Odontoid Synchondrosis Fracture Treated by C1-2 Polyaxial Screw-Rod Fixation

Muthukumar, Natarajan The Korean Neurosurgical Society 2014 Journal of Korean neurosurgical society Vol.55 No.4

The synchondrosis between the dens and the body of axis normally fuses between 5 and 7 years of age. Until this age, synchondrosis fractures can occur in children. Most synchondrosis fractures are conventionally treated by external immobilization alone. We report a 10-year-old child with odontoid synchondrosis fracture who was treated by C1 lateral mass and C2 pars screw rod fixation with a successful outcome and discuss the possible reasons for occurrence of odontoid synchondrosis fracture in this older child as well as the indications for surgery in this condition.

중두개와에 발생한 연골종 1례 : Case Reprot

박우용,윤상열,엄진화,강창구,김동희,김대조 대한신경외과학회 1988 Journal of Korean neurosurgical society Vol.17 No.5

Intracranial chondromas are extremely rare tumors, but when present, are generally related to the synchondrosis of the base, and they have several distinct features. They are slow growing benign tumors, and plain roentgenogram frequently reveals lytic lesion and stippled calcification at base of the skull. They are radioresistant, and surgical removal is treatment of choice. We report the rare case of chondroma arisen from base of the skull which diagnosed by brain CT and confirmed by operation.

The Role of Microtubule in the Regulation of Endochondral Bone Formation in the Developing Mouse Cranial Base

Hyuk-Jae Kwon(권혁제),Jong-Min Lee(이종민),Kyoung-Won Cho(조경원),Jeong-Oh Shin(신정오),Sung-Won Cho(조성원),Min-Jung Lee(이민정),Han-Sung Jung(정한성) 대한체질인류학회 2011 해부·생물인류학 (Anat Biol Anthropol) Vol.24 No.2

머리뼈바닥의 발생 중에 일어나는 연골속뼈발생은 다양한 세포현상과 연골바탕질 대사가 관여하는 매우 복잡한 과정이다. 연골세포에서는 증식, 형태변화, 증식 종료, 비대, 세포자멸사가 일어나고, 연골세포 주변으로는 연골바탕질이 활발하게 생성되었다가 제거되면서 뼈조직에 의해서 대체됨이 밝혀졌다. 그러나 이러한 복잡한 변화가 일어나는 동안 미세관이 어떠한 역할을 하는지에 대해서는 뚜렷하게 밝혀진 바가 없다. 그러므로 본 연구는 머리뼈바닥 발생 중 연골속뼈발생 동안의 미세관의 역할을 밝히고자 하였다. 미세관의 구성 단백질인 튜불린에 결합하는 약물 노코다졸을 사용하여 머리뼈바닥 조직을 기관배양 함으로써 미세관과 머리뼈바닥 발생의 관계를 조사하였다. 배양된 조직에서 일어나는 형태학적, 분자적 변화를 확인하기 위하여 헤마톡실린-에오신염색, 면역조직화학염색, 세포 개수 계산을 통한 분석이 이루어졌다. 나비뒤통수결합 부위 내의 미세관에 가해진 변화에 의해서 Ki67, 뼈발생표지자 BSP, BMP4의 발현이 감소하였다. 비대층 구역에서는 연골세포의 비대과정이 억제되었고, 뼈발생중심에서는 연골조직의 제거 및 뼈조직의 생성이 억제되었다. 본 연구는 미세관이 연골속뼈발생의 다양한 과정에 관여함을 밝혔다. 이러한 결과는 머리뼈 성장 및 머리뼈 바닥 기형과 관련된 질병을 이해하는 자료로 사용될 수 있을 것으로 사료된다. Endochondral bone formation of the developing cranial base is a complex process. This mechanism requires precise orchestration of many cellular events and cartilage matrix metabolism, such as proliferation, becoming round in shape, termination of proliferation, hypertrophic size-increase, and finally programmed cell death. Active formation and degradation of cartilage matrix take place, in which microtubules are involved for intracellular events; bone apposition follows these events. However, the involvement of microtubules during these changes in the developing cranial base has not been identified yet. Thus, we investigated the involvement of microtubules in the regulation of endochondral bone formation during cranial base development. Using tubulin-binding drug nocodazole, we examined the effects of altering the structure and function of microtubules during in vivo organ culture of the mouse cranial base. Cultured specimens were analyzed with HE staining, immunohistochemistry, and cell counting in order to study the morphological and molecular changes that occurred in the tissues. Disruption of the microtubular array by nocodazole reduced cells expressing proliferation marker Ki67, osteogenic marker BSP, and BMP4 within the sphenooccipital synchondrosis region; chondrocyte hypertrophy was ceased in the hypertrophic zone; degeneration of cartilage matrix and bone matrix apposition was inhibited in the ossification center of the basooccipital cranial base. Our data demonstrated that disruption of microtubules by nocodazole have multiple inhibitory effects on the sequential changes that occur during endochondral bone formation, suggesting the importance of normal microtubule-polymerization in cranial base development.

