Abdominal computed tomography (CT) is frequently performed to evaluate gastrointestinal pathologic conditions. The majority of the gastrointestinal radiology literature has concentrated on the colon, stomach, and distal small bowel. The duodenum is often overlooked on imaging, namely on CT, but its anatomy  and location in such close proximity to other viscera results in involvement by a multitude of primary and secondary processes, some of them exclusive to this bowel segment. While some conditions, like duplications, lipomas, and diverticula, are usually asymptomatic and are incidentalomas that have no pathologic significance, others are symptomatic and very relevant and should be recognized by every general radiologist: development conditions such as annular pancreas and gut malrotation; inflammatory processes such as ulcers and secondary involvement from pancreatitis; neoplastic conditions such as adenocarcinoma, lymphoma, or local extension from adjacent malignancies. They all can be reliably diagnosed with CT. In this article, we demonstrate the typical imaging features of various diseases involving the duodenum, such as developmental, traumatic, inflammatory, infectious, neoplastic, and postsurgical pathologic conditions in alphabetical order, focusing mainly on upper gastrointestinal series (UGIS) and CT but also some radiography, ultrasound, and magnetic resonance (MR) imaging. Duodenal pathology is often overlooked on imaging, namely on CT.Its location  results in involvement by a multitude of primary and secondary processes.CT plays a major role in the diagnosis of traumatic duodenal injury and post-operative complications and helps in the diagnosis of other conditions such as infections, inflammations, and neoplams.The duodenum is a C-shaped hollow viscus located at the level of L1-3 vertebrae, that lies in front of the vertebral column, closely related to the head of the pancreas.The duodenum is composed of four parts. It begins at the duodenal bulb as a continuation of the pylorus. The first part (D1) is anterior to the common bile duct, portal vein, and gastroduodenal artery, superior to the pancreatic head, and posterior to the gallbladder and liver. The second part  begins at the superior duodenal flexure and ends at the inferior duodenal flexure. It is anterior to the right kidney, ureter, and adrenal gland, and medial to the ascending colon and hepatic flexure, with the pancreatic head located medially. The major and minor duodenal papillae are situated on its medial wall. The third  part begins at the inferior duodenal flexure and passes transversely to the left, crossing the vertebral column. Posteriorly lies the right psoas muscle, aorta, and inferior vena cava and anteriorly the small bowel mesentery root. The fourth  part passes superiorly and then curves anteriorly and terminates at the duodenojejunal flexure where it continues as the jejunum. The left psoas muscle and the aorta lie posteriorly, with the stomach in a superior position.The arterial supply is provided by the right gastric and right gastroepiploic arteries in the duodenal cap (first 2.5 cm), superior pancreaticoduodenal artery in the remaining D1 to mid-D2, and inferior pancreaticoduodenal from mid-D2 to the ligament of Treitz. Venous drainage occurs through the prepyloric vein in the duodenal cap and superior and inferior pancreaticoduodenal veins in the remaining duodenum. Duodenal nodes drain distally to superior mesenteric nodes and proximally to celiac nodes. It is an intraperitoneal organ for the 2–3 first centimeters with the remaining length being retroperitoneal .Its location, with both intraperitoneal and retroperitoneal segments, and proximity to other organs, results in duodenal involvement by a multitude of primary and secondary processes.In this review, we demonstrate the imaging features of several diseases involving the duodenum, in alphabetical order, to facilitate navigation through this article, focusing mainly on upper gastrointestinal series (UGIS) and computed tomography (CT) but also some radiography, ultrasound (US), and magnetic resonance (MR) imaging.Adenocarcinoma is uncommon  but is the most common malignant tumor of the duodenum . It is mIn UGIS, it may manifest as an annular constricting wall thickening or a polypoid or ulcerative mass. “Apple-core” lesions are a classical finding.CT reflects the same findings seen in the UGI series. It usually manifests as a soft-tissue attenuation thickening of the duodenal wall with enhancement Fig. a–d. It mDifferentiation between adenocarcinoma of the duodenum and other malignant tumors of the ampulla, pancreas, and common bile duct, with duodenum invasion, could be extremely difficult .Periampullary tumors include benign and malignant neoplasms that arise within 2 cm of the ampulla of Vater, such as true ampullary tumors , the differential diagnosis between true ampullary tumors and periampullary tumors is very difficult.Endoscopic cholangiopancreatography (ERCP) and magnetic resonance cholangiopancreatography (MRCP) may be useful in determining the origin of the periampullary tumor Fig. d, e.CT may show wall thickening or a polypoid mass arising from the medial wall of D2, with associated biliary duct dilatation (more commonly), without or with pancreatic duct dilatation (double duct sign) .Afferent loop syndrome (ALS) represents a mechanical obstruction of the afferent loop in 13% of the patients submitted to pancreaticoduodenectomy . Most caThe backpressure from the dilated afferent loop can cause biliary dilatation and acute pancreatitis.Although UGI series can be helpful in the diagnosis by allowing visualization of non-filling of the afferent loop Fig. c, non-obvalvulae conniventes projecting into the lumen, in symptomatic patients after gastroenterostomy, makes the diagnosis.CT is the most important imaging tool in establishing the diagnosis and determining the site, degree, cause, and complications  of ALS . A fluidIt occurs following subacute or chronic cholecystitis that leads to gallstone erosion into the bowel  .The site of obstruction is usually the terminal ileum because it is the narrowest portion of the small bowel.Rigler’s triad encompasses the three typical findings of gallstone ileus on a radiograph: pneumobilia, dilated small bowel loops, and a calcified gallstone in an ectopic location.CT is the modality of choice because it allows a correct diagnosis of gallstone ileus with higher accuracy and detects the exact location of the ectopic stone and the biliary–enteric fistula  Fig. 4)4).Fig. 4Bouveret syndrome is a subtype of gallstone ileus which refers to gastric outlet obstruction due to the impaction of a gallstone in the pylorus or proximal duodenum .The diagnosis was usually made with endoscopy but is increasingly being found on CT in the setting of abdominal pain in emergency departments.On the UGIS, there is an endoluminal filling defect in the duodenum that produces partial or complete gastric outlet obstruction.CT shows to a better extent the gallstone, the cholecystoduodenal fistula, and the gastric dilatation that make up this syndrome Fig. .Fig. 5BoCystic dystrophy of the duodenal wall is related to groove pancreatitis  and is nTwo patterns are described according to the lesion size: the more common cystic pattern, in which the cysts are > 1 cm  and the uncommon solid pattern, in which the lesions are < 1 cm .The cysts are usually located within the thickened submucosa or muscular layers, of the medial wall of the descending duodenum.CT shows multiple small and elongated cysts within the duodenal wall, mural thickening of the descending duodenum, with or without luminal stenosis .MRCP can aid in the diagnosis by displaying cystic changes along the duodenal wall and in the pancreatico-duodenal groove.Choledochal cysts are rare congenital dilatations of the common bile duct. The diagnosis of a choledochal cyst is made based on the disproportional dilatation of the extrahepatic bile ducts after excluding mechanical factors  as the cause of the dilatation.Choledochocele is a cystic/diverticular herniation of the distal common bile duct into the duodenum Fig.  13]. It. It13]. CT shows a fluid-filled sac that protrudes into the duodenal lumen but does not opacify when oral contrast is given. Complications of choledochal cysts such as cholecystitis, cholangitis, biliary stricture, choledocholithiasis, pancreatitis, and malignant transformation into cholangiocarcinoma are promptly diagnosed with CT. MRCP may help when CT is doubtful Fig. d.GI duplication cysts most often originate from the small bowel. Duodenal duplication cysts most commonly arise in the medial wall of the D2 and D3. Most are incidental findings at CT Fig. a, b. SomBecause most duplication cysts do not communicate with the bowel lumen, on CT they typically appear as a well-circumscribed cystic mass with fluid attenuation. Intracystic hemorrhage may occur Fig. c–e. The Endoscopic ultrasonography (EUS) is the diagnostic tool of choice to investigate duplication cysts since it can distinguish between solid and cystic lesions and establish cyst location relative to surrounding tissues .Duodenal extramural diverticula are incidental findings in most of the patients.They may result from mucosal prolapse or the prolapse of the entire duodenal wall  and are Duodenal diverticula may present with serious complications like perforation, duodenal fistulas, intra-abdominal abscesses, and sepsis.UGIS show barium-filled pouch-like projections beyond the normal lumen and can be differentiated from ulcers by visualization of mucosal folds entering the neck of the diverticulum and change in appearance with peristalsis Fig. a.Fig. 9DCT shows as a fluid and/or gas-filled collection in a typical location, that communicate with the duodenal lumen Fig. b. A duodPeriampullary diverticula rarely can cause bile duct obstruction Fig. d with jaEctopic pancreas is defined as abnormally located pancreatic tissue with its own ductal system, with no vascular, neural, or anatomic contact with the normal gland .It is the most common heterotopia within the gastrointestinal system and is usually an incidental finding in CT scans or autopsy Fig. . NeverthThe most common locations are the duodenum (28%), stomach (26%), and jejunum (16%). It most frequently occurs in the submucosa (75%), but can also be present within the muscularis propria or the serosa.On UGIS, a characteristic appearance is a central small collection of barium inside an umbilicated nodule in the duodenal submucosa.On CT, presents as a mass in the duodenal wall with an enhancement pattern similar to that of pancreatic tissue. GIST and leiomyoma have to be differentiated from this condition because they all share a submucosal origin. A flat shape, lobulated contour, and endoluminal growth are most indicative of ectopic pancreas.MRCP may depict the presence of ducts within the tissue, a finding that is pathognomonic for ectopic pancreas .Secondary involvement of the duodenum with malignancy can occur by local extension from adjacent sites or metastases from distant organs .see letter A).Gastric carcinomas may extend through the pylorus into the duodenal bulb or invade D4 Fig. . GastricPancreatic adenocarcinomas can be locally aggressive, and extension to the duodenum is sometimes seen at CT .Colon carcinoma may cause local mass effect or duodenal invasion, occasionally with the formation of a colo-duodenal fistula.An aorto-duodenal fistula should always be suspected in patients who have undergone aortic graft surgery and present with gastrointestinal bleeding .Direct communication between the duodenum and the aorta is one of the most feared clinical situations of vascular surgery, with a very high fatal outcome (near 100% if not promptly operated). The most common locations are the D3 and D4 because these are the sites closer to the aorta.These fistulas can be primary  or secondary (after aortic surgery and/or the placement of a synthetic aortic graft). Since there has been an increase in this kind of surgery, the secondary type is now more common than the primary one.CT is the mainstay of diagnosis Fig. . The dirGIST stands for gastrointestinal stromal tumor, which is the most common benign tumor of the small bowel Fig. .Fig. 13GIt arises from the interstitial cells of Cajal and expresses a tyrosine kinase growth factor—c-KIT (CD117) .Most of GISTs are benign (70–80%). Benign behavior is particularly common in gastric tumors. Tumors of the small-bowel, including the duodenum, are prone to a more aggressive behavior . They moOn UGIS, the mass is typically exophytic but may also be intramural or intraluminal Fig. d.On CT, they are seen as round lesions with patchy/heterogeneous enhancement and central areas of low attenuation (necrosis or cyst formation). Smaller tumors rarely necrose and thus have homogeneous soft-tissue attenuation. Hemorrhage may occur, while calcification is uncommon. It is difficult to differentiate a benign from a malignant GIST from imaging and histologic features .In general, tumors greater than 5 cm have a greater chance of being malignant.Tumor necrosis is seen in both benign and malignant GISTs Fig. b.Fig. 14The most specific sign of malignancy is the presence of synchronous metastases, with the liver and peritoneum most commonly affected Fig. d. Since Brunner gland hamartoma is a very rare condition .It is a proliferation of the Brunner’s glands, which are located mainly in the duodenal bulb and proximal duodenum, at the submucosa layer. Its pathogenesis and pathology remain unclear; it may evolve to adenoma and adenocarcinoma.They are variable in size, typically 1–3 cm. Intussusception has been described in a few cases.On UGIS, there is a nonspecific sessile or pedunculated polypoid-filling defect in the duodenum, pedunculated in the majority of cases.On CT imaging, a nonspecific polypoid lesion with submucosal origin in the duodenum is present Fig. .Fig. 15HThese lesions must be differentiated from adenomas, leiomyomas, neurogenic tumors, aberrant pancreatic tissue, prolapsed pyloric mucosa, and cystic dystrophy of the duodenal wall.Intussusception is an important cause of acute abdomen in children, where no lead point can usually be identified.In adults, however, a specific lead point is identified in more than 90% of cases, and a neoplasm is the culprit in 65%.Gastric intussusception occurs secondary to a mobile gastric tumor 25].Celiac disease is a chronic autoimmune condition of gastrointestinal malabsorption that occurs in genetically susceptible individuals to gluten .The bowel mucosa becomes flattened and villi disappear, while the submucosa, muscularis, and serosa remain normal. This destruction begins in the duodenum and progresses distally to the ileum over time.These patients have an increased risk of lymphoma and gastrointestinal tract carcinoma.The gold standard diagnostic test is a duodenal biopsy.UGIS findings include duodenitis, small bowel dilatation, flocculation, effaced folds, decreased number of folds per inch of jejunum, and increased number of folds per inch in the ileum (≥ 5)—reversed jejunoileal fold pattern .CT may show bowel wall thickening Fig. , dilatedNEN are rare neoplasms that arise from cells of the endocrine and nervous systems. These tumors originate within the pancreas or from similar neuroendocrine cells outside of the pancreas.They are classified as functioning or non-functioning (if they secrete hormones or not); and they can be benign or malignant (60–92%).Insulinomas and gastrinomas are the most common of these rare tumors.While insulinomas are almost always found in the pancreas, gastrinomas are found within the gastrinoma triangle in 90% of cases, an anatomical area which includes the head of the pancreas and D1 and D2 Fig. .Fig. 23NNEN grade 1–2 are usually hyperdense, but NEN grade 3, as a rule, have a heterogeneous structure with a pronounced hypodense component and liver metastases. The higher the NEN grade, the less intense contrast enhancement, and the tumor itself is more heterogeneous. Most often NEN grade 2 mimic GIST. In the absence of intense contrast enhancement, a large tumor can mimic adenocarcinoma.On CT, they manifest as a small nodule in an appropriate location, with enhancement to a greater degree than the pancreas during arterial and portal venous phases. They can be multiple in 10%. They may mimic arterial structures when in a peripancreatic location . Water sInternal hernias (IH) are defined as the protrusion of viscera through a normal or abnormal peritoneal or mesenteric aperture within the peritoneal cavity. Paraduodenal hernias are classically regarded as the most prevalent IH (53% of the cases), with 75% of them occurring on the left.Left paraduodenal IH on the left develops through the fossa of Landzert located at the duodenojejunal junction . Right pOn UGIS crowding of small-bowel loops in the hernial sac, apparent encapsulation of dilated bowel loops in an abnormal location, segmental distension with stasis, and reversed peristalsis may be seen.CT of left paraduodenal IH shows a saclike mass of dilated bowel left to the ligament of Treitz and between the pancreas and the stomach that displaces the transverse colon and duodenal flexure inferiorly Fig. a–c. The Paraduodenal pancreatitis is an uncommon form of segmental chronic pancreatitis that affects the pancreaticoduodenal groove near the minor papilla. It no longer should be called groove pancreatitis.The hallmark of the disease is the presence of scar tissue with fibrosis in the pancreaticoduodenal groove, which may lead to duodenal stenosis .see letter C for cystic dystrophy).On CT, there’s ill-defined sheet-like soft-tissue attenuation material between the pancreatic head and the descending duodenum that may show late enhancement Fig. . DuodenaDifferentiation from duodenal or periampullary tumors is difficult with imaging alone, although the