{
  "abstract": "Application for ESRA Abstract Prizes : I apply as an Anesthesiologist (Aged 35 years old or less)Background and Aims Homozygous achondroplasia (HA) is a rare and distinctly different genetic disorder from heterozygous achondroplasia. Patient features are more severe including a disproportionately larger head, smaller foramen magnum, shorter limbs, and smaller thoracic cavity. Cervicomedullary compression secondary to foramen magnum stenosis can cause respiratory disturbances and obstructive sleep apnea. We report our experience and anesthetic considerations for the management of an infant with HA who presented with increasing respiratory and oxygen requirements and micro aspiration who underwent foramen magnum decompression (FMD) and gastric-tube placement under general anesthesia. The patient and family have provided written HIPPA authorization to publish this case report.Methods An 8-month-old, 3.8 kg female with homozygous achondroplasia, foramen magnum stenosis and micro-aspiration underwent FMD and gastric-tube placement. She required nasal CPAP and oxygen support at baseline. After IV induction, intubation required two attempts due to difficulty acquiring adequate depth of anesthesia. A 3.0 microlaryngeal ETT was placed and post-intubation bronchospasm was treated with epinephrine and propofol. She was positioned prone and TIVA was used for optimal neuromonitoring. After decompression, truncal blocks were performed for pain management before g-tube placement. She was transitioned to sevoflurane, extubated awake and monitoring in the PICU. Postoperative pain was minimal and managed with acetaminophen and ketorolac then discharged home on POD 2.Results Very little is known about managing HA, a rare and typically lethal condition. Only three cases of FMD in HA have been reported. We describe a fourth case, the first to combine FMD with g-tube placement and regional anesthesia to facilitate extubation. HA presents profound anesthetic challenges, including difficult airway, IV access and positioning due to macrocephaly and hypotonia. Indirect laryngoscopy and smaller ETTs are often required. TIVA is preferred for neuromonitoring. Despite surgical success, patients may later require tracheostomy.Abstract P113 Figure 1Sagittal T2 weighted MRI in neutral. (A) flexion; (B) and extension; (C) Scan performed at 4 months of age: Similar narrowing at the craniocervical junction with the aperture measuring 3 mm in flexion, 3.4 mm at the neutral position and 4.9 mm in extension (arrows). Mild compression of the spinal cord is again demonstrated at the craniocervical junction, without myelomalaciaConclusions This case highlights the critical importance of experienced providers and multidisciplinary perioperative planning.",
  "authors": [
    {
      "affiliations": [
        "Anesthesiology, Nemours Children’s Health, Wilmington, USA"
      ],
      "name": "Angela Snow"
    },
    {
      "affiliations": [
        "Anesthesiology, Nemours Children’s Health, Wilmington, DE, USA"
      ],
      "name": "Abraham Oommen"
    }
  ],
  "title": "P113 Anesthetic challenges in rare skeletal dysplasia: emphasizing the value of regional anesthetic approaches",
  "uid": "f48815a1-0ec5-5402-834b-ff8cd55811c3"
}