CONGENITAL LUNG LESION Round Table. Objectives. Congenital Lung Lesions: Anatomy and Physiology Leah Barefoot, DNP, CPNP-PC

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1 CONGENITAL LUNG LESION Round Table L. Barefoot, E. Paton, C. Schultz, R. Caskey, M. O Day Objectives Review the anatomy and pathophysiology of congenital lung lesions List the preoperative evaluation of a patient with a congenital lung lesion Describe the operative procedure for a patient with a congenital lung lesion Describe the postoperative care for children with CPAM and CLE Discuss the short and long term postoperative follow up including complications and outcomes Congenital Lung Lesions: Anatomy and Physiology Leah Barefoot, DNP, CPNP-PC 1

2 Anatomy & Pathophysiology Congenital Pulmonary Malformations (CPMs) Congenital Pulmonary Airway Malformation (CPAM) Bronchopulmonary Sequestrations (BPS) Congenital Lobar Emphysema (CLE) Congenital Pulmonary Airway Malformation (CPAM) Previously known as CCAM Incidence 1:8300 1:35000 Histology Subtypes 0-4 Diagnosis Often prenatal May regress Prognosis Risk of Hydrops (CVR ratio) Surgical Resection Presentation Dependent Bronchopulmonary Sequestrations (BPS) An area of nonfunctioning lung tissue that commonly receives it arterial blood supply from the descending aorta No communication with the tracheobronchial tree Classified as intralobar sequestration (ILS) or extralobar (ELS) ILS: lesion is located within a normal lobe and lacks its own visceral pleura ELS: lesion is located outside the lung and has its own visceral pleura Rare Comprises 1-6% of all congenital pulmonary malformations Associated Congenital Anomalies Diagnosis Often Prenatal May Regress Postnatal diagnosis and presentation varies Treatment Presentation Dependent ELS Serial Monitoring ILS Resection recommended to prevent infections 2

3 Congenital Lobar Emphysema (CLE) Developmental anomaly of lower respiratory tract characterized by hyperinflation of one or more pulmonary lobes Incidence 1: :30000 Male predominance Pathogenesis Intrinsic or extrinsic (most common) Diagnosis Some diagnosis by prenatal ultrasound May regress on prenatal ultrasound Presentation of wheezing and respiratory distress One third symptomatic at birth and almost all children are symptomatic by 6 months Often diagnosed via postnatal CXR Treatment Varies by presentation Resection in symptomatic patients Conservative management for asymptomatic patients No risk for malignancy Congenital Lung Lesions: Preoperative Evaluation Elizabeth Paton, MSN, RN-BC, PPCNP-BC PREOPERATIVE EVALUATION Timing of postnatal follow up Recommended imaging studies Timing for surgical intervention Tips for counselling family 3

4 CLINICAL EVALUATION Evaluation of the infant in the newborn nursery Evaluation after discharge from hospital Recommendations for frequency and timing of follow up visits IMAGING STUDIES CXR CT scan Ultrasound INITIAL VISIT: 1 MONTH OF AGE 4

5 2 ND FOLLOW UP VISIT: 3.5 MONTHS OF AGE 3 RD FOLLOW UP VISIT: 5 MONTHS OF AGE ANOTHER SCENARIO.. 5

6 INITIAL VISIT: 4 WEEKS OF AGE CT SCAN SO, WHEN TO REPAIR? Best to operate when healthy and asymptomatic! If symptomatic- repair sooner If asymptomatic- repair later, but not too much later Best for patient to repair before symptomatic 6

7 FAMILY COUNSELLING TIPS Why surgical intervention is recommended Risks Of immediate surgery Of waiting Of no surgery at all Recovery- to be discussed at another session Congenital Lung Lesions: Intraoperative Care Christine Schultz, MSN, RN, CPNP INTRAOPERATIVE CARE THORACOTOMY Past: Large incision in the chest to remove part/all of a lobe of lung Risks include bleeding, pain, infection, chest wall deformity Present: Muscle-sparing THORACOSCOPY Used in pediatrics since about 1976; growth since 1985 Advantages: cosmetic (small incisions/scars),reduced pain, decrease in infection and dehiscence, superior visibility & precision Disadvantages: longer operating time,lack of three-dimensional vision, reduced feedback from tactile sensation, prolonged learning curve 7

