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What is a dermoid cyst?

Dermoid Cyst

Definition: a benign congenital cyst in the orbit lined by squamous epithelium and containing adnexal

structures in the wall.

Incidence/Prevalence:Dermoid cysts make up between 4-15% o orbital tumors in most series. !he lesions

are present at birth but may become maniest later in childhood or e"en adulthood.

Etiology:Dermoid cysts are belie"ed to arise as embryonic epithelial nests that become entrapped during

embryogenesis and pinched at suture lines o bones.

Clinical Findings:#n clinical grounds dermoid cysts can be di"ided into supericial and deep masses.

Superficialdermoid cysts are usually situated temporally$ and present as painless$ irm$ 1- cm diameter$

immobile masses that are noticed during inancy and originate most commonly rom the ronto&ygomatic

suture or rontolacrimal suture.

Deepdermoid cysts oten present as slow growing masses in later childhood or adulthood and can arise rom

any suture o the orbit$ but most commonly the ronto&ygomatic. Deep dermoid cysts may protrude through

the ronto&ygomatic suture to take a dumbbell shape. 'n"ol"ement o sutures on the ethmoid bone$ or area

around the superior orbital issure ha"e been reported. !he patients present with symptoms and signs

reerable to the mass eect in the orbit (proptosis$ bulging upper eyelid$ downward (or upward) displacemento the eye)$ "isual and ocular motor deects$ etc. *uptured dermoid cysts may present as draining istulas.

Radiologic Findings:

+upericialdermoids are round masses that contain a lucent center (at or muscle density) and are generally

not associated with a deect in the bone.

Deepdermoid cysts are oten accompanied by bone deects at the suture. ,or example$ the classic dumbbell

shaped lesion may extend thru the ronto&ygomatic suture into the temporalis ossa. !here may be

calciication$ or an area where adacent bone is smoothly disrupted.

Histopathology:
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istologically$ dermoid cysts contain desquamated squamous epithelium and keratin in the lumen (arrow 1)

and are encapsulated and lined by keratini&ed stratiied squamous epithelium (arrows and /). 0 key or the

diagnosis is the presence o adnexal structures including sebaceous glands (arrows and 4 here). air roots$sweat glands$ apocrine glands and lacrimal glands may also appear in the wall o the cyst. igher

magniication shows that the lumen contains hair shats and keratin (arrows 5 and $ respecti"ely).

2olari&ation o the sections will oten highlight the hair shats in the lumen.

' the wall does not bear adnexal structures$ the term epidermal or keratin cyst is applied. 'ntraorbital cysts

may also be lined by respiratory epithelium or conuncti"al epithelium. *upture o cyst contents may

produce a marked granulomatous reaction and the residual cyst that orms may ha"e its squamous epithelial

lining completely replaced by epithelioid histiocytes and multinucleated giant cells (number 3 below). ost

ruptured cysts occur between the ages o -4 and some authors note a relationship between the "olume o

the cyst (163 cubic) and the likelihood o inding a hidden rupture (see reerence)

reatment:

+upericialdermoids are oten remo"ed beore the 5th year o lie to a"oid traumatic rupture and some

authors recommend surgery by the age o /-4.

Deepdermoids may be "ery diicult to remo"e and require careul preoperati"e assessment o the anatomicstructures in"ol"ed. 't is important to attempt remo"al o the cyst intact and to remo"e the entire lining.

7esions may extend into the intracranial ca"ity se"erely complicating and compromising the attempts to
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remo"e them.

Prognosis:+upericial dermoids are generally quite easily handled with little morbidity. Deep dermoids

in"ol"ing complicated orbital structures or extending in the intracranial ca"ity are prone to complications

rom either rupture during remo"al or damage to adacent structures. 0 team approach with other ser"ices

may be necessary.

Dermoid Cyst

8hat is a dermoid9

0 dermoid is an o"ergrowth o normal$ non-cancerous tissue in an abnormal location. Dermoids occur all

o"er the body. !he ones in and around the eye are usually comprised o skin structures and at +ee igure 1;.

8here are dermoids ound around the eyes9

!here are two main dermoid types that occur on or around the eyes. 0n orbital dermoid is typically ound in

association with the bones o the eye socket. 0n epibulbar dermoid is ound on the surace o the eye$ either

at the unction o the cornea and sclera (limbal epibulbar dermoid) or more posteriorly on the eye where theconuncti"a that co"ers the eye meets the conuncti"a that co"ers the lid (posterior epibulbar dermoid or

lipodermoid).

8hat does an orbital dermoid look like9

0n orbital dermoid presents as an egg-shaped mass under the skin adacent to the bones o the eye socket.

!he mass is sot. !he skin o"erlying the mass is normal in appearance. Dermoids can remodel the bone

adacent to them so that they oten sit in a depression in the bone. +ometimes dermoids are dumbbell-shaped

with one hal o the mass on the outer part o the rim o the eye socket and the other part in the inside o the

rim o the eye socket. Dermoids are cysts and are typically illed with a greasy material that is yellow in

color.

Where are or!ital dermoids "s"ally fo"nd?

#rbital dermoids usually orm anteriorly in the eye socket where two o the acial bones that orm the eye

socket touch each other. !he most common place or dermoids to occur is in the upper and outer part o the

eye socket near the end o the eyebrow. !hey can also occur adacent to the nose but are rarely ound in

association with the bones in the lower part o the eye socket. *arely orbital dermoids are ound more

posteriorly in the eye socket.


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0re posterior epibulbar dermoids associated with other diseases9

?es$ sometimes. !hey can be ound in persons with @oldenhar syndrome$ linear ne"us sebaceous syndrome$

and encephalocraniocutaneous lipomatosis.

Where are lim!al dermoids "s"ally fo"nd?

!hey are ound on the surace o the eye one the cornea or at the unction o the cornea and sclera.

Do limbal epibulbar dermoids need to be remo"ed9

Aecause they can cause eye irritation and because the appearance is abnormal$ epibulbar dermoids are

usually remo"ed.

ow are limbal epibulbar dermoids remo"ed9

!he dermoids are cut lush with the surace o the eye. +ometimes the dermoid extends into the sclera andBor

the cornea and care must be taken to a"oid entering the eye when excising them. 0ter excision$ the sitewhere the dermoid lay can be co"ered by a piece o transplanted cornea.

Do limbal dermoids cause "ision loss9

#ccasionally the dermoid is so large that it blocks "isual input rom entering the eye. ore oten howe"er$

the "ision loss occurs because the presence o the dermoid causes the cornea o the aected eye to ha"e an

irregular shape. !his warping o the cornea can cause a large amount o astigmatism and a blurred image.

!he blurred image encourages the de"eloping brain to ignore the input rom the aected eye$ thus causing

"ision loss through amblyopia. ,ortunately$ amblyopia i detected during childhood can oten be successully

treated (amblyopia).

Does the risk o "ision loss go away ater the dermoid is remo"ed9

Csually not$ e"en though the dermoid is gone$ the warpage it causes in the cornea remains and the risk o

amblyopia de"eloping remains.

0re limbal dermoids associated with other diseases9

?es$ sometimes. !hey can be ound in persons with @oldenhar syndrome$ linear ne"us sebaceous syndrome$

and encephalocraniocutaneous lipomatosis.

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