Pediatric Cerebral Cavernous Malformations.

Cerebral cavernous malformations are the second most common vascular malformations in the central nervous system, and over one-third are found in children. Lesions may be solitary or multiple, be discovered incidentally, be sporadic, or be secondary to familial cavernomatosis or radiation therapy. Children may present with focal seizures, intracranial hemorrhage, or focal neurological deficits without radiological evidence of recent hemorrhage. We present several children with cerebral cavernous malformations and explore the challenges of their diagnosis in children, their key imaging features, the role of follow-up imaging, and their subsequent management including stereotactic radiosurgery and microsurgical resection. Individual patient risk stratification is advocated for all affected children and their families.

Splenogonadal fusion: a radiologic-pathologic correlation and review of the literature.

We present the case of a 29-year-old male who presented to his General Practitioner with a left testicular lump. Scrotal ultrasound examination revealed 4 well-defined, homogenous, mildly hypoechoic extratesticular mass lesions. He was referred for an urgent urological opinion and underwent local excision. Histologic analysis revealed splenic tissue resulting in the diagnosis of splenogonadal fusion.

Renal vein leiomyosarcoma and renal cell carcinoma presenting together: A case report and discussion on the follow-up.

Leiomyosarcoma affecting the renal vein is rare, with about 30 documented cases in the English literature. The appearance on computed tomography can be difficult to interpret and is often confused with advanced renal cell carcinoma (RCC). This confusion can have implications on the perioperative care of patients presenting with this disease. We report a case with an usual radiological appearance of a renal vein leiomyosarcoma, alongside a separate RCC. This case highlights the need for a high index of suspicion in radiological reporting and provides a dilemma in regards to postoperative surveillance.

Testicular sarcoidosis masquerading as testicular carcinoma.

We present a case of a 27-year-old man with a history of weight loss. A chest x-ray demonstrated hilar lymphadenopathy and he was treated with anti tuberculosis treatment. He noticed a painless left scrotal swelling and a scrotal ultrasound scan raised the possibility of testicular cancer. He underwent an orchidectomy and histology confirmed a testicular sarcoidosis. He was commenced on steroid treatment.

Can endoscopic ultrasound distinguish between mediastinal benign lymph nodes and those involved by sarcoidosis, lymphoma, or metastasis?

BACKGROUND: Lymph nodes (LNs) echofeatures on endoscopic ultrasound (EUS) and concurrent fine needle aspiration (FNA) are alternatives to highly invasive approaches for etiologic diagnosis of mediastinal lymphadenopathy (MLAD). AIMS: To evaluate the efficacy of LNs echofeatures and FNA via EUS to distinguish benign LNs from LNs involved by sarcoidosis, lymphoma, and metastasis in non-lung cancer patients. METHODS: A retrospective review of patients who underwent EUS-FNA for MLAD was performed. Echofeatures of LNs including echogenicity, margins, shape, and LN size were recorded. Final diagnosis was made based on surgical sampling or clinical diagnosis with long-term follow-up. Only patients diagnosed as benign MLAD, sarcoidosis, lymphoma, and metastasis included. Diagnostic value of echofeatures and FNA was evaluated. RESULTS: Included were 162 patients with final diagnosis of benign (68), sarcoidosis (33), lymphoma (20), and metastasis (41). The median LN along axis in the benign group [20.5 mm (6-76)] was significantly shorter than in the metastasis [28 mm (9-82)] and sarcoidosis [27 mm (17-50)] groups (p < 0.05). The median LN short axis in the benign group [11 mm (2-50)] was significantly shorter than in the metastasis [17 mm (5-44)] and lymphoma [16 mm (7-47)] groups (p < 0.05). No other echofeatures showed a discriminant value among the groups. When performing FNA, the sensitivity, specificity, positive predictive value, negative predictive value, and accuracy of EUS-FNA were 73.7, 100, 100, 72.2, and 84.4 %, respectively. CONCLUSION: Although benign MLAD tend to be smaller than other etiologies, echofeatures of LNs are not reliable etiologic diagnostic approach to MLAD. Therefore, FNA is suggested when feasible. However, due to relatively low sensitivity, LNs with benign FNA results should be subjected to further work-up if they are clinically suspicious.

