Saethre-Chotzen syndrome: long-term outcome of a syndrome-specific management protocol.
Bianca K Den Ottelander, Marie-Lise C Van Veelen, Robbin De Goederen and 8 others
PMID 32909287WHAT IT FOUND
Among 32 patients with Saethre-Chotzen syndrome, eight needed a second skull expansion.
Five of nine patients who developed papilledema had head circumference growth falling before papilledema, and abnormal transverse sinuses were more common in patients with papilledema.
Key findings
01Eight patients required a second cranial vault expansion, and six of these were treated according to the centre's protocol.
02Five of nine patients with papilledema who were treated according to the protocol had an occipital frontal head circumference growth curve deflection before papilledema appeared.
03Transverse sinus anomalies were significantly more prevalent in patients with versus without papilledema (p=0.01).
STILL TO COME
How it was doneWhat they foundWhat it means for SLPs
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What it does not show
Only 32 patients were included, and five patients were excluded because of unavailable data, so the sample was small. Children born before 2005 were included retrospectively, so some information came from records rather than planned prospective follow-up. The number of patients was too small to build a trustworthy logistic regression model of all potential intracranial hypertension risk factors. Surgery was performed by four different surgeons, which could affect results. Three patients were under 6 years at last follow-up, so they were not followed until skeletal maturity; the recorded reoperation rate may be a lower bound. Speech referral data were missing for seven patients, and consecutive speech reports were not available for the whole cohort.
The easy way to misread this
Do not read this as proof that the protocol prevents intracranial hypertension. It was a small single-centre cohort, and three patients were under 6 years at last follow-up, so the recorded reoperation rate may be a lower bound.