BACKGROUND: Oculocutaneous albinism (OCA) is a group of autosomal recessive hereditary disorders that affect melanin biosynthesis, resulting in abnormalities in hair, skin, and eyes. Retinopathy of prematurity (ROP) is a proliferative retinopathy mainly observed in premature infants with low birth weight and early gestational age, but it can also affect full-term infants or children with normal weight, particularly in developing countries. The coexistence of ROP and OCA is rare. There is limited documentation regarding treatment approaches, with few studies reporting positive outcomes with laser treatment due to the absence of melanin pigment. This study discusses the treatment challenges in a female infant diagnosed with ROP and OCA, and underscores the importance of genetic analysis in guiding therapeutic decisions for this rare comorbid condition.
METHODS: The study presents a case of ROP occurring concurrently with OCA. Genetic testing revealed two variants, c.727C > T (p.R243C) and c.1832 T > C (p.L611P), in the OCA2 gene, inherited from the patient\'s mother and father, respectively. The identified mutations were consistent with a diagnosis of OCA2, classified as a subtype of OCA. The patient initially received intravitreal anti-vascular endothelial growth factor (anti-VEGF) injection, followed by laser photocoagulation therapy for a recurrent event. A favorable outcome was observed during the 2-month follow-up period.
CONCLUSIONS: The co-occurrence of ROP and OCA is a rare phenomenon, and this is the first recorded case in the Chinese population. The current case supports the use of laser as the primary treatment modality for ROP in OCA2 patients with partial pigmentation impairment. Furthermore, genetic analysis can aid in predicting the effectiveness of laser photocoagulation in this patient population.

To investigate the association of risk factors, including oxygen exposure, for developing retinopathy of prematurity (ROP) in preterm infants at increased risk of ROP.
A case-control study was conducted where each infant born at < 28 weeks gestation with ROP was matched with another without ROP over five years (July 2015 - June 2020). Clinical information about the infants was collected from electronic medical records, including method of oxygen delivery, oxygen saturation (SpO2), fraction of inspired oxygen (FiO2) and mean airway pressure (MAP) measurements. MATLAB was used for a time-averaged analysis. Stata/SE 16.0 was used for statistical analysis.
123 ROP/non-ROP pairs were included in this study. The time-averaged SpO2 analysis showed non-ROP group spent more time in hyperoxia than the ROP group (p < 0.001). The non-ROP group had lower respiratory severity scores and analysis when FiO2 > 21% showed that were was no difference in SpO2 between the two groups when the infants were receiving oxygen support. Conditional logistic regressions showed neonatal surgery significantly increased the risk of ROP (OR = 1.4347, p = 0.010), while the influence of birthweight (odds ratio of 0.9965, p = 0.001) and oxygen exposure (OR = 0.9983, p = 0.012) on ROP outcome was found to be negligible as their odds ratios indicated no influence.
At times when infants were receiving respiratory support (FiO2 > 21%) the SpO2 data indicated no difference in SpO2 between the ROP and non-ROP groups. Analysis of clinical variables found that neonatal surgery increased the odds of developing ROP.

To evaluate respiratory outcomes in preterm infants with retinopathy of prematurity (ROP) following intravitreal bevacizumab injection (IVB).
This single-centre study enroled preterm infants with a gestational age (GA) < 34 weeks or a birth weight (BW) < 1500 g with bilateral type 1 ROP who received a single IVB, and a treatment-free control group matched by GA, postmenstrual age, and respiratory status at the time of the IVB. The primary outcome was serial respiratory changes in mean airway pressure (MAP), fraction of inspired oxygen (FiO2), and respiratory severity score (RSS, MAP x FiO2) during the 28-day post-IVB/matching period and overall respiratory improvement at day 28 and at discharge. The duration of supplemental oxygen therapy following IVB/matching was documented.
A total of 5578 infants were included. Seventy-eight infants were enroled in the IVB group, and another 78 infants were matched as the control group. Both groups had downward trends in the MAP, FiO2, and RSS over the study period (all P < 0.001), but there were no between-group differences in these measures. The percentage of overall respiratory improvement was similar between the IVB and control groups, so was the duration of invasive and in-hospital oxygen ventilation. A lower percentage of oxygen dependence at discharge in the IVB group (P = 0.03) remained significant after adjusting for GA and BW.
This is a matched case study to evaluate respiratory outcomes in preterm infants following IVB for ROP. We found that the IVBs did not compromise respiratory outcomes in preterm infants during the 28-day post-IVB period and at discharge.

To determine whether red blood cell glucose-6-phosphate dehydrogenase (G6PD) activity is associated with retinopathy of prematurity (ROP).
This case-control study was conducted in a Level-3 neonatal unit. Subjects were inborn boys with birth weight <2000 g. \"Cases\" were consecutive subjects with ROP of any severity. \"Controls\" were consecutive unrelated subjects without ROP. Recipients of blood or exchange transfusions were excluded. Sixty cases (out of 98 screened) and 60 controls (out of 93 screened) were enrolled. G6PD activity (quantitative assay) as the candidate risk factor was evaluated.
Sixty cases with 60 controls [mean (SD) gestation 28.80 (2.2) and 30.60 (2.2) wk respectively] were compared. \"Cases\" had a higher median (1st, 3rd quartile) G6PD activity compared to \"controls\" [7.39 (4.7, 11.5) vs. 6.28 (4.2, 8.8) U/g Hb, p = 0.084]. G6PD activity was highest among ROP requiring treatment [8.68 (4.7, 12.3)] followed by ROP not requiring treatment [6.91 (4.4, 11.0)], followed by controls (plinear trend = 0.06). Gestation, birth weight, duration of oxygen, breastmilk feeding, and clinical sepsis were other variables associated with ROP on univariable analysis. On multivariable logistic regression, G6PD activity [Adjusted OR 1.14 (1.03, 1.25), p = 0.01] and gestation [Adjusted OR 0.74 (0.56, 0.97), p = 0.03] independently predicted ROP. C-statistic of the model was 0.76 (95% CI 0.67, 0.85).
Higher G6PD activity was independently associated with ROP after adjusting for confounders. Each 1 U/g Hb increase in G6PD increased the odds of ROP by 14%. Severer forms of ROP were associated with higher levels of G6PD activity.

