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A case of congenital central hypoventilation syndrome
undergoing implantation of phrenic nerve stimulation for diaphragm pacing
Keiko Hirooka, M.D.1, Kotoe Kamata, M.D., Ph.D. 1, Kumi Ono, M.D. 1, Asami Ohashi, M.D. 1, Shiro Horisawa, M.D. 2, Takaomi Taira, M.D., Ph.D. 2, Makoto Ozaki, M.D., Ph.D. 1
Tokyo Women's Medical University, Tokyo, Japan 1. Department of Anesthesiology 2. Department of Neurosurgery
 Postoperative course
Introduction
① Congenital central hypoventilation syndrome (CCHS) is characterized by the lack of adequate autonomic control of respiration with
decreased sensitivity to hypercapnia and hypoxia in the absence of neuromuscular or lung disease or an identifiable brainstem lesion.
Recently, CCHS is considered as a variant of generalized autonomic nervous system dysregulation. Thus, complications occurring in
various organ systems have impacts, despite respiratory dysfunction being pathognomonic of CCHS.
② Patients with CCHS require lifetime ventilation support to avoid pulmonary hypertension and cor pulmonale. Diaphragm pacing improves
quality of life and extend survival in patients with advanced respiratory muscle weakness.
③ The prevalence of late-onset CCHS (LO-CCHS) that is applied to patients who are diagnosed with CCHS after 28 days of age is unknown.
Although, the estimated prevalence of CCHS is one case per 200,000 live births.
 Patient; 5-year-old girl, Ht 103 cm, Wt 17 kg, ASA-PS 3
Perioperative strategies
Born at 40 wks gestation (no fetal distress)
Age 2
Behavior disorders, Repeated respiratory infection
Introduce nocturnal ventilation support
Mental retardation following hypoxic encephalopathy
Age 4
Tracheotomy for chronic home mechanical ventilation
Age 5
Diagnosed with LO-CCHS
Potential mitochondrial dysfunction
(lactic acidosis, 3-hydroxy-3-methylglutaric acidemia)
1. Maintain adequate respiration;
• Keep EtCO2 level within her usual range (>45 mmHg)
• Avoid intraoperative cardiac instability
[no propofol, use sevoflurane]
• Minimize postoperative respiratory depression
2. Avoid developing lactic acidosis;
• Use lactate-free solution, avoid hypoglycemia
[acetate Ringer with 5% dextrose] [no propofol use]
Implantation of phrenic nerve stimulation for diaphragm pacing
(with IRB approval & parental consent)
 Anesthesia
HR [bpm]
BP [mmHg]
【Symbols】
X ; Anesthesia (start/end)
T ; Intubation, Extubation
◎ ; Operation(start/end)
240
SpO2 [%] 100
8
0.3
0.5
0.4 0.3 0.25
6 0.7
0.2
160
92 -
120
88 -
80
84 -
40
80 76 9:00
X
T
10:00
1.0 0.4
100
53
44
190
11:00
◎
12:00
97
48
99
45
44
0.4
192
13:00
42
3. Require surgical site protection;
• Provide postoperative conscious sedation & analgesia
[dexmedetomidine, morphine]
//
//
//
• Premedication; none
• Anesthesia; Sevoflurane was used for induction and maintenance
• Respiratory management; A tracheotomy tube was replaced with
an endotracheal tube to facilitate surgical intervention
-
96 -
FiO2
SpO2 [%]
EtCO2 [mmHg]
Blood glucose [mg/dL]
2.6
17
200
0
5
Intravenous
Suppository
POD 1
POD 2
POD 3
-2 ~ -1
-2 ~ +1
-1 ~ +2
-2 ~ +1
Dexmedetomidine (µg/kg/hr) 0.4 --------------------------- 0.6 -- 1.0 ------- 0.7 -------- 0.5 ------------ 0.4 ------------------------//
Morphine (µg/kg/min)
Phenobarbital (mg/day)
Acetaminophen (mg/day)
0.1 ------------------------------------------------ 0.5 ----------------------//
↓ 60
↓ 60
↓ 600
↓ 600
Mechanical ventilation (FiO2 0.4)
Case presentation
EtSevo [%]
Remifentanil [µg/kg/min]
Dexmedetomidine [µg/kg/hr]
0.3% Ropivacaine [mL]
Richmond Agitation-Sedation Scale
Medication
POD 0
14:00
◎
100
45
46
181
T
X
//
45//
• Fluid balance;
Acetate Ringer solution containing 5% dextrose 1323 mL
Urine output 900 mL
Blood loss 5 mL
• Anesthesia time; 305 min
Operative time; 228 min
Trilogy200Plus
Puritan Bennett 760 (Medotronic)
VCV
↓ 60
↓ 600
(Philips Respironics)
SIMV
SIMV + PS
(same as pre-Op setting)
TV 70-90 mL
IP 14 cmH2O
→
TV 160 mL
PEEP 4 cmH2O
PEEP 4 cmH2O
→
PEEP 5 cmH2O
RR 10/min
RR 10/min
→
PS 6 cmH2O
PS 6 cmH2O
→
7.38
7.47
102 / 66.4
104
12/min
Inspiratory time 0.8s
Arterial blood gas
pH 7.43
PaO2 / PaCO2 136 / 56.2
Discussion
Perioperative management of CCHS should be modified based on coexisting diseases.
 Anesthesia
• Avoid propofol use, which is contraindicated in CCHS patients
• Minimize postoperative respiratory depression by using short-acting agents and infiltration analgesia
• Keep EtCO2 level within her usual range (over 45 mmHg)
• Lactate-free infusion containing 5% dextrose to avoid hypoglycemia and lactic acidosis
 Postoperative management
• Dexmedetomidine and morphine co-administration provided conscious sedation and adequate analgesia to
avoid respiratory depression or tracheostomy dislodgement
• Intravenous medications were replaced by suppositories
Conclusion
CCHS involves profound perioperative concerns.
Respiratory condition as well as systemic comorbidities should be appreciated before anesthesia.