Mathivon L, Carel J C, Coutant R, Derome P, Adamsbaum C, Bougnères P, Chaussain J L
Service d'endocrinologie pédiatrique, hôpital Saint-Vincent-de-Paul, Paris, France.
Arch Pediatr. 1997 Jun;4(6):521-8. doi: 10.1016/s0929-693x(97)87570-7.
Trans-sphenoidal surgery is currently the treatment of choice for Cushing's disease in children.
The results obtained in 20 consecutive patients referred to the Pediatric Endocrinology Department of hôpital Saint-Vincent-de-Paul are reported.
A remission of Cushing's disease was observed in 12/16 (75%) patients in whom surgery was the first treatment. Among these 12 patients, three relapsed (25%) 21 to 80 months after surgery. Four patients were initially treated with steroid synthesis inhibitors: three of those patients were subsequently operated on and their disease remitted. Among the seven patients in whom surgery failed (primary failure or relapse), two were reoperated and also remitted. Taken together, 21 operations were performed and resulted in four immediate failures (19%), three relapses (14%) and 14 long-term remissions (67%, follow-up 40 +/- 35 months). None of the biological, radiological or operative criteria were predictive of the therapeutic results.
Our results illustrate the efficacy and limits of trans-sphenoidal surgery for Cushing's disease of children and emphasize the need for a very long follow-up of these patients. Treatment of patients in whom surgery has failed (initially or secondarily) is particularly difficult and requires a multidisciplinary approach.
经蝶窦手术目前是儿童库欣病的首选治疗方法。
报告了在圣文森特 - 德保罗医院儿科内分泌科就诊的连续20例患者的治疗结果。
在16例首次接受手术治疗的患者中，12例（75%）库欣病缓解。在这12例患者中，3例（25%）在术后21至80个月复发。4例患者最初接受类固醇合成抑制剂治疗：其中3例随后接受手术，疾病缓解。在手术失败的7例患者（原发性失败或复发）中，2例再次手术并缓解。总共进行了21次手术，导致4例即刻失败（19%），3例复发（14%），14例长期缓解（67%，随访40±35个月）。生物学、放射学或手术标准均不能预测治疗结果。
我们的结果说明了经蝶窦手术治疗儿童库欣病的疗效和局限性，并强调对这些患者进行非常长期随访的必要性。对手术失败（原发性或继发性）的患者进行治疗特别困难，需要多学科方法。
