formation. RESULTS: In both cases, ocular surface inflammation resolved within 4 weeks with no progression of conjunctival cicatrization or evidence of limbal epithelial stem cell failure at 1 year follow-up. There were no long-term complications of the Triple-TEN regimen. CONCLUSIONS: Aggressive treatment with the Triple-TEN protocol for acute ocular TEN resistant to first-line therapy, may help prevent long-term blinding sequelae.

Retinoblastoma - current treatment and future direction.

Retinoblastoma is the commonest primary ocular malignancy of childhood. There are two forms - heritable and non heritable. Heritable retinoblastoma is a cancer susceptibility syndrome. Presentation is in the first few years of life, sometimes in the neonatal period. Early detection and prompt treatment can give cure rates up to 95% for intraocular tumours, but extraocular disease carries a very high mortality. The diagnosis is essentially clinical and biopsy is contraindicated due to the risk of extraocular spread. Treatment requires significant multidisciplinary input, with local ophthalmic treatment, systemic chemotherapy and external beam or plaque radiotherapy, or surgery to remove the affected eye. Screening of family members is essential for early detection. Lifelong surveillance of mutation carriers is needed due to the risk of second cancers. Newer treatment modalities including intra-arterial chemotherapy have been added to the therapeutic armamentarium in recent years.

Relationship of infantile periocular hemangioma depth to growth and regression pattern.

PURPOSE: Most infantile periocular hemangiomas undergo rapid growth in the first year of life, followed by gradual resolution over years. Treatment is indicated if vision is compromised and is usually continued through the growth phase. The objective of this study was to determine which clinical characteristics might aid in the prediction of growth and/or regression patterns of periocular hemangiomas. METHODS: Retrospective review of medical records and photographs of children with periocular hemangiomas presenting to a UK pediatric eye unit over a 7-year period. Age at presentation, growth pattern, size, location, amblyopia, and refractive status were documented. RESULTS: Forty-two infants with periocular hemangiomas were evaluated between 2000 and 2007, with a mean follow-up of 24 months (range, 6 months to 5 years). One-third (n=14, 33%) of the hemangiomas were superficial (strawberry nevi); one-third were subcutaneous (n=13, 31%), and the remainder were mixed (n=8, 19%) and orbital (n=7, 17%). There was a marked difference between the growth patterns of superficial (strawberry nevi) and deeper hemangiomas (orbital and subcutaneous), with a more prolonged period of growth noted in the deeper hemangiomas. CONCLUSIONS: Periocular hemangiomas with a deep component tend to have a later onset and prolonged period of growth compared to strawberry nevi. Clinically evident depth of the hemangioma appears to be a valuable predictor of rapidity of resolution. This finding may be useful in assessing prognosis and planning treatment of infantile periocular hemangiomas.

Role of polymerase chain reaction in early diagnosis of herpes zoster ophthalmicus in children.

Herpes zoster ophthalmicus is uncommon in children, and diagnosis may be delayed until the typical skin lesions become apparent. Delay in appropriate treatment may result in sight-threatening complications. We report a case of an 18-month-old child in whom we promptly confirmed the clinical suspicion of herpes zoster ophthalmicus by the identification of viral DNA using polymerase chain reaction. This procedure enabled us to administer prompt antiviral treatment, preventing sight-threatening sequelae. We review the literature regarding the possible mechanism of herpes zoster ophthalmicus in immunocompetent children and discuss the role of polymerase chain reaction in its diagnosis.

Head position-dependent changes in ocular torsion and vertical misalignment in skew deviation.

OBJECTIVES: To investigate whether ocular torsion and vertical misalignment differ in the upright vs supine position in skew deviation and to compare these findings with those in trochlear nerve palsy. METHODS: Ten patients with skew deviation, 14 patients with unilateral peripheral trochlear nerve palsy, and 12 healthy subjects were prospectively recruited. With subjects first in the upright position and then in the supine position, ocular torsion was measured by double Maddox rods and vertical misalignment was measured by the prism and alternate cover test. RESULTS: In patients with skew deviation, the abnormal torsion and vertical misalignment in the upright position decreased substantially with change to the supine position, whereas in patients with trochlear nerve palsy, it changed little between positions. Torsion was decreased by 83% in patients with skew deviation, 2% in patients with trochlear nerve palsy, and 6% in healthy subjects (P < .001). Similarly, vertical misalignment was decreased by 74% in patients with skew deviation and increased by 5% in patients with trochlear nerve palsy and 6% in healthy subjects (P < .001). CONCLUSIONS: Our findings provide the basis for additional clinical tests to support the classic 3-step test: ocular torsion and vertical misalignment that decrease from the upright position to the supine position indicate skew deviation, whereas torsion and vertical misalignment that do not change significantly between positions indicate trochlear nerve palsy.

Tubular aggregate myopathy associated with retinal degeneration.

CASE REPORT: We report a case of congenital tubular aggregate myopathy associated with retinal degeneration. COMMENTS: Bilateral, asymmetric retinal degeneration developed in a 37-year-old woman with a history of congenital tubular aggregate myopathy. The major pathological feature was the presence of tubular aggregates, believed to arise from the sarcoplasmic reticulum, which are present in skeletal muscles only. The abnormal functioning of the smooth muscles of the pupillary dilator, together with retinal degeneration in our patient, suggests that tubular aggregates may represent a more generalized disequilibrium of intracellular calcium homeostasis that may not be confined to skeletal muscles.