Visco-Circumferential-Suture-Trabeculotomy Versus Rigid-Probe Viscotrabeculotomy in Neonatal-Onset Primary Congenital Glaucoma.

PRCIS: Angle procedures are safe and relatively effective for neonatal onset PCG. Watchful delay in intervention to close to the second month of life is helpful in ensuring diagnosis and making surgery more successful and easier. PURPOSE: The purpose of this study was to compare the surgical outcomes of visco-circumferential-suture-trabeculotomy (VCST) to rigid-probe double-entry viscotrabeculotomy (DEVT) and rigid-probe single-entry viscotrabeculotomy (SEVT) in infants with neonatal-onset primary congenital glaucoma (PCG). DESIGN: This was a retrospective chart review. PATIENTS AND METHODS: Retrospective chart review of 64 eyes of 64 infants with neonatal-onset PCG referred to Mansoura Ophthalmic Center in Mansoura, Egypt between February 2008 and November 2018. Study groups included VCST, DEVT, and SEVT, and follow-up covered 4 postoperative years. Complete (qualified) success was defined as intraocular pressure (IOP) ≤18 mm Hg and with 35% IOP reduction from baseline without (with) IOP-lowering medications or further surgical interventions, and without any sign of progression in corneal diameter, axial length, or optic disc cupping and without visual devastating complications. RESULTS: The mean±SD age at presentation and at the surgery of the study children was 3.63±1.74 and 55.23±1.60 days, respectively. The mean±SD IOP and cup/disc ratio of all study eyes at presentation and at final follow-up were 34.91±0.82 mm Hg and 0.70±0.09 and 17.04±0.74 mm Hg and 0.63±0.08, respectively. Complete success was achieved in 54.5%, 43.5%, and 31.6% in the VCST, DEVT, and SEVT groups, respectively. A self-limited hyphema was the commonest complication in all groups. CONCLUSIONS: Angle procedures are safe and marginally effective for the surgical treatment of neonatal-onset PCG, bringing IOP under control for at least 4 years of follow-up. Circumferential trabeculotomy as a first-line treatment has more favorable outcomes than rigid-probe SEVT. Rigid-probe viscotrabeculotomy offers an alternative to the noncompleted circumferential procedure.

Neglected Congenital Glaucoma With Lens Coloboma.

PURPOSE: To report a case of lens coloboma in a case of neglected primary congenital glaucoma. MATERIALS AND METHODS: A 5-year-old boy was brought by the parents with complaints of diminution of vision in both eyes noticed for 8 months. There was a history of enlargement of eyes since 1 year of age. RESULTS: Clinical examination revealed bilateral large eyes with limbal stretching and Haab striae and lens coloboma in the right eye. Dilated examination revealed scalloped border of the crystalline lens superotemporally with broken zonules and lens coloboma in inferotemporal quadrant with absent zonules. There was advanced optic nerve head cupping in both eyes. This lens coloboma is likely an acquired condition due to extensive stretching of the lens and zonules secondary to globe enlargement in neglected buphthalmos. CONCLUSION: A neglected case of congenital glaucoma can lead to lens subluxation along with lens coloboma.

Decompression retinopathy following nonpenetrating deep sclerectomy for primary congenital glaucoma.

BACKGROUND: To describe a unique case of decompression retinopathy manifesting as pre-macular subhyaloid hemorrhage that occurs in a nine-day old child after undergoing a non-penetrating deep sclerectomy for primary congenital glaucoma. CASE PRESENTATION: We report a single case of a 9-day-old boy who was referred to our department of ophthalmology for bilateral buphtalmia and corneal edema. He presented marked elevation of the intraocular pressure in both eyes (22 mmHg and 26 mmHg, in the right eye and left eye respectively) associated with significant optic nerve cupping. Non-penetrating deep sclerectomy was performed for each eye, with effective reduction of the intraocular pressure during the first week postoperatively (11 mmHg and 7 mmHg in the right eye and left eye respectively). The right eye presented an isolated subhyaloid hemorrhage located in the pre-macular area, persisting 3 weeks after the initial surgery and requiring pars-plana vitrectomy to clear the visual axis. This uncommon complication was identified as decompression retinopathy. The intraocular pressure remained controlled in the normal range three years after initial surgery in both eyes, with reversal of optic disc cupping. CONCLUSIONS: Decompression retinopathy is a potential complication after non-penetrating deep sclerectomy in primary congenital glaucoma, requiring prompt treatment strategy to prevent potential organic amblyopia.

