MadSci Network: Medicine
Query:

Re: What is the procedure to diagnose sagittal sinostosis in a new born?

Area: Medicine
Posted By: Theodore VanderVelde, M.D. Surgery, Washington University Medical School, St. Louis, MO
Date: Fri Jan 24 12:22:25 1997
Message ID: 853872207.Me


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.

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