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Nocturnal dyspnea treated by diaphragm pacing
232
Surg Neurol 1983;19:232-6
Nocturnal Dyspnea Treated by Diaphragm Pacing Tomio Sasaki, Hirotake Nakano, Takao Asano, Shinya Manaka, Kintomo Takakura, Haruhiko Tsutsumi, Hidenori Toyooka, and Ikuo Satoh The Departments of Neurosurgery and Emergency Medicine, University of Tokyo Hospital, Tokyo, Japan and the Satoh Clinic, Ube City, YamaguchiJapan
Sasaki T, Nakano H, Asano T, Manaka S, Takakura K, Tsutsumi H, Toyooka H, Satoh I. Nocturnal dyspnea treated by diaphragm pacing. Surg Neurol 232-6.
A case of nocturnal dyspnea secondary to atlantoaxial dislocation is reported. Noctural dyspnea was not alleviated by a decompressive laminectomy of C-1 and a half of C2 vertebrae made by a transoral pharyngeal approach. Diaphragm pacing at night was effective for the longterm management of nocturnal dyspnea. KEY WORDS: Atlantoaxial dislocation; Central hypoventilation; Diaphragm pacing; Phrenic nerve; Dyspnea, nocturnal
Noctural dyspnea is uncommonly encountered in patients with upper cervical lesions of the spinal cord [2,11,12,15,20]. In such cases, positive-pressure respirators are usually employed to assist respiration at night. However, long-term application of such mechanical respirators may lead to various complications including infectious diseases and pulmonary fibrosis. Electrical stimulation of the phrenic nerve (diaphragm pacing) is an attractive method for prolonged ventilatory assistance because it overcomes several physiological drawbacks of positive-pressure respirators [1,3,13,21,23]. Several authors have recently reported that diaphragm pacing was successfully applied in the long-term management of ventilatory insufficiency of various origins, i.e., encephalitis, brain-stem infarction, posterior fossa tumor, the pickwickian syndrome, ventilatory insufficiency due to cordotomy, bulbar poliomyelitis, traumatic injury of the high cervical spinal cord, and spinal muscular atrophy [3-6,8,14,16,17,20,22]. In this paper, we present a case of nocturnal dyspnea secondary to atlantoaxial dislocation, which was not relieved by decompression of the spinal cord with laminectomy of C-1 and a half of C-2 combined with partial Address reprint requests to: Tomio Sasaki, M.D., Department of Neurosurgery, University of Tokyo Hospital, 7-3-1, Hongo, Bunkyoku, Tokyo, Japan. © 1983 by Elsevier Science Publishing Co., Inc.
removal of the odontoid process and suboccipital craniectomy but was successfully managed by diaphragm pacing. A 16-year-old girl was referred to the Neurosurgical Service of the University of Tokyo Hospital on March 12, 1980 with an ll-month history of nocturnal dyspnea. At the age of 11 years, the patient fell from a bed and had a cervical injury. She was admitted to the referring hospital with respiratory distress and tetraparesis, and was diagnosed as suffering from atlanto-axial dislocation. For 3 months her breathing was assisted by a positive-pressure respirator. Although no attempt was made to manage the atlanto-axial dislocation, tetraparesis slowly improved thereafter and the functional ability of both the upper and lower extremities was gradually restored. However, tetraparesis was aggravated after April 1979 and she complained of dyspnea occurring at night. Initial examination revealed hypesthesia below the level of the C-2 dermatome. Both the upper and lower extremities were moderately paralyzed. At night, her breathing had to be assisted by a respirator. Tomograms of the cervical spine revealed an atlanto-axial dislocation (Figure 1A). Computed tomography scan demonstrated that the spinal canal at the level of C-1 was markedly narrowed by a large dislocated odontoid process (Figure l-B). As the patient's neurological condition progressively deteriorated, decompressive laminectomy of C-1 and a half of C-2 combined with suboccipital craniectomy was performed on April 8, 1980. Because the spinal cord was compressed by the posterolaterally disclosed odontoid process, it was partially drilled away. The dura mater was greatly thickened at the level of the C-1 lamina. It was opened and repaired with Lyodura. Postoperatively, she was put in a halo-hoop to provide for immobilization. The C-1 and C-2 vertebral bodies were fused by a transoral pharyngeal approach on July 15, 1980. In spite of these surgical procedures, both her upper and lower extremities remained partially paralyzed. She still complained of nocturnal dyspnea. Therefore, it was decided to employ diaphragm pacing for the management of the 0090-3019/83/030232-0553.00
Diaphragm Pacing
Figure 1. (A) Tomogram of cervical spine: the large odontoid process is posteriorly dislocated. (B) Computed tomography scan demonstrating posterolaterally dislocated odontoid process (arrow): the spinal canal is markedly narrowed. The patient's head was tilted to the right and immobilized so that a part of the posterior fossa is visualized in the right side of the photograph.
