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1 pp QOL IT 10 2 end of life living will living will living will 1 NICU QOL NICU QOL 3 6 kg NICU 42 2 kg 2007 NICU 2 NICU/ GCU A

2 ) 2) 3) 1) 1) 1) 1) 1) 2) 3) 3) Rocky Mountain Soap Rocky Mountain Soap RMS Canmore Banff Pure hand-made 100 toxin free PAPS 10 CIIPS C phallic urethra VSD VACTER mm g 2 3 prolapse prolapse 2 prolapse 9 prolapse QOL 7 1 1) 2) 1) 1) 1) 2) CIC MACE WOC QOL

3 in 2 1) 2) 3) 1) 2) 3) in CISC 8 CISC CISC CISC 9 VUR QOL VUR 22 QOL 1 VUR Grade V VUR Henderen pull-through MRI

4 QOL 13 QOL QOL VUR 4 1 VUR CIC ml 6 5 ST 4 CIC QOL ) 2) 3) 1) 1) 1) 2) 3) VACTERL 1 1 ICU trisomy 1 18 trisomy QOL 16 5

5 ) 2) 1) 1) 1) 1) 1) 1) 1) 2) 2) 2) 2) QOL QOL QOL 1) 2) 3) 1) 1)2) 3) 3) 3) 3) 3) QOL 2012 廔 QOL

6 ) 2) 1) 1) 1) 1) 2) QOL ) 2) 3) 4) 1) 2) 2) 1) 3) 4) 4) 4) 2) 2) cm ICU ICU 1) 2) 3) 1) 2) 3) GER GER QOL GER

7 ) 2) 3) 4) 1) 1) 2) 3) 1) 2) 3) 1) 1) 1) 1) 1) 1) 2) 3) 3) 1) 1) 4) 3) 2012 CLS CLS CLS CLS 7 1 1) 9 2) 1) 2) 3) 3) 4) 1) 2) 2) 3) 1) 2) 3) 4) PRETEXT CT cm 4 7 CITA 2 Day CITA cm CITA 4 on hand position QOL

8 ) 2) 3) 1) 2) 3) 2) 2) 2) 2) 1 W W 0.02 W W MSW ) 2) 1) 1) 1) 2) 2) 13 Nuss 12 1) 2) 3) 4) 5) 1) 1) 2) 3) 3) 3) 4) 5) 5) Nuss A QOL 14 Nuss 1) 2) 3) 1) 2) 3) Nuss QOL A

9 AED Nuss QOL 15 QOL QOL QOL QOL 57 QOL 16 STEP 1) 2) 1) 1) 1) 2) STEP serial transverse enteroplasty ss37w2d 3,091 g 1 2 cm 45 cm 2 47 cm 18Fr. 5 1 STEP VP 1 1) 2) 3) 1) 1) 1) 2) 3) VP 9 7 VP 3 QOL VP 18 CT 1) 2) 3) 1) 2) 3) 2) 2) 2) 3) 2) QOL TIF TIF 20 A B C CT Cobb 9 LTS 7 SCL cm 11 cm Cobb A mm LTS mm SCL mm SCL LTS B mm LTS mm SCL mm C mm LTS mm SCL mm

10 kg kg 7.77 kg 1 5 QOL gastrojejunostomy tube QOL ) 2) 1) 2) 2) 2) F Olney

11 budesonide budesonide budesonide b b 6 Cotton-Myer grade II or III b 1 b cm cm Re-do surgery T T T w3d 1,027 g CT 5 mm 11 1

12 WPC 1 1) 2) 3) 1) 1) 1) 2) 2) 3) PIE PIE WPC g Apgar score 3/6 4 PIE PIE 29 X Allows Wedge Pressure Catheter 4Fr WPC PIE 33 WPC 4 35 WPC PIE PIE WPC 7 17 CCAM CCAM CCAM CCAM BA CCAM 10 CCAM 21 CCAM BA CT mucoid impaction 21 4 BA BA 3/4 CCAM 2/17 mucoid impaction BA 3/4 CCAM 1/17 BA 4/4 CCAM 5/17 BA 3/3 CCAM 6/9 BA CT BA ,223 g 2,190 3,684 g CCAM cm cm CCAM 1 reduction surgery 3 SSI 10 redo surgery 4 23 CCAM type CT

