Treatment of Complex Choristoma by Excision and Amniotic Membrane

A B

C D

Figure 5. A, Findings from histopathologic examination reveal the ciliary body mass (hematoxylin-eosin, original magnification ϫ50). B, The poorly differentiated carcinoma is composed of nonpigmented pleomorphic cells (hematoxylin-eosin, original magnification ϫ100). C, Immunohistochemical stain showing marked reactivity (brown staining) for prostate-specific antigen by tumor (original magnification ϫ50). D, Immunohistochemical stain showing nonreactivity for HMB-45 and melan-A (original magnification ϫ50).

of prostatic adenocarcinoma meta- fully by excision and amniotic mem- static to the ciliary body. Treatment of brane transplantation. Complex Choristoma Shady El-Zayaty, BS Report of Cases. Case 1. A Susan Schneider, MD by Excision and Amniotic Membrane 3-month-old girl had a history of a George K. Mutema, MD, PhD white spot in the left eye that was no- Transplantation James J. Augsburger, MD ticed by her parents on the second Cincinnati, Ohio day after birth. The child was deliv- Epibulbar dermoids are choristomas ered after a full-term pregnancy with This study was supported by an un- that generally arise from the limbus a cesarean delivery. No other re- restricted grant from Research to Pre- and are present at birth.1 Based on markable ocular or systemic medi- vent Blindness, New York, NY. their configuration and the histo- cal history was elicited. Formal vi- Corresponding author and re- logic contents of the lesion, they are sual acuity measurement was not prints: Susan Schneider, MD, Mary classified as dermoids, lipoder- possible; however, the child could Knight Asbury Eye Pathology Labo- moids, or complex choristomas.1,2 fix and follow a source of light. The ratory, University of Cincinnati, They could be either sporadic or as- fixation was steady, central, and A-Pavilion, Room 220, 234 Good- sociated with systemic anomalies like maintained. Ocular motility was full. man St, Cincinnati, OH 45219-0777 Goldenhar syndrome, neurocutane- On external examination there was (e-mail: [email protected]). ous syndromes, or phakomatosis.3 a soft, vascularized mass involving 1. BoringCC,SquiresTS,TongT,MontgomeryS.Can- Though they have almost no inde- the left temporal bulbar conjunc- cer statistics 1994. CA Cancer J Clin. 1994;44:7-26. pendent growth potential, the large tiva extending to the temporal half 2. Dijkman GA, Debrune FMJ. Epidemiology of lesions can protrude through the lid of cornea and encroaching on the prostate cancer. Eur Urol. 1996;30:281-295. 3. Keizur J, Kane C, North R, Leidich R. Adeno- aperture causing mechanical obstruc- pupil. Findings on the left eye were carcinoma of the prostate metastatic to the cho- tion to lid closure and can be associ- otherwise unremarkable. Examina- roid of the eye. Prostate. 1995;27:336-339. 4. Shields C, Shields J, Gross N, Schwartz G, Lally ated with a high degree of corneal tion findings of the right eye were S. Survey of 520 eyes with uveal metastases. Oph- astigmatism, posing a potential threat normal. A diagnosis of dermoli- thalmology. 1997;104:1265-1276. of amblyopia in infants.1-4 We report poma was made, and excision of the 5. De Potter P, Shields CL, Shields JA, Tardio DJ. Uveal metastasis from prostate carcinoma. Can- 2 rare cases of isolated complex cho- mass was advised in view of the risk cer. 1993;71:2791-2796. ristomas that were treated success- of amblyopia. The parents deferred

