Giant sigmoid diverticulum (GSD) is a rare complication of diverticulosis. These lesions arise from herniations of the mucosa through the muscle wall which progressively enlarge with colonic gas to become large air-filled cysts evident on plain X-ray and CT scans. We present a rare case of a 72-year-old female presenting with abdominal distention, abdominal tenderness, and fever who developed a type 1 giant sigmoid diverticulum (pseudodiverticulum) that subsequently formed an intra-abdominal abscess and an accompanying type 2 diverticulum as well. The patient was treated with surgical resection of the diverticulum with a primary anastomosis and abscess drainage. The patient's postoperative course was uneventful. This case helps to support the need for the consideration of GSD in patients aged 60 and older with a history of diverticulosis and presenting with abdominal discomfort and distension. Diverticulosis of the colon is a common clinical entity affecting 35% of individuals over the age of 65 in the western world , 2. DiveA 72-year-old female with hypertension presented with complaints of abdominal distention, fever, decreased appetite, fatigue, and weight loss for 2 months. The patient denied any abdominal pain, cramping, vomiting, constipation, or dysphagia. On physical exam, the patient's abdomen was distended, soft, and diffusely tender with presence of normal bowel sounds. Laboratory results revealed a low hemoglobin value (7.7 g%) with a normal mean corpuscular volume, white blood cell count of 38,200 cells/mL with 21% bands, The patient was started on normal liver function, amylase, and lipase. Patient was started on ciprofloxacin, flagyl, and intravenous fluids. Plain X-ray film of the thorax was normal. A plain abdominal X-ray showed a complete filling of the upper abdomen by a giant radiolucent mass with an air-fluid level . ComputeIn 1943, Bonvin and Bonte reported the first case of a solitary air cyst of the peritoneal cavity attached to the sigmoid colon, subsequently called a giant diverticulum of the colon . Hughes All patients with giant sigmoid diverticula present a common clinical profile. They are generally elderly, with an age range of 40 to 90 years and most occurring after the age of 60. The few reported cases of a true colonic diverticulum occurred at a slightly younger age suggesting a possible congenital component , 12. SexA giant colonic diverticulum (GCD) is defined as a colonic diverticulum measuring 4 cm or larger, with a few described spanning more than 25 cm , 7, 10. McNutt et al. divide giant diverticula into 3 types based on their pathology , 7, 12. Type 1 22%) (pseudodiverticulum) is a type of preexisting pulsion diverticulum that arises gradually, without perforation, and its wall consists of chronic granulation and fibrous tissue with chronic inflammatory cells and remnants of muscularis mucosa [2% (pseudType 2 (66%) (inflammatory diverticulum) is secondary to a local perforation of the mucosa and submucosa which leads to a walled-off abscess cavity that communicates with the bowel lumen and acts as a one-way valve that allows the diverticulum to enlarge. It is lined by fibrous scar tissue without any normal intestinal layer , 7, 12.Type III 12%) (true diverticulum) contains all layers of normal bowel wall and is in continuity with the gut lumen, likely representing a communicating bowel duplication cyst [%  or with more indolent features. The most consistent physical findings of giant colonic diverticula are abdominal tenderness 87%)  or an ab7%  or anComplications of GSD occur in 15–35% of cases such as perforation, intra-abdominal abscess formation, diverticulitis, volvulus, small bowel obstruction (secondary to adhesion to the large bowel), adhesion with the bladder, and lower gastrointestinal bleeding , 15, 19.The investigations of choice for diagnosing GCD include a plain abdominal X-ray and an abdominal CT scan; both can accurately demonstrate the classical “balloon sign” of GCD . Plain aBarium enema can confirm the diagnosis when there is a communication between the diverticulum and colon , 3, 7. CComputed tomography is useful when barium enema fails to make the diagnosis. However, given several reported cases of perforation precipitated by barium enemas, CT scans have been shown to be adequate for the diagnosis of suspected GSD . CT scanThe differential diagnosis for giant colonic diverticulum includes volvulus , bowel duplication, Meckel and duodenal diverticula, infected pancreatic pseudocyst, emphysematous cholecystitis, emphysematous cystitis, vesicoenteric fistula, and intra-abdominal abscess , 12, 15.Resection of the diverticulum and adjacent sigmoid colon is the preferred treatment in uncomplicated GCD –10, 15. Giant sigmoid diverticulum is a rare manifestation of colonic diverticulosis. We present a rare case of a patient who developed a type 1 giant sigmoid diverticulum (pseudodiverticulum), which further developed an accompanying type 2 diverticulum (inflammatory). An intra-abdominal abscess formed as the sequelae of the inflammatory diverticula. Perforation is a known complication of GCD, as evidenced in the patient described above. It is likely that more than one pseudodiverticulum was present, one found incidentally and the other forming an abscess leading to the patients physical findings of abdominal distention.This case helps to support the need for the consideration of GCD in patients aged 60 and older with a history of diverticulosis and presenting with abdominal pain and distension. This case is unique in that it is a rare presentation of a GSD that presented as a type 1 diverticulum with accompanying type 2 diverticula. A left hemicolectomy, rectal Hartman's pouch, and colostomy were performed, and the patient was subsequently discharged without complications.


Background. i-scan is a software-driven technology that allows modifications of sharpness, hue, and contrast to enhance mucosal imaging. It uses postimage acquisition software with real-time mapping technology embedded in the endoscopic processor. Aims. To review applications of i-scan technology in clinical endoscopic practice. Methods. This is a case series of 20 consecutive patients who underwent endoscopic procedures where i-scan image enhancement algorithms were applied. The main outcome measures were to compare mucosal lesions with high-definition white light endoscopy (HD-WLE) and i-scan image enhancement for the application of diagnostic sampling and therapy. Results. 13 cases involving the upper GI tract and 7 cases of the lower GI tract are included. For upper GI tract pathology i-scan assisted in diagnosis or therapy of Barrett's esophagus with dysplasia, esophageal adenocarcinoma, HSV esophagitis, gastric MALT lymphoma, gastric antral intestinal metaplasia with dysplasia, duodenal follicular lymphoma, and a flat duodenal adenoma. For lower GI tract pathology i-scan assisted in diagnosis or therapy of right-sided serrated adenomas, flat tubular adenoma, rectal adenocarcinoma, anal squamous cell cancer, solitary rectal ulcer, and radiation proctitis. Conclusions. i-scan imaging provides detailed topography of mucosal surfaces and delineates lesion edges, which can directly impact endoscopic management. As the field of gastrointestinal endoscopy continues to advance, various methods to enhance mucosal imaging continue to be developed and applied. Besides the progression from fiber optic endoscopes to standard resolution video endoscopes to high-resolution video endoscopes, several other techniques and technologies for enhancing mucosal imaging have been introduced and used. The most widely studied method is dye-based chromoendoscopy. Dye-based chromoendoscopy involves application of dyes or contrast stains directly to the mucosa and is often untidy and time consuming. Potential alternatives to chromoendoscopy involve mucosal enhancing technologies such as Olympus narrow band imaging (NBI), PENTAX i-scan, and FUJI Fice. PENTAX i-scan is a software-driven technology that allows for per pixel modifications of sharpness, hue, and contrast to modify and enhance mucosal imaging. i-scan uses postimage acquisition software with real time mapping technology embedded in the endoscopic processor (EPKi). The computer-controlled digital processing provides resolution of 1.25 megapixels per image, which allows for analysis and modification of the per pixel luminosity data. It does so by using various combinations of three software algorithms: surface enhancement (SE), contrast enhancement (CE), and tone enhancement (TE). Surface enhancement improves light/dark contrast, making dark areas appear darker and light areas appear lighter to better delineate edges and lesion borders. Contrast enhancement slightly suppresses red and green wavelength components of the white light image, while adding a minute blue hue to darker or more depressed areas of mucosa to allow for detailed observation of subtle mucosal irregularities. This helps define “peaks and valleys” within the mucosal surface, resulting in a more detailed topography of the mucosal surface. Tone enhancement analyzes all three components  of the white light image, and then dissects out and suppresses most of the dominant red, creating an image with an elevated blue/green contrast for detecting more subtle mucosal abnormalities . i-scan incorporates these three software algorithms into three distinct modes for the endoscopist: i-scan mode 1, i-scan mode 2, and i-scan mode 3. Each mode can be accessed or changed by a one button press on the endoscope. i-scan 1, designed as a surveillance mode uses SE and CE to provide more detailed topography of the mucosal surface and delineation of lesion edges without altering color. i-scan 2 and 3 also use SE and CE, but adds in TE to dissect out the dominant red and leave an elevated blue/green contrast (i-scan 2 darker contrast compared to i-scan 3). TE enhances vessel structures and minute mucosal structures to further pronounce margins of identified lesions. We report a series of 20 cases from a single academic tertiary care hospital where i-scan technology significantly impacted the management of patients in a wide variety of clinical scenarios. Consecutive cases were selected from our institution's endoscopy archive. Approval to review and report the selected cases was obtained from the University of Wisconsin Health Sciences Institutional Review Board. Informed consent was obtained from all participating patients. Data was collected on location of lesion (upper versus lower GI tract), diagnosis, i-scan mode, mucosal image detected, and impact on management. We compared and contrasted mucosal lesions detected with high-definition white light endoscopy (HD-WLE) versus i-scan image enhancement and the application on diagnostic sampling and therapy.i-scan image enhancement technology was used to diagnose and treat endoscopic findings and pathology in 13 cases involving the upper GI tract and 7 cases of the lower GI tract. Of the upper GI tract pathology, endoscopic image enhancement assisted in diagnosis, and/or therapy of 5 cases of Barrett's esophagus (BE) with dysplasia, 1 case each of T1 esophageal adenocarcinoma, HSV ulcerative esophagitis, gastric MALT lymphoma, distal gastric antral intestinal metaplasia with dysplasia, a flat duodenal adenoma with high grade dysplasia, and 3 cases of duodenal follicular lymphoma. Lower GI tract findings and pathology detected by i-scan imaging included 2 right-sided serrated polyps >1 cm, 1 case each of flat tubular adenoma, T1NO rectal adenocarcinoma, T1NO anal squamous cell cancer, solitary rectal ulcer, and radiation proctitis .The following clinical scenarios illustrate the diverse utility of i-scan in detecting several different types of upper GI tract mucosal abnormalities including neoplasms with dysplasia and those with malignant and infectious etiologies. A 67-year-old man with long-standing GERD was referred for evaluation of short segment Barrett's esophagus with high-grade dysplasia. An endoscopic ultrasound (EUS) was performed and confirmed there was no invading submucosal lesions. EGD with HD-WLE demonstrated 2 cm of Barrett's mucosa . With i-A 71-year-old man with previously diagnosed Barrett's esophagus with low-grade dysplasia (LGD) was referred for possible RFA of the Barrett's. EGD was performed and revealed a 5 cm segment of Barrett's esophagus. WLE revealed a possible small nodule within the segment of Barrett's. i-scan modes 1 and 2 were turned on which clearly defined a nodule within the Barrett's. This allowed for targeted resection via EMR. Pathology from the EMR revealed HGD. He returned for EGD 2 months later and no further nodules were present. The remainder of his Barrett's was successfully treated with endoscopic RFA.Three additional cases of Barrett's with esophageal nodularity and HGD were seen. The first was a 66-year-old female with known 4 cm, long segment Barrett's with HGD. Repeat EGD with i-scan modes 1–3 highlighted nodular areas in her distal esophagus which aided in targeted EMR of those areas. Next, a 78-year-old man with Barrett's with HGD underwent EGD with planned RFA. Upon