13 cm CT CT Congenital central hypoventilation syndrome (CCHS): Clinical aspects, genetics and surgical management with thoracoscopic placement of diaphragm pacing electrodes Regional Chief of Pediatric Surgery, Southern California Kaiser Permanente Medical Group Donald Brian Shaul Congenital Central Hypoventilation Syndrome (CCHS), commonly known as Ondine s Curse, is a clinical syndrome characterized by failure of spontaneous ventilation. It is usually diagnosed in infancy although milder cases can be diagnosed later in life. The syndrome is associated with other autonomic nervous system dysregulations (ANSD) as well as Hirschsprung s Disease. The diagnosis is generally made when hypoventilation is noted in patients who do not respond normally to hypoxemia and hypercarbia. The gene responsible for this syndrome is the PHOX2B gene. Late Onset CCHS (LO-CCHS) is caused by a less severe mutation of the gene. Initial management of the hypoventilation is by positive pressure ventilation usually administered via tracheostomy. Older patients can be managed by thoracoscopic placement of phrenic nerve electrodes allowing ventilation via direct stimulation of the diaphragm. Over a 15 year period, 22 patients in a single institution have undergone successful placement of bilateral diaphragm pacing electrodes with the subsequent establishment of ventilation in all. 14 of these patients required ventilation during sleep only and in 8 of these patients the tracheostomy was successfully removed. Technical refinements to pediatric thoracoscopic instruments and diaphragm pacing equipment, as well as increased knowledge of the associated nervous system dysregulation, have allowed for sophisticated procedures to be safely performed in pediatric patients with CCHS bronchopulmonary foregut malformation: BPFM ,368 g CT 7 tracheal bronchus complete ring BPFM 12 BPFM 1 BPFM ,432 g 6 BPFM CT 11 8 BPFM 13 1

14 a fetoprotein AFP 14,000 ng/ml BEP 1 AFP 5 2 AFP 14 Reccurent respiratory papillomatosis RRP human papilloma virus HPV RRP HLA ) 2) 3) 1) 1) 2) 3) 5 X CT MRI 7 cm CT MRI cm 130 g MIB ) 1) 1) 1) 1) 1) 1) 1) 2) X CT 7 cm CT CT

15 CDH CDH n n n MRI 39 1 BW 3,303 g Apger 8/9 NICU 21 Fontan HLHS 7 1 DORV PS u:m 2 Fontan 2 u:m Nuss CCAM 26 Nuss ,600 g CT CCAM 26 CCAM

16 CCAM 2008 CCAM CCAM CT CCAM kg 9.26 kg ) 2) 3) 4) 1) 1) 1) 1) 1) 1) 1) 2) 2) 3) 3) 3) 4) 4) 1) 1) CPAM 1 1) 2) 1) 1) 2) 1) MRI CPAM ,624 g Apgar score 8/9 CT 12 mm 6 mm CT 25 mm 18 mm CPAM Type I 10 S9/S10

17 CPAM 29 CCAM 2 Hybrid type? 27 2 volume reduction 1 CCAM 2 Hybrid type ,836 g 1 10 X-p CT CCAM 9 CT CO 2 CT S CO CT CPAP 30 1) 2) 3) ) 5) 1) 2) 6) 3) 1) 2) 3) 4) 3) 1) 1) 2) 2) 3) 5) 6) 1) 3) 1) CCLD CCLD 91 CCAM BA / CCAM type 1, 2, BA CCAM type 2 2 n-cpap 13 9 CCAM CT type 1 type 2 BA 2 18

18 ) 2) 3) 1) 2) 2) 2) 2) 2) 2) 2) 2) 2) 2) 2) 3) 3) BA US CT BF BA BA BA BA BF BA BA BA CCAM 2 6/6 CCAM 1 2/6 CCAM 1 2 1/2 BA 24 BA cm 33 ganglioneuroma 10 1) 2) 1) 1) 2) 10 CT 50 mm 3 3 mm 12 mm 3 ganglioneuroma 34 variability g 1 35 VATS 1 8 VATS

19 lost follow cm wound protector EZ access 5 mm port 5 mm 3 4 mmhg A7 A8 A9 10 HS EZ access end-gia V6 HS VATS S6 HS S6 coating ) 2) 1) 1) 1) 1) 1) 2) 2) 1) 11 7 kg 1 BP 116/72 mmhg HR 70 / SpO2 99 room air air X ) 2) 1) 1) 1) 1) 2) 2) 2) 1) BCG 1 2 CT BCG Tokyo MRSA MRSA MRSA 2

QOL 02 QOL QOL UI AI EAThoracotomy TTThoracoscopy TS QOL EA type-c y score UI 3 AI 8 School score QOL score 12

QOL 02 QOL QOL UI AI EAThoracotomy TTThoracoscopy TS QOL EA type-c y score UI 3 AI 8 School score QOL score 12 54 1 2018 2 4-1 OK-432 1 15 14 15 US CT MRI 17 cm 0.16 g/kg/day OK-432 3 1 ke 2 2 ke 3 1 1,150 ml 13 US OK-432 4-2 TJ-28 TJ-28 2003 3 2017 6 250 TJ-28 44 16 28 TJ-28 7 2 46 8.5 2 2 9 34 79.1 10 23.3 34

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