Continuing Education Activity

Dead bag syndrome is a late postoperative complication of cataract surgery that leads to intraocular lens dislocation. This condition is a clinical diagnosis recognized at the later stages of pathology, as there are currently no available screening tests. The etiology of dead bag syndrome is unknown but has been hypothesized to be pathological changes in the capsule, lens epithelial cells, or zonules. Most patients present with visual disturbances, such as decreased visual acuity, diplopia, or light sensitivity, due to lens dislocation. Distinctive clinical findings of this syndrome include a floppy capsular bag that lacks the structural integrity to hold the intraocular lens. Current surgical management includes intraocular lens rescue and repositioning or intraocular lens-bag complex exchange for severe cases of subluxation. Mild cases of dead bag syndrome should be followed closely. 

This activity for healthcare professionals is designed to enhance learners' proficiency in evaluating and managing dead bag syndrome. Participants gain a deeper understanding of the theorized etiology, pathophysiology, epidemiology, evaluation, and treatment options for dead bag syndrome, providing healthcare professionals with the knowledge and tools to identify dead bag syndrome and improve patient care. The activity also highlights the key differences between differential diagnoses to aid in recognizing this condition. Enhanced competence enables healthcare professionals to collaborate within an interprofessional team caring for patients with this condition.

Objectives:

• Identify the signs and symptoms indicative of dead bag syndrome.
• Differentiate dead bag syndrome from other common causes of late intraocular lens dislocation.
• Implement appropriate management strategies for dead bag syndrome, improving individual outcomes.
• Collaborate with other healthcare professionals to manage dead bag syndrome and coordinate a comprehensive ophthalmologic care plan.

Introduction

Dead bag syndrome is a rare cause of spontaneous intraocular lens (IOL) dislocation late after cataract surgery. A distinctive characteristic of dead bag syndrome is the presence of a clear capsule many years postoperatively. Predisposing factors include conditions associated with progressive zonular weakening, such as pseudoexfoliation, uveitis, myopia, connective tissue disorders, a history of vitrectomy, and ocular trauma.[1][2] However, the etiology of dead bag syndrome is still unclear. 

Dead bag syndrome was first identified in the early 2000s and described by Dr. Samuel Masket, who reported IOL dislocation cases where the capsular bag was diaphanous and floppy, unequipped to support the IOL.[3] Patients with dead bag syndrome often present with visual disturbances due to IOL dislocation from the inability to screen for dead bag syndrome. Subluxation may occur either through a defect in the bag's periphery or an in-the-bag dislocation. In contrast to early IOL dislocation, which results from inadequate IOL fixation, dead bag syndrome is suspected to result from zonular weakness that develops as a late postoperative complication.[4][5] Late IOL dislocation occurs at least 3 months after cataract surgery, with reported occurrence between 6 and 12 years after surgery.[6]

Etiology

The exact etiology of dead bag syndrome remains unknown. Current hypotheses revolve around trophic effects between the capsular basement membrane and lens epithelial cells (LECs) comprising the lens capsule. In nonpathologic conditions, the lens capsule is an uninterrupted basement membrane secreted by lens epithelial and fiber cells. To maintain its integrity, the capsule thickens over time due to the deposition of extracellular matrix and capsular components at the basal ends of the cortical lens fibers and LECs.[7][8] LECs are important for capsular development, but whether the condition's etiology arises from the basement membrane or the LECs is unclear.

Leading hypotheses include the following: 

• A problem lies within the capsule, initiating a cycle of LEC damage and capsular degradation.[9][10]
• The problem lies within the LECs, causing degeneration that damages the capsule.
• Late postoperative zonular failure is related to capsule splitting or delamination occurring at the level of zonular attachments.[11]

This syndrome and IOL material or design do not appear to be associated.[12] Capsular structures that provide IOL stability and integrity require further investigation.

