Surgical Management of Polyostotic Craniofacial Fibrous Dysplasia: Long-Term Outcomes and Predictors for Postoperative Regrowth.

BACKGROUND: The mainstay of treatment for craniofacial fibrous dysplasia is surgical; however, optimal indications and techniques are poorly understood, particularly in polyostotic disease and McCune-Albright syndrome. This study investigated surgical indications and risk factors for recurrence in a large cohort. METHODS: One hundred thirty-three craniofacial fibrous dysplasia subjects in a natural history study were evaluated. Radiographic studies, operative reports, and clinical records were reviewed. RESULTS: Thirty-six subjects underwent 103 craniofacial procedures (mean, 2.8 operations per subject), with 13.5 ± 10.5-year follow-up (range, 0 to 39 years). The most common indication was craniofacial deformity (n = 61 operations), including 36 initial operations (59 percent) and 26 reoperations (41 percent). Mean time to reoperation was 3.4 ± 3.2 years (range, 0.3 to 13.3 years). Regrowth occurred after 42 operations (68 percent), and was more frequent after operations in subjects with McCune-Albright syndrome growth hormone excess [22 of 25 operations (88 percent)] than without growth hormone excess [15 of 36 operations (58 percent); p = 0.02]. Of 11 subjects with growth hormone excess, nine (82 percent) were undiagnosed at the time of their initial operation. Regrowth was more frequent after debulking procedures [31 of 38 (82 percent)] than after more aggressive reconstructions [nine of 20 (45 percent); p = 0.007]. Eleven subjects underwent treatment for aneurysmal bone cysts, with recurrence in one subject. Eleven subjects underwent biopsies and none had complications or regrowth. CONCLUSIONS: Craniofacial fibrous dysplasia regrowth and reoperation are common, particularly after debulking procedures. Outcomes are favorable for aneurysmal bone cysts and biopsies. McCune-Albright syndrome growth hormone excess is a risk factor for regrowth, and may be underdiagnosed in surgical patients. Surgeons should be aware of appropriate screening for endocrinopathies in fibrous dysplasia. These findings highlight the importance of a multidisciplinary approach to craniofacial fibrous dysplasia, and individualized care with long-term follow-up. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Contrast Sensitivity Testing in Functional Ptosis and Dermatochalasis Surgery.

PURPOSE: To assess whether taped versus untaped contrast sensitivity testing reliably predicts improvement following eyelid-lifting surgery in patients with ptosis and/or dermatochalasis. METHODS: This prospective study compared pre- and postoperative near contrast sensitivity in patients presenting to 2 ophthalmic plastic surgery practices with functional dermatochalasis and/or ptosis as determined by Humphrey visual field testing. Contrast sensitivity was measured using Mars near cards, held at 40 cm under standard lighting conditions. Logarithmic contrast sensitivity scoring cards were used for analysis. Marginal reflex distance was also collected. Pairwise comparison was used to assess the preoperative and postoperative contrast sensitivity scores. This study was conducted under the approval of the institutional review board. RESULTS: Seventy-eight eyes of 41 patients with ptosis +/ dermatochalasis underwent eyelid-lifting surgery. The mean preoperative untaped contrast sensitivity was 1.30. The mean preoperative taped and postoperative log contrast sensitivities were 1.52 (11.85% increase) and 1.51 (11.44% increase), respectively. The difference between the 2 groups was not statistically significant (p = 0.864). The marginal reflex distance improved by a mean of 2.0 (p < 0.0005). CONCLUSIONS: Contrast sensitivity improves significantly following blepharoplasty and ptosis surgery. The degree of improvement in contrast sensitivity can accurately be predicted by preoperative taped/untaped testing. Contrast sensitivity testing may provide an additional screening modality for functional dermatochalasis and ptosis. In the future, contrast sensitivity may provide an additional preoperative assessment tool in patients who do not meet traditional visual field screening standards or who are unable to perform classic visual field testing.

Intraocular polyimide intraocular lens haptic breakage long-term postoperatively.

