Transcranial orbital decompression for progressive compressive optic neuropathy after 3-wall decompression in severe graves' orbitopathy.

PURPOSE: To determine surgical outcomes after transcranial decompression of the superior orbit in patients with progressive compressive optic neuropathy (CON) secondary to Graves' orbitopathy (GO) who had previously been treated with 3-wall decompression. METHODS: Approval from the West Virginia University Institution Review Board was obtained. A retrospective review of 4 patients with GO who received bilateral transcranial decompression of the orbits for progressive compressive optic neuropathy after bilateral maximal extracranial 3-wall decompression was performed. The patients were treated by the Multidisciplinary Orbit and Skull Base Services at West Virginia University and the University of Michigan. RESULTS: Bilateral transcranial decompression of the orbit for GO was performed on 8 orbits in 4 patients. All 8 orbits had radiographic evidence of compression of the orbital apex, and all patients had been treated with steroids, orbital radiation, and bilateral 3-wall decompression. Preoperative vision ranged from 20/25 to 20/100, which improved to 20/25 or better in all eyes. The visual field mean deviation improved from a mean of -13.05 to -1.67 dB. Hertel measurements improved from a mean of 19.25 to 15.25 mm. Extraocular motility was essentially unchanged. Two patients were noted to have asymptomatic ocular pulsations. There were no other complications, and all patients remained stable during a follow-up period of 5 years (range 2-8 years). CONCLUSIONS: Transcranial decompression is an effective and safe method of salvaging vision when standard treatments fail. This is only the second report of transcranial decompression for refractory compressive optic neuropathy after decompression from a standard approach.

Health-related quality of life after surgical removal of an eye.

PURPOSE: This study compared the general health-related quality of life (HRQOL) and the vision-specific HRQOL in patients following the surgical removal of 1 eye who had good vision in the remaining eye to a group of binocular patients with good vision in OU. METHODS: The Medical Outcomes Study Short Form 12 and the National Eye Institute Visual Function Questionnaire HRQOL surveys were administered to 29 patients who had surgical removal of an eye who attended an ocular prosthetics clinic and to 25 binocular persons who accompanied a patient. All subjects in each group had best-corrected visual acuity of 20/40 or better. Overall statistical significance was tested using Cramer's V followed by individual t tests for independent groups for each of the scales on the 2 questionnaires to determine whether the means between the 2 groups differed statistically. RESULTS: The patient group had a mean age of 50.98 years (range, 19-76 years). The control group had a mean age of 49.46 years (range, 18-76 years). The mean time after loss of vision was 28.03 years (range, 1-71 years), and the mean time from surgical removal of the eye was 23.6 years (range, 0.5-59.5 years). There was an overall significant difference between the 2 groups on the 15 derived subscales of the 2 forms (Cramer's V, p = 0.0025). Three general HRQOL subscales (Short Form 12-mental component summary, Short Form 12 physical component summary, and National Eye Institute Visual Function Questionnaire-General Health) showed no differences between the 2 groups (p = 0.48, p = 0.81, and p = 0.78, respectively). Three of the 12 vision-specific National Eye Institute Visual Function Questionnaire subscales demonstrated statistically significant differences between the patient and control groups: peripheral vision (p = 0.0006), role difficulties (p = 0.015), and the composite score (p = 0.014). In addition, 2 monocular patients had given up driving compared with no binocular subjects (p = 0.056). CONCLUSIONS: This population of monocular patients had general physical and mental HRQOL equivalent to the normal binocular group despite the surgical removal of 1 eye. However, the reduced vision-specific HRQOL of monocular patients on the National Eye Institute Visual Function Questionnaire indicates that there are substantial residual visual deficits even after prolonged monocular status.

Comparison of orbital fracture repair performed within 14 days versus 15 to 29 days after trauma.

PURPOSE: To examine whether orbital floor and/or medial wall fracture repair delayed for 15 to 29 days is as effective as early surgery. METHODS: A retrospective review is reported comparing outcomes of early fracture repairs (performed 1-14 days after trauma) to delayed fracture repairs (performed 15-29 days after trauma). Ocular motility, diplopia, and time to resolution of diplopia postoperatively are the main endpoints. RESULTS: Fifty-eight patients were included in the study: 36 underwent early fracture repair (average 9 days after trauma) and 22 underwent delayed fracture repair (average 19 days after trauma). Ocular motility was equivalent in both groups, both before and after surgery. Patient reports of diplopia and frequency of strabisumus surgery were also equivalent in both groups. The time to resolution or stability of diplopia postoperatively is independent of the time to surgery within the first 29 days after trauma. CONCLUSIONS: Although 14 days after trauma is commonly cited as a timeline target for orbital blowout repair, these data show that effective fracture repair can be performed up to 29 days after trauma. Patients with improving diplopia and at low risk for enophthalmos can therefore be observed for 3 to 4 weeks prior to undergoing surgery. This may help prevent unnecessary surgery in some cases. Fourteen days need not be considered a deadline for orbital floor and/or medial wall fracture repair.