initial evaluation with WLE a 4 cm tongue of Barrett's was noted. Visualization using i-scan modes 1 and 2 highlighted 2 focal nodules that were removed with targeted EMR prior to RFA. Pathology from the nodule resections showed intestinal metaplasia with very high-grade dysplasia and submucosal telangiectasia. Subsequent EGD's have shown reduction in the extent of his Barrett's. Lastly, a 54-year-old man with known Barrett's with high-grade dysplasia and normal EUS had a repeat EGD which showed irregular nodular mucosa in scattered islands. i-scan modes 2 and 3 were used to accentuate the abnormal areas to allow for optimal EMR. A 73-year-old man with a 10 year history of GERD was referred for EGD and EUS for long segment Barrett's esophagus with nodularity. On EGD the patient had 7 cm segment of Barrett's esophagus, with a single are of nodularity as seen on HD-WLE endoscopy image . i-scan An 80-year-old man with history of orthotopic heart transplant presented with progressive dysphagia and odynophagia. Initial EGD demonstrated with WLE alone and biopsy sampling suggested mild, superficial candida esophagitis. He was treated appropriately with a 14-day course of fluconazole. However, his dysphagia and odynophagia persisted. After 10 days of antifungal therapy as an inpatient, a followup EGD was requested. Areas of mucosal irregularity and mild ulceration were visualized with WLE. Further examination with i-scan modes 1–3 showed severe distal esophagitis, with deep ulcerations, which allowed for guided biopsies to be taken for viral culture and staining. Pathology revealed positive immunostaining for HSV I and II. He was started on treatment dose acyclovir and transitioned to valacyclovir prophylaxis with no recurrence of his esophagitis to date and marked improvement in symptoms.A 64-year-old man was referred for epigastric pain, nausea, bloating, and dyspepsia. He denied constitutional symptoms of weight loss, fevers, chills, or rigors. Physical examination findings and index laboratory testing were within normal limits. A two-week trial of PPI prescribed by his primary care physician did not relieve his symptoms. He was referred for an EGD. On EGD, in the body and fundus of the stomach, WLE demonstrated pale, atrophic-appearing mucosa that could be consistent with gastritis or gastropathy . i-scan  A 77-year-old female with history of previous gastric antral intestinal metaplasia with tubulovillous adenoma presented for followup EGD. Initially, WLE showed friable, nodular, and heterogeneous mucosa along the greater curvature of the stomach. i-scan modes 1–3 showed more pronounced involvement of the antrum in an irregular circumferential manner with mucosa that appeared somewhat fibrotic. Biopsies of the abnormal area showed chronic active gastritis with areas of intestinal metaplasia and dysplasia. The patient was then referred for surgical evaluation and is awaiting a subtotal gastrectomy.A 60-year-old woman was referred for EGD for symptoms of atypical, noncardiac chest pain. She also had been experiencing profound weakness without any other specific symptoms. On EGD, she was found to have a normal esophagus and stomach. Incidentally, she had an abnormal appearing polyp in the second portion of the duodenum. She was referred for possible ampullectomy. Upon repeat EGD, there was evidence of not only a large, irregular appearing periampullary polyp, but also multiple small satellite lesions. i-scan modes 1 and 2 were turned on and revealed more widespread distinct lesions. Directed biopsies of these lesions revealed grade 1 follicular lymphoma. The patient was referred to hematology. Staging CT of her chest, abdomen, and pelvis, as well as a bone marrow biopsy, revealed no other evidence of lymphoma. She has not required treatment and has been followed closely with observation and serial EGDs and CT scans. A 52-year-old man with history of alcohol abuse and past pulmonary embolism in the setting of positive lupus anticoagulant underwent EGD for evaluation of dyspepsia. EGD showed erosive esophagitis without Barrett's and a normal stomach. Inspection of the duodenum showed an area of mucosal irregularity with WLE and further visualization with i-scan modes 1–3 showed a sessile polyp that was removed and found to be an adenoma. Follow-up EGD showed a postpolypectomy scar and some areas of nodularity in the duodenum but biopsies obtained were normal. Two additional cases of follicular lymphoma highlighted by i-scan were discovered. First, a 72-year-old man with abdominal pain and a known pancreas head mass was evaluated with EGD and EUS for further diagnosis of the mass. EGD with WLE showed mild gastritis and a hiatal hernia with nodular mucosa in the second portion of the duodenum. i-scan modes 1 and 2 showed additional focal areas of nodularity that upon biopsy revealed grade 1-2 follicular lymphoma. EUS biopsy of the pancreas mass also showed lymphoma. The patient followed up locally for treatment of his lymphoma. Second, a 60-year-old female underwent interval surveillance EGD to monitor her known focal low-grade follicular lymphoma (staging CT and bone marrow biopsy were negative for involvement). Using i-scan modes 1 and 2 slight regression of the burden of lymphoma was seen. The patient has active hematology followup and surveillance with EGD and CT.The following collection of clinical scenarios represent i-scan's varied utility in detecting lower GI tract pathology. The cases range from detection of colonic polyps to that of anorectal malignancies and nonmalignant anorectal pathology.A 66-year-old asymptomatic, average risk woman was referred for a screening colonoscopy. In the ascending colon there was a large sessile polyp visualized under HD-WLE. i-scan mode 1 more clearly visualized the borders of the polyp. The polyp was lifted with injection of 3 mL of saline and was then removed in piecemeal fashion with hot snare polypectomy. i-scan allowed for improved visualization to ensure the polyp was completely removed. The area was then tattooed for future surveillance. Histopathology revealed a serrated polyp. Surveillance colonoscopy 6 months later revealed no residual polyp tissue.A 45-year-old man with no family history of colorectal cancer was referred for colonoscopy due to a change in bowel pattern. A minimally erythematous flat mucosal abnormality was noted in the ascending colon with WLE . FurtherA 64-year-old average risk man underwent routine colonoscopy for colorectal cancer screening. Near the ileocecal junction a fold appeared slightly prominent on WLE. Upon visualization with i-scan modes 1 and 2 a 2 cm sessile polyp was highlighted. Attempts to lift the polyp via saline injection for safe polypectomy were unsuccessful. The polyp was then biopsied and tattooed. An additional 6 mm sigmoid polyp was found and removed. Pathology showed tubular adenoma in both polyps. The patient underwent laparoscopic right hemicolectomy for the cecal adenoma and no higher grade lesions were discovered.A 69-year-old man was referred for routine surveillance colonoscopy. He had a colonoscopy 5 years prior with a single tubular adenoma. Surveillance colonoscopy was normal throughout, including a retroflexed view in the rectum. Upon withdrawal of the scope, there was an area of mucosal irregularity noted at the dentate line. i-scan mode 1 was activated and revealed a small erythematous, friable, ulcerated nodular lesion in the distal anorectum in the posterior wall. This area was targeted for biopsy and the histopathology revealed squamous cell cancer of the anal canal. The patient had a rectal ultrasound which revealed a T1N0 lesion. He was referred to oncology and started on a combined chemotherapy and radiation regimen.A 50-year-old asymptomatic, average risk woman underwent a screening virtual-CT colonography. An 8 mm polyp was seen in the sigmoid colon. She was referred for same day colonoscopy with polypectomy and the 8 mm polyp was removed. Upon retroflexion in the rectum, another 2 cm sessile polyp was seen . i-scan An 81-year-old man with a history of prostate cancer treated with radiation was referred for a colonoscopy for evaluation of hematochezia. His screening colonoscopy five years earlier was normal. A colonoscopy was performed and revealed no polyps, tumors, or evidence of inflammatory bowel disease or ischemic colitis. In the distal rectum, there was the appearance of radiation proctitis seen on initial WLE which suggested the degree of proctitis was mild, encompassing approximately 25% of the surface area. However, i-scan mode 1 was turned on and revealed extensive radiation proctitis involving approximately 60% of the lumen surface area in the anterolateral wall in a semicircumferential fashion. This allowed for better targeting of therapy with argon plasma coagulation (APC). His symptoms of hematochezia improved. Two months later, he returned for one more follow-up flexible sigmoidoscopy with additional APC therapy of the radiation proctitis, which had significantly improved since the first colonoscopy. After followup one year later, he has had no further hematochezia.Finally, a 64-year-old female with history of diarrhea and rectal bleeding was evaluated with colonoscopy. The colon was normal outside of some scattered diverticula and a couple of diminutive polyps. Further evaluation of her rectum with i-scan modes 1 and 2 showed a 6 mm solitary rectal ulcer. Biopsy of the ulcer edge showed benign squamous and columnar mucosa with focal neutrophilic infiltrates.There is an increasing demand for improving mucosal imaging as a means to identify pathology and target diagnostic sampling and guide endoscopic therapy. This has resulted in the development of technology-based methods for enhancing mucosal imaging. Digital filter technologies, such as i-scan, are becoming one of the more common and practical technologies to achieve this goal. We have presented a series of cases that illustrates the applicability of this technology across a broad spectrum of clinical scenarios.Digital filter-based technologies like i-scan were developed out of the concept of dye-based chromoendoscopy. Dye-based chromoendoscopy involves manually applying dye or contrast directly to the mucosa to better delineate normal from pathologic mucosa. It can be done in either an untargeted fashion across the entire mucosal surface, or in a targeted fashion at specific lesions. Dye-based chromoendoscopy leads to improved visualization of mucosal irregularities and has been studied in various situations ranging from screening colonoscopy to ulcerative colitis and Barrett's surveillance –3. HowevThere are several optical and digital filter techniques that have been developed to enhance mucosal imaging without manually applying contrast to the mucosa. Narrow band imaging (NBI) uses optical light filters in front of the light source to narrow the bandwidth of the projected light. This produces a colored image on the screen with enhanced contrast. NBI has not been shown to improve adenoma detection rates , but hasAs opposed to using filters at the light source as in NBI, newer proprietary technologies such as Fuji Intelligent Chromoendoscopy (FICE) (Fuji) and i-scan (Pentax) use postimage acquisition software to modify the images seen. FICE has been shown to be as good as dye chromoendoscopy in detecting colonic adenomas , 10. FICWe have illustrated a variety of cases where i-scan technology can be clinically useful and directly impact management of patients. i-scan was able to better detect and enhance mucosal abnormalities throughout the gastrointestinal tract compared to HD-WLE. Lesions that were highlighted ranged from Barrett's esophagus and gastric MALT lymphoma to duodenal follicular lymphoma and rectal adenocarcinoma. In general i-scan allowed for more precise delineation of lesion borders to allow for more complete mucosal resection and/or biopsy. In several of the cases there were significant changes in outcomes that may have been missed if WLE was used alone. Specifically, in the case involving the periampullary follicular lymphoma i-scan was able to further delineate the extent of a duodenal lymphoma, preventing unnecessary ampullectomy with its potential morbidities.This case series adds to the growing body of literature supporting the clinical utility of enhanced mucosal imaging. The main limitations of this series are that the experiences were limited to a single center and a few endoscopists experienced in the use of i-scan. The validity of i-scan to more readily detect mucosal abnormalities compared to current WLE will need to be addressed in more detail by prospective multicenter studies. Currently there are only single institution studies reported in the literature , 17, 18.i-scan technology shows promise as a useful modality in a wide array of clinical scenarios throughout the gastrointestinal tract to better detect and treat mucosal abnormalities. It can provide more detailed topography of the mucosal surface and delineate lesion edges by enhancing vessel and minute mucosal structures. Utilizing i-scan endoscopic image enhancement may directly impact the management of patients.


