Proptosis in children

PROPTOSIS
children

Dr. Yousaf Jamal
FCPS Resident
Ophthalmology Unit
Hayatabad Medical Complex
06-03-2010

Mar 25, 2013

CONTENTS

• Recap
• Causes in children
• Differentiating features
• Common causes discussion
• Summary
• Take home message
• MCQs
Page 2 Proptosis in Children Mar 25, 2013

Recap

• Causes of Proptosis
• Common causes in adults
• Examination of proptosis


CONTENTS

• Recap
• Summary
• MCQs

Causes in children

• Orbital cellulitis • Retinoblastoma
• Dermoid cyst • Leukemia
• Capillary • Lymphangioma
hemangioma • Metastasis
• Optic nerve glioma – Metastatic
• Rhabdomyosarcoma neuroblastoma
– Ewing's sarcoma


CONTENTS

• Recap
• Summary
• MCQs

CAPILLARY ORBITAL LYMPHANGIOMA
HEMANGIOMA

Pathology Vascular hemartoma Isolated vascular hemartoma

Demographics Infant, commonest Early childhood
benign orbital tumor in
childhood, spontaneous
involution
Associated Visceral, -
nasopharyngeal
hemangioma,
kasabach-merit
syndrome
Presentation Nonaxial, +ve Valsalva, Nonaxial, -ve Valsalva, acute episodes of
superficial/deep spontaneous hemorrhage, may be
superficial/deep
Radiology Intra/extraconal mass, Low density cyst-like mass, enlargement of
poorly defined bony orbit

Tx Observation, surgery Guarded prognosis, surgery, drainage


ON GLIOMA ON MENINGIOMA
Pathology Fusiform enlargement of ON, origin - Tubular enlargement of ON, origin-
glial tissues, meninges show reactive meninges, psammoma bodies
hyperplasia, dura is normal

Demographic Young girls (2-6yrs) Late middle aged woman
s

Associated NF-1 (20-40%) -

Presentation Axial, VA decreases early, EOM Axial, gaze evoked amaurosis,
normal, optociliary shunts - uncommon EOM impaired, optociliary shunts

Radiology Fusiform enlargement, isodense to Tubular enlargement, hyperdense,
bone ON sheath enhancement

Tx Conservative, surgical, radiotherapy Conservative, radiotherapy, surgery


RHABDOMYOSARCOM ORBITAL CELLULITIS
A
Pathology Undifferentiated mesenchymal Infection of soft tissues behind the
cells, most common primary orbital septum
malignancy of childhood
Demographics Boys > girls (avg 7 yrs), Commonest cause of proptosis in
children, adults also affected

Associated Cervical L. nodes involved Usually after trauma, sinus related

Presentation Nonaxial, painful, unilateral, rapid Painful, lids edema with reddish
onset, swollen bluish lids, nose color, Ptosis, malaise, fever, life
bleed, Ptosis, chemosis threatening, VA impaired,
Ophthalmoplegia,
Radiology Moderately well-defined CT-(orbit, sinuses, brain): diffuse
homogenous mass, Bony orbital infiltrate, sinus opacity,
destruction proptosis
MRI for cavernous sinus thrombosis.
Tx Radiotherapy, chemotherapy, Medical, surgical
Exenteration for resistant cases


CONTENTS

• Recap
• Summary
• MCQs

ORBITAL CELLULITIS

• Bacterial orbital cellulitis – life-threatening
• Infection of soft tissues behind septum
• More common in children
• Organisms
– S. pneumoniae
– S. aureus
– S. pyogenes
– H. influenzae


Etiology

• Sinus-related • Post-trauma
– Ethmoidal (90%) – Penetrating septum (48-
• Extension of preseptal 72 hrs)
cellulitis – Retained foreign body

• Local spread • Post-surgical
– Lacrimal
– Dacryocystitis
– Orbital
– Facial cellulitis
– Retinal
– Dental infections
• Systemic bacteremia


Presentation…

• Malaise
• Fever
• Pain
• Visual impairment
• Tender, warm, red periorbital edema
• Proptosis


