Purpose: Controversy continues today regarding the presence of intracranial hypertension in the setting of single-suture craniosynostosis. Clinical experience since the '80's has demonstrated increased ICP in 8-17% of affected individuals. We have currently identified a subset of patients presenting in a delayed fashion with scaphocephaly who appear to be at significantly greater risk for intracranial hypertension. Characteristically, these patients presented with a paucity of clinical findings. In an attempt to better understand clinical parameters associated with intracranial hypertension in this population, patients with delayed presentation of scaphocephaly were evaluated for associated signs and symptoms in addition to determining a safe and effective means of therapeutic intervention contributing to excellent outcomes.
Methods: A retrospective review was undertaken for patients treated over 12 years ('97-'09) at Children’s National Medical Center and Georgetown University Hospital for delayed presentation (surgery >13m) of sagittal synostosis. Preoperative evaluation included eliciting symptoms (headache/vomiting, developmental changes, irritability, oculomotor paresis) and signs (supraorbital retrusion, severe towering, severe frontal/occipital bossing) of intracranial hypertension. Patients were also reviewed for papilledema as well as CT evidence (inner table erosion/compressed subarachnoid spaces). Any clinical evidence of intracranial hypertension led to consideration of intraoperative ICP monitoring via a ventriculostomy which could also be employed to reduce elevated pressures by CSF drainage. This was felt to potentially reduce morbidity when undertaking the cranial reconstruction.
Results: Seventeen patients (88.2%M/11.8%F) underwent primary calvarial reconfiguration for delayed presentation of sagittal synostosis, with a mean age at surgery of 34.4 months (13.8 - 82.9m). All patients had single suture pathology and none manifested hydrocephalus. Eight patients presented with clinical evidence of intracranial hypertension and underwent placement of an intraoperative ventricular drain (mean opening ICP 24.6 cm H₂0 (11.0 - 29.5): mean closing ICP 5.4 cm H₂0 (3.5 - 7.5). Seven patients (7/17, 41%) demonstrated intracranial hypertension (ICP ≥ 20), and one had borderline intracranial hypertension (ICP 15 - 19). Perioperative morbidity was 7.1%, with one patient medically managed briefly for new-onset, postoperative intracranial hypertension. Follow-up ranged from 1.5 - 91.7 months, with a mean of 29.0 months. The long-term complication rate was zero as was mortality. There were no reoperations and patient outcomes were classified as Whitaker I ¼ 94.1%, II ¼ 5.9%, III and IV ¼ 0%.
Conclusions: In view of the continuing controversy over the incidence of intracranial hypertension in single-suture craniosynostosis, we present a subset of patients (41%) with occult intracranial hypertension associated with delayed presentation of sagittal synostosis. Such patients must be thoroughly investigated for the possibility of intracranial hypertension, looking closely for any associated signs or symptoms including radiographic evidence. Patients with delayed presentation of scaphocephaly manifesting clinical features of intracranial hypertension should be considered for placement of a ventriculostomy/ICP monitor during open vault reconstruction. In this manner, cranial vault remodeling may be done with minimal morbidity producing excellent outcomes as well as resolution of their intracranial hypertension.