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Ultrabiomicroscopic-histopathologic correlations in individuals with autosomal dominant congenital microcoria: three-generation family report.

Ramirez-Miranda A, Paulin-Huerta JM, Chavez-Mondragón E, Islas-de la Vega G, Rodriguez-Reyes A - Case Rep Ophthalmol (2011)

Bottom Line: Further, we analyzed novel ultrasound biomicroscopy findings in the family members who did not require surgery.Histopathology of the iris showed a significant reduction of smooth muscle cells, but no alterations of the anterior lens capsule.Ultrasound biomicroscopy could be a great option to confirm the disorder.

View Article: PubMed Central - PubMed

Affiliation: Anterior Segment Department, Instituto de Oftalmología Fundación Conde de Valenciana, Mexico City, Mexico.

ABSTRACT

Background: Congenital microcoria (CMC) is due to a maldevelopment of the dilator pupillae muscle of the iris, with a pupil diameter of less than 2 mm. It is associated with juvenile open angle glaucoma and myopia. We report on a three-generation Mexican-Mestizo family with CMC. The eldest member's iris biopsy proved muscle anomalies. Further, we analyzed novel ultrasound biomicroscopy findings in the family members who did not require surgery.

Patients and methods: A 62-year-old woman, her 41-year-old son and her 9-year-old grandson affected with microcoria since birth, documented by clinical examination and ultrasound biomicroscopy. The eldest member underwent phacoemulsification, and a biopsy of the iris and the anterior capsule of the lens was taken.

Results: Ultrasound biomicroscopy confirmed the CMC diagnosis showing iris thinning and a pupil diameter of less than 2 mm. Histopathology of the iris showed a significant reduction of smooth muscle cells, but no alterations of the anterior lens capsule.

Discussion: Although CMC is a rare disorder, which is due to a maldevelopment of the dilator pupillae muscle of the iris, it could be associated with juvenile open angle glaucoma and myopia; therefore, precise diagnosis is required. Ultrasound biomicroscopy could be a great option to confirm the disorder.

No MeSH data available.


Related in: MedlinePlus

a Histological section of pupillary border showing fusiform and dendritic melanocytes, a few blood vessels, and no smooth muscle cell capsules and an underlying pigmentary epithelial layer (H&E stain). b Histological section of iris middle stroma with a few smooth muscle fibers and a thin appearance. c Masson's trichrome stain showing melanocytes in the iris anterior stroma, smooth muscle fibers, connective tissue and pigmentary epithelium. d PAS stain of the anterior lens capsule showing regular thickness base membrane and cuboidal epithelial cells underneath.
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Figure 3: a Histological section of pupillary border showing fusiform and dendritic melanocytes, a few blood vessels, and no smooth muscle cell capsules and an underlying pigmentary epithelial layer (H&E stain). b Histological section of iris middle stroma with a few smooth muscle fibers and a thin appearance. c Masson's trichrome stain showing melanocytes in the iris anterior stroma, smooth muscle fibers, connective tissue and pigmentary epithelium. d PAS stain of the anterior lens capsule showing regular thickness base membrane and cuboidal epithelial cells underneath.

Mentions: The postoperative recovery was uneventful; 1 day postoperatively the uncorrected Snellen visual acuity was 20/25 in both eyes. At 1 month, the pupil was 5 mm in size, with still no response to mydriatics (fig. 1c, d). The histopathological examination of the removed pupillary margin revealed smooth sphincter muscle fibers, fusiform and dendritic melanocytes and pigmentary epithelium cells, and the iris stroma showed fusiform and dendritic melanocytes, a few blood vessels and no smooth muscle cells (fig. 3a, b). Application of Masson's trichrome histochemical stain showed melanocytes in the iris anterior stroma, smooth muscle fibers, connective tissue, and pigmentary epithelium (fig. 3c). The removed lens capsule was stained with periodic acid-Schiff (PAS) which revealed regular thickness base membrane and cuboidal epithelial cells underneath (fig. 3d).


