Anaesthetic Management of an Achondroplastic Dwarf with Difficult Airway and Spine for Total Hip Replacement: A Case Report

Abstract

Achondroplasia is the commonest form of dwarfism and possesses multiple anesthetic challenges ranging from securing intravenous line, monitoring and calculating drug dosage to abnormalities of the airway, spine, thoracic cage, and obesity, cardiopulmonary and neurological system. Multiple case reports describing neuraxial anaesthesia, particularly in obstetrics patients are available but there are limited literature on airway management and general anaesthesia. We came across an achondroplastic dwarf for hip replacement with spine deformity where neuraxial anaesthesia was impossible and her difficult airway and chronic lung disease imposed serious anesthetic challenges. We describe the importance of awake fiberoptic intubation (FOI) for airway management and general anaesthesia in this patient with difficult airway and spine and restrictive lung disease for total hip replacement.

Anesthetic concerns of achondroplastic patients with hip movement restriction scheduled for retrograde intrarenal surgery

“…Achondroplastic patients need careful preoperative evaluation and intraoperative management for a successful postoperative outcome. [ 2 ]…”

“…These patients have difficult airway features like a premature fusion of the skull base, depressed nasal bridge, maxillary hypoplasia, macroglossia, large mandible, abnormal tooth implantation, tracheal narrowing, short neck with fat deposition, cervical osteophytes, atlantoaxial instability, and limited neck extension. [ 2 ] Neck extension during direct laryngoscopy bears the risk of cervico-medullary compression due to narrow foramen magnum causing quadriparesis or sudden death. [ 2 ] Limited neck extension poses difficulties in visualizing the larynx during direct laryngoscopy.…”

“…[ 2 ] Neck extension during direct laryngoscopy bears the risk of cervico-medullary compression due to narrow foramen magnum causing quadriparesis or sudden death. [ 2 ] Limited neck extension poses difficulties in visualizing the larynx during direct laryngoscopy. [ 3 ] Rib hypoplasia, flattened ribcage, pectus excavatum, and obesity cause restrictive lung disease, resulting in decreased functional residual capacity, increased closing volume, atelectasis, reduced apnea time, sleep apnea, pulmonary hypertension, cor pulmonale, and increased risk of postoperative pulmonary complications.…”

Problematic Airway and Anesthetic Dilemmas for Achondroplastic Dwarfism in the Acute Care Setting: A Case Report

“…In comparison to the average human anatomy, these patients are prone to have difficult airway features (e.g., depressed nasal bridge, maxillary hypoplasia, small oral orifice, macroglossia, adenotonsillar hypertrophy, shortened thyromental, tracheobronchomalacia) that could limit the range of motion in their neck, impede sufficient seal for face mask ventilation, and place them at increased risk for upper airway obstruction [ 3 , 4 ]. Structurally, these patients also have a narrower foramen magnum that is funnel-shaped and special care must be taken during intubation positioning and oropharyngeal manipulation [ 3 , 4 ]. Although statistically unreported in adults, one prospective study in achondroplastic infants reported radiological findings for craniovertebral stenosis and craniomedullary compression in 58% and 35% of their study subjects, respectively [ 5 , 6 ].…”

Continuous Spinal Anaesthesia for Intertrochanteric Femur Fracture in a Patient with Skeletal Dysplasia

“…Spinal deformities in the form of accentuated lumbar lordosis, thoracic scoliosis, and narrowed epidural and intrathecal spaces may be encountered in patients with skeletal dysplasia [3,17]. ese deformities pose a hindrance to a Case Reports in Anesthesiology successful neuraxial block, thereby increasing the risks of complications such as repeated dural puncture, unpredictable LA spread, high block level, and difficult catheter placement [4][5][6][7]18].…”