Ciliary body and choroidal pseudomelanoma from ultrasonographic imaging of hypermature cataract in 20 cases.

PURPOSE: To describe a series of 20 patients with opaque media, referred with uveal melanoma, but subsequently found to have pseudomelanoma from oblique imaging of hypermature cataract on ultrasonography. DESIGN: Case series. PARTICIPANTS: Twenty patients. METHODS: Retrospective chart review. MAIN OUTCOME MEASURES: Ultrasound imaging. RESULTS: All eyes had opaque media from hypermature cataract with no view of the fundus. All were referred because of suspected uveal melanoma, based on ultrasonographic imaging. The echogenic mass appeared to be in the ciliary body (n = 17) or choroid (n = 3). The median patient age was 54 years (range, 17-86 years). Most patients were white (n = 13) or black (n = 4). There was a history of eye trauma (n = 3) and ocular surgery (n = 1). Visual acuity was light perception to hand movements (n = 18) and 20/50 to 20/100 (n = 2). The cataract was in an anatomic position (n = 18) or subluxated (n = 2). On B-scan ultrasonography, the mass was dome-shaped (n = 10) or elliptical (n = 10), displayed an acoustically hollow center with a dense rim (n = 20), and was located in the ciliary body (n = 17) or choroid (n = 3). The mean thickness was 7.2 mm and the mean base was 9.3 mm. Features suggestive of cataract rather than melanoma included a lack of contiguity with the uvea (n = 20) on videoimaging using standard ultrasonography and ultrasound biomicroscopy, a lack of a transillumination shadow, and a lack of a sentinel vessel. For those in the ciliary body region, an additional feature was the ultrasonographic presence of mass in all 4 quadrants (n = 17), representing oblique imaging of the lens equator. For those in the choroid region, the pseudomelanoma shifted when the patient was imaged in a reclined compared with an upright position. After cataract surgery, the lack of melanoma was confirmed. CONCLUSIONS: Dense cataract can preclude a fundus view, necessitating ultrasonography for imaging the posterior segment of the eye. Ultrasonographic confusion with a ciliary body and choroidal melanoma can occur because the dome-shaped cataract can simulate a dome-shaped melanoma.

Vascular tumors of the conjunctiva in 140 cases.

OBJECTIVE: To report clinical and histopathologic features of vascular tumors of the conjunctiva. DESIGN: Retrospective, noninterventional case series. PARTICIPANTS: A total of 140 patients. INTERVENTION: None. MAIN OUTCOME MEASURES: Tumor diagnosis, anatomic location, clinical features, management, and histopathology. RESULTS: There were 140 vascular tumors of the conjunctiva with 93% benign and 7% malignant. The specific diagnoses included lymphangioma/lymphangiectasis (n = 54, 36%), pyogenic granuloma (n = 31, 22%), capillary hemangioma (n = 20, 14%), Kaposi's sarcoma (n = 10, 7%), acquired sessile hemangioma (n = 10, 7%), racemose hemangioma (n = 7, 5%), varix (n = 4, 3%), cavernous hemangioma (n = 3, 2%), and glomangioma (n = 1, <1%). The lesions were unilateral in 89% and discovered at median age of 41 years. Bilateral lesions included lymphangiectasia, Kaposi's sarcoma, and racemose hemangioma. All tumors were typically found in adults with the exception of capillary hemangioma (<1 year) and glomangioma (16 years). The median tumor diameter was 6 mm, with the largest median diameter (15 mm) with Kaposi's sarcoma and (12 mm) glomangioma and capillary hemangioma and the smallest median diameter (3 mm) with cavernous hemangioma. The most common anatomic sites included bulbar conjunctiva, which was extralimbal in 80 patients (57%), limbal in 19 patients (14%), tarsal conjunctiva in 27 patients (19%), and forniceal conjunctival in 24 patients (17%). Clinical features included cystic component in 24% of patients (seen with lymphangioma, glomangioma, and varix), well-defined margins in 64% of patients, and feeder vessels in 39% of patients. CONCLUSIONS: A variety of vascular tumors can occur in both children and adults at various anatomic locations in the conjunctiva. They tend to occur unilaterally on the bulbar conjunctiva, and the majority of tumors are benign.

