Acta Anaesthesiologica Taiwanica xxx (2014) 1e3
Contents lists available at ScienceDirect
Acta Anaesthesiologica Taiwanica journal homepage: www.e-aat.com
Case Report
Arthrogryposis multiplex congenita: Airway concerns in an emergency situation Babita Gupta*, Saurabh Suri, Santvana Kohli, Suma Ahmad, Surender Gupta Department of Anaesthesiology, Jai Prakash Narayan Apex Trauma Center (JPNATC), All India Institute of Medical Sciences (AIIMS), New Delhi, India
a r t i c l e i n f o
a b s t r a c t
Article history: Received 9 October 2013 Accepted 4 December 2013
Difficult airway is always of special concern to anesthesiologists, but in a trauma setting where having a secured airway is most important, the incidence of difficult airway increases manifold. We report a “cannot ventilate cannot intubate” situation in a trauma patient who was later diagnosed to have arthrogryposis multiplex congenita, a syndrome known to affect the airway, and in whom all measures of securing a nonsurgical airway failed. Copyright Ó 2014, Taiwan Society of Anesthesiologists. Published by Elsevier Taiwan LLC. All rights reserved.
Key words: airway assessment; arthrogryposis; difficult airway
1. Introduction Arthrogryposis multiplex congenita (AMC) is a rare, nonprogressive congenital disorder that causes multiple joint contractures, and can be associated with muscle weakness and fibrosis.1 The disease derives its name from Greek, literally meaning “curved or hooked joints.”2 Anesthetic concerns include difficult intravenous access, difficult airway, difficult positioning, and association with other congenital disorders such as congenital heart diseases, gastroschisis, genitourinary defects, and restrictive pulmonary defects.2 Difficult airway, which is more common in a trauma patient, becomes a greater management concern in patients having AMC. We report a case of a 26-year-old female who was brought to our emergency room with severe head injury. She was unable to be ventilated or intubated, and surgical airway was established with difficulty. She was later diagnosed as a case of AMC. 2. Case report A 26-year-old female was brought to the emergency room with a history of motor vehicle accident. On primary survey, she was unresponsive, with a Glasgow Coma Score of E1V1M4. She also had rapid and shallow respiration (respiratory rate >35/minute) with
Conflicts of interest: All authors declare no conflicts of interest. * Corresponding author. Room No. 303, Department of Anaesthesiology, Jai Prakash Narayan Apex Trauma Center (JPNATC), All India Institute of Medical Sciences (AIIMS), New Delhi 110029, India. E-mail address: [email protected] (B. Gupta).
airway obstruction, and pulse oximetry showed oxygen saturation of 75%. Bag valve mask ventilation was initiated immediately to provide oxygenation; however, ventilation could not be established. Endotracheal intubation with a 7.0 mm ID portex tube with stylet in situ using direct laryngoscopy with Macintosh blade number 3 was attempted after administering 100 mg fentanyl, 10 mg etomidate, and 100 mg succinylcholine intravenously. However, the epiglottis and glottis could not be visualized (Cormack-Lehane grade IV), rendering the laryngoscopy and intubation almost impossible. Restricted mouth opening attributed to the difficulty in laryngoscopy and intubation. After three attempts of intubation with two different laryngoscopes and blades (Macintosh and McCoy) by two experienced anesthesiologists, ProSeal laryngeal mask airway insertion was tried. However, restricted mouth opening and severe fibrosis made its placement and manipulation almost impossible. In view of the inability to ventilate or intubate the patient, the emergent pathway of “cannot ventilate cannot intubate” was followed, and an emergency cricothyroidotomy with a cricothyroidotomy needle set of 4.0 mm (Portex) was performed. Oxygen saturation increased from 70% to 95% after being ventilated through an Artificial Manual Breathing Unit (AMBU) bag, and a decision to perform a surgical tracheostomy in the operation room was taken. The surgical tracheostomy was performed with a 7.0 mm tracheostomy tube, and the patient was further evaluated. A computed tomography scan of the head revealed a massive subdural hematoma and multiple brain contusions for which surgical evacuation and decompression was done. The patient was hemodynamically stable intraoperatively. Two days later, she was declared brain dead, which probably could be attributed to traumatic brain injury compounded with hypoxic insult, and thereafter
http://dx.doi.org/10.1016/j.aat.2014.04.003 1875-4597/Copyright Ó 2014, Taiwan Society of Anesthesiologists. Published by Elsevier Taiwan LLC. All rights reserved.
