Ординатура / Офтальмология / Английские материалы / Tumors of the Eye and Ocular Adnexa_Char_2001

been proposed, but the author has not used them.47 Entropion also has been reported after this procedure,48 but the author has not had this problem.

The author has not used the Mustardé technique for the upper lid reconstruction, in which the lower lid is rotated to replace the upper lid and the defect created inferiorly is replaced with a posterior graft of the nasal septal mucosa and a rotational cheek flap. This procedure, while effective in Mustardé’s capable hands, seems much more complex without any obvious advantages.49 Similarly, a variant of the reverse Mustardé—using the upper eyelid to close a lower eyelid defect—seems a less optimal procedure than the others described above.50

OTHER PROCEDURES

As mentioned at the outset of this chapter, the discussion has been limited to the procedures found most useful for the repair of surgically created lid defects by an ophthalmologist. The procedures listed below are less commonly useful in managing either canthal lesions or isolated lid tumors.

Composite grafts taken from the contralateral lid have been used for over 35 years to repair lid defects.51,52 The initial composite grafts were of full thickness and involved both the posterior and anterior lamellae of the lid; necrosis of a portion of the graft was not an infrequent complication. More recently, Putterman and others have suggested using only a posterior lamella from the opposite lid with an advancement flap of skin and muscular tissue to cover the anterior defect.53 The maximum length of a full-thickness composite graft is approximately 8 mm. We have rarely used this procedure since it has a greater risk of necrosis than the other options listed. In a one-eyed patient with a tumor involving the upper lid of the seeing eye, a posterior composite graft from the contralateral lid in combination with lateral cantholysis may be used to avoid closing the lid. As discussed above, we usually elect to use either a modification with a free tarsal conjunctival graft or a rotational tarsal flap.

A larger complete upper lid defect in a one-eyed patient may be one situation where the Mustardé lower lid transposition operation would be another

reasonable alternative. In a large upper eyelid defect where the cancer does not reach more than 10 to 12 mm above the eyelid margin, a variation of this option has been used by the author in a few cases. A free tarsal graft from the contralateral upper eyelid is sewn in place, and the skin from below the brow is brought down in a modified “bucket-handle” approach. A series of these cases will be published from other centers.

If upper lid tarsus is not available for the reconstruction of a lower lid defect, or if an attempt at repair of a large upper lid defect is made without a Cutler-Beard procedure, a free tarsal graft and a nasal septum mucosa free graft to create a posterior lid lamella are other options.54,55 Free chondrodermal auricular cartilage or eye bank sclera grafts have also been used in selected cases.56–58 The technique to obtain a nasal-septal graft is discussed under the Mustardé procedure.

Procedure for

Closing Medial Canthal Defects

Large medial defects involving both the upper and lower lids can be repaired with a free skin graft, a glabellar transposition flap, or a laissez-faire approach.59–61 Figure 3–19 shows a case in which a malignant adnexal tumor was superficial and deep canthal structures were clear of tumor. A free skin graft from the supraclavicular area was used to close the defect. Usually, an upper lid tarsal transposition flap, a free posterior composite graft, or a nasal-sep- tal graft is used to create the posterior lamellae, and a sliding skin flap is used to form the anterior lid structure. If the canthal tendon is sacrificed during tumor resection, the posterior grafts or flaps are

sutured to a raised periosteum. As discussed above, under “Lateral Cantholysis” and “Sliding Tarsal Flap,” the posterior lamellae must be anchored toward the posterior lacrimal crest area to maintain proper lid-eye position. In cases with a medial canthal fistula, more extensive surgery is required, and this is nicely summarized elsewhere.61

The laissez faire technique for repair of lid defects was first described in the 18th century.62 We have used this approach in patients who have had multiple tumors, such as in basal cell nevus syndrome or xeroderma pigmentosum syndrome, and in those who have experienced local recurrence after many surgeries and extensive cryotherapy and radiation treatment. The size of the medial defect is reduced by cutting the lateral canthal tendon and mobilizing both the upper and lower lids medially. The remnants of the upper and lower lids are individually sutured to the remains of the medial canthal tendon or orbital periosteum with a 4-0 nonabsorbable suture (Figure 3–20). The wound is cleaned and allowed to granulate. The cosmetic appearance is adequate, but not excellent (Figure 3–21). In smaller defects, laissez faire cosmesis can be quite good.

