PURPOSE: To present a case of Weill-Marchesani syndrome with corneal endothelial dysfunction due to anterior dislocation of a spherophakic lens and corneolenticular contact. METHODS: A 17-year-old woman presented with high myopia and progressive visual disturbance. She was of short stature and had brachydactyly. Her initial Snellen best corrected visual acuity (BCVA) was 20/50 (-sph 20.50 -cyl 3.00 Ax 180) in her right eye and 20/40 (-sph 16.00 -cyl 6.00 Ax 30) in her left eye. Slit lamp examination revealed a dislocated spherophakic lens touching corenal endothelium. A microspherophakic lens, hypoplastic ciliary body, and elongated zonules were confirmed on rotating Scheimpflug camera (Pentacam(R)) and on ultrasound biomicroscopy. Specular microscopy showed corneal endothealial dysfunction. Systemic evaluation was performed, and chromosomal study showed 46, XX, inv (15) (q13qter). The patient was diagnosed with Weill-Marchesani syndrome. RESULTS: Due to impending corneal decompensation, phacoemulsification and suture fixation of the intraocular lens were performed. The operation and postoperative course were uneventful. Three months postoperatively, the visual acuity was 20/30 (OD) and 20/40 (OS) without correction, and BCVA was 20/20 (+sph 0.50 -cyl 2.00 Ax 160 : OD) and 20/25 (+sph 1.50 -cyl 3.00 Ax 30 : OS). During the follow-up period, increased corneal endothelial counts, hexagonality, and decreased corneal thickness were achieved. CONCLUSIONS: In Weill-Marchesani syndrome with a chromosomal anomaly, a dislocated spherophakic lens may cause severe corneal endothelial dysfunction due to corneolenticular contact, and prompt lensectomy is important to prevent such complications.
*Abnormalities, Multiple ; Adolescent ; *Chromosomes, Human, Pair 15 ; Diagnosis, Differential ; Dwarfism/*genetics ; Endothelium, Corneal/pathology/ultrasonography ; Female ; Fingers/*abnormalities ; Hand Deformities, Congenital/diagnosis/*genetics ; Humans ; Inversion, Chromosome/*genetics ; Lens Implantation, Intraocular/methods ; Lens Subluxation/diagnosis/*genetics/surgery ; Microscopy, Acoustic ; Phacoemulsification/methods ; Syndrome

PURPOSE: To report the experiences at our institute of pars plana vitrectomy (PPV) in combination with intravitreal triamcinolone acetonide (IVTA) injection in patients with retained lens material after cataract surgery. METHODS: The medical records of patients who underwent PPV between January 2005 and December 2006 after complicated cataract surgery in which lens material was dropped into the vitreous cavity were reviewed retrospectively. RESULTS: Five eyes of five patients were treated by vitrectomy and removal of the dislocated lens material and were administered an adjuvant intravitreal injection of 4 mg triamcinolone acetonide at the end of surgery. IVTA administration induced a rapid improvement in vision within six weeks postoperatively. Final visual acuity was 20/32 or better in all patients. Inflammatory cystoid macular edema (CME) that existed preoperatively was promptly improved after IVTA. Intraocular pressure increased transiently in one patient. No procedure-related complications were observed. CONCLUSIONS: PPV with additional IVTA was successfully performed in five eyes with retained lens material and resulted in a marked resolution of vitreous inflammation, an improvement in CME and rapid visual recovery. The authors recommend that IVTA administration should be investigated more thoroughly as an adjunct during the management of retained lens material.
Aged ; Cataract Extraction/adverse effects ; Device Removal/*methods ; Female ; Follow-Up Studies ; Glucocorticoids/*administration & dosage ; Humans ; Injections ; Intraoperative Care/methods ; Lens Subluxation/pathology/*surgery ; Lenses, Intraocular/*adverse effects ; Macular Edema/etiology/pathology/*surgery ; Male ; Middle Aged ; Reoperation ; Retrospective Studies ; Time Factors ; Tomography, Optical Coherence ; Treatment Outcome ; Triamcinolone Acetonide/*administration & dosage ; Visual Acuity ; Vitrectomy/*methods ; Vitreous Body