Anesthetic and Surgical Management of a Bilateral Mandible Fracture in a Patient With Charcot-Marie-Tooth Disease: A Case Report Jeffrey D. Smith, DDS,* Patton Minkin, DDS,y Sean Lindsey, DDS,z and Brian Bovino, DMDx Purpose:
This report describes the case of a 74-year-old man who had been diagnosed with CharcotMarie-Tooth disease as a child. Because the patient had serious motor and sensory neuropathy associated with his disease, special anesthetic and surgical recommendations had to be considered before he underwent general anesthesia to repair his mandibular fracture.
Materials and Methods:
Repair of the mandible was performed under general anesthesia with a nasal endotracheal tube and the use of the nondepolarizing muscle relaxant rocuronium. Open reduction and internal fixation through extraoral approaches were used to fixate the displaced right subcondylar and symphyseal fractures. A closed reduction approach using maxillary fixation screws and a mandibular arch bar with light elastic guidance was used to treat a nondisplaced fracture of the left mandibular ramus. Rigid fixation allowed for avoidance of a period of intermaxillary fixation.
Results:
General anesthesia and muscle relaxant were administered without complication. Treatment of bilateral mandibular fractures with combined open and closed approaches resulted in restoration of premorbid occlusion and masticatory function.
Conclusion:
Repair of mandibular fractures under general anesthesia appears to be a safe procedure in patients with Charcot-Marie-Tooth disease when appropriate anesthetic and surgical methods are used. Ó 2015 American Association of Oral and Maxillofacial Surgeons J Oral Maxillofac Surg 73:1917.e1-1917.e6, 2015
Charcot-Marie-Tooth disease (CMTD) consists of a heterogeneous group of peripheral nerve disorders that has weakness and sensory loss in the distal limbs as the most prominent features. It was first described as a clinical entity in 1886 by the neurologists Charcot and Marie in France and Tooth in England. CMTD affects approximately 1 in 2,500 people and more than 2.8 million worldwide, making it the most common inherited neuromuscular disorder in humans.1 Although the onset and severity of symptoms can vary among those affected, most exhibit a predictable pattern of progressive lower extremity weakness, numbness, contracture, and deformity, including dropfoot and clawed hands.2 Deep tendon reflexes also are lost or diminished in the same distal-to-proximal fashion.
As the disease progresses, muscular atrophy also can occur in the respiratory muscles, diaphragm, and vocal cords.3-5 Disease onset usually occurs in the first or second decades of life and subsequently shows a slow progression over the course of several decades. CMTD is caused by inherited gene mutations affecting proteins involved in the structure and function of peripheral nerves. Disease course is varied, reflecting genotypic and phenotypic heterogeneities. In most types of CMTD, the primary problem lies in the axon, the myelin sheath, or the Schwann cells that lie along the surface of each axon.2 It can be classified according to the pattern of transmission (autosomal dominant, autosomal recessive, or X linked), according to electrophysiologic findings (demyelinating or axonal), or
Received from the Department of Oral and Maxillofacial Surgery,
Luke’s/Mt Sinai-Roosevelt Hospitals, 1111 Amsterdam Avenue,
Mt Sinai-St Luke’s/Mt Sinai-Roosevelt Hospitals, New York, NY. *Resident.
Minturn 207, New York, NY 10025; e-mail: [email protected] Received April 3 2015
yResident.
Accepted June 17 2015
zResident.
Ó 2015 American Association of Oral and Maxillofacial Surgeons
xDirector.
0278-2391/15/00897-6
Address correspondence and reprint requests to Dr Smith:
http://dx.doi.org/10.1016/j.joms.2015.06.162
Department of Oral and Maxillofacial Surgery, Mt Sinai-St
1917.e1
1917.e2
FRACTURE AND CHARCOT-MARIE-TOOTH DISEASE
maintenance of anesthesia. Careful consideration should be used when choosing the method of anesthesia, particularly in regard to depolarizing and nondepolarizing muscle relaxants. The oral and maxillofacial surgeon must obtain a detailed disease history before repair of a mandibular fracture in a patient with CMTD. Information about the patient’s premorbid masticatory function and cranial nerve status must be obtained to establish any baseline deficits that might have existed before injury. Surgical approach and the method of reduction and fixation must be considered because it is difficult to rehabilitate muscles already damaged or atrophied by CMTD. The ideal surgical plan should be minimally invasive and avoid prolonged periods of immobility.
