Childhood cataract is a common cause of visual impairment. Familial types are uncommon among Filipinos. Furthermore, it is not common to have one that follows an autosomal dominant pattern of inheritance but with associated syndromic presentation like Roberts syndrome which is an autosomal recessive disorder. This is a case of a 9-year-old Filipino boy with cataract in the left eye associated with low-set ears, facial asymmetry, underdeveloped nasal ala, cleft lip and palate, macroglossia, micrognathia, short right shin, and absent feet. Patient was clinically diagnosed with Roberts syndrome. We present a clinically diagnosed Roberts syndrome (RS), the first reported RS in a Filipino in local and international literature to our knowledge with an autosomal dominant childhood cataract. Genetic testing can assist in the confirmation of this case.

The detachment and reattachment of inferior oblique (IO) have been done by ophthalmologists in inferomedial orbital wall fracture repair to avoid inducing a new diplopia in patients post-operatively. However, doing them in orbital wall reconstruction in patient who underwent maxillectomy for a malignancy has not been described yet. We describe a case where a disinserted inferior oblique was reattached after titanium mesh implantation to prevent diplopia after the surgery. This is the case of a 40-year-old male diagnosed with recurrent bilateral nasomaxillary ameloblastoma who underwent total maxillectomy of the right with removal of the inferomedial orbital wall, and detachment and reattachment of inferior oblique with no resulting inferior oblique palsy and diplopia from hypotropia or incyclotorsion. This is the first report, to our knowledge, to describe reattachment of IO in its approximate insertion during reconstruction of the inferomedial orbital wall after total maxillectomy from a malignancy with no resulting diplopia.

Background: Retinoblastoma is the most common intraocular cancer in childhood in the Philippines. Most data though on demographics, clinical profile, treatment options, and outcomes in the country are from the National Capital Region. Objectives: This study aimed to describe the demographics, clinical profile, treatment done, and outcomes of retinoblastoma patients seen in a public tertiary referral center in Davao from 2011-2020 to make available literature more representative of the status of retinoblastoma in the Philippines. Methods: An analytical cross-sectional study was conducted using the records of retinoblastoma patients seen in a tertiary government hospital located in Davao Region from January 2011 to December 2020. Results: There were 157 patients included in the analysis. Seventy-three (46%) were female with 44% coming from the Davao Region. One hundred seven (69%) patients had unilateral disease. Median age at initial consultation for patients with unilateral disease was significantly older than those with bilateral disease (p<0.003). Tumors were extraocular in 82 (40%) eyes. In the intraocular group, 36% of the eyes belonged to International Classification of Retinoblastoma (ICRB) Groups D and E. Enucleation was the most commonly performed treatment. Survival rate was 28%. This is the first report to provide epidemiologic and clinical data on retinoblastoma in the literature, including survival data, from Mindanao. Advanced stages and extraocular cases of retinoblastoma remain high. Delay of consultation contributed to the prognosis and clinical outcome of the disease. Conclusion. Advanced stages and extraocular cases of retinoblastoma remain significantly high in the country, even in Mindanao.
Retinoblastoma ; Therapeutics ; Survival Rate

Background and Objective: The Philippines does not have a national congenital rubella syndrome (CRS) surveillance or registry. Regular monitoring of CRS cases in hospitals, including in a Philippine tertiary hospital, helped in the past to provide clinico-epidemiologic data on CRS. This study aimed to continue providing clinico-epidemiologic data on CRS cases seen in the Philippine tertiary hospital from 2009-2012 and 2019-2022 and compare the cases seen from said timelines. Methods: A cross-sectional study was used, employing chart review of patients newly diagnosed with CRS from 2009-2012 and 2019-2022 in the Department of Ophthalmology and Visual Sciences at the Philippine tertiary hospital. Results: Forty-two patients newly diagnosed with CRS from 2009-2012 and 2019-2022 were included. Only 14 (33%) were serologically-confirmed cases (albeit qualitatively). Median age (first and third interquartile ranges) at consult was 1 year (0.4, 2.5). Twenty-four (57%) patients had maternal history of rashes and/or fever. Trimester of pregnancy when mother became symptomatic was not significantly correlated with chief complaint (p=0.20) and numbers of ophthalmic (p=0.68) and systemic manifestations (p=0.32). Cataract was the most common ophthalmic manifestation present in 40 (95%) patients. Twenty-six (62%) patients had other associated systemic findings of which hearing loss was the most common. Only 29 of 40 patients with cataract underwent lensectomy, with 23 patients having poor visual prognosis prior to surgery (5 with nystagmus alone, 10 with nystagmus and strabismus, and 8 with strabismus alone). Discussion: Using ophthalmic manifestations as primary indicator, this study provided an update on the CRS cases in the country. Laboratory confirmation remains a challenge in diagnosing CRS as the tests are costly and not widely available. There was increase from 2009-2012 compared to 2019-2022 in number of patients who underwent surgical treatment for cataract but visual outcomes were suboptimal due to delay in consultation. Although there was a decrease in number of CRS cases seen in the Philippine tertiary hospital, this cannot be attributed to increased rubella-containing vaccine (RCV) coverage alone. Conclusion Provision of data from individual hospital-based studies similar to this highlights the need for a national CRS surveillance system or registry. This can better gauge the burden of CRS and identify the gap in RCV coverage.
Rubella Syndrome, Congenital ; Retinitis Pigmentosa

