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<\/p>\nWeill-Marchesani syndrome (WMS) is a rare connective tissue disorder in which ocular morbidity is largely driven by microspherophakia or spherophakia, lenticular myopia, ectopia lentis, cataract, and secondary glaucoma. In many eyes, the crystalline lens is not merely a displaced structure but the anatomical engine of disease, inducing pupillary block, anterior chamber crowding, angle closure, and progressive glaucomatous damage.<\/p>\n
I report the clinical rationale and surgical strategy adopted in a bilateral case of WMS complicated by spherophakia, glaucoma, cataract, high myopia, and lens dislocation, with established glaucomatous optic neuropathy and visual field loss. The patient was managed in two stages, operating first one eye and then the fellow eye a few months later. In both eyes, pars plana vitrectomy and lensectomy were combined with secondary posterior chamber rehabilitation using a scleral-fixated Carlevale intraocular lens.<\/p>\n
The present case is used as the basis for a focused narrative review of the ocular phenotype of WMS and of current surgical options for the management of unstable spherophakic lenses with inadequate capsular support.<\/p>\n
The available literature supports lens removal as a pathogenetically meaningful step in eyes with pupillary block or angle compromise, while modern scleral fixation techniques offer a physiologic method of visual rehabilitation when the capsular-zonular complex is no longer reliable.<\/p>\n
In this setting, the Carlevale lens appears particularly attractive because it provides sutureless self-anchoring scleral fixation in the posterior chamber. The main message of this paper is that, in WMS, successful surgery requires not only removal of a pathologic lens but reconstruction of a more stable anterior and posterior segment anatomy.<\/p>\n
Weill-Marchesani syndrome is a genetically heterogeneous connective tissue disorder associated with variants in genes including ADAMTS10, ADAMTS17, LTBP2, and FBN1. Although the syndrome has recognizable systemic traits such as short stature, brachydactyly, and joint stiffness, the ophthalmic phenotype is often the most vision-threatening component of the disease. Core ocular manifestations include microspherophakia or spherophakia, lenticular myopia, ectopia lentis, glaucoma, and, less frequently, cataract and corneal endothelial compromise. Recent clinical summaries have reported approximate frequencies of myopia in 94% of cases, microspherophakia in 84%, glaucoma in 80%, ectopia lentis in 73%, and cataract in 23%, underscoring the centrality of the lens in the natural history of WMS. [1,2] Bellone WMS case-based review Page 1<\/p>\n
The pathophysiology of glaucoma in WMS is complex. The abnormally spherical lens has increased anteroposterior thickness and reduced equatorial diameter, while zonular weakness allows progressive forward movement of the lens-iris diaphragm. The resulting pupillary block and angle crowding may evolve into peripheral anterior synechiae, trabecular damage, and chronic intraocular pressure elevation. Consequently, glaucoma in these eyes may initially be lens-driven but later become partly independent of lens position once angle damage is established. This explains why laser iridotomy is sometimes insufficient and why lens surgery may be decisive early yet incomplete in advanced disease. [3-7]<\/p>\n
The surgical challenge is therefore dual: first, removing a crystalline lens that is pathologic, unstable, and often responsible for the pressure problem itself; second, deciding how best to restore optical rehabilitation in the absence of dependable capsular and zonular support. This article presents a bilateral staged surgical strategy using pars plana vitrectomy, lensectomy, and scleral fixation of a Carlevale intraocular lens, and discusses its rationale against the background of the current literature. [3,4,8-12]<\/p>\n
I managed a patient affected by Weill-Marchesani syndrome who presented with severe bilateral ocular involvement. The clinical picture included spherophakia, high lenticular myopia, glaucoma, lens dislocation, and cataract. By the time surgery was planned, glaucoma had already begun to damage the optic nerve, with corresponding visual field impairment, making the case not only refractively and anatomically complex but also functionally urgent. In such eyes, the lens must be regarded as more than a coincidental pathology: it contributes directly to anterior segment crowding, pupillary block, and progressive glaucomatous injury. [2,5,6]<\/p>\n
