CASE REPORT
Conservative Surgical Management of a Grade III Corneal Dermoid _ Ali S¸ ims¸ek, MD,* S¸ emsettin Bilak, MD,* Lokman Balyen, MD,† and Ibrahim Halil Erdo g du, MD‡
Purpose: To present a rare, unique, grade III corneal dermoid treated with conservative surgical management.
Methods: An 8-month-old boy was admitted to our clinic with a lesion on the central cornea of the right eye and a corneal mass in the left eye. The mass had a skin-like surface and protruded to the outside of the palpebral fissure. MRI (magnetic resonance imaging) of the orbits showed a large cyst covering most of the cornea and the absence of the anterior chamber and lens. The dermoid was excised and sent for histopathological examination. The cornea was reconstructed using the partial thickness scleral graft. Results: The histopathology report confirmed the diagnosis of a grade III corneal dermoid. On examination 1 year after the surgery, the orbital and globe volumes were the same, and the intraocular pressure was normal. Conclusions: This is likely the first report of an unusual case in which a grade III corneal dermoid with an anterior staphyloma was treated by anterior segment reconstruction using an autologous partial thickness scleral graft. We believe that this technique has the advantage that it stimulates orbital and facial development and has good cosmetic results. Key Words: large corneal dermoid, conservative surgery, lamellar scleral graft (Cornea 2015;34:1318–1320)
D
ermoids are rare congenital choristomas.1 Choristomas are congenital tumors that consist of overgrowth of normal tissue in an abnormal location and occur in 1 to 3 of every 10,000 newborns.1 Dermoids can occur over the cornea, limbus, or conjunctiva. They can be unilateral or bilateral and have a single lesion or multiple lesions. They most commonly localize at the inferotemporal limbus and can involve the central cornea or can form a ring around the limbus and often have satellite lesions.2,3 Usually, they present as round ovoid,
Received for publication March 31, 2015; revision received May 12, 2015; accepted May 14, 2015. Published online ahead of print July 6, 2015. From the *Department of Ophthalmology, School of Medicine, Adıyaman University, Adıyaman, Turkey; †Department of Ophthalmology, Baglar Hospital, Diyarbakır, Turkey; and ‡Department of Pathology, School of Medicine, Adıyaman University, Adıyaman, Turkey. The authors have no funding or conflicts of interest to disclose. Reprints: S¸emsettin Bilak, MD, Department of Ophthalmology, School of Medicine, Adıyaman University, Adıyaman 02200, Turkey (e-mail: [email protected]). Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved.
1318
| www.corneajrnl.com
yellowish white, solid vascularized nodules with a dome-like shape. We discuss an interesting case of a grade III central corneal dermoid treated with a conservative surgical approach.
CASE REPORT An 8-month-old boy was admitted to our clinic with a lesion on the central cornea of the right eye and a corneal mass in the left eye. The mass had a skin-like surface and protruded outside the palpebral fissure. There were no other findings on systemic examination. During the pregnancy, there was no history of disease, medication, or exposure to radiation. He was born spontaneously and without complication after full-term pregnancy. There was no family history and no intermarriage between the parents. On ocular examination, the patient’s visual acuity was perception of light by the right eye and no perception of light by the left eye. A biomicroscopic evaluation revealed central corneal opacity of the right eye and a large corneal mass covering most of the corneal diameter of the left eye. The mass was 16 · 12 mm in size, painless, soft, and cystic. The optic disc, choroid, and retina were normal on a B-mode ultrasound scan. MRI of the orbits showed the cystic nature of the mass and the unformed anterior chamber and lens. Our prediagnosis was a grade I–II central corneal dermoid in the right eye and a grade III corneal dermoid with a staphyloma in the left eye (Fig. 1), according to the Mann4 classification. We planned penetrating keratoplasty for the right eye and total anterior segment reconstruction for the left eye. Under general anesthesia, complete 360-degree peritomy was performed, and the conjunctiva of the left eye was dissected carefully. Hemostasis was obtained as needed with cautery. The excision diameter of the mass was determined as 1 mm posterior from the mass border. This diameter measured 9.5 · 9.5-mm in size with a caliper. A half-thickness autologous scleral lamellar graft was dissected with a crescent knife after lamellar trephination from the temporal superior sclera. The scleral lamellar graft was oversized (10 · 10 mm) to maximize tissue apposition. The full-thickness excision of the mass was made with corneal scissors and sent for histopathological examination. The vitreous was removed using the open-sky vitrectomy technique, and the excision rim was filled with sodium hyaluronate viscoelastic material. As in penetrating keratoplasty technique, the scleral lamellar graft was brought into the excision rim and sutured using 7-0 vicryl sutures. The tonus of the globe was achieved using sodium hyaluronate viscoelastic material. A 12 · 12-mm single pedicle conjunctival flap from the superonasal area was used to cover the surface of the scleral graft and corneal area and was sutured to the sclera using 8-0 vicryl. The histopathology report confirmed the diagnosis of a grade III corneal dermoid in the left eye. Postoperatively, we followed up the patient monthly and observed no complications. A B-mode ultrasound scan showed the normal structure of the retinal and choroidal tissues (Fig. 2). On examination 1 year after surgery, the orbital and globe volumes were the same, and the intraocular pressure was 14 mm Hg in the left eye, Cornea Volume 34, Number 10, October 2015
Copyright © 2015 Wolters Kluwer Health, Inc. Unauthorized reproduction of this article is prohibited.