… Presentation

• Painful Ophthalmoplegia
• Chemosis
• Headache
• Rhinorrhea
• Elevated IOP


Courtesy: Medline


Work-up

• Hx
• Examination
• Vital signs record
• WBC/blood culture
• Lumbar puncture
• CT orbit
• MRI
– For cavernous sinus thrombosis


Complications

• Ocular • Intracranial
– Exposure – Meningitis (2%)
keratopathy – Brain abscess
– Raised IOP – Cavernous sinus
– CRVO,CRAO thrombosis (1%)
– Endopthalmitis • Orbital abscess
– Optic neuropathy • Subperiosteal
abscess


Treatment

MEDICAL…
• Hospital admission + ENT consultation
• I.V. antibiotics
– Ceftazidime, Cefotaxime, Ceftriaxone
– Flucloxacillin
– Vancomycin
– Ampicillin-Sulbactam
– Piperacillin-Tazobactam
– Ciprofloxacin/Levofloxacin

• Metronidazole
• Nasal decongestant
– Phenylephrine
– Oxymetazoline
• Antibiotic eye ointment


SURGICAL…

• Indications
– Decreasing vision
– Afferent pupillary defect
– No response to antibiotics in 48-72 hrs
– Orbital/Subperiosteal abscess
– Atypical picture…..Needs biopsy


• Canthotomy/Cantholysis in emergency
• Surgical drainage of fluid
• Orbital surgery + sinusotomy
• For fungal
– Debridement
– Exenteration


Doses

• Ceftazidime (Fortum® )
– Adults=1-2g/8hr I.V.
– Pediatric
• Neonates=30mg/kg IV
• 1 month-12 years=30-50mg/kg IV
• Ceftriaxone (Rocephin® )
– Adults=2g/12hr IV
– Pediatric
• 20-50mg/kg/day IV


• Cefotaxime (Claforan® )
– Adults=1-2g/10ml/4hr IV in 3-5 min (max=12g/day)
– Pediatric
• 0-1 week=50mg/kg/12hr IV
• 1-4weeks=50mg/kg/8hr IV
• 1month-12years=50-80mg/kg/day IV in 4-6 doses


• Vancomycin (Vancocin® )
– Adults=1g/12hr IV (over 1 hour with rate <
10mg/min at concentration <5mg/ml)
– Pediatric
• Infants & neonates=15mg/kg loading
• Ciprofloxacin
– In > 17 years age=400mg/12hr IV
• Levofloxacin
– In > 17 years age=750mg/6hr IV


• Ampicillin-Sulbactam (Unasyn® )
– Adults=3g/6hr IV
– Children=300mg/kg/day 4 divided doses
• Piperacillin-Tazobactam (Zosyn® )
– Adults=4.5g/8hr IV
– Children=240mg Piperacillin component/kg/day 3
divided doses
• Metronidazole (Flagyl® )
– 500mg/6-8hr IV


Tx combinations

• Metronidazole to all
• Vancomycin + Ceftriaxone (3rd gen ceph)
– Allergy to penicillin
• Fluoroquinolone
– Allergy to penicillin + cephalosporins
• Vancomycin + Ampicillin-Sulbactam or
Piperacillin-Tazobactam
– For MRSA


Follow-up

• Daily monitor
– Symptoms
– Temperature
– Visual acuity
– Ocular motility
– Proptosis
– Cornea
– IOP
– Retina and optic nerve


• Change to oral to complete 14 days course if
– Afebrile for 48 hours
• Oral Drugs
– Amoxicillin-clavulanate (Augmentin® )
• Adults=500mg TDS
• Children=20-40mg/kg/day 3 divided doses
– Cefaclor (Ceclor® )
• Adults=500mg TDS
• Children=20-40mg/kg/day 3 divided doses
– Metronidazole


DERMOID CYST

• A choristoma, found adjacent to suture lines
• Connected to periorbita by fibrovascular
tissue
• Contain…
– Keratinized stratified squamous epithelium
– Blood vessels, fat, collagen, sebaceous glands,
and hair follicles
– a tan oily liquid to a white or yellow substance


Presentation…

• Mostly in children
• Mostly…Superotemporal aspect of orbit
• A painless mass
• Slowly growing
• Usually < 1 cm in diameter, non-tender


