Citation network analysis on keratoconus

Objective: The high incidence of keratoconus has caused its management, etiology, and pathogenesis to be controversial topics in the ophthalmology field. This study aims to analyze the relationship between the different publications and authors through citation networks, as well as to identify the research areas and determine the most cited article. Methods: The search for publications was carried out through the Web of Science database, using the term “Keratoconus” between 1900 and December 2022. The Citation Network Explorer and CiteSpace software were used for the publication analysis. Results: 9,655 publications were found, with 124,379 citations generated on the network. The year with the highest number of publications was 2021. The most cited publication was “Keratoconus” by Rabinowitz, published in 1998. Cluster function gave five groups of research areas about keratoconus: corneal signs and parameters, cross-linking efficiency and effects, clinical factors, keratoplasty, and treatment. Conclusions: The citation network offers an objective and comprehensive analysis of the papers on keratoconus. (AU)

Large-diameter deep anterior lamellar keratoplasty: A narrative review.

Deep anterior lamellar keratoplasty (DALK) involves the selective replacement of diseased corneal stroma while preserving healthy unaffected endothelium. While DALK has failed to gain widespread popularity, improved visual and refractive outcomes of large-diameter grafts, which patients directly perceive following suture removal, may represent a compelling reason to shift toward DALK. Since the unaffected host endothelium is retained, DALK offers the opportunity to use large-diameter grafts, which reliably achieves maximum visual potential without an increased risk of immune-mediated stromal rejection. In this narrative review, we evaluate the current evidence on large-diameter DALK including surgical technique and clinical outcomes.

Main issues in deep anterior lamellar keratoplasty: A systematic narrative review.

Deep anterior lamellar keratoplasty (DALK) has emerged as a transformative approach in managing corneal pathologies, notably keratoconus (KC), providing a viable alternative to penetrating keratoplasty (PK). This systematic review explores the intricacies of DALK, comparing its preoperative, intraoperative, and postoperative considerations with PK. Extensive literature searches revealed a wealth of data regarding DALK's advantages and challenges, with an emphasis on graft survival, visual outcomes, and complications. In the preoperative phase, DALK showcases its versatility, catering to a wide spectrum of patients, including those with KC and ocular surface disorders. Intraoperatively, it offers innovative techniques to address emphysema, bubble formation, and Descemet's membrane perforation, all while maintaining a strong focus on patient-centered outcomes. Postoperatively, DALK's lower rejection rates and decreased complications underscore its potential superiority over PK, although unique challenges such as graft failure from nonimmunologic factors demand vigilant management. This comprehensive review not only serves as a valuable resource for ophthalmic surgeons but also sheds light on the evolving landscape of corneal transplantation, highlighting DALK's role as a transformative force in the field.

Pre-Descemet's endothelial keratoplasty with glued intraocular lens implantation with pinhole pupilloplasty in a case of ocular comorbidity in achromatopsia.

Ocular comorbidities can happen as congenital defective gene associations. We present a 37-year-old female patient who was mentally challenged and had coexisting achromatopsia gene abnormality on genetic analysis. She was operated in childhood for congenital cataract, and posterior chamber intraocular lens (IOL) was implanted at 10 years of age elsewhere. The patient presented 27 years later with luxated IOL with endothelial decompensation. There was a coexisting steep and thin cornea noted on corneal topography. She was managed with pre-Descemet's endothelial keratoplasty with transpositioning of posterior chamber IOL to glued IOL with single-pass four-throw pupilloplasty. Postoperatively, the cornea was clear with centered glued IOL. The lesser postanesthetic challenges and faster rehabilitation are obtained in combination procedures with reduced complications in such rare scenarios.

Gentian violet (GV) ink associated reaction in a case of preloaded Descemet membrane endothelial keratoplasty: Case report.

Purpose: This report describes a case of Descemet membrane endothelial keratoplasty (DMEK) graft failure after non-clearing bullae over the area of the orientation mark on the graft. Methods: Case report. Results: The summary of the clinical presentation and workup is described, followed by a brief overview of the DMEK procedure and the gentian violet (GV) ink used to ensure the correct orientation of the DMEK graft. Conclusions: GV has a good safety profile; however, there are rare cases of adverse events. Therefore, alternative approaches should be explored, such as the use of intraoperative optical coherence tomography, reviewing a video recording of the insertion step, adjusting the insertion technique, or using asymmetrical trephine marking on graft edges.

Replace or Regenerate? Diverse Approaches to Biomaterials for Treating Corneal Lesions.

