Robinson H B
Perspect Pediatr Pathol. 1975;2:173-206.
Six new cases of the III-IV pharyngeal pouch syndrome were encountered at autopsy among 897 consecutive pediatric autoposies. All occurred in patients with conotruncal cardiac anomalies. The anatomic characteristics of the heart defect suggest a possible mechanism for the pathogenesis of this developmental anomaly. It is postulated that premature involution of the thyroidea ima artery, which is the principal embryonic blood source to the 3rd and 4th pharyngeal pouches and the ultimobranchial body, may be the critical event in the embryogenesis of the syndrome. Hemodynamic alterations in the course of anomalous morphogenesis of conotruncal anomalies may favor premature involution of certain vessels including the left 4th aortic arch and the thyroidea ima. Deficiencies of thymus, parathyroid glands and ultimobranchial tissue ("C" cells) may be the consequence of vascular deprivation during embryogenesis. These cases are discussed with regard to the proposed mechanism of pathogenesis and the influence of varying quantities of thymus upon peripheral lymphoid tissue. Both embryologic and clinical data support the possibility of a deficiency of ultimobranchial tissue. The range of minor and inconstant anomalies seen in patients with this syndrome is presented.
在连续897例儿科尸检中，尸检时发现6例新的III-IV咽囊综合征病例。所有病例均发生在伴有圆锥动脉干心脏畸形的患者中。心脏缺陷的解剖学特征提示了这种发育异常发病机制的一种可能机制。据推测，甲状腺下动脉过早退化可能是该综合征胚胎发生中的关键事件，甲状腺下动脉是第3和第4咽囊及后鳃体的主要胚胎血液来源。圆锥动脉干畸形异常形态发生过程中的血流动力学改变可能有利于包括左第4主动脉弓和甲状腺下动脉在内的某些血管过早退化。胸腺、甲状旁腺和后鳃体组织（“C”细胞）的缺乏可能是胚胎发生期间血管供应不足的结果。就所提出的发病机制以及不同数量的胸腺对外周淋巴组织的影响对这些病例进行了讨论。胚胎学和临床数据均支持后鳃体组织缺乏的可能性。文中还介绍了该综合征患者中出现的轻微和不恒定异常的范围。