Macular hemorrhage after roller coaster riding in a single-eyed patient with congenital glaucoma / Hemorragia macular após passeio em montanha russa em paciente monocular com glaucoma congênito

ABSTRACT A 21-year-old female presented with a 4-day history of decreased vision in her only functional eye (right eye, OD). She had a history of multiple ocular surgeries in both eyes because of congenital glaucoma and had lost light perception in her left eye several years prior. Ophthalmological examination revealed 0.15 Snellen visual acuity, and fundoscopy revealed nearly total cupping and pallor of the optic disc and multiple retinal hemorrhagic foci in the macula in OD. Lesions spontaneously resolved over a few months. Gravitational forces during a roller coaster ride may have caused this macular hemorrhage.

RESUMO Uma paciente de 21 anos de idade se apresentou com perda de visão há quatro dias em seu único olho com visão útil. Ela tinha uma história de cirurgias oculares múltiplas nos dois olhos devido a um glaucoma congênito e perda de percepção luminosa em olho esquerdo há muitos anos. O exame oftalmológico revelou acuidade visual de Snellen de 0,15 e na fundoscopia foi observada escavação do nervo óptico quase total e palidez de papila, assim como focos hemorrágicos múltiplos na região macular. As lesões se resolveram espontaneamente em alguns meses. Acreditamos que essas hemorragias maculares tenham sido causadas pelas forças gravitacionais geradas durante o passeio na montanha russa.

Macular hemorrhage after roller coaster riding in a single-eyed patient with congenital glaucoma.

A 21-year-old female presented with a 4-day history of decreased vision in her only functional eye (right eye, OD). She had a history of multiple ocular surgeries in both eyes because of congenital glaucoma and had lost light perception in her left eye several years prior. Ophthalmological examination revealed 0.15 Snellen visual acuity, and fundoscopy revealed nearly total cupping and pallor of the optic disc and multiple retinal hemorrhagic foci in the macula in OD. Lesions spontaneously resolved over a few months. Gravitational forces during a roller coaster ride may have caused this macular hemorrhage.

Long-term Surgical Outcomes of 180-Degree Suture Trabeculotomy in Korean Patients With Primary Congenital Glaucoma.

PURPOSE: To investigate the surgical outcomes of 180-degree suture trabeculotomy in Korean patients with primary congenital glaucoma (PCG). PATIENTS AND METHODS: This retrospective study included 21 eyes of 17 children with PCG who underwent 180-degree suture trabeculotomy as a first surgical procedure. Surgical success was defined as (1) an intraocular pressure (IOP)<22 mm Hg with or without medication; (2) no additional intraocular surgery; and (3) no evidence of progressive optic disc cupping. The main outcome measure was the success rate of 180-degree suture trabeculotomy. Secondary outcome measures were IOP, number of glaucoma medications, and occurrence of complications. RESULTS: Of the 21 eyes included, 18 (85.7%) were classified as having achieved success at the final visit (mean last follow-up or failure time, 54.91±45.68 mo). Median age at surgery was 11.97 months with mean IOP 29.8±9.6 mm Hg, on average of 1.52 medications. The mean IOP at the final visit was 16.9±5.6 mm Hg on an average of 0.38 medications. The surgical success rate of 180-degree suture trabeculotomy at 1, 3, and 5 years was 95.0%, 87.7%, and 78.9%, respectively (Kaplan-Meier analysis). Three eyes underwent second operations and the mean time to failure was 29.39±19.75 months. Hyphema was observed in 10 eyes, but it cleared up within 7.6 days. CONCLUSION: The 180-degree suture trabeculotomy performed for PCG had a significant IOP-lowering effect and caused no remarkable complications.

