Objective: To describe a novel glaucoma drainage device fashioned from a scleral buckle and nasolacrimal silicone tube. Method: This is a case report and a description of a surgical technique. Results: A 6-year-old boy with congenital glaucoma who underwent 2 failed filter surgeries had medically uncontrolled intraocular pressures (IOP). Due to limited funds for a second glaucoma drainage device, the author utilized a portion of a scleral buckle and nasolacrimal silicone tube – the same raw materials used in the Schocket implant – to fashion a novel, non-valved glaucoma shunt that was implanted in the patient’s eye. Post-operatively, IOP was 8-10mmHg. This was sustained for 12 months without the need for supplemental anti-glaucoma medications. Postoperative complications included tube migration resulting in localized corneal decompensation and cataract formation. Conclusion The alternative GDD made from a portion of a scleral buckle and silicone tube may be an effective and economical alternative to the GDDs available in the market.
congenital glaucoma

Glaucoma ; congenital ; physiopathology ; surgery ; Humans ; Intraocular Pressure ; Trabeculectomy ; methods

The contusive anterior chamber angle deformities after traumatic hyphema probably were related to the late onset of glaucoma but have been overlooked easily. Authors analysed clinically the angle deformities of 53 patients with traumatic hyphema at the Department of Ophthalmology, Pusan National University from May 1983 to April 1986 by gonioscopic findings. The results were as follows: 1. Male to female ratio of 53 patients was 7.8:1, The average age was 23.7 years. 2. The traumatic hyphema was caused by missile injuries(58.5%) and blunt traumas(41.5%). 3. Of the 53 patients with traumatic hyphema, 42(79.2%) patients had angle recession and 9 patients had normal angle. 4. The degree of angle recession consisted of Grade I, 19.0%(8 cases), Grade II, 38.1%(16 cases), and Grade III, 42.9%(18 cases). The circumferential extent of angle recession increased proportionally with the degree of angle recession. 5. The circumferential extent of angle recession in the range of 271 degrees ~ 360 degrees was 57.1%, 181 degrees ~ 270 degrees was 23.8%, 91 degrees ~ 180 degrees was 11.9% and under 90 degrees was 7.1% of the patients. 6. There was no significant correlation between the amount of hyphema and the extent and degree of angle recession. 7. The distribution of angle recession was noted 42 cases in superonasal quadrant, 34 cases in superotemporal quadrant, 29 cases in inferotemporal quadrant and 23 cases in infercnasal quadrant and was not correlative to the amount of hyphema. 8. Of the 42 patients with angle recession, 35 patients developed peripheral anterior synechiae, its average extent was under 50 degrees, mostly being at the lower half of anterior chamber angle. 9. The relationship between the intraocular pressure and the degree of angle recession was not significant. 10. Associated ocular abnormalities other than angle abnormalities were iris damage(32.1%), chorioretinal damage(17.0%) and lens damage(7.5%). 11. The chorioretinal damage was the major cause of decreased visual acuity. 12. The chronic simple glaucoma was developed in 2(4.8%) of 42 patients with angle recession.
Anterior Chamber* ; Busan ; Congenital Abnormalities* ; Female ; Glaucoma ; Glaucoma, Open-Angle ; Humans ; Hyphema* ; Intraocular Pressure ; Iris ; Male ; Ophthalmology ; Visual Acuity