nocturnal dyspnea. Before implantation of a diaphragm pacemaker, transvenous phrenic nerve stimulation was carried out to see if the nerve and diaphragm were intact. A bipolar catheter electrode (007157, USCI) for temporary cardiac pacing was inserted via the right femoral vein. In the superior vena cava, the stimulation, which was provided by the cardiac pacemaker, resulted in favorable rhythmic right diaphragmatic contraction. The patient underwent an operation on the right side of the neck on April 25, 1981. As shown in the postoperative x-ray film of the chest (Figure 2), a bipolar nerve cuff electrode (E-325, Avery Laboratories Inc., Farmingdale, New York) was applied around the right phrenic nerve, with the receiver (I-107, Avery Laboratories Inc.) installed in the subcutaneous tissue of the right anterior chest wall as described by Glenn et al [4]. The electronic parameters were set as follows: the respiratory rate: 15/rain; inspiration time: 1.4 seconds; pulse interval: 40 milliseconds; and the amplitude: 2.5 mA. Pacing of the diaphragm was begun 2 weeks postoperatively. At night, ventilatory studies were performed both with and without diaphragm pacing. A Fleisch pneumotachograph was connected to the expiratory limb of a non-rebreathing valve, and flow velocity through
Surg Neurol 1983;19:232-6
233
Figure 2. X-ray film of the chest anteroposterior view showing an electrode cuff around the right phrenic nerve, a pair of lead wires, and a receiver of phrenic nerve stimulator.
234
Surg Neurol 1983;19:232-6
FIowVelocity(qsec) +0"5t
Sasaki et al
Discussion
Isec.
-0.5
2.o
Volume(l)
1.0[
~~r It
~
e
I~--DiaphragmPacing-~l ASLEEP *I
Figure 3. Respiratory study (May 24, 1981). Without diaphragm pacing, respiration during sleep was irregular. The diaphragm pacing regularized the patient's respiration and increased flow velocity as well as tidal volume.
the tracheostomy tube was measured using a differential pressure transducer and carrier amplifier (Nihon Kohden, AR-600G), signals from which were integrated using an electrical integrator (Nihon Kohden, AQ-600G) to measure the tidal volume. The results of these studies are illustrated in Figures 3 and 4. Without diaphragm pacing, apnea, lasting 10 to 20 seconds, followed by hyperventilation was intermittently observed. The patient sometimes woke up and complained of stifling which was not alleviated by oxygen inhalation. On the other hand, diaphragm pacing corrected her respiration, increased tidal volume and resulted in a modest rise in Pao2 and a fall in Paco2. To avoid nerve fatigue [3], diaphragm pacing was never continued for more than 8 hours. About 2 months after initiating diaphragm pacing, she no longer complained of sleeping difficulty. Moreover, the daytime somnolence and nighttime insomnia present prior to the pacing were alleviated. The patient was discharged from the hospital without anxiety associated with sleep-induced dyspnea on July 9, 1981. Thereafter, her tetraparesis slowly improved. She became able to move around using a wheelchair in the autumn of 1981. About one year after initiating diaphragm pacing, she was readmitted to our hospital for the evaluation of her respiratory condition during sleep. Because her tracheostomy had been closed, a transthoracic impedance pneumograph (Nihon Kohden, OGR-5100) calibrated against a standard Wright's respiratory, was used to measure the tidal volume. As shown in Figure 5, diaphragm pacing regularized her respiration and increased tidal volume. The PaQ and Paco2 were both maintained within normal range during her sleep.