Cornea Volume 34, Number 10, October 2015
FIGURE 1. A, Clinical photograph of the patient with a large central corneal dermoid in the left eye. B, Clinical photograph of the patient 1 year after surgery. as measured by an Icare PRO rebound tonometer. This may be the result of the normal development and function of the ciliary body. Thirteen months after surgery, penetrating keratoplasty was performed on the right eye. Both globes remained the same size at 18 months. We planned a custom ocular prosthesis to improve the cosmetic appearance of the left eye.
DISCUSSION Dermoids can occur alone or in combination with syndromes such as the Goldenhar syndrome, Schimmelpenning-Feuerstein-Mims phakomatosis, and epidermal nevus syndrome.5 A dermoid may be defined as a solid congenital tumor mainly consisting of collagenous connective tissue. It can contain ectodermal derivatives, such as hair follicles, sebaceous glands, sweat glands, and nerve tissue embedded in connective tissue and covered by squamous epithelium.3,5 In our case, histopathology of the mass confirmed an unusual corneal dermoid with a lack of adipose tissue and showed thickened stromal collagen bundles covered by keratinized stratified squamous epithelium (Fig. 3). Immature hair follicles and derivatives embedded in the stroma were seen in some areas. In addition, corneal endothelium and a Descemet membrane were present in some areas but were not persistent in the all histopathological sections. Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved.
Grade III Corneal Dermoid
FIGURE 2. A, Preoperative B-mode ultrasound scan showing the intact globe with the normal choroidal and retinal structure. B, Transverse magnetic resonance image of the orbits showing the round cystic mass with the unformed anterior chamber and lens.
Mann4 anatomically classified corneal dermoids into 3 types. A type III dermoid is the most severe and the rarest. It is characterized by total involvement of the entire anterior segment and is associated with abnormalities such as microphthalmus and posterior segment abnormalities. According to Duke-Elder,6 type III dermoids are divided into 2 subtypes according to the time of development of the anomaly—before or during lens formation. If the anomaly develops before lens formation, it is accompanied by the lack of an anterior chamber, iris, and lens, and the whole eye becomes grossly microphthalmic. If the anomaly develops during lens formation, it is accompanied by an ill-formed lens, rudiments of an iris, but by formed anterior chamber. In both types, the entire anterior segment is totally replaced by a fibro-fatty mass protruding outside the palpebral fissure. In our case, there was no anterior chamber, iris, or lens. Although anterior segment development of the globe was abnormal, it was not microphthalmic. Grade III corneal dermoids with no limbal or conjunctival involvement and without microphthalmus components have not been reported in the literature. To our knowledge, after reviewing the small number of pure large corneal dermoids reported in the literature, if there www.corneajrnl.com |
1319
Copyright © 2015 Wolters Kluwer Health, Inc. Unauthorized reproduction of this article is prohibited.
S¸ ims¸ek et al
Cornea Volume 34, Number 10, October 2015
face gains 90% of its adult dimensions; until this age, bony orbital growth and overall facial development are incomplete.8 Therefore, children younger than 5 years old require an implant that increases in size to stimulate orbital and facial development. These implants can cause many complications and require numerous interventions.8 In our case, the eye was not microphthalmic. However, the anterior chamber and lens were not formed, and the cornea was opaque and vascularized. Therefore, we did not plan penetrating keratoplasty. If a severe and unilateral grade III dermoid accompanied by an anterior staphyloma was present, a keratoprosthesis would have been performed.1 Because our concern for this patient was only in terms of cosmetic appearance, we decided to perform anterior segment reconstruction with an autologous scleral lamellar graft, as used to treat lower-grade dermoids.5 To the best of our knowledge, the present case is the first in which a grade III corneal dermoid with an anterior staphyloma was treated by anterior segment reconstruction using an autologous scleral lamellar graft. This technique has the advantage that it stimulates orbital and facial development and has good cosmetic results.