Phacomatosis pigmentovascularis of cesioflammea type in 7 patients: combination of ocular pigmentation (melanocytosis or melanosis) and nevus flammeus with risk for melanoma.

OBJECTIVE: To describe the features of phacomatosis pigmentovascularis (cesioflammea type). DESIGN: Noninterventional retrospective case series composed of 7 patients. RESULTS: Nevus flammeus combined with ipsilateral ocular melanocytosis or melanosis was seen in all 7 patients. Additional contralateral nevus flammeus was observed in 3 patients. Nevus flammeus (unilateral in 4 patients and bilateral in 3 patients) was distributed in trigeminal nerves V1 (n = 3), V2 (n = 7), and V3 (n = 5). Related findings included diffuse choroidal hemangioma (n = 1) and glaucoma (n = 1), with no patients having brain hemangioma or seizures. Ocular pigmentary abnormalities (unilateral in all 7 patients) included congenital ocular melanocytosis (n = 6) and conjunctival acquired melanosis (n = 1). Pigmentation was sectorial (partial) in 5 patients and complete in 2 patients. Melanocytosis involved the periocular skin in 1 patient, sclera in 2 patients, iris in 2 patients, and choroid in 4 patients. In 3 of 6 patients, melanocytosis was visible in the choroid only on dilated fundus evaluation. Related tumors included choroidal melanoma (n = 3), optic disc melanocytoma (n = 1), and conjunctival melanoma in situ (primary acquired melanosis) (n = 1). Melanoma metastasis developed in 1 patient. CONCLUSIONS: Phacomatosis pigmentovascularis shows features of nevus flammeus and more serious ocular pigmentary abnormalities (uveoscleral melanocytosis and conjunctival melanosis). Melanocytosis may be detected only by dilated ocular fundus examination, as found in 3 of 6 patients. Furthermore, choroidal melanoma can develop from melanocytosis, as noted in 3 of our 6 patients (50%). All patients with nevus flammeus should be examined for phacomatosis pigmentovascularis by an ophthalmologist because ocular melanocytosis and uveal melanoma may remain hidden within the eye.

Acquired sessile hemangioma of the conjunctiva: a report of 10 cases.

PURPOSE: To report 10 cases of an unusual conjunctival lesion that has been termed acquired sessile hemangioma. DESIGN: Retrospective, interventional case series. METHODS: A chart review was conducted on patients coded as having a conjunctival vascular tumor at the Ocular Oncology Service at Wills Eye Institute, Thomas Jefferson University. A cohort of these patients was identified as having a lesion comprising a complex sessile arrangement of conjunctival blood vessels that were remarkably similar to one another, but were distinctly different from other known conjunctival vascular tumors and malformations. These lesions were called acquired sessile hemangioma of the conjunctiva. Each case was evaluated for patient age, gender, race, laterality, and follow-up. Each tumor was assessed for anatomic location in the conjunctiva, quadrant of involvement, diameter, thickness, margin (sharp or ill-defined), and presence of feeder blood vessels. RESULTS: Ten cases were identified. The median age at the time of diagnosis was 58 years (mean, 58 years; range, 31-83 years). There were 8 women and 3 men, and all patients were white. Only 1 patient was referred specifically for the vascular lesion. In the other 9 cases, it was a coincidental finding by the authors on referral for unrelated problems. There were no related systemic or ocular findings that seemed to be related to acquired sessile hemangioma. In each case, acquired sessile hemangioma was characterized by a flat array of intertwining, mildly dilated blood vessels, usually on the bulbar conjunctiva. Fluorescein angiography, performed in 1 case, demonstrated a feeding artery, draining vein, and leakage of dye from the deeper blood vessels in the lesion and minimal or no leakage of dye from the more superficial blood vessels. Histopathologic analysis, obtained in 1 case, disclosed that the lesion comprised 2 to 3 layers of dilated congested blood vessels that were otherwise of normal appearance. A literature review failed to detect any previous reports on this lesion. CONCLUSIONS: Acquired sessile hemangioma is an unusual conjunctival vascular lesion of adults that has characteristic clinical features. It should be differentiated from other conjunctival vascular lesions. There are no systemic associations. The best management is observation only, since most remain stable and have no known complications.