Please cite this article in press as: Gupta B, et al., Arthrogryposis multiplex congenita: Airway concerns in an emergency situation, Acta Anaesthesiologica Taiwanica (2014), http://dx.doi.org/10.1016/j.aat.2014.04.003
2
B. Gupta et al.
was scheduled for organ harvesting. On close examination of the patient, she was observed to have apparent bony deformities affecting all four limbs, bilateral club foot, mandibular hypoplasia, micrognathia, high arched palate, and restricted temporomandibular joint movements (Fig. 1AeC). She also had micrognathia, which along with restricted temporomandibular joint movements probably caused difficult ventilation. Fiberoptic bronchoscopy and laryngoscopy with a C-mac videolaryngoscope were performed, to evaluate the airway and establish the cause of difficulty; however, due to the presence of mucous and thick fibrotic bands in the oral cavity, the epiglottis or the vocal cords could not be visualized (Fig. 2). All these findings suggested that the patient had AMC, which was the cause of failed ventilation and failed intubation. 3. Discussion
Fig. 1. (a) Showing club hand. (b) Showing club foot. (c) Showing mandibular hypoplasia.
Fig. 2. C-mac videolaryngoscopic view showing thick fibrotic bands and nonvisualisation of epiglottis and vocal cords.
AMC is a nonprogressive congenital syndrome complex characterized by contracture of several joints in different parts of the body due to varying degrees of fibrosis of the affected muscles, and thickening and shortening of periarticular capsular and ligamentous tissues.1,2 The primary concern of an anesthesiologist is the potential for airway involvement in such patients, thereby making direct laryngoscopy and endotracheal intubation difficult. Extensive contractures, tense skin, and minimal muscle mass and subcutaneous tissue pose further challenges for positioning prior to ventilation, laryngoscopy, and endotracheal intubation.1e4 Direct laryngoscopy and intubation become more difficult as the patient ages because craniofacial involvement often progresses with growth.5 Trauma commonly compromises the airway in all patients and amplifies the difficulty in this subset of patients; the problem is further compounded in emergency situations. There have been many case reports describing the anesthetic management for elective operative procedures in patients with AMC,6e9 but a situation of “cannot ventilate cannot intubate” in a trauma setting has not been discussed previously. A difficult or rather impossible laryngoscopy and/or intubation can be anticipated in up to 25% of AMC patients. Small mouth opening, micrognathia, high-arched palate, decreased tongue mobility, and short neck with restricted mobility are all causal factors of difficult intubation. Our patient had all the above features, which were only noticed later. Although a trauma patient does not give enough time for detailed evaluation of airway, a rapid assessment of the patient would have helped in anticipating difficult airway. Look criteria of the Lemon Law are important components of airway assessment. Available data suggest that Laryngeal Mask Airway (LMA) insertion and mask ventilation are possible in nearly all cases of AMC, except in one case reported by Mentzelopoulos et al,7 describing failure of ventilation through LMA and unsuccessful fiberoptic bronchoscopy. Their patient underwent magnetic resonance imaging, which revealed long, counterclockwise-rotated, and left-shifted epiglottis; clockwise-rotated hyoid; and short epiglottis tip-toretropharyngeal wall distancedabnormalities that probably led to intubation failure. The patient was rescheduled for surgery, and left molar McCoy-balloon laryngoscopy was described to overcome the anatomic abnormality. This technique was not possible in our patient, as it was an emergency situation and the process would have required time. It has also been recommended to have the pediatric airway equipment available to manage the airway in these patients when intubation is indispensable. Cricothyroidotomy performed by trained personnel enabled us to manage the airway crises. In view of the special considerations for the difficult airway in a trauma patient where there is no option for cancelling the case or awakening the patient, a decision to establish a surgical airway should be taken early instead of making multiple unsuccessful
Please cite this article in press as: Gupta B, et al., Arthrogryposis multiplex congenita: Airway concerns in an emergency situation, Acta Anaesthesiologica Taiwanica (2014), http://dx.doi.org/10.1016/j.aat.2014.04.003
Arthrogryposis multiplex congenita
attempts of intubation by laryngoscopy or other means.10 In patients with features suggestive of AMC, it is recommended to secure the airway by awake intubation or awake tracheostomy when the airway is compromised. In the rare but possible event of a difficult surgical tracheostomy, a percutaneous dilatational technique, which involves cricothyroidotomy, dilatation of the passage, and insertion of a small tube, is described.11 A retrograde intubation technique can also be attempted. Finally, all attending residents and physicians should be well versed with recent guidelines in airway management and develop reasonable skill in performing emergency airway techniques such as cricothyroidotomy.