Report of Case
FIGURE 1. Frontal photograph of a 74-year-old man with CharcotMarie-Tooth disease. Smith et al. Fracture and Charcot-Marie-Tooth Disease. J Oral Maxillofac Surg 2015.
according to the causative mutant genes. Current investigative work into the molecular and cellular disease mechanisms has shown involvement of at least 25 genes and a refinement in the classification schemes to include more than 70 CMTD subtypes.6 Treatment is rehabilitative through physical and occupational therapy, orthopedic devices, and surgical treatment for skeletal deformities and soft tissue abnormalities. Because of the progressive nature of the morbidities associated with CMTD, these patients undergo general anesthesia at a higher frequency than that of the general population. Given this and the relatively common incidence, there are surprisingly few descriptions of the anesthetic management in the literature.7-9 Although this suggests that anesthetic complications are rare, a list of potentially problematic drugs maintained by the Charcot Marie Tooth Association includes some agents commonly used during induction and
This report describes the case of a 74-year-old man (Fig 1) who was diagnosed with type 1 CMTD as a child. He exhibited classic peripheral motor and sensory manifestations of CMTD in the upper and lower extremities (Fig 2), but was otherwise healthy. A serious gait disturbance secondary to a lack of lower extremity muscle tone, pes caves, and claw toes (Fig 3) predisposed him to frequent falls. His most recent fall resulted in facial trauma after hitting his chin on the bathroom sink. General anesthesia was administered twice previously for hip replacements in 2002 and 2010. The patient reported no complications with his previous anesthetic experiences. He also reported adequate masticatory function before his facial injury, with appropriate mouth opening and bite force. He described his premorbid occlusion as stable and reproducible bilaterally.
FIGURE 2. Photograph of right-hand deformities consistent with the diagnosis of Charcot-Marie-Tooth disease. Smith et al. Fracture and Charcot-Marie-Tooth Disease. J Oral Maxillofac Surg 2015.
1917.e3
SMITH ET AL
in distal musculature since his diagnosis, it appeared to have spared the muscles in the head and neck. A head and neck examination was otherwise remarkable for a mild facial droop on the left, although there was no frank paralysis. The patient exhibited no respiratory distress and there was no apparent vocal cord involvement. Extraocular movements, facial sensation, lingual and palatal motion, and trapezius and sternocleidomastoid function were intact and symmetric. A panoramic radiograph depicted a displaced fracture of the right mandibular condyle, a displaced fracture of the left mandibular parasymphysis, and a nondisplaced fracture of the left mandibular ramus (Fig 4).
Materials and Methods FIGURE 3. Photograph of a pes cavus foot deformity seen in the patient with Charcot-Marie-Tooth disease described in this case report. Smith et al. Fracture and Charcot-Marie-Tooth Disease. J Oral Maxillofac Surg 2015.
Clinical examination on presentation to the emergency department disclosed a 4-cm chin laceration and mild edema over the right preauricular area. Intraorally, the patient had malocclusion and associated traumatic step between the mandibular left canine and lateral incisor. Maximum interincisal opening was 20 mm with pain and slight deviation to the right side. Although his disease has progressed considerably
The patient was admitted to the hospital on the evening of injury and started on intravenous antibiotics, pain control, and maintenance fluids. Routine laboratory tests were ordered and a complete blood cell count, comprehensive metabolic panel, and coagulation studies were found to be unremarkable. No other preoperative testing was indicated. Standard anesthetic induction medications were dosed according to the patient’s weight of 65 kg. He was given midazolam 2 mg, propofol 200 mg, and fentanyl 200 mg during induction. In addition, a single 20-mg dose of the nondepolarizing muscle relaxant rocuronium was administered for paralysis. A size 7.0 nasal RAE endotracheal tube was passed atraumatically through the right nostril into
FIGURE 4. Preoperative Panorex radiograph showing bilateral mandible fractures involving the right subcondylar, left parasymphysis, and left ramus regions. Smith et al. Fracture and Charcot-Marie-Tooth Disease. J Oral Maxillofac Surg 2015.
1917.e4
FRACTURE AND CHARCOT-MARIE-TOOTH DISEASE
FIGURE 5. Postoperative Panorex radiograph showing fixation hardware in place in the right subcondylar and left parasymphyseal regions, with good bony reduction of fracture segments. Smith et al. Fracture and Charcot-Marie-Tooth Disease. J Oral Maxillofac Surg 2015.