Adult multilocular cystic nephroma: Report of six cases with clinical, radio-pathologic correlation and review of literature.

BACKGROUND: Cystic renal neoplasms of the kidney can be benign or malignant. Multicystic nephroma (MCN) represents a rare benign cystic lesion of the kidney, which usually presents as a unilateral multicystic renal mass without solid elements. According to the World Health Organization (WHO) classification of the renal neoplasms, it is grouped along with mixed epithelial-stromal tumor of the kidney. MATERIALS AND METHODS: We report a retrospective review of six cases of MCN of kidney. Patient demographics, imaging findings, operative details and final histology were recorded. RESULTS: All patients had suspicious/malignant features on radiological examination, leading to a radical nephrectomy. However, microscopically these lesions were lined by cuboidal epithelium, and in a few places hobnail epithelium. No cells with clear cytoplasm, blastemal or immature elements were seen. In one case, foci of inflammatory cells and histiocytes were present. CONCLUSIONS: MCN is a benign cystic lesion and clinical presentations are nonspecific with symptoms such as abdominal pain, hematuria and urinary tract infection. These nonspecific clinical presentations and confusing radiological features create difficult preoperative differentiation from malignant cystic renal neoplasms.

Changes in efficiency and resource utilization after increasing experience with double balloon enteroscopy.

AIM: To investigate changes in efficiency and resource utilization as a single endoscopist's experience increased with each subsequent 100 double balloon enteroscopy (DBE) procedures. METHODS: We reviewed consecutive DBE procedures performed by a single endoscopist at our center over 4 years. DBE was employed when the clinician deemed the procedure was needed for disease management. The approach (oral, anal or both) was chosen based on suspected location of the target lesion. All DBE was performed in a standard endoscopy room with a portable fluoroscopy unit. Fluoroscopy was used to aid in shortening the small intestine and reducing bowel loops. For oral DBE, measurements were taken from the incisors. For anal DBE, measurements were taken from the anal verge. Enteroscopy continued until the target lesion was reached, until the entire small intestine was examined, or until no further progress was deemed possible. The length of small intestine examined (cm), procedure duration (min), and fluoroscopy time (s) were analyzed for sequential groups of 100 DBE. Sub-groups of diagnostic and therapeutic procedures were analyzed using multivariable linear regression. RESULTS: 802 consecutive DBE procedures were analyzed. For oral DBE, median [interquartile range (IQR)] length of small bowel examined was 230.8 cm (range: 210-248 cm) and for anal DBE was 143.5 cm (range: 100-180 cm). No significant increase in length examined was noted for either the oral or anal approach with advancing position in series. In terms of duration of procedure, the median (IQR) for oral DBE was 86 min (range: 71-105 min) and for anal DBE was 81.3 min (range: 67-105 min). When comparing by the position in series, there was a significant (P value < 0.001) decrease in procedure duration for both upper and lower procedures with increasing experience. Median (IQR) time of exposure to fluoroscopy for oral DBE was 190 s (114-275) compared to anal DBE which was 196.4 s (312-128). This represented a significant (P value < 0.001) decrease in the amount of fluoroscopy used with increasing position in series. For both oral and anal DBE, fluoroscopy time was reduced by greater than 50% over the course of 802 total procedures performed. Sub-group analysis was conducted on therapeutic and diagnostic groups. Out of 802 procedures, a total of 434 were considered therapeutic. Argon plasma coagulation was by far the most common therapeutic intervention performed. There was no evidence of a difference in length examined or fluoroscopy exposure among oral DBE for diagnostic and therapeutic procedures, P = 0.91 and P = 0.32 respectively. The median (IQR) for length was 235 cm (range: 178-280 cm) for diagnostic vs 230 cm (range: 180-275 cm) for therapeutic procedures; additionally, fluoroscopy time median (IQR) was 180 s (range: 110-295 s) and 162 s (range: 102-263 s) for no intervention and intervention. However, there was a significant difference in procedure duration among oral DBE (P < 0.001). The median (IQR) was 80 min (range: 60-97 min) and 94 min (range: 77-110 min) for diagnostic and therapeutic interventions respectively. CONCLUSION: For a single endoscopist, increased DBE experience with number of performed procedures is associated with increased efficiency and decreased resource utilization.

