PMC3656672_01
Right: facial dysmorphism includes an elongated face with prominent broad nose. Left: side view of the face shows a beaked nose.
cop-0004-0084-g01.jpg
PMC3656672_01_cop-0004-0084-g01.jpg
F1
A 16-year-old female orphan (fig. 1) has been complaining of bilateral visual loss for several years. The patient had an elongated face and beaked nose (fig. 1).
Top left: slit lamp photography of the anterior segment of the left eye. The slit passes by the edge of the miotic pupil after dilation. Note the flat anterior chamber. Top right: slit lamp photography of the anterior segment of the right eye shows prominent nasal bleb, flat anterior chamber and central superficial corneal opacity. Bottom left: slit lamp photography of the anterior segment of the right eye shows prominent nasal bleb, flat anterior chamber and central superficial corneal opacity. Bottom right: following injection of hyaluronic acid to deepen the anterior chamber in the right eye, there was a sudden appearance of the clear lens into the anterior chamber. Note the central corneal scar.
cop-0004-0084-g02.jpg
PMC3656672_01_cop-0004-0084-g02.jpg
F2
She also had iridocorneal touch with a diffusely flat anterior chamber (fig. 2). Conjunctival blebs were evident superiorly and nasally (fig. 2) in both eyes with an intraocular pressure of 8 mm Hg. UBM probe (Eye cubed Ellex; Ellex Innovative Imaging Inc., Sacramento, Calif., USA). Under general anesthesia, attempts at deepening the anterior chamber with sodium hyaluronate 3% led to spontaneous dislocation of the lens into the anterior chamber (fig. 2), facilitating its aspiration (fig. 2).
Anterior segment OCT scans show central retrocorneal fibrosis in the right eye by vertical scan (top right) and horizontal scan (bottom).
cop-0004-0084-g03.jpg
PMC3656672_01_cop-0004-0084-g03.jpg
F3
The cornea was visualized using anterior optical coherence tomography (OCT) (Visante OCT; Carl Zeiss Meditec Inc., Jena, Germany) with good delineation of the central retrocorneal fibrosis (fig. 3). Retrocorneal scar tissue persisted after surgery (fig. 3), but the bleb height decreased clinically and by UBM (comparing to the fellow eye) after surgery (fig. 5). Best corrected visual acuity did not improve from the preoperative level beyond 6/60 (20/200) partly from the central retrocorneal scar (fig. 3).
Top: by anterior OCT, the midperiphery of the left cornea shows angle closure, a flat anterior chamber (16 mum) and a peripheral corneal thickness of 625 mum. Bottom: anterior OCT of the angle after surgery to the right eye shows resolution of angle closure configuration.
cop-0004-0084-g04.jpg
PMC3656672_01_cop-0004-0084-g04.jpg
F4
Near apposition of the cornea to the iris was clearly demonstrated with angle closure (fig. 4).
Top: UBM of the right eye after surgery shows the restoration of the normal angle anatomy as well as echogenic central retrocorneal fibrosis. UBM of the left unoperated eye (middle panel) shows a large fistula, while the operated right eye (bottom panel) shows a small fistula.
cop-0004-0084-g05.jpg
PMC3656672_01_cop-0004-0084-g05.jpg
F5
Deepening of the angle was somewhat more evident on UBM than anterior segment OCT after lens removal (fig. 5).