Unilateral phrenic nerve stimulation for neurogenic hypoventilation in Arnold Chiari malformation

Abstract

Long- term ventilator dependence in patients with neurogenic hypoventilation is associated with significant morbidity and restricts mobility. Diaphragmatic pacing by phrenic nerve stimulation (PNS) is a viable alternative. This is a case report of patient with Arnold-Chiari malformation with extensive syrinx who had neurogenic hypoventilation during sleep even after foramen magnum decompression and resolution of the syrinx. Unilateral PNS was done using spinal cord stimulator. With intermittent stimulation for 8 h while asleep, patient could be weaned off the ventilator completely. At 2 years follow- up, patient is ambulant and has returned to his routine activities. PNS is a good treatment tool in patients with neurogenic hypoventilation. Spinal cord stimulator can be used with optimal results. This is first such reported case of using spinal cord stimulator for PNS from India.

Key words

Arnold-Chiari malformation - diaphragmatic stimulation - neurogenic hypoventilation - phrenic nerve stimulation - sleep apnoea - spinal cord stimulator

• References

• 1 Taira T, Hori T. Diaphragm pacing with a spinal cord stimulator: Current state and future directions. Acta Neurochir Suppl 2007; 97: 289-292
• 2 Sitthinamsuwan B, Nunta-aree S. Phrenic nerve stimulation for diaphragmatic pacing in a patient with high cervical spinal cord injury. J Med Assoc Thai 2009; 92: 1691-1695
• 3 Glenn WW, Holcomb WG, McLaughlin AJ, O’Hare JM, Hogan JF, Yasuda R. Total ventilatory support in a quadriplegic patient with radiofrequency electrophrenic respiration. N Engl J Med 1972; 286: 513-516
• 4 Khong P, Lazzaro A, Mobbs R. Phrenic nerve stimulation: The Australian experience. J Clin Neurosci 2010; 17: 205-208
• 5 Le Pimpec-Barthes F, Gonzalez-Bermejo J, Hubsch JP, Duguet A, Morélot-Panzini C, Riquet M. et al Intrathoracic phrenic pacing: A 10-year experience in France. J Thorac Cardiovasc Surg 2011; 142: 378-383
• 6 Yasuma F, Yasuura K, Okamoto H, Miyazaki M, Hirose Y, Akune K. et al Clinical application of diaphragm pacing. J Thoracic Surg 1984; 37: 455-460
• 7 Nannapaneni R, Behari S, Todd NV, Mendelow AD. Retracing “Ondine’s curse”. Neurosurgery 2005; 57: 354-363
• 8 Onders RP, Dimarco AF, Ignagni AR, Aiyar H, Mortimer JT. Mapping the phrenic nerve motor point: The key to a successful laparoscopic diaphragm pacing system in the first human series. Surgery 2004; 136: 819-826
• 9 Glenn WW, Phelps ML. Diaphragm pacing by electrical stimulation of the phrenic nerve. Neurosurgery 1985; 17: 974-984
• 10 Hirschfeld S, Exner G, Luukkaala T, Baer GA. Mechanical ventilation or phrenic nerve stimulation for treatment of spinal cord injury-induced respiratory insufficiency. Spinal Cord 2008; 46: 738-742