Phakic anterior chamber intraocular lens (Verisyse) implantation in children for treatment of severe ansiometropia myopia and amblyopia: Six-month pilot clincial trial and review of literature.

PURPOSE: The current study aims to evaluate both safety and efficacy of Verisyse() (AMO, Irvine, CA) phakic anterior chamber intraocular lens (IOL) in the reduction of clinically significant (>-8 D) myopic anisometropia in children who are noncompliant to traditional medical treatment including spectacle correction or contact lenses. DESIGN: Retrospective interventional case series. METHODS: Six anisometropic myopic pediatric patients in one practice were identified through chart-review. None of the patients were compliant with specatacle correction or contact lens wear and as a result had dense amblyopia of less than 20/400 by Snellen or Allen visual acuity (mean <20/400). All patients underwent Verisyse() phakic IOL implantation in the more myopic eye by one surgeon (AP). Pre- and post-operative visual acuity, anterior/posterior segment examination, stereoacuity, axial biometry measurements, cycloplegic refraction, and endothelial cell counts were performed in all patients whenever feasible. RESULTS: The age of patients ranged from 5-11 years. The mean post-operative follow-up time was six months from the time of IOL insertion. Improvement in visual acuity >6 lines was achieved in four patients (mean visual acuity of 20/70 at six months). Improvement in stereoacuity was noted in all six patients (from total mean zero seconds-arc to six-months post-operative mean of 500 seconds-arc by randot stereoacuity testing). Improvement of >2 lines of visual acuity lines was achieved in the other two patients. No patient lost any lines of visual acuity. Enhanced physical activity, coordination, and improved social interaction were noted in patients and were reported by the parents. No intra/post-operative complications were noted. DISCUSSION: Irreversible or intractable amblyopia secondary to severe anisometropic myopia is a serious medical concern in the pediatric population. Failure of compliance with contact lens therapy consistently leads to visual loss. Anterior chamber phakic IOLs may provide a safe alternative in treatment of noncompliant anisometropic myopic patients who do not accept spectacle wear or contact lens therapy. CONCLUSION: To reduce or eliminate highly significant anisometropic myopia in children who are noncompliant with traditional medical treatment, phakic anterior chamber IOL implantation may be considered as an alternative modality of treatment.

Pellucid marginal degeneration and bilateral corneal perforation: case report and review of the literature.

PURPOSE: To review corneal perforation cases in pellucid marginal degeneration and report a case of bilateral spontaneous perforation at presentation in rigid gas permeable contact lens wear and pellucid marginal degeneration. METHODS: Case report and literature review. RESULTS: The presentation, clinical course, surgical intervention, pathologic analysis, and postoperative outcome of a case of bilateral spontaneous corneal perforation in pellucid marginal degeneration are detailed. All spontaneous corneal perforation cases associated with pellucid marginal degeneration are reviewed. CONCLUSIONS: Pellucid marginal degeneration can lead to spontaneous corneal perforation resulting in the need for urgent therapeutic intervention. A potential for severe ocular morbidity should be considered in all patients with this disorder despite a seemingly stable disease and appropriate gas permeable contact lens wear. Unilateral perforation cases should be watched closely for development of complications in the fellow eye.

Traumatic and spontaneous scleral rupture and uveal prolapse in osteogenesis imperfecta.

Three cases of severe globe injuries due to scleral fragility in osteogenesis imperfecta patients between the ages of 4 and 15 years are reported. Patient 1 had complete loss of vision. Patients 2 and 3 suffered non-sight-threatening scleral perforation. All 3 patients had no previous knowledge of recommendation for eyewear protection.

Ahmed glaucoma valve implants in the pediatric population: the use of magnetic resonance imaging findings for surgical approach to reoperation.

PURPOSE: To describe and to evaluate a novel application of magnetic resonance imaging (MRI) in determining the Ahmed glaucoma valve (AGV) function after implantation. METHODS: Retrospective interventional case series of all surgical glaucoma patients in one practice were identified and orbital MRI series were performed. Orbital MRI findings were evaluated in all children who had undergone Ahmed glaucoma valve insertion. RESULTS: A total of six patients were identified, ranging in age from 1 to 14 years. Mean follow-up time ranged from 1 to 3 years from the time of first tube insertion. Gadolinium enhancement MRIs showed an enhanced large cystic cavity over the plate in five patients, indicating partial to complete patency of the outflow of the tube. One MRI failed to show an enlarged enhanced bleb over the plate region, indicating potential obstruction of outflow. Surgical irrigation of the tube displayed obstruction. Ahmed tube exchange was performed. Enhanced cystic bleb showed compression of the extraocular muscles in four patients. These patients exhibited mild to moderate restrictive strabismus. No lenticular opacification was noted in the four phakic patients. CONCLUSIONS: Early and late AGV failure and subsequent intraocular pressure rise are of great concern after pediatric glaucoma shunt surgery. Orbital MRI series should be considered in the cases of possible AGV drainage failure. In cases of reoperation, orbital MRI findings could be of great clinical value to evaluate for functional patency as well as for the EOM surgical intervention approach. To avoid unnecessary surgical exploration/explantation, orbital MRIs with gadolinium-dye enhancement should be considered in evaluating postshunt glaucoma surgical patients.

A report of canine tooth syndrome.

The authors describe the case of a 5-year-old girl traumatized from a dog bite to the superior aspect of the orbit in the right eye. The dog's canine tooth penetrated deep into the posterior orbit and severed the attachment of the superior oblique muscle from the globe posterior to the trochlea. The management and clinical course of the patient are described and photographs documenting the initial ocular damage and postoperative course are provided. In addition, the entity known as 'canine tooth syndrome' is reviewed.

Ocular findings in Jacobsen syndrome.

PURPOSE: To discuss the ophthalmic findings and their clinical significance in 10 new cases of Jacobsen syndrome (mental retardation, craniofacial anomalies, congenital heart defects, and blood dyscrasias) and to review the ophthalmic findings in all previously reported cases in the literature. METHODS: Ten new cases of Jacobsen syndrome were collected and studied prospectively for detection of abnormal ophthalmologic examination findings. A total of 63 previously reported cases were identified from Medline and analyzed for ophthalmologic abnormalities. RESULTS: The most common ophthalmologic findings in the new cases of Jacobsen syndrome included strabismus (90.0%), refractive error (90.0%), and ptosis (70.0%). Facial dysmorphism was also common and included hypertelorism, epicanthal folds, and down-slanting palpebral fissures. Uncommon ophthalmic findings included 5 patients with retinal vascular tortuosity, 1 with glaucoma, and 3 with amblyopia. In 63 cases reviewed, 36 reported ophthalmologic abnormalities. The most common findings included facial anomalies and ptosis. Only 5 of the 63 patients had evidence of strabismus, and none were reported to have retinal vascular tortuosity. CONCLUSIONS: To prevent unnecessary vision loss in children with Jacobsen syndrome, proper screening for amblyogenic factors is imperative. We recommend a baseline complete ophthalmologic examination with subsequent follow-up examinations depending on the particular findings noted during the initial screening visit.