Joshi Shabin M, Hewitt Richard Jonathan David, Storr Helen L, Rezajooi Kia, Ellamushi Habib, Grossman Ashley B, Savage Martin O, Afshar Fary
Department of Neurosurgery, St. Bartholomew's and The Royal London School of Medicine and Dentistry, London, England.
Neurosurgery. 2005 Aug;57(2):281-5; discussion 281-5. doi: 10.1227/01.neu.0000166580.94215.53.
This is a retrospective analysis of 25 consecutive pediatric patients with Cushing's disease who underwent transsphenoidal surgery performed by a single neurosurgeon in a specialist center during a 20-year period. This article discusses the presentation of Cushing's disease, the endocrinological investigation with particular reference to bilateral inferior petrosal sinus sampling (BIPSS), the operative management with reference to specific pediatric difficulties of the transsphenoidal approach and the use of intraoperative image guidance, and the analysis of these cases as regards postoperative complications and outcomes of this rare condition in young patients.
All patients underwent detailed endocrine investigation and imaging in the form of computed tomography and/or magnetic resonance imaging. BIPSS was performed in 19 patients (76%), with successful lateralization of the side of the microadenoma in 14 (74%) and prediction of a central tumor in four (94% total prediction rate). Surgical removal was via the sublabial, paraseptal, transsphenoidal route.
There were 15 male and 10 female patients, with a mean age of 13.4 years (range, 6.6-17.8 yr). Weight gain was the most common presentation (100%), and then growth impairment (96%), fatigue and skin changes (64%), and hypertension (32%). Postoperative complications included growth hormone deficiency (36%), transient diabetes insipidus (12%), panhypopituitarism (4%), and transient cerebrospinal fluid rhinorrhea (4%). The median follow-up period was 59.5 months (range, 6-126 mo). Overall, 15 patients (60%) achieved surgical cure or remission, of which 14 outcomes were obtained using the results of BIPSS. Ten patients (40%) required postoperative radiotherapy to achieve "remission." There were no cases of meningitis, no neurological deficits, no reoperations, and no mortality.
Cushing's disease in children and adolescents is a rare illness. The accurate preoperative localization of the adenoma is essential for achieving good results. In this series, BIPSS was far more accurate in localizing the adenoma than computed tomography or magnetic resonance imaging. Imaging, however, is useful for the exclusion of other intracranial problems. Transsphenoidal surgery was safe and efficacious in achieving cure in the majority of cases. The challenge of transsphenoidal surgery in this age group is the small pituitary fossa and the absence of sphenoid sinus aeration in some cases. We found the use of intraoperative neuronavigation to be an excellent aid in overcoming such anatomic difficulties.
这是一项对25例连续的库欣病儿科患者进行的回顾性分析，这些患者在20年期间由专科中心的一名神经外科医生进行了经蝶窦手术。本文讨论了库欣病的临床表现、内分泌学检查，尤其提及双侧岩下窦采血（BIPSS），经蝶窦入路的手术管理，涉及该入路在儿科的特殊困难以及术中影像引导的应用，还分析了这些病例的术后并发症以及该罕见疾病在年轻患者中的治疗结果。
所有患者均接受了详细的内分泌学检查以及计算机断层扫描和/或磁共振成像形式的影像学检查。19例患者（76%）进行了BIPSS，其中14例（74%）成功实现了微腺瘤侧别定位，4例（总预测率94%）预测为中央型肿瘤。手术切除通过唇下、鼻中隔旁经蝶窦途径进行。
有15例男性和10例女性患者，平均年龄13.4岁（范围6.6 - 17.8岁）。体重增加是最常见表现（100%），其次是生长发育迟缓（96%）、疲劳和皮肤改变（64%）以及高血压（32%）。术后并发症包括生长激素缺乏（36%）、短暂性尿崩症（12%）、全垂体功能减退（4%）以及短暂性脑脊液鼻漏（4%）。中位随访期为59.5个月（范围6 - 126个月）。总体而言，15例患者（60%）实现了手术治愈或缓解，其中14例缓解结果是依据BIPSS得出。10例患者（40%）术后需要放疗以实现“缓解”。无脑膜炎病例、无神经功能缺损、无再次手术且无死亡病例。
儿童和青少年库欣病是一种罕见疾病。腺瘤的准确术前定位对于取得良好治疗效果至关重要。在本系列中，BIPSS在腺瘤定位方面远比计算机断层扫描或磁共振成像准确。然而，影像学检查对于排除其他颅内问题很有用。经蝶窦手术在大多数病例中安全有效，可实现治愈。该年龄组经蝶窦手术面临的挑战是垂体窝较小以及部分病例蝶窦未气化。我们发现术中神经导航在克服此类解剖学困难方面是一种极好的辅助手段。