Long-Term Outcomes of Trabeculectomy Augmented with Mitomycin C Undertaken within the First 2 Years of Life.

PURPOSE: To evaluate the long-term effectiveness and safety of mitomycin C (MMC)-augmented trabeculectomy undertaken within the first 2 years of life for the surgical management of glaucoma. DESIGN: Retrospective, consecutive, noncomparative case series. PARTICIPANTS: All children who underwent MMC-augmented trabeculectomy within 2 years of birth between May 2002 and November 2012. METHODS: The medical records of 40 consecutive eyes of 26 children who underwent surgery by a single surgeon were reviewed. Data collected during routine clinical care were analyzed. MAIN OUTCOME MEASURES: Assessment of clinical outcomes included intraocular pressure (IOP), final visual acuity, bleb morphology, surgical complications (early and late), postoperative interventions, and further glaucoma surgery performed. Surgical success was defined as final IOP of 5 mmHg or more and of 21 mmHg or less, with anti-glaucoma medications (qualified success) and without (complete success), stable ocular dimensions and optic disc cupping, and no further glaucoma surgery (including needling) or loss of light perception. Surgical outcomes were evaluated using Kaplan-Meier life table analysis. RESULTS: Forty eyes of 26 children were studied over a mean follow-up period of 62.8 months. Most cases (80%) were of primary congenital glaucoma after failed goniotomy surgery. Cumulative probabilities of survival at 1, 5, and 7 years were 78%, 67%, and 60%, respectively. Of eyes regarded as successful, 96% (25/26 eyes) had controlled IOP without topical medication and 44% achieved visual acuity of 20/40 or better. In only 1 of the 40 eyes did a cystic avascular bleb develop, with all the other eyes being non-cystic in nature (diffuse and elevated or flat) at final follow-up. Sixty-four percent (9/14 eyes) of cases regarded as failures ultimately underwent glaucoma drainage device implantation. CONCLUSIONS: A contemporary pediatric trabeculectomy technique augmented with MMC is an effective procedure in the management of glaucoma within the first 2 years of life, as shown by the successful long-term outcomes and low incidence of sight-threatening complications. Trabeculectomy after failed goniotomy surgery or as a primary surgical intervention may offer a phakic infant with glaucoma an excellent opportunity to achieve long-term control of IOP without medications and may be associated with optimal visual outcomes.

Phakomatosis Pigmentovascularis Associated With Sturge-Weber Syndrome, Ota Nevus, and Congenital Glaucoma.

Phakomatosis pigmentovascularis (PPV) is a rare congenital malformation syndrome that is characterized by a combination of capillary abnormalities and dermal melanocytosis.We describe 3 cases of PPV combined with bilateral Sturge-Weber syndrome (SWS), Ota nevus, and congenital glaucoma.Case 1 was a 2-year-old boy. Facial port-wine stains distributed along the 3 branches of his trigeminal nerves, which suggested the existence of SWS. Gray-blue patches were spread over the frontal and temporal areas of bilateral face, waist, buttocks, and thigh. Bilateral triangular alopecia was found on the temporal scalp. The diagnosis of Ota nevus was made by the bilateral scleral malanocystosis. Increased intraocular pressure, enlarged cornea, and pathologic optic disc cupping supported the diagnoses of infantile bilateral glaucoma. Case 2 was a 4-year-old boy. Port-wine stains were found on the face along the 3 branches of the trigeminal nerve and distributed along the trunk, arms, and legs. Mongolian spots spread over his frontal and temporal areas of the bilateral face, waist, buttocks, thigh, abdomen, and back. Infantile glaucoma was found in both eyes. Ota nevus were found in the both eyes. Optic coherent tomography (OCT) scans revealed increased thickness of choroid. Case 3 was a 5-year-old boy. Besides Ota nevus and infantile glaucoma in both eyes, color Doppler ultrasonography showed choroidal hemagioma. OCT scan showed increased choroidal thickness. The bilateral triangular alopecia on the child's temporal scalp was similar to that of Case 1. Cases 1 and 2 presented with port-wine stain patches that were consistent with the characteristic manifestation of PPV type IIb. However, the CMTC of Case 3 met the diagnostic criteria for PPV type Vb.Case 1 was treated with trabeculotomies in both eyes. For Cases 2 and 3, surgical interventions were not considered due to the high risks of antiglaucomatous operation complications. We prescribed them antiglaucoma indications.The simultaneously coexistence of PPV with SWS, Ota nevus, and congenital glaucoma is rare. In the clinic, additional detailed examinations and tests of PPV patients to exclude other ocular abnormalities or extraocular involvements are necessary.