Usually, the lacrimal drainage system must be repaired if an extensive medial canthal tumor is removed. If any of the canalicular system is present, silicone tubes can be used to reconstruct the drainage system.66 We intubate the remaining portion of the system and tie the tubes in the nose in the usual manner. A posterior notch in the reconstructed lid is used to reconstruct the punctum, and the tubes are secured in those positions. If there is no canalicular system left when the tumor is resected, a Jones tube can be placed secondarily. We usually wait until

52 TUMORS OF THE EYE AND OCULAR ADNEXA

Figure 3–20. Laissez-faire approach. The lateral canthal tendons are severed, and the medial lid remnants are sutured to the orbital periosteum. Healing occurs by granulation.

healing of the reconstructed lid has been accomplished before doing this latter procedure.

Ancillary Surgical Procedures

The glabellar forehead flap is not among our frequently used approaches for lid repair. We only use it to reconstruct a medial canthal tumor, if we are unable to obtain skin from lower lid or adjacent sites. Extensive undermining has to be performed, and the length of the flap should be not more than five times its width.

We have tried not to use Mustardé’s lower lid reconstruction technique for several reasons:49 (1) it is a technically more difficult procedure for the ophthalmic surgeon and no more effective than other techniques; (2) it usually results in a long scar line;

(3) there is a necessity for excising a large triangle of normal cheek skin, since the medial vertical lid inci-

Figure 3–21. Laissez-faire healing of a large tumor in xeroderma pigmentosum patient; the tumor involved over 50 percent of both upper and lower lids.

sion must be 1.5 to 2 times the length of the horizontal defect to be closed; (4) the lid is less mobile than we are able to produce with other types of lower lid repair; and (5) with time, most of these reconstructed lower eyelids sag. The Mustardé procedure is shown schematically in Figure 3–22A. Usually the posterior lamella of the lid is recreated with a nasal-septal graft or hard palate (Figure 3–22B). Prior to obtaining the nasal-septal graft, the nose is packed with 4 percent lidocaine and 1:100,000 epinephrine. At least 1 cm of nasal cartilage must be left in situ anterior to the graft donor site to support the nasal bridge. A submucosal injection of 1:100,000 epinephrine in 2 percent lidocaine is given to strip the mucosa from the contralateral cartilage. In some cases, the nasal opening is too small to be separated effectively, and a lateral rhinotomy in the nasal fold can be made to provide easier access to the nasal septum. The rhinotomy is closed in two layers, after the graft is obtained. A scalpel is used to create the nasal-septal flap. The flap is stripped from the opposite mucosa using a periosteal elevator to avoid a through-and- through septal defect (see Figure 3–22B). If the contralateral mucosa is surgically invaded, it should be either sutured or packed. Scissors are then used to complete the resection. This material is used in a manner analogous to a tarsoconjunctival free graft (Figure 3–22C). A very large myocutaneous flap must be created with the superior edge of the flap at the top of the tragus and brought down vertically just in front of the ear. The semicircle’s height, at least to the level of the brow, tends to counteract lower lid sagging (Figure 3–22D). The amount of medial triangle that must be excised varies with the size of the rotation flap. As mentioned above, usually, the medial vertical cut is 1.5 to 2 times the length of the horizontal defect. We have used the Mustardé procedure if there is diffuse involvement of the canthus, if the lid cancer extends down from the lower lid onto the cheek, or if a patient is referred with a large recurrence or incomplete resection of a tumor previously repaired with a Hughes or a Hewes-Beard procedure. The closure of skin should begin with reconstruction of the canthal angle and proceed from there (see Figure 3–22D). Figure 3–23 shows a case in which we used a Mustardé reconstruction. The patient had a malignant melanoma that had been

described by an outside pathologist as clear on frozen sections. The ophthalmic surgeon repaired the lower lid defect with a Hughes procedure, but he found on perusal of the final selections that all three margins were involved by melanoma.

The Gorney procedure (Figure 3–24) for medial lower lid reconstruction was originally described to repair either medial or lateral lower lid defects, but in our unit, we have used it mainly

as an alternative to reconstruct lesions confined to the medial one-half or two-thirds of the lower lid.67 Reconstruction is performed using a full-thickness pedicle flap from the upper lid skin, muscle, tarsus, and conjunctiva.