References 1. Epstein JB, Wittenberg GJ. Maxillofacial manifestations and management of arthrogryposis: literature review and case report. J Oral Maxillofacial Surg 1987;45:274e9. 2. Oberoi GS, Kaul HL, Gill IS. Anaesthesia in arthrogryposis multiplex congenita. Can J Anaesth 1987;34:288e90.
3 3. Thomas JA, Chiu-Yeh M, Moriconi ES. Maxillofacial implications and surgical treatment of arthrogryposis multiplex congenita. Compend Contin Educ Dent 2001;22:588e92. 4. Martin S, Tobias JD. Perioperative care of the child with arthrogryposis. Pediatr Anesth 2006;16:31e7. 5. Jedge P, Aphale S, Bhosale P, Bapat S. Anaesthetic management of a child with arthrogryposis multiplex congenita. Internet J Anesthesiol 2008;21. 6. Thomas PB, Parry MG. The difficult airway: a new method of intubation using the laryngeal mask airway. Cook airway exchange catheter and tracheal intubation fiberscope. Paediatr Anaesth 2001;11:618e21. 7. Mentzelopoulos SD, Armaganidis A, Niokou D, Matsota P, Tzoufi M, Kelekis N, et al. MRI of the upper airway and McCoy-balloon laryngoscopy with left molar approach in a patient with arthrogryposis multiplex congenita and previous unsuccessful endotracheal intubation. Anaesth Analg 2004;99:1879e80. 8. Nguyen NH, Morvant EM, Mayhew JF. Anesthetic management for patients with arthrogryposis multiplex congenita and severe micrognathia: case reports. J Clin Anesth 2000;12:227e30. 9. Chowdhuri R, Samui S, Kundu AK. Anesthetic management of a neonate with arthrogryposis multiplex congenita for emergency laparotomy. J Anaesthesiol Clin Pharmacol 2011;27:244e6. 10. Wilson WC. ASA difficult airway algorithm modified for trauma. Anaesthesiology 2005;69:9e10. 11. Kumar M, Khanna A, Verma S, Jha A, Aggarwal S. Difficult tracheostomy in a case of difficult mask ventilation and difficult intubation? J Anaesthesiol Clin Pharmacol 2013;29:576e7.
Please cite this article in press as: Gupta B, et al., Arthrogryposis multiplex congenita: Airway concerns in an emergency situation, Acta Anaesthesiologica Taiwanica (2014), http://dx.doi.org/10.1016/j.aat.2014.04.003
Contents lists available at ScienceDirect
Acta Anaesthesiologica Taiwanica journal homepage: www.e-aat.com
Case Report
Arthrogryposis multiplex congenita: Airway concerns in an emergency situation Babita Gupta*, Saurabh Suri, Santvana Kohli, Suma Ahmad, Surender Gupta Department of Anaesthesiology, Jai Prakash Narayan Apex Trauma Center (JPNATC), All India Institute of Medical Sciences (AIIMS), New Delhi, India
a r t i c l e i n f o
a b s t r a c t
Article history: Received 9 October 2013 Accepted 4 December 2013
Difficult airway is always of special concern to anesthesiologists, but in a trauma setting where having a secured airway is most important, the incidence of difficult airway increases manifold. We report a “cannot ventilate cannot intubate” situation in a trauma patient who was later diagnosed to have arthrogryposis multiplex congenita, a syndrome known to affect the airway, and in whom all measures of securing a nonsurgical airway failed. Copyright Ó 2014, Taiwan Society of Anesthesiologists. Published by Elsevier Taiwan LLC. All rights reserved.
Key words: airway assessment; arthrogryposis; difficult airway
1. Introduction Arthrogryposis multiplex congenita (AMC) is a rare, nonprogressive congenital disorder that causes multiple joint contractures, and can be associated with muscle weakness and fibrosis.1 The disease derives its name from Greek, literally meaning “curved or hooked joints.”2 Anesthetic concerns include difficult intravenous access, difficult airway, difficult positioning, and association with other congenital disorders such as congenital heart diseases, gastroschisis, genitourinary defects, and restrictive pulmonary defects.2 Difficult airway, which is more common in a trauma patient, becomes a greater management concern in patients having AMC. We report a case of a 26-year-old female who was brought to our emergency room with severe head injury. She was unable to be ventilated or intubated, and surgical airway was established with difficulty. She was later diagnosed as a case of AMC. 2. Case report A 26-year-old female was brought to the emergency room with a history of motor vehicle accident. On primary survey, she was unresponsive, with a Glasgow Coma Score of E1V1M4. She also had rapid and shallow respiration (respiratory rate >35/minute) with
Conflicts of interest: All authors declare no conflicts of interest. * Corresponding author. Room No. 303, Department of Anaesthesiology, Jai Prakash Narayan Apex Trauma Center (JPNATC), All India Institute of Medical Sciences (AIIMS), New Delhi 110029, India. E-mail address: [email protected] (B. Gupta).