UNLABELLED: We present 2 cases of 3-piece silicone intraoculanbsp;r lenses (IOLs) with broken polyimide haptics. In the first case, the IOL was implanted in the anterior chamber after posterior capsule rupture. Twelve years later, it was explanted and exchanged because of bullous keratopathy. However, on careful manipulation for explantation, the haptics shattered into multiple pieces. In the second case, initial in-the-bag implantation of the IOL was uneventful. Fifteen years later, the IOL dislocated into the anterior chamber as a result of spontaneous in situ haptic breakage. The haptics of both IOLs appeared very brittle during careful manipulation for gross and light microscopy, with further breakage. Scanning electron microscopy of both IOLs revealed no evidence of haptic degradation. Their surfaces appeared smooth and regular with jagged edges where the breaks occurred. Few studies have examined the long-term biocompatibility of polyimide, and more research is needed to determine the cause of this phenomenon. FINANCIAL DISCLOSURE: No author has a financial or proprietary interest in any material or method mentioned.

Optic neuropathy in McCune-Albright syndrome: effects of early diagnosis and treatment of growth hormone excess.

CONTEXT: GH excess is a serious complication of McCune-Albright syndrome (MAS) and has been associated with craniofacial morbidity. OBJECTIVE: The aim of the study was to determine whether early diagnosis and treatment of MAS-associated GH excess prevents optic neuropathy and hearing impairment, the major morbidities associated with GH excess. DESIGN AND SETTING: A retrospective cross-sectional analysis was conducted at a clinical research center. PATIENTS: Twenty-two subjects with MAS-associated GH excess and 21 control MAS subjects without GH excess were included in the study. INTERVENTION: Biochemical testing included random GH, nadir GH after glucose load, nadir GH on frequent sampling, and IGF-I Z-score. Subjects underwent imaging, ophthalmological, audiological, and otolaryngological assessment. Treatment included octreotide, pegvisomant, transphenoidal surgery, and/or radiotherapy as indicated. MAIN OUTCOME MEASURE: Association of optic neuropathy and hearing impairment to age at GH excess diagnosis/treatment was measured. RESULTS: Of 129 MAS subjects, 26 (20%) were diagnosed with GH excess based on elevation of two measures of GH function. Of these, 22 subjects were candidates for pharmacological intervention. Optic neuropathy was significantly correlated with intervention status, with no cases in the early intervention group (diagnosed/treated before age 18) or the control group, and four of seven (57%) in the late intervention group (diagnosed/treated after age 18) (Fisher's exact test; odds ratio, 0.027; P = 0.0058). Early diagnosis/intervention was not associated with reduction in hearing deficits (odds ratio, 1.25; P = 1.00). Mean head circumference SD score was significantly higher in the late (6.08; range, 2.70 to 22.56) than the early intervention (2.67; range, -0.65 to 6.72) or control groups (2.13; range, -2.06 to 7.79) (P = 0.003). CONCLUSIONS: Early diagnosis/treatment of GH excess in MAS is important to prevent optic neuropathy and craniofacial expansion. The relationship between hearing deficits and GH excess remains less clear and requires further study.

Lenticular meridional astigmatism secondary to iris mesectodermal leiomyoma.

A 61-year-old African American man presented with decreased vision of 2 months duration. Examination revealed a significant lenticular astigmatism and sectoral cataract as a result of an amelanotic iris lesion. Slitlamp optical coherence tomography (OCT) revealed angle crowding. An excisional biopsy was performed along with phacoemulsification in the right eye, with intraocular lens implantation for meridional lenticular astigmatism. Histopathology and histoimmunochemistry confirmed a diagnosis of uveal mesectodermal leiomyoma. Lenticular astigmatism may be a subtle sign of an anterior segment tumor. Anterior segment slitlamp OCT is an effective tool in diagnosing as well as monitoring small interval changes in these types of tumors.

Selective venous catheterization for the localization of phosphaturic mesenchymal tumors.

