Childhood glaucoma is a rare disorder that occurs from birth until teenage years caused by an abnormality of aqueous humor pathways. About 50-70% of Peters\' anomaly is accompanied by secondary childhood glaucoma. The presence of glaucoma will affect the prognosis. We reported the evaluation and treatment of secondary childhood glaucoma due to Peters\' anomaly. A 5 months-old boy was presented with the complaint of a enlarged left eye since 3 months old. The complaint was accompanied by a watering eye and frequently closed upon light exposure. The left eye looked opaquer than contralateral. Examination under anesthesia showed that the intraocular pressure (IOP) was 35 mmHg in the left eye and the corneal diameter was 14 mm. Other findings were keratopathy, diffuse corneal edema, buphthalmos, shallow anterior chamber, anterior synechiae, and linear slit shaped pupils in the nasal region. Patient was treated with ophthalmic timolol maleate which was later followed by trabeculectomy. After 1 week post-surgery, IOP assessment by palpation suggested the right eye within normal range while the IOP of left eye was higger than normal. Blepharospasm, epiphora, photophobia, bleb on superior, subconjunctiva bleeding, buphthalmos, keratopathy, minimal corneal edema, anterior chamber with shallow image, and posterior synechia were found in left eye anterior segment. In conclusion, trabeculotomy and trabeculectomy are recommended if there is no reduction of IOP observed after receiving timolol maleate therapy. The choice of surgical management is dependent on the feasibility of the protocol.

Peters\' anomaly (PA) is a manifestation of complex disorders in the development of the anterior segment of the eye. The most recognizable feature of the disease is a doughnut-shaped central corneal opacity and adhesions between the opacity and underlying iris. Glaucoma is observed in 30-70% of patients, with up to 50% of the patients showing concomitant vision-threatening disorders. Up to 60% of patients have systemic abnormalities or developmental delays. Being a rare malformation, PA is one of the most common congenital indications for corneal transplantation in infants. Penetrating keratoplasty is used as the primary method of treatment in cases with corneal opacification of a degree that forbids visual development in both eyes. The heterogeneity of co-occurring ophthalmic and systemic malformations in the spectrum of PA determines the wide range of success, defined by various endpoints: graft clarity or visual acuity. Although surgical advancement has made corneal grafting possible in younger children, it has a higher graft failure rate and worse visual prognosis than adult keratoplasty. Optical sector iridectomy, pupil dilation, or cornea rotation can alternatively be performed. Satisfying results of pediatric keratoprosthesis in particular cases of PA have been described. Postoperative treatment of PA aims to maintain a clear optical pathway and prevent amblyopia. This article therefore aims at reporting the ophthalmic treatment and need for multidisciplinary management of PA, including pharmacological and surgical treatment.

BACKGROUND: To evaluate anterior synechiae after penetrating keratoplasty (PK) in patients with Peters\' anomaly using anterior segment optical coherence tomography (OCT).
METHODS: A retrospective cross-sectional study was performed. The medical records of patients diagnosed with Peters\' anomaly who underwent PK between 2013 and 2018 were reviewed. In addition to basic ophthalmic examinations, images of anterior segment structures were obtained via spectral-domain OCT at baseline and during the postoperative follow-up period. The profiles of postoperative anterior synechiae and multiple potential risk factors were analyzed.
RESULTS: Seventy-one eyes of 58 patients, aged 5 to 23 months, were included. Various extent of postoperative anterior synechiae was observed in 59 eyes (83.1%). OCT findings revealed graft-host junction synechiae, peripheral anterior synechiae, and a combination of both. Disease severity and malposition of the internal graft-host junction were significantly associated with the formation of postoperative synechiae. Multivariate regression analysis found that preexisting iridocorneal adhesion [odds ratio (OR) = 16.639, 95% confidence interval (CI) 1.494-185.294, p = 0.022] was positively correlated with postoperative anterior synechiae, whereas anterior chamber depth (OR = 0.009, 95% CI 0.000-0.360, p = 0.012) and graft size (OR = 0.016, 95% CI 0.000-0.529, p = 0.020) were negatively correlated with postoperative synechiae. In addition, quadrants of preexisting iridocorneal adhesion and width of the host corneal bed were identified as risk factors for increased postoperative anterior synechiae.
CONCLUSIONS: Anterior synechiae following PK is a relatively common occurrence in Peters\' anomaly patients and is found to be associated with preexisting iridocorneal adhesion, a shallow anterior chamber, small graft size, graft-host junction malposition, and graft closer to the corneal limbus. These data indicate the need for careful consideration when performing PK on these patients.