In 1948, Sarnoffet al temporarily applied electrophrenic respiration to several patients with ventilatory insufficiency secondary to bulbar poliomyelitis [ 18,19]. However, this method proved impractical for regular use because electrical impulses were delivered either through percutaneous wire electrodes, or through transcutaneous electrodes placed on the surface of the skin. In 1966, van Heeckeren and Glenn demonstrated a new technique of diaphragm pacing by radiofrequency induction [23]. No external wires were necessary because the power source was outside the body in the form of a radio transmitter with easy access for adjustments and battery changes. Signals were transmitted via the antenna to an implanted receiver requiring no batteries. Long-term stimulation of the phrenic nerve became possible with this new technique of diaphragm pacing. Diaphragm pacing overcomes several physiological drawbacks of positive-pressure respiration [1,3,13,21,23]. Langou et al have reported on the hemodynamic changes produced by diaphragm pacing in eight patients with Ondine's curse [13]. Diaphragm pacing caused drops in mean pulmonary arterial and systemic arterial pressure, a fall in arterial carbon dioxide tension, and rises in arterial oxygen tension and pH. Calculated pulmonary arteriolar vascular resistance also decreased wtih diaphragm pacing. In the present case, diaphragm pacing increased the tidal volume, normalized arterial blood gases, and subsequently reduced the patient's discomfort at night. Diaphragm pacing over prolonged periods of time has been employed successfully in the management of ventilatory insufficiency in four types of patients, those with cervical cord injury or disease [5,10,20,22], those
Figure 4. Effect of diaphragm pacing on ventilation during quiet sleep. After the patient fell asleep, mean tidal volume and minute volume decreased. The diaphragm pacing increased ventilation and resulted in a modest rise in Pa02 and a fall in Paco> Pa02 : 91.6 PaC02 : 35.5
Minute volume (L/min)
~]~
Mean tidal volume (ml)
¢ 4.0"
PaOz
: 67.6
elt% , ~ , z ~ .
P.CO2 : 3,0 3.0-
1.0 . . . .
I
"~'~,~
,
'
10
"
20
/
2L/rain
30
Asleep
O2 - ~
40
I
--
"140
-N" "130
e-
.
" ~
50
60
110
100 70 (min) =l
Diaphragm Pacing
Surg Neurol 1983;19:232-6
235
ECG
Volume (too 200 T
IOsec
Diaphragm Pacing ASLEEP
Figure 5. Respiratory study made one year after initiating diaphragm pacing. Diaphragm pacing regularized the patient's respiration. As compared with one year before (Figure 4), tidal volume was even more increased by the diaphragm pacing.
It
Long-term ventitatory support by diaphragm pacing in quadriplegia. Ann Surg 1976;183:566-77. 6. Glenn WWL, Gee JBL, Schachter EN. Diaphragm pacing: application to a patient with chronic obstructive pulmonary disease. J Thorac Cardiovasc Surg 1978;75:273-81. 7. Glenn WWL, Hogan JF, Phelps ML. Ventilatory support of the quadriplegic patient with respiratory paralysis by diaphragm pac~ ing. Surg Clin in North Am 1980;60:1055-78.
with central alveolar hypoventilation [3,8,9,13,14], those with chronic obstructive pulmonary disease [6], and selected cases of neuromuscular disease [17]. However, diaphragm pacing does require that the patient have both a normal, or virtually normal, lung vital capacity and a proven intact phrenic nerve. Regarding the technique of diaphragm pacing, Glenn et al have recently reported that they prefer to place a monopolar electrode on the phrenic nerve in the chest and implant the receiver in the midaxillary line at about the seventh or eighth rib [7]. By properly selecting patients, diaphragm pacing can provide effective long-term relief from ventilatory insufficiency of various origins.