the trachea using a GlideScope. The patient was placed on volume control ventilation with inhalational desflurane used for maintenance of general anesthesia. A peripheral nerve stimulator was applied to the right ulnar nerve. Train-of-4 stimuli were applied 5 minutes after administration of the paralytic and no visible or palpable twitches were noted. The parasymphyseal fracture was manually reduced and secured with a bridal wire. Then, the patient was placed in intermaxillary fixation (IMF) with 24-gauge stainless steel wire using an arch bar in the mandible and 4 intermaxillary fixation screws in the maxilla. Approximately 30 minutes after administration of the paralytic, train-of-4 stimuli were applied. Four strong twitches were noted in addition to sustained tetany. No neuromuscular reversal was given before extraoral skin incisions. The existing chin laceration was used to approach the parasymphyseal fracture and internal fixation was applied with 2 Biomet (Biomet Microfixation, Jacksonville, FL) titanium plates. The displaced right subcondylar fracture was approached through a preauricular incision with facial nerve monitoring. After right subcondylar fracture reduction, a single Biomet miniplate was used for fixation. IMF was released and the patient was found to passively go into occlusion. After layered closure of the surgical wounds, the patient was taken off mechanical ventilation and allowed to breathe spontaneously. Adequate tidal volumes (>550 mL) and rate (>10 breaths/minute) were observed. Before extubation, the patient was able to
follow verbal commands, including a sustained head lift longer than 5 seconds. No neuromuscular reversal agent was given before extubation. Surgical time was 150 minutes, during which fentanyl 200 mg, dexamethasone 10 mg, and ondansetron 4 mg were administered intravenously for postoperative pain, swelling, and nausea control. No other medications were given by the anesthesia team. Subsequent postanesthetic care was uneventful. The patient was discharged home the following day. The patient was seen in clinic for weekly follow-up appointments for 6 weeks postoperatively. Light elastic traction was used during the first 2 weeks to guide the patient into stable occlusion. After 2 weeks, the patient started a physical therapy regimen to improve mouth opening. After 4 weeks, the maxillary fixation screws and mandibular arch bar were removed and a soft diet was started. A postoperative Panorex radiograph displayed the hardware in place and good bony reduction of mandibular fracture segments (Fig 5). Six weeks after surgery, the patient’s interincisal opening was 40 mm and his diet was advanced to regular food.
Discussion CMTD is generally caused by gene mutations that affect the function of nerve cells. CMTD is broadly classified into categories based on subtypes that affect the myelin sheath (demyelinating type) and those that
SMITH ET AL
affect the nerve axon (axonal type). Type 1 CMTD is a demyelinating form, is inherited in autosomal dominant fashion, and causes muscle neuropathy owing to markedly decreased nerve conduction velocities. The clinical symptoms of CMTD typically begin with atrophy in the peroneal muscle, resulting in a ‘‘stork leg’’ or ‘‘inverted champagne bottle’’ lower extremity appearance. Orthopedic devices are often used to improve gait, but trauma related to mechanical falls is frequent. The mandibular fracture described in this report was the patient’s third serious traumatic event related to a fall after hip fractures in 2002 and 2010. Prolonged periods of limited mobility can drastically accelerate CMTD symptoms. Mobility also is important to maintain muscle strength and flexibility. In the setting of mandibular trauma and CMTD, efforts should be made to get the patient’s jaw and masticatory muscles functioning as soon as possible. The postoperative physical therapy program should focus on strengthening unaffected muscles that can help perform the work of those that have atrophied. Atrophy outside the distal musculature tends to occur in late stages of the disease. Before undergoing general anesthesia, special consideration should be given to the respiratory and cardiovascular systems. The respiratory status of the patient with CMTD should be thoroughly assessed because the incidence of dysfunction ranges from 0 to 30%.2 Pulmonary impairment is usually restrictive in nature, associated with weakness in the intercostal muscles and diaphragm.5 If diaphragmatic dysfunction is present, then there be could an increased risk of prolonged neuromuscular blockade. In addition, numerous case reports have shown an association between CMTD and phrenic nerve dysfunction and vocal cord dysfunction. As the disease progresses, paralysis of the vocal cords has been reported in certain subtypes of CMTD, placing these patients at higher risk for aspiration.4 Ideally, all patients with CMTD should undergo direct laryngeal examination, and it is recommended that all respiratory complaints should be thoroughly investigated. CMTD also has been reported to cause cardiac dysrhythmias and conduction disturbances in association with peripheral muscle atrophy.10 An increased incidence of mitral valve prolapse has been documented in several studies. Case reports have linked CMTD with cardiac disorders consisting mainly of supraventricular tachydysrhythmias and conduction defects.11 Although such sporadic case reports exist, prospective studies have not shown an increase in cardiac dysfunction compared with the population at large.10 It is generally believed that CMTD belongs in the group of neuromuscular disorders that are unassociated with cardiac muscle disease.10 When mitral valve prolapse is seen in patients with CMTD, electrocardiography and extensive review of systems should be conducted
