Amyotrophic lateral sclerosis: the Midwestern surgical experience with the diaphragm pacing stimulation system shows that general anesthesia can be safely performed

doi:10.1016/j.amjsurg.2008.11.008

The American Journal of Surgery

Volume 197, Issue 3, March 2009, Pages 386-390

https://doi.org/10.1016/j.amjsurg.2008.11.008 Get rights and content

Abstract

Background

There is a paucity of literature concerning general anesthesia and surgery in patients with amyotrophic lateral sclerosis (ALS or Lou Gehrig's disease). This report summarizes the largest series of surgical cases in ALS during multicenter prospective trials of the laparoscopic diaphragm pacing system (DPS) to delay respiratory failure.

Method

The overall strategy outlined includes the use of rapidly reversible short-acting analgesic and amnestic agents with no neuromuscular relaxants.

Results

Fifty-one patients were implanted from March 2005 to March 2008 at 2 sites. Age at implantation ranged from 42 to 73 years and the percent predicted forced vital capacity (FVC) ranged from 20% to 87%. On preoperative blood gases, Pco₂ was as high as 60. Using this protocol, there were no failures to extubate or 30-day mortalities. The DPS system increases the respiratory system compliance by decreasing posterior lobe atelectasis and can stimulate respirations at the end of each case.

Conclusions

Laparoscopic surgery with general anesthesia can be safely performed in patients with ALS undergoing DPS.

Section snippets

Patients and Methods

This is a prospective evaluation of all ALS patients implanted with the DPS system from March 2005 until March 2008 at University Hospitals Case Medical Center (UHCMC), Cleveland, OH, and Henry Ford Health System (HFHS), Detroit, MI. This was undertaken under FDA Investigational Device Exemption (IDE) G040142 and both sites institutional review boards (IRBs) approved the investigations. The study was registered at http://www.clinicaltrials.gov with the specific identifier NCT00420719. The

Results

For the initial pilot trial, 16 patients were implanted at UHCMC from March 2005 to March 2007 with an average age of 50 years (range 32 to 70 years); 13 of the patients were males. The average predicted FVC at surgery was 56% (range 45% to 89%) with 5 patients having values below 50%. Two patients had hypercarbia before implantation. Six patients underwent simultaneous gastrostomy tube placements. The average time for the operation was 98 minutes (range 60 to 134 minutes).

In September 2007, 2

Comments

This is the largest reported series of ALS patients undergoing general anesthesia safely for a laparoscopic procedure. General anesthesia consisting of remifentanil, sevoflurane, and propofol was effective in facilitating neuromuscular evaluation and laparoscopic surgery in ALS patients without adverse perioperative effects. This strategy may be useful and facilitate other surgery on patients with ALS. The limiting factor in concluding that all surgery in ALS patients can be done safely is that

