Anaesthetic management in a patient with arthrogryposis multiplex congenita

Novoa del Valle, C.; Taboada, M.; Rodríguez, J.; Quevedo, J.E.

doi:10.1016/j.redare.2020.12.005

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Novoa del Valle, M. Taboada, J. Rodríguez, J.E. Quevedo" "autores" => array:4 [ 0 => array:4 [ "nombre" => "C." "apellidos" => "Novoa del Valle" "email" => array:1 [ 0 => "carmennovoavalle@gmail.com" ] "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "*" "identificador" => "cor0005" ] ] ] 1 => array:2 [ "nombre" => "M." "apellidos" => "Taboada" ] 2 => array:2 [ "nombre" => "J." "apellidos" => "Rodríguez" ] 3 => array:2 [ "nombre" => "J.E." "apellidos" => "Quevedo" ] ] "afiliaciones" => array:1 [ 0 => array:2 [ "entidad" => "Departamento de Anestesiología y Reanimación, Complejo Hospitalario Universitario de Santiago de Compostela, Santiago de Compostela, Spain" "identificador" => "aff0005" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Manejo anestésico en un paciente con artrogriposis múltiple congénita" ] ] "textoCompleto" => "Arthrogryposis multiplex congenita (AMC) is a rare, congenital disease present at birth, characterized by limited joint movement or multiple joint contractures. Abnormal mobility is due to fibrous ankylosis, overgrowth, and proliferation of fibrous tissue in the joints. AMC usually affects both the upper and lower extremities, and is associated with complications such as scoliosis, pulmonary hypoplasia, respiratory problems, developmental delay, hemangioma in the midface, facial and mandibular alterations, and abdominal hernias<a class="elsevierStyleCrossRef" href="#bib0005">1</a>. AMC is a rare disease (1/3000 live births) with an unknown aetiology. In Spain, it is estimated to occur in 1/10,000 live births, but there are practically no references in the literature, and few studies or patient records<a class="elsevierStyleCrossRef" href="#bib0010">2</a>.This type of patient is a challenge for the anaesthesiologist, because AMC is associated with a higher incidence of hypotension, respiratory depression, hypotonia, prolonged anaesthetic action, poor peripheral airway, risk of malignant hyperthermia and greater difficulty in airway management<a class="elsevierStyleCrossRef" href="#bib0005">1</a>. Although the risk of malignant hyperthermia is disputed, evidence has confirmed the appearance of a nonspecific intraoperative hypermetabolic reaction with no defined triggers that is controlled with symptomatic measures. The use of succinylcholine can be controversial.We report the case of a 37-year-old man with an avulsion fracture of the upper pole of the left patella who was scheduled for cerclage wire fixation after obtaining written informed consent. His personal history included AMC amyoplasia or classic arthrogryposis since childhood, and he had undergone multiple orthopaedic surgeries to correct his limb deformities. He also presented epilepsy treated with valproic acid, and had been seizure-free for 8 years. The physical examination revealed malformation of the craniofacial skeleton and the upper and lower extremities, and moderate lumbar scoliosis. The airway assessment showed Mallampati 4 with a 1-finger buccal opening, bite test 3, and a thyromental distance of more than 6 cm with good cervical mobility, but torticollis.Upon arrival in the operating room, pulse oximetry, electrocardiogram and non-invasive blood pressure monitoring was started. The patient was preoxygenated with nasal cannulas at 2 lpm, then sedated with 1.5 mg midazolam and 50 μg intravenous fentanyl, and given 2 g intravenous cefazolin for antibiotic prophylaxis. Given his difficult airway and lumbar scoliosis, we decided to perform the surgery under locoregional anaesthesia. Ultrasound-guided left femoral block was performed with 20 mL 0.75% ropivacaine and ultrasound-guided left sciatic block at the subgluteal level - complemented with neurostimulation due to poor ultrasound vision - with 20 mL 1.5% mepivacaine. The ultrasound system used was a BK Flex Focus 1202 with an 8870 linear transducer, and an 80 mm Pajunk SonoPlex 22G needle was inserted in-plane in both nerve blocks. Poor ultrasound vision was due to the replacement of muscle fibres with fibrous, fatty tissue that changes the echogenicity of the muscle and prevents correct identification of the nerve<a class="elsevierStyleCrossRef" href="#bib0015">3</a>. Neurostimulation was performed using the Pajunk MultiStim Sensor neurostimulator with an intensity of between 1 and 1.5 mA and a frequency of 2 Hz delivered for 0.1 ms. This achieves the desired motor response, although joint contractures and immobility can sometimes obstruct this response. Both techniques were well tolerated and did not cause paresthesia. The intraoperative period was uneventful and analgesia was performed with intravenous dexketoprofen and paracetamol.Patients with AMC present several problems for the anaesthesiologist, including difficult airway due to micrognathia, a short neck, and associated malformations. Myopathy associated with skeletal deformities causes respiratory problems, which are aggravated by the presence of scoliosis. This increases the work of breathing and the use of accessory muscles of respiration<a class="elsevierStyleCrossRef" href="#bib0010">2</a>. Due to their low muscle mass and neuropathic and neurogenic changes, AMC patients are prone to respiratory depression, hypotonia, and prolonged action of both inhalational and intravenous anaesthetics. In addition, their myopathy can elicit abnormal reactions to muscle relaxants. Whenever possible, it is advisable to combine narcosis with regional anaesthesia in patients with AMC, because this reduces anaesthetic demand, stabilises circulation, maintains spontaneous breathing, prevents stress, and provides perioperative pain relief<a class="elsevierStyleCrossRefs" href="#bib0015">3,4</a>.Locoregional anaesthesia is