Primary (AL) amyloidosis of the gastrointestinal tract is relatively rare, and symptomatic amyloidosis of the stomach is even more seldom. We present the case of a patient who was referred to upper endoscopy because of weight loss, nausea, and vomiting. Large areas of intramucosal hemorrhages were seen, and biopsies resulted in profuse bleeding stopped with endoscopic clips. The biopsies showed amyloid depositions and further workup revealed that the patient also had cardiac and neuropathic involvements. The patient started treatment with dexamethasone, melphalan and bortezomib. After treatment was started the nausea and epigastric discomfort improved, and a reduction in the biochemical markers troponin T, NT-proBNP, and M-component was observed. Gastric amyloidosis is rarely seen at upper endoscopy in patients without a previously established diagnosis, but the unusual endoscopic findings and bleeding tendency after biopsy should be kept in mind by gastroenterologists. Gastrointestinal involvement in amyloidosis is seen in primary (AL) amyloidosis, secondary (AA) amyloidosis, and dialysis-related (Primary (AL) amyloidosis of the gastrointestinal tract is relatively rare, and only 8% of patients were reported to have amyloidosis in biopsies from the GI tract, whereas only 1% had symptomatic amyloidosis of the stomach in a series of 769 patients , 3, hemaA 74-year-old woman was referred to upper gastrointestinal endoscopy due to weight loss of 10 kg in 6 months, epigastric discomfort, nausea, and episodes of vomiting. She had a previous history of a tachy-brady syndrome resulting in pacemaker implantation two years before and received metoprolol treatment. Upper endoscopy showed large areas of intramucosal hemorrhage, mainly in the corpus and cardia of the stomach, whereas in the duodenal bulb, there was a polypoid lesion . A biopsλ 6.4 g/L. Bone marrow biopsy showed a slight increase in plasma cells positive for light chain λ as a sign of monoclonal plasma cell expansion  xpansion . These bSymptomatic gastric involvement is rare in patients with AL amyloidosis. This patient had large intramucosal hemorrhages and a polypoid lesion in the duodenum, where biopsies at upper endoscopy resulted in bleeding, but revealed amyloid deposits and led to the diagnosis of AL amyloidosis. Large hemorrhages requiring blood transfusion after biopsies from gastric amyloid lesions have been described by others  and may The diagnosis of gastric AL amyloidosis should be considered in patients with plasma-cell disease. Gastric amyloidosis is rarely seen at upper endoscopy in patients without a previously established diagnosis, and only few endoscopic findings have been published, but the differential diagnosis should be kept in mind by gastroenterologists.


If a pediatric abdominal mass is not organomegaly or colonic stool, narrowing the diagnostic possibilities may be difficult, especially in resource-poor areas where ancillary tests and treatment options may be limited. A 2-year-old girl was brought to the rural Kintampo Municipal Hospital in Ghana with a freely moveable, non-tender abdominal mass. A huge mesenteric dermoid cyst was surgically removed. Mesenteric cysts are rare intra-abdominal lesions, most commonly occurring in children <10 years old. Making a preoperative diagnosis is difficult. Dermoid cysts (mature cystic teratoma) rarely occur in the mesentery. Poverty, family circumstances and the rural location led to general physicians doing surgery. As in this case, due to economic, social and transportation issues common throughout Africa, children with abdominal masses may need at least initial surgery in hospitals without dedicated pediatric surgery or even a trained surgeon. Parents often bring their infants and children to clinicians after noticing an abdominal mass. Most of these are either organomegaly - a number that is probably much higher in malaria endemic areas - or colonic stool. Those that cannot be easily identified must be evaluated further, often with surgical exploration. Ninety percent of those requiring surgery are retroperitoneal, with about half originating in the urinary tract . AbdominNarrowing the diagnostic possibilities requires a careful medical history and physical examination. In resource-poor areas, all desired ancillary testing may be difficult or impossible to obtain. Treatment options may also be restricted due to poverty, geographic location, the parents’ personal responsibilities , and the professional staff's capabilities.We describe such a case in a rural district/municipal West African hospital with limited imaging capacity and a general physician who also acts as a surgeon. It illustrates the diagnostic/therapeutic process that led to an unusual diagnosis and a good patient outcome.A 2-year-old girl was brought to the Kintampo Municipal (District) Hospital in rural Ghana because the child's mother noticed a mass in the abdomen. The mother related that she had noticed it for some time, but wasn't sure for how long. She stated that the child had no changes in her eating or defecation pattern, no bleeding or bruising, fever, night sweats, behavioral changes or any other pertinent medical history.On physical exam, the 10 Kg child had a normal physical exam other than a 10 x 5 cm mass overlying the right colon . It appe9/L, and a normal differential. An ultrasound demonstrated a non-specific, probably cystic mass in the abdomen without any other identifiable pathology. An upright radiograph showed displaced bowel. No other laboratory tests or imaging modalities were available at this hospital.Her urinalysis was normal and a complete blood count demonstrated a Hgb 10.1 g/dL, WBC 7.2 x 10Clinicians suggested that the child be taken to another facility, but the mother's finances, family situation, and reluctance to go elsewhere precluded that option. When it became clear that the mother would not take the child to the closest hospital with better diagnostic and treatment facilities, the decision was made to operate. The general physician, who also does surgery, performed the procedure.Given the prevalence of parasitosis in the population and the child's rather benign presentation, the preoperative diagnosis was a probable parasitic cyst. The child was brought to the theater where, under general anesthesia, a midline laparotomy was performed. A large, smooth, glistening mass was immediately apparent occupying the right side and around 1/3 of the total abdomen. It was isolated from the iliac mesentery and removed intact . It weigThe pathology report showed a “huge cystic mass measuring about 10 cm in diameter and roughly spherical in shape containing serous fluid. Cut section reveals copious serous fluid.” The final diagnosis was a mesenteric dermoid cyst without evidence of malignancy.The differential diagnosis for an abdominal mass in a 2-year-old child  often caOn physical examination, the child's mass was large, freely mobile, apparently non-tender, and not associated with other organomegaly. That would seem to eliminate confusing enlarged organs of any etiology, displaced organs , or fixed masses . Its mobility also significantly lessens the probability that the mass was of retroperitoneal origin.The limited available imaging (and expertise at interpretation) suggested that the mass was a single cystic structure. Combined with the other available information, this led to the preoperative presumptive diagnosis of a hydatid  cyst not associated with the liver. Key elements in this presumed diagnosis were that parasitism is rampant in the local population and that patients with hydatid cysts can be asymptomatic, despite very large masses. Admittedly, hydatid cysts are usually parenchymal , so this finding would be unusual. However, the practical importance of considering this diagnosis prior to surgery was that we were intent on keeping the cyst intact, since intraperitoneal rupture might precipitate anaphylactic shock.As pathological evaluation subsequently showed, the cyst was actually a giant mesenteric dermoid cyst, perhaps an even rarer diagnosis.Mesenteric cysts are rare intra-abdominal lesions, occurring most frequently (∼75%) in white patients >10 years old; these lesions are rare in black patients . They caFlorentine anatomist Benevieni first described a mesenteric cyst in 1507, while performing an autopsy on an 8-year-old boy . In 1880Dermoid cysts (mature cystic teratoma) contain developmentally mature skin with its accompanying structures: hair follicles, sweat glands, hair, and often bits of other tissue. Since it contains mature tissue, they are almost always benign. This presentation of a dermoid cyst is very unusual. Dermoid cysts rarely present as mesenteric cysts .Mesenteric cysts have varied presentations, ranging from an asymptomatic mass to an acute abdomen . A lack Upright abdominal radiographs generally demonstrate a homogenous mass displacing bowel loops. Ultrasound examinations can generally diagnose a cystic mass, but may be problematic in further defining the pathology, especially if used by clinicians less experienced in ultrasound diagnosis . ComputeThe treatment of choice for mesenteric cysts is resection. In the resource-poor settings typical of much of the developing world, a child's best option may often be to have surgery, despite a paucity of diagnostic tools and limited surgical capabilities. In this case, surgical intervention was appropriate, given that the diagnosis was unlikely to be a malignant tumor and might have been a low-grade infection requiring removal . That the outcome was a diagnostic rarity was surprising.The literature suggests, however, that, on occasion, the surgery for mesenteric cysts may be more complex, sometimes requiring bowel resection, marsupialization of the cyst, or even a subtotal gastrectomy , 10. YetOne further note is in order concerning the lack of resources. While obtaining pathology reports on surgical specimens is routine in many areas of the world, it was a complex procedure in this setting. The mother had to agree to pay for the analysis. Then the sample had to be sent to a hospital several hours away. Retrieving the pathologist's report was also an ordeal, since they initially couldn't locate it. That, however, is all part of working in rural sub-Saharan Africa.Mesenteric dermoid cysts are uncommon. In rare cases, these cysts can become malignant. They can present asymptomatically or with signs and symptoms suggestive of other intra-abdominal pathology. Preoperative diagnosis can be difficult, but can be made with expert ultrasonography or contrast CT scanning. Neither ultrasound experts nor CT scanning is available in most of sub-Saharan Africa, and especially not in rural areas. Post-operative  diagnosis may be even more difficult, given the costs and logistics involved. Optimal treatment, both for definitive diagnosis and treatment, is intact removal, whenever possible. As in this case, due to economic, social and transportation issues that are common throughout Africa, children with abdominal masses may need at least initial surgery in hospitals without dedicated pediatric surgery or even a trained surgeon.