Glycemic control after total pancreatectomy for intraductal papillary mucinous neoplasm: an exploratory study.

Background. Glycemic control following total pancreatectomy (TP) has been thought to be difficult to manage. Diffuse intraductal papillary mucinous neoplasm (IPMN) is a potentially curable precursor to pancreatic adenocarcinoma, best treated by TP. Objective. Compare glycemic control in patients undergoing TP for IPMN to patients with type 1 diabetes mellitus (DM). Design/Setting. Retrospective cohort. Outcome Measure. Hemoglobin A1C(HbA1C) at 6, 12, 18, and 24 months after TP. In the control group, baseline was defined as 6 months prior to the first HbA1c measure. Results. Mean HgbA1C at each point of interest was similar between TP and type I DM patients (6 months (7.5% versus 7.7%, P = 0.52), 12 months (7.3% versus 8.0%, P = 0.081), 18 months (7.7% and 7.6%, P = 0.64), and at 24 months (7.3% versus 7.8%, P = 0.10)). Seven TP patients (50%) experienced a hypoglycemic event compared to 65 type 1 DM patients (65%, P = 0.38). Limitations. Small number of TP patients, retrospective design, lack of long-termfollowup. Conclusion. This suggests that glycemic control following TP for IPMNcan be well managed, similar to type 1 DM patients. Fear of DM following TP for IPMN should not preclude surgery when TP is indicated.

A case of mesenteric ischemia secondary to Fibromuscular Dysplasia (FMD) with a positive outcome after intervention.

Fibromuscular dysplasia (FMD) is a non-inflammatory, non-atherosclerotic angiopathy, which commonly affects the renal and internal carotid arteries. Although rare, FMD has the potential of involving the mesenteric vasculature. Due its low incidence and relatively little knowledge concerning its risk factors and etiology, actual diagnosis of FMD involving the mesenteric vessels requires a very high degree of suspicion. Upon review of the few reported cases of FMD causing mesenteric ischemia, it is clear that therapeutic interventions are rarely discussed and that positive outcomes are even more uncommon. Herein, we present the case of a 47 year-old female with mesenteric ischemia secondary to FMD, which was diagnosed and treated originally with angioplasty, then repeat angioplasty with stent placement, and finally with a bypass graft. Ultimately, the patient had a positive outcome, including eight month follow-up.

Trainee participation is associated with increased small adenoma detection.

BACKGROUND: Previous studies examining the effect of fellow participation on adenoma detection rate in colonoscopy have yielded conflicting results, and factors such as adenoma size and location have not been rigorously evaluated. OBJECTIVE: To examine whether fellow participation during screening, surveillance, or diagnostic colonoscopy affects overall, size-specific, or location-specific adenoma or polyp detection rate. METHODS: This was a retrospective study of 2430 colonoscopies performed in our ambulatory surgical center between September 2006 and December 2007, comparing adenoma and polyp detection rates of colonoscopies performed by fellows with supervising staff endoscopists (n = 318) with colonoscopies performed by staff endoscopists without fellow participation (n = 2112). Study participants included patients who underwent screening, surveillance, or diagnostic colonoscopies in our GI suite. Logistic regression analysis was used to evaluate the association of fellow participation with adenoma and polyp detection. RESULTS: There was evidence of a higher rate of small (<5 mm) adenoma detection in colonoscopies with a fellow present (25% vs 17%, P = .001). This remained significant after multiple-testing adjustment (P ≤ .003 considered significant). Findings were similar, although not significant for small polyps (36% vs 29%, P = .007). There was a trend toward increased adenoma detection in colonoscopies with a fellow present compared with those without (30% vs 26%, P = .11). Multivariable adjustment for potentially confounding variables did not alter these associations. LIMITATIONS: The study had a retrospective design, and information regarding bowel preparation was not available for 37% of patients. CONCLUSION: Fellow involvement was associated with increased detection rates of small adenomas, providing evidence that the presence of a fellow during colonoscopy plays a role in enhancing the effectiveness of the examination.

