Operative treatment for microcephaly secondary to craniosynostosis at the turn of the twentieth century

Introduction: Microcephaly has been described throughout history, and physicians in the nineteenth and early twentieth centuries struggled to identify the etiology of this condition in order to better treat it. In 1908, Cushing wrote in Surgery of the Head, in Keen's Surgery Its Principles and Practice, that the use of craniotomies for the treatment of microcephaly was a futile practice, with limited post-operative improvement that did not justify the operative risks. Methods: Following IRB approval, and through the courtesy of the Alan Mason Chesney Archives, the surgical files of the Johns Hopkins Hospital, from 1896 to 1912, were reviewed. A single case of operative treatment of microcephaly, performed 3 years after Cushing's published opinion on the procedure, was discovered and is described here. Results: In 1911, a 3-year-old girl from Florida presented with complaints of an "inability to walk" and "backward development." The diagnosis of microcephaly, possibly secondary to craniosynostosis, was made. Cushing operated upon her twice, performing bilateral decompressive craniotomies in a two-stage operation, without excision of the synostosis. Post-operatively, he documented improvement in the child's condition and a resolution of some presenting symptoms. Conclusions: Although Cushing published his disapproval of the use of craniotomies in the treatment of microcephaly in 1908, a review of his early surgical files demonstrates that this opinion was based on cases published in the literature, rather than his own operative experience. In 1911, he performed bilateral craniotomies in a 3-year-old patient with microcephaly and documented post-operative improvement in the patient's general condition.