Long-Term Outcome After Bilateral DMEK for Fuchs Endothelial Corneal Dystrophy.

PURPOSE: The aim of this study was to assess the long-term clinical outcome, complications, and graft survival of bilateral Descemet membrane endothelial keratoplasty (DMEK) in patients with Fuchs endothelial corneal dystrophy. METHODS: This was a retrospective cohort study of 181 patients (362 eyes) with sequential bilateral DMEK for Fuchs endothelial corneal dystrophy. Clinical outcomes were assessed up to 5 years postoperatively. Outcome measures were best-corrected visual acuity, pachymetry, endothelial cell density, graft survival, and complication rates. RESULTS: Contralateral DMEK was performed on average 15 ± 11 months (range: 2-60 months) after the first eye. From 1 until 5 years after DMEK, best-corrected visual acuity, pachymetry, endothelial cell density, and graft survival did not differ between the first and second eyes (all P > 0.05). Graft detachment occurred in 67 eyes (19% [18% first eyes, 19% second eyes], 6% bilateral), graft rejection in 9 eyes (3% [3% first eyes, 2% second eyes], 1% bilateral), glaucoma in 25 eyes (7% [8% first eyes, 6% second eyes], 2% bilateral), and graft failure in 22 eyes (6% [4% first eye, 8% second eye], 2% bilateral). All differences were not significant (all P > 0.05). Five-year graft survival rates were comparable for first and second eyes (0.95 and 0.92, respectively; P = 0.15). CONCLUSIONS: Clinical outcomes after bilateral DMEK are similar in both eyes and sustainable in the longer term. Within the first 5 years, the same complication may rarely occur in the contralateral eye.

What Are the Characteristics of the Upper Airway in Patients With Craniofacial Microsomia?

PURPOSE: Obstructive sleep apnea (OSA) is a common problem in patients with craniofacial microsomia (CFM); however, the exact pathophysiology in patients with CFM remains unclear. The first aim of this study was to evaluate upper airway volume and morphology in patients with CFM. The second aim was to identify risk factors for the presence of OSA. MATERIALS AND METHODS: A cross-sectional study was set up and 3 study groups were identified: 1) CFM with OSA, 2) CFM without OSA, and 3) control. Computed tomographic (CT) scans of the head and neck were included and used to create 3-dimensional models. The age-matched control group consisted of patients evaluated for traumatic head injury or epilepsy. Volumetric and morphologic parameters were measured. The results of patients with CFM were compared among the 3 study groups. Descriptive statistics were computed using the Pearson χ2 test for categorical variables and nonparametric tests for continuous variables. A multiple variable regression model was used to identify risk factors for OSA. RESULTS: In total, 79 patients with CFM were included, of which 25 patients were diagnosed with OSA. A total of 145 CT scans could be analyzed. In addition, a control population of 88 patients was identified. Oropharynx volume, mean cross-sectional area (CSA), minimal CSA, and minimal retropalatal area were found to be markedly smaller in patients with CFM compared with the control population. In contrast, in patients with CFM and OSA, minimal retroglossal area, sphericity, and uniformity markedly differed from those in patients without OSA. Sphericity was identified as the main predicting variable of OSA in patients with CFM. CONCLUSIONS: The upper airway of patients with CFM is markedly smaller and puts them at risk for developing OSA. Patients with CFM diagnosed with OSA have a markedly smaller CSA behind the base of the tongue and a difference in sphericity.

Surgical Correction of Craniofacial Microsomia: Evaluation of Interventions in 565 Patients at Three Major Craniofacial Units.

BACKGROUND: Craniofacial microsomia is characterized by an asymmetric hypoplasia of derivatives of the first and second pharyngeal arch, leading to a variety of phenotypic presentations. Studies on surgical correction of patients with craniofacial microsomia have small cohorts, leaving controversial opinions on the optimal treatment modality, the indication for surgery, and the optimal timing of surgery. The purpose of this study was to evaluate the types of, timing of, and total number of surgical corrections performed and the number of surgical procedures in correlation to the severity of the phenotype. METHODS: A retrospective chart study was conducted including patients diagnosed with craniofacial microsomia from three large craniofacial units. Demographic, radiographic, and clinical information was obtained, including type and number of surgical procedures and age at the time of surgery. RESULTS: A total of 565 patients were included. In total, 443 (78.4 percent) of all patients underwent some form of surgery during their life, varying from skin tag removal to major craniofacial operations. The number of surgical interventions was higher with increasing severity of phenotype, bilateral presentation, and a younger age at the first intervention. CONCLUSIONS: Multiple surgical corrections are frequently seen in patients with a more severe or bilateral presentation. Furthermore, those who are treated earlier in life for correction of asymmetry of the mandible will undergo significantly more surgical procedures to correct the asymmetry later on, independent of the Pruzansky-Kaban type mandible. A prospective international multicenter study is designed with a uniform registration and outcome measurement tool to identify the optimal treatment strategy.