Comparison of 360-degree versus traditional trabeculotomy in pediatric glaucoma.

PURPOSE: To compare intraocular pressure (IOP) control of pediatric glaucoma patients undergoing traditional trabeculotomy (<360 degrees or partial) with those receiving 360-degree circumferential trabeculotomy. METHODS: The medical records of pediatric glaucoma patients receiving trabeculotomy at a single institution from 2000 to 2012 were retrospectively reviewed. Patients were divided into two groups: a traditional trabeculotomy group and 360-degree trabeculotomy group. IOP at baseline and at 1, 3, 6, and 12 months' follow-up were compared within and each groups. RESULTS: A total of 77 eyes of 56 patients (age at surgery, 1.52 ± 2.68 years) in the traditional group and 14 eyes of 10 patients in the 360-degree group (age at surgery, 0.61 ± 0.42 years) were included. Mean baseline IOP was similar in both groups (traditional, 28.75 ± 8.80 mm Hg; 360-degree, 30.35 ± 6.04 mm Hg; t test; P = 0.43). Mean 1-year IOP was 17.05 ± 5.92 mm Hg in the traditional group and 11.0 ± 2.31 mm Hg in the 360-degree group. At 1-year, the surgical success rate was 58.44% in the traditional group and 85.71% in the 360-degree group; 32 eyes in the former and 2 eyes in the latter required another glaucoma procedure within 1 year for IOP control. For both groups, compared to baseline values, IOP decreased significantly with all postoperative measurements (paired t test, all P < 0.01). The 360-degree group had significantly lower IOP compared to the traditional group at 1-year (t test, P < 0.01). CONCLUSIONS: Both 360-degree and traditional trabeculotomy significantly reduced IOP in children through 1 year's follow-up, although the former procedure shows better 1-year postoperative IOP control, with higher rate of surgical success.

Corneal enlargement without optic disk cupping in children with recessive CYP1B1 mutations.

Corneal enlargement during the first 3 years of life can be a sign of early childhood glaucoma and optic nerve head cupping is a useful confirmatory finding. We report 3 children with corneal enlargement without optic nerve head cupping who had recessive CYP1B1 mutations, the most common identifiable cause of primary congenital glaucoma. One child later developed unilateral Haab striae, still in the absence of optic disk cupping. These cases illustrate that CYP1B1-related corneal changes can occur in young children without visible optic nerve head damage.

Loss of function mutations in the gene encoding latent transforming growth factor beta binding protein 2, LTBP2, cause primary congenital glaucoma.