Congenital horizontal tarsal kink: a single-center experience with 6 cases.

PURPOSE: To describe the clinical features, surgical management, and outcome of congenital horizontal tarsal kink. DESIGN: Single-center retrospective interventional case series. PARTICIPANTS: Six patients with congenital horizontal tarsal kink. METHODS: Medical records of all patients with congenital horizontal tarsal kink of the upper eyelid managed at a tertiary care referral center were retrospectively reviewed. The referral diagnosis, presenting clinical features, associated ocular abnormalities, and outcome of entropion surgery were analyzed. Surgical treatment consisted of transconjunctival horizontal tarsotomy with marginal rotation. MAIN OUTCOME MEASURES: Evaluation of clinical presenting features and surgical correction of entropion. RESULTS: Six patients were diagnosed with congenital horizontal tarsal kink. Five of 6 (83%) patients were males. The referral diagnosis was congenital entropion in 2 (33%) patients. Corneal opacity or infiltrate was the commonest associated presenting symptom, seen in 5 of 6 (83%) patients. Absence of upper eyelid crease as compared with contralateral eye was noted in all 6 (100%) patients. The tarsal kink was severe with an acute angle (defined as inability to visualize the inverted upper eyelid margin) in 4 (67%) patients that presented within 8 weeks of birth. The tarsal kink was mild with an obtuse angle (easy visualization of upper eyelid margin) in 2 (33%) patients that presented late, at 3 and 5 years of age. Transconjunctival horizontal tarsotomy with marginal rotation resulted in permanent correction of entropion (mean follow-up, 43.3 weeks; range, 16-89 weeks) in all patients. Visual acuity remained subnormal in 5 of 6 (83%) patients. CONCLUSION: The diagnosis of congenital horizontal tarsal kink was suspected only in one third of patients at referral. Associated corneal scar or infiltrate was seen in 83% of cases, and absent eyelid crease in all cases. Congenital horizontal tarsal kink was severe in patients who presented early (within 8 weeks of birth), and mild in patients who presented late. An absent upper eyelid crease, corneal opacity at birth, and nonvisibility of upper eyelid margin are important diagnostic clues to identify this rare disorder. Transconjunctival horizontal tarsotomy with marginal rotation corrected the entropion in all patients. Visual acuity remained subnormal in 83% of patients owing to corneal opacity.

Ultrasonic bone removal with the Sonopet Omni: a new instrument for orbital and lacrimal surgery.

We used a new instrument that ultrasonically removes bone (Sonopet Omni, model UST-2001 Ultrasonic Surgical Aspirator) in 13 orbital decompressions and 6 dacryocystorhinostomies. We noted no surgical complications of ocular or soft tissue damage, infection, inflammation, or visual loss. Visualization, manipulation, ease of use, and speed were far superior with the Sonopet ultrasonic bone curette compared with drills or rongeurs. The Sonopet Omni is an outstanding innovation in technology for bone removal in surgery.

Modified tarsal resection operation for congenital ptosis with fair levator function.

PURPOSE: There are many options for surgical repair of congenital ptosis with fair levator function. The authors review their 10-year experience with an en bloc resection of tarsus, Müller muscle, and conjunctiva in conjunction with graded levator aponeurosis advancement (a variation of the tarsectomy operation). METHODS: This is a retrospective case series that reviews all cases of ptosis repair performed at West Virginia University from 1994 to 2004 using the "modified tarsal resection method." Thirty patients with congenital ptosis and fair levator function were identified. Follow-up ranged from 6 weeks to 8 years. Charts were reviewed for type of ptosis, pre- and postoperative upper eyelid margin to reflex distance, degree of levator function, amount of operative tarsus and Müller muscle resection, postoperative eyelid symmetry, and postoperative complications. RESULTS: In patients with congenital ptosis and fair levator function, the average preoperative upper eyelid margin to reflex distance was 0.0 mm and the average postoperative upper eyelid margin to reflex distance was 2.8 mm. Twenty-five of 30 (83%) patients were deemed to have a "good" surgical outcome. The amount of tarsus-Müller muscle resection reliably predicts the amount of eyelid elevation. The only complications to date have been transient lagophthalmos with exposure keratitis. CONCLUSIONS: In the authors' hands, an en bloc resection of tarsus, Müller muscle, and conjunctiva combined with levator aponeurosis advancement reliably produces excellent results in the treatment of fair levator function congenital ptosis, superior to their previous experience with isolated maximal levator aponeurosis advancement.