After obtaining clear tumor margins, the length of donor skin tissue needed to fill the defect is marked, and an incision is made in the upper lid just inferior to the lid crease and carried down

through the orbicularis. The upper lid is then everted on a Desmares retractor. The amount of tarsus needed to fill the lower lid tarsal defect is measured, and this is incised using a scalpel and sharp scissors (see Figure 3–24A). The entire flap consisting of skin, muscle, tarsus, and conjunctiva is rotated into the lower lid defect and closed in three layers (see Figures 3–24B and C). Similar to the

Hewes-Beard procedure, the closure should be taut to avoid late development of ectropion. Some additional procedures have been used that are not discussed in this chapter. A number of authors have described the use of island rotational pedicle grafts for reconstruction. This is nicely outlined in another publication.68 In some cases where we have had to remove the benign lid tumors, especially

with neurofibromatosis, the use of a lid expander has been quite helpful, and this is described well in a separate publication.69

REFERENCES

1.Beard C. Observations on the treatment of basal cell carcinoma of the eyelids. The Wendell L. Hughes Lecture. Trans Am Acad Ophthalmol Otolaryngol 1975;79:664–70.

2.Einaugler RB, Henkind P. Basal cell epithelioma of the eyelid: apparent incomplete removal. Am J Ophthalmol 1969;67:413–7.

3.Aurora AL, Blodi FC. Reappraisal of basal cell carcinoma of the eyelids. Am J Ophthalmol 1970;70: 329–36.

4.Rakofsky SI. The adequacy of the surgical excision of basal cell carcinoma. Ann Ophthalmol 1973;5: 596–600.

5.Chalfin J, Putterman AM. Frozen section control in the surgery of basal cell carcinoma of the eyelid. Am J Ophthalmol 1979;87:802–9.

6.Doxanas MT, Green WR, Iliff CE. Factors in the successful surgical management of basal cell carcinoma of the eyelids. Am J Ophthalmol 1981;91:726–36.

7.Gooding CA, White G, Yatsuhashi M. Significance of marginal extension in excised basal cell carcinoma. N Engl J Med 1965;273:923–4.

8.Frank WJ. Frozen section control of excision of eyelid basal cell carcinomas: 8 1/2 years’ experience. Br J Ophthalmol 1989;73:328–32.

9.Older JJ, Quickert MH, Beard C. Surgical removal of basal cell carcinoma of the eyelids utilizing frozen section control. Trans Am Acad Ophthalmol Otolaryngol 1975;79:658–63.

10.Wolfe JT III, Yeatts RP, Wick MR, et al. Sebaceous carcinoma of the eyelid. Errors in clinical and pathologic diagnosis. Am J Surg Pathol 1984;8:597–606.

11.Landthaler M, Braun-Falco O, Leitl A, et al. Excisional biopsy as the first therapeutic procedure versus primary wide excision of malignant melanoma. Cancer 1989;64:1612–6.

12.Mohs FE. Chemosurgery; microscopically controlled method of cancer excision. Arch Surg 1941;42: 279–95.

13.Mohs FE. Chemosurgical treatment of cancer of the eyelid; microscopically controlled method of excision. Arch Ophthalmol 1948;39:43–59.

14.Mohs FE. The chemosurgical method for microscopically controlled excision of external cancer with reference to cancer of the eyelids. Trans Am Acad Ophthalmol Otolaryngol 1958;62:335–6.

15.Mohs FE. Microscopically controlled surgery for periorbital melanoma: fixed-tissue and fresh-tissue techniques. J Dermatol Surg Oncol 1985;11:284–91.

16.Mohs FE. Micrographic surgery for the microscopically controlled excision of eyelid cancers. Arch Ophthalmol 1986;104:901–9.

17.Callahan M, Monheit GD, Callahan A. Mohs histologically controlled excision for management of orbital invasion of eyelid carcinomas. Acta Int Congress Ophthalmol 1982;2:998–1001.

18.Mohs FE. Microscopically controlled excision of medial canthal carcinomas. Ann Plast Surg 1981;7: 308–11.

