Cricopharyngeal Dilatation for the Long-term Treatment of Dysphagia in Oculopharyngeal Muscular Dystrophy

Oculopharyngeal muscular dystrophy (OPMD) is a rare autosomal dominant, progressive degenerative muscle disorder featuring dysphagia with limited therapeutic options. The aim of this study was to evaluate the safety and efficacy of repeated endoscopic dilatation for OPMD over a 15-year period. All patients seen at our Regional Swallowing Clinic with OPMD confirmed by genetic analysis were included. Cricopharyngeal dilatation was performed as an outpatient procedure using a wire-guided 18-mm (54 Fr) Savary-Gilliard bougie with the patient under sedation. Patients were offered repeat endoscopic dilatation when symptoms recurred. Symptom severity prior to initial dilatation and at follow-up was evaluated using the Sydney Swallow Questionnaire (SSQ). Nine patients (7 female, 2 male) were included for analysis. Median total treatment period was 13?years (range?=?3-15), median number of dilatations per patient was 7.2 (range?=?1-16), and median interval between treatments was 15?months (range?=?4.5-45). All patients recorded sustained symptom improvement. Mean SSQ score (out of 1,700) was 1,108.11 (SD?±?272.85) prior to first dilatation and 297.78 (SD?±?189.14) at last follow-up, representing a 73% decrease (95% CI?=?52-94) in degree of dysphagia symptoms (paired t-test, P?=?0.0001). All mean scores for individual questions also showed significant improvement (P?相似文献   

Paediatric traumatic brain injury: A review of pertinent issues

Children with traumatic brain injury (TBI), regardless of the severity of the injury, often face challenges when living in home, school and community. Their needs are often overlooked and recognition of the long-term consequences is not always central to the management of the child in the school or community. This article provides references to pertinent literature and suggestions for intervention from the clinical experiences of four individuals with extensive experience of the family stresses, educational, cognitive-communicative and behavioural challenges that occur after TBI in children. It provides information regarding these issues, particularly educational situations, and suggests methods that may be useful for service providers and family members.     

Composite polymerization stress as a function of specimen configuration assessed by crack analysis and finite element analysis

Objectives

To test the null hypothesis that polymerization-induced stress was not influenced by cavity dimensions and geometries.

Methods

Four experimental groups, with different C-factors and specimen volumes were defined using bottom-less glass disks (height: 1 or 2 mm) with a central hole 3 or 6 mm in diameter, and 3 mm wall thickness. Another four groups were created by bonding a glass plate to the bottom of the disks. Additionally, disks with 2-mm height, 3 mm in cavity diameter, and 4.5-mm thick walls were prepared. Vickers indents (9.8 N, 20 s) were made at the top surface at 600 μm from the cavity margin. The lengths of the indentation diagonal and the corner cracks parallel to the cavity margin were measured. Ten minutes after restoration (Majesty Esthetic, Kuraray), cracks were re-measured. Stresses at the indent site were calculated based on glass fracture toughness and increase in crack length. Data were subjected to ANOVA/Tukey or Kruskal–Wallis/Mann–Whitney tests (alpha: 5%, n = 8). Finite element analysis (FEA) was used to estimate stress at the interface and the effective structural rigidity of the substrate.

Results

Overall, for experimental and FEA results, cavities developed higher stress than bottom-less disks. Increasing wall thickness did not affect stress. When similar geometries and C-factors were compared, higher volumes developed higher stress and had greater incidence of margin cracking.

Clinical significance

C-factor is a suitable predictor for polymerization stress in low compliance environments, particularly due to its simplicity. However, the influence of cavity size cannot be disregarded especially for the development of marginal cracking. The interaction between size, geometry and stiffness is likely to become more complex according to the complexity of the cavity shape.     

Diagnostic and therapeutic direct peroral cholangioscopy using an intraductal anchoring balloon

AIM: To report our experience using a recently introduced anchoring balloon for diagnostic and therapeutic direct peroral cholangioscopy (DPOC).METHODS: Consecutive patients referred for diagnostic or therapeutic peroral cholangioscopy were evaluated in a prospective cohort study. The patients underwent DPOC using an intraductal anchoring balloon, which was recently introduced to allow consistent access to the biliary tree with an ultraslim upper endoscope. The device was later voluntarily withdrawn from the market by the manufacturer.RESULTS: Fourteen patients underwent DPOC using the anchoring balloon. Biliary access with an ultraslim upper endoscope was accomplished in all 14 patients. In 12 (86%) patients, ductal access required sphincteroplasty with a 10-mm dilating balloon. Intraductal placement of the ultraslim upper endoscope allowed satisfactory visualization of the biliary mucosa to the level of the confluence of the right and left hepatic ducts in 13 of 14 patients (93%). Therapeutic interventions by DPOC were successfully completed in all five attempted cases (intraductal biopsy in one and DPOC guided laser lithotripsy in four). Adverse events occurred in a patient on immunosuppressive therapy who developed an intrahepatic biloma at the site of the anchoring balloon. This required hospitalization and antibiotics. Repeat endoscopic retrograde cholangiopancreatography 8 wk after the index procedure showed resolution of the biloma.CONCLUSION: Use of this anchoring balloon allowed consistent access to the biliary tree for performance of diagnostic and therapeutic DPOC distal to the biliary bifurcation.