REFERENCES
is a grade III dermoid accompanied by microphthalmus, the surgical plan is usually an excision of the eye and mass together.1–3,7 By approximately 5.5 years of age, the pediatric
1. Mansour AM, Barber JC, Reinecke RD, et al. Ocular choristomas. Surv Ophthalmol. 1989;33:339–358. 2. Mohammad AE, Kroosh SS. Huge corneal dermoid in a well-formed eye: a case report and review of the literature. Orbit. 2002;21:295–299. 3. Kadri R, Kudva AA, Achar A, et al. Corneal dermoid (Case report). Online J Health Allied Sci. 2011;10:23. Available at: Light House Polyclinic, Light House Hill Road, Mangalore, India. Accessed February 15, 2015. 4. Mann I. Developmental Abnormalities of the Eye. Philadelphia, PA: Lippincott; 1957:357–361. 5. Stergiopoulos P, Link B, Naumann GO, et al. Solid corneal dermoids and subconjunctival lipodermoids: impact of differentiated surgical therapy on the functional long-term outcome. Cornea. 2009;28:644–651. 6. Duke-Elder S. Congenital deformities of the eye: II. Anomalies of differentiation. In: Duke-Elder S, ed. System of Ophthalmology. Volume III. Normal and Abnormal Development. Part 2: Congenital Deformities. St. Louis, MO: Mosby; 1963:512–539. 7. Roncević MB, Dorešić JP. Large congenital corneal dermoid with spontaneous partial regression: the first report. Cornea. 2011;30:219–221. 8. Chen D, Heher K. Management of the anophthalmic socket in pediatric patients. Curr Opin Ophthalmol. 2004;15:449–453.
1320
Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved.
FIGURE 3. Histopathology sections showing (A) increased stromal dense collagen bundles in thickened stroma (arrow), covered by a keratinized stratified squamous epithelium (H&E stain, ·10). B, Immature adnexal structures in the substantia propria (arrow) (vimentin stain, ·4).
| www.corneajrnl.com
Copyright © 2015 Wolters Kluwer Health, Inc. Unauthorized reproduction of this article is prohibited.
Conservative Surgical Management of a Grade III Corneal Dermoid _ Ali S¸ ims¸ek, MD,* S¸ emsettin Bilak, MD,* Lokman Balyen, MD,† and Ibrahim Halil Erdo g du, MD‡
Purpose: To present a rare, unique, grade III corneal dermoid treated with conservative surgical management.
Methods: An 8-month-old boy was admitted to our clinic with a lesion on the central cornea of the right eye and a corneal mass in the left eye. The mass had a skin-like surface and protruded to the outside of the palpebral fissure. MRI (magnetic resonance imaging) of the orbits showed a large cyst covering most of the cornea and the absence of the anterior chamber and lens. The dermoid was excised and sent for histopathological examination. The cornea was reconstructed using the partial thickness scleral graft. Results: The histopathology report confirmed the diagnosis of a grade III corneal dermoid. On examination 1 year after the surgery, the orbital and globe volumes were the same, and the intraocular pressure was normal. Conclusions: This is likely the first report of an unusual case in which a grade III corneal dermoid with an anterior staphyloma was treated by anterior segment reconstruction using an autologous partial thickness scleral graft. We believe that this technique has the advantage that it stimulates orbital and facial development and has good cosmetic results. Key Words: large corneal dermoid, conservative surgery, lamellar scleral graft (Cornea 2015;34:1318–1320)
D
ermoids are rare congenital choristomas.1 Choristomas are congenital tumors that consist of overgrowth of normal tissue in an abnormal location and occur in 1 to 3 of every 10,000 newborns.1 Dermoids can occur over the cornea, limbus, or conjunctiva. They can be unilateral or bilateral and have a single lesion or multiple lesions. They most commonly localize at the inferotemporal limbus and can involve the central cornea or can form a ring around the limbus and often have satellite lesions.2,3 Usually, they present as round ovoid,
Received for publication March 31, 2015; revision received May 12, 2015; accepted May 14, 2015. Published online ahead of print July 6, 2015. From the *Department of Ophthalmology, School of Medicine, Adıyaman University, Adıyaman, Turkey; †Department of Ophthalmology, Baglar Hospital, Diyarbakır, Turkey; and ‡Department of Pathology, School of Medicine, Adıyaman University, Adıyaman, Turkey. The authors have no funding or conflicts of interest to disclose. Reprints: S¸emsettin Bilak, MD, Department of Ophthalmology, School of Medicine, Adıyaman University, Adıyaman 02200, Turkey (e-mail: [email protected]). Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved.
