Chronic pain in Noonan Syndrome: A previously unreported but common symptom.

Noonan syndrome (NS) is a multiple malformation syndrome characterized by pulmonic stenosis, cardiomyopathy, short stature, lymphatic dysplasia, craniofacial anomalies, cryptorchidism, clotting disorders, and learning disabilities. Eight genes in the RAS/MAPK signaling pathway are implicated in NS. Chronic pain is an uncommon feature. To investigate the prevalence of pain in NS, we distributed a two-part questionnaire about pain among NS individuals at the Third International Meeting on Genetic Syndromes of the Ras/MAPK Pathway. The first part of the questionnaire queried demographic information among all NS participants. The second part was completed by individuals with chronic pain. Questions included musculoskeletal problems and clinical features of pain. Forty-five questionnaires were analyzed; 53% of subjects were female. Mean age was 17 (2-48) years; 47% had a PTPN11 mutation. Sixty-two percent (28/45) of individuals with NS experienced chronic pain. There was a significant relationship between prevalence of pain and residing in a cold climate (P = 0.004). Pain occurred commonly in extremities/joints and head/trunk, but more commonly in extremities/joints (P = 0.066). Subjects with hypermobile joints were more likely to have pain (P = 0.052). Human growth hormone treatment was not statistically significant among subjects without chronic pain (P = 0.607). We conclude that pain is a frequent and under-recognized clinical feature of NS. Chronic pain may be associated with joint hypermobility and aggravated by colder climate. Our study is a preliminary investigation that should raise awareness about pain as a common symptom in children and adults with NS.

Adjunctive Pain Control Methods Lower Narcotic Use and Pain Scores for Patients With Adolescent Idiopathic Scoliosis Undergoing Posterior Spinal Fusion.

STUDY DESIGN: This was a retrospective review of patients with adolescent idiopathic scoliosis (AIS) undergoing posterior spinal fusion (PSF). OBJECTIVES: To determine whether the use of adjunctive pain medications (bupivacaine pump, dexmedetomidine, and ketorolac) will reduce the need for opioids, reduce postoperative pain, and shorten length of hospital stay in patients with AIS undergoing PSF. SUMMARY OF BACKGROUND DATA: Posterior spinal fusion and instrumentation for AIS can cause significant postoperative pain. Adjunctive pain control modalities, including the use of ketorolac, dexmedetomidine, and subcutaneous bupivacaine pumps, all can lessen the effects of postoperative pain. METHODS: Retrospective review of adolescents aged 10-18 years with AIS receiving PSF surgery over the past 10 years at a tertiary care children's hospital. All patients with AIS undergoing PSF were included in the study. Patients older than 18 or younger than 10 years and those undergoing PSF for other diagnoses, including neuromuscular scoliosis, congenital scoliosis, and kyphosis, were excluded from the study. Patients' pain was managed postoperatively with adjunctive medications in addition to intravenous and oral opioids. Variables of interest were local anesthetic bupivacaine delivered through a subcutaneous pump, sedative/analgesic dexmedetomidine, and ketorolac. Primary outcomes analyzed were normalized opioid requirement after surgery, visual analog scale (VAS) pain scores, and length of stay in the hospital. RESULTS: A total of 196 children were analyzed with no significant differences in demographics. Univariate analysis showed that all 3 adjunct medications improved outcomes. A multivariate regression model of the outcomes with respect to the 3 medication variables of interest was built, showing that the bupivacaine pump significantly reduced normalized opioid requirement by 0.98 mg/kg (p = .001) and reduced VAS pain scores by 0.67 points (p = .004). Dexmedetomidine significantly reduced the average VAS pain scores in the first 24 hours by 0.62 points (p = .005). CONCLUSIONS: Use of the bupivacaine pump provided improved analgesia with lower pain scores, lower opioid requirements, and a lower length of stay.

Evaluation of dexmedetomidine and postoperative pain management in patients with adolescent idiopathic scoliosis: conclusions based on a retrospective study at a tertiary pediatric hospital.