Epidemiology

Dead bag syndrome is a rare and poorly understood condition. Thus, few epidemiologic factors have been identified. However, predisposing factors include conditions associated with progressive zonular weakening, such as pseudoexfoliation, uveitis, myopia, connective tissue disorders, history of vitrectomy, and ocular trauma. These factors are not unique to dead bag syndrome but are also linked with other conditions causing zonular weakening. The frequency of dead bag syndrome across different sexes and age groups remains undetermined due to its rarity.

Pathophysiology

The currently proposed mechanism underlying dead bag syndrome is the absence or scarcity of LECs, potentially from prior capsular damage or LEC removal from polishing the capsule during cataract surgery. LEC scarcity may play a role in the pathophysiology of dead bag syndrome. However, the association between this condition and capsular polishing during cataract surgery has not been established. Supporting evidence includes the occurrence of dead bag syndrome being documented in cases where surgeons did not practice LEC removal. In addition, capsular polishing cannot remove all LECs and cannot reach the capsular bag equator, as this region is not readily visible.[13]

Histopathology

Dead bag syndrome differs from other causes of lens subluxation because it lacks capsular evidence of fibrosis or opacification. Histopathological findings from suspected cases show little to no LECs in the capsule. Additional findings include degradation signs, such as capsular thinning or splitting at zonular attachment sites, resulting in weak zonular support (see Image. Lens Capsule Splitting). Werner et al hypothesized that late postoperative zonular failure relates to capsule splitting or delamination at the zonular attachments. These features are considered characteristic of the pathology's late stages.

History and Physical

Dead bag syndrome is a disease spectrum most evident once it has progressed several years after cataract surgery. The condition's clinical progression and early manifestations have not been formally established. Consequently, clinicians cannot recognize patients in the perioperative or postoperative period who are likely to develop dead bag syndrome.

Difficulty in early recognition often leads to dead bag syndrome presenting with visual disturbances due to IOL dislocation. Patients may remain unaware of the condition if the IOL dislocation does not compromise central vision.

The capsular bag may appear dilated and diaphanous with clear anterior and posterior capsules without evidence of fibrosis or opacification on slit-lamp examination. Late postoperative evaluation may often show lens subluxation (see Image. Lens Dislocation).

Evaluation

A detailed medical, ocular, and surgical history should be obtained from the patient. Visual acuity, intraocular pressures, and pupillary examination should be performed. A comprehensive slit-lamp and fundus examination should be performed with a 90-diopter lens to assess the capsular bag. The degree of subluxation should be noted, as this clinical sign guides management. If the clinician prefers diagnostic testing, an anterior segment optical coherence tomography or an ultrasound biomicroscopy may be performed to assess the degree of subluxation of the lens and capsule.

Treatment / Management

Several unanswered questions remain regarding the clinical progression and etiology of dead bag syndrome. Therefore, the pathophysiology of dead bag syndrome results in complicated scenarios that often lead to improvisation in surgical technique and management. Based on the assessment of capsular integrity, IOL type, and biometric characteristics, surgical interventions include IOL rescue and repositioning or IOL-bag complex exchange.[14][15] Cases with mild subluxation require adherence to close follow-up. Meanwhile, cases with severe subluxation may necessitate exchanging a 1-piece IOL for a 3-piece IOL that allows for techniques such as sutureless Yamane transscleral fixation, glued or transscleral sutured posterior chamber IOL fixation. Anterior vitrectomy is recommended to thoroughly remove capsular remnants and is essential to prevent any inflammatory response.[16][17]

Differential Diagnosis

Other common causes of late IOL dislocation include true exfoliation syndrome and pseudoexfoliation syndrome.

True Exfoliation Syndrome

This condition typically affects older patients who have had significant exposure to high heat or infrared radiation. Therefore, the causative factor is due to age-related degeneration. Slit-lamp findings include thin capsule delamination, typically without zonular instability. Histopathological findings include lens capsule splitting, delamination, or schisis with varying amounts of LECs.[18][19]

Pseudoexfoliation Syndrome

Pseudoexfoliation is an age-related condition with a strong genetic component involving dysregulated tissue expression of the lysyl oxidase 1 gene. This condition is characterized by the deposition of fibrillar material in the anterior segment with or without the presence of a capsular contraction and rarely capsular phimosis. Histopathological findings often include fibrous metaplasia of anterior LECs or Soemmerring formation.[20][21]

Prognosis

The prognosis of dead bag syndrome depends on the level of zonular support. Currently, long-term data are insufficient to provide an explicit prognosis for patients with this condition.