1318
| www.corneajrnl.com
yellowish white, solid vascularized nodules with a dome-like shape. We discuss an interesting case of a grade III central corneal dermoid treated with a conservative surgical approach.
CASE REPORT An 8-month-old boy was admitted to our clinic with a lesion on the central cornea of the right eye and a corneal mass in the left eye. The mass had a skin-like surface and protruded outside the palpebral fissure. There were no other findings on systemic examination. During the pregnancy, there was no history of disease, medication, or exposure to radiation. He was born spontaneously and without complication after full-term pregnancy. There was no family history and no intermarriage between the parents. On ocular examination, the patient’s visual acuity was perception of light by the right eye and no perception of light by the left eye. A biomicroscopic evaluation revealed central corneal opacity of the right eye and a large corneal mass covering most of the corneal diameter of the left eye. The mass was 16 · 12 mm in size, painless, soft, and cystic. The optic disc, choroid, and retina were normal on a B-mode ultrasound scan. MRI of the orbits showed the cystic nature of the mass and the unformed anterior chamber and lens. Our prediagnosis was a grade I–II central corneal dermoid in the right eye and a grade III corneal dermoid with a staphyloma in the left eye (Fig. 1), according to the Mann4 classification. We planned penetrating keratoplasty for the right eye and total anterior segment reconstruction for the left eye. Under general anesthesia, complete 360-degree peritomy was performed, and the conjunctiva of the left eye was dissected carefully. Hemostasis was obtained as needed with cautery. The excision diameter of the mass was determined as 1 mm posterior from the mass border. This diameter measured 9.5 · 9.5-mm in size with a caliper. A half-thickness autologous scleral lamellar graft was dissected with a crescent knife after lamellar trephination from the temporal superior sclera. The scleral lamellar graft was oversized (10 · 10 mm) to maximize tissue apposition. The full-thickness excision of the mass was made with corneal scissors and sent for histopathological examination. The vitreous was removed using the open-sky vitrectomy technique, and the excision rim was filled with sodium hyaluronate viscoelastic material. As in penetrating keratoplasty technique, the scleral lamellar graft was brought into the excision rim and sutured using 7-0 vicryl sutures. The tonus of the globe was achieved using sodium hyaluronate viscoelastic material. A 12 · 12-mm single pedicle conjunctival flap from the superonasal area was used to cover the surface of the scleral graft and corneal area and was sutured to the sclera using 8-0 vicryl. The histopathology report confirmed the diagnosis of a grade III corneal dermoid in the left eye. Postoperatively, we followed up the patient monthly and observed no complications. A B-mode ultrasound scan showed the normal structure of the retinal and choroidal tissues (Fig. 2). On examination 1 year after surgery, the orbital and globe volumes were the same, and the intraocular pressure was 14 mm Hg in the left eye, Cornea Volume 34, Number 10, October 2015
Copyright © 2015 Wolters Kluwer Health, Inc. Unauthorized reproduction of this article is prohibited.
Cornea Volume 34, Number 10, October 2015
FIGURE 1. A, Clinical photograph of the patient with a large central corneal dermoid in the left eye. B, Clinical photograph of the patient 1 year after surgery. as measured by an Icare PRO rebound tonometer. This may be the result of the normal development and function of the ciliary body. Thirteen months after surgery, penetrating keratoplasty was performed on the right eye. Both globes remained the same size at 18 months. We planned a custom ocular prosthesis to improve the cosmetic appearance of the left eye.
DISCUSSION Dermoids can occur alone or in combination with syndromes such as the Goldenhar syndrome, Schimmelpenning-Feuerstein-Mims phakomatosis, and epidermal nevus syndrome.5 A dermoid may be defined as a solid congenital tumor mainly consisting of collagenous connective tissue. It can contain ectodermal derivatives, such as hair follicles, sebaceous glands, sweat glands, and nerve tissue embedded in connective tissue and covered by squamous epithelium.3,5 In our case, histopathology of the mass confirmed an unusual corneal dermoid with a lack of adipose tissue and showed thickened stromal collagen bundles covered by keratinized stratified squamous epithelium (Fig. 3). Immature hair follicles and derivatives embedded in the stroma were seen in some areas. In addition, corneal endothelium and a Descemet membrane were present in some areas but were not persistent in the all histopathological sections. Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved.