presence of cystic lesions within the mass would favor a diagnosis of paraduodenal pancreatitis. Sometimes, the two entities may coexist, since the inflammation is cause and/or consequence of cancer.Annular pancreas is an uncommon morphologic congenital anomaly that occurs following failure of the pancreatic ventral bud to rotate with the duodenum, resulting in the envelopment of the duodenum .This condition can manifest in the 1st decade of life with duodenal stenosis, although the diagnosis is difficult at this stage due to lack of intra-abdominal fat in children and the narrow width of the pancreatic band.Half of the cases occur in adult life, with 66% of them remaining asymptomatic. When symptomatic, the presentation is usually with abdominal pain, postprandial fullness, vomiting, post-bulbar peptic ulceration, pancreatitis, or biliary obstruction.UGIS show an eccentric or concentric narrowing of D2.On CT, pancreatic tissue completely or incompletely surrounds the descending duodenum Fig. , sometimERCP or MRCP allows for accurate delineation of the annular duct anatomy that usually joins the Wirsung or Santorini ducts.Crohn’s disease is the most common inflammatory condition of the small bowel, with an incidence ranging from 5 to 60% at the duodenum. It usually involves the D1 and D2, almost always associated with continuous involvement of the stomach. The first evidence of the disease tends to manifest as ulcer or stricture formation, while late involvement typically occurs as fistulous tracts from an adjacent diseased loop of small bowel or colon.The UGIS main advantage over CT is the ability to depict aphthous ulcers in the active phase . Other pCT of active disease may reveal hyperenhancing duodenal wall thickening, mesenteric edema, prominent mesenteric vessels (comb sign), and lymphadenopathy Fig. d, as welMR enterography can demonstrate active small-bowel inflammation and complications such as bowel obstruction, penetrating disease, and abscess formation, with multiplanar capability, and functional studies , without the use of ionizing radiation making this method very suitable for a young population with CD that will likely undergo many imaging examinations throughout their lives .Duodenal perforation can result from peptic ulcers, perforated tumors, iatrogenic trauma (ERCP), or non-iatrogenic trauma .see letter T for Trauma and letter U for Ulcers).Duodenal perforation associated with peptic disease is commonly found in the bulb, whereas D2 and D3 are the common sites of perforation caused by blunt trauma .On UGIS during a symptomatic episode, there is strong to-and-fro peristalsis and stomach and duodenal dilatation proximal to a vertical extrinsic impression at the level of D3. Relief of obstruction with postural change may be seen.CT angiography (or MR angiography) allows measurement of the distance between the aorta and the SMA Fig. b. NormalAccessory spleens occur in 10–16% of normal individuals. They may be single or multiple and are most frequently found near the splenic hilum. Occasionally they can be found in the suspensory ligaments of the spleen or the pancreatic tail. Rarely they can occur elsewhere in the abdomen, like near the duodenum .In most patients, they are incidental findings of no clinical significance. Sometimes it is important to identify accessory spleens when they are confused with a mass of another type, particularly when they occur in an atypical location.On CT and MR, they appear as round or ovoid masses, of regular margins, from 1 to 5 cm in size, with the same pattern of contrast enhancement/ dynamic behavior of the normal spleen  Fig. 3030.Fig. 3Technetium sulfur colloid radionuclide scans can be used to confirm the diagnosis in problematic cases.Duodenal trauma may result from penetrating or blunt injury , deformity of the duodenal bulb, and erosions.Duodenal peptic ulcers are almost always caused by H. pylori infection. 5% of them occur at the duodenal bulb and are not considered a premalignant condition.UGIS shows a round or oval pocket of barium filling the ulcer, with an edematous collar of swollen mucosa, absent mucosal pattern, no contraction with the peristalsis, and thickened folds radiating to the ulcer niche .Complications include bleeding, perforation, and stricture, in which cases CT has better accuracy  Fig. 3232. InterSystemic lupus erythematosus is a multisystem autoimmune disorder that induces necrotizing vasculitis. Lupus vasculitis at the gut is usually multifocal, transient, migratory and can occur throughout the GI tract, but is most commonly found in the small bowel.Whenever mesenteric ischemia occurs in young patients, at unusual sites, with a tendency to affect both the small and large intestine, and is associated with genitourinary disease, the hypothesis of vasculitis must be raised.Radiologic findings in the various types of vasculitis often overlap . KnowledUGIS may show evidence of separation of the bowel loops, “thumbprinting,” and mucosal fold thickening.On CT inflammation of the small caliber blood vessels of the GI tract produces a variety of complications, such as intestinal ischemia/infarction, hemorrhage, ileus, ulceration, and perforation, with according imaging findings . BecauseIntraluminal duodenal diverticula are rare malformations that are thought to result from chronic antegrade enteric propulsive forces on an incomplete duodenal congenital diaphragm . Thus, tDespite being congenital, they usually manifest in adulthood in a nonspecific manner. Complications include mechanical obstruction, peptic ulcer disease, hemorrhage, cholangitis, and pancreatitis. They are more common on the descending duodenum.On UGIS, there is evidence of the pathognomonic “windsock sign”, a barium-filled sock within the lumen of the duodenum, whose radiolucent wall is outlined by barium both inside the diverticulum and within the true lumen of the duodenum  Fig. 3434a, d.FiCT images show a low-density endoluminal collection in the duodenum Fig. b, c. TheDuodenal diseases may produce radiographic changes in the thorax and abdomen.In fact, an abnormal radiograph may be the initial clue to the diagnosis of several illnesses of the duodenum Fig. .Fig. 35IA Roux-en-Y enteric anastomosis was first described by surgeon Cesar Roux as a means to bypass an obstructed stomach due to recurrent ulcer attacks. Nowadays, the procedure is used as a means to re-route food or gastrointestinal secretions in other conditions such as gastric bypass (for obesity), following partial or complete gastrectomy , chronic pancreatitis, biliary duct obstruction , etc. It comprises an anastomosis of the distal divided end of the small bowel to another organ such as the stomach or esophagus and an end-to-side anastomosis between the biliopancreatic limb and the small bowel distal to the cut end .The duodenum is in its normal location and is part of the biliopancreatic limb, which measures about 100 cm in length.An opening in the transverse colon mesentery is created for passing the Roux limb of the jejunum, so internal hernias are a possible complication of this procedure.On UGIS, only the Roux loop is opacified; the biliopancreatic limb is usually not opacified Fig. a, b.Fig.CT reflects the new anatomy, with surgical clips seen at the anastomotic sites  Fig. 3636c, d.Malrotation is the congenital abnormal position of the bowel within the peritoneal cavity and usually involves both small and large bowel.The term applies to a wide range of intestinal anomalies, from omphalocele in newborns to asymptomatic nonrotation of the bowel in adults, and the positions of the duodenojejunal junction and colon depend on the developmental stage the embryologic rotation failed.It is usually diagnosed in the first year of life, with a clinical picture of bilious vomiting because of duodenal obstructive bands or midgut volvulus.In nonrotation, an important minority and subtype of malrotation, the small bowel is located predominantly on the right side and the colon on the left side within the peritoneal cavity and complications such as volvulus are less frequent.UGIS may depict a Z-shaped configuration of the duodenum in the presence of obstructing peritoneal bands or a corkscrew-shaped duodenum in the presence of volvulus, in newborns and young infants; the duodenojejunal junction is located lower than the duodenal bulb and to the right of the expected position.On CT, the duodenum does not cross the midline between the aorta and the SM vessels. An abnormally high position of the cecum and the SM vein to the left of the SM artery may also be seen Fig. .Fig. 37GEvery diagnostic radiologist should be aware of both the most frequent and the exclusive pathology of the duodenum.CT plays the pivotal role in the diagnostic imaging of the duodenum. Nevertheless, UGIS still have utility in some conditions and are even better than CT in precise indications . MRI, ultrasound, and radiographs may additionally be useful in a few other settings.