Wide margins of resection and regional lymphadenectomy for GIST are not necessary. Several procedures for rectal GIST have been designed according to the location and size of the tumor to preserve the anal function and decrease the morbidity rate.We report a 61-year-old-man with rectal bleeding. Proctologic examination revealed a small mass of approximately 2 cm in diameter on the anterior wall of the rectum at a distance of 4 cm from the anal verge. Histological examination of the biopsy sample via the rectum led to a diagnosis of GIST due to immunohistochemical positivity for C117 and CD34. Perineal resection was planned because abdominoperineal resection with sacrificing the sphincter function was excessive for this small tumor, and low anterior resection with the double stapling technique was difficult due to the lower position. A hemispheric incision was made from one mid-ischial tuberosity to the other with an apex of approximately 2 cm above the anus. The fascia band and muscles were successively transected in order to expose the anterior wall of the rectum, and excision of the tumor was performed. The postoperative course was uneventful, and the patient remained free from incontinence and recurrence.This perineal approach for a GIST on the anterior wall of the rectum is one option for preserving the anal function and decreasing the morbidity rate. Transanal or transcoccygeal approaches involve common routes for the local excision of rectal tumors. However, these methods are limited to cases of early rectal cancer close to the anal verge without the risk of lymph node metastasis or a non-epithelial tumor at the posterior wall of the rectum. It is difficult to plan the surgical strategy for a gastrointestinal stromal tumor (GIST) at the anterior wall of the lower rectum, as the procedures for the lower rectum are hampered by poor visualization and may cause anal dysfunction or discomfort. Transvaginal excision as described by Hellan is suitable for lesions higher on the anterior wall of the rectum and avoids anal dysfunction . RadicalThe patient presented to our hospital with rectal bleeding. He had neither constitutional symptoms nor a history of note. Proctologic examination revealed a small mass of approximately 2 cm in diameter on the anterior wall of the rectum at a distance of 4 cm from the anal verge. The mass was elastic-hard and non-mobile, with a smooth surface. Routine blood tests, serum chemical analysis, and tumor markers showed no abnormalities. Colonoscopy disclosed a bulge which probably originated in the submucosal layer Figure a. A T2 sUnder general anesthesia, the patient was placed in an exaggerated lithotomy position Figure . The oraThe postoperative course was uneventful. The patient began a meal on the seventh day. He was discharged on the fourteenth day after the operation. Neither local recurrence nor distant metastasis was noted during follow-up for 18 months.et al. reported the treatment of 144 cases of anorectal GIST [Surgery has always been the mainstay of GIST treatment, and the prognosis after surgery is influenced by the malignant potential of the tumor and completeness of the primary resection ,4. A comtal GIST . In thiset al. reported that transanal excision is appropriate for lesions located at an average distance of 3 cm from the dentate line [et al. demonstrated the excision of a rectal leiomyoma through the vagina [There are some reports in the literature describing transanal, transcoccygeal, and transvaginal approaches for the local excision of GISTs located in the lower rectum with the aim of decreasing the morbidity rate. Transanal excision is the most minimally invasive; however, there is a limit to the distance from the dentate line. Koscinski ate line . Furtherate line , but thiate line . Transvae vagina . Transane vagina . This pre vagina . AlthougRadical perineal prostatectomy was first described by Young in 1905 . This prWe conclude that this perineal approach for a GIST on the anterior wall of the rectum is one option for preserving the anal function and decreasing the morbidity rate, similar to transanal or transcoccygeal approaches.Written informed consent was obtained from the patient for publication of the report and any accompanying images.GIST: gastrointestinal stromal tumor; TEM: transanal endoscopic microsurgery.The authors declare that they have no competing interests.All the authors equally participated in preparation of the manuscript. All authors read and approved the final manuscript.


Background. Adult intussusception is a rare entity representing less than 1% of all intestinal obstructions. Diagnosis of the condition is difficult requiring a high index of suspicion and the utilization of imaging studies, especially CT scans. Diagnostic laparoscopy and/or exploratory laparotomy can be used as a diagnostic and therapeutic intervention. In over 90% of cases, an underlying lead point is identified. In the patient described here, it was a gastrointestinal stromal tumor (GIST), a relatively rare mesenchymal tumor comprising only 0.2–1.0% of the gastrointestinal tract neoplasms and believed to originate from neoplastic transformation of the interstitial cells of Cajal. GISTs may occur anywhere along the gastrointestinal tract, but most commonly arise in the stomach and small intestine. Literature review revealed only few cases reporting GISTs as a leading point of adult's intussusception. Case Presentation. In this report, we are presenting a rare case of jejunojejunal intussusception in a 78-year-old female patient with a GIST located in the terminal jejunum being the leading point, demonstrating the importance of imaging studies, especially CT scan, laparoscopy, and exploratory laparotomy as diagnostic and therapeutic interventions. Intestinal invagination or intussusception is the leading cause of intestinal obstruction in children, but it is an uncommon process in adults, accounting for only 5% of all intussusceptions and 1% of all intestinal obstruction. Unlike childhood intussusception which is idiopathic in 90% of cases, 70–90% of adult cases have a demonstrable lead point, with a well-definable neoplastic abnormality being the etiology in 65% of cases .Adult intussusception may present with acute, subacute, or chronic nonspecific symptoms. Therefore, the initial diagnosis often is missed or delayed till the patient is in the operating room. There is a surgical consensus that adult intussusception requires surgical resection because the majority of patients have intraluminal lesions. However, there is controversy about the need for reduction of the intussusception and the extent of resection to be performed .A seventy-eight-year-old female patient with a previous medical history of diabetes mellitus, hypertension, ischemic heart disease, and Hypothyroidism presented with a one-week history of abdominal pain and nausea with vomiting following meals. Her symptoms progressively worsened to severe abdominal distention, anorexia and obstipation on the day of admission. She had no fever, chills, bleeding per rectum, or previous abdominal surgeries. Four weeks earlier the patient had similar symptoms. A plain abdominal X-ray and CT scan which were done at that time showed dilated small bowel loop in right lower quadrant without detected lesions or significant wall thickening, and she had been treated conservatively. After resolution of her symptoms—during the previous episode—an upper and lower GI endoscopy were done and revealed moderate erosive esophagitis, Helicobacter pylori positive gastritis and duodenitis, and an essentially normal colonic exam.3/mm3. Abdominal X-ray revealed dilated proximal bowel loops with multiple air fluid levels. Conservative measures with bowel rest, nasogastric intubation, and intravenous fluids failed to control her symptoms. Abdominal and pelvic CT-scan showed markedly dilated fluid filled small bowel loops with one loop showing central hyperdense area, and a thick soft tissue mass around it with multiple small hypodense areas, referred to as the target sign [Her vital signs on admission were within normal limits. Examination showed a distended abdomen with hyperactive bowel sounds and generalized tenderness without peritoneal signs. Her laboratory investigations showed anemia with hemoglobin of 7.2 g/dL and white cell count of 9.9 × 10Diagnostic laparoscopy which revealed a distal jejuno-jejunal intussusception was followed by limited laparotomy and resection then anastomosis was performed . The leaThe patient had an uneventful postoperative recovery and was discharged in a well condition. She was doing well on subsequent clinic followup.Adult intussusception is an uncommon clinical entity encountered by surgeons. The exact mechanism is unknown, and it is believed that any lesion in the bowel wall or irritant within the lumen that alters normal peristaltic activity is able to initiate invagination. It is most commonly located at the junctions between freely moving segments and retroperitoneally or adhesionally fixed segments .About 90% of occurrences in adults have a well-defined pathological lead point, which may be a benign—such as benign neoplasms, inflammatory lesions, Meckel's diverticuli, appendix, and adhesions—or malignant lesion. In small intestine, malignant lesions (either primary or metastatic) account for 14–47% of cases, while malignant etiology is more prominent in large bowel representing up to 66% of the cases .Most adult patients with intussusception present with chronic and nonspecific symptoms suggestive of intestinal obstruction. Abdominal pain is the most common symptom followed by nausea, vomiting, and a palpable abdominal mass , 2.Preoperative imaging may help in identifying the causative lesion. Plain abdominal X-rays are typically the first diagnostic tool; with barium studies (showing “stacked coin” or “coiled spring” in upper GI series and “cup-shaped defect” in barium enema), ultrasonography , and colonoscopy are also useful tools for evaluating intussusception –7.target sign”) has been reported to be the most useful tool for diagnosis of intestinal intussusception and is regarded superior to the above mentioned studies [In recent years, with a diagnostic accuracy of 58–100% in recent series, abdominal CT-scan . They may occur throughout the GI tract from the esophagus to the anus, but most commonly are found in the stomach (40–60%) and jejunum/ileum (25–30%) . DuodenuSometimes GISTs are asymptomatic and are discovered incidentally during an endoscopy or on a CT done for another purpose. More often, they are associated with nonspecific symptoms  unless complicated with ulceration and overt GI bleeding (40% of the cases) or grow large enough to cause pain, mass or a lead point of intussusception and intestinal obstruction (20% of the cases) .Contrast-enhanced CT is a preferred initial imaging study for screening and staging. However, other procedures such as ultrasound, endoscopy, intestinal capsule, and PET scan may also be used.imatinib may be preferred if a tumor is borderline resectable, or if resection would necessitate extensive organ disruption.Surgical “en bloc” segmental resection with the goal of achieving negative resection margins is the treatment of choice for potentially resectable tumors, while initial therapy with The prognosis of small intestine GISTs depends upon the adequacy of resection, tumor size, mitotic activity, and location within the small bowel, with small intestine having a worse prognosis than stomach –15.Intussusception in adults occurs relatively rarely; however, in 90% of cases a specific lead point is identified. The diagnosis may be challenging because of nonspecific symptoms, and sometimes it is impossible to reach the diagnosis of intussusception as a cause of obstruction until laparotomy is done. CT-scan is the most useful imaging modality in diagnosis. An underlying malignant lesion might be the lead point especially in large bowel. Therefore, surgeons should think of intussusception as a cause of intestinal obstruction, and they should be familiar with the various treatment options. The decision whether to undertake resection or reduction followed by resection is case specific, and it should be tailored according to the situation.


