Orthognathic Correction in Prader-Willi Syndrome: Occlusion and Sleep Restored.

Children with Prader-Willi Syndrome (PWS) may present with a malocclusion and have a high propensity of developing obstructive sleep apnea (OSA). Obstructive sleep apnea is associated with short- and long-term adverse effects that negatively impact children with PWS. A case of a 15-year-old male with PWS, OSA, and a debilitating malocclusion is presented who underwent a combination of Le Fort 1 osteotomy, genioplasty, and tongue reduction to successfully treat his OSA and malocclusion. In select cases, orthognathic correction and other surgical therapies should be considered in patients with PWS.

The relationship between manometric subtype and outcomes of surgical treatment for patients with achalasia : Achalasia: manometric subtypes.

BACKGROUND: The Chicago Classification describes three distinct subtypes of achalasia and it appears to be a promising tool in predicting results of treatment with standard Heller Myotomy. The aim of this study is to analyze the outcomes of surgical treatment for achalasia using an extended Heller myotomy for each subtype and to identify additional parameters that may predict the success of therapy. METHODS: 72 consecutive patients with achalasia were evaluated at the University of Washington between 2008 and 2013. Symptom duration, patient age, and the degree of esophageal dilation (stage 1-3) as assessed by radiography were determined. We defined treatment failure as no improvement in symptoms and/or need for a second therapy within 1 year. Long-term follow-up data of 25 patients were available in the form of a survey evaluating overall satisfaction with the operation. RESULTS: The distribution of patients according to subtype included 13 with type I, 54 with type II, and 5 with type III. All of the type I patients had some degree of esophageal dilation on radiography, whereas no dilation was found in the type III group. All patients underwent uneventful laparoscopic-extended Heller myotomy. Two patients were classified as failures, including one with type I and one with type II achalasia; however, further investigation revealed the cause of both failures to be the development of peptic stricture. Only one of the 25 patients with long-term follow-up reported dissatisfaction with the treatment result and indicated persistent chest pain without dysphagia. CONCLUSIONS: Laparoscopic-extended Heller myotomy is a highly successful treatment for patients with achalasia and outcomes do not appear to vary significantly according to the manometric subtype. Failures may result from reflux in patients who develop esophagitis or stricture. Chest pain is not always responsive to esophagogastric myotomy despite relief of dysphagia.

Laparoscopic anti-reflux surgery for idiopathic pulmonary fibrosis at a single centre.

We sought to assess whether laparoscopic anti-reflux surgery (LARS) is associated with decreased rates of disease progression in patients with idiopathic pulmonary fibrosis (IPF).The study was a retrospective single-centre study of IPF patients with worsening symptoms and pulmonary function despite antacid treatment for abnormal acid gastro-oesophageal reflux. The period of exposure to LARS was September 1998 to December 2012. The primary end-point was a longitudinal change in forced vital capacity (FVC) % predicted in the pre- versus post-surgery periods.27 patients with progressive IPF underwent LARS. At time of surgery, the mean age was 65 years and mean FVC was 71.7% pred. Using a regression model, the estimated benefit of surgery in FVC % pred over 1 year was 5.7% (95% CI -0.9-12.2%, p=0.088) with estimated benefit in FVC of 0.22 L (95% CI -0.06-0.49 L, p=0.12). Mean DeMeester scores decreased from 42 to 4 (p<0.01). There were no deaths in the 90 days following surgery and 81.5% of participants were alive 2 years after surgery.Patients with IPF tolerated the LARS well. There were no statistically significant differences in rates of FVC decline pre- and post-LARS over 1 year; a possible trend toward stabilisation in observed FVC warrants prospective studies. The ongoing prospective randomised controlled trial will hopefully provide further insights regarding the safety and potential efficacy of LARS in IPF.