In Vivo Endothelial Cell Density Decline in the Early Postoperative Phase After Descemet Membrane Endothelial Keratoplasty.

PURPOSE: To evaluate endothelial cell density (ECD) in the first 6 months after Descemet membrane endothelial keratoplasty (DMEK) by eliminating method error as a confounding variable. METHODS: From 24 DMEK eyes operated for Fuchs endothelial corneal dystrophy, from which specular microscopy images could be taken at 1 day and 6 months postoperatively, ECD values were compared between these 2 time points. RESULTS: Using the 1-day ECD measurement as baseline, mean ECD decreased from 1913 (±326) cells/mm to 1524 (±393) cells/mm at 6 months, a decline of -18 (±19)%. With the 1-week ECD as baseline [1658 (±395) cells/mm], the decline at 6 months was -6 (±19)% and when using preoperative ECD as baseline [2521 (±122) cells/mm], the decline was -39 (±16)% at 6 months. CONCLUSIONS: After DMEK, ECD shows an in vivo decline of 18% from 1 day to 6 months postoperatively, with a sharp 13% drop in the first week, and a slower decrease thereafter. The remaining difference of 20% from preoperative ECD values may be attributed to a measurement error in the eye bank with an overestimation of the graft's viable endothelial cell population and/or intraoperative trauma to the graft.

Quantitative Assessment of Aqueous Flare After Descemet Membrane Endothelial Keratoplasty for Fuchs Endothelial Dystrophy.

PURPOSE: To assess aqueous flare as a measure of subclinical inflammation after Descemet membrane endothelial keratoplasty (DMEK) for Fuchs endothelial dystrophy. METHODS: In this prospective cross-sectional and longitudinal case series at a tertiary referral center, 173 DMEK eyes of 169 patients and 19 age-matched healthy control eyes were included. Aqueous flare [photon count per millisecond (ph/ms)] was assessed by laser flare photometry at 1 day, 1 week, and 1 month after DMEK in group I (evaluation of postsurgical blood-aqueous barrier recovery; n = 25) and on average 28 (±19) months (range, 3-86 months) after DMEK in group II (evaluation of long-term inflammation; n = 148). RESULTS: In group I, flare levels decreased from 1 day to 1 week [25.1 (±9.1) ph/ms vs. 13.4 (±4.8) ph/ms; P = 0.003] and remained stable up to 1 month after DMEK [12.1 (±3.2) ph/ms; P = 0.387]. However, average flare at 1 month was higher than that in healthy controls (P < 0.001). The long-term flare value after DMEK (group II) was 9.6 (±4.2) ph/ms and was higher in eyes associated with allograft rejection (n = 6) versus those without rejection [16.7 (±7.8) ph/ms vs. 9.3 (±3.8) ph/ms, respectively, P < 0.001]. All eyes associated with rejection had flare values above 10 ph/ms. CONCLUSIONS: Aqueous flare after DMEK quickly decreased within the first postoperative month, indicating fast recovery of the blood-aqueous barrier. Long-term flare levels were higher in eyes associated with rejection, suggesting persistent subclinical inflammation. A flare level above 10 ph/ms may be used as a threshold for identifying eyes associated with or at risk of allograft rejection after DMEK.

Clinical Outcome of Rebubbling for Graft Detachment After Descemet Membrane Endothelial Keratoplasty.

PURPOSE: To assess the clinical outcome after successful rebubbling procedures for visually significant graft detachment after Descemet membrane endothelial keratoplasty (DMEK). METHODS: From a total of 760 consecutive DMEK surgeries, 41 eyes required rebubbling. Of those, 33 eyes of 31 patients were successful and were included in our retrospective outcome analysis study. Main outcome measures were compared with those of matched controls with uneventful primary DMEK (attached DMEK grafts without rebubbling). Rebubbling was performed on average 25 (±20) days (range 7-91 days) after DMEK. All eyes were evaluated for best-corrected visual acuity, endothelial cell density (ECD), pachymetry, and complications up to 6 months after rebubbling. RESULTS: At 6 months after DMEK, best-corrected visual acuity in rebubbled eyes did not differ from that in control eyes (P = 0.514). The mean ECD decrease was higher in rebubbled than in control eyes (54% vs. 35%, respectively, P = 0.001). Pachymetry did not differ between both groups (P = 0.153). After rebubbling, one buphthalmic eye showed temporary intraocular pressure elevation and 5 eyes had minor graft edge detachment that did not require further treatment. CONCLUSIONS: Rebubbling for DMEK graft detachment may result in similar visual outcomes as in uncomplicated DMEK, when performed within the first 6 to 8 postoperative weeks. However, rebubbled eyes may have lower ECD, which may be attributed to additional air bubble trauma and/or selection bias through more extensive manipulation during initial DMEK or higher risk of graft detachment in more complicated eyes.