1917.e5 to elicit possible congestive heart failure or cardiac decompensation. Owing to the wide variety of CMTD subtypes and clinical signs and symptoms associated with this chronic progressive disease, pulmonary and cardiac needs should be tailored to the individual patient and based on clinical judgment. Perioperative management in patients with CMTD involves judicious use of neuromuscular blocking agents. The chronic denervation associated with CMTD can predispose these patients to considerable release of potassium, and therefore hyperkalemia, after exposure to the depolarizing muscle relaxant succinylcholine (SCH).12 The hyperkalemia occurs through an upregulation of acetylcholine receptors at the neuromuscular junction and from the proliferation of extra-junctional receptors depolarized by SCH. In addition to hyperkalemia, SCH use in denervated muscle can produce sustained muscle contractions lasting several minutes. Case reports have cautioned against of the use of SCH for these reasons, but larger review studies have indicated that SCH is well tolerated.12 If paralysis with SCH is desired, then a small ‘‘defasciculating’’ dose of a nondepolarizing muscle relaxant could lessen the potassium release from diseased muscle.13 In the present case, the nondepolarizing muscle relaxant rocuronium was used to aid in intubation. Although the use of a nondepolarizing muscle relaxant avoids the possible complications associated with SCH, case reports have described prolonged paralysis after the administration of vecuronium.14 Repeated doses of neuromuscular paralysis reversal agents and prolonged periods of mechanical ventilation were required in these cases.9,14 A suitable site for peripheral nerve monitoring should be identified before administering the neuromuscular blocker. Monitoring of the facial nerve is often preferred over the ulnar and tibial nerves because of the clinical wasting seen in the distal extremities.14 Effective nerve monitoring can allow neuromuscular blocking agents to be better titrated to effect and adequate reversal signs can be identified. Agents with the potential to trigger malignant hyperthermia (MH) should be used with caution because a potential link with CMTD has been suggested.15 Although isolated case reports exist and several other neuromuscular diseases are associated with MH, current understanding of the pathophysiology of CMTD and MH does not support a connection between these 2 entities.12 In the present case, SCH was avoided and inhalational desflurane was successfully administered to maintain general anesthesia without any signs or manifestations of MH. Other intraoperative anesthetic considerations include cautious patient positioning and padding of pressure points because nerve compression can exacerbate the underlying neuropathy.16 In conclusion, no evidence of a prolonged response to
1917.e6 rocuronium was found in the present patient with CMTD. Induction and maintenance of general anesthesia were well tolerated and appear to be safe when appropriate preoperative and intraoperative measures are considered. Treatment of the bilateral mandible fracture with internal fixation and postoperative physical therapy resulted in restoration of premorbid function and occlusion.
References 1. Mendell J: Charcot Marie Tooth related disorders. Semin Neurol 18:41, 1998 2. Pareyson D, Marchesi C: Diagnosis, natural history, and management of Charcot-Marie-Tooth disease. Lancet Neurol 8:654, 2009 3. Sugai K, Sugai Y: Epidural anesthesia for a patient with CharcotMarie-Tooth disease, bronchial asthma and hypo- thyroidism. Masui 38:688, 1989 4. Sulica L, Blitzer A, Lovelace RE, et al: Vocal fold paresis of Charcot-Marie-Tooth disease. Ann Otol Rhinol Laryngol 110: 1072, 2001 5. Aboussouan LS, Lewis RA, Shy ME: Disorders of pulmonary function, sleep, and the upper airway in Charcot-Marie-Tooth disease. Lung 185:1, 2007
FRACTURE AND CHARCOT-MARIE-TOOTH DISEASE 6. Berger P, Young P, Suter U: Molecular cell biology of CharcotMarie-Tooth disease. Neurogenetics 4:1, 2002 7. Hirota K, Muraoka M, Sugihara K, et al: Anesthetic experience of a patient with Charcot-Marie-Tooth disease. Masui 37:207, 1988 8. Naguib M, Samarkandt AH: Response to atracurium and mivacurium in a patient with Charcot-Marie-Tooth disease. Can J Anaesth 45:56, 1998 9. Brian JE, Boyles GD, Quirk JG, et al: Anesthetic management for Cesarean section of a patient with Charcot-Marie-Tooth disease. Anesthesiology 66:410, 1987 10. Isner JM, Hawley RJ, Weintraub AM, et al: Cardiac findings in Charcot-Marie-Tooth disease: A prospective study of 68 patients. Arch Intern Med 139:1161, 1979 11. Tetzlaff JE, Schwendt I: Arrhythmia and Charcot-Marie-Tooth disease during anesthesia. Can J Anaesth 47:829, 2000 12. Antognini JF: Anaesthesia for Charcot-Marie-Tooth disease: A review of 86 cases. Can J Anaesth 39:398, 1992 13. Gronert GA, Lambert EH, Theye RA: The response of denervated skeletal muscle to succinylcholine. Anesthesiology 39:13, 1973 14. Pogson D, Tilfer J, Wimbush S: Prolonged vecuronium neuromuscular blockade associated with Charcot Marie Tooth neuropathy. Br J Anaesth 85:914, 2000 15. Roelofse JA, Shipton EA: Anesthesia for abdominal hysterectomy in Charcot-Marie-Tooth disease. A case report. S Afr Med J 67: 605, 1985 16. Pasha TM, Knowles A: Anaesthetic management of a patient with Charcot-Marie-Tooth disease for staged diaphragmatic plication. Br J Anaesth 110:1061, 2013
This report describes the case of a 74-year-old man who had been diagnosed with CharcotMarie-Tooth disease as a child. Because the patient had serious motor and sensory neuropathy associated with his disease, special anesthetic and surgical recommendations had to be considered before he underwent general anesthesia to repair his mandibular fracture.
