Surgical management of ptosis in chronic progressive external ophthalmoplegia

Eshaghi, M; Arabi, Amir; Eshaghi, Sarvin

doi:10.1177/1120672120952344

Cited by 10 publications

(5 citation statements)

References 12 publications

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“…Previous results on ptosis repair in complicated slings mainly include short case series, including BPES, 8 CFEOM 9 , CPEO, 10,11 and others 12 , with each entity enclosing its unique challenges (Fig. 4).…”

Section: Discussionmentioning

confidence: 99%

Outcome of Silicone Sling Frontalis Suspension in Isolated Uncomplicated Congenital Ptosis vs. Complicated Ptosis

Prat

Zhao

Ramakrishnan

et al. 2023

Preprint

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Objectives To compare the outcome of silicone sling frontalis suspension (FS) surgery in children with isolated uncomplicated congenital ptosis (IUCP) vs. children with complicated ptosis. Methods A retrospective medical chart review of all pediatric patients who underwent silicone sling FS surgery between 2009–2020 at a single center were included. Patients were divided according to ptosis type: IUCP vs. complicated ptosis. Pre-and post-operative margin-to-reflex distance (MRD1) measurements were determined from clinical photographs. Main outcome measures were differences in improvement in eyelid height, reoperation rate, and timing between the groups. Results Two-hundred and eight children were included: 139 IUCP and 69 complicated cases, 83 females (40%), mean (± SD) age at intervention was 1.9 ± 2.9 years. Complicated cases included: blepharophimosis epicanthus inversus syndrome (n = 35), Marcus Gunn jaw winking (n = 12), oculomotor palsy (n = 8), congenital fibrosis of extraocular muscles (n = 3), chronic progressive external ophthalmoplegia (n = 3), and others. Mean MRD1 improved by an average of 1.6 mm in both groups. Repeat ptosis repair was performed in 50/171 (29%) patients without a history of failed ptosis procedures, and this rate was similar between IUCP and complicated cases. Children under 3 years of age had higher rates of repeat ptosis repair than older children (n = 59/175, 34% vs n = 5/33, 15%, p = 0.03 chi2). Conclusions Silicone sling frontalis suspension has a favorable outcome in 70% of pediatric patients. Preoperative and final MRD1 and reoperation rates were similar between both groups, suggesting that despite the higher complexity in atypical cases, the outcome is similar.

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Section: Discussionmentioning

confidence: 99%

Outcome of Silicone Sling Frontalis Suspension in Isolated Uncomplicated Congenital Ptosis vs. Complicated Ptosis

Prat

Zhao

Ramakrishnan

et al. 2023

Preprint

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“…Surgical correction of ptosis in CPEO has shown safety and e cacy 20 . Eshaghi et al have reported signi cant improvement in Margin-to-Re ex Distance 1 (MRD1) and chin-up posture at 1-year post-surgical follow up 21 and Doherty et al demonstrated low complication rate and objective bene t following surgery in patients with ocular myopathy and ptosis 22 .…”

Section: Discussionmentioning

confidence: 99%

Healthcare resource utilization of patients with mitochondrial disease in an outpatient hospital setting

Haque

Crawley²,

Shrestha

et al. 2023

Preprint

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Background and Objectives: Mitochondrial diseases present as multi-system disorders requiring a comprehensive multidisciplinary approach. The data on healthcare resource utilization associated with mitochondrial diseases and the clinical drivers of these costs is limited including for the out-patient setting where the majority of the clinical care for mitochondrial patients occurs. We performed a cross-sectional retrospective study of out-patient healthcare resource utilization and costs for patients with a confirmed diagnosis of mitochondrial disease. Methods: We recruited participants from the Adult Mitochondrial Disease Clinic in Sydney and stratified them into three groups: those with mitochondrial DNA (mtDNA) mutations (Group 1), those with nuclear DNA (nDNA) mutations and the predominant phenotype of chronic progressive external ophthalmoplegia (CPEO) or optic atrophy(Group 2) and those without a confirmed genetic diagnosis but clinical criteria and muscle biopsy findings supportive of a diagnosis of mitochondrial disease (Group 3). Data was collected through retrospective chart review and out-patient costs were calculated using the Medicare Benefits Schedule. Results: We analyzed the data from 91 participants and found that Group 1 had the greatest average out-patient costs per person per annum ($838.02; SD 809.72). Neurological investigations were the largest driver of outpatient healthcare costs in all groups (average costs per person per annum: - Group 1: $364.11; SD 340.93, Group 2: $247.83; SD 113.86 and Group 3: $239.57; SD 145.69) consistent with the high frequency (94.5%) of neurological symptoms. Gastroenterological and cardiac-related out-patient costs were also major contributors to out-patient healthcare resource utilization in Groups 1 and 3. In Group 2, ophthalmology was the second-most resource intensive specialty ($136.85; SD 173.35). The Group 3 had the greatest average healthcare resource utilization per person over the entire duration of out-patient clinic care ($5815.86; SD 3520.40) most likely due to the lack of a molecular diagnosis and a more customized management approach. Conclusion: The drivers of healthcare resource utilization are dependent on the phenotype-genotype characteristics and neurological, cardiac, and gastroenterological costs are the top three drivers in the out-patient clinics unless the patient has nDNA mutations with predominant phenotype of CPEO and/or optic atrophy wherein ophthalmological-related costs were the second most resource intensive driver.