19.Grove AS Jr. Staged excision and reconstruction of extensive facial-orbital tumors. Ophthalmic Surg 1977;8:91–109.

20.Anderson RL, Ceilley RI. A multispecialty approach to the excision and reconstruction of eyelid tumors. Ophthalmology 1978;85:1150–63.

21.Robins P, Henkind P, Menn H. Chemosurgery in treatment of cancer of the periorbital area. Trans Am Acad Ophthalmol Otolaryngol 1971;75:1228–35.

22.Harrington JN. Reconstruction of the medial canthus by spontaneous granulation. (Laissez-Faire): a review. Ann Ophthalmol 1982;14:956–60.

23.Leib ML, Johnson DA, Eliezri YD. Mohs histographic surgery and ophthalmic plastic reconstruction. Ophthal Plast Reconst Surg 1992;8:262–70.

24.Bieley HC, Kirsner RS, Reyes BA, Garland LD. The use of Mohs micrographic surgery for determination of residual tumor and incompletely excised basal cell carcinoma. J Am Acad Dermatol 1992;26:754–6.

25.Stephenson C. Reconstruction of the eyelid using a myocutaneous island flap. Ophthalmology 1983;90: 1060–5.

26.Reese AB. Epithelial tumors of the lid, conjunctiva, cornea, and lacrimal sac. In: Reese AB, editor. Tumors of the eye, 3rd ed. New York, NY: Harper and Row; p. 50.

27.Tenzel RR. Reconstruction of the central one half of an eyelid. Arch Ophthalmol 1975;93:125–6.

28.Tenzel RR, Stewart WB. Eyelid reconstruction by the semicircle flap technique. Ophthalmology 1978;85: 1164–9.

29.Hewes EH, Sullivan JH, Beard C. Lower eyelid reconstruction by tarsal transposition. Am J Ophthalmol 1976;81:512–4.

30.Hughes WL. New method for rebuilding a lower lid; report of a case. Arch Ophthalmol 1937;17:1008–17.

31.Levine MR, Buckman G. Semicircular flap revisited. Arch Ophthalmol 1986;104:915–7.

32.Spinelli HM, Jelks GW. Periocular reconstruction: a systematic approach. Plast Reconstruct Surg 1993; 91:1017–24.

33.Bartley GB, Putterman AM. A minor modification of Hughes’ operation for lower eyelid reconstruction. Am J Ophthalmol 1995;119:96–7.

34.Doxanas MT. Orbicularis muscle mobilization in eyelid reconstruction. Arch Ophthalmol 1986;104:910–4.

56 TUMORS OF THE EYE AND OCULAR ADNEXA

35.Cies WA, Bartlett RE. Modification of the Mustardé and Hughes methods of reconstructing the lower lid. Ann Ophthalmol 1975;7:1497–1502.

36.Holds JB, Anderson RL. Medial canthotomy and cantholysis in eye-lid reconstruction. Am J Ophthalmol 1993;116:218–23.

37.Werner MS, Olson JJ, Putterman AM. Composite grafting for eyelid reconstruction. Am J Ophthalmol 1993;116:11–6.

38.Bartley GB, Kay PP. Posterior lamellar eyelid reconstruction with a hard palate mucosal graft. Am J Ophthalmol 1989;107:609–12.

39.Jordan DR, McDonald H, Anderson RL. Irradiated homologous aorta in eyelid reconstruction. Part II. Human data. Ophthal Plast Reconstruct Surg 1994; 10:227–33.

40.Cohen MS, Shorr N. Eyelid reconstruction with hard palate muscosa grafts. Ophthal Plast Reconstr Surg 1992;8:183–95.

41.Beard C. Surgery of lid tumors. In: Fox SA, editor. International ophthalmology clinics, Vol. 4, Number 1. Boston, MA: Little, Brown; 1964.

42.Kersten RC, Anderson RL, Tse DT, Weinstein GL. Tarsal rotational flap for upper eyelid reconstruction. Arch Ophthalmol 1986;104:918–22.

43.Weinstein GS, Anderson RL, Tse DT, Kersten RC. The use of a periosteal strip for upper eyelid reconstruction. Arch Ophthalmol 1985;103:357–9.

