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Case Report: Combined Cataract Surgery and Minimally Invasive Glaucoma Surgery Provide an Alternative Treatment Approach for Lowe Syndrome.

ABSTRACT: We describe the case of a 4-month-old boy who presented with bilateral congenital cataract and high intraocular pressure (IOP) in the left eye, followed by mental retardation and delayed motor development. Genetic investigation revealed the boy had a splicing variant (c.940-11G>A) of the oculocerebrorenal syndrome of Lowe (OCRL) gene. The boy underwent a lensectomy for congenital cataract in his right eye, and lensectomy combined with a 360° suture trabeculotomy to remove the clouded lens and to control IOP of the left eye. During postoperative one-and-a-half-year follow-up, the boy exhibited an improved visual acuity and a well-controlled IOP without the use of topical IOP-lowering medications. Lowe syndrome is a rare multisystemic disorder that is diagnosed through clinical manifestation and genetic testing. The possibility of Lowe syndrome should be considered in patients presenting with typical triad, and genetic analysis should be performed in time to confirm the diagnosis. We recommend combined cataract surgery and minimally invasive glaucoma surgery (MIGS) as a safe, feasible, and efficient method to treat congenital cataract and glaucoma in Lowe syndrome patients.

SUBMITTER: Wang C

PROVIDER: S-EPMC9283680 | biostudies-literature | 2022

REPOSITORIES: biostudies-literature

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Case Report: Combined Cataract Surgery and Minimally Invasive Glaucoma Surgery Provide an Alternative Treatment Approach for Lowe Syndrome.

Wang Chen C Zhang Wenzhe W Wang Leyi L Liu Wenhui W Guo Hui H

Frontiers in medicine 20220701

We describe the case of a 4-month-old boy who presented with bilateral congenital cataract and high intraocular pressure (IOP) in the left eye, followed by mental retardation and delayed motor development. Genetic investigation revealed the boy had a splicing variant (c.940-11G>A) of the oculocerebrorenal syndrome of Lowe (OCRL) gene. The boy underwent a lensectomy for congenital cataract in his right eye, and lensectomy combined with a 360° suture trabeculotomy to remove the clouded lens and to ...[more]

PMID: 35847784

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Project description:BackgroundThe gonio-endoscope is a novel device for use during minimally invasive glaucoma surgery (MIGS) to treat glaucomatous eyes with cloudy cornea. The MIGS procedure requires a surgical gonioprism lens for direct visualization of the angle, intraoperative manipulation of the surgical microscope and the patient's head position, and the patient's eye without a cloudy cornea. In cases with cloudy corneas or limitation of neck movement, MIGS cannot be safely performed. Gonio-endoscopy facilitates clear visualization of the trabecular meshwork (TM) to perform MIGS safely and easily even in a patient with corneal opacities or limitation of neck movement with no additional MIGS procedures. We report the first case in which we performed the newly developed a 10,000-pixel high-resolution 23-gauge gonio-endoscopic operation.Case presentationThe patient was a 58-year-old man with Down syndrome who had secondary glaucoma bilaterally after cataract surgery and long-time use of a steroid for atopic dermatitis and allergic conjunctivitis. His left eye had a cloudy cornea after penetrating keratoplasty for keratoconus with severe corneal residual scarring after prior resolved corneal hydrops. When the intraocular pressure (IOP) in his left eye increased to 41 mmHg despite the maximum use of anti-glaucoma eyedrops, he was referred to our hospital. Anterior-segment optical coherence tomography showed an open angle. We developed a new gonio-endoscope (MACHIDA Endoscope Co., Ltd., Chiba, Japan and NIPRO CORPORATION, Osaka, Japan), the probe of which is bent appropriately to aid visualization of and access to the TM. After obtaining clinical approval from the government and our institution, we could safely perform a high-resolution 23-gauge gonio-endoscopy-assisted microhook ab interno trabeculotomy (μLOT). The IOP decreased to 10 mmHg and the visual acuity has been preserved with no major complications for 1 year postoperatively.ConclusionThis new technique of clear gonio-visualization using a gonio-endoscope might be helpful for a safe and easy μLOT in patients with cloudy corneas. This device can apply to other types of MIGS procedures and cases with pathological diagnoses of glaucoma that are difficult to treat.

Project description:SIGNIFICANCE:Noncompliance is a problem affecting glaucoma patients. Approaches to improve adherence include the use of drug-delivery systems and safer forms of surgery. Minimally invasive glaucoma surgery (MIGS) has reduced complications, particularly in combination with cataract surgery, and with its good intraocular pressure (IOP) reduction may reduce or eliminate glaucoma medications.Glaucoma is a progressive disease and a leading cause of irreversible blindness. Elevated IOP is the most important risk factor, but effective medical management is dependent on patient adherence. This review summarizes the adherence problem in glaucoma and the efforts, including MIGS, to provide effective IOP control that is not dependent on patient compliance.The current understanding of patient adherence to pharmacological treatment of glaucoma is discussed including the challenges facing glaucoma patients. Historical approaches to providing IOP control in a sustained and reliable way are presented culminating in a review of the burgeoning use of MIGS devices.It is estimated that, in the United States, 27% of prescriptions written, across all medications, are not filled or are filled but not taken. For ocular hypotensive medications, even when filled, a large percentage (which varies widely by study) are not instilled as prescribed. To address this problem, methods for sustained drug delivery have been and continue to be developed, as well as surgical and laser approaches. Most recently, MIGS devices have gained popularity because of the ease of implantation during cataract surgery, favorable safety profile, and the possibility for effective and long-lasting IOP lowering, as well as the reduction or elimination of need for IOP-lowering medication.Poor adherence to treatment is relatively common among glaucoma patients and is associated with progression of disease. Recommending MIGS implantation during cataract surgery may offer optometrists a valuable treatment option in managing glaucoma patients, particularly where good adherence is in doubt.

Dataset Information

Case Report: Combined Cataract Surgery and Minimally Invasive Glaucoma Surgery Provide an Alternative Treatment Approach for Lowe Syndrome.

Publications

Case Report: Combined Cataract Surgery and Minimally Invasive Glaucoma Surgery Provide an Alternative Treatment Approach for Lowe Syndrome.

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