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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.
Abstract:
Osteogenesis imperfecta (OI) is a genetic disorder whose primary manifestations are fractures, bone deformity and bone pain. Brainstem compression due to basilar invagination is a rare and potentially life-threatening complication of OI. Children with this condition often require significant medical input and multiple admissions to hospital. Traditionally, anterior decompression is carried out through an open trans-oral route. We describe an endoscopic endonasal approach for resection of the peg in a child with OI and basilar invagination. We believe this approach provides an excellent alternative with minimal morbidity and decreased length of stay especially in the paediatric population.