OBJECTIVE: The purpose of the study was to investigate the association of platelet parameters and postnatal weight gain with treatment-requiring ROP (TR-ROP).
METHODS: In this retrospective matched case-control study, infants with TR-ROP were individually matched, according to gestational age and birth weight, with one or two untreated infants who developed no or spontaneously regressed ROP. Longitudinal data on platelet count (PLT), mean platelet volume (MPV), daily weight and platelet transfusions were collected. Platelet mass index (PMI) and weight standard deviation score (WSDS) were also calculated. Conditional logistic regression analysis was performed to adjust for matching.
RESULTS: Fourteen cases, presenting type I ROP, and 25 matched controls were included. The odds of developing TR-ROP decreased as PLT increased during 31st week of postmenstrual age (PMA) or during 1st and 2nd week of postnatal age (PNA). The odds of developing TR-ROP were 16.7 times higher in infants receiving at least one platelet transfusion compared with those who were not transfused. The odds of developing TR-ROP increased by 31.2% as the mean volume of platelet transfusion per infant increased by 1 ml. The odds of developing TR-ROP decreased as PMI increased during 1st week PNA, and as weight and WSDS increased during 4th -6th week PNA. Analysis of MPV, number of thrombopenic episodes per infant, number of platelet transfusions per infant and days with WSDS < -2 showed no association with TR-ROP.
CONCLUSIONS: To our knowledge, this is the first study ascertaining an association of platelet transfusions with type I ROP. Prospective cohort studies are required to confirm our findings.

Morning glory disc anomaly is associated with serous retinal detachments, high refractive errors, amblyopia, and strabismus. There have been limited reports of an association between morning glory disc and peripheral retinal non-perfusion. The authors report a case of unilateral morning glory disc anomaly associated with markedly asymmetric retinopathy of prematurity. [J Pediatr Ophthalmol Strabismus. 2022;59(5):e55-e57.].

BACKGROUND: Although several clinical studies have analysed the relationship between the levels of vascular endothelial growth factor (VEGF) and apelin-13 in venous blood and retinopathy of prematurity (ROP), no definitive conclusions have been reached. This study aimed to investigate the relationship between apelin-13 levels and VEGF levels and ROP.
METHODS: Differences in plasma apelin-13 and VEGF levels were analysed in two groups of infants born with birth weight < 1500 g and gestational age < 32 weeks at Peking University People\' s Hospital. One group comprised infants diagnosed with ROP and the other group was a control group comprising infants without ROP.
RESULTS: Apelin-13 levels were significantly lower in the ROP group than in the control group, while VEGF levels showed the opposite result (both P < 0.001). Infants with severe ROP had lower apelin-13 levels and higher VEGF levels than with mild ROP (both P < 0.05).The receiver operating characteristic curve for apelin-13 level as the indicator of ROP showed that a cut-off value of 119.6 pg/mL yielded a sensitivity of 84.8% and a specificity of 63.6%, while for VEGF level, the cut-off value of 84.3 pg/mL exhibited a sensitivity of 84.8% and a specificity of 66.7%.
CONCLUSIONS: Plasma apelin-13 and VEGF levels at 4-6 weeks of age may play a role in assisting the diagnosis of ROP.

OBJECTIVE: To report a newborn patient with gastrointestinal (GIS) perforation after intravitreal ranibizumab (RBZ) treatment.
METHODS: The patient was born at 31 gestational week and hospitalized with the diagnosis of small for gestational age and prematurity. In the follow up he underwent GIS surgery due to necrotizing enterocolitis (NEC) and was diagnosed with retinopathy of prematurity (ROP). At 43 weeks of postmenstrual age, he developed intestinal perforation after 12 h of the second low-dose RBZ injection. According to our knowledge, this is the first report of GIS perforation due to low-dose intravitreal RBZ treatment in an infant with severe ROP.
CONCLUSIONS: The risk of GIS perforation should be taken into consideration during the application of intravitreal vascular endothelial growth factor antagonist agents, especially in newborns with previous GIS surgery and a history of NEC, and these patients should be carefully monitored for GIS complications.

Presented here is a case of hybrid retinopathy of prematurity (ROP) with features of both aggressive posterior ROP and ridge-like staged ROP with extensive candle wax-like preretinal deposits plastered on the surface of the retina from optic disc to vascular-avascular junction at a postmenstrual age (PMA) of 34 weeks. The baby was too sick for laser photocoagulation and so underwent intravitreal bevacizumab at half adult doses in both eyes. The deposits melted dramatically within 1-2 weeks of antivascular endothelial growth factor injection without any signs of recurrence till the last visit at 58 weeks of PMA.

Premature infants who require surgery for retinopathy of prematurity often exhibit bronchopulmonary dysplasia. Reactive airway is a clinical manifestation of bronchopulmonary dysplasia. We describe premature infant twins who had difficulty with positive pressure ventilation during anesthesia. Both cases occurred during induction of anesthesia for binocular anterior chamber puncture and vitreous cavity injection. The most likely cause in each case was airway malacia. We recommend that endotracheal intubation is performed in infant patients with low body weight; the possibility of airway malacia occurrence should be considered, especially for infants with comorbid bronchopulmonary dysplasia.