10. Kim JH, Mannelidis EE, Glenn WWL, Kaneyuki T. Diaphragm pacing: histopathological changes in the phrenic nerve following long-term electrical stimulation. J Thorac Cardiovasc Surg 1976;72:602-8. 11. Krieger AJ, Rosomoff HL. Sleep-induced apnea. Part 1: a respiratory and autonomic dysfunction syndrome following bilateral percutaneous cervical cordotomy. J Neurosurg 1974;40:168-80.
References
12. Krieger AJ, Rosomoff HL. Sleep-induced apnea. Part 2: Respiratory failure after anterior spinal surgery. J Neurosurg 1974;40:181-5.
1. Daggett WM, Shanahan EA, Kazemi H, Morgan AP, Austin WG. Intracaval electrophrenic stimulation. II. Studies on pulmonary mechanics, surface tension, urine flow, and bilateral phrenic nerve stimulation. J Thorac Cardiovasc Surg 1970;60:98-107. 2. FieldingJW, Tuul A, Hawkins RJ. Ondine's curse. A complication of upper cervical-spine surgery. J Bone Joint Surg [Br] 1975;57:1000-1. 3. Glenn WWL, Holcomb WG, Gee JBL, Rath R. Central hypoventilation: Long-term ventilatory assistance by radiofrequency electrophrenic respiration. Ann Surg 1970;172:755-73. 4. Glenn WWL, Holcomb WG, Hogan J, Matano I, Gee JBL, Motoyama EK, Kim CS, Poirier RS, Forbes G. Diaphragm pacing by radio-frequency transmission in the treatment of chronic ventilatory insufficiency. Present status. J Thorac Cardiovasc Surg 1973;66:505-20. 5. Glenn WWL, Holcomb WG, Shaw RK, HoganJF, Holschuh KR.
8. Hunt CE, Matalon SV, Thompson TR, Demuth S, LoewJM~ Liu HM, Mastri A, Burke B. Central hypoventilation syndrome: Experience with bilateral phrenic nerve pacing in 3 neonates. Am Rev Respir Dis 1978;118:23-8. 9. Hyland RH, Jones NL, Powles ACP, Lenkie SCM, Vanderlinden RG, Epstein SW. Primary alveolar hypoventilation treated with noctt~rnal electrophrenic respiration. Am Rev Respir Dis 1978;117:165-72.
13. Langou RA, Cohen LS, Sheps D, Wolfson S, Glenn WWL. Ondine's curse: hemodynamic response to diaphragm pacing (electrophrenic respiration). Am Heart J 1978;95:195-300. 14. Mellins RB, Balfour H H Jr, Turino GM, Winters RW. Failure of automatic control of ventilation (Ondine's curse). Medicine (Baltimore) 1970;49:487-504. 15. Mullan S, Hosobuchi Y. Respiratory hazards of high cervical percutaneous cordotomy. J Neurosurg 1968;28:291-7. 16. Richardson RR, Johnson N, Cerullo LJ. Diaphragm pacing in central von Recklinghausen's disease: a case report. Neurosurgery 1978;3:75-8. 17. Richardson RR, Roseman B, Singh N. Diaphragm pacing in spinal muscular atrophy: case report. Neurosurgery 1981;9:317-9. 18. Sarnoff SJ, Hardenbergh E, Whittenberger JL. Electrophrenic respiration. Am J Physiol 1948;155:1-9.
236
Surg Neurol 1983;19:232-6
19. Sarnoff SJ, Hardenbergh E, Whittenberger JL. Electrophrenic respiration. Science 1948;108:482. 20. SeveringhausJW, Mitchell RA. Ondine's curse: Failure of respiratory center automaticity while awake. Clin Res 1962;10:122. 21. Stemmer EA, Crawford DW, List JW, Heber RE, Connolly JE. Diaphragmatic pacing in the treatment of hypoventilation syndrome. J Thorac Cardiovasc Surg 1967;54:649-57.
Sasaki et al
22. Vanderlinden RG, Gilpin L, Harper J, McClurkin M, Twilley D. Electrophrenic respiration in quadriplegia.Can Nurse 1974;70:236. 23. van Heeckeren DW, Glenn WWL Electrophrenic respiration by radiofrequency induction.J Thorac Cardiovasc Surg 1966;52:65565.