The rarity of juvenile psammomatoid ossifying fibroma (JPOF) and lack of cytogenetic studies prompted us to report a novel SETD2 gene mutation in a benign odontogenic tumour.A 21-year-old man presented with a hard, expanded mandibular cortex. Computed tomography revealed multilocular radiopacity in the mandible; this was reconstructed via segmental mandibulectomy using a vascularised iliac crest flap. Based on the clinical and histological findings, we diagnosed JPOF associated with an aneurysmal bone cyst. Microscopically, the solid area was characterised by many rounded or angular ossicles in a cellular fibrous stroma. The stromal cells were spindle-like or stellate. Next-generation sequencing detected a frame shift mutation of the SETD2 gene, while the copy number was normal.Our findings suggest further genetic studies should be performed to assess whether this mutation is related to tumour genesis.  According to the World Health Organization (WHO) classification, there are three subtypes of ossifying fibromas of the craniofacial skeleton and jaw, differing in clinical presentation and histopathological appearance: cemento-ossifying fibroma (COF), juvenile trabecular ossifying fibroma (JTOF), and juvenile psammomatoid ossifying fibroma (JPOF) . JPOFs sA 21-year-old man was transferred to the Department of Oral and Maxillofacial Surgery, Medical University of Graz when a slow-growing tumour in the left mandible, which had been present for 10 years and could not be diagnosed or treated adequately in his home country. On admission, he had facial asymmetry with adequate mouth opening. The intraoral examination revealed left-sided, hard, expanded buccal and lingual mandibular cortices. Computed tomography showed an 8.5-cm multilocular radiopacity in the left mandibular neck extending to the midline. Despite occupying the soft tissues, the mainly cystic, focal, solid lesion (arrow) was completely covered by thin, non-perforated, pre-existing cortical bone (Fig. The surgical specimen was 12 cm in its greatest longitudinal diameter and dominated by an 8-cm-diameter, nodular, bone-distending tumour. On cross-sections, the tumour consisted mainly of cystic spaces varying in size and sometimes filled with blood clots; a solid, nodular area measuring 3 cm was seen only at the lingual aspect of the lower part Fig. . The ossOnly a few JPOFs located in the mandible associated with an aneurysmal bone cyst have been reported –11. The On searching the literature, we found no cytogenetic studies of a mandibular JPOF with an associated aneurysmal bone cyst. However, we found cases that were diagnosed retrospectively as psammomatoid ossifying fibromas on re-evaluation their histopathological appearance. One of these was located in the mandible and had an interstitial deletion on chromosome 2 between q31–32 and q35–36 ; three cKnocking out Hrpt2 in the mouse results in preimplantation lethality as well as when conditionally deleted in adult animals . De MesqIn our case, the SETD2 gene mutation likely played an important role in tumourigenesis. Therefore, it would be interesting to determine whether this mutation is typical of JPOF in general. To this end, further genetic studies on a JPOF series are required.


Congenital extrahepatic portosystemic shunts are a rare cause of lower gastrointestinal bleeding in children. They result from the connection of a systemic vessel with the portomesenteric vasculature before the division of the main portal vein. Herein, we report a case of a congenital extrahepatic portosystemic shunts type II in a 4-year-old male diagnosed by Doppler ultrasonography during the investigation of abdominal pain and recurrent hematochezia, later confirmed by computed tomography. Conventional angiography with a balloon occlusion test revealed patent intrahepatic portal branches not depicted by previous imaging techniques. Successful shunt closure was achieved by endovascular approach with an Amplatzer Septal Occluder without complications. A portosystemic shunt consists in an abnormal communication between the portal venous system and the systemic circulation, allowing venous flow to bypass the liver in a variable degree. Although most cases result from chronic portal hypertension (acquired shunts), they may also be of congenital origin Congenital extrahepatic portosystemic shunts (CEPS), also known as Abernethy malformations, are rare vascular anomalies that result from the connection of a systemic vessel with the portomesenteric vasculature before the division of the main portal vein (MPV) The clinical presentation of CEPS is highly variable, reflecting different anatomic configurations and coexistent pathologies. They may be diagnosed on neonatal screening tests due to high galactosemia or later in life after the development of complications, such as portal hypertension, hepatic encephalopathy, pulmonary hypertension and/or hepatopulmonary syndrome The initial presentation of CEPS may be secondary to associated congenital cardiac, abdominal and skeletal malformations, which often coexist ,3. ThereA 4-year-old male with a history of asthma was admitted to the emergency department with hematochezia and diffuse abdominal pain. His mother recalled two previous episodes of hematochezia in the last 5 months. Physical examination revealed facial telangiectasia and palmar erythema. His abdomen was soft and non-tender and no jaundice, fever, or peripheral edema were present.Laboratory evaluation showed normocytic normochromic anemia  but no other abnormalities in the remaining blood count and liver biochemical and function tests. An abdominal ultrasonography was performed to rule out intussusception. No distended or wall thickened bowel loops were noted. However, hepatic evaluation showed a direct communication between the MPV and the inferior vena cava (IVC) with flow on color and spectral Doppler . No intrSubsequent computed tomography (CT) angiography confirmed the presence of a portocaval shunt and showed a normal origin of the MPV, resulting from the confluence of the superior mesenteric vein and splenic vein . CT alsoLiver magnetic resonance imaging (MRI) confirmed the absence of parenchymal lesions. Since no portal branches were detected by Doppler ultrasonography, CT and MRI, a type I CEPS was suspected.Further investigation with colonoscopy found rectal varices and a bleeding varix was successfully sclerosed with polidocanol. Hemorrhoids and a nonbleeding anal fissure were also evident. Upper endoscopy was normal. Meckel diverticulum was ruled out on tc-99 m pertechnetate scintigraphy.Serum alpha-fetoprotein and ammonia levels were normal. Transthoracic echocardiography and ventilation/perfusion scintigraphy did not reveal additional anomalies.Portal venography with a percutaneous transjugular approach showed patent but hypoplastic intrahepatic portal branches, allowing the definitive diagnosis of CEPS type II . Portal The shunt was closed 1 month later, in a single session, with an Amplatzer Septal Occluder , without complications . A Doppler ultrasound of the liver performed 6 months later showed absence of flow between the MPV and IVC . At 1-yeHerein we describe a type II CEPS with direct drainage of portal blood into the IVC. The shunt manifested itself as variceal lower gastrointestinal hemorrhage due to significant portal hypertension (16 mmHg).Gastrointestinal hemorrhage is the presenting symptom in 8.1% of cases with CEPS and in 10.5% of CEPS with direct drainage to the IVC Cross-sectional imaging plays an important role in the work-up of these patients. Multidetector CT angiography is a valuable technique in the evaluation of the shunt anatomy due to its high spatial resolution Conventional angiography remains the best imaging technique for assessment of portomesenteric vasculature and allows the depiction of vessels before and after a shunt occlusion test. This temporary closure may reveal intrahepatic portal vessels not seen by other imaging techniques and it is thus essential for a correct classification of CEPS ,10. MeasThe classification of CEPS has important therapeutic implications. Liver transplantation is the only curative treatment for type I shunts, while patients with preservation of some intrahepatic venous flow (type II) are candidates for surgical or endovascular shunt closure Shunt closure should be performed in type II shunts as early as possible after the first year of life, in order to prevent long-term complications In conclusion, this report presents a rare cause of lower gastrointestinal bleeding in children. It also highlights how Doppler ultrasonography and cross-sectional imaging techniques are essential in the study of these patients but are not sensitive enough to rule out intrahepatic portal flow. Hence, management of CEPS should always include conventional angiography with shunt occlusion for an adequate classification and appropriate treatment.The authors declare that written informed consent was obtained from the patient's mother for publication of the case with accompanying images.