Materials and Methods:
Repair of the mandible was performed under general anesthesia with a nasal endotracheal tube and the use of the nondepolarizing muscle relaxant rocuronium. Open reduction and internal fixation through extraoral approaches were used to fixate the displaced right subcondylar and symphyseal fractures. A closed reduction approach using maxillary fixation screws and a mandibular arch bar with light elastic guidance was used to treat a nondisplaced fracture of the left mandibular ramus. Rigid fixation allowed for avoidance of a period of intermaxillary fixation.
Results:
General anesthesia and muscle relaxant were administered without complication. Treatment of bilateral mandibular fractures with combined open and closed approaches resulted in restoration of premorbid occlusion and masticatory function.
Conclusion:
Repair of mandibular fractures under general anesthesia appears to be a safe procedure in patients with Charcot-Marie-Tooth disease when appropriate anesthetic and surgical methods are used. Ó 2015 American Association of Oral and Maxillofacial Surgeons J Oral Maxillofac Surg 73:1917.e1-1917.e6, 2015
Charcot-Marie-Tooth disease (CMTD) consists of a heterogeneous group of peripheral nerve disorders that has weakness and sensory loss in the distal limbs as the most prominent features. It was first described as a clinical entity in 1886 by the neurologists Charcot and Marie in France and Tooth in England. CMTD affects approximately 1 in 2,500 people and more than 2.8 million worldwide, making it the most common inherited neuromuscular disorder in humans.1 Although the onset and severity of symptoms can vary among those affected, most exhibit a predictable pattern of progressive lower extremity weakness, numbness, contracture, and deformity, including dropfoot and clawed hands.2 Deep tendon reflexes also are lost or diminished in the same distal-to-proximal fashion.
As the disease progresses, muscular atrophy also can occur in the respiratory muscles, diaphragm, and vocal cords.3-5 Disease onset usually occurs in the first or second decades of life and subsequently shows a slow progression over the course of several decades. CMTD is caused by inherited gene mutations affecting proteins involved in the structure and function of peripheral nerves. Disease course is varied, reflecting genotypic and phenotypic heterogeneities. In most types of CMTD, the primary problem lies in the axon, the myelin sheath, or the Schwann cells that lie along the surface of each axon.2 It can be classified according to the pattern of transmission (autosomal dominant, autosomal recessive, or X linked), according to electrophysiologic findings (demyelinating or axonal), or
Received from the Department of Oral and Maxillofacial Surgery,
Luke’s/Mt Sinai-Roosevelt Hospitals, 1111 Amsterdam Avenue,
Mt Sinai-St Luke’s/Mt Sinai-Roosevelt Hospitals, New York, NY. *Resident.
Minturn 207, New York, NY 10025; e-mail: [email protected] Received April 3 2015
yResident.
Accepted June 17 2015
zResident.
Ó 2015 American Association of Oral and Maxillofacial Surgeons
xDirector.
0278-2391/15/00897-6
Address correspondence and reprint requests to Dr Smith:
http://dx.doi.org/10.1016/j.joms.2015.06.162
Department of Oral and Maxillofacial Surgery, Mt Sinai-St
1917.e1
1917.e2
FRACTURE AND CHARCOT-MARIE-TOOTH DISEASE
maintenance of anesthesia. Careful consideration should be used when choosing the method of anesthesia, particularly in regard to depolarizing and nondepolarizing muscle relaxants. The oral and maxillofacial surgeon must obtain a detailed disease history before repair of a mandibular fracture in a patient with CMTD. Information about the patient’s premorbid masticatory function and cranial nerve status must be obtained to establish any baseline deficits that might have existed before injury. Surgical approach and the method of reduction and fixation must be considered because it is difficult to rehabilitate muscles already damaged or atrophied by CMTD. The ideal surgical plan should be minimally invasive and avoid prolonged periods of immobility.
