Differential reversibility of anterior and posterior pituitary dysfunction in delayed recurrent pediatric Rathke's cleft cyst.

IF 0.9 Q4 ENDOCRINOLOGY & METABOLISM
Atsuko Ogasawara, Shozo Yamada, Go Matsuoka
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引用次数: 0

Abstract

Summary: Rathke's cleft cyst (RCC) is a benign sellar or suprasellar lesion increasingly detected by magnetic resonance imaging (MRI). Although surgery is indicated for symptomatic or enlarging RCCs, recurrence may occur and reoperation may be required, particularly in pediatric patients, for whom long-term postoperative data remain limited. We report a pediatric case with RCC associated with growth hormone (GH) deficiency. GH replacement therapy was started at 5 years of age. At 8 years of age, MRI revealed a sellar and suprasellar cystic lesion diagnosed as RCC, and endoscopic transsphenoidal surgery was performed. Following the initial surgery, GH secretion showed no improvement, and desmopressin therapy was initiated due to postoperative arginine vasopressin (AVP) deficiency. Gradual cyst recurrence became evident, necessitating reoperation seven years after the initial surgery. After the second surgery, GH secretion improved, whereas postoperative AVP deficiency persisted. This case demonstrates delayed recurrence of pediatric RCC and dynamic changes in anterior and posterior pituitary function, highlighting axis-specific reversibility of pituitary dysfunction and the need for long-term follow-up.

Learning points: Pediatric RCC may demonstrate delayed recurrence many years after initial surgery, requiring long-term endocrine follow-up. Anterior pituitary dysfunction, including GH deficiency, may be reversible following adequate decompression. Persistent postoperative arginine vasopressin deficiency may reflect irreversible posterior pituitary or stalk injury and may not recover after reoperation. Pituitary function in RCC can evolve over time and should be reassessed even after apparent surgical success.

迟发性复发儿童拉克氏裂囊肿前后垂体功能障碍的差异性可逆性。
摘要:Rathke's cleft囊肿(RCC)是一种良性鞍或鞍上病变,磁共振成像(MRI)越来越多地检测到。尽管有症状的或增大的rcc需要手术治疗,但可能会发生复发并需要再次手术,特别是儿科患者,他们的长期术后数据仍然有限。我们报告一个小儿病例与RCC与生长激素(GH)缺乏。生长激素替代疗法开始于5岁。8岁时,MRI显示鞍上和鞍上囊性病变,诊断为肾细胞癌,并进行内镜下经蝶窦手术。初始手术后,GH分泌未见改善,由于术后精氨酸抗利尿激素(AVP)缺乏,开始去氨加压素治疗。囊肿逐渐复发,首次手术后7年再次手术。第二次手术后,生长激素分泌改善,而术后AVP缺乏持续存在。本病例显示小儿RCC的延迟复发和垂体前后部功能的动态变化,强调垂体功能障碍的轴特异性可逆性和长期随访的必要性。学习要点:小儿肾细胞癌可能在初次手术多年后延迟复发,需要长期的内分泌随访。垂体前叶功能障碍,包括生长激素缺乏,在适当的减压后可能是可逆的。术后持续的精氨酸抗利尿激素缺乏可能反映不可逆的垂体后叶或垂体柄损伤,再手术后可能无法恢复。RCC的垂体功能可以随着时间的推移而变化,即使在明显的手术成功后也应重新评估。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
CiteScore
1.50
自引率
0.00%
发文量
142
审稿时长
9 weeks
期刊介绍: Endocrinology, Diabetes & Metabolism Case Reports publishes case reports on common and rare conditions in all areas of clinical endocrinology, diabetes and metabolism. Articles should include clear learning points which readers can use to inform medical education or clinical practice. The types of cases of interest to Endocrinology, Diabetes & Metabolism Case Reports include: -Insight into disease pathogenesis or mechanism of therapy - Novel diagnostic procedure - Novel treatment - Unique/unexpected symptoms or presentations of a disease - New disease or syndrome: presentations/diagnosis/management - Unusual effects of medical treatment - Error in diagnosis/pitfalls and caveats
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