Craniosynostosis. Trigonocephaly Associated with Symptoms. Mainly Nonsyndromic Type.

Accession number;00A0582413
Title;Craniosynostosis. Trigonocephaly Associated with Symptoms. Mainly Nonsyndromic Type.
Author;SHIMOJI TAKEYOSHI(Naha Hfosp., Okinawa Prefect. Gov.)   YAMADA MIKITO(Asahi Univ., Murakami Mem. Hosp.)   HARA SHIGERU(Daiyukai Hosp.)   
Journal Title;Nervous System in Children
Journal Code:G0347B
ISSN:0387-8023
VOL.25;NO.1;PAGE.43-48(2000)
Figure&Table&Reference;FIG.6, TBL.2, REF.21
Pub. Country;Japan
Language;Japanese
Abstract;Our report concerns sixteen cases (13 boys, 3 girls) of trigonocephaly, mainly of the nonsyndromic type (Table 1). All patients had such symptoms as language development delay, motor delay, hyperactivities and autistic tendencies, etc., in addition to facial features characterized by depressed temples, heel-shaped forehead, and slight hypotelorism (Fig. 1). The most important finding was that the closed metopic suture ridge could be palpated. Ten patients aged more than one did not show any apparent symptoms. Skull X-rays, CT, 3D-CT, and MRI demonstrated sclerotic changes of the metopic suture, hypotelorism, shortened lateral orbit, narrowed anterior fossa, and constricted frontal lobes (Fig. 2). There were no abnormal brain findings of any patient. Seven patients were subjected to SPECT, and five demonstrated decreased CBF in the bilateral frontal lobes (Table 2). Cranioplasty of the frontal bone involving the skull base was carried out for all patients (Fig. 3). Good results aesthetically were obtained in all patients postoperatively. There also was some degree of clinical symptom improvement in all patients (Table 1). Postoperative SPECT demonstrated increased CBF in the frontal lobes of five patients (Table 2). Our patients with trigonocephaly of the nonsyndromic type has such as sociated clinical symptoms as were reported by Sidoti [20] and Kapp-Simon [12]. If patients older than one have language problems, motor development delay, hyperactive and autistic tendencies, trigonocephaly should be suspected as it can easily be diagnosed by palpating the metopic suture ridge in the center of the forehead. (author abst.)