Perioperative Management of Patients with Down Syndrome: A Review

Abstract

Introduction: Down syndrome (DS) or Trisomy 21 is associated with a higher morbidity, mortality and surgery need. This syndrome presents a characteristic set of morphologic features regarding several systems: cardiovascular, respiratory, gastrointestinal, nervous, musculoskeletal, immune, hematologic, endocrine, ophthalmic and hearing. Therefore, special care is required to maximize safety before, during and after surgery. This study’s objective is to systematically review these needs in the perioperative (pre, intra and postoperative) period, and how to approach them. Methods: PubMed and Web of Science were searched and 32 articles selected for this revision. Results: DS patients have commonly pulmonary arterial hypertension and congenital heart defects. They may have swallow function abnormalities or gastro-esophageal reflux disease. Airway and respiratory tract conditions, such as aspiration pneumonia, obstructive sleep apnea, congenital tracheal stenosis, and recurrent infections, are common. In addition, cervical instability and nociception disorders may be present. Discussion: In order to prevent perioperative complications, several practices are suggested. In the preoperative period: assess the surgical risk using Aristotle and RACHS-1 scoring systems, analyze a recent echocardiogram, consider prophylactic antibiotic therapy and take strict aseptic precautions. Performing an X-ray looking for cervical instability is a controversial topic. In the intraoperative period: administer intravenous sedation in dental treatments, have anticholinergic agents available, consider aspiration prophylaxis, and position the neck particularly. However, there is no agreement on the best airway device. In the postoperative period: provide longer hospitalizations or stay in intensive unit care, remove catheters as soon as possible, assess the pain with specific tools, administer lower weight-adjusted doses of dexmedetomidine (controversial topic) and use morphine, once no opioid resistance was found. Conclusion: There are variations in the approach to the perioperative period, and even lack of agreement in some topics, making clear the need for specific guidelines to standardize this process and reduce morbidity.