Reply to Dr. Ijichi’s group letter

We appreciate Dr. Ijichi’s group for being interested in our study. Since you warned our way of treatment of the mild trigonocephaly in 2000, we tried to follow asmuch as possible your suggestions. We went through the appropriate procedure for being approved by the ethical committee in our hospital with an informed consent form in which it is written that this treatment is not generally accepted. We would like to make this clear again that we are not treating autism. We were pointing out this from the very beginning. We are treating mild trigonocephaly with developmental delays and those symptoms mimicking autism. Maybe this may make Dr. Ijichi’s group misunderstand the situation. Besides these symptoms, the children we treated do have further clinical manifestations such as language delays; hyperactivities; motor delays; self-mutilations; sleeping disturbances, especially night terror; and drooling. Most of these symptoms are considered to be caused by the frontal lobe dysfunction. Finally, signs of increased intracranial pressure are also seen in some patients. Indeed, the treatment of this mild form of craniosynostosis started with our personal experience. The first several cases were done to improve the cosmetic deformity. Incidentally, these cases were associated with clinical symptoms, namely language delays and hyperactivity, but we did not think at that time that the surgical procedure would have improved them. However, there was an improvement in a short term in our first children which prompted us to plan since the sixth girl we operated on a preoperative and postoperative psychological evaluation based on several tests. In other words, we intentionally treated this sixth patient in order to improve her symptoms. After surgery, she made glorious improvement in short time period after surgery and continued developing well successively. Actually, she graduated high school last spring. This case was already introduced in reply to Dr. Ijichi’s comment in 2002 (14, reference no. from Dr. Ijichi’s letter). After treating many further cases, all of them accurately evaluated for psychomotor development both prior and after the surgical correction, we were convinced that the surgical procedure played a positive role for the improvement of the symptoms. We tried to understand the mechanisms causing their symptoms. On the neuro-radiological examinations, the subjects that had a median bony ridge in the forehead presented also on the CTscan examination a narrow forehead, smaller frontal lobes and tight anterior fossa, and profound digital markings. Also in surgery, the wide and thin sphenoid ridges compressing the opercular regions were noticed in many of the cases (Fig. 1). We also measured the intracranial pressure epidurally during surgery. We had seen raised intracranial pressures above 11 mmHg in more than 90 % of the cases (Table 1). We mention this observation to emphasize that we are not treating some obscure asymptomatic condition. Additionally, in CT scans carried out 6 months after surgery, it could be noted that there were remarkably reduced digital markings which led us to believe that surgery had decreased an abnormally high intracranial pressure (Fig. 1). * Takeyoshi Shimoji tk_simoji2008@angel.ocn.ne.jp