Most common malignant cause of bilateral proptosis in children? (AIPG 2011)
|A||Acute leukemic leukem|
|D||Acute Myeloid leukemia|
(Ref. Indian journal of Medical and Paediatric Oncology; Vol. 25 Suppl. 2, 2004)
Indian journal of Medical and Paediatric Oncology; Vol. 25 Suppl. 2, 2004; “Approach by K. Ganessan and Sameer Bakshi”
1. Orbital lymphomas are the most common malignant orbital tumors in adults.
2. Unilateral > bilateral
3. Typically superior orbit is the site
4. Slow onset and progression
5. Subconjunctival “salmon-colored" mass in the fornix
6. CT scan:
a. Poorly defined mass conforming to the shape of the orbital bones and globe without bony erosion; orbital biopsy necessary.
7. Definitive treatment is radiation.
1. The most common primary orbital malignancy of childhood.
2. The average age of presentation is 7 yrs.
3. rapidly progressive mass in the superior orbit with proptosis, globe displacement, and eyelid swelling
4. Prompt diagnosis with orbitotomy and biospy is crucial, as overall mortality is 60% once the disease extends to orbital bones.
5. Current Rx strategy is chemoradiation.
1. multisystem. autoimmune disorder.
2. hyperthyroid, hypothyroid, euthyroid.
3. inflammation and enlargement EOM.
4. fusiform enlargement sparing the tendon (“Coke-bottle sign”).
5. C/f: Proptosis, Eyelid retraction, Corneal exposure, Diplopia, Optic nerve compression.
6. Treatment depending on the severity
Orbital inflammatory pseudotumor:
1. Nonspecific idiopathic inflammatory
2. Localized to muscle, lacrimal gland, sclera or diffuse
3. Eyelid erythema or edema, Palpable mass, Decreased vision, uuveitis, hyperopic shift,optic nerve edema
4. Bilateral disease more common in children
5. CT scan diagnostic study:
a. Thickening 1+ EOM (inc. tendons)
b. Lacrimal gland enlargement
c. Thickening of the posterior sclera
6. Treatment corticosteroids +/- radiation
Optic nerve glioma (juvenile pilocytic astrocytoma)
1. slow-growing tumor of childhood
2. Decreased visual acuity with a RAPD
3. CT scan or MRI
a. “fusiform” enlargement of the ON with/without kinking of the nerve with good contrast enhancement.
4. associated with NF1 - if bilateral.