Severe Multilevel Tracheal Stenosis with Significant Twisting in a Patient with Spondylometaphyseal Dysplasia: A Case Report.

Spondylometaphyseal dysplasia (SMD) is a rare genetic disorder affecting skeletal growth and development presenting anesthesiologists with many perioperative challenges. We present a case of a patient found to have multilevel tracheal stenosis due to twisting and folding of his trachea. This was discovered on imaging during a research review of SMD cases at our institution. Structural and functional abnormalities of the trachea have not been reported in SMD. This is the first description of a patient with SMD with severe multilevel tracheal disease requiring tracheal reconstructive surgery.

Metatropic dysplasia-a skeletal dysplasia with challenging airway and other anesthetic concerns.

BACKGROUND: Metatropic dysplasia is a rare form of skeletal dysplasia requiring multiple anesthetics for surgical and imaging procedures, most of which are orthopedic procedures. We provide centralized care to patients with skeletal dysplasia at our tertiary care pediatric hospital, and we were able to collect the largest number of metatropic dysplasia patients reported to date. AIM: The aim of this retrospective study was to describe and characterize the anesthetic difficulties in this high-risk population. METHODS: Medical charts of all patients with metatropic dysplasia were reviewed to collect data, including anesthetics performed, difficulties, and complications related to the anesthetic care, co-morbid conditions, and related events. RESULTS: Twenty-three patients with metatropic dysplasia underwent 188 anesthetics with 61% of the anesthetics having been administered for orthopedic procedures. Fourteen of 23 (60.8%) progressively became difficult to intubate over the course of their care, with 12 out of 14 having undergone cervical spine fusion. These 14 patients had a total of 133 procedures. Sixty procedures (45.1%) had an airway described as difficult. Glidescope was the difficult airway tool most commonly used (68%) with flexible fiberoptic scope used 12% and Miller or Macintosh blade used 18% of the time. In addition to the airway difficulties, spinal canal narrowing or stenosis was widely prevalent, and no neuraxial anesthetic was performed in any of our patients. CONCLUSION: Difficult airway is the most common co-morbid condition present in patients with metatropic dysplasia, especially if their cervical spine has been fused. Familiarity with the difficulties involving the airway and its management is critical in safe and successful management of anesthesia in this high-risk population.

Clinical relevance of echocardiogram in patients with cerebral palsy undergoing posterior spinal fusion.

BACKGROUND: Spinal deformity is one of the secondary musculoskeletal problems that occur with cerebral palsy (CP). Of the co morbidities associated with CP and spinal deformity, cardiac function is of theoretical concern. OBJECTIVE: The goal of our study was to determine the clinical relevance of routine preoperative cardiology evaluation via echocardiogram for patients with CP presenting for posterior spine fusion (PSF) surgery. METHODS: A retrospective chart review was performed of CP patients presenting for scoliosis surgery. The data collected for each patient included: age, sex, height, weight, Cobb angle, and medical history. All patients had a preoperative cardiac evaluation. RESULTS: Seventy-two patients were included. The mean age was 13.6 ± 3.4 years. Left ventricular systolic function was normal in all patients; the mean shortening fraction was 39.3 ± 6.2%. No patient had more than mild insufficiency of either the semilunar or atrioventricular valve. One patient was diagnosed with aortic root dilation as well as aortic valve insufficiency. All patients had PSF surgery without changes in anesthetic or surgical plans, and no patient experienced complications attributable to a cardiac origin. CONCLUSION: The results suggest that routine preoperative cardiology evaluation via echocardiogram for children with CP in the absence of clinical history or physical examination findings suggestive of cardiac disease is not necessary.

Anesthesia management of a patient with a laryngotracheo-esophageal cleft.

Laryngotracheo-esophageal cleft is a rare congenital anomaly that results from complete or partial failure of the development of the tracheoesophageal septum. The presenting symptoms include stridor, respiratory distress, and coughing or cyanotic episodes with feeding. There are four classifications for laryngeal clefts; the severity depends on the type present. We discuss the anesthesia management of a neonate with a Type IV cleft who presented for an emergency gastric division to prevent pulmonary aspiration and later returned for final repair of the defect.