156 51 1 2015 2 3 6 1 1) 2) 3) 1) 1) 1) 1) 1) 2) 3) 3) 3 1 4 6 2 3 4 Rocky Mountain Soap Rocky Mountain Soap RMS Canmore Banff Pure hand-made 100 toxi

51 1 2015 2 pp. 155-173 155 25 QOL 26 10 18 1993 2000 23 25 3 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 25 4 26 3 3 A

156 51 1 2015 2 3 6 1 1) 2) 3) 1) 1) 1) 1) 1) 2) 3) 3) 3 1 4 6 2 3 4 Rocky Mountain Soap Rocky Mountain Soap RMS Canmore Banff Pure hand-made 100 toxin free PAPS 10 CIIPS 21 5 1 3 C phallic urethra VSD VACTER 2 3 36 mm 0 6 1 8 26 0 898 g 2 3 prolapse prolapse 2 prolapse 9 prolapse QOL 7 1 1) 2) 1) 1) 1) 2) 21 1 2 CIC 20 19 MACE WOC QOL

51 1 2015 2 8 in 2 1) 2) 3) 1) 2) 3) 20 1 7 in 157 10CISC 8 CISC CISC CISC 9 VUR QOL VUR 22 QOL 1 VUR Grade V 2 1 1 6 VUR Henderen 9 20 11 9 7 19.1 3 pull-through 4 2 7 11 12 4 2 1 1 2 MRI 12 1 19

158 51 1 2015 2 9 12 18 2 QOL 13 QOL 1991 48 QOL 1991 2014 5 1 1 23 3 VUR 4 1 VUR 1 1 3 CIC 100 300 ml 6 5 ST 4 CIC QOL 1 14 3 1) 2) 3) 1) 1) 1) 2) 3) VACTERL 1 1 ICU 60 1 5 15 18 trisomy 1 18 trisomy 0 10 1 4 1 6 QOL 16 5

51 1 2015 2 17 6 1) 2) 1) 1) 1) 1) 1) 1) 1) 2) 2) 2) 2) 24 1 18 QOL QOL 2010 12 2014 7 10 105 3 4 159 19 QOL 1) 2) 3) 1) 1)2) 3) 3) 3) 3) 3) QOL 2012 廔 QOL 1 1 1 2 20

160 51 1 2015 2 2 5 1) 2) 1) 1) 1) 1) 2) QOL 2 4 1 1) 2) 3) 4) 1) 2) 2) 1) 3) 4) 4) 4) 2) 2) 1 5 1 40 cm 3 1 5 6 2 3 1 3 ICU ICU 1) 2) 3) 1) 2) 3) 2013 4 2014 3 5 GER 2012 2012 16 1 1 16 9 7 GER 5 2 1 QOL GER

51 1 2015 2 161 6 8 1) 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 1 2 3 CLS CLS 7 1 1) 9 2) 1) 2) 3) 3) 4) 1) 2) 2) 3) 1) 2) 3) 4) 1 7 5 PRETEXT 5 2 5 6 1 CT 13 10 8 cm 4 7 CITA 2 Day 10 1 1 1 3 8 CITA 3 6.7 7.6 8.0 cm CITA 4 on hand position 10 3 1 14 QOL

162 51 1 2015 2 10 1) 2) 3) 1) 2) 3) 2) 2) 2) 2) 1 W 0.02 7 W 0.02 W 0.05 0.02 W 0.05 11 2013 4 2014 3 99 MSW 98 12 1) 2) 1) 1) 1) 2) 2) 13 Nuss 12 1) 2) 3) 4) 5) 1) 1) 2) 3) 3) 3) 4) 5) 5) Nuss 12 24 8 26 3 A 42 3 10 6 50 3 40 6 100 3 6 3 QOL 14 Nuss 1) 2) 3) 1) 2) 3) Nuss QOL A 2013 1 2 70

51 1 2015 2 163 32 41 15 20 AED Nuss QOL 15 QOL QOL 2003 7 2 4 QOL 7 0 6 3 6 1 4 3 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 3 1 17 VP 1 1) 2) 3) 1) 1) 1) 2) 3) 11 16 VP 9 7 VP 3 QOL 1 2 2 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 4 7 39 13 1 20 5 6.5 20 cm 11 cm Cobb 0 113 28 A 50.2 10.6 mm LTS 40.8 11.4 mm SCL 58.1 11.8 mm SCL LTS B 16.0 5.08 mm LTS 17.1 6.36 mm SCL 16.0 0.74 mm C 29.6 12.0 mm LTS 38.4 9.85 mm SCL 27.0 4.57 mm

164 51 1 2015 2 19 2009 12 2013 12 6 5 1 2 4 5 2 10 1 1 3.85 kg 12.24 kg 7.77 kg 1 5 QOL 21 1 20 6 14 gastrojejunostomy tube QOL 1 20 1) 2) 1) 2) 2) 2) 5 3 8 1 3 4 1 2 4 19 1 3 4 3 10 7 1 2 1 1 4 5 1 3 3 3 4 2 2 1 2 1 1 5 13 1 4 9 3 4 4 8 1 2 3 11 24 25 10 25 2F 2 2-4 1 1 Olney 15 6 6 3 2 1 1 1 5 2 10 3 5 2 12 11 3 1 15 12