airway obstruction, and pulse oximetry showed oxygen saturation of 75%. Bag valve mask ventilation was initiated immediately to provide oxygenation; however, ventilation could not be established. Endotracheal intubation with a 7.0 mm ID portex tube with stylet in situ using direct laryngoscopy with Macintosh blade number 3 was attempted after administering 100 mg fentanyl, 10 mg etomidate, and 100 mg succinylcholine intravenously. However, the epiglottis and glottis could not be visualized (Cormack-Lehane grade IV), rendering the laryngoscopy and intubation almost impossible. Restricted mouth opening attributed to the difficulty in laryngoscopy and intubation. After three attempts of intubation with two different laryngoscopes and blades (Macintosh and McCoy) by two experienced anesthesiologists, ProSeal laryngeal mask airway insertion was tried. However, restricted mouth opening and severe fibrosis made its placement and manipulation almost impossible. In view of the inability to ventilate or intubate the patient, the emergent pathway of “cannot ventilate cannot intubate” was followed, and an emergency cricothyroidotomy with a cricothyroidotomy needle set of 4.0 mm (Portex) was performed. Oxygen saturation increased from 70% to 95% after being ventilated through an Artificial Manual Breathing Unit (AMBU) bag, and a decision to perform a surgical tracheostomy in the operation room was taken. The surgical tracheostomy was performed with a 7.0 mm tracheostomy tube, and the patient was further evaluated. A computed tomography scan of the head revealed a massive subdural hematoma and multiple brain contusions for which surgical evacuation and decompression was done. The patient was hemodynamically stable intraoperatively. Two days later, she was declared brain dead, which probably could be attributed to traumatic brain injury compounded with hypoxic insult, and thereafter
http://dx.doi.org/10.1016/j.aat.2014.04.003 1875-4597/Copyright Ó 2014, Taiwan Society of Anesthesiologists. Published by Elsevier Taiwan LLC. All rights reserved.
Please cite this article in press as: Gupta B, et al., Arthrogryposis multiplex congenita: Airway concerns in an emergency situation, Acta Anaesthesiologica Taiwanica (2014), http://dx.doi.org/10.1016/j.aat.2014.04.003
2
B. Gupta et al.
was scheduled for organ harvesting. On close examination of the patient, she was observed to have apparent bony deformities affecting all four limbs, bilateral club foot, mandibular hypoplasia, micrognathia, high arched palate, and restricted temporomandibular joint movements (Fig. 1AeC). She also had micrognathia, which along with restricted temporomandibular joint movements probably caused difficult ventilation. Fiberoptic bronchoscopy and laryngoscopy with a C-mac videolaryngoscope were performed, to evaluate the airway and establish the cause of difficulty; however, due to the presence of mucous and thick fibrotic bands in the oral cavity, the epiglottis or the vocal cords could not be visualized (Fig. 2). All these findings suggested that the patient had AMC, which was the cause of failed ventilation and failed intubation. 3. Discussion
Fig. 1. (a) Showing club hand. (b) Showing club foot. (c) Showing mandibular hypoplasia.
Fig. 2. C-mac videolaryngoscopic view showing thick fibrotic bands and nonvisualisation of epiglottis and vocal cords.
