Anesthetic Management of an Infant With Kleefstra Syndrome During Direct Laryngoscopy and Rigid Bronchoscopy: A Case Report.

Kleefstra syndrome (KS) is an autosomal dominant disorder caused by a chromosomal deletion at 9q34.3 resulting in pathogenic variants of the gene that codes for the enzyme, euchromatin histone methyltransferase 1 (EHMT1). KS is a rare, yet clinically relevant congenital disorder for anesthesiologists because of its potential for cardiac and craniofacial involvement. We present a 3-month-old patient with KS who required anesthetic care for diagnostic laryngoscopy and rigid bronchoscopy. The end-organ effects of KS are reviewed and our anesthetic care presented.

Regional Anesthesia for Pain Management After Orthopedic Procedures for Treatment of Lower Extremity Length Discrepancy.

INTRODUCTION: The use of regional anesthesia techniques continues to expand in a wide variety of surgical procedures as the benefits and safety are increasingly appreciated. Limb-lengthening procedures are often associated with significant postoperative pain and high opioid requirements which may impact patient's recovery and increase risk of chronic pain and long-term opioid use. METHODS: The current study retrospectively reviews our experience utilizing a novel peripheral nerve catheter (PNC) protocol for postoperative pain management in patients undergoing elective limb-lengthening procedures. We measure total opioid consumption following 48 hrs in the postoperative period between groups. RESULTS: A total of 70 patients were included from which 41 received general plus regional anesthesia (RA) and 29 were managed with general anesthesia alone (NORA). Postoperative pain needs were calculated as morphine equivalents (ME). There were no differences in the demographic characteristics between the groups. Over the first 48 postoperative hours, opioid use was 0.5 mg/kg ME (IQR 0.3, 0.9) in the RA group versus 1.7 mg/kg ME (IQR 1.1, 3.1) in the NORA group (p<0.001). Subgroup analysis between femoral lengthening and tibial-fibular lengthening procedures demonstrated the same opioid-sparing effect favoring the RA group compared to the NORA group. Hospital length of stay was significantly shorter in the femoral lengthening RA group compared to NORA group (32 hrs [IQR 29, 35] versus 53 hrs [IQR 33, 55], respectively). There was no significant difference in length of stay between the RA group and NORA group after tibial-fibular lengthening procedures. DISCUSSION: Regional anesthesia via continuous catheter infusions has a clinically significant opioid-sparing effect for postoperative pain management after limb-lengthening procedures and may facilitate earlier hospital discharge.