The inner structures of the eye are protected by the cornea, which is a transparent membrane exposed to the external environment and subjected to the risk of lesions and diseases, sometimes resulting in impaired vision and blindness. Several eye pathologies can be treated with a keratoplasty, a surgical procedure aimed at replacing the cornea with tissues from human donors. Even though the success rate is high (up to 90% for the first graft in low-risk patients at 5-year follow-up), this approach is limited by the insufficient number of donors and several clinically relevant drawbacks. Alternatively, keratoprosthesis can be applied in an attempt to restore minimal functions of the cornea: For this reason, it is used only for high-risk patients. Recently, many biomaterials of both natural and synthetic origin have been developed as corneal substitutes to restore and replace diseased or injured corneas in low-risk patients. After illustrating the traditional clinical approaches, the present paper aims to review the most innovative solutions that have been recently proposed to regenerate the cornea, avoiding the use of donor tissues. Finally, innovative approaches to biological tissue 3D printing and xenotransplantation will be mentioned.

Development of acute hydrops in eye with infectious keratitis: A case report.

Purpose: To determine the characteristics of an eye that developed acute hydrops while being treated for infectious keratitis. Observation: A 35-year-old man presented with pain and blurred vision in his left eye. He had undergone cataract surgery seven years earlier and was being treated for poorly controlled atopic dermatitis. The decimal best-corrected visual acuity (BCVA) of the left eye was 0.01. Slit-lamp microscopy showed conjunctival injection, corneal opacification, and a corneal ulcer. The patient was diagnosed with infectious keratitis and was treated with topical and systemic antibiotics. During the hospitalization, the patient was noted to rub his eyes frequently and vigorously. Five days after the first visit, the cornea protruded markedly, and the stroma surrounding the ulcerated area was edematous. These findings led to a diagnosis of acute hydrops.Penetrating keratoplasty was performed to prevent corneal perforation. Histopathological study of the excised cornea showed stromal edema, infiltration of leukocytes, and a tear in Descemet's membrane. Unfortunately, the patient developed endophthalmitis the day after the surgery. The anterior chamber was irrigated with antibiotics, and antibiotics were also injected into the vitreous. The endophthalmitis gradually subsided, and two years after the surgery, the patient's decimal BCVA had improved to 0.6. Conclusion and importance: Vigorous eye rubbing in cases of infectious keratitis can induce acute hydrops, and timely surgical intervention is recommended.

Descemet Membrane Detachment Triggered by Contrast-Enhanced Computed Tomography in a Patient with a History of Penetrating Keratoplasty for Keratoconus Treatment.

Introduction: We report a case of late-onset Descemet membrane detachment triggered by contrast-enhanced computed tomography more than 30 years after penetrating keratoplasty for keratoconus and describe its successful treatment with air tamponade. Case Presentation: A 53-year-old woman with a history of uneventful penetrating keratoplasty more than 30 years ago for keratoconus presented with acute vision loss caused by corneal edema 2 days after undergoing contrast-enhanced computed tomography. Anterior-segment optical coherence tomography (AS-OCT) revealed corneal stromal edema in the graft and Descemet's fold and partial Descemet membrane detachment without a tear. The patient received 0.1% betamethasone eye drops once every hour, along with sub-Tenon's triamcinolone acetonide injection. Anti-inflammatory treatment improved corneal edema; however, the detachment area widened. Air tamponade facilitated complete Descemet membrane reattachment and improved corneal thickness with complete restoration of visual acuity. Conclusion: An immune response may have been involved in the progression of Descemet membrane detachment in this patient. Anti-inflammatory treatment may have facilitated Descemet membrane reattachment prior to air or gas tamponade. AS-OCT is an excellent imaging modality to detect Descemet membrane detachment in eyes with presumed late penetrating graft rejection or failure.

Open-source, 3D printable IOL holder for detailed, smartphone-based anterior segment photography.

Smartphones are increasingly relevant resources in medical practice as they are ubiquitous and reasonably cheap. Among the advantages of using smartphones in medical practise, there is the possibility of obtaining reproducible photographic documentation of various conditions. This is particularly true in the ophthalmic field, where anterior segment color photography plays a significant role in the diagnosis and the management of ocular surface diseases. Here we propose an original design for an open-source smartphone accessory for taking and sharing high-definition photographs of the anterior segment. It can be easily reproduced via 3D printing, and it only needs to be integrated with an intraocular lens (IOL), widely available to the majority of ophthalmologists. Compared to other solutions described previously, it allows a precise and reproducible placement of the IOL on the smartphone camera, avoiding manual positioning that could result tricky and time-consuming. The IOL holder is cheap, scalable, portable and it can be quickly assembled and disassembled, without permanently modifying the smartphone camera.

