Orbital Lipodermoid

From EyeWiki


Cam Loveridge-Easther, MD and Rona Z Silkiss, MD, FACS 10 January 2022

• 1 Disease Entity

o1.1 Disease

o 1.2 Etiology

o 1.3 General Pathology

• 2 Diagnosis

o 2.1 History

o 2.2 Physical examination

o 2.3 Signs

o 2.4 Symptoms

o 2.5 Clinical diagnosis

o 2.6 Diagnostic procedures

o 2.7 Differential diagnosis

• 3 Management

o 3.1 General treatment

o 3.2 Surgery

• 4 Additional Resources

• 5 References

• 6 Images

1. Disease entity

Lipodermoid, Dermolipoma

1.1 Disease

Lipodermoids are abnormal epibulbar growths of the adipose tissue. A conjunctival lesion, the lipodermoid (dermolipoma) is usually located near the temporal fornix and is composed of adipose tissue and dense connective tissue. Lipodermoids are congenital choristomas (1). Choristomas are histologically normal tissue found at an abnormal location, in contrast to hamartomas which are benign (not cancer) growths made up of an abnormal mixture of cells and tissues normally found in the body where the growth occurs (2). Lipodermoids are localized yellowish-white, soft, smooth masses firmly adherent to the overlying conjunctiva. They can be contiguous with orbital fat posteriorly and tend not to involve the cornea anteriorly (3). They are considered distinct but similar entities to epibulbar dermoids and complex choristomas (3). Choristomatous lesions make up 10-30% of childhood tumors of the conjunctiva (4).

1.2 Etiiology

Lipodermoids are congenital choristomas derived from sequestration of ectoderm at the time of embryonic development (5). Lipodermoids can be associated with Goldenhar syndrome, organoid nevus syndrome (Linear Nevus of Jarassohn), Treacher- Collins or arise sporadically (3, 6). Of 57 cases of Goldenhar syndrome, 32% had epibulbar dermoids or lipodermoids, whereas conversely in a series of lipodermoids 35% had Goldenhar syndrome (7, 8). Lipodermoids are also associated with colobomas of the eye, osteomas, limbal dermoids and facial nerve palsy (1, 8).

1.3 General Pathology

Lipodermoids consist of adipose tissue, collagenous tissue and skin adnexa covered by connective tissue and surface epithelium of the conjunctiva (3). Pilosebaceous structures and lacrimal gland tissue may also be present (1, 4). Lipodermoids may be adherent to the lacrimal gland or to an underlying osteoma. The high amount of adipose tissue and the contiguous nature of the epithelium with the conjunctiva make them distinct from dermoids (8). Dermoids by contrast consist of collagen connective tissue resembling dermis covered by epidermal epithelium (3). As choristomas, they are normal tissue and do not harbor malignant potential (9).

2. Diagnosis

Given the superficial location of lipodermoids, they are easily diagnosed on clinical exam though imaging modalities may be utilized. More details are presented below.

2.1 History

Though lipodermoids are believed to be congenital, they classically remain asymptomatic for years and may not be detected until adulthood (4). Median age of presentation is 16 years They are more common in females (8, 10). Often, the patient first notices the presence of the lipodermoid when lifting the lid to apply makeup or place a contact lens. Lipodermoids may cause irritation and can disrupt vision either through irritation by lash growth, tear disruption, ptosis, or refractive error, though they induce much less astigmatism than dermoids (11).

2.2 Physical exam

Lipodermoids are normally yellow-white and firm. They are adherent to the conjunctiva, not freely mobile and cannot be repositioned into the orbit (5). They are usually unilateral though can be bilateral and the lateral canthus may be disrupted (1). Visual acuity and refraction are important given the risk of amblyopia. Retropulsion will help differentiate a lipodermoid from orbital fat prolapse, as orbital fat will increase on retropulsion (11). Other ocular abnormalities including coloboma, osteoma, ear tags and micrognathia may be associated. A complete anterior segment, dilated fundus and strabismus examination should be performed.

2.3 Signs

• Yellow-white palpable firm mass • Distortion of the lateral canthus • Astigmatism • Inflammation • Strabismus

2.4 Symptoms

Usually none however on occasion, there may be ocular surface irritation due to pilosebaceous structures causing mechanical irritation and disruption of the tear film.

2.5 Clinical diagnosis

Lipodermoids are usually diagnosed on clinical exam though they may be associated with syndromic features.

2.6 Diagnostic procedures

Lipodermoids are usually easily diagnosed on clinical examination, but orbital imaging may be of use to confirm the diagnosis and for surgical planning. CT or MRI can both reliably distinguish a lipodermoid from orbital fat prolapse and can delineate posterior extension (5). CT and MRI images will show a crescent or triangular fatty mass, usually at the supertemporal aspect of the globe, anterior to the insertion of the lateral rectus and medial to the lacrimal gland, usually with no connection to the intraconal fat (5). Lipodermoids attenuate identically to intraconal fat though may show some stranding (5). Of course, CT should be avoided, if possible, in children especially given the benign nature of these lesions.

2.7 Differential Diagnosis Dermolipoma- this term is used interchangeably with lipodermoid Herniated orbital fat Lacrimal gland cyst Lacrimal gland prolapse Conjunctival lymphangioma Conjunctival lymphoma

3. Management

3.1 General treatment

Most and especially small, asymptomatic lipodermoids do not require treatment. Lubrication and occasional anti-inflammatories may suffice.