Nutcracker syndrome is a condition in which the left renal vein is pinched between the abdominal aorta and the superior mesenteric artery, resulting in an increase in renal vein pressure and certain symptoms. We report a very rare case of retroperitoneal hematoma caused by the rupture of varicose veins of the left ovary.A 77-year-old Japanese woman, para 7, experienced sudden left lower abdominal pain. She had no history of trauma or treatment complications. Computed tomography revealed a left retroperitoneal hematoma, but her abdominal pain subsided quickly; thus, urgent treatment was not required. We then scheduled her for an assessment regarding the cause of her bleeding. However, 6 days after the pain onset, abdominal pain symptoms recurred, confirming hematoma regrowth. Magnetic resonance imaging and three-dimensional computed tomography revealed an abnormal vascular network from the left side of the uterus to the left adnexa. Subsequent angiography revealed that the retroperitoneal bleeding originated from rupture of the distended left ovarian vein, which caused blood reflux from the left renal vein to the left ovarian vein. Although angiography confirmed a passage between the left renal vein and inferior vena cava, computed tomography showed obvious stenosis in the left renal vein. In accordance with these findings, we diagnosed the cause of the distention and rupture of the left ovarian vein as nutcracker syndrome. She underwent embolization of the left ovarian vein as hemostasis treatment, and had a good course thereafter.This is the first report of a spontaneous rupture of the left ovarian vein caused by nutcracker syndrome. Nutcracker syndrome is not yet well known to clinicians and should be considered as part of the differential diagnosis when an abnormal vascular network in the pelvis is found. The left renal vein (LRV) anatomically passes between the abdominal aorta and the superior mesenteric artery (SMA). The nutcracker phenomenon is the condition in which this gap narrows for some reason and causes stenosis in the LRV, and when it is accompanied by certain clinical symptoms, it is called nutcracker syndrome (NCS) , 2.In 1937, the anatomist John C. Boileau Grant first reported the phenomenon of LRV pinching, and in 1972, de Schepper named the condition “nutcracker syndrome” . BecauseElevated blood pressure in the LRV causes certain signs and symptoms of NCS, hematuria being the most common, but distension and dysfunction of the gonadal vein also cause pelvic congestion syndrome (PCS) in women and varicocele in men. In recent years, there have some reports on NCS but, to our knowledge, this report is the first that focuses on spontaneous rupture of the ovarian vein caused by NCS.We report this case to raise awareness of the pathophysiology of NCS, and to suggest that ovarian varicose veins may rupture spontaneously.A 77-year-old Japanese woman, para 7, who went through menopause at age 48, suffered sudden onset of left lower abdominal pain and visited a primary care doctor. Computed tomography (CT) revealed torsion of a left ovarian cyst, and she was transferred to our hospital for surgery. She had a history of left-side breast cancer and underwent total left mastectomy at the age of 67, with no recurrence thereafter. Although she was a carrier of hepatitis C, her liver function and coagulation remained normal, and she was being followed-up without medication. Furthermore, she was taking nifedipine and candesartan cilexetil for hypertension. Meanwhile, her family history and psychosocial history were unremarkable. She also had no history of trauma.2; blood pressure, 112/50 mm Hg; pulse, 72 bpm; and body temperature, 37.1 °C. Physical examination detected no significant findings. On external (body surface) and internal examinations, no palpable masses or tenderness were noted in the left pelvic area. Transvaginal ultrasound revealed an atrophied uterus; however, the bilateral adnexa could not be identified due to atrophy. The hematoma in the left pelvis could be identified. These findings were identical to those obtained by CT. Blood test results were a hemoglobin value of 9.5 g/dL, a hematocrit value of 28.4%, a white blood cell count of 7970/μL, and a C-reactive protein level of 0.05 mg/dL. Urinalysis revealed mild occult blood. After admission, the hematoma did not enlarge, and the hemoglobin and hematocrit levels did not decrease. After 11 days of bed rest, with no exacerbation of the inflammatory response, lower abdominal pain was relieved, and she was discharged. After discharge, we planned to investigate the cause of her bleeding on an outpatient basis, but 6 days after discharge, she was readmitted to our hospital after relapse of symptoms caused by retroperitoneal bleeding from the same site. The hematoma had grown to a size of 11.7 cm, and magnetic resonance imaging showed an abnormal vascular network extending from the left side of the uterus to the left adnexal area. Three-dimensional CT (3D-CT) similarly showed an abnormal vascular network on the left side of the uterus, and we suspected a possible arteriovenous malformation (AVM) or arteriovenous fistula (AVF) from the uterine artery to the left ovarian vein  but flowed back into the LOV Fig. a. Subseqxus Fig. d. CT on xus Fig. . The patIn this case, angiography confirmed the continuity of LRV and IVC but demonstrated no antegrade blood flow to the IVC. Therefore, we attributed LOV regurgitation to extravascular physical compression of LRV. After close examination of the CT images, we determined that LOV rupture was caused by NCS because the LRV was severely stenotic between the abdominal aorta and the SMA Fig. .Fig. 5CoThis case was an emergency of retroperitoneal hemorrhage with a background of NCS, but it was necessary to rule out various diseases before we could establish the final diagnosis. The referring physician diagnosed her condition as torsion of a left ovarian cyst, and we observed bleeding from what we thought was an AVM or AVF in her pelvis at her first visit. Additional diagnostic imaging and angiography finally demonstrated left ovarian venous regurgitation and rupture. These studies were extensive because of our lack of knowledge of NCS. NCS is not a well-known disease, and it is observed mainly by pediatricians, urologists, vascular surgeons, and radiologists; it is less familiar to gynecologists. In recent years, PCS has begun to attract attention in the field of gynecology as a cause of chronic pelvic pain in women. NCS can be part of the cause of PCS, but alone it is not the same condition . MoreoveSpontaneous rupture of the ovarian vein was, in one case, caused by an abnormal vascular network associated with liver cirrhosis in a patient with a history of ruptured esophageal varices , and in NCS diagnosis is confirmed mainly by image evaluation, but no unified diagnostic standard exists , 2, 11. The prevalence of the nutcracker phenomenon ranges from 4.1% to 7.8% among healthy individuals, and of these cases, 8.8% or fewer are symptomatic NCS , 11. YouTo treat NCS, the timing and method of intervention must be individualized. For young patients, conservative treatment with monitoring for changes is the first choice, and for mild symptoms, follow-up monitoring for weight gain is the first choice. Although no clear criteria have been established, surgical and endovascular treatment can be used for severe symptoms , 4, 13. To our knowledge, spontaneous rupture of the LOV caused by NCS has not been reported in the past; this case is the first report. Because NCS is a disease that has not yet been fully characterized, it should be considered in the differential diagnosis of bleeding emergencies. Knowledge of NCS may be helpful in the appropriate and prompt treatment if an abnormal vascular network in the pelvis is found, especially in bleeding emergencies, such as this case.


