Modified DCER technique for Basilar invagination and atlantoaxial dislocation in neurofibromatosis type 1: A Case Report

Sirawich Paokunta; Todsapon Praphanuwat; Chumpon Jetjumnong

Authors

Sirawich Paokunta Neurosurgery Unit, Department of surgery, Faculty of Medicine, Chiang Mai University, Thailand
Todsapon Praphanuwat Neurosurgery Unit, Department of surgery, Faculty of Medicine, Chiang Mai University, Thailand
Chumpon Jetjumnong Neurosurgery Unit, Department of surgery, Faculty of Medicine, Chiang Mai University, Thailand

Keywords:

Neurofibromatosis Type 1 (NF-1), Atlantoaxial Dislocation (AAD), Basilar Invagination (BI), DCER technique, Craniovertebral Junction Anomalies

Abstract

Introduction: Spinal deformities are common in Neurofibromatosis Type 1 (NF-1), a rare autosomal dominant disorder, however, craniovertebral junction anomalies such as atlantoaxial dislocation (AAD) and basilar invagination (BI) are uncommon. Surgical management is indicated in symptomatic cases. Although combined anterior–posterior fixation remains the standard approach, the distraction, compression, and extension reduction (DCER) technique offers a safe and effective alternative in selected patients. Case presentation: A 21-year-old woman with clinical features of Neurofibromatosis Type 1 (NF-1) presented with sudden-onset quadriparesis following a fall to the ground. Neuroimaging revealed atlantoaxial dislocation (AAD) and basilar invagination (BI). She was initially managed with cervical traction, followed by a single-stage posterior fixation and fusion using a modified distraction, compression, and extension reduction (DCER) technique. Postoperatively, spinal alignment and stability were restored, and the patient demonstrated marked neurological improvement. Conclusion: Surgical management of atlantoaxial dislocation (AAD) and basilar invagination (BI) in Neurofibromatosis Type 1 (NF-1) is particularly challenging due to the presence of dystrophic anatomy. The posterior-only distraction, compression, and extension reduction (DCER) technique offers an effective, patient-specific approach for deformity correction and achieving optimal outcomes.

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References

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