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Ординатура / Офтальмология / Английские материалы / Applied Pathology for Ophthalmic Microsurgeons_Naumann, Holbach, Kruse_2008

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188 5.4 Ciliary Body

a

b

c

d

Fig. 5.4.10. Diffuse shedding of malignant melanocytes into aqueous and adjacent structures in different patients showing unilateral ocular hypertension or glaucoma. a Malignant melanocytes on the surface of the iris recognized by biocytology. b Progressive diffuse malignant melanoma with seeding into the surface of the iris. c Diffuse malignant melanoma of iris in 35-year-old male. d Same eye 33 months later

a

b

Fig. 5.4.11. Diffuse non-pigmented malignant melanoma of the iris (ring melanoma) masquerading as “glaucoma.” a Preoperatively. b After filtering procedure “cystic” bleb

5.4.2 Surgical Pathology 189

c

d

Fig. 5.4.11. c, d Filtering channel lined by malignant melanocytes: anterior chamber (AC), Descemet membrane (DM). e, f Ring melanoma invading ciliary body and outflow channels in

opposite chamber angles e (PAS)

190 5.4 Ciliary Body

f

Fig. 5.4.11 (Cont.)

a

b

Fig. 5.4.12. Recurrence and subconjunctival extension of malignant melanoma of the anterior uvea 14 years after sector iridectomy via corneal scleral incision. Starting at age 33 years this patient was observed for 10 years because of an iris tumor. Then secondary glaucoma (32 mm Hg) and a pigmented tumor of the iris roof was described. Histologically the tumor was considered a “nevus of the iris.” Twelve years later he developed diffuse malignant melanoma of the anterior uvea, pressure of 40 mm Hg and advanced cupping. He refused enucleation, which was performed later, 14 years after the

c sector iridectomy for the initial biopsy. a Diffuse malig-

nant melanoma of iris and ciliary body with extrascleral extension. b Gonioscopy showing invasion of the ciliary body. c Histopathology of sector iridectomy specimen, 14 years before enucleation

5.4.2 Surgical Pathology 191

d

Fig. 5.4.12. d Higher power

 

from c. Relatively benign

 

looking melanocytes.

 

e–g Recurrence of malignant

 

melanoma in the iris root

 

and ciliary body within the

 

intracorneal scar and with

 

extrascleral extension (ar-

 

rows) (PAS stain), also high-

 

er power. This case illus-

 

trates the need to perform

 

“biopsy” of the iris only

 

through the avascular corne-

 

al access to allow earlier rec-

 

ognition of recurrence.

 

(Case reported to Joint Meet-

 

ing of Verhoeff Society and

 

European Ophthalmic Pa-

 

thology Society, Philadel-

 

phia, 1986)

e

 

f

g

192 5.4 Ciliary Body

a

c

b

d

Fig. 5.4.13. Primary malignant rhabdomyosarcoma of the “iris root” in 5-year-old girl treated by sector iridectomy followed by four repeated recurrences within 4 years in spite of various types of radiotherapy requiring enucleation (see also Fig. 5.3.16).

a Preoperatively. b Highly cellular neoplasm reaching beyond the iris root. c Recurrence in iris root and ciliary body. d Massive infiltration of the ciliary body. Processes of the iris root cannot be removed completely with a sector iridectomy, they require block excision including the adjacent pars plicata of the ciliary body. After 36-year- old follow-up patient is in good health. (Reported by Naumann et al. 1972)

5.4.3 Indications for Procedures Involving the Ciliary Body

193

5.4.2.7

Epithelial Ingrowth Involving the Anterior Chamber Angle

Any epithelial ingrowth both of the diffuse and cystic type involving the anterior chamber angle covers not only the surface of the iris root and the back of the cornea but – by necessity – also the “face of the ciliary body,” or in other words: the insertion of the ciliary muscle to the scleral spur. As direct manipulation and separation of the delicate corneal, conjunctival or epidermal epithelial layers – often only one to two cell layers – is impossible, the adjacent tissues must be “used as a shell,” resulting in block excision together with cornea, sclera, and pars plicata of the ciliary body and iris (Fig. 5.4.8). Partial excision attempted by sector iridectomy or laser application will convert the cystic into a diffuse type of epithelial ingrowth – both approaches are contraindicated (Tables 5.4.6, 5.4.8).

5.4.2.8

Zonular Apparatus in Pseudoexfoliation Syndrome and Homocystinuria

Both in pseudoexfoliation syndrome and homocystinuria basement membrane material and microfibrillar intercellular matrix is interposed between the origin of the zonular fibers and the non-pigmented ciliary epithelium. This causes weakening of the anchoring of the zonules at their origin in the ciliary body leading to phacodonesis or spontaneous dislocation or even luxation of the lens (see Chapter 5.5).

5.4.2.9

Non-invasive In Vivo Diagnostic Procedures

Echography, ultrasound biomicroscopy, optical coherence tomography (OCT), diaphanoscopy in addition to gonioscopy and funduscopy with indentation of the ora serrata are used. Laser tyndallometry measures the involvement of the blood-aqueous barrier.

5.4.3

Indications for Procedures Involving the Ciliary Body

The ciliary body itself until recently was considered a “taboo zone” for direct intraocular microsurgery because of concerns about hemorrhage, vitreous loss, lens subluxation and their complications. We shall show that these concerns can be controlled.

