3/28/18. Navigating the Bumps and Dips: The Ins and Outs of Pectus Excavatum and Pectus Carinatum. Disclosure Information.

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1 Navigating the Bumps and Dips: The Ins and Outs of Pectus Excavatum and Pectus Carinatum Beth Orrick, MSN, APRN, FNP-BC Amy Pierce, MSN, APRN, PPCNP-BC Children s Mercy Hospital Kansas City, MO Disclosure Information No disclosures Objectives Identification of pectus excavatum and pectus carinatum Identify key points to diagnosis, work-up and perioperative considerations Describe the surgical management of pectus excavatum with newest developments Treatment options for pectus carinatum 1

2 Pectus Excavatum Inward growth of costal cartilages that pushes the sternum posteriorly Most common chest wall abnormality with incidence of 1 in 1000 children having it More common in males 4 males: 1 female Can exhibit functional impairment as well as psychosocial impact Pectus Excavatum Work-Up History and Physical Symptoms Shortness of breath Chest pain Co-morbidities Scoliosis Connective tissue disorders Marfan s Ehler s Danlos Loeys Dietz Family History Metal Allergy AGE 2

3 Work-Up CT Scan mm 69.8 mm Haller Index Standard metric for severity > 3.25 severe = 2.7 (posterior sternum to anterior spine) Work-Up CT Scan 69.8 mm 90.3 mm Correction Index More accurate Percentage of chest depth lost due to excavatum depth > 16% considered severe ( ) 90.3 x 100= 22.7 Pectus Bar Insertion 3

4 Cryoablation Newest modality for postoperative pain control Freeze (-60 degrees C) intercostal nerves 3 or 4 through 7 or 8 using a scope through lateral chest incisions Analgesia occurs by destroying neural pathways or sympathetic structures involved in pain transmission The full effect may take hours for optimal pain control The cryoablation will last between 2-3 months Patients may experience some skin numbness to chest wall. The numbness should resolve within 3 months when the nerve regenerates Can get off oral narcotics approx. 1 week earlier Cryoablation Retrospective study: 8 out of 28 patients undergoing excavatum repair received cryoablation Mean operative time was 20 minutes longer in the cryoablation group The days to only oral pain medication was over 1 day less in the cryoablation group Length of stay was over 2 days shorter with cryoablation There were no reported complications from cryoablation or bar placement during the study period In follow up the median days to discontinuation of oral narcotics was a week less in the cryoablation Cryoablation 4

5 Cryoablation Medication Protocol 1) Pre-op Gabapentin mg/kg (max 800 mg) PO (capsules preferred in 100 mg increments, can give liquid if patient can t take pills) 2) Intra-op: a. Methadone 0.1 mg/kg (max 10 mg) IV during or as soon as possible after or induction (in lieu of spinal narcotic). b. Acetaminophen (10-15 mg/kg) IV one hour before case ends or after induction if case expected to last < 1 hr. c. Ketorolac 0.5 mg/kg (max 30 mg) IV at closing (check with surgeon first before giving); instead of this we could consider giving celecoxib 2-3 mg (max 200 mg) preop so NSAID is always given [see above]. d. Lidocaine 1 mg/kg IV with induction. Cryoablation Medication Protocol e. Dexmedetomidine mcg/kg IV bolus at or soon after induction. May give additional 0.1 mcg/kg IV at or near emergence if felt necessary to mitigate emergence delirium. f. Fentanyl titrated intraoperatively at discretion of anesthesiologist. g. Consider Ketamine 1mg/kg IV with induction or infusion of 0.5 mg/kg/hr and/or Dexmedetomidine infusion of 0.5 mcg/kg/hr and/or Lidocaine infusion 1 mg/kg/hr (reducing induction bolus to 0.5 mg/kg, and foregoing local anesthetic infiltration at closure) through case if patient had pain preop or is very anxious, which are predictors of poor pain trajectory, which may correlate with chronic post-surgical pain (CPSP). Cryoablation Medication Protocol 3) PACU: a) Fentanyl 0.5 mcg/kg q 5 min PRN pain in PACU order IV PRN pain b) Diazepam 0.05 mg/kg (max 5 mg) IV x 1 PRN muscle spasm 5