The learning curve of in vivo probe-based confocal laser endomicroscopy for prediction of colorectal neoplasia.

BACKGROUND: Probe-based confocal laser endomicroscopy (pCLE) is an emerging tool for in vivo imaging of the GI tract that requires the endoscopist to interpret microscopic images. The learning curve for interpretation of pCLE images is unknown. OBJECTIVE: To examine the learning curve of correctly identifying benign and neoplastic colorectal lesions by using pCLE and to evaluate the learning curve of obtaining high-quality images. DESIGN: Prospective, double-blind review of pCLE images of 76 colorectal lesions by using corresponding polypectomies as the reference standard. A training set of 20 images with known histology was first reviewed to standardize image interpretation, followed by blinded review of 76 unknown images. SETTING: Eleven endoscopists from 3 different endoscopy centers evaluated the images obtained by 1 endoscopist using the high-definition confocal probe. PATIENTS: Patients undergoing screening and surveillance colonoscopies. INTERVENTION: Intravenous fluorescein pCLE imaging of colorectal lesions followed by polypectomies. MAIN OUTCOME MEASUREMENTS: Accuracy of image interpretation with constructing learning curve for pCLE image interpretation and acquisition. RESULTS: Of the 76 colorectal lesions, 51 (67%) were neoplastic and 25 (33%) were benign, based on histopathology. Accuracy for the overall group was 63% for lesions 1 to 20, 64% for lesions 21 to 40, 79% for lesions 41 to 60, and 86% for lesions 61 to 76. The ability to obtain high-quality images was stable over the 76 cases. LIMITATIONS: Small sample size and use of offline video sequences. CONCLUSIONS: Accurate interpretation of pCLE images for predicting neoplastic lesions can be learned rapidly by a wide range of GI specialists. Furthermore, the ability to acquire high-quality pCLE images is also quickly learned.

An evaluation of risk factors for inadequate cytology in EUS-guided FNA of pancreatic tumors and lymph nodes.

BACKGROUND: The factors associated with maximizing the cytological adequacy of EUS-guided FNA (EUS-FNA) in pancreatic tumor evaluation are not well-known. OBJECTIVE: To examine associations of physician and procedural factors with the endpoint: the presence of an adequate cytological specimen found by using EUS-FNA in patients with pancreatic tumors and lymph nodes. DESIGN: Retrospective cohort study. SETTING: A U.S. tertiary care center. PATIENTS: Patients undergoing EUS-FNA of pancreatic masses and lymph nodes. INTERVENTIONS: Analysis of EUS-FNA procedures performed in our institution from 1997 to 2007. MAIN OUTCOME MEASUREMENTS: Associations were evaluated between the primary endpoint of cytological adequacy and factors including the endoscopist, needle gauge, the number of needle passes attempted, the pathologist, and the presence of an onsite cytotechnologist to confirm an adequate specimen. EUS-FNA adequacy was determined by a pathologist based on the presence of definite benign or malignant tissue. RESULTS: EUS-FNA was performed in 247 pancreatic masses and 276 lymph nodes. An adequate cytological sample was obtained in 240 (97%) pancreatic tumors (95% CI, 94%-99%) and 252 (91%) lymph nodes (95% CI, 87%-94%). For pancreatic tumors, there was no evidence of any associations between factors and cytological adequacy. For lymph nodes, cytological adequacy was improved when an onsite cytotechnologist was present (96% vs 84%, P = .002); no other factors showed statistically significant associations with cytological adequacy. LIMITATIONS: Retrospective study, low power to detect associations. CONCLUSIONS: The presence of an onsite cytotechnologist is an important factor in achieving successful EUS-FNA of suspicious lymph nodes in patients with pancreatic masses.

Endosonographic features predictive of malignancy in mediastinal lymph nodes in patients with lung cancer.

