Taguchi Tomomi, Kimizuka Sachiko, Takano Koji
Department of Endocrinology, Diabetes and Metabolism, Kitasato University School of Medicine.
Department of Plastic and Aesthetic Surgery, Kitasato University School of Medicine, Sagamihara, Kanagawa, Japan.
Endocrinol Diabetes Metab Case Rep. 2021 Apr 1;2021. doi: 10.1530/EDM-21-0001.
Acromegaly is associated with a low quality of life (QoL), which is partially attributable to appearance. However, appearance changes are only partially reversible with treatments of growth hormone excess. This case study describes a 41-year-old Japanese man who presented with mandibular prognathism. Acromegaly was suspected because of the patient's facial features. Subsequent examination revealed a pituitary tumour with elevated levels of growth hormone and insulin-like growth factor 1 (IGF1), confirming a diagnosis of acromegaly. We assessed his QoL with the acromegaly QoL questionnaire (AcroQoL) before transsphenoidal surgery, and all AcroQoL scores were low. Although the pituitary adenoma was resected, his serum IGF1 levels started to increase again and MRI identified a residual pituitary lesion. After lanreotide and pegvisomant injection therapies improved his serum IGF1 levels, we reassessed his AcroQoL scores, however, the results showed worsening scores regarding appearance and personal relationships, and the patient expressed interest in surgery for mandibular prognathism. We performed sagittal split ramus osteotomy (SSRO) with Le Fort I osteotomy, thus, the patient's AcroQoL scores improved. This case shows that SSRO with Le Fort I osteotomy for mandibular prognathism, rather than control of serum IGF1 level alone, improved the patient's AcroQoL score, especially for both psychological well-being and approval of appearance. Acromegaly has many complications, including its negative impact on patients' perception of their appearance and patients' QoL can be improved using multiple options, in addition to controlling growth hormone levels.
The patient's AcroQoL scores worsened despite biochemical normalization of IGF-1 levels. Mandibular prognathism due to acromegaly can be successfully operated by performing sagittal split ramus osteotomy with Le Fort I osteotomy. Frequent monitoring of AcroQoL scores and appropriate response to negative results can improve the overall QoL.
肢端肥大症与生活质量(QoL)低下相关,部分原因是外观问题。然而,生长激素过多的治疗只能部分逆转外观变化。本病例研究描述了一名41岁的日本男性,他表现为下颌前突。由于患者的面部特征,怀疑患有肢端肥大症。随后的检查发现垂体肿瘤,生长激素和胰岛素样生长因子1(IGF1)水平升高,确诊为肢端肥大症。在经蝶窦手术前,我们使用肢端肥大症生活质量问卷(AcroQoL)评估了他的生活质量,所有AcroQoL评分都很低。尽管切除了垂体腺瘤,但他的血清IGF1水平再次开始升高,MRI检查发现垂体有残留病变。在使用兰瑞肽和培维索孟注射治疗改善了他的血清IGF1水平后,我们重新评估了他的AcroQoL评分,然而,结果显示在外观和人际关系方面的评分恶化,患者对下颌前突手术表示感兴趣。我们进行了下颌升支矢状劈开截骨术(SSRO)联合Le Fort I截骨术,因此,患者的AcroQoL评分得到改善。本病例表明,下颌前突的SSRO联合Le Fort I截骨术,而非仅控制血清IGF1水平,改善了患者的AcroQoL评分,尤其是在心理健康和外观认可度方面。肢端肥大症有许多并发症,包括对患者外观认知的负面影响,除了控制生长激素水平外,还可以使用多种方法改善患者的生活质量。
尽管IGF-1水平生化指标正常,但患者的AcroQoL评分仍恶化。肢端肥大症导致的下颌前突可通过下颌升支矢状劈开截骨术联合Le Fort I截骨术成功手术治疗。频繁监测AcroQoL评分并对负面结果做出适当反应可改善总体生活质量。