[Effect of keratoplasty on the quality of life of keratoconus patients]. / Effet de la kératoplastie sur la qualité de vie des patients atteints de kératocône.

Corneal deformations caused by keratoconus produce high levels of optical aberration (OA). Despite appropriate optical correction, these alter the quality of vision and diminish the patient's quality of life, especially since the affected population is predominantly young and of working age. When thinning is too severe or corneal transparency too impaired, a corneal transplant may be considered. In this study, we compare the quality of life of patients with keratoconus in the early (stages 1 and 2) or advanced (stages 3 and 4) stages of the Krumeich classification, as well as patients who have had keratoconus treated by keratoplasty. Quality of life was assessed using the NEI-VFQ 25 questionnaire, the most widely used for keratoconus. An aberrometric examination (OQAS®; HD Analyser, Visiometrics, Terrassa, Spain) was also performed to assess patients' quality of vision. Our results show that keratoplasty provides an improvement in quality of life compared with advanced-stage keratoconus in the areas of distance (p=0.0083) and near vision (p=0.029) activities. This improvement also applies to Best-Corrected Visual Acuity (BCVA) (p=0.032) and transparency (OSI) (p=0.049). Our study shows that keratoplasty improves corneal transparency, and it is interesting to note that it improves patients' quality of life over the long term.

The surgical management of a patient with Fuchs endothelial dystrophy and cataracts.

Objective: To report the two different surgical approaches in the case of a patient with Fuchs endothelial dystrophy with low endothelial cell count and advanced cataracts. Methods: The chosen surgical approach differed between eyes, with the right eye undergoing a combined approach consisting of cataract surgery, intraocular lens implantation, and penetrating keratoplasty in 2022. One year later, for the left eye, a different approach was decided: cataract surgery followed by Descemet membrane endothelial keratoplasty (DMEK). The Descemet membrane graft was prepared by the surgeon using the liquid bubble technique. AS-OCT was used to monitor the patient before and after surgery. Results: Visual recovery was excellent for both eyes, however, visual acuity improved quickly in the left eye (DMEK), while, in the right eye (PK), the best corrected visual acuity was reached after several months post-surgery. Conclusion: Advanced stages of Fuchs dystrophy patients will most likely need corneal transplantation. Each type of corneal transplantation procedure comes with unique challenges, both intraoperative and postoperative. DMEK is a very good treatment option for patients with Fuchs endothelial dystrophy, with excellent visual recovery and good graft survival at the 10-year mark. Abbreviations: DMEK = Descemet membrane endothelial keratoplasty, PK = penetrating keratoplasty, AS-OCT = anterior segment optical coherence tomography, FECD = Fuchs endothelial corneal dystrophy, BCVA = best corrected visual acuity, US = ultrasound, CDE = cumulative dissipated energy, IOL = intraocular lens.

Femtosecond thin-flap laser assisted in situ keratomileusis for correction of post-penetrating keratoplasty ametropia: long-term outcome.

PURPOSE: To evaluate the long-term clinical outcomes of femtosecond thin-flap LASIK (femto-LASIK) for correction of refractive error after penetrating keratoplasty in keratoconus-affected eyes. SETTING: a private ophthalmology clinic. DESIGN: Prospective interventional case series. METHODS: This prospective interventional case series enrolled 22 eyes of 22 patients who underwent femto-LASIK for the management of post-penetrating keratoplasty ametropia. The refractive error, uncorrected (UDVA), and corrected (CDVA) distance visual acuities and vector analysis were reported in short-term and long-term period after surgery. RESULTS: The mean age was 32.7 ± 7.5 years (range, 23 to 47 years) at the surgery time. The average time between PK and femto-LASIK was 42.5 ± 31.7 months. The average follow-up duration after femto-LASIK was 81.2 ± 18.6 months. The mean preoperative UDVA significantly improved from 0.47 ± 0.15 logMAR to 0.35 ± 0.14 logMAR at 12 months (P = 0.048) and 0.4 ± 0.17 at final follow-up exam (P = 0.007). CDVA was 0.22 ± 0.1 at baseline which improved to 0.18 ± 0.15 and 0.15 ± 0.1 logMAR at 12 and 81 months, respectively. (Ps = 0.027, 0.014). The mean cylinder before surgery was - 5.04 ± 1.4D which significantly decreased to -1.5 ± 0.8 D at 12 months postoperatively. (P < 0.001). There was a significant increase in refractive astigmatism from 12 months to 81 months postoperatively (-3.1 ± 2.0, P = 0.002). At the final visit, the efficacy index was 0.83, and the safety index was 1.16. CONCLUSIONS: Despite the short-term outcome indicated that femo-LASIK was effective for correction of post-keratoplasty ametropia during short-term period, a notable regression in its effect was observed in the long-term follow-up. Therefore, the predictability of this technique might decrease in the long-term.