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“…Surgical correction of ptosis in CPEO has shown safety and efficacy [ 23 ]. Eshaghi et al have reported significant improvement in Margin-to-Reflex Distance 1 (MRD1) and chin-up posture at 1-year post-surgical follow up [ 24 ] and Doherty et al demonstrated low complication rate and objective benefit following surgery in patients with ocular myopathy and ptosis [ 25 ]. In our cohort, 7 participants in Group 2 had ptosis correction surgery (41.2%) of which 3 patients required more than one operation.…”

Section: Discussionmentioning

confidence: 99%

Healthcare resource utilization of patients with mitochondrial disease in an outpatient hospital setting

Haque

Crawley

Shrestha

et al. 2023

Orphanet J Rare Dis

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Background and objectives Mitochondrial diseases present as multi-system disorders requiring a comprehensive multidisciplinary approach. The data on healthcare resource utilization associated with mitochondrial diseases and the clinical drivers of these costs are limited including for the out-patient setting where the majority of the clinical care for mitochondrial disease patients occurs. We performed a cross-sectional retrospective study of out-patient healthcare resource utilization and costs for patients with a confirmed diagnosis of mitochondrial disease. Methods We recruited participants from the Mitochondrial Disease Clinic in Sydney and stratified them into three groups: those with mitochondrial DNA (mtDNA) mutations (Group 1), those with nuclear DNA (nDNA) mutations and the predominant phenotype of chronic progressive external ophthalmoplegia (CPEO) or optic atrophy (Group 2) and those without a confirmed genetic diagnosis but clinical criteria and muscle biopsy findings supportive of a diagnosis of mitochondrial disease (Group 3). Data was collected through retrospective chart review and out-patient costs were calculated using the Medicare Benefits Schedule. Results We analyzed the data from 91 participants and found that Group 1 had the greatest average out-patient costs per person per annum ($838.02; SD 809.72). Neurological investigations were the largest driver of outpatient healthcare costs in all groups (average costs per person per annum:—Group 1: $364.11; SD 340.93, Group 2: $247.83; SD 113.86 and Group 3: $239.57; SD 145.69) consistent with the high frequency (94.5%) of neurological symptoms. Gastroenterological and cardiac-related out-patient costs were also major contributors to out-patient healthcare resource utilization in Groups 1 and 3. In Group 2, ophthalmology was the second-most resource intensive specialty ($136.85; SD 173.35). The Group 3 had the greatest average healthcare resource utilization per person over the entire duration of out-patient clinic care ($5815.86; SD 3520.40) most likely due to the lack of a molecular diagnosis and a less customized management approach. Conclusion The drivers of healthcare resource utilization are dependent on the phenotype–genotype characteristics. Neurological, cardiac, and gastroenterological costs were the top three drivers in the out-patient clinics unless the patient had nDNA mutations with predominant phenotype of CPEO and/or optic atrophy wherein ophthalmological-related costs were the second most resource intensive driver.

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Surgical management of ptosis in chronic progressive external ophthalmoplegia

Cited by 10 publications

References 12 publications

Outcome of Silicone Sling Frontalis Suspension in Isolated Uncomplicated Congenital Ptosis vs. Complicated Ptosis

Outcome of Silicone Sling Frontalis Suspension in Isolated Uncomplicated Congenital Ptosis vs. Complicated Ptosis

Healthcare resource utilization of patients with mitochondrial disease in an outpatient hospital setting

Healthcare resource utilization of patients with mitochondrial disease in an outpatient hospital setting

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