Complex limbal choristomas in linear nevus sebaceous syndrome.
OBJECTIVE
This study aimed to describe the clinical and histopathologic findings in four patients with complex limbal choristomas associated with linear nevus sebaceous syndrome (LNSS), a rare disorder including nevus sebaceous, seizures, and mental retardation, and often accompanied by ocular anomalies.


METHODS
Small observational case series.


METHODS
A retrospective review of the clinical and histopathologic records of four patients.


RESULTS
Each of four patients had complex limbal choristomas in the setting of clinical and histopathologic LNSS. The limbal choristomas were multiple in three patients and bilateral in two patients. Most choristomas involved the superotemporal limbus (6 of 10), although nasal (3 of 10) and inferior (1 of 10) limbal tumors also were present. Three patients had significant corneal astigmatism or involvement of the central cornea requiring surgical removal of their choristomas, one accompanied by a lamellar keratoplasty and another accompanied by two consecutive penetrating keratoplasties. Each graft eventually vascularized, reducing vision. One patient's vision was limited by amblyopia and another by occipital cortical dysgenesis with visual impairment. Histopathologic examination of the excised choristomas showed foci of lacrimal gland (3 of 4 patients), adipose tissue (3 of 4), neural tissue (1 of 4), cartilage (1 of 4), lymphoid follicles (1 of 4), skin adnexal tissue (1 of 4), and smooth muscle (1 of 4). Other associated ocular findings included an eyelid mass (1 of 4), colobomas of the eyelid (3 of 4), colobomas of the choroid and retina (2 of 4), nonparalytic strabismus (2 of 4), scleral ectasia (1 of 4), partial oculomotor palsy with ptosis and anisocoria (1 of 4), microphthalmia (1 of 4), hypertelorism (1 of 4), and cortical visual impairment (1 of 4).


CONCLUSIONS
Complex limbal choristomas, although rare, can occur in the setting of LNSS and can be associated with multiple ocular and systemic abnormalities. Visual prognosis appears poor in most cases despite aggressive management.
Department of Ophthalmology, University of California, San Francisco, School of Medicine 94143-0944, USA.
J L
JL
Duncan
M
M
Golabi
D R
DR
Fredrick
C S
CS
Hoyt
D G
DG
Hwang
S G
SG
Kramer
E L
EL
Howes
E T
ET
Cunningham
D002675:Child, Preschool; D002828:Choristoma / Q000150:complications* / Q000473:pathology / Q000601:surgery; D003316:Corneal Diseases / Q000150:complications* / Q000473:pathology / Q000601:surgery; D005124:Eye Abnormalities / Q000150:complications / Q000473:pathology; D005134:Eye Neoplasms / Q000150:complications* / Q000473:pathology / Q000601:surgery; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008607:Intellectual Disability / Q000150:complications* / Q000473:pathology; D015948:Keratoplasty, Penetrating; D016850:Limbus Corneae / Q000473:pathology; D008297:Male; D018193:Neoplasms, Complex and Mixed / Q000150:complications* / Q000473:pathology / Q000601:surgery; D009378:Neoplasms, Multiple Primary / Q000150:complications / Q000473:pathology; D009508:Nevus, Pigmented / Q000150:complications* / Q000473:pathology; D012189:Retrospective Studies; D012626:Sebaceous Gland Neoplasms / Q000150:complications* / Q000473:pathology; D012640:Seizures / Q000150:complications* / Q000473:pathology; D013577:Syndrome