Small Bowel Imaging from Stepchild of Roentgenology to MR Enterography, Part II: The Reliable Disclosure of Crohn's Disease and Non-Inflammatory Small Bowel Disorder Plot through MRI Findings.

MRE has become a standard imaging test for evaluating patients with small bowel pathology, but a rigorous methodology for describing and interpreting the pathological findings is mandatory. Strictures, abscess, inflammatory activity, sinus tract, wall edema, fistula, mucosal lesions, strictures, and mesentery fat hypertrophy are all indicators of small bowel damage in inflammatory and non-inflammatory small bowel disease, and they are all commonly and accurately explained by MRE. MRE is a non-invasive modality that accurately assesses the intra-luminal, parietal, and extra-luminal small bowel. Differential MRE appearance allows us to distinguish between Crohn's disease and non-inflammatory small bowel disorder. The purpose of this paper is to present the MRE pathological findings of small bowel disorder.

Small Bowel Imaging from Stepchild of Roentgenology to MR Enterography: Part I: Guidance in Performing and Observing Normal and Abnormal Imaging Findings.

MRE has become a standard imaging test for evaluating patients with small bowel pathology, but the indications, interpretation of imaging findings, methodology, and appropriate use must be standardized and widely known. Several signs of small bowel damage in inflammatory and non-inflammatory small bowel pathology include strictures, abscess, inflammatory activity, sinus tract, wall edema, fistula, mucosal lesions, and mesentery fat hypertrophy, all of which are widely and accurately explained by MRE. MRE is a non-invasive modality that accurately assesses the intra-luminal, parietal, and extra-luminal small bowel. The differential MRE appearance allows us to distinguish between different small bowel pathologies, such as neoplastic and non-neoplastic small bowel diseases. The purpose of this paper is to present the MRE technique, as well as the interpretation of imaging findings, through the approach of a rigorous stepwise methodology.

Colitis cystica profunda of the rectum with adenomatous dysplastic features: Radiologic-pathologic correlation.

Colitis cystica profunda is a rare nonneoplastic condition characterized by the presence of mucus-containing cysts in the submucosa of the right colon and rectum. The etiology is unclear, with a few cases reported in the literature. The presenting symptoms and signs may mimic colorectal adenocarcinoma. We report a case of colitis cystica profunda localized in the rectum, investigated by colonoscopy, CT, MRI, and subsequently surgically treated.

Bayesian multi-dipole modelling in the frequency domain.

BACKGROUND: Magneto- and Electro-encephalography record the electromagnetic field generated by neural currents with high temporal frequency and good spatial resolution, and are therefore well suited for source localization in the time and in the frequency domain. In particular, localization of the generators of neural oscillations is very important in the study of cognitive processes in the healthy and in the pathological brain. NEW METHOD: We introduce the use of a Bayesian multi-dipole localization method in the frequency domain. Given the Fourier Transform of the data at one or multiple frequencies and/or trials, the algorithm approximates numerically the posterior distribution with Monte Carlo techniques. RESULTS: We use synthetic data to show that the proposed method behaves well under a wide range of experimental conditions, including low signal-to-noise ratios and correlated sources. We use dipole clusters to mimic the effect of extended sources. In addition, we test the algorithm on real MEG data to confirm its feasibility. COMPARISON WITH EXISTING METHOD(S): Throughout the whole study, DICS (Dynamic Imaging of Coherent Sources) is used systematically as a benchmark. The two methods provide similar general pictures; the posterior distributions of the Bayesian approach contain much richer information at the price of a higher computational cost. CONCLUSIONS: The Bayesian method described in this paper represents a reliable approach for localization of multiple dipoles in the frequency domain.

Giant angioleiomyoma of uterus: A case report with focus on CT imaging.

We report a rare case of giant angioleiomyoma located in the uterus and detected in a 37-year-old woman. The uterus is an extremely rare location for angioleiomyoma. The definitive diagnosis is usually obtained only after the histopathologic examination because the imaging criteria are challenging for this disease. We focused our attention on the main computed tomography features able to provide a robust preoperative diagnosis of this rare clinical entity.

The network sustaining action myoclonus: a MEG-EMG study in patients with EPM1.