(REPRINTED) ARCH OPHTHALMOL / VOL 121, FEB 2003 WWW.ARCHOPHTHALMOL.COM 278

©2003 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/27/2021 surgery at that time but 3 months A B later, they brought the child back with the complaint that the mass had increased. The mass was larger in size, soft, vascular, and involved two thirds of cornea, obscuring the pupillary area (Figure 1A). The child had a strong fixation preference with the right eye and poor fixation with the left eye. The central fundus was normal in the right eye while fun- Figure 1. A, The clinical picture of a 3-month-old infant with a yellow-gray soft lesion resembling a dus examination of the left eye was lipodermoid, encroaching to the pupillary margin. B, The postoperative picture of the child showing an not possible. intact ocular surface at 6 months after surgery shows peripheral corneal scarring. The lesion was excised in toto under general anesthesia. After excision of the mass, a conjunctival- limbal graft (CLAG) was harvested from the nasal and upper nasal area and was sutured on top of the amniotic graft between the 2- and 4-o’clock positions. The limbal side of the graft was sutured at the limbus with 10-0 monofilament interrupted sutures. The peripheral edge of the graft was anchored to the conjunctiva with 8-0 interrupted Vic- ryl sutures. A bandage contact lens was inserted after surgery. Postop- eratively, steroid and antibiotic drops Figure 2. The histologic section of the lesion shows a lining epithelium of stratified squamous and columnar cells with few goblet cells. There are lobules of lacrimal gland, cartilage, adipose tissue, and were administered and all sutures ϫ were removed after 1 month, at smooth muscle bundles (hematoxylin-eosin, original magnification 125). which time the ocular surface had Under general anesthesia, the fied squamous as well as columnar healed and the amniotic membrane mass was excised piecemeal, and the epithelium-containing goblet cells. had disintegrated. Seven months corneal surface was smoothened with The underlying stroma contained postoperatively, there was scar for- a No. 15 surgical blade on a Bard lacrimal gland elements, lobules of mation with no evidence of recur- Parker knife (Khosla Eye Instru- adipose tissue, cartilage, nerve rence (Figure 1B). The child was ments, Mumbai, India), cauterizing bundles, and bundles of smooth fixing light poorly with the left eye the prominent blood vessels. The sur- muscle (Figure 2). In the second and is now receiving amblyopia face was covered with 2 layers of pre- case, there were lymphoid follicles therapy. served human amniotic membrane with germinal centers in the subepi- Case 2. A 3-year-old girl was with stromal side down, which was thelial stroma with mild diffuse seen with a history of a white spot sutured to the temporal limbus and lymphoplasmacytic infiltrates in the in the black of the right eye since to the corneal surface nasally with cir- surrounding stroma. birth. She was a healthy, full-term cumferential 10-0 monofilament ny- child from a consanguineous mar- lon sutures. Postoperatively, steroid Comment. Dermoids are fairly com- riage. Her visual acuity could not and antibiotic drops were adminis- mon limbal lesions that are classi- be evaluated. Refraction revealed an tered for 1 week and the loose su- fied as choristomas because they error of +4 diopter (D)/+3 D cylin- tures were removed 2 weeks later un- contain displaced epithelial and der- der in the right eye and +0.5 D cyl- der general anesthesia. Three weeks mis-like elements normally not found inder in the left eye. There was a after surgery, the amniotic mem- in these areas.3 There are 3 types of right convergent squint; ocular brane was in place and had covered dermoids recognized: (1) the solid, movements were full in both eyes. the cornea well. On last follow-up, well-defined limbal dermoid; (2) the Anterior segment evaluation of the about 8 weeks after surgery, the eye more diffuse dermolipoma; or (3) the right eye revealed a whitish mass was quiet and there was corneal scar- complex choristomas that contain with patches of keratinization cov- ring involving the temporal cornea. more than 2 elements of ectopic tis- ering the temporal half of the cor- The child was advised to follow-up sues (lacrimal gland tissue, nerve nea and extending to the lateral at the pediatric ophthalmology clinic bundles, cartilage, bone, etc). It is be- fornix and superiorly to the supe- for amblyopia treatment. lieved that epibulbar choristomas rior and medial fornix. With a prob- share a common origin from ectopic able diagnosis of lipodermoid, ex- Histopathologic Characteristics. pluripotential cells capable of devel- cision with lamellar keratoplasty was Both lesions showed similar fea- oping into either a complex growth planned. tures and were covered by strati- composed of several elements or le-

(REPRINTED) ARCH OPHTHALMOL / VOL 121, FEB 2003 WWW.ARCHOPHTHALMOL.COM 279

©2003 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/27/2021 sions containing a predominance of limbal autograft, is an alternative sive neoplasm that rarely invades the a single tissue.1 The complex choris- modality to achieve good ocular eyeororbit.1,2 Immunosuppressedpa- toma can be isolated or associated surface construction. With timely in- tients are at increased risk to develop with linear nevus sebaceous syn- tervention, amblyopia may be pre- conjunctival squamous cell carcino- drome.4 We report 2 rare cases of iso- vented or treated early. The histo- ma.3 Tumors in immunosuppressed lated complex choristoma that were logic confirmation of an unsuspected patients can behave aggressively.3 We treated with amniotic membrane complex choristoma warrants a mul- describe a patient receiving immuno- grafting, supplemented with CLAG in tidisciplinary approach. suppression treatment after liver 1 case. Thorough examination of both transplantation,whodevelopedanag- cases failed to reveal any associated Virender S. Sangwan, MS gressive conjunctival squamous cell ocular or neurologic abnormalities. M. S. Sridhar, MD carcinoma that caused his death Treatment of dermoid de- Geeta K. Vemuganti, MD within 1 year of diagnosis. pends on the size, location, and the Hyderabad, India mechanical effects of the lesion on Report of a Case. A 56-year-old man the surrounding structures. Most This work was funded by the was referred for additional therapy dermoids are 2 to 3 mm, but they Hyderabad Eye Research Foundation. after a biopsy of a conjunctival le- may sometimes be large (12-15 We thank the Ramayamma Eye sion revealed a poorly differenti- mm), causing mechanical obstruc- Bank, L. V. Prasad Eye Institute, ated invasive squamous cell carci- tion or corneal astigmatism. Many Hyderabad, India, for providing us noma that had been incompletely of the reported cases were treated by the preserved human amniotic mem- excised. His medical history in- excision, though some were treated brane. cluded a liver transplantation for with a combination of lamellar or Corresponding author and chronic sclerosing cholangitis 3 years penetrating keratoplasty2,4; how- reprints: Geeta K. Vemuganti, MD, previously and treatment with im- ever, the results of both of these pro- Ophthalmic Pathology Service, L.V. munosuppressive drugs (azathio- cedures are not encouraging and Prasad Eye Institute, L.V. Prasad prine and tacrolimus). have led to failed grafts. Marg, Banjara Hills, Hyderabad On examination, his visual acu- The general principles of treat- 500034, India (e-mail: geeta@lvpeye ity was 20/20 OU. The left eye was un- ment of limbal-based ocular sur- .stph.net). remarkable. The right eye showed a face lesions include wide excision of temporal conjunctival wound with-