OBJECTIVE

This study compared the demographics, clinical profile, treatment, and outcomes of retinoblastoma patients seen at medical institutions in the Philippines between two time periods: 2010 to 2015 and 2016 to 2020.

This was a multicenter, analytical, cohort study using review of medical charts and databases of retinoblastoma patients seen in 11 medical institutions from 2010 to 2020.

There were 636 patients (821 eyes) included in this study: 330 patients were seen in 2010 to 2015 while 306 in 2016 to 2020. More cases per annum were seen in the latter timeline. The number of patients with unilateral disease was not significantly different between the two time periods (p=0.51). Age at onset of symptom, age at initial consultation, and delay in consult were also not significantly different between the two time periods (p > 0.05). Patients had significantly different distributions of intraocular grades (p < 0.0001) and systemic staging (p < 0.0001) between the two time periods. Enucleation was the most common surgical treatment performed in both timelines. There was significant difference in the status of patients based on the need for systemic chemotherapy (p < 0.01). There was significant difference in outcome between the two time periods, including the proportions of living and deceased patients.

This study compared the most comprehensive data on retinoblastoma patients in the country. There was no improvement in early health seeking behavior based on similar age at initial consult and delay in consult. Enucleation remained the most common treatment mode as opposed to chemotherapy due to similar percentage of patients with unilateral disease, an indication for enucleation rather than chemotherapy.

Uncorrected or unaddressed refractive error (URE) is the leading cause of treatable visual impairment (VI) globally. A significant factor is the prohibitive costs of corrective options. The World Health Organization recently recommended the use of effective refractive error coverage (eREC) to determine the burden and management of URE. To increase eREC, spectacles should be made available and affordable. Most developing countries use ready-made glasses produced in bulk to address presbyopia. Timor-Leste employed a tiered- pricing for these ready-made spectacles which were found to be effective. The Philippines can adopt similar initiatives considering that prescription spectacles are not covered by its national health insurance. Prescription spectacles should also receive coverage from the national insurance. Policies should also be created that will set-up optical units inside government hospitals and primary health care units that can dispense low cost or free prescription spectacles. Dedicated government posts for optometrists should be created to man the said units. Existing colleges of optometry can partner with nearby public health facilities to man their optical units similar to partnerships made for other health professions. State universities can also consider opening colleges of optometry where they can tie up return services conditions or follow ladderized programs based on community demands similar to existing ones for other health professions
Refractive Error ; spectacles ; cost

