关键词: abnormalities congenital abnormalities conjoined multiples neural tube defects twins

来  源:   DOI:10.1111/scd.12991

Abstract:
Diprosopus is a congenital anomaly in which partial or complete duplication of craniofacial structures occurs. Because it is rare, the mortality rate is high, and information concerning this anomaly is scarce. This study describes a case of human diprosopus in a 9-year-old male individual, who has severe complications associated with the central nervous, cardiovascular, respiratory, and digestive systems. Since birth, he has been monitored in a specialized hospital environment, where he has undergone several surgeries and multidisciplinary treatments. Regarding the craniofacial aspects, he had agenesis of the corpus callosum, floor of the nasal cavity, and floor of the anterior cranial fossa, in addition to the presence of bone dysplasia, ocular hypertelorism and cleft palate with nasal and oral teratoma. Regarding dental characteristics, the patient has duplication of the maxilla, mandible, tongue, and some teeth. After complementary imaging exams, several supernumerary teeth were found, with some being impacted and in complex regions, with an indication for extraction due to the risks of impaction, irruptive deviation, root resorption, and associated cystic or tumoral lesions. Because of the numerous complications, knowledge, and preparation of the entire team is necessary for the correct management of the case.
摘要:
Diprosopus是一种先天性异常,其中发生部分或完全的颅面结构重复。因为它很罕见,死亡率很高,关于这种异常的信息很少。这项研究描述了一个9岁男性的人类diprosopus病例,有严重的中枢神经并发症,心血管,呼吸,和消化系统。自出生以来,他在专门的医院环境中接受了监控,在那里他经历了几次手术和多学科治疗。关于颅面方面,他有call体发育不全,鼻腔的地板,和前颅窝的地板,除了骨发育不良,眼球过度过度和腭裂伴鼻腔和口腔畸胎瘤。关于牙齿特征,病人的上颌骨有重复,下颌骨,舌头,还有一些牙齿.补充影像学检查后,发现了几颗多余的牙齿,有些受到影响,在复杂的地区,由于撞击的风险,有提取的迹象,颠覆性偏差,根吸收,和相关的囊性或肿瘤性病变。由于众多的并发症,知识,整个团队的准备对于正确管理案件是必要的。
公众号