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イチハラ アツヒロ
ICHIHARA Atsuhiro
市原 淳弘 所属 医学部 医学科（東京女子医科大学病院） 職種 教授・基幹分野長 |
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| 論文種別 | 症例報告 |
| 言語種別 | 英語 |
| 査読の有無 | 査読あり |
| 表題 | Pituitary dysfunction in trans-sellar encephalocele: a two-case report and a literature review. |
| 掲載誌名 | 正式名：Endocrine Journal 略 称：Endocr J ISSNコード：13484540/09188959 |
| 掲載区分 | 国内 |
| 巻・号・頁 | pp.ahead of print |
| 著者・共著者 | Kosaku Amano†, Yasufumi Seki*, Shinichiro Koizumi, Yuto Kawauchi, Miho Yamashita, Kazuhiko Kurozumi, Atsuhiro Ichihara, Takakazu Kawamata |
| 発行年月 | 2026/01 |
| 概要 | Trans-sellar encephalocele (TSE) is a rare congenital anomaly of the cranial base characterized by herniation of intracranial contents through the sellar floor. Although clinical presentations are diverse, the prevalence and characteristics of endocrine dysfunction due to hypothalamic-pituitary involvement are unclear. This study aimed to clarify endocrine function in patients with TSE and emphasized the importance of multidisciplinary management. Here, we present two adult cases of TSE with progressive visual impairment and evident pituitary hormone deficiencies, and conducted a comprehensive literature review to investigate endocrine function and its postoperative outcomes in patients with TSE. Both cases presented with growth hormone (GH) deficiency and diabetes insipidus and showed deterioration in GH secretion, which was postoperatively assessed using dynamic testing, despite minimal or no direct manipulation of the pituitary gland. In a literature review, 87 cases from 41 case reports and 3 review articles were identified. Among the 87 cases, pituitary dysfunction was identified in 41 (47.1%); however, detailed hormonal assessments were performed in only 25 (28.7%) of the cases. In 33 cases with documented preoperative and postoperative endocrine status, pituitary function worsened in 16 (48.5%), improved in 6 (18.2%), and remained unchanged in 11 (33.3%) postoperatively. Pituitary dysfunction occurred in nearly half of patients with TSE, but was frequently underdiagnosed due to inadequate endocrine assessment. Comprehensive endocrine evaluation, including dynamic GH testing, performed at diagnosis and perioperatively, may be essential in optimizing patient outcomes. |
| DOI | 10.1507/endocrj.EJ25-0480 |
| PMID | 41526235 |