Background: Infants with congenital rubella syndrome (CRS) often require multiple diagnostic procedures and interventions that include cataract surgery and procedures for congenital heart abnormalities. CRS is a vaccine preventable disease. Objective: This study aimed to determine the costs incurred by the parents, Philippine Health Insurance Corporation (PHIC), hospital's medical social service (MSS), and non-governmental organization (NGO) in the management of CRS. Methods: This is a costing study of five children diagnosed with probable CRS who were managed in a tertiary government hospital in Northern Luzon, Philippines. The parents or guardians of the patients were interviewed on the cost incurred particularly on non-medical related expenses during their outpatient department consultations and admissions. Hospital bills from our institutions and those from the previous institutions, if available, were retrieved. Expenses incurred from procedures or medical supplies relating to known complications of CRS were included in the computation. Results: All five patients diagnosed with CRS had cardiac, ear, and eye manifestations. Two patients had postnatal complications. The average cost spent by the five patients' early years of life (mean age of patients was 16 ± 14 months) was ₱409,740.84. A quarter of the cost was out-of-pocket expenses while a third was covered by the hospital's MSS where the patients were seen. Another third was shouldered by an NGO. Most expenses were from the treatment of cardiac complications at 42% of the cost and had the highest average cost at ₱116,586.59. Case 1 had the highest financial cost at ₱833,514.24 mainly from the cardiac complications of CRS. Conclusion The cost of CRS in the early years of life is high. This is a significant financial burden to parents, PHIC, hospital's MSS, and NGO.
Congenital rubella syndrome ; cataract

Objective: To present a rare case of Netherton Syndrome (NS) in a Filipino child. Case: This is a case of an 11-year-old girl with elevated immunoglobulin E, trichorrhexis nodosa, and ichthyosis linearis circumflexa. She had dry skin with fine scaling at birth with recurrent pruritic, erythematous papules coalescing to plaques mainly on the face and extensors. The skin dryness turned to generalized redness with fine scaling, and the skin started getting tight. She could no longer completely extend both knees, but she was still able to walk. She also started having difficulty closing both eyes. She developed migratory serpiginous erythematous plaques with peripheral double-edged scaling. At six years old, she developed ulcers in the scalp, trunk, and extremities, which resulted in admission to our institution. She was managed for multiple skin infections, pneumonia, sepsis, seizure, severe malnutrition, joint contracture, atopy, and bilateral cicatricial ectropion. Conclusion Management of NS remains challenging. Common management options include emollients, topical corticosteroids, calcineurin, and protease inhibitor, and phototherapy while newer ones that need further validation include intravenous immunoglobulins and biologics such as infliximab. However, until specific recommendations are made, overall management for NS remains challenging. Regular multidisciplinary monitoring of the manifestations of NS is central to its management.
Netherton syndrome ; ichthyosis ; trichorrhexis nodosa ; atopy

Background: Intravitreal chemotherapy has been an effective addition in treating vitreous seeding in retinoblastoma. However, it was only in 2020 that it was used in the Philippines. There is no literature on its use in multiple Filipino retinoblastoma patients. Objectives: To describe the clinical course of the four patients who are the first to undergo intravitreal chemotherapy for vitreous seeding of retinoblastoma in the Philippine tertiary hospital. Methods: A case series of four eyes of four patients with retinoblastoma who underwent intravitreous injection of melphalan and topotecan for vitreous seeding at the Department of Ophthalmology and Visual Sciences of a Philippine tertiary hospital. Results: Two eyes, with International Intraocular Retinoblastoma Classification (IIRC) Group C with vitreous seeding, responded well to intravitreous melphalan and topotecan. One eye had recurrent vitreous seeding despite 10 intravitreal injections. One eye with IIRC Group E, did not respond to intravitreous chemotherapy and was eventually enucleated. This is the first case series on the local use of intravitreous chemotherapy in the country for vitreous seeding in retinoblastoma. The control of 50% achieved in this case series is lower than in other series due to longer treatment interval from poor follow-up and the presence of advanced disease. Conclusion The use of intravitreous melphalan and topotecan can be an effective adjuvant for systemic chemotherapy in controlling vitreous seeding in eyes with IIRC Group C. It is not effective in controlling IIRC Group E disease.
intravitreous ; melphalan ; topotecan ; retinoblastoma ; Philippines

Cranial nerve (CN) III palsy is rarely caused by intraorbital compression, let alone from a subgaleal abscess. We present a case of a hypertensive 55-year-old man with an acute isolated pupillary-sparing left CN III palsy from a left subgaleal abscess with associated pterional osteomyelitis and frontotemporal mass. This is the first reported such case and the third reported case of a chronic spontaneous subgaleal abscess. A seemingly routine case of an acute, pupillary-sparing, isolated CN III palsy from hypertension turned out to be a rare case in terms of etiology of the palsy and of the source of the abscess.
Cranial Nerve III ; palsy ; ptosis ; ophthalmoplegia