Cerebrotendinous xanthomatosis (van Bogaert-Scherer-Epstein disease): CT and MR findings.

PURPOSETo describe the CT and MR findings in the brain and spinal cord of patients with cerebrotendinous xanthomatosis and to seek possible correlations between clinical, biochemical (cholestanol levels), and neuroimaging findings.METHODSTen patients with well-defined clinical and biochemical diagnoses of cerebrotendinous xanthomatosis were examined. Brain CT was performed in eight cases. In all patients MR was obtained using spin-echo and gradient-echo sequences. In eight patients spine MR was also performed.RESULTSNeuroradiologic findings included diffuse cerebral and cerebellar atrophy. In half the cases, atrophy of the brain stem and corpus callosum was also found. In the majority of patients cerebellar bilateral focal lesions and mild white matter signal alterations were present. Spinal cord MR did not show signal abnormalities or atrophy.CONCLUSIONSWe found cranial alterations in patients with severe neurologic impairment, but there was no correlation with cholestanol plasma levels. No spinal cord abnormalities were present.     

Vacuum-assisted closure, dermal regeneration template and degloved cryopreserved skin as useful tools in subtotal degloving of the lower limb

The standard management of degloving injuries involves either immediate grafting with the avulsed skin or full- or split-thickness grafts at a later date. Alternative methods include pedicle and free flaps and revascularisation. The authors present an innovative technique of treating degloving injuries with cryopreserved split-thickness skin grafts harvested from degloved flap, artificial dermal replacement and vacuum-assisted closure (VAC therapy). To the authors' knowledge, this is the first reported case of such bilaminar reconstruction of a degloving injury.     

Prognostic Influence of Loss of Blood Group A Antigen Expression in Pathologic Stage I Non-Small-Cell Lung Cancer

IntroductionIn the scientific literature, contradictory results have been published on the prognostic value of the loss of expression of blood group antigen A (BAA) in lung cancer. The objective of our study was to analyze this fact in our surgical series.Patients and methodsIn a multicenter study, 402 non-small-cell lung cancer (NSCLC) patients were included. All were classified as stage-I according to the last 2009-TNM classification. We analyzed the prognostic influence of the loss of expression of BAA in the 209 patients expressing blood group A or AB.ResultsThe 5-year cumulative survival was 73% for patients expressing BAA vs 53% for patients with loss of expression (P=.03). When patients were grouped into stages IA and IB, statistical significance was only observed in stage I-A (P=.038). When we analyzed the survival according to histologic type, those patients with adenocarcinoma and loss of expression of BAA had a lower survival rate that was statistically very significant (P=.003). The multivariate analysis showed that age, gender and expression of BAA were independent prognostic factors.ConclusionsThe loss of expression of blood group antigen A has a negative prognostic impact in stage I NSCLC, especially in patients with adenocarcinoma.     

The growing spine: how spinal deformities influence normal spine and thoracic cage growth

Purpose  

This article aims to provide an overview of how spinal deformities can alter normal spine and thoracic cage growth.     

Gonadotropic pituitary carcinoma: HER-2/neu expression and gene amplification. Report of two cases

The authors report on two gonadotropic carcinomas of the adenohypophysis that occurred in a55-year-old man (Case 1) and a 53-year-old woman (Case 2), with signs of mass effect and amenorrhea, respectively. Both lesions were macroadenomas. The tumor in Case 1 metastasized to dura mater, skull, nasal sinus, and larynx 2 years after patient presentation, whereas that in Case 2 spread to vertebral bodies and ribs after a 19-year latency. Histologically, the primary, recurrent, and metastatic lesions in Case 1 featured brisk mitotic activity and high MIB-1 levels as well as p53 labeling indices. Immunoreactivity for HER-2/neu was assessable only in rare neoplastic cells of the second recurrence and in 80% of cells of the dural metastasis. Low-level HER-2/neu gene amplification was evident in the recurrent tumors and metastasis. The sellar and metastatic tumors in Case 2 resembled benign gonadotropic adenoma with oncocytic change; p53 accumulation, HER-2/neu overexpression, and HER-2/neu gene amplification were not present. The results indicate that low-level amplification of the HER-2/neu gene might be associated with pituitary carcinomas in which more aggressive behavior is seen. Further studies are needed to determine whether HER-2/neu plays a role in the pathogenesis of pituitary carcinoma.     

Comparison of computed tomographic angiography with digital subtraction angiography in the diagnosis of cerebral aneurysms: a meta-analysis

OBJECTIVE: To compare a novel diagnostic radiological technique, computed tomographic angiography (CTA), with the standard method, namely digital subtraction angiography (DSA), in the diagnosis of cerebral aneurysms. METHODS: A comprehensive search of the world literature on CTA was performed. Articles that reported on prospective comparisons of CTA and DSA in the evaluation of patients suspected of harboring cerebral aneurysms were selected for data extraction. Suitable statistical methods were applied to the extracted data for meta-analysis. RESULTS: Twenty-one references met the criteria for use in the meta-analysis. Unweighted calculations based on data for 1251 patients resulted in a sensitivity of 0.933 (93.3%; range, 75.4-100%) and a specificity of 0.878 (87.8%; range, 0-100%). When the studies were weighted for the number of patients in each study, the sensitivity decreased slightly, to 0.927 (92.7%), and the specificity decreased more substantially, to 0.772 (77.2%). CONCLUSION: On the basis of this meta-analysis, DSA remains the standard method. However, many who use CTA have reported it to be as good as or better than DSA in the diagnosis and treatment of cerebral aneurysms, as well as being of less risk and discomfort to their patients and easier and less expensive to perform.     