Glaucoma is a heterogeneous group of optic neuropathies that manifests by optic nerve head cupping or degeneration of the optic nerve, resulting in a specific pattern of visual field loss. Glaucoma leads to blindness if left untreated, and is considered the second leading cause of blindness worldwide. The subgroup primary congenital glaucoma (PCG) is characterized by an anatomical defect in the trabecular meshwork, and age at onset in the neonatal or infantile period. It is the most severe form of glaucoma. CYP1B1 was the first gene genetically linked to PCG, and CYP1B1 mutations are the cause of disease in 20-100% of patients in different populations. Here, we report that LTBP2 encoding latent transforming growth factor beta binding protein 2 is a PCG causing gene, confirming results recently reported. A disease-associated locus on chromosome 14 was identified by performing whole genome autozygosity mapping in Iranian PCG families using high density single nucleotide polymorphism chips, and two disease-segregating loss of function mutations in LTBP2, p.Ser472fsX3 and p.Tyr1793fsX55, were observed in two families while sequencing candidate genes in the locus. The p.Tyr1793fsX55 mutation affects an amino acid close to the C-terminal of the encoded protein. Subsequently, LTBP2 expression was shown in human eyes, including the trabecular meshwork and ciliary processes that are thought to be relevant to the etiology of PCG.

[Diagnosis and treatment of tearing in infancy]. / Larmoiement du nourrisson : conduite à tenir et thérapeutique.

Tearing in infancy is a very frequent symptom, particularly during the first year of life. A definite diagnosis can be made easily, only by questioning the parents about the characteristics and the evolution of the tearing since the birth and with a simple examination. The main causes are congenital nasolacrimal duct obstruction account of his high frequency in infant and congenital glaucoma account of his seriousness.

Progressive optic neuropathy in congenital glaucoma associated with the Sirsasana yoga posture.

The authors describe a case of progressive optic neuropathy in a patient with congenital glaucoma who had routinely practiced the Sirsasana (headstand) yoga posture for several years. Ophthalmic examination included best-corrected visual acuity, anterior segment examination, indirect ophthalmoscopy, ultrasound pachymetry for central corneal thickness, and intraocular pressure before, during, and after maintaining the Sirsasana posture for 5 minutes. Intraocular pressure increased significantly during the Sirsasana posture. Transient elevation in intraocular pressure during yoga exercises may lead to progressive glaucomatous optic neuropathy, especially in susceptible patients with congenital glaucoma.

Heterozygous FOXC1 mutation (M161K) associated with congenital glaucoma and aniridia in an infant and a milder phenotype in her mother.

PURPOSE: To report the genetic basis for congenital glaucoma with clinical aniridia in an infant and a milder phenotype in her mother. METHODS: Prospective case series. RESULTS: An infant girl with almost complete lack of iris tissue was referred and treated for congenital glaucoma. Although the presumed clinical diagnosis was aniridia (On-line Mendelian Inheritance in Man [OMIM] AN2, # 106210), PAX6 sequencing was normal. Examination of the infant's mother was significant for Axenfeld-Rieger malformation (ARM): prominent Schwabe line, subtle iris hypoplasia, iris stands bridging the angle, increased intraocular pressure, and glaucomatous optic nerve cupping. Both parents and the infant underwent diagnostic FOXC1 DNA sequencing. A heterozygous M161K FOXC1 mutation was found in the infant and her mother but not in the father, who had a normal ocular examination. DISCUSSION: The spectrum of intrafamilial phenotypic variation associated with heterozygous FOXC1 mutation can be wide. FOXC1 mutation can be a cause of congenital glaucoma with clinical aniridia. Although such infants resemble the AN2 phenotype, the glaucoma of AN2 due to PAX6 mutation is typically secondary with onset several years after birth.

Cataracts and glaucoma in patients with oculocerebrorenal syndrome.

