Management of Cervical Kyphosis in Larsen Syndrome: A Case Report

Abstract

Introduction: Larsen syndrome is a congenital skeletal disorder manifested by several facial, ligamentous and spinal complications. Cervical kyphosis is one of the serious manifestations of the Larsen syndrome. However, there is no consensus regarding the best procedure of cervical kyphosis management in these patients. Case Presentation: A 1-year-old boy with the diagnosis of the Larsen syndrome was admitted to our hospital and undergone several corrective surgeries for knee, hip and foot deformities. At the age of 2 years, scoliosis was diagnosed and surgically managed. At the same time, cervical kyphosis was observed and monitored until the symptoms of neurological deficit due to cord compression led to the correction of cervical Kyphosis at the age of 4.5 years. Accordingly, an anterior/posterior (360 degree) cervical spinal fusion surgery was performed. Subsequently, cervicothoracic fusion was performed to correct cervicothoracic instability. No neurological complications were reported afterward. Conclusions: In spite of existing controversy around the best method of cervical kyphosis management in Larsen syndrome's patients older than 2-year old, anterior release and posterior fixation followed by anterior spinal fusion and strut grafting led to the satisfactory result in our case.

Craniovertebral junction instability in Larsen syndrome: An institutional series and review of literature

“…Single-stage 360° fixation (corpectomy with implant or graft anteriorly along with C1/occipital plate to C6 fixation) is a popular option, tailored to the complexity of cervical sub-axial spinal involvement. [ 18 ] Moreover, the choice of approach depends on the familiarity of a surgeon with a corridor, the extent of radiological involvement, availability of pediatric intensivist, and age of the child. In a 360° approach, which corridor should be taken first is again a matter of debate.…”

“…[ 5 ] Even the authors who recommend 360° fixation, failed to demonstrate any substantial improvement. [ 18 21 ] Anterior spinal fusions alone are not advised in young children with LS, owing to high risk for spinal cord injury and an arrest of anterior growth. [ 5 ] Exceptionally, in patients with severe CK with fixed variety and myelopathic symptoms, an anterior decompression along with circumferential fusion is beneficial.…”