Fourth branchial pouch anomalies are rare congenital disorders of the neck and are a consequence of abnormal development of the branchial apparatus during embryogenesis. Failure to appropriately recognize these anomalies may result in misdiagnosis, insufficient treatment, and continued recurrence. Here, we present an unique presentation of two cases, describe their diagnosis, clinical course, and management, and review the literature regarding these interesting anomalies. The human branchial apparatus, which develops in early gestation, is comprised of six paired mesodermal arches, separated by endodermal and ectodermal invaginations known as pouches and clefts, respectively . Each arCongenital lateral cervical cysts, fistulae, and sinuses are thought to arise from the branchial apparatus. Approximately 95% of congenital anomalies of the branchial apparatus involve the second branchial arch, pouch, or cleft, while the remaining mostly arise from the first and third arches  and accoThe origin and course of the anomalies are determined by the mesodermal derivatives of adjacent arches; fistulae of the fourth branchial arch have an external opening in the neck on the anterior border of the lower sternocleidomastoid muscle and an internal opening in the pyriform sinus . We present two rare cases of fourth branchial pouch abnormalities, the second of which represents a true, complete fistula, an anomaly rarely reported in the literature. The purpose of this manuscript is to present our experience with these cases, both of which demonstrate long-term followup. We have also emphasized the diagnosis, clinical course, and management of these interesting anomalies.A 9-year-old white male presented with recurrent neck swellings, since the age of 2. During that time, he had a history of 27 procedures on his neck, ranging from explorations, drainages, and attempted excisions, with recurrence of signs and symptoms after every intervention. Upon presentation to our clinic, he complained of dysphagia and painful swelling of his neck.Physical examination of his neck revealed swelling of the anterior left neck that was tender to palpation and was approximately 3 cm in diameter. Several computerized tomography (CT) scans had been performed in the previous years, all of which only revealed a diffuse anterior neck cystic structure, which was superficially located left of the midline.In the operating room, direct laryngoscopy revealed a sinus tract originating from the left side of the pyriform sinus apex ; a cholaA 42-year-old male presented with recurrent neck infections, left-sided neck pain, and a draining cutaneous pit to our clinic. The patient described a history of multiple procedures on his neck starting 1 year ago for what was thought to be a recurrent deep neck infection. He also recently became alarmed when blue fluid began emerging from his neck after drinking a blue-colored liquid, worrisome for a sinus tract. He was a previously healthy nonsmoker.Flexible laryngoscopy revealed a small mucosal depression in the lateral posterior left pyriform sinus apex. He also had a draining neck wound on the anterior portion of his left sternocleidomastoid muscle . A bariuIn the operating room, a size-three foley catheter stylet was used to cannulate the tract. A curvilinear incision was made around the neck wound including the external fistula, the strap muscles were sectioned, and an oblique incision through the thyroid cartilage exposed the apex of the pyriform sinus, allowing the cannulated tract to become palpable. The fistulous tract was found to exit the pharynx inferior to the superior laryngeal nerve during exploration, consistent with an anomaly of the fourth branchial pouch . HistoloFourth branchial pouch anomalies are rare and usually present as lateral neck masses, abscesses, or acute suppurative thyroiditis . These aA fistulous tract of a third branchial apparatus abnormality has a similar course to a fourth arch anomaly, but exits the pharynx superior to the superior laryngeal nerve , 10. ParThe common presenting symptoms of a fourth branchial pouch anomaly include recurring deep neck infections or abscesses, as well as soft fluctuant masses. Third and fourth branchial arch anomalies may also lead to acute suppurative thyroiditis, and for this reason, some authors recommend that in all cases of thyroiditis, the presence of a branchial arch anomaly should be investigated . DiagnosOur cases present an important issue in the treatment of fourth branchial pouch anomalies-prompt recognition. Both patients had undergone extensive medical treatment and surgical excisions to remove what were thought to be simple branchial cysts or thyroglossal duct cysts. The mainstay of treatment for fourth branchial anomalies is complete surgical excision because of the risk of infection and life-threatening abscesses , 8. The Fourth branchial arch anomalies are rare and fascinating aberrations of fetal development that may present in many different ways. Combining a proper preoperative evaluation with careful surgical planning may result in the proficient eradication of these lesions, offering the patient relief from this source of recurrent infection. Definitive management is achieved by complete surgical excision of the anomaly.The authors have no conflict of interest to disclose.


Partially or completely invaginated appendix mistaken for a polyp during colonoscopy and leading to intussusception is a rare situation. This paper describes our experience with two cases of appendiceal intussusception. In the first case, there was no underlying ileocecal abnormality, and, in the second case, histologic examination of the resected appendix and cecum revealed widespread foci of angiodysplasia, and this was thought to be the basis for the intussusception. The authors present reviews of the literature concerning clinical features and associated conditions and emphasize that failure to recognize this condition may result in unexpected complications such as consequent peritonitis in case of endoscopic removal. Intussusception of the appendix is an extremely rare condition. It affects all ages but is usually associated with males in the first decade . DespiteA 48-year-old man with several years history of abdominal pain and transit disturbances was admitted to our hospital for further investigations. Physical examination and laboratory data were within normal limits. Colonoscopy revealed a sessile, dimpled 20 mm cecal polyp  covered  (hemoglobin 8 g dL-1). Gastroscopy was normal but colonoscopy showed an actively bleeding, sessile, cecal polyp located in the usual site of the appendiceal orifice (A 65-year-old man was admitted to our hospital with a six-month history of melena with transfusion requirement. Hemogram showed hypochromic microcytic anemia orifice . There wAppendiceal inversion was first described in 1858 . It is a


Medulloepithelioma is a rare congenital tumor of the primitive medullary neuroepithelium. A significant proportion of patients with medulloepithelioma arising from the optic nerve die from intracranial spread or cerebral metastasis. Because it has no known distinct clinical features and because of its low frequency, this tumor presents within the first two to six years of life and is usually misdiagnosed clinically as a different type of optic nerve tumor. Here, we describe a new and atypical case of medulloepithelioma of the optic nerve in a 12-year-old boy. To the best of our knowledge, he is the oldest reported patient to present with this disease and, now as an adult, has the longest documented period of disease-free survival.A 12-year-old Caucasian boy with headache and unilateral amaurosis was referred for a presumed optic nerve glioma to our hospital. A computed tomography scan showed optic nerve enlargement, and fundoscopy showed a whitish mass at the optic disc. Our patient had been followed at his local hospital for four years for an 'optic disc cyst' with no change or progression. He experienced mild progressive visual impairment during that period. He was admitted for resection, and a histopathological analysis revealed a medulloepithelioma of the optic nerve. Supplemental orbital radiotherapy was performed. He remained disease-free for 25 years.Medulloepithelioma of the optic nerve can clinically mimic more common pediatric tumors, such as optic glioma, meningioma, or retinoblastoma. Thus, medulloepithelioma should be included in the differential diagnoses of pediatric optic nerve lesions. Fundoscopy in these patients may provide relevant information for diagnosis. Anterior optic nerve medulloepitheliomas may behave differently from and have a better prognosis than medulloepitheliomas that have a more posterior location. Our case report illustrates that long-term survival can be achieved in patients with this malignant tumor. Medulloepithelioma is a rare congenital tumor derived from the primitive medullary epithelium lining the neural tube. This is the most frequent congenital tumor of the ciliary body but is extremely infrequent in the optic nerve (ON) . To the A 12-year-old Caucasian boy was referred with a presumed diagnosis of right ON glioma to our hospital. Four years earlier, his local ophthalmologist had diagnosed a 'yellow-whitish, well-demarcated papillary cyst with central retinal vessel displacement' in his right eye  and documented a visual acuity of 20/30 in both eyes. He was examined twice a year, and his vision remained stable. Four years later, his ophthalmologist documented 'visual acuity of 20/80 and concentric visual field reduction to the central 10° isopter in OD'. Fundoscopic variation of the lesion was not noted.One month later, after a severe headache, visual loss progressed and no light perception with an absolute afferent pupillary defect was present in the OD. A computed tomography (CT) scan showed right ON thickening, and the boy was referred and admitted to our center.At admission, the described neuro-ophthalmologic findings were confirmed, and on CT, the optic disc appeared elevated and enhanced after intravenous contrast was administered. Fundoscopy revealed a well-defined whitish mass without macroscopic calcification at the ON head, dilated and tortuous retinal vessels, and small superficial retinal hemorrhages Figure . A complAfter informed consent was obtained from our patient's parents, a right transfrontal exploratory orbitotomy with canalicular unroofing was performed, and a normal chiasm, prechiasmatic and canalicular ON, and grayish infiltration in the proximal intraorbital portion were revealed. A frozen intraoperative biopsy of the tumor was diagnosed as an 'unrecognized tumoral process', and 0.8 cm of distal ON was resected. Because the eye had no light perception and the tumor was potentially malignant, the remaining ON and globe were enucleated with excision of the retrobulbar orbital fat.A gross examination of the eye revealed an ON with a width of 6 to 7 mm and a length of 6 mm. A sagittal section through the globe revealed a small, whitish, rounded tumor of 2 to 3 mm with a soft surface at the optic disc. No lesions of the cornea, iris, lens, or ciliary processes were detected. The tumor had areas composed of poorly differentiated neuroblastic cells with nuclear pleomorphism but no mesenchymal elements. Thus, the final histopathological diagnosis was malignant non-teratoid medulloepithelioma of the ON . There w60) was applied in two fields. Our patient was closely followed with annual orbital CT and half-yearly echography for six years without evidence of recurrence. At present, he is 37 years old and has a disease-free survival of 25 years.Systemic work-up was negative for any evidence of metastatic disease. In view of the significant mortality rate of this tumor, orbital exenteration was suggested, but our patient's parents declined -5. FiftyMedulloepithelioma of the ON remains a challenge because of its low frequency. The clinical presentation is similar to that of other neoformations of the ON during childhood. When its location involves the orbital portion of the ON, proptosis and optic disc swelling may occur; lesions that are more posterior can produce a progressive retrobulbar optic neuropathy . InteresDifferential diagnoses of growing ON masses with reduced visual function and an intraocular component should include glioma, retinoblastoma, medulloepithelioma, and meningioma. Glioma and retinoblastoma with ON infiltration are the most frequently presumed diagnoses. Other processes, such as intraocular lesions extending onto the ON , metastasis , or inflammatory disorders (mainly granulomatosis), are rare but should also be properly ruled out.There are no distinct neuroimaging features of medulloepitheliomas of the ON. Calcification can be detected, as in retinoblastoma . In addiIn patients with medulloepitheliomas arising from the posterior ON, mortality is usually caused by untreatable intracranial invasion or cerebral metastasis ,4,9,10. Our patient was remitted with presumed glioma of the ON. However, the white mass in the optic disc was not typical for a glioma, and the four-year history of a growing papillary mass was not typical for a malignant process. This could be caused by low aggressiveness of the tumor or late malignant transformation . DespiteThe most effective management of medulloepitheliomas of the ON remains unclear. It seems that total excision is curative but, because of extension to adjacent structures, is not always possible. Adjuvant chemotherapy, radiotherapy, or extensive resection can be effective in cases of tumor infiltration at the resection margin , but theON alterations with continued, even mild, visual loss in children should be considered for neuroimaging. Medulloepithelioma of the ON should be included in the differential diagnoses of ON masses. In addition to ON enlargement, an intraocular component (papillary mass) may be present. Surgery is the first and most important therapeutic step, but the need for associated adjuvant treatments, even without residual disease, and the most effective treatment still cannot be specified. The long-term prognosis of these rare tumors is still unknown. However, our case report reveals, for the first time, that successful treatment can achieve a long-term survival.CT: computed tomography; OD: oculus dexter (right eye); ON: optic nerve.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.NP-S, JP-C, and MA-D performed examinations, interpreted data regarding clinical and neuroimaging findings of the patient, and contributed to the conception and design of the manuscript. JA-G and FC-B operated on the patient and made major contributions to the writing of the manuscript. EG-F and CM-B performed the histological examination of the lesion and wrote the text regarding the histopathological findings. All authors read and approved the final manuscript.