44.Jordan DR, Anderson RL, Nowinski TS. Tarsoconjunctival flap for upper eyelid reconstruction. Arch Ophthalmol 1989;107:599–603.

45.Cutler NL, Beard C. A method for partial and total upper lid reconstruction. Am J Ophthalmol 1955;39:1–7.

46.Wesley RE, McCord CD Jr. Transplantation of eyebank sclera in the Cutler-Beard method of upper eyelid reconstruction. Ophthalmology 1980;87:1022–8.

47.Hecht SD. An upside-down Cutler-Beard bridge flap. Arch Ophthalmol 1970;84:760–4.

48.Carroll RP. Entropion following the Cutler-Beard procedure. Ophthalmology 1983;90:1052–5.

49.Mustardé JC. Repair and reconstruction in the orbital region. New York, NY: Churchill Livingstone Inc.; 1971.

50.Papp C, Maurer H, Geroldinger E. Lower eyelid reconstruction with the upper eyelid rotational flap. Plast Reconstruct Surg 1990;86:563–5.

51.Callahan A. Free composite lid graft. Arch Ophthalmol 1951;45:539–45.

52.Fox SA. Autogenous free full-thickness eyelid grafts. Am J Ophthalmol 1969;67:941–5.

53.Putterman AM. Viable composite grafting in eyelid reconstruction. Am J Ophthalmol 1978;85:237–41.

54.Stephenson CM, Brown BZ. The use of tarsus as a free autogenous graft in eyelid surgery. Ophthal Plast Reconstr Surg 1985;1:43–50.

55.Brown BZ. The use of homologous tarsus as a donor graft in lid surgery. Ophthal Plast Reconstr Surg 1985;1:91–5.

56.Robbins TH. Chondrodermal graft reconstruction of the lower eyelid. Br J Plast Surg 1981;34:140–1.

57.Beyer CK, Albert DM. The use and fate of fascia lata and sclera in ophthalmic plastic and reconstructive surgery. (The Wendel Hughes Lecture 1980). Ophthalmology 1981;88:869–86.

58.Baylis HI, Rosen N, Neuhaus RW. Obtaining auricular cartilage for reconstructive surgery. Am J Ophthalmol 1982;93:709–12.

59.Leone CR Jr, Hand SI Jr. Reconstruction of the medial eyelid. Am J Ophthalmol 1979;87:797–801.

60.Mehta HK. Simultaneous, spontaneous, and primary surgical repair of eyelids. Br J Ophthalmol 1989;73: 488–93.

61.Putterman AM. Reconstruction of nasal fistulas of the medial canthus. Am J Ophthalmol 1989;108:68–74.

62.Marmelzat W. “Noli me tangere” circa 1754; Jacques Daniel’s forgotten contribution to skin cancer. Arch Dermatol 1964;90:280–3.

63.Fox SA, Beard C. Spontaneous lid repair. Am J Ophthalmol 1964;58:947–52.

64.Fier RH, Older JJ. Spontaneous repair of the medial canthus after removal of basal cell carcinoma. Ophthal Surg 1982;13:737–40.

65.Mehta HK. Spontaneous reformation of lower eyelid. Br J Ophthalmol 1981;65:202–8.

66.Quickert MH, Dryden RM. Probes for intubation in lacrimal drainage. Trans Am Acad Ophthalmol Otolaryngol 1970;74:431–3.

67.Gorney M, Falces E, Jones H, Manis JR. One-stage reconstruction of substantial lower eyelid margin defects. Plast Reconstr Surg 1969;44:592–6.

68.Beyer-Machule CK, Grewers H, Kestember E. Island rotational pedicle graft for reconstruction. Orbit 1994;13:183–6.

69.Tse DT, McCafferty LR. Controlled tissue expansion in periocular reconstructive surgery. Ophthalmology 1993;100:260–8.

The pathogenesis and pathophysiology of squamous cell carcinoma, melanoma, sebaceous cell carcinoma, and Kaposi’s sarcoma (KS) are covered in the chapters on lid tumors. The oncogenesis and progression of melanomas are discussed in the chapters on eyelid and uveal melanomas, and the mechanism of lymphoid tumor development is discussed in the section on the orbit.