BACKGROUND: To explore the cortical network sustaining action myoclonus and to found markers of the resulting functional impairment, we evaluated the distribution of the cortico-muscular coherence (CMC) and the frequency of coherent cortical oscillations with magnetoencephalography (MEG). All patients had EPM1 (Unverricht-Lundborg) disease known to present with prominent and disabling movement-activated myoclonus. METHODS: Using autoregressive models, we evaluated CMC on MEG sensors grouped in regions of interests (ROIs) above the main cortical areas. The movement was a repeated sustained isometric extension of the right hand and right foot. We compared the data obtained in 10 EPM1 patients with those obtained in 10 age-matched controls. RESULTS: As expected, CMC in beta band was significantly higher in EPM1 patients compared to controls in the ROIs exploring the sensorimotor cortex, but, it was also significantly higher in adjacent ROIs ipsilateral and contralateral to the activated limb. Moreover, the beta-CMC peak occurred at frequencies significantly slower and more stable frequencies in EPM1 patients with respect to controls. The frequency of the beta-CMC peak inversely correlated with the severity of myoclonus. CONCLUSIONS: the high and spatially extended beta-CMC peaking in a restricted range of low-beta frequencies in EPM1 patients, suggest that action myoclonus may result not only from an enhanced local synchronization but also from a specific oscillatory activity involving an expanded neuronal pool. The significant relationship between beta-CMC peak frequency and the severity of the motor impairment can represent a useful neurophysiological marker for the patients' evaluation and follow-up.

Altered EEG resting-state effective connectivity in drug-naïve childhood absence epilepsy.

OBJECTIVE: We investigated interictal EEG activity in patients with childhood absence seizures with the aim of detecting markers of network defects generating "idiopathic" hyperexcitability in this form of epilepsy. METHODS: We included 11 drug-naïve patients with childhood absence epilepsy (CAE), and 11 age matched controls (CTRL). We analyzed interictal EEG using partial directed coherence (PDC), a connectivity estimator in frequency domain based on autoregressive multivariate (MVAR) modeling giving the advantage of indicating the direction and strength of the interactions between multiple variables. RESULTS: Our results revealed the presence of an abnormal cortico-cortical network occurring in the interictal condition in CAE and involving a large span of frequencies, with prominence in the alpha band; the most evident finding was a highly significant increase of out-going connectivity involving frontal and central cortical areas in CAE patients compared to CTRL subjects. CONCLUSIONS: Our observation indicates that, in interictal conditions, a distorted network characterizes CAE, and a hyperconnected network is already detectable under resting conditions in the delta, theta and alpha bands. SIGNIFICANCE: The increased interictal EEG connectivity demonstrated here provides support for a persistent abnormal relationship between the thalamus and a hyperexcitable cortex outside the ictal phase.

Identification of the Epileptogenic Zone from Stereo-EEG Signals: A Connectivity-Graph Theory Approach.

In the context of focal drug-resistant epilepsies, the surgical resection of the epileptogenic zone (EZ), the cortical region responsible for the onset, early seizures organization, and propagation, may be the only therapeutic option for reducing or suppressing seizures. The rather high rate of failure in epilepsy surgery of extra-temporal epilepsies highlights that the precise identification of the EZ, mandatory objective to achieve seizure freedom, is still an unsolved problem that requires more sophisticated methods of investigation. Despite the wide range of non-invasive investigations, intracranial stereo-EEG (SEEG) recordings still represent, in many patients, the gold standard for the EZ identification. In this contest, the EZ localization is still based on visual analysis of SEEG, inevitably affected by the drawback of subjectivity and strongly time-consuming. Over the last years, considerable efforts have been made to develop advanced signal analysis techniques able to improve the identification of the EZ. Particular attention has been paid to those methods aimed at quantifying and characterizing the interactions and causal relationships between neuronal populations, since is nowadays well assumed that epileptic phenomena are associated with abnormal changes in brain synchronization mechanisms, and initial evidence has shown the suitability of this approach for the EZ localization. The aim of this review is to provide an overview of the different EEG signal processing methods applied to study connectivity between distinct brain cortical regions, namely in focal epilepsies. In addition, with the aim of localizing the EZ, the approach based on graph theory will be described, since the study of the topological properties of the networks has strongly improved the study of brain connectivity mechanisms.

A very advanced case of a T cell peritoneal lymphomatosis.

Small-bowel lymphoma is not a common disease, accounting for 15-20% of primary extranodal gastrointestinal lymphomas. Peritoneal lymphomatosis is considered a rare and aggressive presentation. We describe the case of a 55 years-old man affected by T-cell intestinal lymphoma, presenting with diffuse abdominal involvement, bowel dysfunction, severe ascites and pleural effusion, who underwent surgery. Clinical course led dramatically to death. Preoperative cytology and radiologic investigations did not yield diagnosis and were unable to differentiate between peritoneal carcinosis and lymphomatosis. It is suggested that, in such advanced cases, with rapidly deteriorating clinical conditions and huge systemic involvement, surgery is not indicated. On the contrary, maximum effort has to be spent to obtain a preoperative diagnosis.