<\/p>\n
Because the disease was bilateral, I deliberately adopted a staged strategy. One eye was operated on first, and the fellow eye was addressed a few months later after confirming the anatomical and functional course of the first procedure. This choice was motivated by prudence and by the unusually hostile anatomy of WMS eyes, where shallow anterior chambers, zonular incompetence, lens instability, glaucoma, and cataract can coexist. Even in the absence of the original surgical videos, the clinical sequence and operative rationale remain clear and, in my view, are the most meaningful scientific elements of the case.<\/p>\n
The preoperative reasoning was straightforward. A capsular bag-based solution was not considered dependable because the capsular-zonular complex was intrinsically compromised by the spherophakic and dislocated lens. An anterior chamber IOL would have placed a prosthesis in the least forgiving compartment of an eye already predisposed to glaucoma and endothelial stress, while an iris-fixated option would have relied on iris tissue in a setting where a posterior chamber solution appeared more physiologic. Accordingly, I opted for pars plana vitrectomy and lensectomy followed by secondary posterior chamber rehabilitation with a scleral-fixated Carlevale lens in both eyes. [3,4,8-12]<\/p>\n
In eyes with marked lens instability or true dislocation, a pars plana route offers several conceptual advantages. It permits controlled removal of the crystalline lens, comprehensive management of the vitreous, reduction of vitreous traction on residual zonular structures, and safer completion of surgery when the lens cannot be reliably approached from the limbus. The literature on microspherophakia consistently Bellone WMS case-based review Page 2 emphasizes that the degree of subluxation or dislocation and the quality of the capsular-zonular apparatus should dictate whether a limbal or pars plana strategy is preferable. [3,4]<\/p>\n
Once lensectomy has been completed and aphakia created, visual rehabilitation becomes the next major decision. In the present case, I selected the Carlevale lens because it was specifically designed for sutureless scleral fixation in eyes without capsular support. Its self-anchoring plugs allow posterior chamber positioning without long-term suture-related concerns and avoid the angle-related drawbacks of anterior chamber lenses. In practical terms, this design is well suited to eyes in which long-term anatomic stability is more important than merely completing the operation. [9-12]<\/p>\n
The goal of surgery was therefore threefold: first, eliminate the lens-induced component of pupillary block and angle crowding; second, remove a cataractous and dislocated lens that had become visually and anatomically harmful; and third, reconstruct a stable posterior chamber optical system. In WMS, this sequence is particularly coherent because it addresses both the mechanism of disease and the need for durable rehabilitation.<\/p>\n
One of the most useful ways to interpret WMS is to recognize that the crystalline lens is central to pathogenesis. High myopia in these patients is often predominantly lenticular rather than axial, and the discrepancy between marked myopia and a non-proportionate posterior segment phenotype should alert the clinician to a lens-based disorder. In the same eye, the spherical lens also reduces anterior chamber depth and mechanically contributes to pupillary block and secondary angle closure. This integrated view of refraction, anatomy, and pressure is essential because it clarifies why lens surgery can be both visually rehabilitative and pressure-modifying. [1,3,5,6]<\/p>\n
The presence of established glaucomatous optic neuropathy and visual field loss in my patient is a critical point. In WMS and related microspherophakic states, delayed recognition may allow repeated or chronic pressure elevation to produce irreversible optic nerve injury. Guo and colleagues described advanced WMS glaucoma with severe optic neuropathy, a cup-to-disc ratio of 0.99, and an extremely narrow tubular visual field, emphasizing how chronically elevated intraocular pressure can translate into permanent structural and functional loss. [6] A more recent review of secondary angle-closure glaucoma in WMS reiterated that prompt diagnosis and treatment are mandatory because glaucoma is the most threatening ocular complication and may progress to irreversible blindness if managed too late. [2]<\/p>\n
