Bovendeert Jeske F M, Nievelstein Rutger A J, Bleys Ronald L A W, Cleypool Cindy G J
Department of Pediatrics, Diakonessenhuis, Utrecht, The Netherlands.
Department of Radiology & Nuclear Medicine, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht, The Netherlands.
J Med Case Rep. 2020 Oct 3;14(1):176. doi: 10.1186/s13256-020-02501-x.
Conjoined twinning is a rare congenital malformation with an incidence of about 1.5 per 100,000 births. Because no consensus has been reached regarding the dysmorphology, thorough descriptions of conjoined twins as part of teratological collections can be useful to increase knowledge of this congenital malformation. In this case report, we describe a parapagus dicephalus twin from the collection of the Department of Anatomy of the University Medical Center Utrecht in the Netherlands. External anatomical characteristics were assessed through a detailed macroscopic examination and internal characteristics by means of whole-body computed tomography and magnetic resonance imaging (3 Tesla).
Macroscopic examination showed a Caucasian male parapagus dicephalus tripus tribrachius conjoined twin a type of conjoined twinning in which there are two heads side by side, one rump, and three upper and three lower limbs. In addition, anencephaly was observed in the left twin. Radiological imaging showed a normal central nervous system in the right twin and absence of the calvaria, cerebrum, diencephalon, and mesencephalon in the left twin. There was clear duplication of the vertebral column, rib cage, respiratory system, and gastrointestinal system at least up to and including the first part of the duodenum. The heart consisted of a monoatrium with two separate ventricles. There was a fused liver with a single gallbladder, a single spleen, three kidneys, two bladders, and duplication of the penis. The third upper and lower extremities were articulating with a fused glenoid and acetabulum, respectively. The third foot showed both polydactyly and syndactyly of the toes.
This case report describes a unique case of a male dicephalus parapagus tripus tribrachus conjoined twin discordant for anencephaly. Radiological imaging proved to be an adequate noninvasive method to provide insights into the internal (dys)morphology of this specific specimen, improving its scientific and educational value. This approach could be generally applied to other teratological specimens, thereby strengthening arguments regarding pathogenetic hypotheses, which may lead to new or improved insights into both normal and abnormal embryonic development.
联体双胎是一种罕见的先天性畸形，发病率约为每10万例出生中有1.5例。由于在联体双胎的畸形学方面尚未达成共识，因此作为畸形学标本集一部分对联体双胎进行详尽描述，有助于增加对这种先天性畸形的认识。在本病例报告中，我们描述了荷兰乌得勒支大学医学中心解剖学系标本集中的一例双头联体双胎。通过详细的大体检查评估外部解剖特征，借助全身计算机断层扫描和磁共振成像（3特斯拉）评估内部特征。
大体检查显示为一名白种男性双头联体三臀三臂联体双胎，这是联体双胎的一种类型，有两个并列的头、一个臀部以及三条上肢和三条下肢。此外，在左侧双胎中观察到无脑畸形。放射学成像显示右侧双胎中枢神经系统正常，左侧双胎无颅骨、大脑、间脑和中脑。至少直至十二指肠第一部，脊柱、胸廓、呼吸系统和胃肠道系统均明显重复。心脏为单心房双心室。肝脏融合，有一个胆囊、一个脾脏、三个肾脏、两个膀胱，阴茎重复。第三条上肢和下肢分别与融合的肩胛盂和髋臼相连。第三只脚显示有多指和并指畸形。
本病例报告描述了一例罕见的男性双头三臀三臂联体双胎且伴有无脑畸形差异的病例。放射学成像被证明是一种合适的非侵入性方法，可深入了解该特定标本的内部（畸形）形态，提高其科学和教育价值。这种方法可普遍应用于其他畸形学标本，从而加强关于发病机制假说的论据，这可能会对正常和异常胚胎发育带来新的或更好的认识。
