Aberrant growth of the anterior cranial base relevant to severe midface hypoplasia of Apert syndrome / 대한악안면성형재건외과학회지

BACKGROUND: A 9-year-old male showed severe defects in midface structures, which resulted in maxillary hypoplasia, ocular hypertelorism, relative mandibular prognathism, and syndactyly. He had been diagnosed as having Apert syndrome and received a surgery of frontal calvaria distraction osteotomy to treat the steep forehead at 6 months old, and a surgery of digital separation to treat severe syndactyly of both hands at 6 years old. Nevertheless, he still showed a turribrachycephalic cranial profile with proptosis, a horizontal groove above supraorbital ridge, and a short nose with bulbous tip. METHODS: Fundamental aberrant growth may be associated with the cranial base structure in radiological observation. RESULTS: The Apert syndrome patient had a shorter and thinner nasal septum in panthomogram, PA view, and Waters’ view; shorter zygomatico-maxillary width (83.5 mm) in Waters’ view; shorter length between the sella and nasion (63.7 mm) on cephalogram; and bigger zygomatic axis angle of the cranial base (118.2°) in basal cranial view than a normal 9-year-old male (94.8 mm, 72.5 mm, 98.1°, respectively). On the other hand, the Apert syndrome patient showed interdigitating calcification of coronal suture similar to that of a normal 30-year-old male in a skull PA view. CONCLUSION: Taken together, the Apert syndrome patient, 9 years old, showed retarded growth of the anterior cranial base affecting severe midface hypoplasia, which resulted in a hypoplastic nasal septum axis, retruded zygomatic axes, and retarded growth of the maxilla and palate even after frontal calvaria distraction osteotomy 8 years ago. Therefore, it was suggested that the severe midface hypoplasia and dysostotic facial profile of the present Apert syndrome case are closely relevant to the aberrant growth of the anterior cranial base supporting the whole oro-facial and forebrain development.

Prevention & Management of Complications in Craniofacial Surgery Involving the Anterior Cranial Base

Despite its obvious advantages craniofacial surgery still remains a high-risk procedure with major complications. The purpose of this article is to review craniofacial surgery involving the anterior cranial base performed at Severance hospital from 1986 to 2000, focusing on complications and their management. The cases reviewed included not only craniofacial anomalies, but also curative procedures for trauma and tumor resection. This article retrospectively analyzed 136 procedures performed in 126 patients. Patients were classified into 5 groups on a clinical basis : craniosynostosis, craniofacial synostosis syndrome, orbital hypertelorism, tumor, and trauma. There were 58 complications in 36 patients. Complications were most frequent in group 2(craniofacial synostosis syndrome) which showed a complication rate of 50%, followed in order by orbital hypertelorism, trauma, craniosynostosis, and tumor. There were two deaths, and the mortality rate was 1.5% and the complication rate was 26%. Complications were divided into surgical and medical ones. The majority of surgical complications had serious morbidity potential and infection was most frequent. Medical complications were largely minor, with postoperative pneumonia being most common. Infection-related complications were frequently life-threatening and shown to have decisive influence on postoperative results. We therefore propose the following guide lines for the prevention and management of infection-related complications: 1) extensive antibiotics, 2) atraumatic dural dissection around crista gali, 3) water-tight closure of anterior cranial base defect with well vascularized flap, 4) secure bony fixation with minimal miniplate usage, 5) early detection of infection and continuous intracranial irrigation, 6) surgical skill, 7) short operative time.

Comparision of two cephalometric superimposition methods nasion-sella line method and anterior cranial base method / 대한치과교정학회지

Spatial change of craniofacial structures from growth or orthopedic force is usually evaluated by cephalometric superimpositions at different points in time.As the evaluation can be changed according to cephalometric superimposition method,selection for correct superimposition method is very important. Double registration of nineteen pairs of female lateral head films,their growth period is average 3.7 years and age is average 10.7 years, were performed by two observers. Comparisions was made between two methods and the results revealed the following; 1. NS line and ACE superimpositions are not markedly different in reliability due to almost same registration error of them. 2. The investigation was undertaken to examine interobserver difference. In NS line method, there is no significance in all measurements. In ACE method, significant difference was revealed in 4 measurements of 7 measurements was 3. In the investigation of intermethod difference, there is no significance between NS line and ACB superimpositions