Glioblastoma (GBM) is the most common primary intracranial tumor and originates from the small pool of adult neural stem and progenitor cells (NSPCs). According to the World Health Organization (WHO) classification of brain tumors, gliomas are classified into grades I–IV, and GBM is defined as the highest grade (IV). GBM can be disseminated by cerebrospinal fluid (CSF), but extracranial metastasis is rare. Additionally, the pathway and mechanism involved remain unclear.We report a rare case of left temporal lobe GBM with multiple bone metastases and soft tissue metastasis. This 49-year-old right-handed man who was diagnosed with GBM underwent surgery on May 9, 2017, followed by radiochemotherapy in June 2017. On August 13, 2019, local relapse was found. Then, the patient received a second surgery but not radiochemotherapy. In November 2019, the patient was reported to be suffering from low back pain for nearly 1 month. On December 6, 2019, magnetic resonance imaging (MRI) of the thoracolumbar vertebrae and abdominal computed tomography (CT) confirmed metastases on the ninth posterior rib on the right, the third anterior rib on the left, and the T7 and T10 vertebrae and their appendages. CT-guided rib space-occupying puncture biopsy was performed, and GBM was identified by pathology.We should pay attention to extracranial metastasis of GBM. Timely detection and early treatment improve overall quality of patients’ life. The extracranial metastasis in this patient may have occurred through the spinal nerve root or intercostal nerve. Further clinical observations are required to clarify the pathway and mechanism involved. Malignant brain tumors are among the most feared types of cancer, not only because of their poor prognosis but also because of their direct repercussions on quality of life and cognitive function. Glioblastoma (GBM) is the most notorious intracranial tumor in adults ; after s2/day) as an adjunct to radiotherapy and then sequentially  between June 5, 2017, and December 9, 2017. There were no obvious signs of recurrence or metastasis on craniocerebral MRI every 3 months after the operation.On first admission (May 2017), a 47-year-old man presented with a 3-month history of headache, nausea, vomiting, and memory loss. On May 7, 2017, craniocerebral magnetic resonance imaging (MRI) revealed space-occupying lesions in the left temporal lobe Figure 1On second admission , MRI demonstrated recurrence of the tumor in the postoperative residual cavity Figure 32/day). Oral analgesics were recommended during treatment from December 2019 to January 2020. At the end of treatment, the patient’s symptoms were slightly relieved, but there was still pain, with an NRS score of 2. The patient did not come for further consultation for personal reasons. According to a telephone follow-up, the patient’s pain worsened again in August 2020, and he received analgesic treatment at a local hospital. He eventually developed cachexia and died of systemic failure in December 2020. The progression-free survival (PFS) period was 27 months, and the overall survival (OS) period was 43 months.On third admission (November 2019), the patient presented with a 1-month history of back pain affecting sleep and aggravating in the recumbent position, with a numerical rating scale (NRS) score for the first 24 h of 4. On December 4, 2019, he underwent brain MRI. The intracranial conditions are shown in via neurosurgical management  tumors and 47.7% of all primary malignant CNS tumors . Surgerynagement . GBM hasnagement , 16, andnagement .via the movement of CSF through shunts to the peritoneum or pleura (via the CSF, lymph drainage that ends in the lymph nodes or blood, and invasive procedures increase the access of tumor cells to metastasize (Although GBM cells can pass through the BBB and be disseminated throughout the peripheral blood , extracrr pleura . Previour pleura , 28. Addastasize . Howeverastasize . Later sastasize . Malte Mastasize . GBM metastasize .via multiple possible pathways, including those described above. However, interestingly, the two metastases that were seen on imaging in December 2019 and the soft tissue metastasis in the ninth posterior costal ribs and T10 metastasis (Approximately 10.5% of extracranial metastases of GBM involve multiple metastases, with or without intracranial recurrence . The mostastasis Figure 6tastasis , so the The survival time of GBM patients is related to many factors. Research has shown that GBM with extracranial metastases most often involves the temporal lobe, either coincidentally or factually . For IDHAlthough the number of reports of GBM metastases has increased over time, the pathogenesis of such metastases remains elusive, and this diagnosis remains rare , 46. OurThe original contributions presented in the study are included in the article/supplementary material. Further inquiries can be directed to the corresponding authors.The studies involving human participants were reviewed and approved by the Ethics Committee of Weifang People’s Hospital. Written informed consent for participation was not required for this study in accordance with the national legislation and the institutional requirements.WZ, YYC, and FRH performed the chemoradiotherapy in this case. WZ, XLW, and XXW drafted the manuscript and performed the literature review. YL and YJC retrieved the clinical and the image information, GYH and YXZ retrieved and analyzed the pathological information. FRH and YXZ conceived, designed, and supervised this study. The paper properly credits the meaningful contributions of the coauthors and coresearchers. All authors have been personally and actively involved in substantial work leading to the publication of paper and take public responsibility for its content. All authors contributed to the article and approved the submitted version.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article, or claim that may be made by its manufacturer, is not guaranteed or endorsed by the publisher.


