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Perioperative care of the child with arthrogryposis
1Department of Anesthesiology, University of Missouri, Columbia, MO 65212, USA.
Insights
Perioperative care for arthrogryposis multiplex congenita (AMC) patients requires attention to airway management, IV access, and hyperthermia. These common issues impact surgical outcomes in AMC patients.
Area of Science:
- Anesthesiology
- Pediatric Surgery
- Genetics and Inherited Diseases
Background:
- Arthrogryposis multiplex congenita (AMC) is a congenital condition characterized by multiple joint contractures, often stemming from fetal akinesia.
- Fetal akinesia in AMC can be caused by extrinsic factors like oligohydramnios or intrinsic fetal conditions, including neurogenic or myopathic disorders.
Purpose of the Study:
- To retrospectively analyze the perioperative experience of patients diagnosed with arthrogryposis multiplex congenita (AMC).
- To identify common intraoperative and postoperative challenges encountered in the surgical management of AMC patients.
Main Methods:
- Retrospective review of perioperative data from 12 patients with arthrogryposis multiplex congenita (AMC).
- Documentation and analysis of intraoperative events, including intravenous access, airway management, and temperature fluctuations.
- Recording of postoperative complications such as stridor and atelectasis.
Main Results:
- Difficulties were noted in peripheral IV placement (3/12 patients), tracheal intubation (4/12 patients), and intraoperative hyperthermia (4/12 patients).
- Tracheal intubation challenges were more prevalent in older AMC patients (11.0 +/- 2.9 years) compared to those without intubation difficulties (5.8 +/- 3.9 years).
- Postoperative complications included stridor (2/12) and atelectasis (3/12).
Conclusions:
- Perioperative management of AMC patients must address physical manifestations and underlying neuromuscular disease implications.
- Key perioperative challenges include airway management difficulties, intravenous access problems, and intraoperative hyperthermia.
- Proactive strategies are essential for optimizing surgical outcomes in patients with arthrogryposis multiplex congenita.
Background:
Arthrogryposis multiplex congenita (AMC) is a congenital symptom complex characterized by multiple joint contractures. AMC results from fetal akinesia due to extrinsic factors (oligohydramnios) or primary neurogenic or myopathic conditions of the fetus.
Methods:
We retrospectively reviewed our perioperative experience in 12 patients with AMC.
Results:
Intraoperative issues noted in our patients included difficulties with peripheral IV placement (n = 3), difficult tracheal intubation (n = 4), and intraoperative hyperthermia (n = 4). Difficulties with peripheral IV placement were more common in younger patients (2.3 +/- 0.6 vs 9.2 +/- 3.5 years). Although easy bag-valve-mask ventilation was present in all 12 patients, difficulty with tracheal intubation was noted in four patients. In these four patients, the airway was secured using an LMA (n = 3) or a facemask (n = 1). The patients in whom difficulties with tracheal intubation were encountered were older than patients in whom no difficulties were noted (11.0 +/- 2.9 vs 5.8 +/- 3.9 years). Four patients developed intraoperative temperatures > or =37 degrees C; however, there was no evidence of hypercarbia or tachycardia suggestive of malignant hyperthermia. Postoperative issues included stridor (n = 2) and postoperative atelectasis (n = 3).
Conclusions:
The perioperative care of patients with AMC should consider not only the physical manifestations of the disease process, but also the implications of the underlying neuromuscular disease. The most common perioperative issues included difficulties with airway management, problematic intravenous access, and intraoperative hyperthermia.
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