Isolated Lambdoid Craniosynostosis.

Introduction: Lambdoid craniosynostosis is an extremely rare anomaly in which there is premature fusion of one or both lambdoid sutures. The mainstay of treatment is surgical intervention, for which various procedures have been described, but there is a paucity of data on long-term outcomes. This study examines the long-term outcomes in the surgical management of this challenging condition, showing that accurate diagnosis and careful planning can lead to safe and consistent results.

Materials and methods: A retrospective chart review was performed looking at all cases of isolated lambdoid craniosynostosis treated with surgical intervention by the senior author from 1999 to 2016. Data collected included gender, age at diagnosis, age at surgery, length of follow up, method of diagnosis, side of affected suture, pre-operative and post-operative physical exam findings, surgical technique, complications, re-operation rate, and associated torticollis.

Results: Twenty-five patients (N = 25) were included in the study. All patients underwent posterior calvarial remodeling with/without barrel stave osteotomies and full thickness calvarial bone grafts. Mean length of follow up after operative intervention was 43.8 months (+/- 23.2 months). All patients were judged to have significantly improved head contour which was near-normal at conversational distance during post-operative follow up by the senior author. Residual plagiocephaly was present in 24% of patients. There were no major complications in this series. Reoperation rate was 8%. Seventy-six percent of patients also presented with torticollis, of which 37% had refractory torticollis that required sternocleidomastoid (SCM) release by the senior author.

Discussion: The authors present one of the largest series of operative cases of isolated lambdoid craniosynostosis to date. Our data show that with accurate diagnosis and careful planning, safe and consistent long-term results can be achieved with surgical intervention. A significant number of patients in our series also presented with concomitant torticollis. The authors recommend that all patients being evaluated for posterior plagiocephaly should also be evaluated for torticollis, because without recognition and intervention, patients may continue to have residual facial asymmetry and head shape abnormalities despite optimal surgical correction of the lambdoid synostosis.