Perioperative Management of Presumed Schwartz-Jampel Syndrome During Complex Pediatric Spinal Fusion: A Case Report

Schwartz-Jampel syndrome (SJS) is associated with neuromuscular and structural abnormalities that can complicate perioperative and anesthetic management. The syndrome has been noted to create increased difficulty in anesthetic airway management with age. We report the perioperative course of a 13-year-old female with presumed SJS undergoing combined anterior and posterior spinal fusion for severe progressive congenital scoliosis. Preoperative evaluation revealed features predictive of a difficult airway, including limited mouth opening and restricted neck mobility, requiring video laryngoscopy for successful intubation. During the anterior thoracoscopic phase, dense pleural adhesions were encountered, requiring extensive pleural dissection and adhesiolysis to allow completion of the release of the anterior spinal column. After repositioning prone for the posterior procedure, the patient developed acute ventilation difficulty with markedly elevated airway pressures and severe hypercarbia. Flexible bronchoscopy revealed a nearly occlusive sanguineous mucus plug within the endotracheal tube, prompting abortion of the procedure. The patient returned 1 week later for completion of surgery, and difficulty with endotracheal tube depth was encountered due to a foreshortened trachea. She otherwise recovered without major complications. This case highlights the perioperative management of a complex pediatric neuromuscular scoliosis patient undergoing combined anterior and posterior spinal procedures. It underscores the importance of anticipating unexpected pulmonary complications in patients with difficult airway anatomy and severe restrictive lung disease. Heightened awareness of endotracheal tube obstruction and malposition is imperative.