Rupture of the urinary collecting system associated with perinephric or retroperitoneal extravasation of the urine is an unusual condition and it is commonly associated with renal obstructing disease. Perforation could occur at any level from the calix to the bladder but it is usually seen at the fornices and upper ureter. It may lead to several serious consequences including urinoma, abscess formation, urosepsis, infection, and subsequent irreversible renal impairment. We report a case of a 69-year-old woman who presented at the emergency department of our institution with severe abdominal pain. Due to symptomatology worsening, complete laboratory evaluation was performed and the patient underwent abdominal contrast enhanced computed tomography (CT) evaluation which showed contrast agent extravasation outside the excretory system without any evidence of renal calculi at basal acquisition. It was decided to perform a double-J stent placement which was followed by complete healing of the ureter and its removal was performed 8 weeks later. Diagnosis and therapeutic approaches are discussed.  Obstruction of the genitourinary system is followed by an increase of intraluminal pressure which may lead to rupture of the collecting system and consequently urine extravasation with urinoma formation. Spontaneous rupture of the ureter is rare and can be traumatic or nontraumatic. Calculi represent the most frequent cause of ureteral and pelvis rupture in the nontraumatic group. Urine extravasation may be clinically occult or may lead to symptoms of acute abdomen. We report a rare case of spontaneous ureteric rupture in a patient with a clinical history of recurrent renal colics in the younger age treated with double-J stent placement. We further describe this minimally invasive interventional approach.3), with 48,2% of neutrophils. Urine analysis showed the presence of leukocytes and erythrocytes. Other values were within the normal limits.A 69-year-old woman was admitted to the emergency department of our institution with a severe right-sided flank pain that started 6 hours before, nausea, and history of renal calculi in the younger age. In suspicion of an episode of renal colic, analgesic drugs therapy and complete laboratory evaluation were performed. Her vital signs were as follows: heart ratio: 89 beats per minute and regular; blood pressure: 145/90 mmHg; respiration: 18 per minute; and body temperature: 36,8°C. She had leukocytosis in the blood  was intended to get a complete overview of the entire excretory system and showed in the delayed phase (10 minutes after contrast medium injection) a late filling of the right lower ureter and a perinephric fluid collection. Contrast medium extravasation extended from the vertebral body of L2 to L5 and crossed the midline at the level of the aortic bifurcation .No evidence of factors that caused ureteric rupture was identified at CT evaluation.Two hours later the lower calyceal group of the right kidney was punctured with Chiba needle  under ultrasonographic guidance. Contrast media injection under fluoroscopy confirmed the presence of an extravasation localized in proximity of the pyeloureteral junction . After tUrine extravasation is the result of a leakage of the urinary collecting system at any level from the calix to the urethra. This is defined as “spontaneous” if it is not induced by external trauma, iatrogenic manipulation, degenerative kidney diseases, or previous surgery.First cases were described in 1856 by Diaz and Buenrostro –4.Lymphoma and chemotherapy have been also described as a rare case of renal pelvis rupture. Usually collecting system rupture is monolateral, but Niggeman et al.  describePathogenetic pathway underlying urinary collecting system nontraumatic disruption can be identified in an increase of intraluminal pressure. The fornix is the most common site of rupture followed by the upper ureter when pressure exceeds a critical level reported from 20 to 75 mmHg. Urine extravasation may lead to urinoma formation , 7. ThisSymptomatology usually could not be differentiated from a renal colic but sometimes could mime an acute abdomen. In some cases it could be very difficult due to lack of symptoms.Differential diagnosis includes diverticulitis, cholecystitis, appendicitis, and others.Ultrasonography represents the first line of investigation for renal colic . It can In our case, ultrasonography was not performed because we preferred to perform immediately abdominal contrast enhanced CT due to symptomatology worsening and to gain a panoramic view of the entire excretory system. Computed tomography is the technique of choice in the diagnosis of urinary collecting system leaks and urinomas. The latter could appear as confined, encapsulated fluid collections or as free fluid. Delayed acquisitions (5–20 minutes after contrast medium administration) are mandatory to identify the attenuation increase of the urinoma which can range from 0 to 20 HU before intravenous contrast administration and then enhance up to 200 HU after contrast administration , 10. RefTreatment options included surgery or interventional radiology –14 and sIn this case, a minimally invasive procedure can consist in percutaneous urinoma drainage using a nephrostomy catheter, with a double-J stent placement. Fluoroscopy permits to achieve an unobstructed urinary outflow, the healing of the perforation, and the regression of the urinoma in the majority of cases. Its placement may be performed either retrograde through the bladder or antegrade, as in our case, through a percutaneous nephrostomy . To faciA diversionary nephrostomy catheter could be left in place to gain an access to the collecting system so that contrast medium could be injected to confirm the ureteral injury has completely healed.As the incidence of late complications after surgery such as ureteric stricture, ureteropelvic stenosis, or periureteric fibrosis remains unknown, percutaneous interventional minimally invasive techniques are the first choice of treatment option nowadays. Spontaneous rupture of the ureter should always be considered in the differential diagnosis of a patient presenting with complex symptoms after renal colic.


Extraneural and extracranial metastases of glioblastoma (GB) are very rarely reported in the literature. They occur in only 0.2% of all GB patients.We present a 40 year old caucasian male with secondary GB and first diagnosis of an astrocytoma world health organisation (WHO) grade II through stereotactic biopsy in 2006. He presented a new hemiparesis and a progress of the known mass lesion in 2008. Subtotal tumor resection was performed and the histological examination verified a GB. After combined radio- and chemotherapy the adjuvant temozolomide therapy was not started because of non-compliance.In 2011 a second local relapse was resected and 4 month later the patient presented a fast progressing tetraparesis. Cervical CT and MRI scan showed a mass lesion infiltrating the fifth and sixth vertebra with infiltration of the spinal canal and large paravertebral tumor masses. Emergency surgery was performed. By additional screening further metastases were detected in the thoracal and lumbal spine and surprisingly also in the lung and pulmonary lymphnodes. Palliative radio- and chemotherapy of the pulmonal lesions was completed, further antitumor therapy was rejected. The patient died 10 months after diagnosis of the extraneural metastases.Especially young “long-term-survivors” seem to have a higher risk of extraneural metastasis from a GB and appropriate staging should be performed in these cases. Glioblastoma (GB) is known as a highly aggressive neuroepithelial tumor almost exclusively growing in the neural tissue. Extraneural metastases were rarely reported in the literature. The incidence of extraneural metastases is 0.2% of all GB patients Hsu E, 1998) [998 [1]. We present a case of a 40 year old patient with first diagnosis of an Astrocytoma World Health Organization (WHO) grade II in 2006, a secondary GB in 2008 and multiple extraneural metastases in 2011. Although several cases of extraneural metastases of GB have been reported, to our best knowledge this is the first report of a case with so many metastases in different tissues  on admission was 90 and his antiepileptic drug therapy was based on oxcarbazepine and clobazam. Magnetic resonance imaging (MRI) showed a mass lesion (3 centimeter (cm) × 2 cm) in the right central region  was performed, but because of non-compliance the planned adjuvant temozolomide chemotherapy was not started.A debulking with subtotal tumor resection was performed and the histological examination verified a secondary glioblastoma multiforme (GB). After the surgery a combined radiation therapy (RT) (5 × 2 Gray (Gy) per week, ad total dose of 60 Gy) and chemotherapy , was performed once, a repetition was again rejected by the patient.A combined RT and chemotherapy was subsequently performed in September 2011 with irradiation of the metastatic mediastinal, infracarinal, pretracheal, retrocaval and hilar lesions in a fractionation of 15 × 3 Gy up to a total target volume dose of 45 Gy in a palliative intention. RT was well tolerated without significant acute radiation-induced toxicities. Additional chemotherapy with temozolomide  reported patients with extraneural metastases underwent cranial GB surgery beforehand  , therefo6% reportAnother special feature in our patient was the fact that the last tumor resection surgery was at the same time a revision for a chronic wound healing disturbance.By enhancing global immune responses patients with wound infections after GB resection are discussed to have a longer overall survival compared to patients without wound infections, although there is still no clear proof for this theory  . The comThe fact that the overall survival in patients with GB increased in the last decade  brings uWe can reaffirm with our case that especially young glioblastoma patients have the potential to develop extraneural metastasis. We conclude that especially in the case of the so called “long-term survivors” with repeated surgeries the possibility of extraneural metastasis should be anticipated and an appropriate staging should be performed early when suspected.Based on the experience with our special case and according to the current literature, we recommend appropriate routine staging consisting of holospinal MRI and CT of thorax and abdomen beside the routine cranial MRI for “long-term survivors” with GB in case of clinical suspicion of extraneural metastasis.Written informed consent was obtained from the next of kin of the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the editor of this journal.MRI: Magnetic resonance imaging; CT: Computed tomogram; RT: Radiotherapy; Gy: Gray; KPS: Karnofsky performance index; GB: Glioblastoma; WHO: World health organization; cm: Centimeter; CSF: Cerebrospinal fluid; GFAP: Glial fibrillary acidic protein; IDH: Isocitrate dehydrogenase; MAP: Microtubule-associated protein.The authors declare that they have no competing interests.FvL contributed and listed up the different histological figures. SG contributed and listed up the radiological figures. MvL performed surgery and contributed the intraoperative pictures. JB accompanied the post surgical therapy of the patient in terms of RT and chemotherapy and as senior author participated significantly in the drafting. CB conceived and drafted the case report as main author. All authors read and approved the final manuscript.