Grade III Corneal Dermoid
FIGURE 2. A, Preoperative B-mode ultrasound scan showing the intact globe with the normal choroidal and retinal structure. B, Transverse magnetic resonance image of the orbits showing the round cystic mass with the unformed anterior chamber and lens.
Mann4 anatomically classified corneal dermoids into 3 types. A type III dermoid is the most severe and the rarest. It is characterized by total involvement of the entire anterior segment and is associated with abnormalities such as microphthalmus and posterior segment abnormalities. According to Duke-Elder,6 type III dermoids are divided into 2 subtypes according to the time of development of the anomaly—before or during lens formation. If the anomaly develops before lens formation, it is accompanied by the lack of an anterior chamber, iris, and lens, and the whole eye becomes grossly microphthalmic. If the anomaly develops during lens formation, it is accompanied by an ill-formed lens, rudiments of an iris, but by formed anterior chamber. In both types, the entire anterior segment is totally replaced by a fibro-fatty mass protruding outside the palpebral fissure. In our case, there was no anterior chamber, iris, or lens. Although anterior segment development of the globe was abnormal, it was not microphthalmic. Grade III corneal dermoids with no limbal or conjunctival involvement and without microphthalmus components have not been reported in the literature. To our knowledge, after reviewing the small number of pure large corneal dermoids reported in the literature, if there www.corneajrnl.com |
1319
Copyright © 2015 Wolters Kluwer Health, Inc. Unauthorized reproduction of this article is prohibited.
S¸ ims¸ek et al
Cornea Volume 34, Number 10, October 2015
face gains 90% of its adult dimensions; until this age, bony orbital growth and overall facial development are incomplete.8 Therefore, children younger than 5 years old require an implant that increases in size to stimulate orbital and facial development. These implants can cause many complications and require numerous interventions.8 In our case, the eye was not microphthalmic. However, the anterior chamber and lens were not formed, and the cornea was opaque and vascularized. Therefore, we did not plan penetrating keratoplasty. If a severe and unilateral grade III dermoid accompanied by an anterior staphyloma was present, a keratoprosthesis would have been performed.1 Because our concern for this patient was only in terms of cosmetic appearance, we decided to perform anterior segment reconstruction with an autologous scleral lamellar graft, as used to treat lower-grade dermoids.5 To the best of our knowledge, the present case is the first in which a grade III corneal dermoid with an anterior staphyloma was treated by anterior segment reconstruction using an autologous scleral lamellar graft. This technique has the advantage that it stimulates orbital and facial development and has good cosmetic results.
REFERENCES
is a grade III dermoid accompanied by microphthalmus, the surgical plan is usually an excision of the eye and mass together.1–3,7 By approximately 5.5 years of age, the pediatric
1. Mansour AM, Barber JC, Reinecke RD, et al. Ocular choristomas. Surv Ophthalmol. 1989;33:339–358. 2. Mohammad AE, Kroosh SS. Huge corneal dermoid in a well-formed eye: a case report and review of the literature. Orbit. 2002;21:295–299. 3. Kadri R, Kudva AA, Achar A, et al. Corneal dermoid (Case report). Online J Health Allied Sci. 2011;10:23. Available at: Light House Polyclinic, Light House Hill Road, Mangalore, India. Accessed February 15, 2015. 4. Mann I. Developmental Abnormalities of the Eye. Philadelphia, PA: Lippincott; 1957:357–361. 5. Stergiopoulos P, Link B, Naumann GO, et al. Solid corneal dermoids and subconjunctival lipodermoids: impact of differentiated surgical therapy on the functional long-term outcome. Cornea. 2009;28:644–651. 6. Duke-Elder S. Congenital deformities of the eye: II. Anomalies of differentiation. In: Duke-Elder S, ed. System of Ophthalmology. Volume III. Normal and Abnormal Development. Part 2: Congenital Deformities. St. Louis, MO: Mosby; 1963:512–539. 7. Roncević MB, Dorešić JP. Large congenital corneal dermoid with spontaneous partial regression: the first report. Cornea. 2011;30:219–221. 8. Chen D, Heher K. Management of the anophthalmic socket in pediatric patients. Curr Opin Ophthalmol. 2004;15:449–453.
1320
Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved.
FIGURE 3. Histopathology sections showing (A) increased stromal dense collagen bundles in thickened stroma (arrow), covered by a keratinized stratified squamous epithelium (H&E stain, ·10). B, Immature adnexal structures in the substantia propria (arrow) (vimentin stain, ·4).
| www.corneajrnl.com
Copyright © 2015 Wolters Kluwer Health, Inc. Unauthorized reproduction of this article is prohibited.