6 Cryoablation Medication Protocol POD #0 (Traditional Floor Status) 1) Ketorolac 0.5mg/kg (max 30 mg) IV q6hr SCHEDULED (Check first if OK with surgeon). Begin first dose 6 hours after the dose in the OR (or 8 hours after celecoxib preop dose). Hold for UO <0.5 ml/hr. 2) Diazepam 0.05 mg/kg IV (max 3 mg) q6hr prn muscle spasm 3) Ondansetron mg/kg IV (max 4mg) q4hr PRN nausea/vomiting. 4) Diphenhydramine 0.5 mg/kg IV (max 25 mg) q6hr PRN pruritus or nausea/vomiting not treated by ondansetron Cryoablation Medication Protocol 5) Gabapentin 5 mg/kg PO TID for 3 days post op (continue for 3 mos. in those with demonstrated poor pain trajectories, esp. with neuropathic component, as inpatient) 6) PRN Pain (mild): Acetaminophen mg/kg (max 1000 mg) PO q4hr PRN mild pain or temp >38.5 C, not more than 4 doses per day 7) PRN pain (moderate or severe): Oxycodone 0.1mg/kg (usually 5-10 mg) PO Q4hrs PRN moderate or severe pain Oxycontin 10 mg PO BID, wean upon home discharge (usually 10 mg PO BID x 3 days, then 10 mg PO daily x 3 day, then off. Can wean faster if tolerated) Hydromorphone IV mg/kg/dose q3hr PRN moderate or severe pain (max 0.4 mg) or Morphine IV 0.1 mg/kg/dose q3hr PRN moderate or severe pain (max 3 mg) 8) Consider Ketamine infusion mg/kg/hr postop X hrs for patients with expectation of poor pain trajectory (may correlate with CPSP) Postoperative Considerations Wean opioids as tolerated ADL s and normal activities when tolerated Back to school/work when off opioids and pain tolerable with Tylenol/Ibuprofen Clinic follow-up 2-4 weeks postop No sports for 2-3 months No contact sport for 6 months 6

7 Complications Bar infection or allergic reaction to bar Pre-op screening and possible formal testing Pain Stabilizer sites Rest, cold/warm compresses, NSAIDS Interventional Radiology Pain med & steroid injections Cryoablation (if did not receive in OR) Stabilizer removal Recurrence after bar removal Bar in for 3 years Increased risk with growth spurt Pectus Carinatum: 2 major subtypes Chondrogladiolar Protrusion of inferior costal cartilages (most common) Chondromanubrial Protrusion of superior costal cartilages Pectus Carinatum 7

8 Pectus Carinatum Less common than pectus excavatum Prevalence 0.6%, with 80% being male Usually presents during puberty at the time of rapid linear growth, but can also be present at younger ages. Grows as they grow, may become masked with muscle, adipose or breast tissue. Cartilage is pliable during adolescence, therefore, remodeling is possible. Pectus Carinatum No significant physiologic symptoms (shortness of breath, exercise intolerance, chest pain). Psychosocial ramifications: poor body image, low self esteem. Children may avoid activities or social interaction due to embarrassment of their chest. Pectus Carinatum: Treatment Options Mild cases without symptoms/cosmetic concerns, no treatment may be necessary. Traditionally, correction was a surgical approach (open/modified Ravitch or reversed Nuss). Need for operation is now less common. Non-surgical bracing is preferred avoidance of surgical risks and scarring less costly no downtime excellent cosmetic outcomes 8

9 Pectus Carinatum: Bracing The chest wall is flexible during childhood until skeletal maturity is reached, then becomes rigid. Bracing during adolescence-remodels the abnormal cartilage into a neutral position using external pressure by a brace. Gentle pressure gradually corrects the protrusion. Worn continuously and adjusted regularly the chest slowly redevelops into a more normal shape. Pectus Carinatum Bracing Pectus Carinatum: Bracing Dynamic compression device (DCD) Custom-fitted brace Measuring device monitors brace pressure (psi) on the thoracic wall and objectively measure pressure for complete correction. Once corrected, brace intervals and pressures are decreased into "retainer mode. Lower PIC and increased brace time significantly impacts likelihood of resolution. Brace pressure Initial correction pressure 9

10 Pectus Carinatum: Bracing 503 patients evaluated for PC, 340 (68%) underwent bracing Results: 47% achieved complete correction (neutral position or retainer mode) with average bracing time of 7.5 mos. Median duration of bracing: 16 mos No recurrence after bracing, 1 failed bracing and required operative correction. Minimal complications: skin breakdown, mild discomfort, mechanical problems. >75% reported no issues during bracing. Clinical criteria: Motivated to wear brace (23 hrs/d) Age>10 years Correction pressure <7.5 psi, (not exclusive) Pectus Carinatum Bracing Before After Pectus Carinatum Bracing Before After 10