Surg Neurol 1983;19:232-6
Nocturnal Dyspnea Treated by Diaphragm Pacing Tomio Sasaki, Hirotake Nakano, Takao Asano, Shinya Manaka, Kintomo Takakura, Haruhiko Tsutsumi, Hidenori Toyooka, and Ikuo Satoh The Departments of Neurosurgery and Emergency Medicine, University of Tokyo Hospital, Tokyo, Japan and the Satoh Clinic, Ube City, YamaguchiJapan
Sasaki T, Nakano H, Asano T, Manaka S, Takakura K, Tsutsumi H, Toyooka H, Satoh I. Nocturnal dyspnea treated by diaphragm pacing. Surg Neurol 232-6.
A case of nocturnal dyspnea secondary to atlantoaxial dislocation is reported. Noctural dyspnea was not alleviated by a decompressive laminectomy of C-1 and a half of C2 vertebrae made by a transoral pharyngeal approach. Diaphragm pacing at night was effective for the longterm management of nocturnal dyspnea. KEY WORDS: Atlantoaxial dislocation; Central hypoventilation; Diaphragm pacing; Phrenic nerve; Dyspnea, nocturnal
Noctural dyspnea is uncommonly encountered in patients with upper cervical lesions of the spinal cord [2,11,12,15,20]. In such cases, positive-pressure respirators are usually employed to assist respiration at night. However, long-term application of such mechanical respirators may lead to various complications including infectious diseases and pulmonary fibrosis. Electrical stimulation of the phrenic nerve (diaphragm pacing) is an attractive method for prolonged ventilatory assistance because it overcomes several physiological drawbacks of positive-pressure respirators [1,3,13,21,23]. Several authors have recently reported that diaphragm pacing was successfully applied in the long-term management of ventilatory insufficiency of various origins, i.e., encephalitis, brain-stem infarction, posterior fossa tumor, the pickwickian syndrome, ventilatory insufficiency due to cordotomy, bulbar poliomyelitis, traumatic injury of the high cervical spinal cord, and spinal muscular atrophy [3-6,8,14,16,17,20,22]. In this paper, we present a case of nocturnal dyspnea secondary to atlantoaxial dislocation, which was not relieved by decompression of the spinal cord with laminectomy of C-1 and a half of C-2 combined with partial Address reprint requests to: Tomio Sasaki, M.D., Department of Neurosurgery, University of Tokyo Hospital, 7-3-1, Hongo, Bunkyoku, Tokyo, Japan. © 1983 by Elsevier Science Publishing Co., Inc.
removal of the odontoid process and suboccipital craniectomy but was successfully managed by diaphragm pacing. A 16-year-old girl was referred to the Neurosurgical Service of the University of Tokyo Hospital on March 12, 1980 with an ll-month history of nocturnal dyspnea. At the age of 11 years, the patient fell from a bed and had a cervical injury. She was admitted to the referring hospital with respiratory distress and tetraparesis, and was diagnosed as suffering from atlanto-axial dislocation. For 3 months her breathing was assisted by a positive-pressure respirator. Although no attempt was made to manage the atlanto-axial dislocation, tetraparesis slowly improved thereafter and the functional ability of both the upper and lower extremities was gradually restored. However, tetraparesis was aggravated after April 1979 and she complained of dyspnea occurring at night. Initial examination revealed hypesthesia below the level of the C-2 dermatome. Both the upper and lower extremities were moderately paralyzed. At night, her breathing had to be assisted by a respirator. Tomograms of the cervical spine revealed an atlanto-axial dislocation (Figure 1A). Computed tomography scan demonstrated that the spinal canal at the level of C-1 was markedly narrowed by a large dislocated odontoid process (Figure l-B). As the patient's neurological condition progressively deteriorated, decompressive laminectomy of C-1 and a half of C-2 combined with suboccipital craniectomy was performed on April 8, 1980. Because the spinal cord was compressed by the posterolaterally disclosed odontoid process, it was partially drilled away. The dura mater was greatly thickened at the level of the C-1 lamina. It was opened and repaired with Lyodura. Postoperatively, she was put in a halo-hoop to provide for immobilization. The C-1 and C-2 vertebral bodies were fused by a transoral pharyngeal approach on July 15, 1980. In spite of these surgical procedures, both her upper and lower extremities remained partially paralyzed. She still complained of nocturnal dyspnea. Therefore, it was decided to employ diaphragm pacing for the management of the 0090-3019/83/030232-0553.00
Diaphragm Pacing
Figure 1. (A) Tomogram of cervical spine: the large odontoid process is posteriorly dislocated. (B) Computed tomography scan demonstrating posterolaterally dislocated odontoid process (arrow): the spinal canal is markedly narrowed. The patient's head was tilted to the right and immobilized so that a part of the posterior fossa is visualized in the right side of the photograph.
