Anaesthetic Challenges in a Paediatric Patient with Escobar Syndrome-Difficult Airway and Postoperative Pneumothorax

Waleed Bin Ghaffar1, Irfan Ul Haq1, Arham Shahid1

  • 1Department of Anaesthesiology, Aga Khan University Hospital, Karachi, Pakistan.

Insights

This case report details managing anesthesia for a child with Escobar syndrome (ES), highlighting difficult airway techniques and rare postoperative complications like pneumothorax. It introduces oxygen insufflation during fiberoptic intubation for pediatric ES patients.

Area of Science:

  • Anesthesiology
  • Genetics
  • Pediatric Surgery

Background:

  • Escobar syndrome (ES) presents significant airway challenges for anesthesiologists due to craniofacial and cervical malformations.
  • Associated anomalies include cleft lip/palate, micrognathia, and limited neck mobility, complicating intubation.
  • Perioperative management requires careful consideration of these anatomical variations.

Purpose of the Study:

  • To report a pediatric case of Escobar syndrome undergoing general anesthesia.
  • To describe successful airway management using fiberoptic intubation with oxygen insufflation.
  • To document rare postoperative complications, including pneumothorax with mediastinal shift.

Main Methods:

  • A pediatric patient with Escobar syndrome underwent laparoscopic inguinal hernia repair and orchidopexy.
  • Initial airway management attempts with video laryngoscopy and fiberoptic bronchoscopy were challenging.
  • Successful intubation was achieved via nasal fiberoptic bronchoscopy with oxygen insufflation.

Main Results:

  • Video laryngoscopy and standard fiberoptic bronchoscopy failed due to poor visualization and desaturation.
  • Nasal fiberoptic intubation with oxygen insufflation using a 3mm endotracheal tube was successful.
  • The patient experienced intraoperative hyperthermia and postoperative pneumothorax with mediastinal shift.

Conclusions:

  • This case highlights the successful use of oxygen insufflation during fiberoptic intubation in a pediatric Escobar syndrome patient.
  • Pneumothorax with mediastinal shift is a newly reported postoperative complication in pediatric Escobar syndrome.
  • Anesthesiologists must be prepared for complex airway and perioperative challenges in Escobar syndrome.

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