11 Pectus Carinatum Pearls Must be compliant and willing to wear brace Brace boys later and girls earlier My job as APRN with screwdriver developmental aspect, how to empower teenagers, impact on self esteem, develop process for success, set expectations early. References Colozza, S., & Butter, A. (2013). Bracing in pediatric patients with pectus carinatum is effective and improves quality of life. Journal of Pediatric Surgery, 48, De Beer, S., Gritter, M., De Jong, J., & van Heurn, E. (2017). The Dynamic Compression Brace for Pectus Carinatum: Intermediate Results in 286 Patients. Annals of Thoracic Surgery, 103, Fonkalsrud, E., & Beanes, S. (2001). Surgical management of pectus carinatum: 30 years experience. World Journal of Surgery, 25, Gould, J., Sharp, R., St. Peter S., Snyder, C., Juang, D., Aguayo, P., Holcomb, G. (2017). The minimally invasive repair of pectus excavatum using a subxiphoid incision. European Journal of Pediatric Surgery, 27, 2-6. Haje, S., Harcke, H., & Bowen, J., (1999). Growth disturbance of the sternum and pectus deformities: imaging studies and clinical correlation. Pediatric Radiology, 29, Kim, S., Idowu, O., Palmer, B., & Lee, S. (2016). Use of transthoracic cryoanalgesia during the Nuss procedure. Journal of Thoracic Cardiovascular Surgery, 151, References Keller, B., Kabagame, S., Becker, J., Chen, Y., Goodman, L., Clark-Wronski, J., Raff, G. (2016). Intercostal nerve cryoablation versus thoracic epidural catheters for postoperative analgesia following pectus excavatum repair: Preliminary outcomes in twenty-six cryoablation patients. Journal of Pediatric Surgery, 51, Knudsen, M.J., Grosen, K., Pilegaard, H.K., & Laustsen, S. (2015). Surgical correction of pectus carinatum improves perceived body image, mental health and self-esteem. Journal of Pediatric Surgery, 50, Pinson, M., Coop, C., & Webb, C. (2014). Metal hypersensitivity in total joint arthroplasty. Annals of Allergy, Immunology & Asthma, 113, Moorjani, N., Zhao, F., Tian, Y., Liang, C., Kaluba, J., & Maiwand, M. (2001). Effects of cryoanalgesia on post-thoracotomy pain and on the structure of intercostal nerves: a human prospective randomized trial and histological study. European Journal of Cardiothoracic Surgery, 20, Nuss, D., & Kelly, R.E. (2014). Congenital Chest Wall Deformities. In Holcomb, G.W., Murphy, J.P., & Ostlie, D.J. (Eds.), Ashcraft s Pediatric Surgery ( ). Philadelphia, PA: Elsevier Health Sciences. 11

12 References Obermeyer, R.J., Gaffar, S., Kelly, R.E. Jr., Kuhn, M.A., Frantz, F.W., McGuire, M.M., Kelly, C.S. (2018). Selective versus routine patch metal allergy testing to select bar material for the Nuss procedure in 932 patients over 10 years. Journal of Pediatric Surgery, 53(2), Poola, A.S., Pierce, A.L., Orrick, B.A., St. Peter, S.D., Snyder, C.L., Juang, D., Holcomb, G.W. (2018). A Single-Center Experience with Dynamic Compression Bracing for Children with Pectus Carinatum. European Journal of Pediatric Surgery, 28, St. Peter, S.D., Weesner, K.A., Weissend, E.E., Sharp, S.W., Valusek, P.A., Sharp, R.J., Ostlie, D.J. (2012). Epidural vs patient-controlled analgesia for postoperative pain after pectus excavatum repair: a prospective, randomized trial. Journal of Pediatric Surgery, 47, St. Peter, S.D., Juang, D., Garey, C.L., Laituri, C.A., Ostlie, D.J., Sharp, R.J., Snyder, C.L. (2011). A novel measure for pectus excavatum: The correction index. Journal of Pediatric Surgery, 46, References Sujka, J., Benedict, L.A., Fraser, J.D., Aguayo, P., Millspaugh, D.L., & St. Peter, S.D. (2018). Outcomes Using Cryoablation for Post-Operative Pain Control in Children Following Minimally Invasive Pectus Excavatum Repair. Manuscript submitted for publication. Trescot, A.M. (2003). Cryoanalgesia in interventional pain management. Pain Physician, 6,