nocturnal dyspnea. Before implantation of a diaphragm pacemaker, transvenous phrenic nerve stimulation was carried out to see if the nerve and diaphragm were intact. A bipolar catheter electrode (007157, USCI) for temporary cardiac pacing was inserted via the right femoral vein. In the superior vena cava, the stimulation, which was provided by the cardiac pacemaker, resulted in favorable rhythmic right diaphragmatic contraction. The patient underwent an operation on the right side of the neck on April 25, 1981. As shown in the postoperative x-ray film of the chest (Figure 2), a bipolar nerve cuff electrode (E-325, Avery Laboratories Inc., Farmingdale, New York) was applied around the right phrenic nerve, with the receiver (I-107, Avery Laboratories Inc.) installed in the subcutaneous tissue of the right anterior chest wall as described by Glenn et al [4]. The electronic parameters were set as follows: the respiratory rate: 15/rain; inspiration time: 1.4 seconds; pulse interval: 40 milliseconds; and the amplitude: 2.5 mA. Pacing of the diaphragm was begun 2 weeks postoperatively. At night, ventilatory studies were performed both with and without diaphragm pacing. A Fleisch pneumotachograph was connected to the expiratory limb of a non-rebreathing valve, and flow velocity through
Surg Neurol 1983;19:232-6
233
Figure 2. X-ray film of the chest anteroposterior view showing an electrode cuff around the right phrenic nerve, a pair of lead wires, and a receiver of phrenic nerve stimulator.
234
Surg Neurol 1983;19:232-6
FIowVelocity(qsec) +0"5t
Sasaki et al
Discussion
Isec.
-0.5
2.o
Volume(l)
1.0[
~~r It
~
e
I~--DiaphragmPacing-~l ASLEEP *I
Figure 3. Respiratory study (May 24, 1981). Without diaphragm pacing, respiration during sleep was irregular. The diaphragm pacing regularized the patient's respiration and increased flow velocity as well as tidal volume.
the tracheostomy tube was measured using a differential pressure transducer and carrier amplifier (Nihon Kohden, AR-600G), signals from which were integrated using an electrical integrator (Nihon Kohden, AQ-600G) to measure the tidal volume. The results of these studies are illustrated in Figures 3 and 4. Without diaphragm pacing, apnea, lasting 10 to 20 seconds, followed by hyperventilation was intermittently observed. The patient sometimes woke up and complained of stifling which was not alleviated by oxygen inhalation. On the other hand, diaphragm pacing corrected her respiration, increased tidal volume and resulted in a modest rise in Pao2 and a fall in Paco2. To avoid nerve fatigue [3], diaphragm pacing was never continued for more than 8 hours. About 2 months after initiating diaphragm pacing, she no longer complained of sleeping difficulty. Moreover, the daytime somnolence and nighttime insomnia present prior to the pacing were alleviated. The patient was discharged from the hospital without anxiety associated with sleep-induced dyspnea on July 9, 1981. Thereafter, her tetraparesis slowly improved. She became able to move around using a wheelchair in the autumn of 1981. About one year after initiating diaphragm pacing, she was readmitted to our hospital for the evaluation of her respiratory condition during sleep. Because her tracheostomy had been closed, a transthoracic impedance pneumograph (Nihon Kohden, OGR-5100) calibrated against a standard Wright's respiratory, was used to measure the tidal volume. As shown in Figure 5, diaphragm pacing regularized her respiration and increased tidal volume. The PaQ and Paco2 were both maintained within normal range during her sleep.