Tumor-induced osteomalacia (TIO) is characterized by renal phosphate wasting, hypophosphatemia, and aberrant vitamin D(3) metabolism and is caused by fibroblast growth factor 23 (FGF-23)-producing mesenchymal tumors, which are often difficult to locate. We investigated the utility of selective venous sampling in tumor localization. The primary endpoint was identification of the FGF-23 concentration ratio between the venous drainage of the tumor bed and the general circulation that was diagnostic of the location of an FGF-23-secreting tumor. Fourteen subjects underwent 15 sampling procedures after functional and anatomic imaging studies. Subjects fit into three imaging categories: no suspicious site, multiple sites, and single site (positive controls). FGF-23 levels were measured by ELISA. Suspicious tumors were resected for diagnosis, confirmation, and cure. In subjects with a positive venous sampling study and subsequent cure, a minimum ratio of 1.6 was diagnostic. In 7 of 14 subjects there was suggestive imaging, a diagnostic ratio, and an associated TIO tumor (true positive). Four of these required complicated resection procedures. In 4 of 14 subjects with no suspicious site on imaging studies, an FGF-23 diagnostic ratio was not detected (true negative). Biopsy or resection of a single lesion in 2 of 14 subjects with a diagnostic ratio failed to identify a TIO tumor (false positive). A diagnostic FGF-23 ratio was absent in 1 of 14 subjects whose tumor was a single highly suspicious lesion on imaging studies (false negative). These data yield a sensitivity of 0.87 [95% confidence interval (CI) 0.47-0.99] and a specificity of 0.71 (95% CI 0.29-0.96). Selective venous sampling for FGF-23 was particularly useful in subjects with multiple suspicious sites or an anatomically challenging planned resection but not in the absence of a suspicious lesion on imaging studies.

Accelerated 20-year sunlight exposure simulation of a photochromic foldable intraocular lens in a rabbit model.

PURPOSE: To assess the long-term biocompatibility and photochromic stability of a new photochromic hydrophobic acrylic intraocular lens (IOL) under extended ultraviolet (UV) light exposure. SETTING: John A. Moran Eye Center, University of Utah, Salt Lake City, Utah, USA. DESIGN: Experimental study. METHODS: A Matrix Aurium photochromic IOL was implanted in right eyes and a Matrix Acrylic IOL without photochromic properties (n = 6) or a single-piece AcrySof Natural SN60AT IOL (n = 5) in left eyes of 11 New Zealand rabbits. The rabbits were exposed to a UV light source of 5 mW/cm(2) for 3 hours during every 8-hour period, equivalent to 9 hours a day, and followed for up to 12 months. The photochromic changes were evaluated during slitlamp examination by shining a penlight UV source in the right eye. After the rabbits were humanely killed and the eyes enucleated, study and control IOLs were explanted and evaluated in vitro on UV exposure and studied histopathologically. RESULTS: The photochromic IOL was as biocompatible as the control IOLs after 12 months under conditions simulating at least 20 years of UV exposure. In vitro evaluation confirmed the retained optical properties, with photochromic changes observed within 7 seconds of UV exposure. The rabbit eyes had clinical and histopathological changes expected in this model with a 12-month follow-up. CONCLUSIONS: The new photochromic IOL turned yellow only on exposure to UV light. The photochromic changes were reversible, reproducible, and stable over time. The IOL was biocompatible with up to 12 months of accelerated UV exposure simulation.

Toxic anterior segment syndrome: common causes.