3.2 Surgery

If required, treatment involves surgical debulkment without necessarily aiming for complete excision (1, 9-12). Lesions which affect the visual axis, induce astigmatism, cause constant irritation or are cosmetically unacceptable in primary or medial gaze are the main indications for surgery (1, 11). The method for resection involves removal of only the anterior portion of the mass, with care taken to conserve as much conjunctiva as possible while removing all pilosebaceous units and as much lipodermoid anterior to the orbital rim as possible (1). Lipodermoids are adherent to the conjunctiva with no accessible cleavage plane and given their proximity to the lacirmal gland and lateral rectus surgery may be more difficult than it initially seems. Aggressive surgical reduction may lead to a shortage of conjunctiva and a post-operative restrictive strabismus (9-12). Ptosis has also been described (11). Post operatively, the patient may notice the persistence of the lesion, with less visible thickness.

Surgical technique

Under intravenous sedation or general anesthesia, patient age and maturity dependent. Tetracaine drops are administered, then a 50:50 mixture of 2% xylocaine with 1:100,000 epinephrine, 0.5 Marcaine with 1:200, 000 epinephrine, sodium bicarbonate and hyaluronidase is injected into the area of the lipodermoid. Usually, 1 cc will suffice to raise the conjunctiva in the area of the lipodermoid. The patient is them prepped and draped in the usual sterile fashion. A small wire eyelid speculum or Desmarres retractor is positioned.

Westcott scissors are used to make an incision just medial to the lesion through the conjunctiva. Dissection is then carried out on either side of the lesion. Anteriorly, the dissection is performed between the conjunctival covering and the underlying lesion. Conjunctiva is preserved, though this is not easy given the adherent nature of the conjunctiva to the underlying tissue. If the lesion is supertemporal, superior dissection should be limited to avoid damage to the lacrimal gland ductules

Posteriorly the dissection occurs between the lipodermoid and the Tenon's. Avoid dissection too close to the lateral rectus muscle. If necessary, a muscle hook can be placed to identify the muscle. The goal of Lipodermoid surgery is functional and cosmetic improvement without post-operative issues such as a symblepharon, dry eye or strabismus.

The color and to some extent texture of the area will be similar post-operatively, but the thickness will be improved. The part of the lipodermoid that can visualized with the patient in primary gaze, is the main area for removal consideration. Removal of the hidden sections is optional and entirely dependent on the ease and safety of removal.

The conjunctiva can then be closed with superficial interrupted 6-0 plain gut or 7-0 Vicryl sutures placed in a buried fashion

At the conclusion of the case, a steroid/antibiotic ointment is placed into the eye and the patient returns in one week for reevaluation. The patient should be counseled that a post operative “red eye” is to be expected. Cool compresses as well as the ointment are utilized for one week post operatively.

4. Additional resources

Surgical video. https://webeye.ophth.uiowa.edu/eyeforum/video/plastics/6/6-lipodermoid.htm

Surgical video. https://oculoplastic.eyesurgeryvideos.net/dermolipoma-excision/

5. References.

1. Fry CL, Leone CR. Safe Management of Dermolipomas. Archives of ophthalmology (1960). 1994;112(8):1114-6. 2. Welsh CT. Hamartomas and choristomas in the nervous system. Semin Diagn Pathol. 2019;36(1):62-70. 3. Rajabi MT, Ramezani K. Lipodermoid Cyst: A Report of a Rare Caruncular Case. Middle East Afr J Ophthalmol. 2015;22(4):528-30. 4. Shields CL, Shields JA. Conjunctival tumors in children. Current opinion in ophthalmology. 2007;18(5):351-60. 5. Kim E, Kim HJ, Kim YD, Woo KI, Lee H, Kim ST. Subconjunctival Fat Prolapse and Dermolipoma of the Orbit: Differentiation on CT and MR Imaging. American Journal of Neuroradiology. 2011;32(3):465. 6. Insler MS, Davlin L. Ocular findings in linear sebaceous naevus syndrome. Br J Ophthalmol. 1987;71(4):268-72. 7. Mansour AM, Wang F, Henkind P, Goldberg R, Shprintzen R. Ocular findings in the facioauriculovertebral sequence (Goldenhar-Gorlin syndrome). Am J Ophthalmol. 1985;100(4):555-9. 8. Khong JJFM, Hardy TGFM, McNab AAFM. Prevalence of Oculo-auriculo-vertebral Spectrum in Dermolipoma. Ophthalmology (Rochester, Minn). 2013;120(8):1529-32. 9. Mahanta K, Laskar H, Saharia B, Sarmah P. Conjunctival Dermolipoma. Journal of evolution of medical and dental sciences. 2019;8(43):3279-81. 10. McNab AA, Wright JE, Caswell AG. Clinical features and surgical management of dermolipomas. Australian and New Zealand journal of ophthalmology. 1990;18(2):159-62. 11. Matsuo T. Clinical decision upon resection or observation of ocular surface dermoid lesions with the visual axis unaffected in pediatric patients. Springerplus. 2015;4:534-. 12. Bilateral Lipoderma In Adult: A Rare Case. The Internet journal of ophthalmology & visual science. 2006;4(1)

6. Images.

Image I showing left temporal lipodermoid with pilosebaceous structures.

Images: Image I showing left temporal lipodermoid with pilosebaceous structures. Image courtesy of Dr Rona Silkiss, California Pacific Medical Centre, CA, USA.

Image II showing child with Oculo-auricular-vertebral syndrome following corrective L upper lid coloboma repair. Note subtle R temporal lipodermoid. Image courtesy of Dr Rona Silkiss, California Pacific Medical Centre, CA, USA.

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