Five malignancies often involve the conjunctiva: squamous cell carcinoma, melanoma, lymphoid tumors, KS, and sebaceous cell carcinoma, secondarily from contiguous lid involvement. Squamous cell carcinoma is the most common malignant lesion of the conjunctiva. Melanomas occur less frequently, followed by lymphoid lesions, sebaceous gland carcinoma, and KS. Sebaceous gland carcinoma is discussed in Chapters 1 and 3. Although basal cell carcinoma of the conjunctiva has been reported, the estimated ratio of squamous cell conjunctival carcinoma to basal cell conjunctival carcinoma is greater than 20:1.1–3 In one large series of conjunctival malignancies, the 6 patients with basal cell carcinoma all had a history of an identical neoplasm involving the eyelid.4 There are some epidemiologic data on squamous cell carcinoma of the conjunctiva. Newton and co-workers studied the geographic distribution of this malignancy in relation to ambient solar ultraviolet (UV) radiation. They noted that the incidence of squamous cell carcinoma declined by approximately 49 percent for each 10-degree increase in latitude. In Uganda, there are 12 cases per million per year in contrast to 0.2 cases per million per year in the United Kingdom.5 In a study from the United States, Sun and co-workers noted that the incidence was 0.3 per million per year, with

the rate being fivefold higher in Caucasian males. They also noted a very strong UV-B exposure and both conjunctival melanoma as well as squamous cell carcinoma of the conjunctiva.6

Unlike both cutaneous and uveal melanomas, where several cytogenetic abnormalities have been documented, there is a paucity of data on conjunctival melanoma. McCarthy and colleagues noted a translocation in chromosome 1 and 14 in a patient with dysplastic nevus syndrome.7 Dahlenfors and colleagues have noted a small abnormality in a few cells, but it appears that conjunctival melanomas probably have a different genomic alteration than uveal melanomas.8,9

BENIGN CONJUNCTIVAL TUMORS

A number of benign lesions can occur in the conjunctiva and simulate malignancy. The most common simulating lesions include pterygium, viral papilloma (Figure 4–1), amelanotic nevus, dermolipoma (Figure 4–2), pyogenic granuloma (Figures 4–3A and B), and corneal pannus. In most cases, these lesions do not require biopsy, but in uncommon presentations, incisional biopsy or cytopathologic evaluation of surface scrapings is necessary.

Viral papillomas are one of the more frequent simulating conjunctival lesions (see Figure 4–1).1–4,10 They most commonly occur in young children than in adults, often in multiple areas.11 Recently, workers have demonstrated that some benign conjunctival papillomas appear to be caused by the human papillomavirus (HPV). Management of these lesions is often quite difficult.12 Surgery, topical chemotherapy, cimetidine, and cryotherapy have been effective

58 TUMORS OF THE EYE AND OCULAR ADNEXA

Figure 4–1. Nonmalignant viral conjunctival papilloma.

in some patients.13–15 As discussed below, viruses also may be important in the pathophysiology of squamous cell carcinomas of the conjunctiva.16–19

A number of less common benign conjunctival lesions can simulate a malignancy; many can only be correctly diagnosed on histologic evaluation. As an example, we have seen a discoid lupus of the eyelid and conjunctiva simulate a malignancy.20

Ectopic lacrimal gland tissue has been described at the corneal limbus.21 Similarly, respiratory epithelium has been reported to produce a limbal cystic choristoma in a neonate.22 Localized amyloid deposits can occur on the bulbar conjunctiva, although this process more commonly involves either the palpebral or forniceal conjunctiva or the lid (Figure 4–4).23 Unlike a carcinoma that is initially epithelial in origin, often amyloid appears as a subepithelial mass, usually without intrinsic vasculature. Pyogenic granulomas

Figure 4–2. Conjunctival limbal dermoid.

can simulate a malignancy. They usually occur in young patients after strabismus or ptosis surgery (see Figure 4–3B) and in older patients following chronic infection or trauma.24 In older patients, a biopsy is usually necessary to differentiate a pyogenic granuloma at the corneal limbus from the squamous cell carcinoma it mimics.25

