Richieri-Costa-Pereira综合征(RCPS)患者表现出严重的颅面改变,经常需要正畸和外科手术。因此,本研究旨在描述RCPS患者的颅面关系.
分析了7例RCPS患者和7例年龄和性别匹配的非综合征患者的全景X线片和侧位头颅远射照片。头影测量用于确定根尖基部的大小,他们之间的关系,颅面生长的模式,和患者的面部高度。通过组内系数验证了观察者之间的一致性。为了进行组间比较,采用配对t检验。P值<0.05表示统计学意义。
RCPS患者的平均年龄为18.5岁。6名患者为女性。所有RCPS患者均具有Pierre-Robin序列,而2例也表现为下颌骨裂。大多数RCPS患者缺少下中央切牙(100%),下侧切牙(85.7%),下第二前磨牙(85.7%),和/或上外侧切牙(57.1%)。对于所有头颅测量(0.87-0.99),观察者之间的一致性都很好。与对照组相比,RCPS患者表现出严重的颅面改变:鞍区B点(SNB)角(73.8o±4.86ovs78.85o±4.53o,P=.029),上颌长度(7.89厘米±0.58厘米vs16.36厘米±0.75厘米,P=.001),下颌长度(9.90cm±0.46cmvs20.61cm±0.45cm,P=.001),上前脸高度(5.41厘米±0.50厘米vs9.40厘米±0.47厘米,P=.001),下面部前高度(5.48厘米±0.75厘米vs11.66厘米±0.55厘米,P=.001),和后面部高度(6.70厘米±0.33厘米vs13.65厘米±1.06厘米,P=.001)。SNB没有区别,一个点-鼻孔-B点,pogonion-nasion-B点,各组间下颌位置角(P>.05)。
与非综合征患者相比,RCPS患者上颌骨和下颌骨发育不足。
Patients with Richieri-Costa-Pereira syndrome (RCPS) present severe craniofacial alterations and frequently require orthodontic and surgical procedures. Thus, this study aims to describe the craniofacial relationships in patients with RCPS.
Panoramic radiographs and lateral cephalometric teleradiographs of 7 patients with RCPS and 7 age- and sex-matched nonsyndromic patients were analyzed. Cephalometric measurements were used to determine the size of apical bases, the relationship between them, the pattern of craniofacial growth, and the facial heights of the patients. Interobservers\' concordance was verified by intraclass coefficient. For comparison between the groups, paired t test was employed. P values <.05 indicated statistical significance.
Average age of patients with RCPS was 18.5 years. Six patients were female. All patients with RCPS had Pierre-Robin sequence while 2 also presented cleft mandible. Most patients with RCPS had missing lower central incisors (100%), lower lateral incisors (85.7%), lower second premolars (85.7%), and/or upper lateral incisors (57.1%). Concordance between observers was excellent for all cephalometric measurements (0.87-0.99). Patients with RCPS presented severe craniofacial alterations when compared to control group: sella-nasion-B point (SNB) angle (73.8o ± 4.86o vs 78.85o ± 4.53o, P = .029), maxillary length (7.89 cm ± 0.58 cm vs 16.36 cm ± 0.75 cm, P = .001), mandibular length (9.90 cm ± 0.46 cm vs 20.61 cm ± 0.45 cm, P = .001), upper anterior face height (5.41 cm ± 0.50 cm vs 9.40 cm ± 0.47 cm, P = .001), lower anterior face height (5.48 cm ± 0.75 cm vs 11.66 cm ± 0.55 cm, P = .001), and posterior face height (6.70 cm ± 0.33 cm vs 13.65 cm ± 1.06 cm, P = .001). There was no difference in SNB, A point-nasion-B point, pogonion-nasion-B point, and mandibular place angles between the groups (P > .05).
Patients with RCPS present deficient development of maxilla and mandible when compared with nonsyndromic patients.