Main issues in penetrating keratoplasty.

This review explores contemporary challenges in penetrating keratoplasty (PK), focusing on technical intricacies, technological advancements, and strategies for preventing graft rejection. A systematic literature search from January 2018 to July 2023 was conducted across PubMed, Cochrane, Web of Science, Scopus, and EMBASE. The inclusion criteria comprised studies on PK and its comparison with other corneal pathologies, with emphasis on keratoconus (KC). Two independent reviewers screened studies, extracting relevant data. The review covers PK evolution, highlighting infra-red femtosecond lasers' impact on graft shapes, minimizing astigmatism, and enhancing wound healing. Graft rejection, a primary complication, is examined, detailing risk factors and preventive measures. Preoperative considerations, diagnostic techniques for rejection, and PK in KC are discussed. Postoperative care's significance, including intraocular pressure monitoring and steroid administration, is emphasized. The paper concludes with a comprehensive approach to prevent graft rejection, involving topical and systemic medications. An outlook on evolving monoclonal antibody research is presented. As the field progresses, personalized approaches and ongoing therapeutic exploration are expected to refine strategies, enhancing PK outcomes.

Phakic anterior chamber intraocular lens removal with simultaneous posterior chamber phakic intraocular lens implantation and Descemet membrane endothelial keratoplasty.

The purpose of this study was to describe a case and clinical course of simultaneous anterior chamber phakic intraocular lens (AC-pIOL) removal, implantation of a posterior chamber phakic intraocular implantable collamer lens (ICL), and Descemet membrane endothelial keratoplasty (DMEK). This was a case report of a 44-year-old male with a unilateral decompensated cornea from a displaced Duet-Kelman lens on his left eye that underwent pIOL extraction and implantation of a posterior chamber phakic intraocular ICL and simultaneous DMEK. After 6 months, the cornea of the left eye had regained clarity, the Descemet membrane was graft stable, and the ICL implanted was centered with good vault. Postoperative anisometropia was avoided. There was no development of cataracts or other complications. The simultaneous pIOL extraction, ICL implantation, and DMEK in our case showed good results with full restoration of anterior segment anatomy and return of transparency of the cornea. The current case shows the feasibility of the simultaneous approach instead of sequential as an alternative for patients with endothelial disease associated with AC pIOLs, restoring vision and anatomy and also avoiding postoperative anisometropia.

Phacoemulsification Wound Burn and Its Management.

Introduction: The production of ultrasonic energy during phacoemulsification is associated with heat generation that could damage ocular tissues, particularly at the corneoscleral wound site. Case Presentation: This study presents an 89-year-old patient with senile hypermature cataract and Fuchs endothelial dystrophy developing severe thermal corneoscleral injury during phacoemulsification. At presentation, visual acuity was finger count at 40 cm and there was a 1 × 2-mm area of corneal melting at the corneal tunnel with diffuse corneal oedema. After 1 month, a temporal circular corneal patch graft was applied to the corneal burn. A penetrating keratoplasty was performed 16 months after the first surgery. Conclusion: Corneal surgery, including lamellar patch grafts and full-thickness penetrating grafts, could be used, when necessary, to restore the cornea's integrity. These procedures could eliminate corneal scarring, decrease astigmatism, and improve vision in patients with phacoemulsification burns.

An injectable thermoresponsive-hydrogel for lamellar keratoplasty: In-situ releases celastrol and hampers corneal scars.