Craniofacial anomalies associated with spondyloenchondrodysplasia : Two case reports

Hong, Seok Woo,Huh, Kyung-Hoe,Lee, Jeong Keun,Kang, Jeong-Hyun,NA., Williams & Wilkins Co 2018 Medicine Vol.97 No.50

<P><B>Abstract</B></P><P><B>Rationale:</B></P><P>Spondyloenchondrodysplasia (SPENCD) is an autosomal recessive skeletal dysplasia by biallelic mutations in <I>ACP5</I> gene encoding tartrate-resistant acid phosphatase (TRAP). The extra-osseous phenotype of SPENCD is pleiotropic and involves neurological impairment and immune dysfunction. Dentofacial abnormalities and orofacial symptoms in SPENCD patients have been little discussed in the literature.</P><P><B>Patients concerns:</B></P><P>Herein we present clinical and radiological data regarding 2 siblings with SPENCD. Both patients exhibited short stature, cervical platyspondyly, growth disturbance with multiple skeletal deformities of the wrist, and systemic lupus erythematosus related autoimmunity. They experienced prolonged pain in the temporomandibular joint (TMJ) area and exhibited delayed dental development. One patient presented with midface hypoplasia, retrognathic mandible, and anterior openbite. Computed tomographic images demonstrated delayed spheno-occipital synchondrosis, obtuse cranial base angle, overdeveloped and anteriorly displaced sphenoidal sinuses, and compressed ethmoidal sinuses.</P><P><B>Diagnosis:</B></P><P>The genetic analysis revealed heterozygous for a missense mutations at <I>ACP5</I> in both probands.</P><P><B>Interventions:</B></P><P>Routine follow-up with conservative treatment were conducted for 12 months.</P><P><B>Outcomes:</B></P><P>The elder sister's orofacial pain was relieved but the boy showed sustained masticatory and cervical muscle pain and TMJ arthralgia which had changed in accordance with systemic condition. No further teeth eruption or skeletal growth was observed in 2 siblings during the follow-up period.</P><P><B>Lessons:</B></P><P>These findings extend the phenotypic spectrum of SPENCD and indicate that compromised endochondral ossification and the loss of TRAP activity may affect altered dentofacial development and orofacial symptoms.</P>

완전 편측성 순구개열자의 두개저 성장과 상,하악골 위치의 상관 관계에 관한 연구

백재호,손우성 대한치과교정학회 2000 대한치과교정학회지 Vol.30 No.4

본 연구는 연령 증가에 따른 구순구개열자의 두개저 성장 특징과 비상악복합체 및 하악골의 위치 변화와의 상관관계를 알아보고자 시행되었다. 교정치료 및 악정형적 치료의 경험이 없는 100명의 완전 편측성 구순구개열 남녀와 악골 부조화가 없으며 교정치료, 악정형적 치료 및 악교정 수술의 경험이 없는 100명의 정상 남녀를 선정하고 이들을 접형 사골연골결합 성장기, 접형후두연골결합 성장기 그리고, 연골결합 성장종료기의 세 연령집단으로 구분하였다. 이들의 측모두부규격방사선사진을 연령별로 비교하여 두개저와 비상악복합체 및 하악골 위치와의 관계를 비교하였고 이를 통해 완전 편측성 구순구개열자가 정상인에 비하여 다음과 같은 차이가 있음을 알 수 있었다. 1.완전 편측성 구순구개열군에서 사춘기 시기까지 사대 길이 및 두께가 작게 나타났고, 사대 길이의 사춘기 시기 증가량 또한 정상군 보다 작게 나타났다. 두개저 전방 길이 및 사대의 연령에 따른 회전 양상은 정상군과 차이가 없었다. 2.완전 편측성 구순구개열군에서 비상악복합체는 모든 연령에서 정상군 보다 후방 위치를 보였고, 특히 성장이 활발한 사춘기 시기까지 정상군과의 차이가 보다 크게 나타났다. 반면 수직적 위치는 정상군과 차이가 없었다. 이와 같은 비상악복합체의 전방 성장 결여는 두개저 형태와 깊은 상관관계를 나타내었다. 또한 상악을 자체의 후방성장을 나타내는 PtmS은 정상군과 달리 연령에 따른 증가량이 작아 상악결절부의 성장 결여를 나타내었다. 3.완전 편측성 구순구개열군에서 하악골은 정상군과 차이가 적었으나, Articular angle이 정상군 보다 매우 작게 나타났고, 하악골 위치와 비상악복합체 및 두개저 계측 항목간에 높은 상관관계를 보였다. This study was designed to evaluate the correlations between the morphology and growth of cranial base and the position of nasomaxillary complex and mandible in complete unilateral cleft lip and palate patients. Craniofacial skeletal morphology pattern was analyzed on the lateral cephalometric radiographs of the 100 subjects of complete unilateral cleft lip and palate group and the 100 normal grower group and each group was divided three sub-groups by age-range like spheno-ethmoidal synchodrosis glowing group, spheno-occipital synchondrosis growing group and finished synchondrosis growth group. These data were statistically analyzed to examine significant difference between both groups and between each sub-groups. The results of this study were as follows: 1.In complete unilateral cleft group, the length, thickness of clivus, and the rate of increase in length of clivus showed smaller amount by adolescence. The anterior length of cranial base and the rotation pattern of clivus with age showed no significant difference between two group. 2.In complete unilateral cleft group, nasomaxillary complex were located more posteriorly. This difference between two groups is larger by adolescent group than adult. Vertical position showed no significant difference between two groups. There was significant correlation between the cranial base of cleft group and the horizontal measurements(p〈0.01). PtmS showed no significant increment in cleft group. This showed the deficiency of growth in posterior part of maxilla. 3.In mandible, there was no significant difference between normal group and complete unilateral cleft group but articular angle showed significantle smaller in cleft group than in normal one. And the measurements of nasomaxillary complex position, cranial base and the position of mandible had significant correlation(p〈0.01).