BACKGROUND: Oculocerebrorenal syndrome is an X-linked recessive hereditary oculocerebrorenal disorder characterized by congenital cataract, mental retardation, and Fanconi syndrome of the proximal renal tubules. Other ocular findings include glaucoma, corneal opacity (keloid), enophthalmos, and hypotonia. OBJECTIVE: To describe the treatment of 7 patients (14 eyes) with bilateral cataracts associated with oculocerebrorenal syndrome. Method Retrospective review. RESULTS: Seven patients with oculocerebrorenal syndrome had visually significant bilateral cataracts detected on their first full ophthalmic examination. All underwent bilateral cataract surgery. The mean age (of 14 eyes) at cataract extraction was 1.25 (median, 1.1) months. Glaucoma diagnosis and treatment were more variable. The mean age (of 11 eyes) at glaucoma diagnosis was 24.1 (range, 0.2-70.0) months: the mean age was 0.2 month (of 4 eyes) when glaucoma was diagnosed before cataract extraction, and the mean age was 37.7 months (of 7 eyes) when glaucoma was diagnosed after cataract extraction. All eyes were followed up for a mean of 100.8 (range, 38-190) months. Main Outcome Measure Treatment of 7 patients (14 eyes) with bilateral cataracts associated with oculocerebrorenal syndrome. CONCLUSIONS: Early identification and surgical removal of cataracts is recommended in patients with oculocerebrorenal syndrome. Despite this, visual acuity results will only rarely be better than 20/70, and nystagmus is likely. Patients should be monitored closely and regularly for changes in intraocular pressure, optic nerve cupping, and refractive error to rule out the development of glaucoma.

[Combined deep sclerectomy and trabeculotomy in congenital glaucoma with complications]. / Kombinierte tiefe Sklerektomie und Trabekulotomie beim komplizierten kongenitalen Glaukom.

BACKGROUND: Lately there has been growing interest in deep sclerectomy as a therapeutic approach for the management of congenital glaucoma.In theory, its pressure-lowering effect is independent of external filtration, which minimizes the risk of failure due to conjunctival or episcleral scarring. In patients who have failed to benefit from previous filtering procedures and have refractory congenital glaucoma,deep sclerectomy could become an alternative. PATIENTS AND METHODS: A 3-year-old girl with refractory congenital glaucoma underwent deep sclerectomy in her left eye. Intraoperatively, percolation through the trabeculodescemetic window was weak, so that the procedure was supplemented by a trabeculotomy. RESULTS: By now, throughout a follow-up period of 20 months, stabilisation of disc cupping and ocular axial length has been achieved without additional glaucoma surgery or medications. CONCLUSIONS: In patients with congenital glaucoma,the combination of deep sclerectomy with trabeculotomy may provide an additional therapeutic approach whenever the prospect of success appears to be limited with deep sclerectomy alone.

Reversal of optic disc cupping after trabeculotomy in primary congenital glaucoma.

BACKGROUND: The phenomenon of reversal of glaucomatous cupping of the optic disc following lowering of the intraocular pressure (IOP) was originally recognized in infants. We evaluated the change in optic disc cupping with normalization of the IOP after trabeculotomy in primary congenital glaucoma and assessed the factors associated with reversal of cupping. METHODS: We reviewed the records of 17 patients (24 eyes) who underwent trabeculotomy between July 1993 and June 1999 and who had been followed for at least 1 year. Surgical success was defined as IOP less than 22 mm Hg without anti-glaucoma medication, stable or reduced optic disc cupping, and lack of further corneal enlargement disproportionate to normal growth. Patients who required more than one surgical procedure to control the IOP and those with cloudy media that precluded documentation of cupping were excluded from analysis. Optic disc cupping was assessed independently before and after surgery by two clinicians. The cup:disc ratio was estimated as the percentage of surface area of the optic disc occupied by cupping in the vertical axis. We accepted a difference of 0.1 or 0.2 in the cup:disc ratio between the two observers in each subjective assessment and used the mean value of the two results for data analysis. If the difference was more than 0.2, the eye was excluded from further study. RESULTS: Of the 17 patients 4 were excluded: 2 because they received antiglaucoma medication to control the IOP postoperatively, 1 because he underwent more than one surgical procedure to control the IOP during follow-up, and 1 owing to disagreement in the assessment of the cup:disc ratio between the two observers. Eighteen eyes of 13 patients were thus included in the analysis. Twelve eyes were from boys and six, from girls. The patients were followed for a mean of 43.2 (standard deviation [SD] 30.4) months (range 12 to 90 months). The mean cup:disc ratios pre- and postoperatively were 0.74 (SD 0.20) and 0.60 (SD 0.21) respectively (p = 0.003). Of the 18 eyes 11 (61.1%) had documented reduction in optic disc cupping. The mean time to stabilization of cupping reversal was 4.8 (SD 2.8) months (range 2 to 12 months). In multivariable analysis the age of the patient at surgery was the only variable significantly associated with reversal of cupping (p = 0.027). The mean age at surgery for the 11 eyes with reduction in cupping was 6.9 (range 3 to 15) months, compared with 23.4 (range 12 to 42) months for the 7 eyes with unchanged cupping. The mean preoperative cup:disc ratio was 0.67 (SD 0.17) in the former group and 0.83 (SD 0.17) in the latter group. Six of the seven eyes with unchanged cupping had advanced cupping. INTERPRETATION: Optic disc cupping can be reversed at an early stage of primary congenital glaucoma following successful reduction of IOP. Younger age at surgery was associated with reversal of cupping.