Langerhans cell histiocytosis of the uvea with neovascular glaucoma: diagnosis by fine-needle aspiration biopsy and management with intraocular bevacizumab and brachytherapy.

A 6-year-old boy with known multisystem Langerhans cell histiocytosis developed photophobia, conjunctival injection, iris neovascularization, and an iridociliochoroidal mass. Fine-needle aspiration biopsy revealed mononucleated and multinucleate histiocytes that demonstrated positive immunostaining for CD68 and S100 consistent with Langerhans cell histiocytosis. Management with intracameral bevacizumab (1.25 mg/0.05 mL) resolved the iris neovascularization, and plaque radiotherapy (brachytherapy) resolved the mass rapidly and completely, preserving the patient's visual acuity and preventing glaucoma during the 10-month follow-up.

Vascular tumors of the iris in 45 patients: the 2009 Helen Keller Lecture.

OBJECTIVE: To report on a series of vascular tumors of the iris. DESIGN: Noncomparative case series. A retrospective medical record review of all patients with an iris vascular tumor was performed to identify the clinical features and develop a simple classification of these lesions. Included were demographics, clinical features, systemic associations, complications, management, and histopathology. RESULTS: There were 54 eyes in 45 patients with an iris vascular tumor. These were categorized as racemose hemangioma (41 eyes: 29 simple and 12 complex), cavernous hemangioma (3 eyes: 2 localized and 1 systemic), capillary hemangioma (1 eye, localized), varix (3 eyes, localized), and microhemangiomatosis (6 eyes, localized). The hemangiomas occurred in adults at a median age of 55 years, whereas capillary hemangioma occurred in infancy and cavernous hemangioma with systemic involvement occurred in a child. Of the 41 eyes with iris racemose hemangioma, none showed systemic involvement. Of all 54 eyes, transient hyphema was the main complication, found at some point in 30% or more of each affected eye except for iris capillary and racemose hemangioma. Surgical resection was necessary in 1 cavernous hemangioma and 1 varix. The remainder were managed with observation. CONCLUSIONS: There are now well-documented examples of iris racemose hemangioma, cavernous hemangioma, capillary hemangioma, varix, and microhemangiomatosis. Transient hyphema is the main complication. Observation is usually advised. Most are solitary lesions confined to the iris and some (cavernous hemangioma and microhemangiomatosis) can have important systemic associations.

Lower extremity arterial injury patterns and reconstructive outcomes in patients with severe lower extremity trauma: a 26-year review.

BACKGROUND: Management of severe traumatic lower extremity injuries remains a considerable challenge. Free tissue transfer is now a standard part of reconstruction for Gustilo IIIB and IIIC injuries. There is limited information on arterial injury patterns in this population. We undertook a review of our experience to gain insight on vascular injury patterns and surgical outcomes. STUDY DESIGN: A 26-year retrospective analysis was performed of all lower extremity Gustilo IIIB and IIIC injuries requiring microvascular reconstruction at New York University Medical Center. Patient demographics, Gustilo classification, angiographic findings (conventional/computed tomographic angiography/magnetic resonance angiography), recipient vessels, elapsed time from injury, flap choices, and outcomes were examined. RESULTS: Two hundred twenty-two free flaps on 191 patients were performed from September 1982 until March 2008. There were 151 males and 40 females ranging in age from 4 to 83 years (median age 33 years). Patients sustained either Gustilo IIIB (170 patients) or IIIC (21 patients) open fractures. One hundred fifty-four patients had angiograms (78.2% IIIB, 100% IIIC). Sixty-six (42.9%) had normal 3-vessel runoff and 88 (57.1%) were abnormal. Sixty-one patients (31.9%) had anterior tibial injuries, 17 patients (8.9%) had posterior tibial injuries, and 30 (15.7%) had peroneal injuries. Sixty-three complications occurred (11 early thrombosis, 33 requiring secondary procedures, and 10 requiring amputation). CONCLUSIONS: Angiography of severe lower extremity injuries requiring free flap reconstruction usually revealed arterial injury and is generally indicated. In our experience, the anterior tibial artery is most commonly injured and the posterior tibial artery is most likely to be spared and used as a recipient.