Craniosynostosis Management

Most of the modern understanding of craniosynostosis is referenced from the 1851 writings of Virchow. His understanding and descriptions of irregular calvarial growth patterns were the basis of the law of Virchow. According to his observations, the abnormal cranial growth observed in persons with craniosynostosis occurs perpendicular to the involved calvarial sutures. Therefore, if a suture line is prematurely ossified, no growth is present in the direction perpendicular to that suture. The law was too simplistic in its explanation of the growth patterns of the skull; later studies demonstrated conflicting data (Moss, 1959). The presence of compensatory growth patterns in patients with craniosynostosis was described later (Delashaw, J Neurosurg, 1989; Delashaw, Neurosurg Clin N Am, 1991).

Surgical treatment for craniosynostosis was initially advocated by Lannelongue in 1890. His patients had microcephaly from craniosynostosis and were thought to be imbeciles. These patients accordingly underwent craniectomy to remove the involved suture line and to "release the brain" (Lannelongue, 1890). Soon after, in 1891, linear craniectomy was introduced. As with any new procedure, this one met with much resistance. However, the resistance to a surgical intervention was slowly put to rest with mounting evidence. Several studies indicated that craniosynostectomy was the treatment of choice for the release of fused suture lines in the skull (Faber, 1927; Dandy, 1943; Ingraham, 1948; Shillito, 1968).

Although strip craniectomy was used often, it lost much support with the advent of cranial vault reconstruction, in which the calvarial bones were excised, reshaped, and trimmed. Studies showed that, over time, cranial suture areas excised during strip craniectomy still became fused and led to an abnormal cranial contour (Venes, 1976). Strip craniectomy was easier and involved less blood loss compared with the newer cranial vault reconstruction. Strip craniectomy also did not address the frontal bossing and associated abnormalities in calvarial shape and relied on the rapid growth of the brain to correct it. Strip craniectomy was optimal only in the first few months of infancy, while surgeons could use cranial vault reconstruction throughout infancy. Consequently, strip craniectomy lost favor, and the surgical treatment has been modified to include cranial vault remodeling.

Recently, with the advent of endoscopy, attention has returned to endoscopic strip craniectomy. The endoscopic technique has only been tried over the last several years, but it offers the advantages of a shorter and safer operation, less cost, less in-hospital time, and less blood loss. The operation was shown to be a success in a study of 12 patients, all younger than 8 months (Barone, 1999). Critical to this success and a departure from the standard strip craniosynostectomy was the extensive use of a postoperative remodeling helmet. Although first introduced by Persing et al in 1986, helmet therapy has not been used as extensively as a postoperative therapeutic intervention (Persing, 1986). Following the endoscopic technique, helmets were used for several months and showed promising early results.