Concomitant boost radiotherapy and multidrug chemotherapy in the neoadjuvant treatment of locally advanced rectal cancer: results of a phase II study.

BACKGROUND: An intensified multidrug chemotherapy regimen (raltitrexed plus oxaliplatin, Tom-Ox) plus concomitant boost radiotherapy, in the neoadjuvant treatment of locally advanced rectal cancer patients, was shown feasible in our previous study. The aim of this study was to evaluate the efficacy in terms of pathologic complete response to pre-operative therapy. MATERIAL AND METHODS: A Phase II study was designed and clinical stage T3-T4 and/ or N ≥ 1 patients were treated with concomitant boost radiotherapy (55 Gy/5 weeks) plus concurrent chemotherapy (Tom-Ox). The primary endpoint was the assessment of efficacy in terms of clinical and pathologic response to pre-operative therapy. According to the Gehan's design study, 25 patients were enrolled. Toxicity was assessed according to the RTOG-EORTC and CTCAE v.3.0 criteria. RESULTS: Twenty-five consecutive patients were treated. Twenty-two of the 25 (88%) patients had a partial clinical response at the time of pre-operative magnetic resonance imaging (MRI). Only one patient showed progressive systemic disease at pre-surgical revaluation and was subjected only to biopsy to evaluate pathological response. Twenty-four patients (96%) underwent surgery. Overall, pathologic complete response was observed in eight patients (32%; CI 0.95:12-55%) and only microscopic tumor foci (pTmic) in two patients (pT0-mic: 40%; CI 0.95:18-63%). Nineteen patients (76%) showed tumor down-staging. Proctitis and/or diarrhea were the most frequent acute side effects experienced. Eighteen patients had grade 1-2 toxicity (77%); whereas two patients experienced grade 3 toxicity (8%). Two-year Local control and actuarial Disease Free Survival were 100% and 91%, respectively. CONCLUSION. An intensified regimen of concomitant boost radiotherapy plus concurrent raltitrexed and oxaliplatin, can be safely administered in patients with locally advanced rectal cancer. This regimen produces high rates of pathological complete response. Based on available data, this type of treatment could be offered to patients with more advanced tumors (T4 or local recurrence).

[Gastrointestinal stromal tumors]. / Tumori stromali gastrointestinali.

Gastrointestinal stromal tumor (GIST) account for 1% of all gastrointestinal neoplasms and are the most common mesenchymal tumor of gastrointestinal tract. There are considered to originate fom the intestinal cell of Cajal, an intestinal pacemaker cell, characterized usually express the KIT protein on immunohistochemistry. The stomach (40-60%) and small intestine (30-40%) are the most common locations. Diagnosis of these tumors is difficult to establish, because symptoms are vague and traditional diagnostic tests are not specific. GISTs shows a wide variety of clinical behaviours ranging fom benign to frankly malignant, making the outcome totally unpredictable. Surgery is the standard treatment of local GIST while Imatinib (tyrosine kinasi inhibitor) is considered as the standard treatment of metastatic disease. Resistence to Imatinib is also becoming a major clinical problem but new tirosyne kinase inibitor are being studied to improve the treatment and survival. The present paper is a review of the salient features of epidemiology, pathophysiology, diagnosis, therapy and prognostic factors of GIST

Emergency high-loop jejunostomy as enteral nutrition access: a simple and safe method.

BACKGROUND: Emergency high-loop jejunostomies are seldom used for nutrition access in the clinical practice. METHODS: This paper describes the results of a simple and safe technique that uses emergency high-loop jejunostomy as an enteral feeding access. A feeding tube is inserted into the efferent loop of the jejunostomy and then subcutaneously tunneled. In this way, whenever it becomes necessary, the bag collecting fluids from the afferent loop can be changed without removing the tube, which remains permanently inserted into the efferent loop and secured to the skin in order to avoid displacements. RESULTS: Twenty-nine patients with high-loop jejunostomy were consecutively treated with the described technique during the period 2000-2006. The mean distance between the ligament of Treitz and tube was 38.3 +/- 16.2 cm. After an induction period, all patients received full-strength enteral nutrition and were discharged after a mean of 25.1 +/- 19.5 days of treatment. All patients were subsequently readmitted to our unit, and their ostomies were successfully closed. No major early and late complications were observed; particularly, no patient experienced local or systemic septic complications. CONCLUSION: From the analysis of our results, the described method for delivering enteral nutrition through an emergency high-loop ostomy proves easy to apply and clinically effective. Enteral nutrition can be started as soon as possible after operation through the efferent loop of the ostomy; the management of the jejunostomy is simple and safe, with no additional discomfort for the patients.