BACKGROUND: EUS is useful in determining mediastinal lymph node (LN) metastases in patients undergoing staging for lung cancer. However, FNA of LNs is often performed only if suspicious features are present. The utility of individual LN features in predicting malignant cytology remains unclear. OBJECTIVE: To evaluate the utility of EUS-determined LN features for predicting malignant cytology. DESIGN: Prospective observational study. SETTING: Two U.S. tertiary-care centers. PATIENTS: This study involved 425 patients with primary lung cancer who underwent EUS. INTERVENTION: All mediastinal LNs were described according to size, shape, echogenicity, and margin characteristics. FNA was performed on LNs with any features suggestive of malignancy. EUS-guided FNA cytology was classified as benign or abnormal (suspicious/malignant). The utility of LN features in predicting malignant cytology was determined and further analyzed by logistic regression, and a predictive model was established. MAIN OUTCOME MEASUREMENTS: Accuracy of individual LN features for predicting malignancy. RESULTS: EUS detected 836 LNs in 425 patients, and FNA was obtained in 698 patients. On multivariable analysis, only round shape, a short axis of >8.3 mm, and sharp margins were predictive of malignant cytology. According to the predictive model, the calculated probability of having malignancy is less than 4% (95% confidence interval [CI], 0.022-0.064) when none of the LN features are present and 63% (95% CI, 51%-72.2%) when all features were seen. LIMITATIONS: No surgical histology as the criterion standard. CONCLUSION: Among patients with lung cancer, EUS features of round shape, sharp margins, and short axis of >8.3 mm are significant predictors of malignancy. The probability of malignancy is low when none of the features are present.

Endoscopic spray cryotherapy for esophageal cancer: safety and efficacy.

BACKGROUND: Few options exist for patients with localized esophageal cancer ineligible for conventional therapies. Endoscopic spray cryotherapy with low-pressure liquid nitrogen has demonstrated efficacy in this setting in early studies. OBJECTIVE: To assess the safety and efficacy of cryotherapy in esophageal carcinoma. DESIGN: Multicenter, retrospective cohort study. SETTING: Ten academic and community medical centers between 2006 and 2009. PATIENTS: Subjects with esophageal carcinoma in whom conventional therapy failed and those who refused or were ineligible for conventional therapy. INTERVENTIONS: Cryotherapy with follow-up biopsies. Treatment was complete when tumor eradication was confirmed by biopsy or when treatment was halted because of tumor progression, patient preference, or comorbid condition. MAIN OUTCOME MEASUREMENTS: Complete eradication of luminal cancer and adverse events. RESULTS: Seventy-nine subjects (median age 76 years, 81% male, 94% with adenocarcinoma) were treated. Tumor stage included T1-60, T2-16, and T3/4-3. Mean tumor length was 4.0 cm (range 1-15 cm). Previous treatment including endoscopic resection, photodynamic therapy, esophagectomy, chemotherapy, and radiation therapy failed in 53 subjects (67%). Forty-nine completed treatment. Complete response of intraluminal disease was seen in 31 of 49 subjects (61.2%), including 18 of 24 (75%) with mucosal cancer. Mean (standard deviation) length of follow-up after treatment was 10.6 (8.4) months overall and 11.5 (2.8) months for T1 disease. No serious adverse events were reported. Benign stricture developed in 10 (13%), with esophageal narrowing from previous endoscopic resection, radiotherapy, or photodynamic therapy noted in 9 of 10 subjects. LIMITATIONS: Retrospective study design, short follow-up. CONCLUSIONS: Spray cryotherapy is safe and well tolerated for esophageal cancer. Short-term results suggest that it is effective in those who could not receive conventional treatment, especially for those with mucosal cancer.

Variation in Barrett's esophageal wall thickness: is it associated with histology or segment length?

