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Background <p>Fibrodysplasia ossificans progressiva is a rare genetic disorder characterized by the progressive heterotopic ossification of soft connective tissues, ultimately leading to cumulative disability and ankylosis. Airway management during general anesthesia in patients with fibrodysplasia ossificans progressiva is particularly challenging because of anatomical abnormalities and the risk of triggering flare-ups due to minor trauma. However, reports on the anesthetic management of pediatric patients remain limited.</p> Case presentation <p>We present the case of a 10-year-old Japanese girl with genetically confirmed fibrodysplasia ossificans progressiva who underwent an uneventful elective inguinal hernia repair under general anesthesia. She had hallux valgus at birth and was diagnosed with fibrodysplasia ossificans progressiva at the age of 2&#xa0;years. Preoperative imaging revealed temporomandibular joint ankylosis with limited mouth opening (interincisal distance ~2&#xa0;cm), cervical spine fusion, and thoracic heterotopic ossification. A high-flow nasal cannula was used for preoxygenation and apneic oxygenation. Mask ventilation was confirmed to be adequate after induction with remifentanil and propofol. Gentle mandibular elevation and nasally guided fiberoptic intubation were successfully performed while maintaining peripheral oxygen saturation levels above 99%. Anesthesia was maintained using remifentanil and sevoflurane. Perioperative corticosteroids and nonsteroidal anti-inflammatory drugs were administered to prevent flare-ups. The surgery lasted 82&#xa0;minutes, and the patient was discharged uneventfully the following day without any signs of flare-up or airway complications.</p> Conclusion <p>General anesthesia in pediatric patients with fibrodysplasia ossificans progressiva requires meticulous planning to address the dual challenges of difficult airway management and the prevention of flare-ups. This case demonstrates that nasal fiberoptic intubation under high-flow nasal cannula oxygenation, combined with pharmacological prophylaxis and atraumatic handling, can contribute to safe anesthetic care. This patient case contributes to the limited body of literature on pediatric fibrodysplasia ossificans progressiva anesthesia, underscoring the practical strategies for managing the airway and minimizing the risk of flare-ups.</p>

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Anesthetic and airway management for inguinal hernia repair in a pediatric patient with fibrodysplasia ossificans progressiva: a case report

  • 期刊论文

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  • 发表日期：

    2026年1月28日

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  • Saori Ozaki,
  • Satoshi Kamiya,
  • Noriko Takeno,
  • Tomomi Ishii,
  • Yukari Toyota,
  • Hiroshi Yokomi,
  • Takahiro Kato,
  • Yasuo M. Tsutsumi

摘要

Background

Fibrodysplasia ossificans progressiva is a rare genetic disorder characterized by the progressive heterotopic ossification of soft connective tissues, ultimately leading to cumulative disability and ankylosis. Airway management during general anesthesia in patients with fibrodysplasia ossificans progressiva is particularly challenging because of anatomical abnormalities and the risk of triggering flare-ups due to minor trauma. However, reports on the anesthetic management of pediatric patients remain limited.

Case presentation

We present the case of a 10-year-old Japanese girl with genetically confirmed fibrodysplasia ossificans progressiva who underwent an uneventful elective inguinal hernia repair under general anesthesia. She had hallux valgus at birth and was diagnosed with fibrodysplasia ossificans progressiva at the age of 2 years. Preoperative imaging revealed temporomandibular joint ankylosis with limited mouth opening (interincisal distance ~2 cm), cervical spine fusion, and thoracic heterotopic ossification. A high-flow nasal cannula was used for preoxygenation and apneic oxygenation. Mask ventilation was confirmed to be adequate after induction with remifentanil and propofol. Gentle mandibular elevation and nasally guided fiberoptic intubation were successfully performed while maintaining peripheral oxygen saturation levels above 99%. Anesthesia was maintained using remifentanil and sevoflurane. Perioperative corticosteroids and nonsteroidal anti-inflammatory drugs were administered to prevent flare-ups. The surgery lasted 82 minutes, and the patient was discharged uneventfully the following day without any signs of flare-up or airway complications.

Conclusion

General anesthesia in pediatric patients with fibrodysplasia ossificans progressiva requires meticulous planning to address the dual challenges of difficult airway management and the prevention of flare-ups. This case demonstrates that nasal fiberoptic intubation under high-flow nasal cannula oxygenation, combined with pharmacological prophylaxis and atraumatic handling, can contribute to safe anesthetic care. This patient case contributes to the limited body of literature on pediatric fibrodysplasia ossificans progressiva anesthesia, underscoring the practical strategies for managing the airway and minimizing the risk of flare-ups.