beneficial in high-risk patients. Nerve block is best performed under ultrasound-guidance, which is not impaired by muscle contractures and joint immobility<a class="elsevierStyleCrossRef" href="#bib0015">3</a>. Although the presence of spinal deformities and joint contractures in the extremities can complicate the administration of regional anaesthesia, some cases of successful spinal anaesthesia for caesarean section in adults with AMC or for tibia fracture in a paediatric patient have been published<a class="elsevierStyleCrossRef" href="#bib0020">4</a>. Tuta et al. administered an iliac fascia block in a neonate with AMC to perform a muscle biopsy, thereby avoiding the risks associated with general anaesthesia<a class="elsevierStyleCrossRef" href="#bib0025">5</a>.Patients with AMC can pose quite a challenge to the anaesthesiologist. They can present many of the complications associated with general anaesthesia, in addition to difficult airway. It is important to perform a thorough preoperative evaluation to anticipate these complications and prepare a good anaesthesia management plan. In our case, the alternative plan included awake intubation with fibreoptic bronchoscope due to the presence of a difficult airway, and we had prepared everything that would be required for this eventuality. Regional anaesthesia is a good alternative and provides effective postoperative analgesia. In this case in particular, the association of ultrasound and neurostimulation was fundamental due to the patient’s characteristics<a class="elsevierStyleCrossRef" href="#bib0015">3</a>." "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "Please cite this article as: Novoa del Valle C, Taboada M, Rodríguez J, Quevedo JE. Manejo anestésico en un paciente con artrogriposis múltiple congénita. Rev Esp Anestesiol Reanim. 2022;69:54–55." ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:5 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Anesthesia in patients with arthrogryposis multiplex congénita: a report of ten patients" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:4 [ 0 => "J. Woong Jung" 1 => "B. Young Heo" 2 => "E. Jung Oh" 3 => "Y. Hoon Chung" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.4097/kjae.2014.67.S.S89" "Revista" => array:8 [ "tituloSerie" => "Korean J Anesthesiol." "fecha" => "2014" "volumen" => "67" "numero" => "Suppl" "paginaInicial" => "S89" "paginaFinal" => "S90" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/25598924" "web" => "Medline" ] ] "itemHostRev" => array:3 [ "pii" => "S0026049517302160" "estado" => "S300" "issn" => "00260495" ] ] ] ] ] ] ] 1 => array:3 [ "identificador" => "bib0010" "etiqueta" => "2" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Artrogryposis: an update on clinical aspects, etiology and treatment strategies" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:2 [ 0 => "B. Kowalczyk" 1 => "J. Felus" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.5114/aoms.2016.57578" "Revista" => array:5 [ "tituloSerie" => "Arch Medical Sci." "fecha" => "2016" "volumen" => "12" "paginaInicial" => "10" "paginaFinal" => "24" ] ] ] ] ] ] 2 => array:3 [ "identificador" => "bib0015" "etiqueta" => "3" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Comparison of success rate of ultrasound-guided sciatic and femoral nerve block and neurostimulation in children with arthrogryposis multiplex congenita: a randomized clinical trial" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "V. Ponde" 1 => "A.P. Desai" 2 => "D. Shah" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1111/pan.12022" "Revista" => array:6 [ "tituloSerie" => "Paediatr Anaesth." 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"fecha" => "2012" "volumen" => "26" "paginaInicial" => "283" "paginaFinal" => "285" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/22354670" "web" => "Medline" ] ] "itemHostRev" => array:3 [ "pii" => "S0749379716303853" "estado" => "S300" "issn" => "07493797" ] ] ] ] ] ] ] 4 => array:3 [ "identificador" => "bib0025" "etiqueta" => "5" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Fascia iliaca block for an infant with arthrogryposis multiplex congénita undergoing muscle biopsy" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:4 [ 0 => "I. Tuta" 1 => "S.D. Cook-Sather" 2 => "R.S. Finkel" 3 => "G. Cucchiaro" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1213/01.ANE.0000138036.83973.9A" "Revista" => array:6 [ "tituloSerie" => "Anesth Analg." "fecha" => "2005" "volumen" => "100" "paginaInicial" => "82" "paginaFinal" => "84" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/15616056" "web" => "Medline" ] ] ] ] ] ] ] ] ] ] ] ] ] "idiomaDefecto" => "en" "url" => "/23411929/0000006900000001/v1_202201270542/S234119292200004X/v1_202201270542/en/main.assets" "Apartado" => array:4 [ "identificador" => "66474" "tipo" => "SECCION" "en" => array:2 [ "titulo" => "Letter to the Director" "idiomaDefecto" => true ] "idiomaDefecto" => "en" ] "PDF" => "https://static.elsevier.es/multimedia/23411929/0000006900000001/v1_202201270542/S234119292200004X/v1_202201270542/en/main.pdf?idApp=UINPBA00004N&text.app=https://www.elsevier.es/" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S234119292200004X?idApp=UINPBA00004N"]

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Revista Española de Anestesiología y Reanimación (REDAR) -the Spanish Journal of Anesthesiology and Resuscitation- is the scientific body of the Spanish Society of Anesthesiology, Resuscitation and Pain Therapy. This is a monthly journal (10 issues) that publishes scientific articles of all areas within its specialty: clinical anesthesia, resuscitation-intensive medicine and critical care, diagnosis and treatment of acute and chronic pain, urgent care and emergencies and studies on basic science and related fields. The journal accepts work in both Spanish and English.

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Vol. 69. Issue 1.