Corneal stromal fibrosis is a common cause of visual impairment resulting from corneal injury, inflammation and surgery. Therefore, there is an unmet need for inhibiting corneal stromal fibrosis. However, bioavailability of topical eye drops is very low due to the tear and corneal barriers. In situ delivery offers a unique alternative to improve efficacy and minimize systemic toxicity. Herein, a drug delivery platform based on thermoresponsive injectable hydrogel/nano-micelles composite with in situ drug-controlled release and long-acting features is developed to prevent corneal scarring and reduce corneal stromal fibrosis in lamellar keratoplasty. The in-situ gelation hydrogels enabled direct delivery of celastrol to the corneal stroma. In vivo evaluation with a rabbit anterior lamellar keratoplasty model showed that hydrogel/micelles platform could effectively inhibit corneal stromal fibrosis. This strategy achieves controlled and prolonged release of celastrol in the corneal stroma of rabbit. Following a single corneal interlamellar injection, celastrol effectively alleviated fibrosis via mTORC1 signal promoting autophagy and inhibiting TGF-ß1/Smad2/3 signaling pathway. Overall, this strategy demonstrates promise for the clinical application of celastrol in preventing corneal scarring and reducing corneal stromal fibrosis post-lamellar keratoplasty, highlighting the potential benefits of targeted drug delivery systems in ocular therapeutics.

Re-Descemet Membrane Endothelial Keratoplasty (DMEK) con preservación del injerto original tras free roll en cámara anterior: a propósito de un caso / Re-descemet membrane endothelial keratoplasty (DMEK) with preservation of the original graft after free-roll in the anterior chamber: a case report

Introducción: Las queratoplastias lamelares han supuesto un gran impacto en el manejo del edema corneal por disfunción endotelial. Las técnicas de trasplante mínimamente invasivo como la Descemet Membrane Endothelial Keratoplasty (DMEK) han permitido reducir la morbilidad que suponía la realización de una queratoplastia penetrante en este tipo de pacientes. Aun así, se trata de técnicas complejas que no están exentas de complicaciones, y que requieren una larga línea de aprendizaje quirúrgico y una aún más exigente experiencia en el manejo postoperatorio.Caso clínicoUna mujer de 89 años afecta de distrofia endotelial de Fuchs e intervenida de cirugía combinada de catarata y DMEK, presentó a las 24h de la intervención un edema estromal de predominio inferior y un despegamiento sectorial del injerto. Tras un re-bubbling en consultas y 4 días más tarde, se observó el injerto enrollado y libre en cámara anterior.Se intervino de re-DMEK con preservación del injerto original tras 24h, con desepitelización para optimizar la visualización. Se tiñó el injerto con azul tripán y se protegió el estroma posterior con aire. Se reimplantó el injerto bajo maniobras intraoculares y con burbuja de aire.A las 24h de la cirugía se observó el injerto adherido, con una gran disminución del edema estromal. Un mes después, la paciente presentaba una córnea transparente, una persistente adhesión completa del injerto y una agudeza visual de 0,9.ConclusiónEl hallazgo del free roll en cámara anterior tras cirugía de DMEK constituye la forma más compleja de despegamiento del injerto. El edema corneal, así como la disposición de las diferentes estructuras intraoculares son condicionantes a tener en cuenta para la resolución quirúrgica de esta complicación. En muchos casos el reposicionamiento quirúrgico del injerto es factible, hecho que implica ahorrar costes sin necesidad de utilizar nuevos tejidos corneales donantes. (AU)

Introduction: Lamellar keratoplasties have had a great impact in the management of corneal edema due to endothelial dysfunction. Minimally invasive transplant techniques such as descemet membrane endothelial keratoplasty (DMEK) have helped to reduce the morbidity involved in performing penetrating keratoplasty in this type of patient. Even so, these are complex techniques that are not free of complications and require a long line of surgical learning and an even more demanding experience in postoperative management.Clinical caseAn 89-year-old woman suffering from Fuchs endothelial dystrophy and undergoing combined cataract and DMEK surgery presented stromal edema predominantly inferior and sectoral detachment of the graft 24h after the intervention. After re-bubbling in consultations and 4 days later, the graft was observed rolled and free in the anterior chamber.She underwent re-DMEK with preservation of the original graft after 24h, with de-epithelialization to optimize visualization. The graft was stained with trypan blue and the posterior stroma was protected with air. The graft was reimplanted under intraocular maneuvers and with an air bubble.Twenty four hours after surgery, the adhered graft was observed, with a great decrease in stromal edema. One month later, the patient had a clear cornea, persistent complete graft adhesion, and visual acuity of 0.9.ConclusionThe discovery of free roll in the anterior chamber after DMEK surgery constitutes the most complex form of graft detachment. Corneal edema as well as the arrangement of the different intraocular structures are conditions to be considered for the surgical resolution of this complication. In many cases, surgical repositioning of the graft is feasible, which means saving costs without the need to use new donor corneal tissues. (AU)

Comparison of therapeutic effects between big-bubble deep anterior lamellar keratoplasty and penetrating keratoplasty for medically unresponsive Acanthamoeba keratitis.