PURPOSE: To identify the most common risk factors associated with toxic anterior segment syndrome (TASS). SETTING: Ophthalmic surgical centers in the United States, Argentina, Brazil, Italy, Mexico, Spain, and Romania. METHODS: A TASS questionnaire on instrument cleaning and reprocessing and extraocular and intraocular products used during cataract surgery was placed on the American Society of Cataract and Refractive Surgery web site. A retrospective analysis of questionnaires submitted by surgical centers reporting cases of TASS was performed between June 1, 2007, and May 31, 2009, to identify commonly held practices that could cause TASS. Members of the TASS Task Force made site visits between October 1, 2005, and May 31, 2009, and the findings were evaluated. RESULTS: Data from 77 questionnaires and 54 site visits were analyzed. The reporting centers performed 50 114 cataract surgeries and reported 909 cases of TASS. From January 1, 2006, to date, the 54 centers reported 367 cases in 143 919 procedures; 61% occurred in early 2006. Common practices associated with TASS included inadequate flushing of phaco and irrigation/aspiration handpieces, use of enzymatic cleansers, detergents at the wrong concentration, ultrasonic bath, antibiotic agents in balanced salt solution, preserved epinephrine, inappropriate agents for skin prep, and powdered gloves. Reuse of single-use products and poor instrument maintenance and processing were other risk factors. CONCLUSIONS: The survey identified commonly held practices associated with TASS. Understanding these findings and the safe alternatives will allow surgical center personnel to change their practices as needed to prevent TASS. FINANCIAL DISCLOSURE: No author has a financial or proprietary interest in any material or method mentioned.

Do calcium channel blockers rescue dying photoreceptors in the Pde6b ( rd1 ) mouse?

Retinitis pigmentosa (RP) is a genetically heterogeneous set of blinding diseases that affects more than a million people worldwide. In humans, ~5-8% of recessive and dominant RP cases are caused by nonsense mutations in the Pde6b gene coding for the ss-subunit of the rod photoreceptor cGMP phosphodiesterase 6 (PDE6-ss). The study of the disease has been greatly aided by the Pde6b ( rd1 ) (rd1) mouse model of RP carrying a null PDE6ss allele. Degenerating rd1 rods were found to experience a pathological increase in intracellular calcium concentration ('Ca overload') when they enter the apoptotic process at postnatal day 10. A 1999 study suggested that the Ca(2+) channel antagonist D-cis diltiazem delays the kinetics of rd1 rod degeneration, conferring partial rescue of scotopic vision. Subsequent reports were mixed: whereas several studies failed to replicate the original results, others appeared to confirm the neuroprotective effects of Ca(2+) channel antagonists such as diltiazem, nilvadipine and verapamil. We discuss the discrepancies between the results of different groups and suggest plausible causes for the discordant results. We also discuss potential involvement of recently identified Ca(2+)-dependent mechanisms that include protective calcium ATPase mechanisms, ryanodine and IP3 calcium stores, and store operated channels in Pde6b ( rd1 ) neurodegeneration.

Scanning electron microscopy visualization of methicillin-resistant Staphylococcus aureus after contact with gatifloxacin with and without preservative.

PURPOSE: To test a visual model by looking at the differences in effect of Zymar((R)) (gatifloxacin plus benzalkonium chloride [BAK]) when compared to gatifloxacin and a normal saline (NS) control upon a methicillin and gatifloxacin-resistant Staphylococcus aureus (MRSA) species. METHODS: An ocular isolate of gatifloxacin-resistant (minimal inhibitory concentration >2 to 4 microg/mL) MRSA was grown to confluency. Chambered slides were prepared with bacterial culture smears, and then incubated with either gatifloxacin at the concentrations of 1 and 10 microg/mL, Zymar containing equivalent concentrations of gatifloxacin, or NS. Bacterial cultures were fixed after 10, 30, and 60 min. Fixed slides were coated in gold sputter for examination. Bacteria were visually evaluated with scanning electron microscopy (SEM) at 50,000x. Blinded review of SEM images compared structural changes and mitotic activity across samples. RESULTS: MRSA exposed to 10 microg Zymar for 60 min showed significantly greater pleomorphism and cell wall surface changes when compared to gatifloxacin (P < 0.0001) and NS (P = 0.001), and significantly less mitotic activity than NS (P = 0.002). CONCLUSION: Using SEM, the topical formulation of gatifloxacin 0.3% (Zymar), which contains BAK, had greater antibacterial activity than did gatifloxacin alone in gatifloxacin and methicillin-resistant S. aureus, thereby illustrating potential advantages of the preservative in the commercial formulation. We further show that these effects can be visualized and quantified.