… Presentation

• Globe displacement
• Inflammation if ruptures
• May
– Compress optic nerve
– Diplopia


Courtesy: Medline


Proptosis in Children Mar 25, 2013

Courtesy: Medline


Work-up

CT/MRI

– Indicated if posterior extent not palpated
– Cystic appearance
– Well circumscribed lesion


Courtesy: eyetext.org


Treatment

Surgical
• Excision in Toto


CAPILLARY HEMANGIOMA

• Most common orbital tumor of infancy
• Benign endothelial cells neoplasms
• Absent usually at birth
• Rapid growth in infancy
• Involution later on


Pathophysiology

• Thought … of placental origin
• 02 phases of growth
– Proliferative
• Rapid growth…8-18 months
• Increase in no. of endothelial & mast cells (stimulus for
vessel growth)
– Involutional
• 30-50% involute by 5 yrs
• 75% by 7 yrs

Kavanagh EC, Heran MK, Peleg A, et al. Imaging of the natural history of an orbital capillary
hemangioma. Orbit. Mar 2006;25(1):69-72


Mortality/Morbidity

• Kasabach-Merritt syndrome
– Coagulopathy, thrombocytopenia
– large visceral / Nasopharyngeal hemangiomas
– DIC may occur, high output CCF
– Mortality…30-50%
• Ophthalmic morbidity
– Space occupying
– Amblyopia


Presentation

• F:M…3:1
• 1-2% in neonates
• All develop by 6 months
• Typically…red spot growing in size in
Periorbital area
• Inability to open lids
• Bulging of the eye


Classified as…

1. Cutaneous
2. Purely preseptal
3. Preseptal with extraconal element
4. Combination of preseptal, extraconal and
intraconal


Signs

• Usually…superonasal, brow or lid lesion
• Typically blanches with pressure
• Ptosis & proptosis if posterior extension
• Visual loss
• Preseptal…Dark blue or purple
• Enlarge & change color while crying


Work-up

CT
– poorly circumscribed mass with no bony erosion
– Homogenous enhancing lesion
MRI
– hypointense to fat on T1-weighted & hyperintense
on T2-weighted scans
US & Doppler
– For anatomic relations & extent


Treatment

Medical
• Observation
• Steroids
– Clobetasol propionate cream
– Injectable steroid formulations
– Systemic corticosteroids are used for amblyogenic
lesions
• Interferon alfa-2a
– Resistant to steroid treatment

Surgery
• Laser surgery
– carbon dioxide laser
– Argon laser
– Nd:YAG laser
• Primary excision


OPTIC NERVE GLIOMA

• Most common primary tumor of ON
– In children…mostly benign
– In adults…Aggressive glioma
• WHO classify it as
– Grade I astrocytomas (pilocytic astrocytomas) bcz
slow growing & tend not to metastasize
• Generalized hyperplasia of glial cells in the
nerve to complete disorganization


• 10-38% of pediatric pts with ON glioma have
NF-1
• Bilateral ON gliomas…almost pathognomonic
for NF-1

Listernick R, Charrow J, Greenwald MJ, et al. Optic gliomas in children with neurofibromatosis type 1. J
Pediatr. May 1989;114(5):788-92.


Presentation…

• ON gliomas are…*
– 4% of orbital tumors
– 4% of intracranial gliomas
– 2% of intracranial tumors
– 2/3rd of all primary optic nerve tumors
• 20% gliomas extend to the optic chiasm or
beyond into the optic radiations & aggressive
* Hollander MD, FitzPatrick M, O''Connor SG, et al. Optic gliomas. Radiol Clin North Am. Jan 1999;37(1):59-71.


… Presentation…

• F>M
• In pediatrics
– Median age…5 yrs
– 80% pts present before age 15
• In adults
– 22-79 yrs with a mean age of 52 yrs

Hollander MD, FitzPatrick M, O''Connor SG, et al. Optic gliomas. Radiol Clin North Am. Jan 1999;37(1):59-71.
Millar WS, Tartaglino LM, Sergott RC, et al. MR of malignant optic glioma of adulthood. AJNR Am J
Neuroradiol. Sep 1995;16(8):1673-6.


