Endoscopic endonasal resection of the odontoid peg for paediatric basilar invagination

Saurabh Sinha1, Showkat Mirza, Nick Bishop

  • 1Department of Neurosurgery, Sheffield Children's NHS Foundation Trust, Sheffield, UK. saurabh.sinha@sth.nhs.uk

Insights

Osteogenesis imperfecta (OI) patients with basilar invagination can now undergo safer decompression surgery. An endoscopic endonasal approach offers a minimally invasive alternative to traditional open surgery for children with this rare OI complication.

Area of Science:

  • Neurosurgery
  • Genetics
  • Pediatrics

Background:

  • Osteogenesis imperfecta (OI) is a genetic disorder characterized by bone fragility, fractures, deformity, and pain.
  • Basilar invagination, leading to brainstem compression, is a rare but severe complication in children with OI.
  • Conventional anterior decompression for basilar invagination involves an open trans-oral approach, often associated with significant morbidity.

Observation:

  • A rare, life-threatening complication of Osteogenesis imperfecta (OI) is brainstem compression caused by basilar invagination.
  • Children with OI and basilar invagination frequently require extensive medical care and hospitalizations.
  • The traditional surgical method for anterior decompression involves an open trans-oral route.

Findings:

  • This study details an endoscopic endonasal approach for resecting the odontoid peg in a pediatric patient with OI and basilar invagination.
  • The endoscopic endonasal technique was successfully employed for decompression in a child with OI and basilar invagination.
  • This minimally invasive approach facilitated the removal of the odontoid peg causing brainstem compression.

Implications:

  • The endoscopic endonasal approach presents a viable alternative to open trans-oral surgery for OI-related basilar invagination in pediatric patients.
  • This technique may significantly reduce patient morbidity and shorten hospital stays compared to traditional methods.
  • Further adoption of this endoscopic approach could enhance surgical outcomes and patient recovery in pediatric OI cases with basilar invagination.

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