In 1948, Sarnoffet al temporarily applied electrophrenic respiration to several patients with ventilatory insufficiency secondary to bulbar poliomyelitis [ 18,19]. However, this method proved impractical for regular use because electrical impulses were delivered either through percutaneous wire electrodes, or through transcutaneous electrodes placed on the surface of the skin. In 1966, van Heeckeren and Glenn demonstrated a new technique of diaphragm pacing by radiofrequency induction [23]. No external wires were necessary because the power source was outside the body in the form of a radio transmitter with easy access for adjustments and battery changes. Signals were transmitted via the antenna to an implanted receiver requiring no batteries. Long-term stimulation of the phrenic nerve became possible with this new technique of diaphragm pacing. Diaphragm pacing overcomes several physiological drawbacks of positive-pressure respiration [1,3,13,21,23]. Langou et al have reported on the hemodynamic changes produced by diaphragm pacing in eight patients with Ondine's curse [13]. Diaphragm pacing caused drops in mean pulmonary arterial and systemic arterial pressure, a fall in arterial carbon dioxide tension, and rises in arterial oxygen tension and pH. Calculated pulmonary arteriolar vascular resistance also decreased wtih diaphragm pacing. In the present case, diaphragm pacing increased the tidal volume, normalized arterial blood gases, and subsequently reduced the patient's discomfort at night. Diaphragm pacing over prolonged periods of time has been employed successfully in the management of ventilatory insufficiency in four types of patients, those with cervical cord injury or disease [5,10,20,22], those
Figure 4. Effect of diaphragm pacing on ventilation during quiet sleep. After the patient fell asleep, mean tidal volume and minute volume decreased. The diaphragm pacing increased ventilation and resulted in a modest rise in Pa02 and a fall in Paco> Pa02 : 91.6 PaC02 : 35.5
Minute volume (L/min)
~]~
Mean tidal volume (ml)
¢ 4.0"
PaOz
: 67.6
elt% , ~ , z ~ .
P.CO2 : 3,0 3.0-
1.0 . . . .
I
"~'~,~
,
'
10
"
20
/
2L/rain
30
Asleep
O2 - ~
40
I
--
"140
-N" "130
e-
.
" ~
50
60
110
100 70 (min) =l
Diaphragm Pacing
Surg Neurol 1983;19:232-6
235
ECG
Volume (too 200 T
IOsec
Diaphragm Pacing ASLEEP
Figure 5. Respiratory study made one year after initiating diaphragm pacing. Diaphragm pacing regularized the patient's respiration. As compared with one year before (Figure 4), tidal volume was even more increased by the diaphragm pacing.
It
Long-term ventitatory support by diaphragm pacing in quadriplegia. Ann Surg 1976;183:566-77. 6. Glenn WWL, Gee JBL, Schachter EN. Diaphragm pacing: application to a patient with chronic obstructive pulmonary disease. J Thorac Cardiovasc Surg 1978;75:273-81. 7. Glenn WWL, Hogan JF, Phelps ML. Ventilatory support of the quadriplegic patient with respiratory paralysis by diaphragm pac~ ing. Surg Clin in North Am 1980;60:1055-78.
with central alveolar hypoventilation [3,8,9,13,14], those with chronic obstructive pulmonary disease [6], and selected cases of neuromuscular disease [17]. However, diaphragm pacing does require that the patient have both a normal, or virtually normal, lung vital capacity and a proven intact phrenic nerve. Regarding the technique of diaphragm pacing, Glenn et al have recently reported that they prefer to place a monopolar electrode on the phrenic nerve in the chest and implant the receiver in the midaxillary line at about the seventh or eighth rib [7]. By properly selecting patients, diaphragm pacing can provide effective long-term relief from ventilatory insufficiency of various origins.