[Presentation, treatment and prognosis of intraductal papillary mucinous neoplasm]. / Aspetti clinici, trattamento e prognosi della neoplasia papillare intraduttale mucosecernente.

Intraductal papillary mucinous neoplasms (IPMNs) are rare tumours rising from the pancreatic duct epithelium. They are characterized by intraductal papillary growth and thick mucin secretion; mucin fills the Wirsung and/or branch pancreatic ducts and may cause ductal dilatation. IPMNs are classified into three types, according to the site of involvement: main duct type, branch duct type, and combined type. Most branch type IPMNs are benign, while the other two types are frequently malignant. Recent advances in diagnostic imaging have led to an increased frequency of diagnosis of IPMNs, but the clinical features of them can range broadly from benign, borderline, and malignant non-invasive to invasive lesions, and their management has not yet been clearly defined. The most of patients are asymptomatic. The possibility of malignancy is increased in cases which large mural nodules are presented. Presence of a large branch type IPMN and marked dilatation of the main duct indicate the existence of adenoma. Not infrequently, synchronous or metachronous malignancies may be developed in various organs. Endoscopic retrograde cholangiopancreatography (ERCP), endoscopic ultrasonography (EUS), intraductal ultrasonography, and magnetic resonance cholangiopancreatography (MRCP) are the most valuable imaging techniques for diagnosis of these lesions. Prognosis is excellent after complete resection of benign and non-invasive malignant IPMNs. Total pancreatectomy should be reserved for patients with resectable but extensive IPMN involving the whole pancreas; its benefits must be balanced against perioperative risks.

Risk factors associated with pancreatic fistula after distal pancreatectomy, which technique of pancreatic stump closure is more beneficial?

AIM: To identify risk factors related to pancreatic fistula in patients undergoing distal pancreatectomy (DP) and to determine the effectiveness of using a stapled and a sutured closed of pancreatic stump. METHODS: Sixty-four patients underwent DP during a 10-year period. Information regarding diagnosis, operative details, and perioperative morbidity or mortality was collected. Eight risk factors were examined. RESULTS: Indications for DP included primary pancreatic disease (n=38, 59%) and non-pancreatic malignancy (n=26, 41%). Postoperative mortality and morbidity rates were 1.5% and 37% respectively; one patient died due to sepsis and two patients required a reoperation due to postoperative bleeding. Pancreatic fistula was developed in 14 patients (22%); 4 of fistulas were classified as Grade A, 9 as Grade B and only 1 as Grade C. Incidence of pancreatic fistula rate was significantly associated with four risk factors: pathology, use of prophylactic octreotide therapy, concomitant splenectomy, and texture of pancreatic parenchyma. The role that technique (either stapler or suture) of pancreatic stump closure plays in the development of pancreatic leak remains unclear. CONCLUSION: The pancreatic fistula rate after DP is 22%. This is reduced for patients with non-pancreatic malignancy, fibrotic pancreatic tissue, postoperative prophylactic octreotide therapy and concomitant splenectomy.

Gastrointestinal involvement in a case of hereditary angioedema: could the early weaning have had a role?

Hereditary angioedema (HAE) is a noninflammatory disorder due to reduced C1-inhibitor level and/or function and characterized by recurrent, circumscribed, and self-limiting episodes of cutaneous and mucous membrane swellings involving different organs. A heterogeneous group of mutations in the C1-inhibitor gene have been found. HAE might present with diverse clinical pictures, even within families with the same mutation, but the cause of this variability is not known yet. We describe the case of type II HAE in a young adult presenting with recurrent abdominal pain for many years, occasionally associated with ascites. We suppose that an early weaning might have influenced his phenotype, making his gastrointestinal tract a "vulnerable organ," in which hereditary angioedema could express itself.

Retroperitoneal schwannomas: diagnostic and therapeutic implications.

AIMS AND BACKGROUND: Schwannomas are a rare group of soft-tissue tumors that are derived from the peripheral nerve sheath and rarely develop in the retroperitoneum. METHODS AND STUDY DESIGN: We reviewed the clinicopathological features of 4 patients referred to our unit between October 1999 and March 2004 who on radiological examination were diagnosed with pancreatic, adrenal, psoas and retroperitoneal fat tissue tumors and subsequently underwent surgical treatment. RESULTS: The preoperative diagnosis was incorrect in all cases. At time of surgery, we found a mass probably arising from the adrenal gland in 2 patients, a lesion originating from the femoral nerve in 1 patient, and a retroperitoneal mass without a clear site of origin in 1 patient. Pathological evaluation revealed schwannomas in all cases, with no signs of malignancy. Complete surgical excision was performed in all patients without any major postoperative complications. At the time of writing all patients are alive with no evidence of local or distant recurrence. CONCLUSIONS: Radical surgical excision is considered the best treatment for these neoplasms, resulting in a very good longterm prognosis.

