Treatment for delayed presentation of sagittal synostosis: challenges pertaining to occult intracranial hypertension

Abstract

Patients with delayed presentation of isolated sagittal synostosis (ISS) pose unique surgical challenges. Intracranial hypertension can be missed in the absence of overt findings. Here, an algorithm is presented for the elucidation of intracranial hypertension and approaches to surgical treatment during calvarial reconstruction. Patients with delayed presentation (age > 15 months) of ISS between 1997 and 2009 were identified. Symptoms, signs, and radiological evidence of intracranial hypertension were noted. Intraoperative management included calvarial reconstruction in conjunction with ventriculostomy for intracranial pressure (ICP) monitoring and CSF drainage in the setting of suspected intracranial hypertension. Seventeen patients underwent calvarial reconstruction for delayed presentation of ISS. The mean surgical age was 40.5 months (16.2-82.9 months), and the average follow-up was 34.2 months (0.6-92.2 months). Eleven patients with subtle findings of intracranial hypertension underwent ICP monitoring during calvarial reconstruction. The mean opening ICP was 23.5 cm H(2)O (16.5-29.5 cm H(2)O), and the mean closing ICP was 7.0 cm H(2)O (3.5-17.0 cm H(2)O). Nine (81.8%) of 11 monitored patients demonstrated intracranial hypertension (ICP ≥ 20 cm H(2)O); the other 2 had borderline increased ICP. Perioperative morbidity was 5.9%, with 1 patient medically treated for transient, new-onset intracranial hypertension. The postoperative Whitaker category was I in 94.1% of patients, II in 5.9%, and III/IV in 0%. There were no reoperations or deaths. In this consecutive series, 11 of 17 patients with delayed presentation of ISS underwent ICP monitoring during calvarial reconstruction as a result of subtle clinical findings of intracranial hypertension. Nine (81.8%) of 11 monitored patients demonstrated intracranial hypertension. Calvarial reshaping along with ICP monitoring and CSF drainage facilitated reconstruction and resulted in good outcomes and the resolution of intracranial hypertension.