Grand Rounds Brooke LW Nesmith, M.D. University of Louisville School of Medicine Department of Ophthalmology & Visual Sciences 1/16/2015

Presentation CC: Enlargement of right upper eyelid mass x 2 months CC: Enlargement of right upper eyelid mass x 2 months HPI: 10mo male referred to oculoplastics clinic for evaluation of right upper eyelid mass. Patient has had fullness of right upper lid laterally since approx 2 months of age, has become progressively larger in the past 2 months. HPI: 10mo male referred to oculoplastics clinic for evaluation of right upper eyelid mass. Patient has had fullness of right upper lid laterally since approx 2 months of age, has become progressively larger in the past 2 months.

Presentation PMHx:Full term with uncomplicated delivery; Asthma PMHx:Full term with uncomplicated delivery; Asthma Meds:Albuterol, Zyrtec Meds:Albuterol, Zyrtec Allergies: Cephalexin Allergies: Cephalexin

Exam Visual acuity: Central steady and maintained OU Pupils: 4mm OU, no RAPD Motility: Full OU Globes soft to palpation OU External Exam: Soft firm mobile mass at superior aspect of right lateral orbital rim. No edema or erythema. No tenderness to palpation. No globe displacement. Anterior Segment Exam: WNL OU

Assessment 10 month old male with congenital right superolateral orbital mass of the upper eyelid with recent enlargement. 10 month old male with congenital right superolateral orbital mass of the upper eyelid with recent enlargement.  congenital cyst –dermoid –epidermal Right anterior orbitotomy to excise lesion Right anterior orbitotomy to excise lesion Plan

Surgical Course 1 x 0.5cm tan-white to tan-yellow cyst was successfully excised near the right zygomaticofrontal suture line without rupture 1 x 0.5cm tan-white to tan-yellow cyst was successfully excised near the right zygomaticofrontal suture line without rupture

Pathology

Pathology

Orbital Cysts of Childhood surface epithelium   dermoid- (epidermal and conjunctival)   simple epithelial (epidermal, conjunctival, respiratory, apocrine gland) teratomatous neural (associated with ocular maldevelopment) secondary (mucocele) inflammatory (parastic) noncystic lesions with a cystic component – –adenoid cystic carcinoma, rhabdomyoscarcoma Shields J, Shields C. Orbital cysts of childhood-classification, clinical features, and management. Surv Ophthalmol 2004;49:

Orbital Cysts of Childhood Shields J, Shields C. Orbital cysts of childhood-classification, clinical features, and management. Surv Ophthalmol 2004;49:

Dermoid Cyst Most common Most common –40% of all orbital lesions of childhood –89% of all orbital cystic lesions of childhood that come to biopsy or surgical removal Congenital choristomas Congenital choristomas –embryonic epithelial nests – entrapped during embryogenesis –along suture lines Shields J, Shields C. Orbital cysts of childhood-classification, clinical features, and management. Surv Ophthalmol 2004;49:

Dermoid Cyst Anterior lesions Anterior lesions –generally becomes apparent during 1 st decade of life –most common location – superolateral aspect of orbit at frontozygomatic suture Shields J, Shields C. Orbital cysts of childhood-classification, clinical features, and management. Surv Ophthalmol 2004;49:

Dermoid Cyst Deeper lesions Deeper lesions –may remain clinically occult until adulthood – present with painless, progressive proptosis, motility deficits, or diplopia Shields J, Shields C. Orbital cysts of childhood-classification, clinical features, and management. Surv Ophthalmol 2004;49:

Dermoid Cyst Dumbbell-shaped Dumbbell-shaped –cyst in orbit and temporal fossa – connected by defect in bone –pulsating proptosis with mastication Missotten G, Van Santbrink-Bakker H., de Keizer R. Dumbbell-shaped dermoid cysts. Acta Ophthalmologica 2010;doi:10.111/j

Dermoid Cyst Workup and Imaging Workup and Imaging –classic dermoid cysts at frontozygomatic suture may be diagnosed clinically without imaging –imaging required for medial lesions (rule out encephalocele or mucocele) and deep lesions Treatment Treatment –surgical excision – remove with cyst wall intact without causing iatrogenic rupture Missotten G, Van Santbrink-Bakker H., de Keizer R. Dumbbell-shaped dermoid cysts. Acta Ophthalmologica 2010;doi:10.111/j

Thank you.