the lesion over cornea, conjunc- 1. Pokorny KS, Hyman BM, Jakobiec FA, Perry HD, out clinical evidence of tumor. Based tiva, and sclera, followed by alco- Caputo AR, Iwamoto T. Epibulbar choristoma on a review of the previous histo- hol epitheliectomy, wide resection containing lacrimal tissue: clinical distinction pathologic findings, wide surgical ex- 5 from dermoids and histologic evidence of an ori- cryotherapy, or closure. Amniotic gin from the palpebral lobe. Ophthalmology. 1987; cision of the wound, double freeze- membrane has also been used as an 94:1249-1257. thaw cryotherapy, and superficial adjunct procedure in treating dif- 2. Mansour AM, Barber JC, Reinecke RD, Wang FM. alcohol keratectomy were performed Ocular choristomas. Surv Ophthalmol. 1989;33: fuse ocular surface neoplasias for 339-358. to remove residual microscopic dis- surface reconstruction after exci- 3. SpencerWH.Conjunctiva.In:SpencerWH,ed.Oph- ease and prevent recurrence. Histo- 6 thalmic Pathology: An Atlas and Text Book. 4th ed. sion of the lesion. Keeping in mind Philadelphia, Pa: WB Saunders Co; 1996:48-56. pathologic examination showed a the large size of the lesion and the 4. Duncan JL, Golabi M, Fredrick DR, et al. Complex completely resected tumor with un- age of the patient, these 2 cases were limbal choristomas in linear nevus sebaceous syn- involved surgical margins. drome. Ophthalmology. 1998;105:1459-1465. treated with excision and amniotic 5. Shields JA, Shields CL, DePotter P. Surgical man- Six months later, the patient membrane transplantation. In case agement of conjunctival tumors: the 1994 Lynn developed sudden redness and 1, CLAG was used to prevent lim- B. McMahan Lecture. Arch Ophthalmol. 1997; swelling of the right upper eyelid. 115:808-815. bal stem cell deficiency. In case 2, 6. Tseng SCG, Prabhasawat P, Lee SH. Amniotic An external examination showed two layers of amniotic membrane membrane transplantation for conjunctival sur- hemorrhagic axial proptosis and a face reconstruction. Am J Ophthalmol. 1997;124: were used for better results (as 765-774. palpable firm mass near the su- proven in the treatment of deep cor- 7. Kruse FE, Rohrschneider K, Volcker HE. Mul- perolateral margin of the orbit neal and scleral ulcers) with the in- tilayered amniotic membrane transplantation for (Figure 1). Ocular motility was reconstruction of deep corneal ulcers. Ophthal- tention of providing collagen layer mology. 1999;106:1504-1511. restricted in all gazes and corneal supplementation, basement mem- sensation was intact. Magnetic brane reconstruction, promotion of resonance imaging of the brain and epithelialization, and wound heal- orbits disclosed a diffuse, enhanc- ing.7 In both cases, visual potential ing mass that extended along the was hampered by the development lateral wall of the right orbit from of amblyopia; therefore, the pa- the rim to the apex (Figure 2), tients were advised to undergo an- Aggressive Conjunctival without radiologic evidence of cav- tiamblyopia therapy. Squamous Cell Carcinoma ernous sinus thrombosis. Based on our experience with in a Patient Following An orbital exenteration was these 2 cases, we believe that surgical Liver Transplantation performed using an eyelid-splitting excision of large dermoids followed technique after an incisional bi- by amniotic membrane transplan- Squamous cell carcinoma of the con- opsy revealed poorly differentiated tation, with or without conjunctival- junctiva is usually a slowly progres- squamous cell carcinoma. The small,

(REPRINTED) ARCH OPHTHALMOL / VOL 121, FEB 2003 WWW.ARCHOPHTHALMOL.COM 280