Trigonocephaly – our experience and treatment in the Republic of Macedonia

Abstract

Introduction: Prematurely fused metopic suture results in developmental anomaly named trigonocephaly. The treatment of trigonocephaly is a surgical reconstruction, starting from the simple suturectomy toward the complicated cranial vault reconstructions with aim to obtain enough endocranial space for normal development of the brain and aesthetic correction as well. The aim: The aim of our paper is to present our experience on this pathology in the Republic of Macedonia, stressing the trigonocephaly as one of the rare forms of craniosynostosis. Our material: During a period of 20 years (from 1996 to 2015) at the Pediatric department of the Clinic for Neurosurgery in Skopje, we observed 18 babies with trigonocephaly, including one with Carpenter syndrome and trigonocephaly, 14 males and 4 females. All children had simple trigonocephaly, one had syndromic trigonocephaly (Carpenter’s syndrome). According to Oi and Matsumoto classification done in 19865 severe trigonocephaly is observed in 11 cases and, moderate trigonocephaly in 7 cases. Our method: Our treatment consisted of slightly modified Di Rocco’s3 surgical procedure named “shell” operation, adding transposition of the “bone flap”. Results: The postoperative period was uneventful except for the expected forehead swelling. The babies were discharged from the hospital on average at the 8th postoperative day. At the three months control after the surgery, the head had excellent aesthetic appearance, with regular psychomotor development according to the age of the patient (Fig 3а and 3b). We had no serious complications except the expected postoperative swelling of the forehead. All operated children had excellent “long term” aesthetic effect and normal psychomotor development. Conclusion: The early recognition of these anomalies including all craniosynostoses, the deformities of the newborn and infant’s head and the preventive operative reconstruction would prevent abnormal disturbance of the psychomotor development during the child’s growth. The multidisciplinary approach can prevent new disabled individuals in the society. Our technique allows shortening the entire surgical procedure, especially in the departments where blood saving devices are not available.