Single-incision, off-pump repair of a right aortic arch with an aberrant left subclavian artery and diverticulum of Kommerell in an adult.

Repairing a complete vascular ring in adults can be challenging. A right aortic arch with an aberrant retro-oesophageal left subclavian artery and persistent diverticulum of Kommerell represents one of the most common variants seen in adults, and the ring is completed by the left-sided ligamentum arteriosum. Most presentations in adults occur secondary to oesophageal compression, resulting in varying degrees of dysphagia. Owing to the difficulty and challenges associated with exposure in adults, it is not unusual for surgeons to offer a two-incision approach or to stage the procedure. We present a detailed surgical technique for a single-incision repair of a right aortic arch with an aberrant retro-oesophageal left subclavian artery via a left posterolateral thoracotomy.

Clinical Discordance in Monozygotic Twins With Autism Spectrum Disorder.

There is a significant concordance of autism spectrum disorder in monozygotic (MZ) twins, where behavioral manifestations are heavily influenced by genetic factors. We describe a case of male monozygotic twins with autism spectrum disorder (ASD), raised in the same household, that present with different clinical manifestations. One of the twins presents with intermittent frank syncopal episodes, sinus bradycardia, and elevated alkaline phosphatase (ALP), while the other has symptoms of attention-deficit/hyperactivity disorder (ADHD), normal cardiological findings, and normal ALP level. The clinical discordance in this pair of monozygotic twins may be due to any of the following: 1) neuroanatomic cerebellar differences, 2) variable expression of genotype, and 3) inconsistent neurotransmitter regulation.

The Safety and Effectiveness of High-Dose Propranolol as a Treatment for Challenging Behaviors in Individuals With Autism Spectrum Disorders.

PURPOSE/BACKGROUND: Individuals with autism spectrum disorders present with social communication deficits and a rigid adherence to sameness. Along with these symptoms, many individuals also present with severe challenging behaviors that place themselves as well as their families and communities at risk for injury. For these individuals, new and effective treatments are acutely needed. Propranolol has been used worldwide for over 50 years. Its primary indication is for hypertension, but there is evidence that, at higher doses, propranolol inhibits rage and anger through its effects on the central nervous system. This effect has been demonstrated in a variety of neuropsychiatric disorders. METHODS/PROCEDURES: Here, we present 46 retrospective analyses of clinical cases that were followed by a psychiatrist. Propranolol was prescribed as an add-on to the patients' existing medications. The doses ranged from 120 to 960 mg per day (mean = 462 mg). FINDINGS/RESULTS: Thirty-nine (85%) of 46 patients were found to be much improved or very much improved on the physician-rated Clinical Global Impression Improvement scale. There were few side effects noted, with only 2 subjects unable to tolerate the propranolol. IMPLICATIONS/CONCLUSIONS: It appears that high-dose propranolol can be given safely with minimal adverse cardiovascular problems, provided that close clinical monitoring is maintained. A more rigorous clinical trial is needed to elucidate and verify its clinical utility, clinical practice parameters, and the effects of propranolol as a monotherapy versus as an add-on to the patient's existing medication regimen.