GOALS: To measure esophageal wall thickness (EWT) with endoscopic ultrasound (EUS) in patients with and without Barrett's esophagus (BE). BACKGROUND: Segment length and histology are used to evaluate BE. The development of varying depths of ablation therapy has renewed interest in using EUS for BE. STUDY: In this prospective study, EWT measurements were taken from the balloon-mucosal interface to the outer most hyper-echoic line. These measurements were correlated with the highest grade of BE dysplasia and segment length, and then compared with the measurements from control group. RESULTS: Between 2004 to 2007, 76 BE patients (69 men, mean age 68 y, 4 ND, 14 low-grade dysplasia, 52 high-grade dysplasia, 6 carcinoma in situ) and 53 normal controls (18 men, mean age 60 y) underwent EUS. The mean EWT was 2.4 mm for controls, 3.1 mm for nondysplastic BE, 3.2 mm for low-grade dysplasia, 3.4 mm for high-grade dysplasia, and 3.9 mm for carcinoma in situ. In the control group of 53 patients, the mean EWT was 2.4 mm. Compared with normal controls, the mean EWT was significantly greater in all histologic subgroups of BE patients (P<0.001). No statistically significant correlation was seen between EWT and BE histology grade. There were no correlations between age, gender, or BE segment length and EWT (P=0.55). CONCLUSIONS: EWT is greater among patients with BE compared with control patients; however, there were no systematic differences in EWT were found among BE patients, based on histology and segment length.

Lung cancer staging by combined endobronchial ultrasound (EBUS) and endoscopic ultrasound (EUS): The gastroenterologist's perspective.

This review deals with the combined approach of endoscopic ultrasound and endobronchial ultrasound for lung cancer staging. The review provides an overview for the gastroenterologist who performs endosonography with regard to the current evidence supporting the use of endoscopic ultrasound and endobronchial ultrasound in clinical practice.

High-definition colonoscopy detects colorectal polyps at a higher rate than standard white-light colonoscopy.

BACKGROUND & AIMS: Adenoma detection rates might be improved through use of high-definition colonoscopy, which can detect subtle mucosal changes. We investigated whether the use of high-definition white-light (HDWL) colonoscopy resulted in a higher rate of adenoma detection than standard-definition white-light (SDWL) colonoscopy in a clinical practice setting. METHODS: This retrospective study included 2430 patients who underwent colonoscopies from September 2006 to December 2007; 1226 received SDWL colonoscopy and 1204 received HDWL colonoscopy. We analyzed data from consecutive screening, surveillance, and diagnostic colonoscopies, comparing adenoma and overall polyp detection between procedures. Potentially confounding variables were controlled using multivariable logistic regression analysis. RESULTS: The adenoma detection rate was higher among patients who underwent HDWL compared with SDWL colonoscopies (28.8% vs 24.3%; P = .012), as was the polyp detection rate (42.2% vs 37.8%; P = .026). These findings remained after adjustments for potentially confounding variables (P = .018 and .022, respectively). CONCLUSIONS: In a general clinical practice setting, HDWL colonoscopy resulted in a higher adenoma detection rate compared with SDWL colonoscopy. The use of SDWL colonoscopy could reduce the number of missed adenomas and the subsequent risk for colorectal cancer.

Economic analysis of combined endoscopic and endobronchial ultrasound in the evaluation of patients with suspected non-small cell lung cancer.

Lung cancer remains the most common cause of cancer-related death in the United States. This study evaluated the costs of alternative diagnostic evaluations for patients with suspected non-small cell lung cancer (NSCLC). Researchers used a cost-minimization model to compare various diagnostic approaches in the evaluation of patients with NSCLC. It was less expensive to use an initial endoscopic ultrasound (EUS) with fine needle aspiration (FNA) to detect a mediastinal lymph node metastasis ($18,603 per patient), compared with combined EUS FNA and endobronchial ultrasound (EBUS) with FNA ($18,753). The results were sensitive to the prevalence of malignant mediastinal lymph nodes; EUS FNA remained least costly, if the probability of nodal metastases was <32.9%, as would occur in a patient without abnormal lymph nodes on computed tomography (CT). While EUS FNA combined with EBUS FNA was the most economical approach, if the rate of nodal metastases was higher, as would be the case in patients with abnormal lymph nodes on CT. Both of these strategies were less costly than bronchoscopy or mediastinoscopy. The pre-test probability of nodal metastases can determine the most cost-effective testing strategy for evaluation of a patient with NSCLC. Pre-procedure CT may be helpful in assessing probability of mediastinal nodal metastases.