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Purpose To provide clinical and pathological features of posterior persistent fetal vasculature (PFV) presenting with vitreous hemorrhage. Observations Case 1 was a one-year old male with PFV reaching up to the posterior lens capsule. Case 2 and 3 both had history of blunt trauma. B-scans in cases 2 and 3 revealed vitreous hemorrhage and an intravitreal tissue attached to the optic disc. Pre-operative visual acuity in cases 1, 2 and 3 was undetermined due to age, hand motion and light perception, respectively. During vitrectomy, a fibrotic stalk attached to the optic nerve was removed, which consisted of fibrovascular tissue enveloping pockets of hemorrhage histopathologically. The fibrovascular tissue contained smooth muscle actin (SMA) positive spindle-shaped myofibroblasts in one case and hemosiderin-laden macrophages in another case. Glial fibrillary acidic protein (GFAP) stain was focally positive in two specimens. The proliferation index was low using Ki-67 stain in all cases. Post-operative visual acuity in case 3 remained unchanged, while improved in case 2 from hand motion to 20/70. There was no recurrence of the vitreous hemorrhage. Conclusion and Importance Vitreous hemorrhage may occur in cases of PFV with or without history of blunt trauma. Hemorrhage within the persistent fetal vasculature may become organized with reactive process in the hyaloid stalk. The fibrovascular stalk contained astrocytes and myelofibroblasts which contribute to the formation and contractile function of PFV, respectively. The outcomes following vitrectomy seemed to be satisfactory.

Purpose Myeloid sarcoma (MS) of the orbit is an uncommon condition in occurring in children, generally coupled to myeloproliferative neoplasms. Observations We describe two rare cases of orbital MS in young boys with aggressive local symptoms but without evidence of acute myeloid leukemia (AML), both patients underwent orbitotomy for gross-tumor resection and biopsy. At follow up, there was no evidence of recurrence nor evolution of the myeloproliferative neoplasms clinically and by radiological and laboratory work-up. We also provide a detailed description of the magnetic resonance imaging presentation, with an extensive pathological analysis correlation. Conclusions and importance A comprehensive revision of the literature on isolated orbital MS was carried out with particular emphasis on clues for differential diagnosis and treatment options, stressing the need to consider MS even in the absence of sign and symptoms of an underlying myeloproliferative disorders.