Cases reported "Scleral Diseases"

Filter by keywords:



Filtering documents. Please wait...

1/7. Posterior scleral choristoma in the organoid nevus syndrome (linear nevus sebaceus of Jadassohn).

    PURPOSE: To highlight the association of posterior osseous and/or cartilaginous ocular choristomas with epibulbar choristomas and the nevus sebaceus of Jadassohn. DESIGN: Small case series. PARTICIPANTS: Four patients with the organoid nevus syndrome. methods: Clinical and histopathologic studies in four patients with epibulbar lesions and nevus sebaceus of Jadassohn. MAIN OUTCOME MEASURES: Ophthalmoscopic findings of peripapillary lesions. Computed tomographic and ultrasonographic characteristic of posterior scleral lesions. Ocular histopathologic findings in one globe from one of the study subjects. RESULTS: Three patients had the triad of posterior osseous/cartilaginous ocular choristomas, anterior epibulbar choristomas, and nevus sebaceus of Jadassohn and one patient had anterior epibulbar choristomas and posterior osseous/cartilaginous ocular choristomas. ultrasonography and computed tomography were valuable in detecting scleral ossification or epibulbar cartilage or both. The ophthalmoscopic findings were similar to those of a choroidal osteoma. CONCLUSIONS: The presence of posterior osseous/cartilaginous ocular choristomas in a patient with epilepsy or epibulbar lesions or both suggests the diagnosis of nevus sebaceus of Jadassohn. Osseous/cartilaginous ocular choristomas should be suspected in patients with nevus sebaceus of Jadassohn and peripapillary hypopigmented fundus lesions.
- - - - - - - - - -
ranking = 1
keywords = choristoma
(Clic here for more details about this article)

2/7. Complex ocular choristomas in linear nevus sebaceus syndrome: a report of two cases.

    Two cases of linear nevus sebaceus syndrome (LNSS) are described in which ipsilateral facial nevus of Jadassohn was associated with complex ocular choristoma. One patient also had scleral osteomas, a rare occurrence in LNSS.
- - - - - - - - - -
ranking = 0.41666666666667
keywords = choristoma
(Clic here for more details about this article)

3/7. Epibulbar osseous choristomas with scleral involvement.

    Epibulbar osseous choristomas are rare congenital collections of mature compact bone most often located in the superotemporal fornix. Most cases are not recognized preoperatively, as the lesion is usually felt to represent a dermatolipoma or epibulbar dermoid. Although most previous reports have described a loose attachment to the surrounding orbital structures, we report two cases in which intimate scleral incorporation was present. In one case, preoperative computerized tomography and echography allowed the diagnosis and probable scleral involvement to be considered preoperatively, and surgery was deferred. The embryogenesis of these lesions is uncertain, although they seem to correlate with the scleral ossicles seen in avian anatomy, and may represent abnormal activation of embryonic pluripotential mesenchymal cells.
- - - - - - - - - -
ranking = 0.41666666666667
keywords = choristoma
(Clic here for more details about this article)

4/7. Epibulbar complex choristoma associated with nevus sebaceus.

    We encountered an infant with nevus sebaceus of Jadassohn who was born with a striking pedunculated mass on the superotemporal aspect of the anterior sclera and limbus of the left eye. In addition, two small choroidal colobomas were seen in the papillomacular bundle area of the same eye. Histopathologic examination of the excised mass revealed a mixture of ectodermal and mesodermal elements, including cartilage and bone. These findings led to the rare diagnosis of epibulbar complex choristoma.
- - - - - - - - - -
ranking = 0.41666666666667
keywords = choristoma
(Clic here for more details about this article)

5/7. Episcleral osteocartilaginous choristoma.

    PURPOSE/METHOD: A 31-year-old woman had a hypertropia and an episcleral choristoma in the superotemporal quadrant of the globe. The choristoma was examined. RESULTS/CONCLUSIONS: Gross and histopathologic examination showed bone and cartilage components in the tumor, which refutes the previously accepted membranous growth pattern of episcleral osseous choristomas.
- - - - - - - - - -
ranking = 0.58333333333333
keywords = choristoma
(Clic here for more details about this article)

6/7. Ophthalmic features of the organoid nevus syndrome.

