MadSci Network: Medicine |
Dear Telma: I’m not a neurosurgeon, but I’ll try to answer your question adequately. Sagittal synostosis (also known as scaphocephaly) is the most common of the craniosynostoses. These are a group of diseases which involve premature fusion of the cranial sutures. They can occur in isolation or as a part of various syndromes which I won’t elaborate on for the sake of time. The premature fusion can be partial or complete. Sagittal synostosis involves the sagittal suture, which is the most superior suture connecting the anterior and posterior fontanels in the sagittal plane. There is no one test to diagnose this disorder. It can be diagnosed antenatally if the fusion is severe enough to cause hydrocephalus which would be seen on ultrasound. Often it escapes the perinatal period if there are no gross external abnormalities. However the first year of life involves a rapid growth in the size of the brain and thus the cranium. Since the synostosis limits the expansion of the cranium in the lateral dimension, these children will develop abnormal longitudinal growth. The head takes a very narrow elongated shape with frontal bossing (sphenocephaly) and parietal-occipital prominence (bathmocephaly). The face is generally normal. Therefore the diagnosis is generally made by recognition of the physical signs. Confirmation is made by radiology. An AP skull radiograph may demonstrate absence of the gap between the parietal plates. CT or MRI will be even more conclusive. Commonly, if this is the only problem, the disorder is primarily cosmetic. Usually the fontanels remain open. However in more severe cases the fusion can extend into the fontanels as well. If this is the case, there is a higher likelihood for neurological developmental abnormalities and increased intracranial pressure (ICP). Even in simple sagittal synostosis, authors have reported a 10 - 60% incidence of elevated ICP. Therapy is surgical. This involves opening the fused suture and taking steps to ensure that the plates do not fuse again. To go into any more detail would be getting out of my league. I know I gave you more information than you asked. I hope this explains a complex problem a little better. I ran across the story of your son you posted on the web and am glad that things have turned out well for you both. Click here for more info. Also, there is a recent abstact on late repair of this problem.