Table 1.
Clinical Features of 5 patients with ALHE
| Case/Sex/ Age, y/Race |
Symptoms/Duration/ Side of involvement |
Visual acuity* |
Pertinent ophthalmic exam findings* |
Radiologic/laboratory findings |
Suspected diagnosis |
Intervention (s) | Histopathology findings* |
Follow-up examination |
|---|---|---|---|---|---|---|---|---|
| 1/F/12/H | Upper eyelid ptosis and swelling/Six months/Right | 20/20 | Upper lid ptosis, inferior globe displacement, 5mm proptosis | CT: left lacrimal gland swelling and proptosis Lab: elevated serum ACE levels |
Sarcoidosis | Oral steroids, Incisional biopsy | Fibrovascular tissue infiltrated with eosinophils, lymphocytes and plasma cells | Persistent lesion at 3- month follow- up |
| 2/F/11/H | Intermittent periorbital swelling and redness/One year/Left | 20/25 | 1.5 mm proptosis with normal motility, ptosis, palpable lacrimal gland mass | MRI: diffuse enlargement of the left lacrimal gland Lab: mildly elevated ACE levels but normal pulmonary evaluation |
Sarcoidosis | Incisional biopsy | Fibrovascular tissue with numerous eosinophils and lymphoid follicles surrounding blood vessels-- rare follicles | No recurrence at 1-year follow-up |
| 3/M/31/W | Ptosis/One month/Right | 20/50 | −3 restriction in all fields of gaze, ptosis | CT: well circumscribed extraconal mass measuring 40 × 24 × 23 mm | Lymphoma | Incisional biopsy | Fibrovascular tissue with sporadic lymphoid follicles infiltrated with numerous eosinophils, lymphocytes, and plasma cell--rare follicles | No recurrence at 6-year follow-up |
| 4/M/86/W | Upper lid ptosis/Three years/Left | 20/40 | Upgaze restriction, ptosis, palpable well circumscribed mass | CT: well circumscribed anterior orbital mass | Lymphoma Metastasis Hemangioma Cyst | Incisional biopsy | Proliferation of small and medium blood vessels and foci of inflammatory cells compose of lymphocytes and eosinophils | No recurrence at 5-year follow-up |
| 5/M/50/B | Left upper and lower lid lesions/Six years/left | NA | Upper and lower eyelid lesions, ptosis | Not performed | ALHE | Excisional biopsy | Vascular hyperplasia with plumped endothelial cells and numerous eosinophils, lymphocytes and plasma cells--rare follicles | No recurrence at 1-year follow-up |
*
The visual acuity and ophthalmic exam finding are reported for the involved side only. The vascular endothelium in all cases demonstrated atypia (histiocytes-like with intracytoplasmic vacuoles)
W: White
H: Hispanic
B: Black
NA: Not available