PMC6525849_01
PMC6525849_02
Patient 1 UBM before surgery and after uveo-scleral excision en bloc. (a) Left: longitudinal scan at the 6 o'clock position (L6) shows a solid thickening of the ciliary body and the anterior choroid; the corresponding anterior chamber angle is infiltrated, the acoustic structure is heterogeneous, and the internal reflectivity is medium. Right: transversal scan at the 5:30 o'clock position (T5:30) shows the double dome-shaped morphology of the lesion at the level of the anterior choroid and the circumscribed growth into the overlying sclera, which appears hypoechoic (white arrow). (b) Patient 1: slit lamp examination presurgery. Image shows a confined pigmented nodular growth of uveal melanoma through the sclera, at about 6 mm from the limbus, under the conjunctival layer. (c) After Hanna trephination, block excision is completed with scissors until removing the entire lesion and the overlying infiltrated sclera. (d) Tectonic corneal graft covers the scleral defect and is sutured with 16 interrupted Nylon 10.0 sutures. (e) Corneal graft is well integrated into the sclera, under the conjunctiva, about 4 years after surgery.
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fig1
Ultrasound examination and UBM showed in the left eye a solid irregular-shaped thickening starting at the level of the choroidal layer of the preequatorial part of the eye, between the 5 and 6:30 o'clock positions (Figure 1(a) left), associated with circumscribed extraocular growth, into the overlying sclera under the conjunctiva, 2 mm thick (Figure 1(a) right). This nodule was at about 6 mm from the limbus (Figure 1(b)); the adjacent ciliary body was thickened, and the basal diameter of the entire lesion was large about 13 mm. The patient was submitted to scleral full-thickness block excision (8.50 mm large) of tumor, combined to the corneal graft, 16 interrupted Nylon 10.0 sutures (Figures 1(c)-1(e)).
PMC6525849_03
Patient 2 before treatment. (a) Gonioscopy shows a pigmented fusiform thickening of the iris root (white arrow) and a dome-shaped pigmented mass behind the pupil foramen (dashed line and double white arrow). (b) UBM longitudinal scan at the 2:30 o'clock position (L2:30) showing a circumscribed dome-shaped mass at the level of the ciliary body, extended to the iris root, which touches the anterior surface of the lens. (c) Scleral thinning and prolapse of the uveal tissue at the limbus, one year after brachytherapy. (d) UBM L2:30 scan shows a local scleral extension of the tumor, under the conjunctival layer (white asterisk).
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fig2
She was a 48-year-old woman affected by a pigmented fusiform thickening of the iris root in the left eye, as shown by gonioscopy (Figure 2(a)). UBM revealed the presence of a well-circumscribed dome-shaped mass at the level of the ciliary body, between the 2:30 and 3 o'clock positions (Figure 2(b)). One year later, the patient showed a scleral thinning and a prolapse of uveal tissue at the limbus, corresponding to the site of the preexisting lesion (Figure 2(c)). UBM showed a local scleral extension of the tumor, under the conjunctival layer, 0.88 mm thick (Figure 2(d)).
Patient 2 block excision of sclera and ciliary melanoma. (a) Removal of the ciliary melanoma en bloc after marking the margins and after Hanna trephination. (b) Corneal graft is sutured to the adjacent sclera; basal iridectomy. (c) Corneal graft is in situ 7 years after surgery well visible under the conjunctiva. (d) The perfect clearance of trephination on healthy scleral and ciliary body margins. (e, f) Postirradiation necrotic aspect of spindle melanoma's cells.
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fig3
The patient underwent a radical block excision of sclera, ciliary melanoma, and adjacent iris tissue, by a peripheral iridectomy performed through the opening of the sclera, combined to the corneal tectonic graft (8.50 mm large), 16 single stitches (Figures 3(a)-3(c)).
PMC6525849_04
Patient 3 before and after surgery. (a) Large epithelial cysts of the iris involving the upper nasal quadrant of the right eye. Cysts stretch the pupil foramen and cause circumscribed corneal failure. (b) Block excision of the cysts, iris, cornea, and limbal sclera after Hanna trephination. (c) Eccentric corneal graft is clear one-year postoperatively, and sutures are still in situ; a large iris coloboma is observable. (d) Two months after penetrating keratoplasty and cataract surgery. Photo shows that the graft is clear and the cosmetic IOL is well positioned in the sulcus; double continuous Nylon 10.0 suture is in situ.
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fig4
A corneal graft (8.50 mm large), 16 interrupted Nylon 10.0 sutures, was contemporary sutured to the sclera and residual cornea to restore the bulbar wall (Figures 4(b) and 4(c)). About 3 years later, she underwent a penetrating keratoplasty centered on the visual axis, combined to phacoemulsification of the cataract and refractive IOL implantation in the capsular bag, together with cosmetic neutral IOL implantation in the sulcus (Figure 4(d)).
PMC6525849_05
Patient 4 before surgery and after uveo-sclero-corneal block excision. (a) Epithelial iris cysts between the 8 and 12 o'clock positions of the right eye, involving the anterior chamber angle, partially stretching the pupil foramen. (b) UBM transversal scan at the 11 o'clock position (T11) showing multiple optically empty cysts into the anterior chamber occluding the chamber angle, in contact with the corneal endothelium. (c) UBM axial scan at the 12 o'clock position (A12) showing how the large cyst touches the equator and the anterior surface of the lens. (d) Removal of the major cyst at 12 o'clock after creating a scleral fornix-based flap and a limbal incision to enter into the anterior chamber. (e) Block excision of cysts from 8 to 11 o'clock after core dry vitrectomy and manual incision of the marked sclera with a diamond knife. Viscoelastic substance is injected into the anterior chamber. (f) Corneal graft is sutured to the sclera and to the patient's cornea with interrupted Nylon 10.0 sutures. (g) Corneal graft is clear two years after surgery. Sutures are still in situ. (h, i) Perfect integrity of the cyst's wall after block excision, outlined by the inner surface of cornea, angle, iris, and ciliary body. The empty, clear spaces outlined by the cyst's walls are related to the cyst's serous content.
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fig5
She was a 17-year-old girl who was sent to our clinic for diffuse recurrence of epithelial iris cysts in the right eye, which involved the anterior chamber structures of the temporal side between the 8 and 12 o'clock positions (Figure 5(a)). UMB showed multiple iris cysts extended into the anterior chamber in contact with the corneal endothelium; major cysts touched the equator and the anterior surface of the lens, occluding the anterior chamber angle and stretching the pupil foramen (Figures 5(b) and 5(c)). Removal of cysts at 12 o'clock was possible by creating a scleral rectangular fornix-based flap, 5 x 4 mm large, and entering into the anterior chamber through a limbal linear incision, as in transcleral local resection (Figure 5(d)). An uveo-sclero-corneal block excision, including cysts from 8 to 11 o'clock, was then completed, after a core, decompressive, dry pars plana vitrectomy, and covered by the tectonic corneal graft, 8.50 mm large, 16 single stitches (Figures 5(e)-5(g)). Histopathology confirmed the epithelial origin of the cysts (Figures 5(h) and 5(i)).