    BACKGROUND/PURPOSE: The organoid nevus (sebaceous nevus) syndrome is characterized primarily by cutaneous sebaceous nevus, seizures, and epibulbar choristomas. On the basis of ophthalmoscopic and computed tomographic studies, a yellow fundus lesion recently observed in this syndrome has been called a coloboma by some investigators and a choroidal osteoma by others. This study was undertaken to review our personal experience with the organoid nevus syndrome, to review the English language literature on the subject, and to address some misconceptions regarding its ocular manifestations. methods: We reviewed the records of patients with the organoid nevus syndrome who were personally evaluated by the investigators. The ocular findings were studied in more detail, with emphasis on the epibulbar and fundus lesions. RESULTS: We identified five patients with the organoid nevus syndrome. Four had a classic sebaceous nevus in the facial and scalp area, and 2 had seizures and arachnoid cysts. All 5 patients had an epibulbar tumor, which proved to be a complex choristoma in one case that was studied histopathologically. A characteristic ophthalmoscopic feature, observed in the 4 patients with clear ocular media, was a flat yellow discoloration of the posterior fundus, of variable size and shape, which appeared to correlate with a dense plaque noted on ultrasonography and computed tomography. In 1 case, histopathologic examination showed that this posterior lesion contained intrascleral cartilage. CONCLUSIONS: Our observations and a review of the literature indicated that the organoid nevus syndrome has varied manifestations. Like the closely related phakomatoses, it often occurs as a forme fruste, without full expression of the syndrome. The most important ocular manifestations are an epibulbar mass, compatible with a complex choristoma, and focal yellow discoloration in the fundus, probably related to intrascleral cartilage.
- - - - - - - - - -
ranking = 0.25
keywords = choristoma
(Clic here for more details about this article)

7/7. Ocular manifestations of the organoid nevus syndrome.

    BACKGROUND: The organoid nevus (sebaceous nevus) syndrome is characterized primarily by cutaneous sebaceous nevus, seizures, and epibulbar choristomas. Based on ophthalmoscopy and computed tomography (CT), a yellow fundus lesion recently observed in this syndrome has been called a coloboma by some authors or a choroidal osteoma by others. This study was undertaken to review the authors' personal experience with the organoid nevus syndrome, to review the English language literature on the subject, and to address some misconceptions regarding its ocular manifestations. methods: The authors reviewed the records of patients with the organoid nevus syndrome who were personally evaluated by the authors. The ocular findings were studied in more detail, with emphasis on the epibulbar and fundus lesions. RESULTS: The authors identified five patients with the organoid nevus syndrome. Four had a classic sebaceous nevus in the facial and scalp area and two had seizures and arachnoid cysts. All five patients had an epibulbar tumor, which proved to be a complex choristoma in one case that was studied histopathologically. A characteristic ophthalmoscopic feature, observed in the four patients with clear ocular media, was a flat, yellow discoloration of the posterior fundus, of variable size and shape, that appeared to correlate with a dense plaque noted on ultrasonography and CT. In one case, histopathologic studies showed that this posterior lesion contained intrascleral cartilage. CONCLUSIONS: The authors' observations and a review of the literature indicated that the organoid nevus syndrome has varied manifestations. Just like the closely related phakomatoses, it often occurs as a forme fruste, without full expression of the syndrome. The most important ocular manifestations are an epibulbar mass, compatible with a complex choristoma, and focal, yellow discoloration in the fundus, probably related to intrascleral cartilage.
- - - - - - - - - -
ranking = 0.25
keywords = choristoma
(Clic here for more details about this article)


Leave a message about 'Scleral Diseases'


We do not evaluate or guarantee the accuracy of any content in this site. Click here for the full disclaimer.