Peters\' anomaly (PA) is a rare form of anterior segment dysgenesis characterized by central corneal opacity accompanied by iridocorneal or lenticulo-corneal adhesions. Although genetic mutations, particularly those affecting transcription factors that function in eye development, are known to cause PA, the etiology of this disease remains poorly understood. In this study, 23 patients with PA were recruited for panel sequencing. Four out of 23 patients were found to carry variants in known PA causal genes, PITX2 and PITX3. More importantly, two homozygous mutations (NM_057164: p.Val86Ala and p.Arg689Cys) in the COL6A3 gene (collagen type VI alpha-3 chain) that correlated with the phenotype of type I PA were identified, and then validated by following whole-exome sequencing. The expression profile of the COL6A3 gene in the cornea and the impact of the mutations on protein physiological processing and cellular function were further explored. It was shown that COL6A3 presented relatively high expression in the cornea. The mutant COL6A3 protein was relatively retained intracellularly, and its expression reduced cellular resistance to oxidative stress through an enhanced endoplasmic reticulum stress response. Taken together, our findings expanded the known genetic spectrum of PA, and provided evidence for the involvement of COL6A3 or collagen VI in ocular anterior segment development, thereby offering new insight for future investigations targeting PA.

Primary hyperplastic persistent vitreous (PHPV) or persistent fetal vasculature is a rare clinical entity that presents with leucocoria, microphthalmos, and cataract. It is mostly unilateral. Here we present a report of two cases of bilateral PHPV. One of the patients had associated Peters\' anomaly. The entity closely mimics retinoblastoma and should be kept as a differential diagnosis of bilateral leucocoria. Examination under anesthesia, ultrasound B-scan, and aqueous lactate dehydrogenase levels helped us reach the diagnosis and differentiate it from the more serious entity retinoblastoma.

Peters\' anomaly is a rare congenital eye condition characterized by anterior segment dysgenesis and commonly presents as unilateral or bilateral corneal opacity in the early neonatal period. Peters\' anomaly is often associated with congenital brain and skull abnormalities, which are frequently overlooked. In this paper, we present a case of a 5-day-old female neonate with Peters\' anomaly, and review the literature for similar reports that describe associated brain imaging findings. In our case, imaging studies show abnormalities involving the anterior segments of both globes with absent intracranial manifestations. Although Peters\' anomaly is a condition of interest for ophthalmologists, radiological studies should be performed, and neuroradiologists should be aware of the imaging findings associated with this rare entity.

Penetrating keratoplasty in children is associated with very specific difficulties for the surgeon as well as for the patient and the parents. Special features are specific pediatric indications, which do not occur in adults, a more difficult examination and treatment adherence depending on the parents. Diseases with a favorable prognosis include keratoconus and herpetic keratitis. Especially sclerocornea and the Peters\' anomaly often have a limited prognosis regarding vision and graft survival due to secondary malformations of the eye. In addition, younger age represents a risk factor. This is most likely due to the impaired examination during follow-up and reduced compliance. For successful penetrating keratoplasty in children the timing for the operation, in which the risk for the graft is weighed up against the risk for amblyopia, is crucial.

Peters\' anomaly accounts for the highest type of Anterior Segment Dysgenesis (ASD). The main features of Peters\' anomaly are: congenital corneal opacity centrally, defect in the posterior stroma and absence of Descemet\'s membrane and the endothelium. However, this condition has wide clinical and histopathological variations in appearance, associations and severity. In this case series, we summarize 6 corneas in 5 Saudi cases of Peters\' anomaly (and describe 2 in detail) with unique histopathological findings that are additional to the typical known ones, shedding some light on the nomenclature of these variants according to the reported cases in the English-written literature. This will widen the spectrum of findings known to ophthalmic pathologists and ophthalmologists about this anomaly. This is also of importance in the assessment of the congenital glaucoma cases commonly seen in Saudi Arabia that often happens in association with ASD.

The treatment of congenital glaucoma requires special expertise and often novel surgical approaches. The combined use of a XEN and a Baerveldt implant is an alternative to conventional tube implants and may be less harmful for long-term corneal endothelial damage. If this technique does not provide sufficient long-term control of intraorbital pressure (IOP), a direct implantation of the Baerveldt tube can be easily performed in a second intervention.

A seven-month-old female domestic shorthaired cat was presented for buphthalmos in the right eye and corneal cloudiness in the left eye. Full ophthalmic examinations were performed for both eyes and enucleation was done for the right nonvisual eye. Congenital glaucoma caused by anterior segment dysgenesis was confirmed for the right eye. In the left eye, slit-lamp examination revealed focal corneal edema with several iris strands from iris collarette to the affected posterior corneal surfaces. Circular posterior corneal defect was suggested to be the cause of edema. Goniodysgenesis, additionally, was identified. Taken together, the diagnosis of Peters\' anomaly which is a subtype of anterior segment dysgenesis was suggested in the left eye.