… Presentation

• Painless proptosis with inferior dystopia
• Decreased vision
• Nystagmus
• Changes in appetite or sleep
• Large lesion can obstruct 3rd ventricle & ICP
• Optic atrophy
• Opticociliary collaterals
• CRVO


Work-up

CT
• Diffuse Fusiform enlargement of the optic
nerve
MRI
• T1-weighted images
– Isointense to hypointense
• T2-weighted images
– Isointense to hyperintense


Treatment

• Not required if
– Not growing
– Good vision
– No cosmetic blemish
• Surgical excision
– Large & growing lesions
– Confined to orbit
• Radiotherapy + chemotherapy
– Intracranial extension

EMBRYONAL SARCOMA

• Most common primary orbital malignancy of
childhood
• Origin…undifferentiated mesenchymal cells
• Called Rhabdomyosarcoma if differentiate
into striated muscle


Presentation…

• Usually first decade (average…7yrs)
• Boys > girls
• Proptosis
– Rapid (days to weeks)
– Unilateral
– Painful
– Non-axial
• Mimics orbital cellulitis


… Presentation…

• Swollen lids but bluish appearance
• Ptosis
• Location
– Superonasal
– Retro bulbar
– Superior
– Inferior
• Chemosis
• Sinuses involvement & nose bleeds

… Presentation

• Other findings
– Disc edema
– Choroidal folds
– Decreased vision
– Cervical lymph nodes involvement


Courtesy: medline


Work-up

USG
• Relatively well-circumscribed mass
• Low-medium amplitude echoes
CT & MRI
• Moderately well-defined homogenous mass
• Bony destruction
Biopsy
Systemic investigations

Treatment

• Referred to pediatric oncologist
• Radiotherapy
• Chemotherapy
• Surgery
– Exenteration for recurrent & resistant cases


LYMPHANGIOMA

• Introduction
– Non-Neoplastic
– Non-functional
– Abortive
– Vascular malformation
• Hemodynamically isolated
• Bleeding into lumen…chocolate cysts


Presentation…

• Early childhood (average=5-6 yrs)
• Multiple bluish soft masses
• Upper nasal quadrant
• Cystic conjunctival component
• Proptosis
– Due to spontaneous hemorrhage
– Painful
– Axial/non-axial


… Presentation

• Worsened by RTI
• Palatal involvement
• Ptosis
• Restricted eye movements
• Optic nerve compression
• Papilledema
• Secondary glaucoma


Work-up

CT
• Multilobulated cystic mass within the orbit
and/or intralesional calcifications
MRI
• Hemorrhagic cyst…Hyperintense
• Fluid level


Treatment

• Conservative • Intralesional
• Partial surgical injection of
resection of the sclerosing agents
major cyst – sodium tetradecyl
sulfate (Sotradecol®)
• Needle aspiration
– OK-432 (Picibanil®)
• CO2 laser
• local radiotherapy
• Cryotherapy


• Sodium tetradecyl sulfate (Sotradecol®)
• An anionic surface-active agent used for its wetting properties in
industry and used in medicine as an irritant and sclerosing
agent for hemorrhoids and varicose veins
• $259.00


• OK-432 (Picibanil®)
• lyophilized preparation of a low-virulence strain of S. pyogenes
(S. hemolyticus), inactivated by heating with penicillin G. It has
been proposed as a noncytotoxic antineoplastic agent because
of its immune system-stimulating activity.


CONTENTS

• Recap
• Summary
• MCQs

PROPTOSIS

Lid edema Little/no inflammation
Sinus disease Orbital lesion
Restricted motility Young child/adult
Pain/Tender Slow growing
Lid trauma Located near bone
Fever sutures

ORBITAL CELLULITIS Dermoid cyst


PROPTOSIS

Child (5 yrs) Child (7-8yrs)
Upper nasal quadrant mass Hx of nose bleeds
Painful Bluish color lids
Few episodes of proptosis Unilateral
Worsened by RTI Lymph node involved
MRI shows fluid levels Painful

ORBITAL LYMPHANGIOMA Rhabdomyosarcoma


CONTENTS

• Recap
• Summary
• MCQs

TAKE HOME MESSAGE

• Proptosis in children may be
– Benign as … Dermoid
– Malignant as … Rhabdomyosarcoma
• Thorough Hx & clinical approach
• Pediatric ophthalmologist & oncologist


