Mild Trigonocephaly - Autism - Ken

Most cases of craniosynostosis are diagnosed during early infancy, but occasionally craniosynostosis evolves with minimal cranial involvement and goes unnoticed until late childhood. Seemingly these mild forms of craniosynostosis cause few, if any, symptoms of neurological involvement. We describe the cases of a 9-year-old girl and a 6-year-old boy who presented with evident signs of raised intracranial pressure (ICP), together with a negligible skull deformity. We have termed these cases as occult craniosynostosis. Differential diagnosis in our patients was established against known causes of benign intracranial hypertension. Bilateral expanding craniotomies afforded total relief from the symptoms and signs of raised ICP. Neurosurgeons treating children with symptoms and signs of benign intracranial hypertension should be aware of the possibility of minimal forms of craniosynostosis evolving with marked manifestations of raised ICP.

In this study, intracranial pressure (ICP) was recorded with an epidural sensor for periods of 12 to 24 hours in 92 cases of craniosynostosis. Pre- and postoperative recordings were performed in 23 patients, and 55 children underwent preoperative psychometric testing. The ICP was found to be normal in one-third of the cases, was obviously elevated in one-third, and was borderline in one-third. Waves of increased ICP were recorded during rapid eye movement (REM) sleep. After surgery, ICP decreased progressively and returned to normal in several weeks. A significant statistical relationship was found between the patients' ICP and their mental level: the higher the ICP the lower the mental level. The regression curve of ICP as a function of age shows that ICP is maximal at the age of 6 years and decreases later. The significance of these results is discussed. The authors recommend that ICP be recorded in cases of craniosynostosis since it is of some help in deciding whether patients should undergo surgery.

The indications for surgical correction of craniosynostosis in which there is involvement of only one of the cranial vault sutures have traditionally been based upon the cosmetic merits of the deformity alone. Whilst it is now appreciated that intracranial hypertension is commonly associated with the more complex forms of craniosynostosis, this aspect has not previously been addressed in detail among cases of single-suture craniosynostosis. This retrospective study reports our experience of overnight subdural intracranial pressure monitoring in 74 children with premature closure of a single cranial suture. A single coronal suture was involved in 37 patients, the sagittal suture in 25 and the metopic suture in 12. Intracranial pressure was raised in 13 (17%), borderline in 28 (38%) and normal in 33 (45%). Elevated intracranial pressure was seen more commonly where a midline suture was involved (sagittal or metopic) than when a single coronal suture was fused. We conclude that intracranial hypertension occurs in a significant proportion of children with single-suture craniosynostosis and suggest that this factor should be borne in mind during the initial assessment of these children so as to enable timely intervention where required and appropriate counselling of parents.

Craniosynostosis, the premature fusion of one or multiple cranial sutures, an be complicated by visual failure resulting from raised intracranial pressure (ICP). Of the 290 children operated on at the Birmingham Children's Hospital between 1978 and 1995 for craniosynostosis, 9 were found to have defective visual acuity attributable to raised ICP. Mean age at presentation was 3.11 years (range: 1-6 years) and mean follow-up, 7.33 years (range: 1.5-16 years). All these patients presented significantly later than usual, and 5 of them developed recurrent craniosynostosis. At the initial examination bilateral papilloedema was seen in 4 patients, unilateral disc oedema in 1 patient, bilateral optic atrophy in 3 patients and unilateral optic atrophy in 1 patient. Following decompressive craniofacial surgery, the visual outcome was good in 4 out of 5 patients with papilloedema and poor in all patients with optic atrophy. visual failure resulting from raised ICP in craniosynostosis is a devastating complication, which appears to be associated with late presentation and recurrent craniosynostosis.