Report of Case
FIGURE 1. Frontal photograph of a 74-year-old man with CharcotMarie-Tooth disease. Smith et al. Fracture and Charcot-Marie-Tooth Disease. J Oral Maxillofac Surg 2015.
according to the causative mutant genes. Current investigative work into the molecular and cellular disease mechanisms has shown involvement of at least 25 genes and a refinement in the classification schemes to include more than 70 CMTD subtypes.6 Treatment is rehabilitative through physical and occupational therapy, orthopedic devices, and surgical treatment for skeletal deformities and soft tissue abnormalities. Because of the progressive nature of the morbidities associated with CMTD, these patients undergo general anesthesia at a higher frequency than that of the general population. Given this and the relatively common incidence, there are surprisingly few descriptions of the anesthetic management in the literature.7-9 Although this suggests that anesthetic complications are rare, a list of potentially problematic drugs maintained by the Charcot Marie Tooth Association includes some agents commonly used during induction and
This report describes the case of a 74-year-old man (Fig 1) who was diagnosed with type 1 CMTD as a child. He exhibited classic peripheral motor and sensory manifestations of CMTD in the upper and lower extremities (Fig 2), but was otherwise healthy. A serious gait disturbance secondary to a lack of lower extremity muscle tone, pes caves, and claw toes (Fig 3) predisposed him to frequent falls. His most recent fall resulted in facial trauma after hitting his chin on the bathroom sink. General anesthesia was administered twice previously for hip replacements in 2002 and 2010. The patient reported no complications with his previous anesthetic experiences. He also reported adequate masticatory function before his facial injury, with appropriate mouth opening and bite force. He described his premorbid occlusion as stable and reproducible bilaterally.
FIGURE 2. Photograph of right-hand deformities consistent with the diagnosis of Charcot-Marie-Tooth disease. Smith et al. Fracture and Charcot-Marie-Tooth Disease. J Oral Maxillofac Surg 2015.
1917.e3
SMITH ET AL
in distal musculature since his diagnosis, it appeared to have spared the muscles in the head and neck. A head and neck examination was otherwise remarkable for a mild facial droop on the left, although there was no frank paralysis. The patient exhibited no respiratory distress and there was no apparent vocal cord involvement. Extraocular movements, facial sensation, lingual and palatal motion, and trapezius and sternocleidomastoid function were intact and symmetric. A panoramic radiograph depicted a displaced fracture of the right mandibular condyle, a displaced fracture of the left mandibular parasymphysis, and a nondisplaced fracture of the left mandibular ramus (Fig 4).
Materials and Methods FIGURE 3. Photograph of a pes cavus foot deformity seen in the patient with Charcot-Marie-Tooth disease described in this case report. Smith et al. Fracture and Charcot-Marie-Tooth Disease. J Oral Maxillofac Surg 2015.
Clinical examination on presentation to the emergency department disclosed a 4-cm chin laceration and mild edema over the right preauricular area. Intraorally, the patient had malocclusion and associated traumatic step between the mandibular left canine and lateral incisor. Maximum interincisal opening was 20 mm with pain and slight deviation to the right side. Although his disease has progressed considerably
The patient was admitted to the hospital on the evening of injury and started on intravenous antibiotics, pain control, and maintenance fluids. Routine laboratory tests were ordered and a complete blood cell count, comprehensive metabolic panel, and coagulation studies were found to be unremarkable. No other preoperative testing was indicated. Standard anesthetic induction medications were dosed according to the patient’s weight of 65 kg. He was given midazolam 2 mg, propofol 200 mg, and fentanyl 200 mg during induction. In addition, a single 20-mg dose of the nondepolarizing muscle relaxant rocuronium was administered for paralysis. A size 7.0 nasal RAE endotracheal tube was passed atraumatically through the right nostril into
FIGURE 4. Preoperative Panorex radiograph showing bilateral mandible fractures involving the right subcondylar, left parasymphysis, and left ramus regions. Smith et al. Fracture and Charcot-Marie-Tooth Disease. J Oral Maxillofac Surg 2015.
1917.e4
FRACTURE AND CHARCOT-MARIE-TOOTH DISEASE
FIGURE 5. Postoperative Panorex radiograph showing fixation hardware in place in the right subcondylar and left parasymphyseal regions, with good bony reduction of fracture segments. Smith et al. Fracture and Charcot-Marie-Tooth Disease. J Oral Maxillofac Surg 2015.