Corneal dermoids are a rare cause of corneal opacification, consisting of abnormal mesoblastic tissue surrounded by epithelium. Here, we describe the case of a 1-year-old child who had a congenital corneal dermoid in the left eye since birth; thus, the patient underwent tumor excision followed by keratoplasty.A 1-year-old girl was brought to the hospital by her parents, who had been noticing a mass on the surface of her left eyeball since birth. The patient had no other previous or concurrent disease nor family history for dermoids.No abnormalities were present in the cornea and the anterior and posterior segments of the right eye. Eye movement, intraocular pressure, and the position of the upper eyelid of the left eye were normal. No signs of conjunctival hyperemia were present. The tumor presented as a yellowish-pink mass with hair and veins on the surface.The patient was initially diagnosed with a keratoconjunctival tumor of the left eye by a clinical doctor.Corneal tumor resection combined with keratoplasty was performed in the patient. Eye drops with 1% cyclosporine were administered 3 times per day to prevent immune rejection.Based on postoperative pathological examinations, the final diagnosis was a corneal dermoid. The patient had an uneventful healing process and rapid corneal re-epithelization. The ocular surface was stable during the follow-up visits, and no complications emerged.We report a rare case of congenital corneal dermoid. We learned that close follow-up is needed after surgery in such cases. The lesion generally invades the superficial layer of the cornea, though occasionally involves the entire corneal thickness and the anterior chamber. Different surgical techniques for dermoid removal have been described in the past decades, including bare excision, amniotic membrane transplantation, and lamellar or penetrating keratoplasty.,2Corneal dermoids are congenital benign tumors that usually manifest in the subtemporal quadrant of the eye. They present as pale or yellow semi-circular masses with a keratinized surface and occasionally with hair follicles and cilia. Associated ocular abnormalities include eyelid colobomata, lacrimal anomalies, and scleral and corneal staphylomata.Keratoplasty is used to improve sight, relieve pain, and treat severe infection or damage. The choice of treatment depends on the tumor grading system based on the regions involved, as described by Mann.The treatment of corneal dermoids is particularly challenging in young children because of several concerns, such as the tendency for low visual acuity and astigmatism-related amblyopia in this population. Recently, a young child with a congenital corneal dermoid was admitted to our department. The manifestation, treatment, and prognosis of this patient are reported below.2The patient was a 1-year-old girl. Her parents had noticed a mass on the surface of the left eyeball that had been present since birth. The patient had no history of other diseases, no relevant family history, and no genetic diseases.3Physical and eye examinations were performed when the patient was admitted to the hospital. Eye movement and intraocular pressure in both eyes were normal. The upper left eyelid was in the correct position; mass compression signs were apparent at the eyelid margin, and the mass was slightly exposed when the eyelids were closed. No evident conjunctival hyperemia was noted; however, a small round mass was visible in the 4 to 5 o’clock direction of the temporal cornea. The tumor size was approximately 7 mm × 5 mm × 5 mm, and the appearance was yellowish pink. Hair and veins were also observed on the mass surface. The tumor had clear boundaries, and the conjunctiva was invaded. The remaining cornea was transparent, with no apparent abnormalities in the anterior segment Fig. A. The opDue to the young patient's age, the surgery required general anesthesia, and standard preoperative tests were performed. Orthogonal radiographic chest examination showed that the lungs had a clear texture and no abnormalities. There were also no abnormalities on routine blood examinations, virus screening, or tests for blood clotting disorders.4The patient was diagnosed with a keratoconjunctival tumor of the left eye by a clinical doctor based on the appearance and location of the mass.5Before the operation, the treatment methods, postoperative precautions, and prognosis were discussed with the patient's parents, and consent was obtained before the operation. Corneal tumor resection combined with keratoplasty was performed under general anesthesia. The superior rectus muscle was fixed using a 5 to 0 nylon wire. The bulbar conjunctiva surrounding the mass was cut to expose the tumoral tissue completely. The tumor and turbid cornea were removed, and a lamellar keratectomy was performed on 1/3 of the thickness of the cornea.During the operation, a trephine, with a diameter 0.25 mm larger than the implantation bed, was used to drill the corneal graft at the appropriate depth; micro scissors were used to trim the edge and thickness of the graft to fit the implantation bed. The graft was carefully spread onto the implantation bed. The corneal and sclerotic sides were diagonally sutured with 10 to 0 nylon sutures, and the whole graft circumference was intermittently and evenly sutured. At the end of the operation, the corneal graft was smooth and fitted adequately in the implantation bed  occur at the inferotemporal bulbar location. However, these masses may occasionally be present entirely within the perilimbal cornea or confined to the conjunctiva and sclera. Limbal dermoids mainly include 4 pathological types: dermoids, lipodermoids, complex choristomas, and epibulbar osseous choristomas. Most of these tumors manifest as dermoids containing pilosebaceous structures, stratified squamous epithelium, and choristomatous tissue, a dense connective tissue similar to the skin corium.Corneal dermoids are unusual congenital anomalies formed by residual primitive tissues. These formations are the most common congenital orbital lesions and account for 25% of invasive orbital growths and 75% of cystic lesions. Among grade I tumors, the most frequent are the limbal or epibulbar dermoid types. Grade I tumors are less than 5 mm in size and are usually located in the inferior temporal quadrant of the corneal limbus. They present as a round, flattened, yellowish-pink mass not affecting the entire cornea. Currently, the standard medical approach for grade I disease is observation. Grade II tumors comprise large limbal dermoids that can affect the corneal stroma or Descemet's membrane. Most patients diagnosed with these tumors have difficulties in closing their eyes. Grade III tumors are the rarest and most serious type. They may affect the entire corneal tissue, damage the anterior chamber structure, and occasionally invade the lens.Corneal dermoids are classified into 3 major grades based on their size, anatomical location, and the severity of symptoms they cause. Furthermore, approximately 30% of patients with corneal dermoids have other congenital abnormalities, the most common being Goldenhar–Gorlin syndrome.Overall, corneal dermoids may cause visual abnormalities by the gradual development of corneal astigmatism, caused by encroachment in the visual axis, and by corneal infiltration with fatty components. These abnormalities may result in anisometropic amblyopia. The invaded angular axis of the dermoid is the most significant factor associated with postoperative visual acuity, amblyopia development, and postoperative scarring.; thus, stem cell transplantation can be performed to minimize the incidence of postoperative defects. In fact, in a recent study, researchers performed amniotic membrane transplantation to remove limbal dermoids and obtained promising postoperative results. Autologous limbal stem cell transplantation has also been successfully implemented in removing corneal dermoids in 2 patients without any of the complications above and no visual loss. Therefore, at present, the primary treatment for this disease is surgical resection combined with corneal or stem cell transplantation. For example, surgical approaches for grade II and III limbal dermoids include excision, lamellar or penetrating keratoplasty, and amniotic membrane transplantation.–11 Customized preoperative treatment plans are also available and have been proposed depending on the characteristics of the lesion and the extent of invasion.Due to their location on the eye surface, corneal dermoids can be easily detected early; thus, most patients can receive timely diagnosis and treatment. Removal of corneal dermoids can lead to persistent epithelial defects, corneal vascularization, and scar formation. All these complications are believed to occur because of focal marginal limbal stem cell deficiency at the site of dermoid excisionIn conclusion, we reported the rare case of a young patient diagnosed with congenital corneal dermoid. A prompt surgery was performed, resulting in a positive outcome; this case helps us acquire increased knowledge of this disease. Complications such as corneal scarring, neovascularization, pseudopterygium, and eyelid adhesion are common with simple surgical excision. Therefore, we chose to perform tumor resection combined with lamellar keratoplasty for this patient. However, such a procedure is not devoid of postoperative complications, including corneal astigmatism, rejection, and implantation bed penetration. Therefore, we will follow-up with this patient with a complete optical inspection when the child's visual system is fully developed. Refractive correction should be performed to avoid amblyopia if the astigmatism is severe.Data curation: Yanyan Cui, Shan Yin, Bojun Zhao.Formal analysis: Yanyan Cui, Shan Yin.Investigation: Yanyan Cui.Resources: Xuewei Yin.Software: Yanyan Cui, Shan Yin, Xuewei Yin.Supervision: Xuewei Yin, Yonghui Liu.Visualization: Yonghui Liu, Bojun Zhao.Writing – original draft: Yanyan Cui.Writing – review & editing: Yanyan Cui, Bojun Zhao.