10. Kim JH, Mannelidis EE, Glenn WWL, Kaneyuki T. Diaphragm pacing: histopathological changes in the phrenic nerve following long-term electrical stimulation. J Thorac Cardiovasc Surg 1976;72:602-8. 11. Krieger AJ, Rosomoff HL. Sleep-induced apnea. Part 1: a respiratory and autonomic dysfunction syndrome following bilateral percutaneous cervical cordotomy. J Neurosurg 1974;40:168-80.
References
12. Krieger AJ, Rosomoff HL. Sleep-induced apnea. Part 2: Respiratory failure after anterior spinal surgery. J Neurosurg 1974;40:181-5.
1. Daggett WM, Shanahan EA, Kazemi H, Morgan AP, Austin WG. Intracaval electrophrenic stimulation. II. Studies on pulmonary mechanics, surface tension, urine flow, and bilateral phrenic nerve stimulation. J Thorac Cardiovasc Surg 1970;60:98-107. 2. FieldingJW, Tuul A, Hawkins RJ. Ondine's curse. A complication of upper cervical-spine surgery. J Bone Joint Surg [Br] 1975;57:1000-1. 3. Glenn WWL, Holcomb WG, Gee JBL, Rath R. Central hypoventilation: Long-term ventilatory assistance by radiofrequency electrophrenic respiration. Ann Surg 1970;172:755-73. 4. Glenn WWL, Holcomb WG, Hogan J, Matano I, Gee JBL, Motoyama EK, Kim CS, Poirier RS, Forbes G. Diaphragm pacing by radio-frequency transmission in the treatment of chronic ventilatory insufficiency. Present status. J Thorac Cardiovasc Surg 1973;66:505-20. 5. Glenn WWL, Holcomb WG, Shaw RK, HoganJF, Holschuh KR.
8. Hunt CE, Matalon SV, Thompson TR, Demuth S, LoewJM~ Liu HM, Mastri A, Burke B. Central hypoventilation syndrome: Experience with bilateral phrenic nerve pacing in 3 neonates. Am Rev Respir Dis 1978;118:23-8. 9. Hyland RH, Jones NL, Powles ACP, Lenkie SCM, Vanderlinden RG, Epstein SW. Primary alveolar hypoventilation treated with noctt~rnal electrophrenic respiration. Am Rev Respir Dis 1978;117:165-72.
13. Langou RA, Cohen LS, Sheps D, Wolfson S, Glenn WWL. Ondine's curse: hemodynamic response to diaphragm pacing (electrophrenic respiration). Am Heart J 1978;95:195-300. 14. Mellins RB, Balfour H H Jr, Turino GM, Winters RW. Failure of automatic control of ventilation (Ondine's curse). Medicine (Baltimore) 1970;49:487-504. 15. Mullan S, Hosobuchi Y. Respiratory hazards of high cervical percutaneous cordotomy. J Neurosurg 1968;28:291-7. 16. Richardson RR, Johnson N, Cerullo LJ. Diaphragm pacing in central von Recklinghausen's disease: a case report. Neurosurgery 1978;3:75-8. 17. Richardson RR, Roseman B, Singh N. Diaphragm pacing in spinal muscular atrophy: case report. Neurosurgery 1981;9:317-9. 18. Sarnoff SJ, Hardenbergh E, Whittenberger JL. Electrophrenic respiration. Am J Physiol 1948;155:1-9.
236
Surg Neurol 1983;19:232-6
19. Sarnoff SJ, Hardenbergh E, Whittenberger JL. Electrophrenic respiration. Science 1948;108:482. 20. SeveringhausJW, Mitchell RA. Ondine's curse: Failure of respiratory center automaticity while awake. Clin Res 1962;10:122. 21. Stemmer EA, Crawford DW, List JW, Heber RE, Connolly JE. Diaphragmatic pacing in the treatment of hypoventilation syndrome. J Thorac Cardiovasc Surg 1967;54:649-57.
Sasaki et al
22. Vanderlinden RG, Gilpin L, Harper J, McClurkin M, Twilley D. Electrophrenic respiration in quadriplegia.Can Nurse 1974;70:236. 23. van Heeckeren DW, Glenn WWL Electrophrenic respiration by radiofrequency induction.J Thorac Cardiovasc Surg 1966;52:65565.