the trachea using a GlideScope. The patient was placed on volume control ventilation with inhalational desflurane used for maintenance of general anesthesia. A peripheral nerve stimulator was applied to the right ulnar nerve. Train-of-4 stimuli were applied 5 minutes after administration of the paralytic and no visible or palpable twitches were noted. The parasymphyseal fracture was manually reduced and secured with a bridal wire. Then, the patient was placed in intermaxillary fixation (IMF) with 24-gauge stainless steel wire using an arch bar in the mandible and 4 intermaxillary fixation screws in the maxilla. Approximately 30 minutes after administration of the paralytic, train-of-4 stimuli were applied. Four strong twitches were noted in addition to sustained tetany. No neuromuscular reversal was given before extraoral skin incisions. The existing chin laceration was used to approach the parasymphyseal fracture and internal fixation was applied with 2 Biomet (Biomet Microfixation, Jacksonville, FL) titanium plates. The displaced right subcondylar fracture was approached through a preauricular incision with facial nerve monitoring. After right subcondylar fracture reduction, a single Biomet miniplate was used for fixation. IMF was released and the patient was found to passively go into occlusion. After layered closure of the surgical wounds, the patient was taken off mechanical ventilation and allowed to breathe spontaneously. Adequate tidal volumes (>550 mL) and rate (>10 breaths/minute) were observed. Before extubation, the patient was able to
follow verbal commands, including a sustained head lift longer than 5 seconds. No neuromuscular reversal agent was given before extubation. Surgical time was 150 minutes, during which fentanyl 200 mg, dexamethasone 10 mg, and ondansetron 4 mg were administered intravenously for postoperative pain, swelling, and nausea control. No other medications were given by the anesthesia team. Subsequent postanesthetic care was uneventful. The patient was discharged home the following day. The patient was seen in clinic for weekly follow-up appointments for 6 weeks postoperatively. Light elastic traction was used during the first 2 weeks to guide the patient into stable occlusion. After 2 weeks, the patient started a physical therapy regimen to improve mouth opening. After 4 weeks, the maxillary fixation screws and mandibular arch bar were removed and a soft diet was started. A postoperative Panorex radiograph displayed the hardware in place and good bony reduction of mandibular fracture segments (Fig 5). Six weeks after surgery, the patient’s interincisal opening was 40 mm and his diet was advanced to regular food.
Discussion CMTD is generally caused by gene mutations that affect the function of nerve cells. CMTD is broadly classified into categories based on subtypes that affect the myelin sheath (demyelinating type) and those that
SMITH ET AL
affect the nerve axon (axonal type). Type 1 CMTD is a demyelinating form, is inherited in autosomal dominant fashion, and causes muscle neuropathy owing to markedly decreased nerve conduction velocities. The clinical symptoms of CMTD typically begin with atrophy in the peroneal muscle, resulting in a ‘‘stork leg’’ or ‘‘inverted champagne bottle’’ lower extremity appearance. Orthopedic devices are often used to improve gait, but trauma related to mechanical falls is frequent. The mandibular fracture described in this report was the patient’s third serious traumatic event related to a fall after hip fractures in 2002 and 2010. Prolonged periods of limited mobility can drastically accelerate CMTD symptoms. Mobility also is important to maintain muscle strength and flexibility. In the setting of mandibular trauma and CMTD, efforts should be made to get the patient’s jaw and masticatory muscles functioning as soon as possible. The postoperative physical therapy program should focus on strengthening unaffected muscles that can help perform the work of those that have atrophied. Atrophy outside the distal musculature tends to occur in late stages of the disease. Before undergoing general anesthesia, special consideration should be given to the respiratory and cardiovascular systems. The respiratory status of the patient with CMTD should be thoroughly assessed because the incidence of dysfunction ranges from 0 to 30%.2 Pulmonary impairment is usually restrictive in nature, associated with weakness in the intercostal muscles and diaphragm.5 If diaphragmatic dysfunction is present, then there be could an increased risk of prolonged neuromuscular blockade. In addition, numerous case reports have shown an association between CMTD and phrenic nerve dysfunction and vocal cord dysfunction. As the disease progresses, paralysis of the vocal cords has been reported in certain subtypes of CMTD, placing these patients at higher risk for aspiration.4 Ideally, all patients with CMTD should undergo direct laryngeal examination, and it is recommended that all respiratory complaints should be thoroughly investigated. CMTD also has been reported to cause cardiac dysrhythmias and conduction disturbances in association with peripheral muscle atrophy.10 An increased incidence of mitral valve prolapse has been documented in several studies. Case reports have linked CMTD with cardiac disorders consisting mainly of supraventricular tachydysrhythmias and conduction defects.11 Although such sporadic case reports exist, prospective studies have not shown an increase in cardiac dysfunction compared with the population at large.10 It is generally believed that CMTD belongs in the group of neuromuscular disorders that are unassociated with cardiac muscle disease.10 When mitral valve prolapse is seen in patients with CMTD, electrocardiography and extensive review of systems should be conducted