Complications

The primary complication of dead bag syndrome is lens dislocation, which can lead to visual disturbances and decreased visual acuity. Dislocations can lead to retinal detachment, bleeding, and inflammation that can result in corneal or macular edema in some cases.

Deterrence and Patient Education

Surgeons may consider including this pathology in the informed consent process if a patient possesses various factors predisposing to its development. Awareness of dead bag syndrome should prompt cataract surgeons to reassess the diagnosis and treatment of patients with late IOL dislocations. Continuity of care and adherence to close follow-up must be emphasized to patients with an established diagnosis to prevent complications from dead bag syndrome. Patients should be aware of any visual disturbances they may experience after cataract surgery. Patients are encouraged to undergo comprehensive ophthalmic examinations to assess and screen for IOL and capsule positioning changes.

Enhancing Healthcare Team Outcomes

Effective communication among team members is crucial for managing dead bag syndrome. Ophthalmologists and optometrists must promptly recognize the symptoms and clinical signs of the condition. A detailed history of visual symptoms must be obtained before examination. Open and clear communication facilitates rapid diagnosis and treatment decisions, preventing errors and ensuring a coordinated response.

Ethical considerations guide care decisions. Informed consent is essential to respect patient autonomy and ensure beneficence and nonmaleficence. Patient preferences are central to decisions, promoting shared decision-making. Education and training keep the team updated on best practices. Ongoing professional development ensures that healthcare practitioners are equipped to manage dead bag syndrome. A patient-centered approach places the patient's well-being and preferences at the forefront of all decisions. An interprofessional healthcare team ensures a comprehensive response, minimizes complications, and prioritizes patient safety and care quality in managing dead bag syndrome.

Review Questions

• Access free multiple choice questions on this topic.
• Click here for a simplified version.
• Comment on this article.

References

1.

Subasi S, Yuksel N, Karabas VL, Yilmaz Tugan B. Late in-the-bag spontaneous IOL dislocation: risk factors and surgical outcomes. Int J Ophthalmol. 2019;12(6):954-960. [PMC free article: PMC6580206] [PubMed: 31236352]

2.

Werner L. The dead bag syndrome. J Cataract Refract Surg. 2022 May 01;48(5):517-518. [PubMed: 35703837]

3.

Culp C, Qu P, Jones J, Fram N, Ogawa G, Masket S, Mamalis N, Werner L. Clinical and histopathological findings in the dead bag syndrome. J Cataract Refract Surg. 2022 Feb 01;48(2):177-184. [PubMed: 34261985]

4.

Ascaso FJ, Huerva V, Grzybowski A. Epidemiology, Etiology, and Prevention of Late IOL-Capsular Bag Complex Dislocation: Review of the Literature. J Ophthalmol. 2015;2015:805706. [PMC free article: PMC4698990] [PubMed: 26798506]

5.

McKellar MJ, Elder MJ. The early complications of cataract surgery: is routine review of patients 1 week after cataract extraction necessary? Ophthalmology. 2001 May;108(5):930-5. [PubMed: 11320024]

6.

Kristianslund O, Dalby M, Drolsum L. Late in-the-bag intraocular lens dislocation. J Cataract Refract Surg. 2021 Jul 01;47(7):942-954. [PubMed: 33750091]

7.

Yan Q, Blake D, Clark JI, Sage EH. Expression of the matricellular protein SPARC in murine lens: SPARC is necessary for the structural integrity of the capsular basement membrane. J Histochem Cytochem. 2003 Apr;51(4):503-11. [PubMed: 12642629]

8.