The contusive anterior chamber angle deformities after traumatic hyphema probably were related to the late onset of glaucoma but have been overlooked easily. Authors analysed clinically the angle deformities of 53 patients with traumatic hyphema at the Department of Ophthalmology, Pusan National University from May 1983 to April 1986 by gonioscopic findings. The results were as follows: 1. Male to female ratio of 53 patients was 7.8:1, The average age was 23.7 years. 2. The traumatic hyphema was caused by missile injuries(58.5%) and blunt traumas(41.5%). 3. Of the 53 patients with traumatic hyphema, 42(79.2%) patients had angle recession and 9 patients had normal angle. 4. The degree of angle recession consisted of Grade I, 19.0%(8 cases), Grade II, 38.1%(16 cases), and Grade III, 42.9%(18 cases). The circumferential extent of angle recession increased proportionally with the degree of angle recession. 5. The circumferential extent of angle recession in the range of 271 degrees ~ 360 degrees was 57.1%, 181 degrees ~ 270 degrees was 23.8%, 91 degrees ~ 180 degrees was 11.9% and under 90 degrees was 7.1% of the patients. 6. There was no significant correlation between the amount of hyphema and the extent and degree of angle recession. 7. The distribution of angle recession was noted 42 cases in superonasal quadrant, 34 cases in superotemporal quadrant, 29 cases in inferotemporal quadrant and 23 cases in infercnasal quadrant and was not correlative to the amount of hyphema. 8. Of the 42 patients with angle recession, 35 patients developed peripheral anterior synechiae, its average extent was under 50 degrees, mostly being at the lower half of anterior chamber angle. 9. The relationship between the intraocular pressure and the degree of angle recession was not significant. 10. Associated ocular abnormalities other than angle abnormalities were iris damage(32.1%), chorioretinal damage(17.0%) and lens damage(7.5%). 11. The chorioretinal damage was the major cause of decreased visual acuity. 12. The chronic simple glaucoma was developed in 2(4.8%) of 42 patients with angle recession.
Anterior Chamber* ; Busan ; Congenital Abnormalities* ; Female ; Glaucoma ; Glaucoma, Open-Angle ; Humans ; Hyphema* ; Intraocular Pressure ; Iris ; Male ; Ophthalmology ; Visual Acuity

Axenfeld-Rieger syndrome is a rare autosomal dominant inherited developmental disorder characterized by ocular and systemic abnormalities. In ocular anomaly, it includes a prominent and anteriorly displaced Schwalbe line and an iridocorneal synechiae, iris hypoplasia, corectopia and secondary glaucoma. Extraocular developmental abnormalities of the syndrome are hearing loss, congenital heart disease, dental anomalies, developmental delay, and a characteristic facial appearance. We herein reported a familial case of Axenfeld-Rieger syndrome that had a mitral valve prolapse with severe mitral regurgitation finally requiring mitral valve replacement.
Glaucoma ; Hearing Loss ; Heart Defects, Congenital ; Iris ; Mitral Valve ; Mitral Valve Insufficiency* ; Mitral Valve Prolapse

Aniridia or irideremia is characterized by reduction of iris or absence of total iris, but a rudimentary iris is always present in most cases. This congenital anomaly is familial and the transmission is strongly dominant by an autosomal gene. It demonstrates high penetrance and variable expression. Aniridia is mainly due to a primary defect in development of the neural ectoderm and an aberrant development of the mesoderm. Glaucoma and cataract in the eye is frequently complicated with other deformities of the body and the treatment is not satisfactory. The author have experienced a case of aniridic family which is composed of 7 persons. Of these, the father, one son and 3 daughters are affected with aniridia, cataract, nystagmus and amblyopia and the mother and one son are healthy. The author reports this cases with the review of literature.
Amblyopia ; Aniridia* ; Cataract ; Congenital Abnormalities ; Ectoderm ; Fathers ; Glaucoma ; Humans ; Iris ; Mesoderm ; Mothers ; Nuclear Family ; Penetrance

BACKGROUNDGlaucoma is one of the leading causes of blindness in the world. Primary open-angle glaucoma (POAG) and primary congenital glaucoma (PCG) are subtypes of glaucoma. Myocillin is the first gene identified to be involved in POAG. Recently, myocillin mutation has been found in PCG. In this context, we reported a special glaucoma pedigree, which was composed of both PCG and POAG patients, and analyzed the mutation of myocillin in this pedigree.

METHODSThe family was composed of the parents, a son and a daughter. All members of the family underwent the complete ophthalmologic examinations. All coding exons 1 - 3 and flanking introns of myocilin gene were screened for sequence alterations by polymerase chain reaction and direct DNA sequencing.

RESULTSThe son was the proband, who was diagnosed as PCG in both eyes. The father was diagnosed as POAG in the right eye, the left eye was still normal. Both the sister and the mother of the proband had normal intraocular pressure without glaucomatous optic disc changes. The mutations in intron 2 of myocilin gene were detected in the family. While the proband and the father were homozygous, the mother and the sister were heterozygous for the mutation.