Primary leiomyoma of the pleura is extremely rare. A 45-year-old man presented with a complaint of right chest pain. Chest computed tomography demonstrated a solid, round pleural mass in the right anterior chest wall. The mass was completely resected, and histopathological examination revealed a localized primary pleural leiomyoma. The patient was followed and has been disease-free for over 15 months. This is the first report of primary leiomyoma of the pleura in China. A review of the literature on primary leiomyoma of the pleura is presented. Leiomyomas are benign smooth muscle tumors that are commonly encountered in the urogenital tract, occasionally in the gastrointestinal tract, and rarely in the respiratory tract . HoweverA 45-year-old man without any significant past history was admitted to our department complaining of right chest pain persisting for 3 days. A mass was seen in the right middle lung field on chest roentgenogram Figure . HoweverThe patient underwent a right posterolateral thoracotomy. A giant, well-capsulated smooth-surfaced tumor was observed in the anterior chest wall. It was easily resected en-bloc with surrounding tissue. Grossly, the tumor measured 9 cm × 6 cm × 5 cm and was covered with pleura. The cut tumor was solid white with cystic cavities. Pathological examination revealed a proliferation of interlaced fascicles of spindle cells showing moderate atypia and less than 1 mitotic figure per 50 high power fields Figure . ImmunohThe patient had an uneventful recovery, and there has been no sign of recurrence 15 months after surgery. Since the malignant potential of the tumor could not be determined, close follow-up of the patient was advised.Primary intrathoracic soft tissue tumors are unusual. They can originate from the upper or lower respiratory tract or from the mediastinum. The differential diagnosis for pleural spindle cell neoplasms includes solitary fibrous tumor, smooth muscle tumor, spindle cell carcinoma, thymoma, sarcomatoid variant of mesothelioma, and leiomyoid variant of mesothelioma -5. LeiomAlthough this leiomyoma of the pleura appeared benign, with a smooth, well-capsulated surface and unremarkable histologic findings, it has a low but definite malignant potential. Pleural leiomyomas may increase in size with local invasion to the mediastinum and may not be possible to resect completely . They maMost tumors can be completely resected if the contrast-enhanced chest CT scan shows a well-capsulated pleural mass with minimal invasion of the adjacent organs, and the patient does not have signs of invasion, such as very severe pain. In general, most primary pleural leiomyomas can be easily and completely resected. If the tumor is small and localized, complete resection can be achieved with minimally invasive surgery such as video-assisted thoracic surgery (VATS) ,9,14. InIn summary, because primary pleural leiomyoma is an extremely rare tumor with low malignant potential, complete resection and close follow-up is advised. Transthoracic fine-needle biopsy is contraindicated if it is possible that the tumor can be completely resected.Written informed consent was obtained from the patient for publication of this Case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.XQ, DZ, JC and QZ did the surgery, SW and GC collected the data. XQ, JC and QZ wrote the article. All authors read and approved the final manuscript.


Gastric outlet obstruction caused by duodenal impaction of a large gallstone migrated through a cholecystoduodenal fistula has been referred to as Bouveret's syndrome. We present a case of gallstone-induced duodenal obstruction in an elderly female patient, diagnosed on a 64-slice MDCT scanner. One-stage surgery, that is, stone removal and cholecystectomy, was performed resulting in relief of obstruction and complete cure. Clinical features, multidetector computed tomography (MDCT) findings, and surgical management are discussed. The first case of obstruction of duodenum by a gallstone was reported by Beaussier in 1770, but the syndrome came to be recognized only after Leon Bouveret published his two cases in 1896 . When th Since this syndrome is usually observed in older patients with poor medical status, a nonsurgical approach such as endoscopic stone removal has been used as the first-line treatment. The main purpose of surgical intervention should be to remove the obstructing stone by enterotomyotomy without cholecystectomy and cholecystoduodenal fistula breakdown to minimize the risks of surgery . However9/L. Liver function tests were normal.A 60-year-old female presented with a two-week history of occasional vague abdominal pain that, over a period of four days, had become more severe and localized to the right upper quadrant of the abdomen. This was associated with vomiting, which relieved pain. There was history of dyspepsia for the past 4 years. On examination, the patient was mildly dehydrated without any icterus. The abdomen was distended and tender without evidence of hernia. Bowel sounds were present. Ryle's tube aspirate was bilious and around 1 litre/24 hours. Plain radiographs of abdomen and chest were normal. Potassium and sodium levels were decreased . Amylase was slightly raised to the level of 125.5 IU/L. Urea and creatinine levels were 84 and 1.7 milligram/dL. Her haemoglobin level was 11.2 g/dL. The white cell counted 10 × 10Ultrasound examination  revealed dilated stomach and duodenum till its 3rd part. No calculus was detected in gallbladder or duodenum. Liver and kidneys were normal. There was no ascites. Contrast enhanced computed tomography (CT) scan was performed on a multidetector CT scanner . The scan parameters were as follows: 5 mm collimation, 2.5 mm interslice interval, 0.6 seconds rotation time, 15 mm table speed per rotation at 120 KV and automatic mA. Images were acquired in portal venous phase after injecting 90 mL of nonionic iodinated contrast material intravenously at 2.5 mL/s with a pressure injector followed by 20 mL of normal saline flush at the same rate. The multidetector computed tomography (MDCT) scan revealed a cholecysto-duodenal fistula with second part of duodenum. There was a large gallstone measuring 3.2 cm in the proximal 3rd part of the duodenum. Pneumobilia was also seen. Stomach and duodenum were dilated (Figures On laparotomy, multiple adhesions were found between stomach, gallbladder, duodenum, and omentum with inferior surface of liver. Fundus of gallbladder was adherent to 2nd part of duodenum, suggestive of a fistulous tract. The fistulous tract was excised, and the 2nd part of duodenum was opened simultaneously . DuodenoBouveret's syndrome is gastric outlet obstruction caused by a large gallstone passing into the duodenal bulb through a biliogastric or bilioduodenal fistula . Bouvere Endoscopic lithotomy is the first-line approach to treatment; however, it may be unsuccessful in some cases particularly with impacted large stones. Smaller stones generally pass through the duodenum and do not cause gastric outlet obstruction . However Enterotomy carries less morbidity rate when compared to duodenotomy particularly in patients with duodenal ulcer due to the erosion of duodenal wall by an impacted large stone. Intraoperative endoscopy may facilitate recognition and removal of remaining stones in the proximal gastrointestinal lumen. Intraoperative ultrasound is advised in cases where the surgeon is unable to localize exactly the site of the remaining stone fragments . In our


Lesions located in the area of the tibial spines are rare. In most cases, treatment follows histological diagnosis, but when imaging and clinical data are considered to be "very" characteristic for benign lesions, such as chondroblastoma or osteoid osteoma, treatment may be performed without biopsy. Traditional curettage requires opening the joint, which presents a high risk of contamination of the joint itself and surrounding structures, such as the popliteal area, with possible contamination of the neurovascular bundle when performing curettage with the posterior approach. In this case, the re-excision of a local recurrence would be extremely difficult.We describe a technique using arthroscopic guidance for radiofrequency thermoablation of a benign lesion in the tibial spines area. We report on an illustrative case. The patient so treated, reported immediate relief from the pain, and after two weeks, was free of pain. The biopsy performed before the treatment confirmed the radiological diagnosis of chondroblastoma. At one year of follow-up, the patient is without pain, with a 0-130°range of motion, has no activity limitations and is apparently free of disease.This technique allows a radiofrequency thermoablation of a lesion in the tibial spines area and in the posterior tibial surface to be performed without opening the joint, monitoring the tibial plateau surface, probably decreasing the risk of cartilage damage. Unfortunately, in the case presented, the high pressure from the arthroscopy's pump broke the tibial plateau surface creating a communication to the tibial tunnel used for thermoablation. Tumors located in the tibial spines area are unusual. Biopsy is difficult because of the high risk of contamination of the joint, therefore, it should be done under guidance of a CT-scan. In some cases where radiologic imaging is characteristic for benign lesions, such as chondroblastoma or osteoid osteoma, treatment may be performed at the same time as biopsy, ideally with an expert pathologist available for intraoperative histology.This paper reports an unusual case of chondroblastoma located in the tibial spines and describes an arthroscopic technique used to guide an extra-articular biopsy, followed immediately by Radiofrequency Thermoablation (RT) of the tumor.A 28 year-old male was referred to our department complaining of pain in his right knee for the last four years. During that time, he had undergone three arthroscopies for suspected meniscopathy without any results.A new CT-scan and a MRI-scan of the patient's right knee evidenced the presence of a neoformation located in the proximal tibia, posterior to the insertion of the cruciate ligaments, which had been misinterpreted at the previous scans when it was not clearly visible Figure . The imaThe exploratory arthroscopy confirmed the absence of intra-articular disease, therefore a biopsy was carried out and an arthroscopic-guided RT was performed. A frozen section was not obtained because the imaging and clinical aspect were considered clear.Unfortunately, after the procedure, the high pressure from the arthroscopy's water pump broke the tibial plateau surface and created a communication to the tunnel used for the procedure Figure . NeverthThe patient gave his permission for publication of his images and his caseFor knee arthroscopy, the patient is positioned as required by the surgeon. In this case, the patient was supine with hip and knee flexed to facilitate the access to the knee joint. An X-ray image intensifier was located as shown in Figure Access was provided via the routine parapatellar inferior anterolateral and anteromedial portals, avoiding the fat