MCQs

1. A 1-month old infant presents with a
purplish, spongy eyelid mass. Most likely
diagnosis:
a. Rhabdomyosarcoma
b. Neurofibroma
c. Dermoid cyst
d. Capillary hemangioma
e. Metastatic Ewing sarcoma

Ans. d


2. In the evaluation of child with unilateral
Proptosis, which assumption is true?
a. Cavernous hemangioma is the most common cause of
unilateral Proptosis in children
b. Thyroid ophthalmopathy is the most common cause of
unilateral Proptosis in children
c. Neurofibroma is the malignant tumor that most commonly
produce Proptosis in children
d. ON meningioma are more common than gliomas in children
e. None of the above

Ans: e


3. The management of Rhabdomyosarcoma of
the orbit usually involves
a. Lumbar puncture to rule out CNS Mets
b. Exenteration
c. Enucleation & orbital radiation
d. Systemic chemotherapy & orbital radiation
e. Radical neck dissection if cervical L. nodes are involved

Ans: d


4. All of the following are true regarding optic
nerve tumors except:
a. ON gliomas in children are associated with NF-1
b. ON meningioma in children are associated with NF-2
c. Radiation therapy is an accepted therapy for ON sheath
meningioma
d. ON gliomas of childhood can be malignant

Ans: d


FOR MCQs 5-8

5. An 11-year old girl presents with acute,
unilateral, left-sided periocular pain,
Proptosis and double vision. Which
condition would NOT be included in D. Dx?
a. Cavernous hemangioma
b. Sinusitis with orbital abscess
c. Traumatic retrobulbar hemorrhage
d. Orbital Lymphangioma

Ans: a


…Continued…
6. 24 hrs later & without any treatment, the pain
has resolved. Periocular ecchymosis
develops, & the double vision stabilized.
MRI shows cystic mass & layered
hemorrhage. Most likely Dx based on
clinical Hx & MRI findings is:
a. Rhabdomyosarcoma
b. Capillary hemangioma
c. Orbital abscess
d. Lymphangioma

Ans: d

…Continued…

7. If the patient was losing vision bcz of this
process, you would consider:
a. Intravenous antibiotics
b. Open surgery to excise the lesion in its entirety
c. CT-guided drainage of encysted blood
d. Biopsy of the lesion to establish Dx

Ans: c


…Continued

8. The disease process is an example of :
a. The most common cause of proptosis in children
b. The most common primary orbital malignancy in children
c. A tumor that may enlarge with upper RTI
d. An orbital vascular lesion that will involute after intralesional
corticosteroids

Ans: c


9. Which one of the following is the most
common primary malignancy of the orbit in
children?
a. Neuroblastoma
b. Rhabdomyosarcoma
c. Ewing’s sarcoma
d. None of the above

Ans: b


10. what is the most common cause of
unilateral childhood proptosis?
A. capillary hemangioma
B. Thyroid related orbitopathy
C. Orbital hemorrhage
D. Orbital cellulitis

Ans: D


11. In orbital cellulitis, which of the following is
false:
a. Common cause of proptosis in children
b. Seen due to infection spread from surrounding sinusitis
c. Commonly bilateral
d. Can cause cavernous sinus thrombosis

Ans: c


12. Most common organism causing orbital
cellulitis in children < 5 yrs:
a. Pneumococci
b. Staph aureus
c. Strep pyogenes
d. H. influenzae

Ans: d


13. ON glioma includes following:
a. Usually seen in 1st decade of life
b. Can be seen either in the intraorbital or intracranial part of
optic nerve
c. Generally causes painless, bilateral proptosis
d. Proptosis is axial, non-Pulsatile & irreducible

1. a+b+c 2. a+b+c+d 3. a+b+d 4. a+c+d

Ans: 3


14. A young child presents with sudden loss of vision &
proptosis of right eye. On examination, the direct
pupillary reflex is absent but consensual pupillary
reflex is present. He is having:
a. ON glioma
b. ON meningioma
c. Retinoblastoma
d. ON angioma
e. Capillary hemangioma

Ans: a


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