This point is also supported by larger spherophakia cohorts. Rao et al. showed that, after lensectomy in 52 eyes with secondary glaucoma, younger age, higher presenting intraocular pressure, and larger cup-to-disc ratio at presentation were significant risk factors for failure. [7] In other words, once glaucomatous damage is advanced, surgery may still be necessary and beneficial, but the ceiling of functional recovery becomes lower. This is directly relevant to the present case, where surgery was undertaken not only to improve anatomy and vision, but also to arrest further glaucomatous loss.<\/p>\n
The current literature strongly favors lens extraction when microspherophakia or spherophakia produces pupillary block, significant subluxation, lens dislocation, intolerable lenticular myopia, cataract, or uncontrolled glaucoma. A systematic review by Venkataraman et al. proposed an integrated decision-making pathway in which the extent of zonular weakness, lens position, degree of angle damage, and severity of glaucoma guide the procedure. [3] In early disease, lens removal alone may be sufficient to reduce the lens-driven component of pressure elevation; in advanced eyes with extensive peripheral anterior synechiae or severe glaucoma, combined glaucoma surgery may also be required. [3,6,7]<\/p>\n
Importantly, not all series show the same magnitude of pressure benefit after lensectomy, and this heterogeneity should not be ignored. Muralidhar et al. reported that glaucoma in microspherophakia can remain difficult to control despite medical and surgical management. [5] By contrast, Rao et al. demonstrated a median reduction of intraocular pressure from 22 to 14 mmHg after lensectomy, with a complete success probability of 69% at one year and 51% at five years. [7] These apparently different results are not contradictory; rather, they likely reflect different stages of disease at presentation, especially the degree of pre-existing angle damage and optic nerve compromise.<\/p>\n
Once the pathologic lens has been removed, the surgeon must choose how to rehabilitate aphakia. Anterior chamber IOLs are less attractive in WMS because they require larger incisions and may worsen endothelial stress, induce astigmatism, and pose angle-related concerns in eyes already susceptible to glaucoma. Iris-fixated IOLs can be effective in selected cases but depend on adequate iris tissue and may be associated with iris trauma, pupil distortion, or endothelial cell loss. Posterior chamber scleral fixation, in contrast, places the prosthesis in a more physiologic position and avoids direct occupation of the anterior chamber angle. [10-12]<\/p>\n
Evidence specifically addressing scleral fixation in spherophakia is increasingly encouraging. Kannan et al. reported that lensectomy with pars plana vitrectomy and sutureless scleral-fixated IOL implantation in 26 spherophakic eyes improved mean best-corrected visual acuity from $0.43 \\pm 0.32$ to $0.19 \\pm 0.21$ loqMAR while also markedly improving refractive status. [8] Similarly, Burugupally et al. found that lensectomy, vitrectomy, and glued IOL implantation provided significant visual and refractive improvement in spherophakia-associated glaucoma, although many eyes still required postoperative pressure-lowering medication. [13] Collectively, these studies support the broader concept that posterior chamber scleral fixation is a sound reconstructive strategy in the absence of capsular support.<\/p>\n
The Carlevale lens deserves particular attention because it was designed specifically for sutureless posterior chamber scleral fixation. In the largest published adult series, scleral fixation of the Carlevale IOL after pars plana vitrectomy in 169 eyes with inadequate capsular support led to improvement in mean postoperative visual acuity from approximately 20\/80 to 20\/25, with good lens stability and no need for reoperation. [9] A pediatric series also showed good IOL positioning and meaningful visual improvement in children without capsular support. [10]<\/p>\n
A dedicated review concluded that the Carlevale lens is among the best current options for eyes with absent capsular support because of its foldable single-piece design, Bellone WMS case-based review Page 4 self-centering behavior, and favorable safety profile. [11] A more recent review comparing modern scleral fixation strategies highlighted that, versus anterior chamber or iris-fixated alternatives, Carlevale implantation may induce less astigmatism and offer easier, faster implantation with very good centration. [12] For a WMS eye characterized by a fundamentally unreliable zonular architecture, these attributes are highly relevant.<\/p>\n