5.4.3.1

Posterior Sclerotomy

Penetrating incisions of the sclera 4 mm behind the limbus open the supraciliary space and allow drainage of fluid from the choroidal detachment particularly with postoperative or post-traumatic defects in the anterior segment causing acute or persisting ocular hypotony. The anterior ciliary arteries are avoided by entering in the sectors between the rectus muscles. Choroidal detachments develop after all perforating injuries that do not seal spontaneously. If wound closure is delayed, reforming the anterior chamber may be possible only after the fluid of the choroidal detachment is released by posterior sclerotomy.

5.4.3.2

Pars Plana Vitrectomy

This approach, pioneered by Machemer (1972), has revolutionized not only vitreal surgery but has also set an example for minimally invasive microsurgery in other organs of the body. It starts with posterior sclerotomies and traverses both the sclera and the pars plana of the ciliary body.* It is indicated: (1) in acute ciliary block angle closure glaucomas with abnormal accumulation of aqueous in the vitreous behind the lens and (2) to treat a multitude of vitreoretinal pathologies via multiple ports combining infusion, cutting, suction and illumination – and implantation of gas and silicone (see Chapter 5.6).

5.4.3.3

Direct Cyclopexy for Treating Persisting Ocular Hypotony Resulting from Traumatic or Iatrogenic Cyclodialysis

Cyclodialysis for up to 2 h (60°) may close and heal spontaneously or after thermal argon laser coagulation. Closure of larger clefts by direct thermic lasers usually is not successful – nor is intravitreal gas injection, with or without capsular tension rings placed in the sulcus ciliaris. Direct cyclopexy describes the reattachment of the anterior attachment line of the ciliary muscle to the scleral spur by suturing under direct microsurgical control (Fig. 5.4.7, 5.4.8 and Table 5.4.2). This procedure is only indicated: (1) if there is functional impairment, (2) morphologic consequences of ocular hypotony like macular star with cystoid maculopathy, choroidal edema and papilloedema ex vacuo persist, (3) the cyclodialysis

*Historically a similar entry into the globe was suggested by Celsus (Chapter 1) to achieve couching of the cataractous lens (see Chapter 1).

194 5.4 Ciliary Body

Fig. 5.4.14. Block excision of tumors of the anterior uvea: ciliary body with adjacent iris and/or choroid and full thickness cornea and sclera, followed by corneoscleral graft. Sketches of surgical steps. a Transillumination outlining the borders of the tumor by scleral sutures. c Removal of the tumor with adjacent tissue of ciliary body, iris, full thickness sclera and cornea en block usually with sector iridectomy and anterior vitrectomy

a

b

c

d

 

e

 

Fig. 5.4.14. d Excised tumor and shell. e Corneoscleral graft

 

from donor eye corresponding to the defect of the wall result-

 

ing from the block excision. f Corneoscleral graft in place

f

(modified from Naumann, 1987)

5.4.3 Indications for Procedures Involving the Ciliary Body

195

a

b

Fig. 5.4.15. Highly vascularized malignant melanoma of the iris extending into the ciliary body in a 47-year-old male. a Preoperatively.

b Block excision of 7,5 mm. c Histopathology of block consisting of the tumor and adjacent full-thickness sclera and cornea. Iris (thick arrow). d After follow-up of 13 years partially vascularized tectonic corneoscleral graft. Iron staining of corneal epithelium of host (arrow)

– not to be confused with recurrence! Extent of graft (dots). Tumor anterior (TA) and posterior to iris (TP). Pars plicata (PP) of ciliary body (CB)

c

extends for more than 60° (2 clock hours), and (4) no spontaneous improvement has occurred within 6 weeks. Our technique avoids anterior synechiae resulting in persisting closure angle glaucoma (Table 5.4.3) (Naumann and Völcker 1981; Küchle and Naumann 1991).

Transitory acute pressure spikes in the first days after direct cyclopexy should be treated medically. In more than 60 consecutive patients no persisting secondary glaucomas developed.

d

5.4.3.4

“Block Excision” of Tumors and Epithelial Ingrowth of Anterior Uvea

Block excision is defined as in toto removal of the process involving the anterior uvea and angle structures together with the adjacent iris, pars plicata of the ciliary body, full thickness sclera and cornea up to 150° of the circumference.

The defect in the eye wall then is closed by a corresponding corneoscleral tectonic graft (Tables 5.4.4 – 5.4.8; Naumann and Rummelt 1996; Rummelt et al. 1994) (Fig. 5.4.15 – 22).

196 5.4 Ciliary Body

a

b

c

d

e

Fig. 5.4.16. Large malignant melanoma of the anterior uvea extending to the level of the inferior temporal retinal vessel arcade located inferiorly in 55 years old male. a Preoperatively with infrared photography. b Excised tumor with adjacent full thickness sclera and cornea and retina (preceded by retinopexy 2 weeks earlier): 18 × 18 × 9 mm. Retina (R) over tumor detached. Ciliary Body (CB). c Histopathology. d Extensive chorioretinal scarring following broad cryoretinopexy. e Sixteen years postoperatively, external aspect with visual acuity of 0.2 – 0.3. Extent of graft (dots)

5.4.3 Indications for Procedures Involving the Ciliary Body

197

c

a

b

d

e

f

g

Fig. 5.4.17. Malignant melanoma of the anterior uvea with extension into the iris root, 74-year-old male: microsurgical steps. a Preoperative gonioscopy: tumor in iris root (arrow). b Flieringa ring after phacoemulsification and implantation of rigid PMMA lens. c Block excision 9 mm in diameter. d Excised tumor with adjacent structure and peripheral iris. e With tectonic corneoscleral graft scleral cataract incision (In), Sclera (S), Cornea (C), Choroid (Ch), Retina (R). f One year later corneoscleral graft, clear optic media, peripheral iris coloboma. g Fifteen months postoperatively: good function