Intra-operative MRI facilitates tumour resection during trans-sphenoidal surgery for pituitary adenomas

Background During trans-sphenoidal microsurgical resection of pituitary adenomas, the extent of resection may be difficult to assess, especially when extensive suprasellar and parasellar growth has occurred. In this prospective study, we investigated whether intra-operative magnetic resonance imaging (iMRI) can facilitate tumour resection. Methods Twenty patients with macroadenomas, (16 non-functioning, three growth-hormone secreting and one pharmaco-resistant prolactinoma) were selected for surgery in the iMRI. The mean tumour diameter was 27 mm (range 11–41). The mean parasellar grade, according to the Knosp classification, was 2.3. Pre-operative coronal and sagittal T1-weighted and T2-weighted images were obtained. The trans-sphenoidal tumour resection was performed at the edge of the tunnel of a Signa SP 0.5-Tesla MRI. The surgeon aimed at a radical tumour resection that was followed by a peri-operative MRI scan. When a residual tumour was visualised and deemed resectable, an extended resection was performed, followed by another MRI scan. This procedure was repeated until the imaging results were satisfactory. In all patients, we were able to obtain images to assess the extent of resection and to classify the resection as either total or subtotal. Results After primary resection, eight out of 20 cases were classified as total resections. A second resection was performed in 11 of 12 cases classified as subtotal resections, and in four of these, total resection was achieved. A third resection was performed in three of the remaining seven cases with subtotal resections, but we did not achieve total resection in any of these cases. Therefore, the use of iMRI increased the number of patients with total resection from 8/20 (40%) to 12/20 (60%). The only observed complication was a transient spinal fluid leakage. Conclusion Intra-operative MRI during trans-sphenoidal microsurgery is useful in selected patients for a safe and more complete resection. This article is distributed under the terms of the Creative Commons Attribution Noncommercial License which permits any noncommercial use, distribution, and reproduction in any medium, provided the original author(s) and source are credited

Interstitial lung disease in children - genetic background and associated phenotypes

Interstitial lung disease in children represents a group of rare chronic respiratory disorders. There is growing evidence that mutations in the surfactant protein C gene play a role in the pathogenesis of certain forms of pediatric interstitial lung disease. Recently, mutations in the ABCA3 transporter were found as an underlying cause of fatal respiratory failure in neonates without surfactant protein B deficiency. Especially in familiar cases or in children of consanguineous parents, genetic diagnosis provides an useful tool to identify the underlying etiology of interstitial lung disease. The aim of this review is to summarize and to describe in detail the clinical features of hereditary interstitial lung disease in children. The knowledge of gene variants and associated phenotypes is crucial to identify relevant patients in clinical practice

Intraoperative magnetic resonance imaging-guided transsphenoidal surgery for giant pituitary adenomas

Giant pituitary adenomas (GPAs), defined as >/=40 mm in one extension, present a challenging subgroup of pituitary adenomas in terms of radical tumor removal and complication rates. The potential impact of intraoperative magnetic resonance imaging (iMRI) is investigated in a consecutive series and the results compared to the literature. From November 2004 until February 2005, six (five male) patients were operated for GPAs via an iMRI-guided transsphenoidal approach in the PoleStar N20. Clinical, endocrinological, and neuroradiological outcomes (at 3 months and yearly postoperative over 4 years) were assessed. Mean age was 46 years (range, 34-60). All patients presented with preoperative visual field defects, five with pituitary failure. Five adenomas were clinically nonfunctioning, one was producing GH and TSH. Preoperative imaging showed invasion of the cavernous sinus in all and extension to the interventricular foramen in two patients (one with occlusive hydrocephalus). Resection was total in four and subtotal (small cavernous sinus remnants) in two patients, leading to transsphenoidal reoperation in one patient. Visual acuity and fields improved in all six patients. The patient with occlusive hydrocephalus developed a postoperative cerebrospinal fluid leak (subsequently revised), two patients developed temporary, one permanent central diabetes insipidus, and one of them transient hyponatremia. Compared to the preoperative situation, endocrine status in the long-term follow-up (mean, 25 months) remained unchanged in four and worsened in two. Two patients were considered not to require hormone replacement therapy. IMRI supports transsphenoidal resections of GPAs because residual adenoma and related risk structures are easily detected and localized intraoperatively, extending the restricted visual access of the microscope beyond mere surface anatomy to a three-dimensional view. More radical removal of adenomas in a single surgical session combined with low complication rates are accomplished. This may add to a favorable clinical and endocrinological outcome in GPAs