1917.e5 to elicit possible congestive heart failure or cardiac decompensation. Owing to the wide variety of CMTD subtypes and clinical signs and symptoms associated with this chronic progressive disease, pulmonary and cardiac needs should be tailored to the individual patient and based on clinical judgment. Perioperative management in patients with CMTD involves judicious use of neuromuscular blocking agents. The chronic denervation associated with CMTD can predispose these patients to considerable release of potassium, and therefore hyperkalemia, after exposure to the depolarizing muscle relaxant succinylcholine (SCH).12 The hyperkalemia occurs through an upregulation of acetylcholine receptors at the neuromuscular junction and from the proliferation of extra-junctional receptors depolarized by SCH. In addition to hyperkalemia, SCH use in denervated muscle can produce sustained muscle contractions lasting several minutes. Case reports have cautioned against of the use of SCH for these reasons, but larger review studies have indicated that SCH is well tolerated.12 If paralysis with SCH is desired, then a small ‘‘defasciculating’’ dose of a nondepolarizing muscle relaxant could lessen the potassium release from diseased muscle.13 In the present case, the nondepolarizing muscle relaxant rocuronium was used to aid in intubation. Although the use of a nondepolarizing muscle relaxant avoids the possible complications associated with SCH, case reports have described prolonged paralysis after the administration of vecuronium.14 Repeated doses of neuromuscular paralysis reversal agents and prolonged periods of mechanical ventilation were required in these cases.9,14 A suitable site for peripheral nerve monitoring should be identified before administering the neuromuscular blocker. Monitoring of the facial nerve is often preferred over the ulnar and tibial nerves because of the clinical wasting seen in the distal extremities.14 Effective nerve monitoring can allow neuromuscular blocking agents to be better titrated to effect and adequate reversal signs can be identified. Agents with the potential to trigger malignant hyperthermia (MH) should be used with caution because a potential link with CMTD has been suggested.15 Although isolated case reports exist and several other neuromuscular diseases are associated with MH, current understanding of the pathophysiology of CMTD and MH does not support a connection between these 2 entities.12 In the present case, SCH was avoided and inhalational desflurane was successfully administered to maintain general anesthesia without any signs or manifestations of MH. Other intraoperative anesthetic considerations include cautious patient positioning and padding of pressure points because nerve compression can exacerbate the underlying neuropathy.16 In conclusion, no evidence of a prolonged response to
1917.e6 rocuronium was found in the present patient with CMTD. Induction and maintenance of general anesthesia were well tolerated and appear to be safe when appropriate preoperative and intraoperative measures are considered. Treatment of the bilateral mandible fracture with internal fixation and postoperative physical therapy resulted in restoration of premorbid function and occlusion.
References 1. Mendell J: Charcot Marie Tooth related disorders. Semin Neurol 18:41, 1998 2. Pareyson D, Marchesi C: Diagnosis, natural history, and management of Charcot-Marie-Tooth disease. Lancet Neurol 8:654, 2009 3. Sugai K, Sugai Y: Epidural anesthesia for a patient with CharcotMarie-Tooth disease, bronchial asthma and hypo- thyroidism. Masui 38:688, 1989 4. Sulica L, Blitzer A, Lovelace RE, et al: Vocal fold paresis of Charcot-Marie-Tooth disease. Ann Otol Rhinol Laryngol 110: 1072, 2001 5. Aboussouan LS, Lewis RA, Shy ME: Disorders of pulmonary function, sleep, and the upper airway in Charcot-Marie-Tooth disease. Lung 185:1, 2007
FRACTURE AND CHARCOT-MARIE-TOOTH DISEASE 6. Berger P, Young P, Suter U: Molecular cell biology of CharcotMarie-Tooth disease. Neurogenetics 4:1, 2002 7. Hirota K, Muraoka M, Sugihara K, et al: Anesthetic experience of a patient with Charcot-Marie-Tooth disease. Masui 37:207, 1988 8. Naguib M, Samarkandt AH: Response to atracurium and mivacurium in a patient with Charcot-Marie-Tooth disease. Can J Anaesth 45:56, 1998 9. Brian JE, Boyles GD, Quirk JG, et al: Anesthetic management for Cesarean section of a patient with Charcot-Marie-Tooth disease. Anesthesiology 66:410, 1987 10. Isner JM, Hawley RJ, Weintraub AM, et al: Cardiac findings in Charcot-Marie-Tooth disease: A prospective study of 68 patients. Arch Intern Med 139:1161, 1979 11. Tetzlaff JE, Schwendt I: Arrhythmia and Charcot-Marie-Tooth disease during anesthesia. Can J Anaesth 47:829, 2000 12. Antognini JF: Anaesthesia for Charcot-Marie-Tooth disease: A review of 86 cases. Can J Anaesth 39:398, 1992 13. Gronert GA, Lambert EH, Theye RA: The response of denervated skeletal muscle to succinylcholine. Anesthesiology 39:13, 1973 14. Pogson D, Tilfer J, Wimbush S: Prolonged vecuronium neuromuscular blockade associated with Charcot Marie Tooth neuropathy. Br J Anaesth 85:914, 2000 15. Roelofse JA, Shipton EA: Anesthesia for abdominal hysterectomy in Charcot-Marie-Tooth disease. A case report. S Afr Med J 67: 605, 1985 16. Pasha TM, Knowles A: Anaesthetic management of a patient with Charcot-Marie-Tooth disease for staged diaphragmatic plication. Br J Anaesth 110:1061, 2013
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