Liu W, Huang D, Guo R, Ji J. Pathological Changes of the Anterior Lens Capsule. J Ophthalmol. 2021;2021:9951032. [PMC free article: PMC8113000] [PubMed: 34055399]

9.

Shentu XC, Zhu YN, Gao YH, Zhao SJ, Tang YL. Electron microscopic investigation of anterior lens capsule in an individual with true exfoliation. Int J Ophthalmol. 2013;6(4):553-6. [PMC free article: PMC3755321] [PubMed: 23991396]

10.

Thakur A, Bansal M, Challa D, Malhotra C, Jain AK. Dead bag syndrome-in the capsular bag subluxated intraocular lens centration and refixation: A new technique. Indian J Ophthalmol. 2023 Oct;71(10):3412-3414. [PMC free article: PMC10683701] [PubMed: 37787245]

11.

Danysh BP, Duncan MK. The lens capsule. Exp Eye Res. 2009 Feb;88(2):151-64. [PMC free article: PMC2674021] [PubMed: 18773892]

12.

Sumioka T, Werner L, Yasuda S, Okada Y, Mamalis N, Ishikawa N, Saika S. Immunohistochemical findings of lens capsules obtained from patients with dead bag syndrome. J Cataract Refract Surg. 2024 Aug 01;50(8):862-867. [PubMed: 38711217]

13.

Darian-Smith E, Safran SG, Coroneo MT. Comment on: Clinical and histopathological findings in the dead bag syndrome. J Cataract Refract Surg. 2022 Jul 01;48(7):871-872. [PubMed: 35514046]

14.

Cooke CA, Lum DJ, Wheeldon CE, Teoh H, McGhee CN. Surgical approach, histopathology, and pathogenesis in cataract associated with true lens exfoliation. J Cataract Refract Surg. 2007 Apr;33(4):735-8. [PubMed: 17397753]

15.

Wong AL, Chan TC, Fong AH, Lam BN, Yuen HK. Clinical characteristics and surgical outcomes of phacoemulsification in true exfoliation syndrome. J Cataract Refract Surg. 2014 Jan;40(1):82-6. [PubMed: 24238944]

16.

Darian-Smith E, Safran SG, Coroneo MT. Lens Epithelial Cell Removal in Routine Phacoemulsification: Is It Worth the Bother? Am J Ophthalmol. 2022 Jul;239:1-10. [PubMed: 35081415]

17.

Fernández-Buenaga R, Alio JL, Pérez-Ardoy AL, Larrosa-Quesada A, Pinilla-Cortés L, Barraquer R, Alio JL, Muñoz-Negrete FJ. Late in-the-bag intraocular lens dislocation requiring explantation: risk factors and outcomes. Eye (Lond). 2013 Jul;27(7):795-801; quiz 802. [PMC free article: PMC3709407] [PubMed: 23764989]

18.

Senthilkumar VA, Krishna MS, Krishnadas R. True exfoliation with double delamination of the anterior lens capsule. Indian J Ophthalmol. 2020 May;68(5):919. [PMC free article: PMC7350437] [PubMed: 32317488]

19.

Karp CL, Fazio JR, Culbertson WW, Green WR. True exfoliation of the lens capsule. Arch Ophthalmol. 1999 Aug;117(8):1078-80. [PubMed: 10448754]

20.

Olawoye OO, Pasquale LR, Ritch R. Exfoliation syndrome in sub-Saharan Africa. Int Ophthalmol. 2014 Oct;34(5):1165-73. [PubMed: 24844849]

21.

Liu E, Cole S, Werner L, Hengerer F, Mamalis N, Kohnen T. Pathologic evidence of pseudoexfoliation in cases of in-the-bag intraocular lens subluxation or dislocation. J Cataract Refract Surg. 2015 May;41(5):929-35. [PubMed: 25935341]

Disclosure: Majid Moshirfar declares no relevant financial relationships with ineligible companies.

Disclosure: Kayvon Moin declares no relevant financial relationships with ineligible companies.

Disclosure: Yasmyne Ronquillo declares no relevant financial relationships with ineligible companies.