[Primary congenital glaucoma. Long-term success after goniotrepanation with scleral flap]. / Primäres kongenitales Glaukom. Langzeiterfolg nach primärer Goniotrepanation mit Skleradeckel.

BACKGROUND: The long-term success of primary goniotrephining with scleral flap was evaluated in the treatment of primary congenital glaucoma. PATIENTS AND METHODS: Retrospective analysis was carried out in 31 eyes of 18 patients with primary congenital glaucoma (mean follow-up 63 months, range 10-161). Surgical success was defined by an intraocular pressure less than 18 mmHg without medication, stabilization, or reduction in horizontal corneal diameter and stabilization or reversal of disc cupping. Surgical complications were also noted. RESULTS: In 25 eyes control of the glaucoma was achieved with a single goniotrephining procedure (80.6% success rate). In 31 eyes control was achieved with two goniotrephinings (100% success rate). No serious surgical complications were noted. CONCLUSIONS: Primary goniotrephining with scleral flap is a successful and safe method of primary congenital glaucoma treatment.

[Modern therapeutic approach to congenital abnormalities of the anterior eye segment in pediatric patients]. / Approches thérapeutiques modernes des anomalies congénitales du segment antérieur de l'enfant.

Modern functional and anatomic tests allow a reproducible approach of congenital abnormalities of the anterior segment in pediatric patients. Bidimensional ultrasonography allows to perform accurate IOL determination based on precise localization of the macula. In congenital glaucoma, it allows to evaluate optic disc cupping when retinal biomicroscopy is not possible. Color doppler ultrasonography enables to assess blood flow of intra-ocular vessels (in case of persistence of primitive vitreous) or of retrobulbar vessels (in eyes with congenital glaucoma). Miniaturization has allowed to adapt a great number of devices, such as an automatic refractometer, a tonometer, a retinograph or an angiograph, for the use in patients in prone position. These techniques have allowed to assess the anatomic and functional status of eyes undergoing surgery for congenital cataract, which is based on an anterior and posterior capsulorhexis followed by central vitrectomy, for congenital glaucoma, using modern goniotomy or external trabeculotomy, or for anterior persistence of primitive vitreous treated with membranectomy.

[A case of congenital uveal ectropion with glaucoma]. / Przypadek wrodzonego wywiniecia listka barwnikowego blony naczyniowej z jaskra.

A case of 21-year-old man with a rare congenital syndrome of uveal ectropion with glaucoma is described. The visual acuity was 1.0 with correction cyl. -1.0 axis 100 degrees. The highest intraocular pressure was 55 mm Hg. The optic nerve was damaged with glaucomatous cupping (cup/disc ratio 0.8). The visual field presented adequate few insular scotomas. The glaucoma filtering surgery was performed: goniotrepanatio by Fronimopoulos. After the surgery in one year observation intraocular pressure was about 15 mm Hg. The perimetry showed no increase in optic disc damage. We wanted to present this case because of its rare occurrence and good results of the surgery.