AMC is a nonprogressive congenital syndrome complex characterized by contracture of several joints in different parts of the body due to varying degrees of fibrosis of the affected muscles, and thickening and shortening of periarticular capsular and ligamentous tissues.1,2 The primary concern of an anesthesiologist is the potential for airway involvement in such patients, thereby making direct laryngoscopy and endotracheal intubation difficult. Extensive contractures, tense skin, and minimal muscle mass and subcutaneous tissue pose further challenges for positioning prior to ventilation, laryngoscopy, and endotracheal intubation.1e4 Direct laryngoscopy and intubation become more difficult as the patient ages because craniofacial involvement often progresses with growth.5 Trauma commonly compromises the airway in all patients and amplifies the difficulty in this subset of patients; the problem is further compounded in emergency situations. There have been many case reports describing the anesthetic management for elective operative procedures in patients with AMC,6e9 but a situation of “cannot ventilate cannot intubate” in a trauma setting has not been discussed previously. A difficult or rather impossible laryngoscopy and/or intubation can be anticipated in up to 25% of AMC patients. Small mouth opening, micrognathia, high-arched palate, decreased tongue mobility, and short neck with restricted mobility are all causal factors of difficult intubation. Our patient had all the above features, which were only noticed later. Although a trauma patient does not give enough time for detailed evaluation of airway, a rapid assessment of the patient would have helped in anticipating difficult airway. Look criteria of the Lemon Law are important components of airway assessment. Available data suggest that Laryngeal Mask Airway (LMA) insertion and mask ventilation are possible in nearly all cases of AMC, except in one case reported by Mentzelopoulos et al,7 describing failure of ventilation through LMA and unsuccessful fiberoptic bronchoscopy. Their patient underwent magnetic resonance imaging, which revealed long, counterclockwise-rotated, and left-shifted epiglottis; clockwise-rotated hyoid; and short epiglottis tip-toretropharyngeal wall distancedabnormalities that probably led to intubation failure. The patient was rescheduled for surgery, and left molar McCoy-balloon laryngoscopy was described to overcome the anatomic abnormality. This technique was not possible in our patient, as it was an emergency situation and the process would have required time. It has also been recommended to have the pediatric airway equipment available to manage the airway in these patients when intubation is indispensable. Cricothyroidotomy performed by trained personnel enabled us to manage the airway crises. In view of the special considerations for the difficult airway in a trauma patient where there is no option for cancelling the case or awakening the patient, a decision to establish a surgical airway should be taken early instead of making multiple unsuccessful
Please cite this article in press as: Gupta B, et al., Arthrogryposis multiplex congenita: Airway concerns in an emergency situation, Acta Anaesthesiologica Taiwanica (2014), http://dx.doi.org/10.1016/j.aat.2014.04.003
Arthrogryposis multiplex congenita
attempts of intubation by laryngoscopy or other means.10 In patients with features suggestive of AMC, it is recommended to secure the airway by awake intubation or awake tracheostomy when the airway is compromised. In the rare but possible event of a difficult surgical tracheostomy, a percutaneous dilatational technique, which involves cricothyroidotomy, dilatation of the passage, and insertion of a small tube, is described.11 A retrograde intubation technique can also be attempted. Finally, all attending residents and physicians should be well versed with recent guidelines in airway management and develop reasonable skill in performing emergency airway techniques such as cricothyroidotomy.
References 1. Epstein JB, Wittenberg GJ. Maxillofacial manifestations and management of arthrogryposis: literature review and case report. J Oral Maxillofacial Surg 1987;45:274e9. 2. Oberoi GS, Kaul HL, Gill IS. Anaesthesia in arthrogryposis multiplex congenita. Can J Anaesth 1987;34:288e90.
3 3. Thomas JA, Chiu-Yeh M, Moriconi ES. Maxillofacial implications and surgical treatment of arthrogryposis multiplex congenita. Compend Contin Educ Dent 2001;22:588e92. 4. Martin S, Tobias JD. Perioperative care of the child with arthrogryposis. Pediatr Anesth 2006;16:31e7. 5. Jedge P, Aphale S, Bhosale P, Bapat S. Anaesthetic management of a child with arthrogryposis multiplex congenita. Internet J Anesthesiol 2008;21. 6. Thomas PB, Parry MG. The difficult airway: a new method of intubation using the laryngeal mask airway. Cook airway exchange catheter and tracheal intubation fiberscope. Paediatr Anaesth 2001;11:618e21. 7. Mentzelopoulos SD, Armaganidis A, Niokou D, Matsota P, Tzoufi M, Kelekis N, et al. MRI of the upper airway and McCoy-balloon laryngoscopy with left molar approach in a patient with arthrogryposis multiplex congenita and previous unsuccessful endotracheal intubation. Anaesth Analg 2004;99:1879e80. 8. Nguyen NH, Morvant EM, Mayhew JF. Anesthetic management for patients with arthrogryposis multiplex congenita and severe micrognathia: case reports. J Clin Anesth 2000;12:227e30. 9. Chowdhuri R, Samui S, Kundu AK. Anesthetic management of a neonate with arthrogryposis multiplex congenita for emergency laparotomy. J Anaesthesiol Clin Pharmacol 2011;27:244e6. 10. Wilson WC. ASA difficult airway algorithm modified for trauma. Anaesthesiology 2005;69:9e10. 11. Kumar M, Khanna A, Verma S, Jha A, Aggarwal S. Difficult tracheostomy in a case of difficult mask ventilation and difficult intubation? J Anaesthesiol Clin Pharmacol 2013;29:576e7.
Please cite this article in press as: Gupta B, et al., Arthrogryposis multiplex congenita: Airway concerns in an emergency situation, Acta Anaesthesiologica Taiwanica (2014), http://dx.doi.org/10.1016/j.aat.2014.04.003