pad. Exploratory arthroscopy identified the tibial spines.A drill guide was then put through the medial port Figure , aimed a®" with a "StarBurst®"multi-tined expandable probe with seven tips.Following extraction of the biopsy specimen, the same access was used for the RF thermoablation of the tumor. The system used was a monopolar "angiodynamics RITA Model 1500XThe radiofrequency probe was placed at the lesion; placement and expansion were again confirmed radiographically by the C-arm, and the RT was done, reaching a temperature of 100°C for ten minutes.The arthroscopy permitted the monitoring of the articular surface during the entire procedure and no changes were visible. Unfortunately, in this case, the high pressure from the arthroscopy's water pump broke the tibial plateau surface after the RT, creating a communication between the joint and the access tunnel used for the treatment Figure .Fortunately, this did not influence the results, and after one year of follow-up no sign of any recurrence is present at MRI and CT-scans Figure . It is aThe preferred approach to biopsy of lesions in the tibial spines area is by CT-guidance, using an access point, which avoids joint space to decrease the risk of neoplastic contamination. Nevertheless, in selected cases, radiologic findings are so strongly characteristic of benign lesions that biopsy and ablative therapy may be planned as a single, two-step operative procedure. Here we have presented a case in which the imaging and clinical data (very slow growth) was highly suggestive of chondroblastoma, a benign tumor typical of the epiphysis. In this case, treatment was carried out at the same time as the biopsy.Benign chondroblastomais often localized in epiphyseal areas. It is usually found in patients between 10 and 20 years of age, and less frequently in patients between 21 and 30 years of age. The imaging is typical of an osteolytic rounded lesion, with smooth, well-defined borders and sometimes calcifications. On CT and MRI scans, the lesion appears to be parenchymatous, occasionally with cystic cavities and fluid levels [The gold standard in the treatment of chondroblastoma is curettage, with or without the use of local adjuvants.When the lesion is located under the anterior part of the tibial articular surface the surgical approach is anterior; when the lesion is located under the tibial spines area or posterior, the approach should be posterior because of the presence of cruciate ligaments, identifying the popliteal neurovascular bundles.Today, RT has become a minimally invasive alternative to surgery , eliminaA CT-guided RT is probably easier than the arthroscope-guided procedure described herein, because it allows for more accurate verification of the correct placement of the RT probe. However, CT-guidance does not allow for the cartilage surface to be directly monitored during the RT, otherwise possible with the arthroscopic procedure described here, probably decreasing the risk of damage to these tissues during the ablation therapy. Unfortunately, no studies about the changes induced by RT are available in literature, nevertheless, in our case, no changes were noted during the procedure. If any variation in the aspect and color of the cartilage were noted, radiofrequency thermoablation could be stopped and restarted after pulling the probe backward and positioning it farther from the cartilage or after decreasing the working temperature.Unfortunately, in the case presented here, the high pressure from the arthroscopy's water pump broke the tibial joint surface and created a communication between the joint and the disease area. This happened because the water pressure was not decreased during and after the procedure, rather than as a direct consequence of the treatment. The rupture occurred after RT when theoretically the tumoral cells had died, therefore local relapse risk is, definitely, less than that of traditional curettage: the high temperature achieved by RT created a significant necrosis, so the patient remains disease-free at one year of follow-up.Attention must be paid to minimize the intra-articular water pressure during and after the procedure of arthroscope-guided radiofrequency thermoablation.The arthroscopic procedure can be performed in any hospital where arthroscopic and RF instruments are available. However, the CT-guided procedure requires, in addition to RF instrumentation, an anesthesia-supported CT suite, which is not widely available. Indeed both procedures require general or epidural anesthesia.The main advantage of our technique is to allow the treatment of benign lesions in the tibial spines area and in the posterior part of the tibial articular surface where a posterior approach is needed without opening the joint for curettage. Moreover, this technique should permit the cartilage to be directly monitored during the procedure, and because of its safety and relative ease, it can be performed by surgeons at varying levels of expertise in arthroscopic procedures. Nevertheless, more studies need to be conducted to describe the possible cartilage damage.The upper size limit of the lesions potentially treated by RF depends on the opening of the probe tips technically possible up to 5 cm; nevertheless this size cannot be obtain within the bone and the radiofrequency application would not be uniform, therefore, in our opinion, lesions bigger than 3 cm in diameter should not be treated.A fundamental requirement of this treatment is that it must only be done after histologic confirmation of a benign lesion, or when radiologic imaging is strongly characteristic of a benign lesion.The patient has given his consent for the publication of his case and his images.The authors declare that they have no competing interests.CZ drafted the manuscript, GT designed the procedure, NS performed the procedure, BDP revised the paper, EA performed the procedure and supervised the manuscript. All authors read and approved the final manuscript.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-2474/13/52/prepub


We report an unexpected presentation of metastatic renal cell carcinoma (RCC) to the ciliary body and an interesting response to proton beam radiotherapy.We encountered a case of angle-closure glaucoma as the initial presentation of ocular metastasis to the ciliary body in a 65-year-old Caucasian man who had undergone right radical nephrectomy for RCC 15 years earlier. He underwent YAG  laser peripheral iridotomy while further metastatic workup took place. His condition was eventually diagnosed as stage IV metastatic RCC of the clear cell type and involved multiple sites, including the ciliary body, brain, lungs, liver, and pancreas. The progression of RCC metastasis to the ciliary body was studied for 16 months. The ciliary body mass continued to grow despite systemic treatment with temsirolimus and interleukin-2 and intravitreal injections of bevacizumab. The tumor size peaked at 6.11 × 6.06 mm before the start of proton therapy, which reduced the tumor size to 5.07 × 4.39 mm.RCC can produce metastases involving unusual sites many years after resection of the primary tumor. Proton therapy was found to be effective in treating RCC metastasis to the ciliary body in settings in which other treatment modalities failed. Renal cell carcinoma (RCC) accounts for approximately 85% of primary renal neoplasms and represents approximately 3% of all adult malignancy . The mosWe report a rare case of angle-closure glaucoma secondary to ciliary body metastasis in a patient who underwent radical nephrectomy for RCC 15 years earlier. Also, we review the different treatment modalities we used to treat the ocular metastasis.A 65-year-old Caucasian man presented to our emergency department and complained of sudden-onset blurry vision and pain in his right eye. He reported associated nausea and a right-sided headache. His medical history was significant for RCC, for which he had undergone right radical nephrectomy 15 years earlier. After the initial nephrectomy, he had completed 10 years of surveillance, after which he was thought to be disease-free.oculus dexter, or OD) and 20/20 in his left eye . The intraocular pressures by applanation tonometry were 50 mm Hg OD and 14 mm Hg OS. The results of a left eye exam were unremarkable. A slit-lamp exam of his right eye demonstrated a red mass that was located in the superior nasal aspect of his iris at one to three o'clock and that was associated with dilated episcleral feeder vessels  secondary to a ciliary body mass in his right eye. He was started on timolol, acetazolmide, and latanoprost followed by a YAG  laser peripheral iridotomy to lower the intraocular pressure.The results of a further metastatic workup were consistent with a stage IV RCC that was of the clear cell type Figure  and thatThe ciliary body mass continued to grow despite systemic treatment with temsirolimus   and inteLastly, proton radiotherapy was administered. Tantalum clips were placed to outline the posterior margin of the tumor. Our patient received a total of 30 cobalt gray equivalents (CGE) in three fractions given over the course of six calendar days. As a result, the tumor size decreased to 5.07 × 4.39 mm Figures  and 2b. The mechanism of ocular spread in RCC is presumed to take place through venous diffusion within the small chroidal vessels, and neoplastic cells travel as emboli . SimultaPrimary RCC produces angiogenic growth factors , which are responsible for tumor growth, proliferation, metastases, and survival through high serum levels . UnfortuThe prognosis of metastatic RCC is generally poor; median survival is 10 months and five-year survival is less than 5% ,14. Our Several urologists advocated RCC surveillance for at least 10 years after the initial nephrectomy. Others concluded that follow-up for life is reasonable -20. Our We present an interventional case report in which the progression of RCC metastasis to the ciliary body was studied for 16 months. The response to different treatment modalities was investigated. The ciliary body mass continued to grow despite systemic treatment with temsirolimus and IL-2 and intravitreal injections of bevacizumab. The tumor size peaked at 6.11 × 6.06 mm before the start of proton therapy, which reduced the tumor size to 5.07 × 4.39 mm. To the best of our knowledge, this is the first case report of a ciliary body metastasis from RCC that responded to proton radiotherapy after other treatment modalities had failed.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.oculus dexter (right eye); OS: oculus sinister (left eye); RCC: renal cell carcinoma.AACG: acute angle-closure glaucoma; IL-2: interleukin-2; OD: The authors declare that they have no competing interests.TA analyzed and interpreted the patient data and wrote the manuscript. BK helped to gather the data and write the manuscript. LL was the radiation oncologist who treated our patient with proton beam radiotherapy. MER was the attending ophthalmologist who evaluated our patient, performed the bevacizumab intravitreal injections, and coordinated the proton beam radiotherapy. All authors have read and approved the final manuscript.