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El marcapasos diafragmático (MD) ha demostrado su utilidad en la sustitución de la ventilación mecánica en pacientes afectos de lesiones medulares crónicas (LMC) y esclerosis lateral amiotrófica (ELA), mejorando la calidad de vida y reduciendo la morbimortalidad y los costes sanitarios asociados. El manejo anestésico de dichos pacientes y las particularidades del procedimiento quirúrgico suponen un reto anestésico. El objetivo de nuestro estudio es el análisis del manejo y las complicaciones intraoperatorias de los pacientes sometidos a MD en nuestra institución.
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La colocación de MD bajo anestesia general es una intervención segura, tanto en el paciente adulto como en el pediátrico, en la que pueden aparecer complicaciones derivadas tanto de la enfermedad de base como de la técnica quirúrgica, siendo necesaria una rápida identificación y tratamiento de las mismas para el buen desarrollo del procedimiento.
The diaphragm pacemaker (DP) has proven its utility in replacing mechanical ventilation in patients with chronic spinal cord injury (SCI) and amyotrophic lateral sclerosis (ALS). The DP improves patient quality of life and reduces morbidity and mortality and their associated health care costs. The anesthetic management of these patients and the particularities of the surgical procedure are challenging. The aim of our study is to analyze anesthetic management and intraoperative complications in patients undergoing DP placement in our hospital.
We performed a chart review of patients treated between December 2007 and July 2017, recording the patients’ preoperative status, anesthetic management and intraoperative complications.
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DP placement under general anesthesia is a safe intervention in both adult and pediatric patients. Complications derived from both the underlying disease and the surgical technique may appear, and must be rapidly identified and treated to obtain a satisfactory surgical outcome.
Anesthetic considerations for laparoscopy for rectal cancer in patient with amyotrophic lateral sclerosis: A case report
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A 60-year-old man with stage IV rectal cancer who developed ALS after palliative surgery has been described [4]. In addition, given the potential risk of general anesthesia [5], curative laparoscopy for rectal cancer arising in an ALS patient has not previously been reported in an English-language publication [4]. Here, we report a case involving rectal cancer arising in an ALS patient who subsequently underwent curative laparoscopic surgery and adjuvant chemotherapy.
Amyotrophic lateral sclerosis, which is also known as motor neuron disease, is a chronic neurodegenerative disease characterized by progressive muscular weakness, respiratory muscle disability, and eventual death. Previous epidemiologic studies have shown no association between cancer and amyotrophic lateral sclerosis. Colorectal cancer arising in patients with amyotrophic lateral sclerosis has rarely been reported. Here, we report a case involving rectal cancer arising in a patient with amyotrophic lateral sclerosis who subsequently underwent curative laparoscopic surgery and adjuvant chemotherapy. Amyotrophic lateral sclerosis causes the deterioration of respiratory function by compromising expiratory and inspiratory muscles; accordingly, patients with amyotrophic lateral sclerosis are at high anesthetic risk, especially with respect to general anesthesia. Careful airway management is essential, and intraoperative neuromuscular monitoring is important. A depolarizing muscle relaxant such as succinylcholine should not be used because of the potential risk of developing hyperkalemia or rhabdomyolysis. Thus, a nondepolarizing muscle relaxant (rocuronium) was used at a low dose in this case. In addition, fentanyl for postoperative patient-controlled analgesia should be used cautiously because fentanyl can cause respiratory muscle rigidity, which may reduce postoperative respiratory function in patients with amyotrophic lateral sclerosis.
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These concerns are alleviated by abundant reports of anesthesia in a variety of surgeries in ALS patients, without evidence of decline. Surgeries have included feeding tube placement, diaphragmatic pacemaker implantation, spinal stem cell transplantation, and various other procedures [18–21]. Concern for renal dysfunction or uropathy after kidney donation is an unlikely issue as there are multiple reports that living kidney donors have an extremely low peri-operative risk [22], and no increased risk of end stage renal disease (ESRD) when compared to the general population [23–26].
Patients with Amyotrophic Lateral Sclerosis (ALS) have expressed desire to become living organ donors but are unable to do so with current organ donation policies. Our objective is to assess ALS patient’s interest in organ donation, and perceived concerns of this practice by ALS neurologists.
An electronic survey was administered to ALS neurologists across the United States regarding living organ donation in ALS patients prior to respiratory failure.
52 complete responses were received from 121 invites. 67% (35/52) of neurologists expressed no concerns about living organ donation in ALS patients, and 33% had concerns. The concerns related to respiratory failure, anesthesia exposure and renal dysfunction. With their concerns addressed, 71% of neurologists reported that they would endorse living organ donation.
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2017, Essentials of Neuroanesthesia
The term neuromuscular disorder encompasses a heterogeneous group of disorders affecting skeletal muscles due to abnormalities in nerve, neuromuscular junction, or ion channels or due to metabolic derangements. Management of these patients is a challenge in the perioperative period and also in neuro–intensive care units. Various neurological disorders and medications may influence the preoperative evaluation, conduct of anesthesia, and postoperative management of these patients. Myasthenia gravis and Guillain–Barré syndrome are the two most frequently encountered neurologic disorders by neuroanesthesiologists and neurointensivists in their clinical practice. These patients may be on multiple medications for the primary neurologic disorder, which may influence the use of anesthetic medications. Also, it is challenging to provide perioperative care for deep brain stimulation for patients with Parkinson’s disease, when they are scheduled for awake craniotomy for stimulator lead placement. In this chapter, the authors discuss some of the important neurologic disorders and their anesthetic management.
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American Academy of Neurology suggests that patients with ALS with a preoperative FVC < 50% should use postoperative noninvasive positive-pressure ventilation [17]. Fortunately, preoperative FVC of our patient (63%) was above that threshold, indicating a possible postoperative extubation [18]. We therefore chose general anesthesia to avoid ALS exacerbation.
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: Dr. Onders, University Hospitals of Cleveland, and Case Western Reserve University School of Medicine have intellectual property rights involved with the diaphragm pacing system and equity in Synapse Biomedical who manufactures the device.

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The Midwest Surgical AssociationAmyotrophic lateral sclerosis: the Midwestern surgical experience with the diaphragm pacing stimulation system shows that general anesthesia can be safely performed

Abstract

Background

Method

Results

Conclusions

Section snippets

Patients and Methods

Results

Comments

Chest

Surgery

Anesthesiol Clin

Neurol Clin. N Am

General anaesthesia in a patient with motor neuron disease

Eur J Anaesthesiol

Epidural anesthesia combined with propofol sedation fro abdominal hysterectomy in a patients with amyotrophic lateral sclerosis-a case report

Acta Anaesthesiol Sin

Anesthesia in two patients with motor neuron disease

Rev Esp Anestesiol Reanim

The Midwest Surgical Association
Amyotrophic lateral sclerosis: the Midwestern surgical experience with the diaphragm pacing stimulation system shows that general anesthesia can be safely performed