Vastardis Heleni, Spyropoulos Meropi N, Burdi Alphonse R
Department of Oral Biology, School of Dentistry, National and Kapodistrian University of Athens Athens, Greece.
Front Physiol. 2012 Aug 24;3:333. doi: 10.3389/fphys.2012.00333. eCollection 2012.
The rare opportunity to study a human fetus showing bilateral clefting of the lower lip along with other associated anomalies resembling those of the equally rare Pena-Shokeir phenotype prompts this report. The scarcity of reports on bilateral clefts of the lower lip has strengthened the conventional understanding or, perhaps even dogma that the lower lip and jaw develop from the progressive midline merging of just two mandibular prominences in the embryo. On the basis of observations stemming from this case report, it is proposed that yet another developmental event or process (in addition to the midline merging of the mandibular prominences) may be operable in the normal morphogenesis of the lower lip and anterior mandibular region. The bilateral paramedian clefting observed provides evidence that another distinct developmental region, a small medial process complements mandibular morphogenesis.
有机会研究一名下唇双侧唇裂且伴有其他类似同样罕见的佩纳-肖克综合征(Pena-Shokeir phenotype)相关畸形的人类胎儿,这一罕见机遇促成了本报告。关于下唇双侧唇裂的报道稀少,强化了传统认知,甚至可能是一种教条观念,即下唇和下颌在胚胎期由仅两个下颌突的渐进性中线融合发育而来。基于本病例报告的观察结果,有人提出,在正常下唇和下颌前部区域形态发生过程中,可能还有另一个发育事件或过程(除下颌突的中线融合之外)在起作用。观察到的双侧旁正中唇裂提供了证据,表明另一个独特的发育区域,即一个小的内侧突起,对下颌形态发生起到补充作用。