Original Article

Clinical features and surgical management of dermolipomas

Alan A. McNab FRACO Orbital Clinic, Moorfields Eye Hospital, London John E. Wright MD, FRCS Orbital Clinic, Moorfields Eye Hospital, London Anthony G. Caswell FRCS Orbital Clinic, Moorfields Eye Hospital, London

Abstract
Dermolipomas are solid choristomas which present as a lateral canthal mass. A series of 35 cases is reported, 27 of whom were female. Six patients had features of Goldenhars syndrome. Proximity of the dermolipoma to the lacrimal ductules, the lateral rectus and upper lid structures makes extensive surgical excision dangerous. A microsurgical technique of partial excision of the lesion is described and was used successfully in 13 cases without significant complications.

Key words: Dermolipoma, Goldenhars syndrome.

Dermolipomas are uncommon tumours. They have a characteristic appearance and patients usually complain of a cosmetic blemish. Direct excision with the overlying conjunctiva often causes significant complications. We present a series of 35 patients and describe a technique of microsurgical removal which has proved safe and effective.

Patients and methods Thirty-five patients with dermolipomas seen in the Orbital Clinic at Moorfields Eye Hospital between 1970 and 1988 were reviewed. In all patients a detailed history was obtained and a full ocular examination performed. In particular, features of Goldenhars syndrome were sought.

The diagnosis was made on the basis of the characteristic appearance of the tumour (Figure 1). Surgery was offered to those patients who considered the lump to be a significant cosmetic blemish. Only that part of the lesion that was visible anteriorly was removed (Figure 2), so that in all except three cases a subtotal excision was performed. The microsurgical technique began with a lateral peritomy with relieving incisions directed superolaterally and inferolaterally (Figure 3). The conjunctiva was carehlly dissected free of Tenons capsule leaving this layer and the underlying lateral rectus undisturbed. The openings of the lacrimal ductules in the superolateral fornix were identified, sometimes with the aid of a drop of 2% fluorescein. Their position marked the maximal extent of the dissection superolaterally. Having reached the anterior edge of the dermolipoma, its subepithelial components were removed by sharp dissection leaving the overlying epithelial surface intact. In some patients a small ellipse of closely adherent squamous epithelium was removed. Only those parts that were readily visible in the palpebral aperture were excised. The conjunctiva was closed with an absorbable 6/0 suture and antibiotic drops instilled during the postoperative period. Excised tissue was submitted for pathological examination.

Results A total of 35 patients was seen with dermolipomas. The age at presentation ranged from one to 68 years

Reprint requests: Dr Alan McNab, 21 Erskine Street, North Melbourne 3051.

Clinical features and surgical management of dermolipomas 159

Fig. 1. -A typical dermolipoma shows a definite rounded anterior border behind the lateral limbus, with no visible posterior limit.

(mean 22.5, median 17 years). Twenty-seven patients were female and eight male. All patients or their parents had noticed a pale lump at the lateral canthus. T w o had bilateral lesions. There were no symptoms of diplopia, ptosis or dry eye. One patient had irritation from a long, thick hair growing from the surface of the lesion and several others complained of similar symptoms from multiple fine surface hairs. The only positive family history was in two female siblings with Goldenhars syndrome. There were no other affected family members. T h e lesions were white to pale yellow-pink with a definite anterior border at least 5 mm from the

lateral limbus. There was no posterior limit visible. T h e mass was either directly temporal with equal parts above and below the horizontal meridian, or mainly superotemporal. Thirteen patients had several fine hairs on the surface of the mass and one had a single, long, dark hair. T h e surface epithelium was pale, moist and non-keratinised and the underlying tissue varied from solid white to glistening yellow fat. Commonly, a band of epithelium extended from the surface of the lesion to the lateral canthus, obliterating the lateral fornix. Ocular motility was normal except in two patients with Goldenhars syndrome who had an ipsilateral Duanes syndrome. N o patients had ptosis and the tear film was normal. A slight medial displacement of the globe was common, but no patient had a measurable proptosis (2 mm or more). No other ocular abnormalities were found. Six patients had Goldenhars syndrome, including two sisters who were the only patients with bilateral lesions. The diagnosis of Goldenhars syndrome was based on the presence of pre-auricular skin tags or sinuses. In addition, hemifacial microsomia and mild abnormalities of the pinna were seen in three cases. None of these patients had concurrent limbal dermoids or lid colobomas. Surgical excision was performed to improve cosmesis in 13 patients. Complete excision without extensive dissection was possible in three cases, while the remainder underwent partial excision. A good cosmetic result was achieved in 12 patients without complications. In one, the dermolipoma was still visible following surgery and further debulking was carried out. Postoperatively the patient had diplopia caused by contraction of scarred conjunctiva at the lateral canthus. T h e patients symptoms were relieved by a z-plasty of the scarred conjunctiva. N o patients developed dry eye or ptosis. Excised tissue showed a mixture of fat and fibrous tissue with occasional interspersed sebaceous glands and hair follicles. If any overlying epithelium had been excised, it was found to be stratified squamous cells and lacked goblet cells.