Dermoids and osseous choristomas can involve the conjunctival limbus (see Figure 4–2). Usually, these lesions do not grow and are not difficult to differentiate from malignancies, since they appear in a younger age group.26–28 They can be confused, however, with fibromas, lipomas, or nonpigmented nevi.26–29 As Crawford has emphasized, excision of these benign pediatric lesions, especially if they involve the lateral fornices, has been associated with significant ocular morbidity. Similarly, limbal dermoids, when excised too deeply, can result in a filtering wound, staphyloma, or corneal ulcer.30–32 As discussed in the section on anterior uveal tumors, high-frequency ultrasonography has sufficient reso-

A

B

Figure 4–3. A, Pyogenic granuloma of the conjunctiva after ptosis. B, Pyogenic granuloma of the conjunctiva after strabismus surgery.

lution to delineate the intracorneal borders of such lesions. In a study by Elsas and Green, < 1.5 percent of pediatric conjunctival tumors that were biopsied were malignant.33 Several clinicians have reported “teddy bear” granulomas of the conjunctiva that can simulate a tumor. These granulomas are caused by the synthetic fibers of the toy.34 Rarely, a meningoencephalocele can simulate a conjunctival mass in an infant.35 Occasionally, a conjunctival dermoid cyst can produce a chronic red eye.27

Fibrous histiocytomas can sometimes simulate a lymphoid lesion. However, they are more tan and yellow in color, whereas lymphoid tumors are salmon-pink. Unlike malignant processes, these lesions generally do not have intrinsic tumor vessels or episcleral vessels feeding the tumor.36–39 An isolated episcleral neurofibroma can also have a yel- lowish-white appearance and, if incompletely excised, has a tendency to recur.39–41 These tumors have been associated with both neurofibromatosis types 1 and 2 (NF1, NF2).42 Only 13 cases of benign myxoma involving the conjunctiva have been reported. In appearance, myxomas are smooth, fleshy, or gelatinous, and they are often pink in color. Unlike their behavior in other locations, myxomas of the conjunctiva usually do not regrow after they are excised.43,44 Nodular fasciitis can rarely involve the conjunctiva; this author has usually seen it in the orbit.45 Usually, the diagnosis of lymphangiomas of the conjunctiva in the orbit is straightforward; rarely, in an older patient, because of hemorrhage, they can simulate a malignancy.46

Neurilemmomas and trans-scleral leiomyomas can also rarely simulate a conjunctival mass; usually, these appear to be vascularized.47–49 In contrast, while the orbital hemangioperiocytoma is vascularized, the rare conjunctival manifestations of the tumor often are not, although there is usually dilated overlying vasculature.

There are several benign variants of systemic lymphoid lesions and other simulating tumors that can mimic a conjunctival lymphoid lesion.50,51 Rarely, a conjunctival salmon-patch mass may be the first manifestation of a recurrence of systemic acute leukemia.52 Other rare simulating lesions include dacryoadenoma, sinus histocytosis, and, rarely, infectious mononucleosis.53–56

Several indeterminate squamous cell lesions of the conjunctiva have been described. As has been described in the cutaneous literature, the spectrum between keratoacanthoma and squamous cell carcinoma, inverted papillomas, and recurrent papillomas in adults is poorly delineated. The paucity of these possibly benign squamous cell proliferations and their limited follow-up make it difficult to draw conclusions about their management.57–60 In the few that the author has, on the basis of very little data, the tendency has been to manage them as if they were squamous cell carcinomas.

SQUAMOUS CELL CARCINOMA

The nomenclature of conjunctival epithelial neoplasia is confusing. McGavic coined the term “Bowen’s disease” for this condition, since in situ conjunctival dysplasias have bizarre cells, often with giant nuclei or multinucleated configurations.61 Unfortunately, this has proven to be a misnomer. Bowen’s disease was initially thought to be a skin cancer associated with visceral malignancy, but more recent studies have cast doubts on that association.62,63

Most clinicians now use the term “conjunctival intraepithelial neoplasia” (CIN) for what was previously referred to as Bowen’s disease, conjunctival dysplasia, or conjunctival epithelioma.64–67 CIN usually connotes partialto full-thickness intraepithelial neoplasia, whereas carcinoma has full-thickness involvement with invasion. Neither CIN nor invasive conjunctival squamous cell carcinoma is associated with visceral malignancies.63,68 Several workers

Figure 4–4. Amyloid involvement of the conjunctiva. The patient had no systemic abnormalities