CONCLUSIONSHomozygous mutation in intron 2 of myocilin gene is involved in both POAG and PCG. It is suggested that the pathogenesis might be overlapping in POAG and PCG.

Cytoskeletal Proteins ; genetics ; Eye Proteins ; genetics ; Female ; Glaucoma ; congenital ; genetics ; Glaucoma, Open-Angle ; genetics ; Glycoproteins ; genetics ; Humans ; Introns ; Male ; Mutation ; Pedigree

Sturge-Weber syndrome (SWS) is a rare congenital neurocutaneous disorder that causes congenital glaucoma. Previous experiences have shown that drainage procedures are often required to control associated glaucoma. The conventional surgical approach in trabeculectomy carries a significant risk of intraoperative expulsive hemorrhage. Here, we describe a modified approach of the conventional trabeculectomy technique, which may lower the risk of expulsive hemorrhage. A viscoelastic device was employed to maintain a steady intraocular pressure throughout the procedure. Details of the surgical technique and material used are described. One patient with congenital glaucoma associated with SWS underwent a successful trabeculectomy using the modified technique. Postoperative intraocular pressure was successfully reduced and no intraoperative complications occurred. We describe a successful case of trabeculectomy in a SWS case where a modified technique was applied.
Glaucoma/*congenital ; Humans ; Infant, Newborn ; *Intraocular Pressure ; Male ; Sturge-Weber Syndrome/*complications/diagnosis/surgery ; Trabeculectomy/*methods ; Visual Acuity

BACKGROUNDEarly surgical intervention is required for the primary congenital glaucoma (PCG). There are currently few reports on the surgical outcomes in infants with PCG. This study aimed to evaluate the efficacy and safety of trabeculotomy and the postoperative visual outcomes in Chinese newborns with PCG within 4 weeks of birth.

METHODSA total of 21 eyes of 12 patients with PCG who underwent primary trabeculotomy within 4 weeks of birth were retrospectively studied. Preoperative and postoperative intraocular pressure (IOP), corneal clarity and diameter, axial length and optic disc cupping, visual acuity and postoperative refractive error, success rates, and complications were evaluated. Kaplan-Meier survival analysis was applied to evaluate the success rates.

RESULTSThe mean follow-up time was 46.9 ± 34.4 months (range: 12-122 months). The postoperative IOP was significantly lower than the preoperative IOP at all of the follow-up visits (P < 0.001). The complete success rates for all eyes at 1, 2, 3, and 5 years postoperatively were 90.5%, 85.7%, 85.7%, and 85.7%, respectively. The IOPs of the three patients who needed antiglaucomatous medications postoperatively were also well controlled. At the last visit, the cornea became clear, and the cup-to-disc ratio decreased significantly (P = 0.01) although the horizontal corneal diameter did not change significantly (P = 0.11). Visual acuities were able to be recorded in eight eyes at the last visit, among which six eyes had a best-corrected visual acuity of 20/40 or better. There were no severe intraoperative or postoperative complications.

CONCLUSIONSTrabeculotomy proves to be a safe and effective treatment in reducing IOP in this group of Chinese newborns with PCG. The outcomes of vision function were satisfactory in most of the patients.

Child ; Child, Preschool ; Congenital Abnormalities ; surgery ; Female ; Glaucoma ; surgery ; Humans ; Infant ; Male ; Postoperative Complications ; Retrospective Studies ; Trabeculectomy ; Treatment Outcome

Sturge-Weber syndrome is a congenital disorder characterized by facial nevus flammeus along the distribution of the trigeminal nerve, ipsilatal leptomeningioma, buphthalmos, and choroidal angioma. The Authors experienced a case of Sturge-Weber syndrome in 9 year old female patient and glaucoma was controlled by trabecuectomy without any complications.
Child ; Choroid ; Congenital, Hereditary, and Neonatal Diseases and Abnormalities ; Female ; Glaucoma ; Hemangioma ; Humans ; Hydrophthalmos ; Port-Wine Stain ; Sturge-Weber Syndrome* ; Trigeminal Nerve