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Bilateral Opposing Lens Dislocation and Retinal Detachment in LTBP2-Associated Weill-Marchesani Syndrome: Surgical
Adriana P Perez Negron1, Arnulfo Garza Reyes, Joaquin Sosa Lockward
1Department of Ophthalmology, Bascom Palmer Eye Institute, University of Miami Miller School of Medicine, Miami, FL, USA.
Purpose:
To report the presentation and surgical management of LTBP2-associated Weill-Marchesani syndrome with bilateral lens dislocation and unilateral rhegmatogenous retinal detachment.
Methods:
A detailed clinical and surgical case analysis was performed in an 8-year-old male with confirmed biallelic LTBP2 mutations. Ophthalmic examination, multimodal imaging, and intraoperative optical coherence tomography (iOCT) were utilized. The patient underwent single-session bilateral pars plana lensectomy and vitrectomy, with scleral buckle placement and silicone-oil tamponade in the eye with total retinal detachment. iOCT-guided viscoelastic dissection was employed to manage lenticulo-corneal adhesion in the fellow eye.
Results:
The right eye demonstrated a posteriorly dislocated lens with an associated open-funnel rhegmatogenous retinal detachment, while the left eye exhibited anterior lens dislocation with firm adherence to the corneal endothelium and an attached retina. Postoperatively, both retinas remained attached. Transient ocular hypertension was successfully managed medically. The cornea in the left eye-maintained clarity, developing a limited posterior stromal scar without endothelial decompensation. At four months, visual acuity improved to 20/60 in the right eye and 20/100 in the left.
Conclusion:
Severe LTBP2-associated Weill-Marchesani syndrome may require individualized bilateral surgical strategies. iOCT guidance and multidisciplinary care can support favorable anatomical and functional outcomes in complex pediatric cases.