51 1 2015 2 2 budesonide budesonide budesonide b 2007 4 2013 3 1 27 b 6 Cotton-Myer grade II or III 2 3 1 b 1 b 3 1 6 2 10 4 3 8 1 cm 2 8 2 3 2.5 5 1 1 1 cm 2 1 1.5 2 6 165 4 Re-do surgery 6 28 14 T- 9 64 T- 12 60 T- 1 5 3 9 29w3d 1,027 g 0 1 2 4 4 CT 5 mm 11 1

166 51 1 2015 2 2 6 WPC 1 1) 2) 3) 1) 1) 1) 2) 2) 3) PIE PIE WPC 1 23 2 496 g Apgar score 3/6 4 PIE PIE 29 X Allows Wedge Pressure Catheter 4Fr WPC 1 10 24 PIE 33 WPC 4 35 WPC PIE PIE WPC 7 17 CCAM 2000 1 2013 7 CCAM 17 11 6 6 11 3 14 9 2 8 1 6 3 1 6 2 4 6 4 1 8 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 9 12 12 24 21 36 39.3 37.86 40.71 3,223 g 2,190 3,684 g CCAM 10 1 2 7 0 26 5.0 cm 3.5 6.0 cm 13.5 8 5 CCAM 1 reduction surgery 3 SSI 10 redo surgery 4 23 CCAM type 1 38 5 CT

51 1 2015 2 6 cm CT 3 9 4 CT 5 26 12 4 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. 167 3 11bronchopulmonary foregut malformation: BPFM 1 7 38 5 2,368 g CT 7 tracheal bronchus complete ring BPFM 12 BPFM 1 BPFM 1 6 38 4 2,432 g 6 BPFM 2 4 6 CT 11 8 BPFM 13 1

168 51 1 2015 2 11 a fetoprotein AFP 14,000 ng/ml BEP 1 AFP 5 2 AFP 14 Reccurent respiratory papillomatosis RRP 1 8 1 3 human papilloma virus HPV 11 1 9 1 2 RRP HLA 15 1 1) 2) 3) 1) 1) 2) 3) 5 X CT MRI 7 cm CT MRI 4 8 7 6 cm 130 g MIB-1 1 3 16 1 1) 1) 1) 1) 1) 1) 1) 1) 2) 1 11 3 X CT 7 cm 3 37 4 17 1991 2012 23 12 11 0 10 1 22 CT 12 2 1 CT

51 1 2015 2 169 18 CDH 700 1997 10 3 CDH 31 75 1997 2003 n 22 2004 2005 n 10 2006 2013 n 43 1 3 63 90 85 21 28 22 17 1 35 44 17 5 23 19 MRI 39 1 BW 3,303 g Apger 8/9 NICU 21 Fontan 98 2008 5 13 1 4 HLHS 7 1 DORV PS 1 8 5 6 4 22 1 2.25 1.55 u:m 2 Fontan 2 u:m 2 25 20 Nuss CCAM 26 Nuss 39 4 3,600 g CT CCAM 26 CCAM

170 51 1 2015 2 8 23 1989 1 2013 3 65 2 34 19 15 2 3 14 2 1 3 31 3 7 21 1 13 2 1 13 5 65 26 19 11 6 24 CCAM 2008 CCAM 1 2008 1 2013 6 CCAM 8 1 9 1 6 CT CCAM 10 1 5 1 2 7.65 10.3 kg 9.26 kg 1 1 1 4 1 25 1) 2) 3) 4) 1) 1) 1) 1) 1) 1) 1) 2) 2) 3) 3) 3) 4) 4) 1) 1) 2002 20 15 5 3 17 18 90 22 32 25.5 3 5 9 45 6 30 1 3 15 2 9 122 7 11 5 9 4 10 2 26 CPAM 1 1) 2) 1) 1) 2) 1) 10 21 30 MRI CPAM 41 3 3,624 g Apgar score 8/9 CT 12 mm 6 mm CT 25 mm 18 mm CPAM Type I 10 S9/S10

51 1 2015 2 171 CPAM 29 CCAM 2 Hybrid type? 27 2 volume reduction 1 CCAM 2 Hybrid type 0 34 1 2,836 g 1 10 X-p CT CCAM 9 CT CO 2 CT 10 30 S6 63 2 2 CO 2 133 1 CT 2 11 1 10 CPAP 30 1) 2) 3) 28 2 4) 5) 1) 2) 6) 3) 1) 2) 3) 4) 3) 1) 1) 2) 2) 3) 5) 6) 1) 3) 1) CCLD 1 22 40 4 CCLD 91 CCAM BA 2 30 18 10/9 3 32 CCAM type 1, 2, 3 38 4 32 2 2 4 3 BA CCAM type 2 2 n-cpap 13 9 CCAM CT type 1 type 2 BA 2 18

172 51 1 2015 2 31 1) 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 2002 4 2013 6 52 CCAM 2 6/6 CCAM 1 2/6 CCAM 1 2 1/2 BA 24 BA 3 32 1 13 4 cm 33 ganglioneuroma 10 1) 2) 1) 1) 2) 10 CT 50 mm 3 3 mm 12 mm 3 ganglioneuroma 34 variability 5 7 2 1 1 1 5 2 2 21 12 g 1 35 VATS 1 8 VATS

51 1 2015 2 173 lost follow 7 6 3 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 7 36 1 1) 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 4 3 37 2 1) 2) 1) 1) 1) 1) 2) 2) 2) 1) 1 1 6 BCG 1 2 CT BCG Tokyo 172 2 43 MRSA MRSA MRSA 2