Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038379 (strabismus)
 9,317 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Three cases of histologically proven limbal xanthogranuloma are presented. All three cases presented with a yellow raised swelling at the limbus and no other ocular or systemic abnormality. A simple excision of the lesion was carried out in all cases and histological examination revealed features typical of xanthogranuloma. Two patients had a recurrence at the original site, necessitating keratectomy, with insertion of a lamellar graft. We suggest that a primary keratectomy with insertion of a lamellar graft should be performed in cases of limbal xanthogranuloma in order to lessen the risk of recurrence and to provide a better cosmetic result.
J Pediatr Ophthalmol Strabismus
PMID:Limbal xanthogranuloma. 190 55

Uveal juvenile xanthogranuloma (JXG) is a rare intraocular tumor which usually occurs in very young children. Most reported cases of successfully treated uveal juvenile xanthogranuloma have received systemic steroids, irradiation or excision. Some cases have responded to topical steroids alone. We report a case of JXG with recurrent hyphemas and elevated intraocular pressures despite the use of topical steroid. This patient responded to the addition of subconjunctival steroid injection. This is the first report to our knowledge demonstrating a response to periocular steroid supplementation for uveal JXG unresponsive to topical steroids.
J Pediatr Ophthalmol Strabismus
PMID:Subconjunctival steroid in the management of uveal juvenile xanthogranuloma: a case report. 211 75

An 11-month-old boy presented with uniocular hypopyon, elevated intraocular pressure, and iris nodules. A differential diagnosis of physical abuse, infection, retinoblastoma, juvenile xanthogranuloma, and histiocytosis X were considered but initial physical and laboratory investigations all had normal results. Three weeks following initial presentation, the child developed fulminant acute myelogenous leukemia. The clinical course, investigations, and outcome are reviewed.
J Pediatr Ophthalmol Strabismus
PMID:Pseudohypopyon in acute myelogeneous leukemia. 762 68

We describe an unusual presentation of juvenile xanthogranuloma in a 4-year-old girl. Juvenile xanthogranuloma may be associated with posterior manifestations including optic nerve and choroidal granulomas, panuveitis, and vitreous hemorrhage in the absence of the classic anterior segment manifestations.
J Pediatr Ophthalmol Strabismus
PMID:Chorioretinal involvement and vitreous hemorrhage in a patient with juvenile xanthogranuloma. 1612 56

A 3-year-old boy was referred because of the presence of a non-pigmented, highly vascular mass with tapioca appearance involving the superior portion of the left iris. Iris fluorescein angiography revealed early hyperfluorescence of the iris tumor with diffuse, intense late leakage of dye throughout the entire iris, not just in the region of the tumor. Cytopathologic examination revealed Touton giant cells and the presence of histiocytes, confirming the diagnosis of iris juvenile xanthogranuloma. The patient was prescribed topical prednisolone acetate, and the lesion resolved during the next 3 months. Fluorescein angiography may be useful in differentiating iris juvenile xanthogranuloma from malignant or non-inflammatory benign iris lesions.
J Pediatr Ophthalmol Strabismus
PMID:Fluorescein angiography of iris juvenile xanthogranuloma. 1840 59

Juvenile xanthogranuloma is a benign non-Langerhans cell histiocytosis characterized by skin lesions that tend to be self-limited. Ocular lesions can occur in juvenile xanthogranuloma, most commonly presenting as an iris granuloma. Skin lesions of juvenile xanthogranuloma may appear similar to lesions of mastocytosis. Mastocytosis includes a heterogeneous group of diseases characterized by the proliferation and abnormal infiltration of mast cells. Rubbing of cutaneous lesions leads to the release of histamine, causing the lesions to urticate. Juvenile xanthogranuloma and mastocytosis skin lesions occurring concurrently is extremely rare, with only four cases reported. Ocular juvenile xanthogranuloma and cutaneous lesions of mastocytosis have never been described in the same patient in the literature. The authors describe a patient with an ocular juvenile xanthogranuloma presenting at birth with cutaneous mastocytosis developing several years later.
J Pediatr Ophthalmol Strabismus 2014 Dec 12
PMID:Presumed ocular juvenile xanthogranuloma and biopsy-proven cutaneous mastocytosis occurring sequentially in a young boy. 2549 Feb 41

A 6-month-old female infant was referred following three episodes of spontaneous hyphema. Iris juvenile xanthogranuloma was suspected but not clinically visible. Multimodal imaging with fluorescein angiography, anterior segment optical coherence tomography, and ultrasound biomicroscopy were used to detect the flat, transparent lesion and cytopathology confirmed juvenile xanthogranuloma.
J Pediatr Ophthalmol Strabismus 2015 May 14
PMID:Multimodal imaging for detection of clinically inapparent diffuse iris juvenile xanthogranuloma. 2599 41