OBJECTIVES: This study evaluated the effectiveness of dexmedetomidine in decreasing opioid use in children with adolescent idiopathic scoliosis following posterior spinal fusion surgery at a pediatric tertiary care hospital over the past 10 years. DESIGN: This was a retrospective chart review. Patients were separated into two groups: those that received opioid via patient-controlled analgesia pain therapy alone and those that received opioid via patient-controlled analgesia pain therapy with dexmedetomidine. SETTING: A tertiary pediatric free-standing hospital. The study focused on care administered in the perioperative period, including the operating room, ICU, and general hospital floor. PATIENTS: One hundred sixty-three children with adolescent idiopathic scoliosis. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Measurements included patient demographics, American Society of Anesthesiologists Physical Status Classification System, levels of spinal fusion, length of hospital stay, complications, numeric pain scores, opioid requirement, elastomeric pain pump use, length of time until ambulation, adverse effects, and naloxone use. Data were collected through the first 72 hours of the perioperative period. One hundred six patients received opioids via patient-controlled analgesia therapy with dexmedetomidine and 57 received opioids via patient-controlled analgesia alone. Within the groups, there were 46 patients who received local anesthetic infusions via elastomeric pumps in the patient-controlled analgesia with dexmedetomidine group and 16 patients had pumps in the patient-controlled analgesia-alone group. There was no overall difference in postoperative use of morphine (or equivalents) between the two groups. However, the use of elastomeric pain pumps demonstrated a statistically significant decrease in mean overall opioid consumption (42.6 mg vs 63.1 mg, p < 0.001). CONCLUSIONS: There was no difference in opioid use related to dexmedetomidine on any postoperative day. The only variable showing a significant opioid sparing effect was the use of local anesthetic infusions via elastomeric pumps. Using continuous local anesthetic infusions instead of dexmedetomidine could eliminate the need for ICU admission, require shorter hospital stays, and reduce costs while still providing safe and effective pain control.

Validation and incremental value of the hybrid algorithm for CTO PCI.

OBJECTIVES: To evaluate the outcomes and benefits of using the hybrid algorithm for chronic total occlusion (CTO) percutaneous coronary intervention (PCI). BACKGROUND: The hybrid algorithm harmonizes antegrade and retrograde techniques for performing CTO PCI. It has the potential to increase success rates and improve efficiency for CTO PCI. No previous data have analyzed the impact of this algorithm on CTO PCI success rates and procedural efficiency. METHODS: Retrospective analysis of contemporary CTO PCI performed at two high-volume centers with adoption of the hybrid technique was compared to previously published CTO outcomes in a well matched group of patients and lesion subsets. RESULTS: After adoption of the hybrid algorithm, technical success was significantly higher in the post hybrid algorithm group 189/198 (95.4%) vs the pre-algorithm group 367/462 (79.4%) (P < 0.001). Procedural success in the post hybrid algorithm group 175/198 (88.3%) when compared to the pre-algorithm group 360/462 (77.9%) (P < 0.001) was also significantly improved. Failure rates were significantly lower. Efficiency parameters including procedure time, contrast volume, fluoroscopy time, and radiation doses all favored the post hybrid group but did not reach statistical significance. CONCLUSIONS: The validation of the hybrid algorithm has the potential to disseminate adoption of CTO PCI.

Are We Undermedicating Patients With Neuromuscular Scoliosis After Posterior Spinal Fusion?

STUDY DESIGN: Retrospective, matched study of patients with adolescent idiopathic scoliosis (AIS) and patients with cerebral palsy (CP) undergoing (PSF). OBJECTIVES: To compare pain management, through measurement of the amount of narcotic used and pain scores, for patients with neuromuscular (NM) scoliosis undergoing PSF to a cohort of patients with AIS. SUMMARY OF BACKGROUND DATA: Posterior spinal fusion for children with severe NM scoliosis carries a high risk of complications. Appropriate assessment of pain is crucial; undertreatment of pain leads to anxiety whereas overtreatment can lead to respiratory depression and additional complications. METHODS: A series of patients with NM scoliosis was matched for age, gender, and weight with a group of patients with AIS. Data collection included age, curve type and magnitude, and instrumentation type and levels fused. The total opioid used (TOU) was determined by summing all narcotics given during the hospital stay and converting them to morphine equivalent units. The data were then analyzed to determine differences in TOU. RESULTS: A total of 25 patients with NM scoliosis were included in the study. This group was matched with 25 patients with AIS scoliosis. The TOU for the NM group was 1.2 mg morphine/kg (range, 0.28-4.21 mg morphine/kg) whereas the TOU for the AIS group was 3.52 mg morphine/kg (range, 0.71-15.51 mg morphine/kg) (p < .0000001). CONCLUSIONS: In this case-control analysis, patients with AIS undergoing PSF received more than twice the amount of narcotic compared with a matched group of patients with NM scoliosis. These data suggest that NM patients' pain may be undertreated compared with AIS patients. More study is indicated to investigate pain assessment and pain control in this vulnerable patient population to improve care.