PURPOSE: To compare the outcomes of big-bubble deep anterior lamellar keratoplasty (BB-DALK) and penetrating keratoplasty (PKP) in the management of medically unresponsive Acanthamoeba keratitis (AK). METHODS: This retrospective study included 27 eyes of BB-DALK and 24 eyes of PKP from a tertiary ophthalmology care centre. Glucocorticoid eye drops were subsequently added to the treatment plan 2 months postoperatively based on the evaluation using confocal laser scanning microscopy. The clinical presentations, best-corrected visual acuity (BCVA), postoperative refractive outcomes, graft survival, and Acanthamoeba recurrence were analyzed. RESULTS: The AK patients included in the study were in stage 2 or stage 3, and the percentage of patients in stage 3 was higher in the PKP group (P = 0.003). Clinical presentations were mainly corneal ulcers and ring infiltrates, and endothelial plaques, hypopyon, uveitis and glaucoma were more common in the PKP group (P = 0.007). The BCVA and the graft survival rate showed no statistically significant differences between the two groups at 1 year after surgery. However, 3 years postoperatively, the BCVA of 0.71 ± 0.64 logMAR, the graft survival rate of 89.5%, and the endothelial cell density of 1899 ± 125 cells per square millimeter in the BB-DALK group were significantly better than those of the PKP group (P = 0.010, 0.046, and 0.032, respectively). 3 eyes (11.1%) in the BB-DALK group and 2 eyes (8.3%) in the PKP group experienced Acanthamoeba recurrence, but the rates showed no statistically significant difference between the two groups (P = 1.000). In the PKP group, immune rejection and elevated intraocular pressure were observed in 5 and 6 eyes, respectively. CONCLUSION: Corneal transplantation is recommended for AK patients unresponsive to antiamoebic agents. The visual acuity and graft survival can be maintained after BB-DALK surgery. Acanthamoeba recurrence is not related to the surgical approach performed, whereas complete dissection of the infected corneal stroma and delayed prescribing of glucocorticoid eye drops were important to prevent recurrence.

Herpetic Keratouveitis: Missed Diagnosis Leading to Corneal Perforation.

Herpetic uveitis is an easy diagnosis to miss, which can lead to devastating consequences. The aim of this report is to create awareness of how this disease can present, appropriate clues to the diagnosis, and how it should be managed. We report a case of a 70-year-old female who presented with redness and painless blurry vision in her right eye and was treated with topical corticosteroid drops for presumed idiopathic anterior uveitis. Despite initial symptomatic improvement, she reattended with a significant deterioration in vision and was found to have a large corneal infiltrate and associated perforation. The perforation was sealed with corneal gluing, and she was treated for presumed herpetic anterior uveitis with oral acyclovir. Corneal polymerase chain reaction (PCR) specimen was positive for herpes simplex virus DNA. The perforation started to leak again despite repeat corneal gluing, so an emergency therapeutic penetrating keratoplasty was performed. She has remained on prophylactic oral acyclovir for the last 24 months, with no recurrence and the graft remains clear.

Familial primary calcific band-shaped keratopathy with late onset systemic disease: a case series and review of the literature.

BACKGROUND: Familial calcific band-shaped keratopathy (BSK) is a very rare disease, with no underlying cause. There is no underlying disease in this form of the disease. This article introduces a family with seven children, three of whom were diagnosed with familial primary calcific BSK. One of them developed a systemic disease 38 years after ocular manifestation. CASE PRESENTATION: In this case report, three Iranian siblings from a family with familial calcific band-shaped keratopathy (BSK) are introduced. Systemic and ocular examinations performed on these patients indicated the occurrence of chronic kidney disease in the older child, a 41-year-old woman, 38 years after ocular manifestation. The examinations conducted on the other two siblings revealed no pathological findings. The 41-year-old sister and 37-year-old brother underwent unilateral deep anterior lamellar keratoplasty (DALK), while the 33-year-old sister underwent bilateral superficial keratectomy (SK). CONCLUSION: Considering the late onset of systemic disease in one of the siblings diagnosed with familial calcific band-shaped keratopathy (BSK), it is crucial to emphasize the necessity of long-term follow-up for these patients and their families.