8 INTRAOPERATIVE CARE Congenital Lung Lesions: Postoperative Care Ronelle Caskey, MSN, PNP 8

9 PACU CXR Stable: Transfer to floor Post-operative Nursing Assessment Pain Management- Pain Ladder (tylenol RTC, prn morphine, morphine drip), medicate prior to CT removal ADAT Respiratory Assessment- Rate, work of breathing, auscultate breath sounds, oxygen saturation, Chest tube- Unclamped, dressing intact, CT drainage (amount and consistency), fluctuation in water seal chamber with respirations, suction set to 20 cm water In 1967 we moved from bottles to a box 9

10 POD 1: Patient placed to water seal Bubbling visualized in water seal chamber, mostly with cough Assess for air leak (from lung or system) Clamp CT by patient (if bubbling persists, then system leak) Place pressure over dressing (if bubbling stops, replace occlusive dressing, if bubbling continues possible leak from lung) Obtain chest xray Pneumothorax What next? Place back to suction hours Retrial a water seal challenge Reimage If lung remains up and chest tube drainage is less than 2ml/kg/8 hours, remove CT 10

11 Chest Tube Removal Confirm no air leak and pre-medicate Prepare new occlusive dressing Remove anchoring suture (except U stitch if used) Apply pressure above the CT site with vaseline gauze, sterile 2x2 and tegaderm Remove tube when lungs fully expanded (older child can hold breath while performing the valsalva maneuver. Infants pull quickly with occlusive dressing on site as tube exits Xray 4 hours after CT removal Occlusive dressing may be removed after 48 hours Congenital Lung Lesions: Long Term Follow-Up Maura O Day, MS, RN, CPNP, CWON Long Term Follow Up Long term follow up for congenital lung lesions What can we gain from long-term follow up? Timing of follow up and duration? Structure of visit? 11

12 Long Term Follow Up Outcomes excellent survival rates documented for most congenital lung lesions, particularly those without presence of hydrops prenatally Potential associated symptoms/health issues: Lung hypoplasia Pneumonia Asthma/Reactive airway Long Term Follow Up Experience + long-term follow up allows clinicians to: Collect clinical data Assess for any potential associated symptoms Provide more comprehensive prenatal and postnatal counseling for families on expected outcomes Improve our overall care of these patients in the future How many of your centers curently offer long-term follow up? 12

13 References Browne, N, Flanigan, L, McComiskey, C, Pieper, P. Nursing care of the Pediatric Surgical Patient. 3 rd ed. Burlington, MA: Jones & Bartlett Chandran-Mahaldar, D., Kumar, S., Balamurugan, K., Raghuram, A. R., Krishnan, R., & Kannan (2009). Congenital lobar emphysema. Indian Journal of Anaesthesia, 53(4), Retrieved from Congenital Pulmonary Airway Malformation (CPAM) (2016). Paper presented at the Cardinal Glennon St. Louis Fetal care Institute, St. Louis, IL. Durai, R, Hoque, H, Davies, TW. Managing a chest tube and drainage system. AORN Journal. 2010; 91(2): Durell, J., Thakkar, H., Gould, S., Fowler, D., & Lakoo, K. (2016). Pathology of asymptomatic, prenatally diagnosed lung malformations. Journal of Pediatric Surgery, 51(), References Fievet, L., Natale, C., D Journo, X., Coze, S., Dubus, J., Guys, J.,... De Lagausie, P. (2015, April- June). Congenital pulmonary airway malformation and sequestration: two standpoints for a single condition. Journal of Minimal Access Surgery, 11(2), Kapralik, J., Wayne, C., Chan, E., & Nast, A. (2015). Surgical versus conservative management of congenital pulmonary airway malformation in children: a systematic review and meta-analysis. Journal of Pediatric Surgery. Shah, R., Reddy, A. S., & Dhends, N. P. (2007, October-December). Video assisted thoracic surgery in children. Journal of Minimally Invasive Surgery, 3(4), Stone, A. E. (2015). Cystic adenomatoid malformation. Retrieved March 29, 2016, from References Sfakianaki, A. K., & Copel, J. A. (2012). Congenital Cystic Lesions of the Lung: Congenital Cystic Adenomatoid Malformation and Bronchopulmonary Sequestration. Reviews in Obstetrics and Gynecology, 5(2), Waldhausen, J, Cusick, R, Graham, D, Pittinger, Sawin, R. Removal of Chest Tubes in Children without water seal after elective thoracic procedures: A Randomized Prospective Study. American College of Surgeons. 2002; 194(4) 13