[Retroperitoneal Castleman's disease. A report of two cases and analysis of the literature]. / Malattia di Castleman retroperitoneale. Due casi clinici e analisi della letteratura.

Castleman's disease is a rare disorder of the lymphoid tissue with three possible histological variants--the hyalin-vascular type, with a good prognosis, the plasma-cellular type and the mixed type; the latter two are both more aggressive than the hyalin-vascular type. Two clinical types of this disease have already been described: the localized or unifocal type and the multicentric or multifocal type. The aetiology of Castleman's disease remains unclear due to polymorphic clinical features that give rise to many diagnostic and treatment problems. Its diagnosis, therefore, can only be confirmed by histological examination. Surgical treatment is the treatment of choice in patients with Castleman's disease, but radical removal of the tumour mass, especially in the multicentric type, is not always possible. We are still in no position to draw definitive conclusions as to treatment, because there are only a few reports with different regimens regarding patients with multicentric Castleman's disease. A better understanding of the pathogenesis of this rare disorder may help in deciding the best treatment approach. In this study, we report two cases of Castleman's disease, one hyalin-vascular and the other plasma-cellular, both of which were unifocal and located in a retroperitoneal-pararenal site. We also analyse the main clinical, diagnostic and treatment problems associated with this rare condition, with an overview of the literature.

[Chronic pain after inguinal hernia mesh repair: possible role of surgical manipulation of the inguinal nerves. A prospective multicentre study of 973 cases]. / Il dolore cronico dopo ernioplas ica inguinale con protesi: il possibile ruolo della manipolazione chirurgica dei nervi del canale inguinale.

The aim of the study was to evaluate whether the surgical treatment reserved for the ilioinguinal, iliohypogastric and genital branches of the genitofemoral nerves, during open hernia mesh repair, is effective in reducing chronic post-operative pain. A multicentre prospective study involving 11 Italian Institutions led to the recruitment of 973 cases of hernioplasty. All surgeons were asked to report whether or not each nerve had been identified and preserved or divided. The main endpoint of the study was the evaluation of moderate-severe chronic pain at 6 months and 1 year. Overall, presence of groin pain at 6 months and 1 year follow-up was 9.7% and 4.1%, respectively. Pain was mild in 7.9% and moderate-to-severe in 2.1% at 6 months, and mild in 3.6% and moderate-to-severe in 0.5% at 1 year. Univariate and multivariate analysis showed that lack of identification of nerves is significantly correlated with presence of chronic pain, the risk of developing inguinal pain increasing with the number of nerves not detected. Likewise, division of nerves was clearly correlated with presence of chronic pain. The present findings indicate that identification and preservation of nerves during open inguinal hernia repair reduce chronic incapacitating groin pain.

Influence of preservation versus division of ilioinguinal, iliohypogastric, and genital nerves during open mesh herniorrhaphy: prospective multicentric study of chronic pain.

OBJECTIVE: To evaluate whether the various surgical treatment reserved for ilioinguinal, iliohypogastric, and genital branch of the genitofemoral nerves, during open hernia mesh repair, is effective in reducing chronic postoperative pain. BACKGROUND: Interest in chronic groin pain following herniorrhaphy has escalated, in recent years, due both to treatment and legal implications. However, much debate still exists concerning which treatment to reserve for the 3 inguinal sensory nerves. METHODS: A multicentric prospective study involving 11 Italian institutions led to the recruitment of 973 cases of hernioplasty. All surgeons were asked to report whether or not each nerve had been identified and preserved or divided. The main endpoint of the study was the evaluation of moderate to severe chronic pain at 6 months and 1 year. RESULTS: Overall, the presence of groin pain at the 6-month and 1-year follow-up was 9.7% and 4.1%, respectively. Pain was mild in 7.9% and moderate to severe in 2.1%, at 6 months, and mild in 3.6% and moderate to severe in 0.5%, at 1 year. Univariate and multivariate analysis showed that lack of identification of nerves is significantly correlated with presence of chronic pain, the risk of developing inguinal pain increasing with the number of nerves not detected. Likewise, division of nerves was clearly correlated with presence of chronic pain. CONCLUSIONS: The present findings indicate that identification and preservation of nerves during open inguinal hernia repair reduce chronic incapacitating groin pain and that, in the majority of patients with chronic pain at 6 months, the pain at 1 year is resolved only with conservative or medical treatment.