Jensen B L, Jensen K E, Kastrup K W, Pedersen S A, Wagner A
School of Dentistry, University of Copenhagen, Denmark.
J Craniofac Genet Dev Biol. 1997 Oct-Dec;17(4):190-7.
Seventeen patients (twelve males and five females) with craniopharyngioma were studied by retrospective review (stature, bone age, and hormone therapy) and by follow-up assessment in all seventeen survivors (stature and craniofacial development). Roentgencephalometric films in the lateral and frontal projections were analyzed. Individual and mean facial diagrams were produced based on 221 reference points in the individual patients and compared to normative data. The posterior cranial base was significantly reduced in length and the cranial base angle was significantly increased. In the facial regions great variations in size and prognathy of the jaws were recorded; on average the patients' maxilla and especially the mandible were short and retrognathic in relation to the anterior cranial base when compared to average adults. Average size and shape of the calvaria, cranial base, and facial regions in the adult male craniopharyngioma group corresponded closely to the average male at the stage of maximum growth in body height, i.e., around 14 years of age. It was concluded that size and morphology of the sphenoid and basioccipital bones were severely affected, possibly as a result of the interfering growth of a craniopharyngioma in childhood. The retrusion of the facial regions might be present as a result of the flattening of the posterior cranial base, but the relatively short and retruded mandible could also be caused by growth hormone deficiency before diagnosis/operation and in periods of sub-optimal therapy. The close resemblance of craniofacial morphology between adult males with craniopharyngioma and normal boys at the time of peak height velocity might reflect the fact that imitation of the natural, optimal balance between growth hormone and sex steroid in puberty is difficult to obtain in therapy.
对17例颅咽管瘤患者（12例男性，5例女性）进行了回顾性研究（身高、骨龄和激素治疗情况），并对所有17名幸存者进行了随访评估（身高和颅面发育情况）。分析了头颅侧位和正位X线测量片。根据个体患者的221个参考点绘制个体和平均面部图，并与标准数据进行比较。后颅底长度明显缩短，颅底角明显增大。在面部区域，记录到颌骨大小和前突程度有很大差异；与成年平均水平相比，患者的上颌骨，尤其是下颌骨相对于前颅底较短且后缩。成年男性颅咽管瘤组的颅骨、颅底和面部区域的平均大小和形状与身高增长最快阶段（即14岁左右）的平均男性密切对应。研究得出结论，蝶骨和枕骨基底部的大小和形态受到严重影响，可能是儿童期颅咽管瘤生长干扰的结果。面部区域的后缩可能是后颅底变平的结果，但下颌骨相对较短且后缩也可能是诊断/手术前及治疗效果欠佳期间生长激素缺乏所致。颅咽管瘤成年男性与身高增长高峰期正常男孩颅面形态的密切相似性可能反映了一个事实，即在治疗中难以模拟青春期生长激素和性类固醇之间自然、最佳的平衡。
