| |
Epidemiology |
Clinical features |
Investigations |
Management |
| Vascular |
| Capillary hemangioma |
Incidence 1-5% [21-23] |
Ocular: Superficial hemangioma (strawberry nevus)
Subcutaneous hemangioma [12,21]
Enlarges during first year, followed by gradual resolution [22] |
Clinical diagnosis
CT or MRI if deeper lesion [22] |
Ocular:
Monitor for local complications [22,23]
Systemic:
Surgery [20]
Pulsed-dye laser [20]
Steroids [20]
Beta blockers [25] |
| Lymphangioma |
5.6% of benign childhood tumors [26] |
Ocular: Orbital, eyelid, or conjunctival vascular mass [23,26,27]
Acute proptosis triggered by upper respiratory infection [27] |
CT/MRI [22] |
Ocular:
Monitor for local complications [23,27,28]
Surgical debulking [27,28]
Local radiotherapy and intralesional sclerotherapy [23,28]. |
| Orbital varix |
2% of orbital tumors [29,30] |
Ocular: Venous vascular mass dilates during increased venous return–crying, straining, Valsalva [29,30]
Systemic: Cranioorbital or cranionasal anomalies in 5% [29] |
CT/MRI. [33]
Sequential CT scans with and without Valsalva maneuver demonstrate enlargement [33] |
Ocular: Monitor for local complications [13,29]
Surgical debulking [13,31]
Endovascular catheterization [32,34]. |
| Malignant |
| Neuroblastoma |
Incidence: 9 per million [10]
7.5% of pediatric cancers [11] |
Ocular: Spontaneous periocular ecchymosis (SPE) [4,36,37], subconjunctival hemorrhage, proptosis, strabismus, opsoclonus, anisocoria, heterochromia, choroidal metastasis, optic neuropathy [4,38-40].
Systemic: abdominal pain, distension, mass effect, bone pain, thrombocytopenia [4,39,44]. |
Blood tests for catecholamine metabolites
Tissue biopsy
Abdominal plain film, ultrasound, MRI for primary [35].
Whole-body MRI and nuclear medicine for staging [35] |
Ocular: Monitor for local complications [13]
Systemic: multimodal chemotherapy and resection of primary tumor [43,44]
Adjuvant myeloablative and autologous stem cell therapies [45] |
| Rhabdomyosarcoma |
Incidence: 4 per million [11,46]
3% of all pediatric cancers [5] |
Ocular: SPE [5,9,47], palpable mass, periocular edema, ptosis, proptosis, chemosis, choroidal folds, optic disc edema [5].
Systemic: soft tissue mass |
CT/MRI [49]
Incisional or excisional biopsy [49]
Whole body imaging with bone scan for staging [48] |
Ocular/systemic: Surgical debulking, external-beam radiation, systemic chemotherapy [9,50]. |
| Leukemia |
Incidence: 3,000 children per year [52] |
Ocular: infiltration to orbit, conjunctiva, iris, ciliary body, choroid, retina, optic nerve [53]
Retinal vascular tortuosity and occlusion, retinal and vitreous hemorrhage, opportunistic infection, SPE6 [53],
Systemic: anemia, thrombocytopenia, hyperviscosity, and immunosuppression [6,53] |
Bone marrow biopsy [54]
Serum electrolytes and uric acid, complete blood count, coagulation studies, metabolic and viral panel [54]
Ocular exam for papilledema and cranial nerve palsies [6,53]
|
Ocular: Local irradiation and systemic chemotherapy (emergent if optic nerve infiltrate) [55-57]
Systemic: Induction, consolidation, and maintenance chemotherapy [54] |
| Coagulation disorders |
| Aplastic anemia |
Incidence: 2 per million per year [58] |
Ocular: retinal and vitreous hemorrhage, neovascularization, cotton-wool spots, SPE7 [60-62],
Systemic: Anemia, neutropenia, thrombocytopenia, fatigue, pallor, recurrent infections, mucosal hemorrhage, menorrhagia, petechiae [7]. |
CBC, work-up for hepatitis, human immunodeficiency virus, Ebstein-Barr virus |
Ocular: Monitor for local complications
Surgical decompression in rare cases of orbital hemorrhage [7]
Systemic: allogenic bone marrow transplantation [59,63] |
| Thrombocytopenia |
Incidence: 4-6 children per year [64,65] |
Ocular: SPE, mucosal bleeding [8]
Systemic: purpura, petechial rash, mucosal bleeding, history of recent infection, epistaxis, gastrointestinal bleeding, intracranial hemorrhage [65]. |
Platelet count
Complete blood count
Peripheral blood smear [64] |
Systemic: Intravenous immunoglobulin, anti-D immunoglobulin, platelet transfusions for severe cases [66] |
| Inflammatory |
| Orbital myositis |
Female predilection of 2:1 [67,68] |
Ocular: Diplopia, painful proptosis, SPE, ptosis [9,69,70] |
CT of orbits [69] |
Ocular: systemic steroids [69]
Intralesional triamcinolone with prolonged oral steroid taper [72]
Radiotherapy, immunomodulatory drugs, surgical debulking for recalcitrant cases [71,72] |
| Amyloidosis |
Incidence 9 per 100,000 [74] |
Organ-dependent
Ocular: periocular pain, palpable mass, SPE, ptosis, proptosis, motility restriction [17,75,78]
Systemic: congestive heart failure, edema, petechiae, diarrhea, proteinuria, hepatosplenomegaly [17,75,78] |
Biopsy
Subcutaneous fat aspiration with Congo red staining [78] |
Ocular: monitor for complications
Local debulking [77]
Systemic: steroids, mephalan, autologous stem cell transplantation [77,79,80] |
| Miscellaneous |
| Pertussis |
3055 cases per year in the U.S. [82] |
Ocular: SPE and subconjunctival hemorrhage [81,83]
Systemic: series of coughs during a single expiration, followed by a vigorous whooping inspiration [18].
Post-tussive vomiting |
Clinical diagnosis [81] |
Systemic: antibiotics (macrolide, trimethoprim-sulfamethoxazole) [84]
Consider hospitalization if increased work of breathing, cyanosis, apnea, poor feeding. |
| Blue rubber bleb nevus syndrome |
200 reported cases [86] |
Ocular: proptosis, subconjunctival hemorrhage, SPE [19,87,88]
Systemic: Cutaneous lesions resembling blue rubber blebs [85]
Gastrointestinal bleeding [85] |
CT / MRI
Endoscopy [85] |
Ocular: Monitor for local complications
Surgical debulking if exposure keratopathy [88]
Systemic: gastroenterology evaluation, endoscopic removal if bleeding [85] |
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