Surgical management of cervical kyphosis in larsen syndrome. Case report and review of literature.

INTRODUCTION

and importance: Larsen syndrome is a rare genetic disorder that is characterized by multiple joint dislocations, flat faces that can also be referred to as "dish face", kyphoscoliosis, and anomalies of the vertebrae. Patients with this syndrome frequently develop various spinal deformities, one of them being kyphosis of the cervical spine. This deformity can lead to serious health manifestations if not surgically treated.

CASE PRESENTATION: We report a case of a 6-month-old female, diagnosed with Larsen syndrome. She presented with progressive upper and lower limbs spasticity, flexed neck, and bilateral resistant developmental dysplasia of the hip. A C3 corpectomy with iliac crest allograft was done and 2.7 plates with screws in C2 and C4 were placed. An abduction brace (Pavlik harness) was used for 3 months after the surgery to prevent early collapse.

CLINICAL DISCUSSION: Our patient was the youngest patient reported in the literature to be operated on. Although the type of surgery for patients with Larsen who suffer from spinal deformities is dictated by the severity of the deformity; the literature agrees that surgical intervention is the most important step in its management.

CONCLUSION

If cervical kyphosis in a patient with Larsen syndrome is left untreated; the progression of the condition can eventually lead to paralysis. Early surgical correction can spare the patient future deterioration due to chronic cord compression.