Although the literature specifically addressing Carlevale implantation in WMS-related spherophakia remains limited, the conceptual match is strong. The disease creates exactly the type of aphakic eye in which a stable posterior chamber, scleral-fixated, sutureless implant is desirable. In my view, this is the major original contribution of the present case: it shows how a modern self-anchoring scleral-fixated lens can be integrated into the surgical management of a classical yet mechanically challenging inherited lens disorder.<\/p>\n
The originality of this manuscript lies less in the rarity of WMS alone than in the surgical reasoning it illustrates. This was a bilateral, staged case in which the same disease mechanism was addressed in two eyes by the same reconstructive logic: pars plana lensectomy\/vitrectomy to remove the pathologic lens, followed by posterior chamber rehabilitation with a Carlevale IOL. The case also includes two clinically important aggravating features-cataract and already manifest glaucomatous optic nerve\/visual field damage-which strengthen the indication for timely intervention.<\/p>\n
The main limitation is that this manuscript was reconstructed without the original surgical videos. Nevertheless, the scientific value of the case rests primarily on the clinical phenotype, the treatment rationale, the choice of surgical sequence, and the consistency of the bilateral staged approach. A final journal submission could be further strengthened by adding preoperative and postoperative numerical data from the medical charts, including visual acuity, intraocular pressure, glaucoma medications, optic nerve imaging, and visual field indices.<\/p>\n
In Weill-Marchesani syndrome, the crystalline lens is often the anatomical fulcrum of ocular disease, simultaneously driving myopia, instability, pupillary block, angle compromise, cataract-related visual decline, and secondary glaucoma. When spherophakia is accompanied by lens dislocation and inadequate capsular support, pars plana vitrectomy with lensectomy offers a rational means of removing the principal pathological substrate.<\/p>\n
In such eyes, secondary scleral fixation of a posterior chamber IOL provides a more physiologic reconstruction than anterior chamber or iris-dependent solutions. The Carlevale lens appears especially suitable for this task because it combines posterior chamber positioning with sutureless self-anchoring scleral fixation. The present bilateral staged case supports the view that surgery in WMS should aim not merely to extract an abnormal lens, but to rebuild a more stable ocular anatomy before progressive glaucoma causes further irreversible optic nerve and visual field loss.<\/p>\n
Abstract Weill-Marchesani syndrome (WMS) is a rare connective tissue disorder in which ocular morbidity is largely driven by microspherophakia or spherophakia, lenticular myopia, ectopia lentis, cataract, and secondary glaucoma. In many eyes, the crystalline lens is not merely a displaced structure but the anatomical engine of disease, inducing pupillary block, anterior chamber crowding, angle closure, […]<\/p>\n","protected":false},"author":2,"featured_media":22109,"comment_status":"closed","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[3715],"tags":[],"class_list":["post-22108","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-news-3"],"_links":{"self":[{"href":"https:\/\/albertobellone.it\/en\/wp-json\/wp\/v2\/posts\/22108","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/albertobellone.it\/en\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/albertobellone.it\/en\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/albertobellone.it\/en\/wp-json\/wp\/v2\/users\/2"}],"replies":[{"embeddable":true,"href":"https:\/\/albertobellone.it\/en\/wp-json\/wp\/v2\/comments?post=22108"}],"version-history":[{"count":5,"href":"https:\/\/albertobellone.it\/en\/wp-json\/wp\/v2\/posts\/22108\/revisions"}],"predecessor-version":[{"id":22214,"href":"https:\/\/albertobellone.it\/en\/wp-json\/wp\/v2\/posts\/22108\/revisions\/22214"}],"wp:featuredmedia":[{"embeddable":true,"href":"https:\/\/albertobellone.it\/en\/wp-json\/wp\/v2\/media\/22109"}],"wp:attachment":[{"href":"https:\/\/albertobellone.it\/en\/wp-json\/wp\/v2\/media?parent=22108"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/albertobellone.it\/en\/wp-json\/wp\/v2\/categories?post=22108"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/albertobellone.it\/en\/wp-json\/wp\/v2\/tags?post=22108"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}