Ultrabiomicroscopic-histopathologic correlations in individuals with autosomal dominant congenital microcoria: three-generation family report.

Ramirez-Miranda A, Paulin-Huerta JM, Chavez-Mondragón E, Islas-de la Vega G, Rodriguez-Reyes A - Case Rep Ophthalmol (2011)

a Histological section of pupillary border showing fusiform and dendritic melanocytes, a few blood vessels, and no smooth muscle cell capsules and an underlying pigmentary epithelial layer (H&E stain). b Histological section of iris middle stroma with a few smooth muscle fibers and a thin appearance. c Masson's trichrome stain showing melanocytes in the iris anterior stroma, smooth muscle fibers, connective tissue and pigmentary epithelium. d PAS stain of the anterior lens capsule showing regular thickness base membrane and cuboidal epithelial cells underneath.
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC3104862&req=5

Figure 3: a Histological section of pupillary border showing fusiform and dendritic melanocytes, a few blood vessels, and no smooth muscle cell capsules and an underlying pigmentary epithelial layer (H&E stain). b Histological section of iris middle stroma with a few smooth muscle fibers and a thin appearance. c Masson's trichrome stain showing melanocytes in the iris anterior stroma, smooth muscle fibers, connective tissue and pigmentary epithelium. d PAS stain of the anterior lens capsule showing regular thickness base membrane and cuboidal epithelial cells underneath.
Mentions: The postoperative recovery was uneventful; 1 day postoperatively the uncorrected Snellen visual acuity was 20/25 in both eyes. At 1 month, the pupil was 5 mm in size, with still no response to mydriatics (fig. 1c, d). The histopathological examination of the removed pupillary margin revealed smooth sphincter muscle fibers, fusiform and dendritic melanocytes and pigmentary epithelium cells, and the iris stroma showed fusiform and dendritic melanocytes, a few blood vessels and no smooth muscle cells (fig. 3a, b). Application of Masson's trichrome histochemical stain showed melanocytes in the iris anterior stroma, smooth muscle fibers, connective tissue, and pigmentary epithelium (fig. 3c). The removed lens capsule was stained with periodic acid-Schiff (PAS) which revealed regular thickness base membrane and cuboidal epithelial cells underneath (fig. 3d).

Bottom Line: Further, we analyzed novel ultrasound biomicroscopy findings in the family members who did not require surgery.Histopathology of the iris showed a significant reduction of smooth muscle cells, but no alterations of the anterior lens capsule.Ultrasound biomicroscopy could be a great option to confirm the disorder.

View Article: PubMed Central - PubMed

Affiliation: Anterior Segment Department, Instituto de Oftalmología Fundación Conde de Valenciana, Mexico City, Mexico.

ABSTRACT

Background: Congenital microcoria (CMC) is due to a maldevelopment of the dilator pupillae muscle of the iris, with a pupil diameter of less than 2 mm. It is associated with juvenile open angle glaucoma and myopia. We report on a three-generation Mexican-Mestizo family with CMC. The eldest member's iris biopsy proved muscle anomalies. Further, we analyzed novel ultrasound biomicroscopy findings in the family members who did not require surgery.

Patients and methods: A 62-year-old woman, her 41-year-old son and her 9-year-old grandson affected with microcoria since birth, documented by clinical examination and ultrasound biomicroscopy. The eldest member underwent phacoemulsification, and a biopsy of the iris and the anterior capsule of the lens was taken.

Results: Ultrasound biomicroscopy confirmed the CMC diagnosis showing iris thinning and a pupil diameter of less than 2 mm. Histopathology of the iris showed a significant reduction of smooth muscle cells, but no alterations of the anterior lens capsule.

Discussion: Although CMC is a rare disorder, which is due to a maldevelopment of the dilator pupillae muscle of the iris, it could be associated with juvenile open angle glaucoma and myopia; therefore, precise diagnosis is required. Ultrasound biomicroscopy could be a great option to confirm the disorder.

No MeSH data available.


Related in: MedlinePlus