Fig. 2.-Top: A diagram ofthe visible part of the dermolipoma that is debulked for patients requesting cosmetic improvement. Fig. 3.-Bottom: The conjunctival incision used to approach and debulk subepithelial components of the dermolipoma.
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Discussion Dermolipomas have a characteristic appearance. They are often quite small and asymptomatic (Figures 4 5), however a proportion are larger and , visible and patients seek cosmetic improvement. Surgery should be conservative and aimed at reducing visible tumour rather than achieving complete removal.
Australian and New Zealand Journal of Ophthalmology 1990; 18(2)

Fig. 4.-A patient with a small dermolipoma completely hidden by the upper lid. There is no indication for surgery.

Fig. 5.-A patient with a small dermolipoma completely hidden by the upper lid. There is no indication for surgery.

A dermolipoma should be differentiated from orbital fat prolapsed through Tenons capsule at a similar position, which may occur in the elderly. The overlying conjunctiva is normal, however, and the fat is mobile beneath it. Enlargement of the palpebral lobe of the lacrimal gland by inflammation, infiltration or tumour should be readily differentiated. Dermolipomas are solid choristomas and in that respect similar to limbal dermoids, but unlike cystic dermoids which have an inward-facing epithelium and continue to enlarge by the accumulation of keratin debris and the products of adnexal structures within their walls. The epithelial surface of a dermolipoma is continuous with the conjunctiva, with fat, fibrous tissue and often adnexal structures making up the deeper aspects of the mass. Posteriorly they appear to blend imperceptibly with the orbital fat, unlike dermoid cysts where total removal of the epithelial lining is required to avoid recurrence. The pathogenesis of dermolipoma is uncertain, but they may represent a minor manifestation of a branchial arch anomaly. When associated with Goldenhars syndrome they certainly form part of a recognised first pharyngeal arch and six of our patients showed this association. Goldenhar4 originally described a triad of epibulbar dermoids, pre-auricular appendages and pretragal fistulas. Gorlin et U P further defined the syndrome and noted the marked variation in its expression. Pre-auricular skin tags are the commonest manifestations. Baum and Feingold3 analysed 13 cases of Goldenhars syndrome and reviewed 114 other cases from the literature. They found dermolipomas in 4770, which were bilateral in 19% and
Clinical features and surgical management of dermolipomas

unilateral in 28%. Limbal dermoids occurred in 76%, being bilateral in only 23%. Duanes retraction syndrome, as seen in two of our patients, also has been found in association with Goldenhars Decreased corneal sensation and neurotrophic keratitis occasionally occur in Goldenhars syndrome9~0 did not affect any but of our patients. Recently, decreased visual acuity, tilted or hypoplastic discs, microphthalmia and anophthalmia have been reported in association with Goldenhars syndrome. All our patients had normal visual acuity and ocular fundi. Upper lid colobomas, another recognised feature of Goldenhars syndrome, were also not seen in this series. Isolated dermolipomas appear to occur sporadically with no specific inheritance. However, in association with Goldenhars syndrome, a positive family history is occasionally obtained. We saw two sisters with bilateral dermolipomas and Goldenhars syndrome. O t h e d 3 have noted Goldenhars syndrome in families, but monozygotictwins have been reported in whom only one of each pair was afXected.14 One pedigree of three generations affected with dermolipomas has been rep~rted.~ the vast majority of In both isolated dermolipoma and Goldenhars syndrome, there appears to be no inheritance. The unequal sex incidence of dermolipoma was first commented upon by Lagrange,16 who noted a female preponderance. Baum and FeingoldY3 in their extensive literature review of Goldenhars syndrome, found no sex difference. In our own series, only eight of the 35 cases occurred in males. Five of the six patients with Goldenhars syndrome were female. Surgical removal of dermolipomas has a reputa161

