Craniosynostosis is a condition characterized by premature closure of one or more of the sutures of the cranial vault. The most common variety involves the sagittal suture (sagittal synostosis), and is characterized by a long, narrow head (scaphocephaly or dolichocephaly), with ridging over the sagittal suture. Less common are coronal synostosis, characterized by asymmetry of the forehead and orbits, and metopic synostosis, in which there is a triangular appearance to the forehead with ridging in the middle of the forehead over the metopic suture.
Craniosynostosis of all types is managed by the pediatric neurosurgeons at BC Children’s Hospital. Since 1982, our group has operated on over 325 children with craniosynostosis, including 170 with sagittal synostosis, 140 with unilateral or bilateral craniosynostosis, and 20 with metopic synostosis. Children with coronal synostosis included those with Crouzon, Apert, Pfeiffer, Saethre Chotzen and other syndromes. The vast majority of these children have had their surgical procedures in the 1st year of life. Children with sagittal synostosis are operated on by a neurosurgeon alone, whereas those with coronal or metopic synostosis are operated on as a team procedure with our plastic surgical craniofacial experts. Pre and post operative follow-up by the multidisciplinary team is provided to syndromic patients.
There have been no mortalities or serious infections after surgical procedures for craniosynostosis, attesting not only to the expertise of the surgeons, but of the entire team, including the pediatric anesthesiologists. Except for the most complex craniofacial syndromic cases, children are not admitted to the ICU setting after surgery, and generally are discharged within 72 hours of surgery.
Over the years we have reduced the rate of blood transfusions during these procedures by meticulous attention to limiting intraoperative blood loss and by accepting a lower hemoglobin level in the postoperative period. The rate of blood transfusion in surgery for all types of craniosynostosis at . BCCH is now less than 20%, and this is lower than that in any reported series. We have reviewed in detail our series of 118 children having surgery for sagittal synostosis between January 1986 and December 1999, and have documented a reduction in the rate of transfusions from 42% before 1996 to 11% from 1997-1999. This low rate of transfusion has continued since 1999 to the present.
More recently we have introduced minimally invasive endoscopically assisted surgery for selected infants with sagittal synostosis and this is supplemented by the use of helmets after surgery. The children have much smaller scalp incisions and tolerate this surgery very well and are often discharged from hospital within 24 hours. This option is used especially in very young infants.
The outcomes after surgery for craniosynostosis have been documented, where appropriate, using anthropometric measurements. We have also been interested in developing consistent 3D imaging paradigms as a tool in outcome assessment. We are currently studying the reproducibility of some of the anthropometric measurements that have been used in the assessment of children with asymmetric heads with positional occipital plagiocephaly. In the management of positional plagiocephaly, we have had a long interest in the concept of remodelling of the head shape using orthotic devices, and have been using either helmets or head bands for over 15 years. Other research efforts will assess the long term outcomes in our population.