Infiltrating orbital astrocytic proliferation associated with congenitally malformed eyes.

This report describes the clinical and pathologic findings in two adults with orbital infiltration by astrocytic cells associated with congenitally malformed eyes. Both cases had enlarging orbital masses and underwent complete resection. Histopathology revealed proliferation of astrocytes (fibrillary acidic protein-positive) that invaded the orbital tissues. To the best of our knowledge, there are no similar cases in the literature.

Bilateral optic nerve sheath enhancement from giant cell arteritis.

An 83-year-old man presented with acute bilateral visual loss to no light perception (NLP) OD and 20/50 OS. His fundus examination showed moderate bilateral pallid disc edema. A sedimentation rate was 60 mm/h. Magnetic resonance imaging of the brain and orbits with gadolinium revealed marked bilateral enhancement of the optic nerve sheaths and adjacent orbital fat. He underwent biopsies of the optic sheath OD and bilateral temporal arteries. Histopathology of the optic nerve sheath area revealed fibroadipose tissue containing numerous arteries with intimal thickening, and mild mural inflammation consisting predominantly of lymphocyte with occasional giant cells. The bilateral temporal artery biopsies revealed focal disruption of the elastic lamina with rare giant cells. His vision had since stabilized on IV methypdnisolone therapy. The biopsies of the nerve sheath suggest that the radiologic finding of optic nerve sheath enhancement in giant cell arteritis is caused by tbe same pathophysiology, and therefore may be a manifestation of this systemic disease.

Lower eyelid reverse ptosis repair.

PURPOSE: To report the results of the surgical repair of lower eyelid reverse ptosis. METHODS: Retrospective case series. Eight patients ranging in age from 31 to 77 years underwent surgical repair of lower eyelid reverse ptosis. The pupillary axis of the affected eye(s) in each patient was obscured in downgaze, interfering with reading. The lower eyelid reverse ptosis resulted from involutional changes in 3 patients, previous orbital decompression in 3 patients, multiple prior retinal and extraocular muscle operations in 1 patient, and previous orbital floor fracture and repair in 1 patient. Transcutaneous advancement of the lower eyelid retractors was performed in 12 eyelids of the 8 patients. RESULTS: The mean preoperative vertical eyelid fissure was 6.2 mm (median, 6 mm; range, 3-9 mm), increasing after surgery to a mean of 7.7 mm (median, 8 mm; range, 5-11 mm). The mean preoperative distance between the central light reflex and the lower eyelid margin was 1.7 mm (median, 1.25 mm; range, 1-4 mm); this distance increased to a mean of 3.3 mm (median, 3.25 mm; range, 2.5-4.5 mm) after surgery. Symptoms improved in all patients, and there were no perioperative complications. Follow-up intervals ranged from 2 months to 24 months (mean, 9 months; median, 13 months). CONCLUSIONS: Analogous to upper eyelid ptosis repair by advancement of the levator aponeurosis, lower eyelid reverse ptosis may be corrected effectively and safely by advancing the lower eyelid retractors.

Mutations in the human RAX homeobox gene in a patient with anophthalmia and sclerocornea.

Anophthalmia and microphthalmia are among the most common ocular birth defects and a significant cause of congenital blindness. The etiology of anophthalmia and microphthalmia is diverse, with multiple genetic mutations associated with each of these conditions, along with potential environmental causes. Based on findings that mutations in the Rx/Rax homeobox genes in mice and fish lead to defects in retinal development and result in animal models of anophthalmia, we screened 75 individuals with anophthalmia and/or microphthalmia for mutations in the human RAX gene. We identified a single proband from this population who is a compound heterozygote for mutations in the RAX gene. This individual carries a truncated allele (Q147X) and a missense mutation (R192Q), both within the DNA-binding homeodomain of the RAX protein, and we have characterized the biochemical properties of these mutations in vitro. Parents and grandparents of the proband were found to be carriers without visible ocular defects, consistent with an autosomal recessive inheritance pattern. This is the first report of genetic mutations in the human RAX gene.