Combined meeting 2015/7/2 R2 潘妤玟

Patient Informations Name:蔡X祺 Age: 3 month 16 day old Gender: Male Admission Date: 2015/3/16 Chief Problem: Respiratory distress after birth

Present Illness Maternal history: Birth history: Prenatal care at 婦兒安 and 新樓: unremarkable. Birth history: G3P1A2, GA 36+5 weeks BBW: 2878g Apgar score: 7->9 FHB poor variation at 新樓H. Emergent C/S then Delay of initial crying s/p ambu bagging Poor activity admitted to NICU+NCPAP Blood gas: pH 7.188, CO2 59.7, HCO3 22.2, BE -7 Intubation done and transferred to our NICU

Physical Examination T/P/R: 36.8/163/44, BP: 70/47mmHg General appearance: Pink appearance, fair activity HEENT: Conjunctiva: not pale Sclera: anicteric Neck: supple, No JVE, no LAP Chest: Symmetric expansion, retraction(+) Slightly decreased right lower BS, no bowel sound could be heard Skin: no edema, petechiae or ecchymosis Heart: regular heart beat, no murmur Abdomen: flat and soft, normoactive bowel sound Tenderness (-) Rebound tenderness (-) Muscle guarding (-) Flank pain(-) Extremities: warm, pitting edema (-)

Hospital course 2015/3/17 1033: 2015/3/17-3/18 2015/3/17 1033: Echo: liver parenchyma in right thoracic cavity Consult CS arrange surgery 2015/3/17-3/18 Aggitation and fighting ventilator desaturation Sedation Cardiac echo: PPHN、TR iNO but poor response Suspect Right& left pul. artery hypoplasia FiO2 65% pH=7.204 ，pCO2=62.8，HCO3-24.2 ，BE=-3.8 2015/3/17 1033: Echo: liver parenchyma in right thoracic cavity Cardiac echo: PDA R->L shunt FiO2 35% Consult CS arrange surgery 2015/3/17 2030: Frequent bradycardia CXR: no pneumothorax ↑Stroke volume 2015/3/18 Aggitation and fighting ventilator desaturation Sedation Cardiac echo: PPHN、TR iNO but poor response Right& left pul. artery atrophy FiO2 65% pH=7.204 ，pCO2=62.8，HCO3-24.2 ，BE=-3.8 Adjust stroke volume

Hospital course 2015/3/19 surgery: Right side diaphragm eventration without diaphragm herniation Perform diaphragm plication

Admission course 2015/3/21 HFOV shift to IMV mode 3/20, 3/21, 3/23

Admission course 2015/3/26 Extubation + NIV NAVA support Diaphragm elevation gradually 3/26, 3/29, 4/3

Hospital course 4/3 Tachypnea + sputum 4/5 frequent ABD  Intubation Consult CS surgeon for re-eventration 4/5 frequent ABD  Intubation (NIV NAVA x 9 days)

Hospital course 4/11 Sputum culture Antibiotics Unasyn use Klebsiella pneumoniae Antibiotics Unasyn use Blood culture: negative 4/11 Fever + Frequent ABD CRP: 25.666.989.8 Blood culture: Shift to Fortum use

Hospital course 4/10 chest CT 4/16 Extubation  NIV NAVA support hypoplasia of RML, RLL PDA 4/16 Extubation  NIV NAVA support

Hospital course 5/4 Intubation due to CO2 retention Apnea/bradycardia/desaturation when agitation Chlohydrate prn use for sedation

Hospital course 6/12 Extubation  NIV NAVA support Cardiac echo: BiPAP tried but failed Still CO2 retention Cardiac echo:  Add Viagra 6/24 Intubation

Lung perfusion scan Diffusely lower lung perfusion in the right lung

Hospital course 3/16 Admission 3/18 Diaphragm plication 3/26 Extubation 4/5 Intubation due to frequent ABD CXR: Re-eventration + RUL collaspe 4/10 Chest CT 4/11 K.P. Bacteremia 4/16 Extubation FiO2 around 25-30%, CO2 around 45-55mmHg FiO2 around 30-40%, CO2 around 60-70mmHg

Hospital course 5/4 Intubation due to progressive CO2↑ ABD while agitation, bagging prn 6/3 Lung perfusion scan 6/12 Extubation Cardiac echo: pulmonary hypertension Add Viagra 6/24 Intubation FiO2 around 40-50%, CO2 around 70-80mmHg Currently, FiO2 around 50-60%, CO2 around 70-80mmHg

Next step? Surgery again? Lung hypoplasia? Tracheostomy? Younger children (under 1 year of age) had a higher complication rate than older cases. Lung transplantation? Complications Early Emphysema Pneumomediastinum pneumothorax Wound complications Bleeding Late Granulations/laryngeal stenosis/tracheal stenosis

Prognosis Severe pulmonary hypoplasia was a major contributing factor to mortality in this series. Pulmonary hypoplasia similar to that associated with diaphragmatic hernias (CDH) also occurs in CDE, albeit more rarely and to a lesser degree [4, 12, 17].

Thank you!