tion for producing major ocular morbidity. We have seen two patients (operated upon elsewhere and not included in this series) who have had dry eye, ptosis and diplopia. Others have reported similar c ~ m p l i c a t i o n sClearly, ~ . ~ ~ ~ ~ such complications are unacceptable for what is essentially a cosmetic problem. Attempts at a wide or complete surgical excision are bound to result in complications as the deeper aspects of the dermolipoma are in close relation to the lacrimal ductules, the lateral rectus muscle and the upper lid. Removal of the surface epithelium as well as the underlying solid componentsz0 runs the risk of symblepharon formation and if there is extensive epithelial loss, cover with rotated conjunctival flaps or free conjunctival grafts should be obtained. Excision down to and including Tenons capsule is also to be avoided as the exposed lateral rectus will adhere by scar to the lateral canthus causing limitation of movement. Dissection into the region of the lacrimal ductules is unnecessary as this part of the lesion is hidden under the upper lid. Hairs on the surface of dermolipomas may cause irritation, and if this is the major concern, hair follicles can be excised, electrolysed or frozen, leaving the bulk of the dermolipoma intact. If patients find their dermolipoma a sufficient cosmetic blemish to warrant surgery, surgical excision should be confined to the readily visible portions of the mass. T h e surgical technique we have used gave good results and avoided the complications caused by excessive removal of tissue and damage to the lacrimal ductules.

Acknowledgements Alan McNab was supported by the RACO-OPSM Travelling Fellowship. References
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4. Goldenhar h l . Associations malformatives de Ioeil et de loreille en particulier le syndrome dermoide epibulbaireappendices auriculaires - fistula auris congenita et ses relations avec la dysostose mandibulo-faciale. J Genet Med 1952;1:243-282. 5. Gorlin RJ, Jue KL, Jacobsen U, Goldenschmidt E. Oculoauriculovertebral dysplasia. J Pediat 1963;63:991-999. 6. Mattson R. Bilateral epibulbar dermoids and eyelid coloboma, caruncular malformation, anterior polar cataract, abductional insufficiency and acquired conjunctival cyst in the same patient. Acta Ophthalmol 1947;25:321-323. 7. Hoffman-Egg L, Velissaropoulos P. Malformations oculoauriculaire (lipodermoide epibulbaire, appendice preauriculaire, colobome de la paupiere superieure) et leurs relstions avec la dysostose mandibulo-faciale. Ann Oculist 1953;196: 155-169. 8. Pieroni D. Goldenhars syndrome associated with bilateral Duanes retraction syndrome. J Paed Ophthalmol 1969;6: 16-18. 9. von Bijsterveld OP. Unilateral corneal anesthesia in oculoauriculovertebral dysplasia. Arch Ophthalmol 1969;82: 189-190. 10. Mohandessan MM, Romano PE. Neuroparalytic keratitis in Goldenhar-Gorlin syndrome. Am J Ophthalmol 1978;78:111-113. 1 I . Margolis S, Aleksic J, Charles N, er a / . Retinal and optic nerve findings in Goldenhar-Gorlin syndrome. Ophthalmology 1984;91: 1327-1333. 12. Krause V. The syndrome of Goldenhar affecting two siblings. Acta Ophthalmol 1970;48:494-499. 13. Bock RH. Ein Fall von epibulbaren Dermolipom mit Missbildung einer Gesichtschalfie Diskordantes vorkommen bei einem eineiigen Zwillingspaar. Ophthalmologica 1953;122:86-90. 14. Cordier J, Stricker M, Remy A, Raspiller A. Syndrome de Franceschetti-Goldenhar discordant chez deux jumelles monozygotes. Arch Ophthalmol (Paris) 1970;30:321-328. 15. Saebo J. Lipoma conjunctivae in three generations. Acta Ophthalmol (Kbh) 1948;26:447-450. 16. Lagrange F. Traite des tumeurs de Ioeil, Vol 1. Paris: G Steinheil, 1901;50. 17. Crawford JS, Pashby RC, Apt RK. Embryology and congenital anomalies. In: Stewart WB, ed. Ophthalmic plastic and reconstructive surgery. San Francisco: American Academy of Ophthalmology 1984;96-97. 18. Reeh MJ. Treatment of lid and epibulbar tumours. Springfield, Illinois: Charles C Thomas, 1963; 5 I . 19. Swan KC, Emmens TH, Christensen L. Experiences with tumors of the limbus. Trans Am Acad Ophthalmol 1948;52:458-466. 20. Reese AB. Tumors of the eye. 3rd edn. Hagerstown: Harper and Row, 1976;420.

(Received 7 November 1989)

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