'Spindle cell oncocytoma' of the adenohypophysis: a tumor of folliculostellate cells?

We describe five primary tumors of the adenohypophysis featuring mitochondrion-rich spindle cells. The patient ages ranged from 53 to 71 years (mean 61.6 years); two were female. All presented with panhypopituitarism. Two also had visual field defect. On neuroimaging all tumors showed suprasellar extension and were indistinguishable from pituitary adenoma. None showed imaging or operative evidence of dural involvement. All were gross totally removed: four by transsphenoidal surgery and one by frontal craniotomy. Follow-up ranged from 2 to 68 months (mean 35.4 months). No recurrences were noted. The clinical workup was noncontributory in all but two patients: one (case no. 4) with an oncocytic thyroid adenoma and another (case no. 5) with squamous carcinoma of both the uterine cervix and of vocal cord. Histologically, the five tumors were composed mainly of fascicles of spindle cells with eosinophilic, granular cytoplasm. Mitoses were rare and necrosis was absent. Neoplastic cells were immunoreactive for vimentin, epithelial membrane antigen, S-100 protein, and galectin-3. Stains for pituitary hormones, synaptophysin, chromogranin, glial fibrillary acidic protein, cytokeratin CAM5.2, smooth muscle actin, CD34, and CD68 were negative. No thyroglobulin immunoreactivity was noted in the tumor of case no. 4. Ultrastructurally, the neoplastic cells contained numerous mitochondria with lamellar cristae. The neoplastic cells were linked by intermediate junctions and desmosomes. No secretory granules were noted. The histologic, immunohistochemical, and fine structural features of these tumors were unlike those of pituitary adenoma or any other primary sellar tumor. A derivation from adenohypophyseal folliculostellate cells is suggested.     

Acute Appendicitis: Still a Surgical Disease? Results from a Propensity Score-Based Outcome Analysis of Conservative Versus Surgical Management from a Prospective Database

Objective

The aim of the present study was to compare the outcomes of conservative versus surgical treatment for acute appendicitis.

Background

Although acute appendicitis is a common disease, great debate exists regarding the appropriate management of patients. Conservative treatment has shown positive results in several RCTs, eliciting questions about indications to surgery, therapeutic appropriateness and ethical conduct.

Methods

Data were prospectively collected; a Propensity Score-based matching method was implemented in order to reduce bias arising from characteristics of the patients; a proportion of patients (69 in total) were excluded to obtain two comparable groups of study (1a). Main outcomes of the study were: failure rate, in-hospital length of stay (at first admission and cumulative), post-discharge absence from work. Within the medical group, failure was defined as the necessity for appendectomy after conservative treatment, while it was identified with complications and negative appendectomy within the surgical group (Failure 1). In parallel, an additional definition of failure was proposed (Failure 2) and excluded negative appendectomy from the reasons for failure within the surgical group (5b).

Results

The failure rate for the conservative treatment resulted to be inferior, as compared to the surgical treatment (16.5 vs. 28.4%, OR 0.523 p = 0.019), considering negative appendectomy as a reason for failure. When excluding negative appendectomy from the definition of failure, medical and surgical treatment appeared to perform equally (failure rate: 16.5 vs. 18.3%, OR 1.014 p = 0.965). Patients managed conservatively showed to have a shorter length of stay at first admission than the patients who underwent appendectomy (3.11 vs. 4.11 days, β = ?0.628 days, p < 0.0001). A lower number of lost work days after discharge resulted from a conservative approach (6 vs. 14.64 days, β = ?8.7 days, p < 0.0001).

Conclusions

Considering each outcome as part of a wide-angle analysis, the conservative management of acute appendicitis resulted to be safe and effective in the selected group of patients. In terms of failure rate, the medical treatment resulted to perform as effectively as surgical treatment, if negative appendectomy was excluded from failure, or better, when negative appendectomy was included in the definition of failure. A diminished length of stay during the first admission and a reduced number of lost work days were evident with a conservative approach. The comparison between medical and surgical treatment for acute appendicitis requires a change in perspective, from a spare ‘effectiveness analysis’ to a more thorough ‘appropriateness analysis’: in the present study, the conservative treatment showed to address the clinical requirements in terms of therapeutic appropriateness. Although acute appendicitis is considered a ‘surgical disease’, increasing evidence supports the effectiveness and safety of a conservative approach for selected groups of patients.

     

Transvaginal uterosacral ligament hysteropexy: a video tutorial

Introduction and hypothesis

Uterine-sparing procedures could be attractive in patients concerned about fertility preservation and corporeal image changes. Transvaginal uterosacral ligaments (USLs) hysteropexy can provide a mesh-free technique for uterine suspension. This video is intended to serve as a tutorial for surgical steps.

Methods

A 38-year-old woman with symptomatic stage III POP desired preserving fertility. After proper counseling, the patient was admitted for vaginal hysteropexy through bilateral high USL suspension according to the featured technique.

Results

Prolapse repair was successfully achieved without complications. We had already published a series of 20 cases that confirmed that transvaginal USLs hysteropexy is a promising technique for correcting genital prolapse with uterus preservation.

Conclusion

Transvaginal USLs hysteropexy provides a feasible technique for apical support without the use of prosthetic material. This procedure could be attractive to women who desire a uterine-sparing surgical option.