Neoplasms of the urachus are exceedingly rare, representing 0.17% of all bladder cancers. The mucinous cystic tumor of low malignant potential (MCTLMP) subtype is particularly rare with just 25 previous cases reported in the literature. Although rare, MCTLMPs are important to identify due to potential devastating complications and good cure rates with surgical removal. We present a 43 year old female with a nuanced constellation of comorbidities and confirmed MCTLMP following a workup for abdominal pain and irritative lower urinary tract symptoms. Notably, this tumor did not change in size over a 3-year course of serial imaging prior to surgical excision. This urachal MCTLMP represents roughly the 26th and one of the smallest of its subtype reported in the literature. This case illustrates the diagnosis and management of this rare urachal MCTLMP. Individual patient medical history, clinical considerations, and neoplasm characteristics are examined. Although rare, the potential for increased malignancy and potential complications necessitates surgical management and further investigation by the academic community. The urachus is an embryologic remnant of the allantois, which connects the anterior dome of the bladder to the umbilicus and rarely persists after early infancy. In the event of incomplete atresia, the urachus has the potential to transform into numerous pathologies including fistula, diverticulum, or tumor. Among the potential pathologies of urachal remnants, urachal neoplasms are exceedingly rare and are of significant interest due to their potentially aggressive invasive potential. Invasive mucinous cystadenocarcinomas have a poor prognosis with only 45% 5 year survival rates. , 2Isolated cases of urachal neoplasms have presented as pseudomyxoma peritonei and mucosal urinary secretion. Symptomatic presentation varies significantly, but most manifestations include some form of nonspecific abdominal pain . SurgeryThe inner wall of the urachus is lined by epithelium, which may transform into non-glandular, glandular, or mixed neoplasms . GlandulA 43 year-old female with a history of newborn gastroschisis, appendectomy, cholecystectomy, colectomy, Roux-en-Y Gastric Bypass, and multiple psychiatric diagnoses had a CT abdomen/pelvis with contrast that incidentally reported a small diverticulum near the bladder dome. A subsequent CT of the abdomen and pelvis without IV contrast 2 years later once again identified the lesion unchanged in size, although suggested a small bladder saccule, diverticulum, or urachal remnant as possible etiologies. Repeat imaging reports failed to identify this abnormality or found it to be stable. Shortly after, the patient was referred to Urology by her primary care physician due to persistent idiopathic lower abdominal pain following an extensive workup. Symptoms at time of urologic consultation included diffuse low- to-mid abdominal pain with associated nausea, vomiting, anorexia, dysuria, and irritative lower urinary tract symptoms.Cystoscopic evaluation demonstrated a saccular cystic lesion consistent with a urachal remnant cyst with associated tenderness upon compression. 3 years after initial detection on imaging, an open partial cystectomy for excision of the suspicious region was offered as robotic operation was excluded due to prior abdominal surgical history. Intraoperatively, the urachal remnant was palpable and grossly visible on the anterior bladder wall. Abdominal adhesions were extensive as expected from her significant past abdominal surgical history. A partial cystectomy at the dome of the bladder was performed with careful removal of the involved tissue and without any major intraoperative complications.Gross examination of the excised specimen revealed a 3.5 cm area of nodularity believed to represent the actual size of the tumor. Histopathological analysis revealed cystic structures lined by mucinous epithelium with focal areas exhibiting a pseudostratified cyst lining and at least mild-to-moderate nuclear atypia, consistent with a mucinous cystadenoma of low malignant potential of the urachus Figs. , 2. HistUnfortunately, as of roughly two months status-post surgical excision, the patient has reported no symptomatic relief in the symptoms she believed were caused by the excised urachal remnant.Interestingly, this patient’s history of newborn gastroschisis necessitates consideration of a connection with her subsequent MCTLP. It is unclear if any of the cases in the literature had a known history of gastroschisis. Although the specific pathogenesis of gastroschisis is unclear, hypotheses such as mesenchymal failures, amniotic rupture, persistence of umbilical veins, and vitelline artery disruption should be considered in the conversation of urachal transformation and carcinogenesis. With relation to the urachus, some hypotheses suggest a connection to ventral folding, ventral structures, and the yolk sac . Among tFurthermore, this patient’s neoplasm was sized at 1.9 × 2 cm on imaging and 3.5 cm on pathological analysis, differing greatly from reported average size of published mucinous urachal cysts of low malignant potential is 5.4 cm (2.0–14 cm)  analysis was not completed on initial or confirmatory analyses as it does not appear to add any diagnostic value for urachal neoplasms . A studyGiven the potential for complications, growth, and increased malignancy, surgical management of mucinous urachal neoplasms is necessary. MCTLMP should be surgically excised with partial cystectomy followed by periodic imaging and cystoscopy to monitor for recurrence. Future cases concerning for urachal neoplasm should include gross imaging and cystoscopy imaging to contribute to the medical literature, improve characterization of these masses, and improve our overall understanding of these neoplasms. Although data is limited, it appears that the prognosis of MCTLMP is excellent following adequate surgical removal.This case illustrates the diagnosis and management of this rare urachal MCTLMP. Individual patient medical history, clinical considerations, and neoplasm characteristics are examined. Although rare, the potential for increased malignancy and potential complications necessitates surgical management and further investigation by the academic community.


Echinococcus multilocularis), determined that she had hepatic alveolar echinococcosis (AE). The patient underwent surgical resection, and hepatic AE was confirmed by a histopathological examination. A 10-year-old girl had a history of epigastric pain for three months. A laboratory investigation revealed mild leukocytosis with eosinophilia. Abdominal gray-scale ultrasonography showed a heterogeneous mass lesion in the left lobe of the liver. The mass was generally hypoechoic; however, it contained cystic components and hyperechoic foci, representing calcifications . MultideE. multilocularis. The correct identification of hepatic AE allows for appropriate treatment. Abdominal ultrasonography is the first-line imaging technique for the evaluation of patients in whom the presence of AE is suspected. MDCT and magnetic resonance imaging techniques could be performed for a preoperative evaluation. Liver lesions are seen as heterogeneous cystic-necrotic masses with calcifications on ultrasonography and MDCT. Hepatic AE lesions usually have irregular margins, scattered calcifications, central necrosis, and no significant enhancement in MDCT images,AE is caused by infection with the larvae of


Unexpected bone lesions of the skull present a common dilemma, where radiological appearance and patient’s clinical background are crucial to avoid misdiagnosis.Enostosis are benign sclerotic bone lesions; its aetiology is still unknown and its management is usually conservative, with good prognosis. Most of these lesions are asymptomatic and neurological involvement is rare. We present the first report of enostosis-related epilepsy. A 64-year-old male was referred to our emergency department (ED) presenting with altered state of consciousness. He was found unconscious by his daughter at home who also described a “generalized shaking”. She denied signs of trauma, sphincter incontinence and confirmed that he was fine the day before. By the time the patient got to the hospital, he spontaneously recovered and had no memories of the event. There was no history of previous events, drug or alcohol abuse. His past medical history included arterial hypertension with long-term use of perindopril 5 mg bid.−1 for a reference range of <145 U l−1), with normal renal and hepatic function tests as well as preserved complete blood count. During his time in ED, he had a brain CT that detected a tubular 20.8 × 14.5 mm enostosic formation in the right temporal fossa with resulting mass effect on the adjacent temporal lobe  was performed that detected generalized epileptiform activity derived from right temporal lobe .The patient recovered after treatment with levetiracetam and was then observed by neurosurgery who decided to evaluate the case later on. The patient was discharged with long term use of levetiracetam 500 mg bid and is pending final decision on whether surgical treatment should be performed.1 The pathogenesis is uncertain, but it is thought to be congenital or developmental in nature, where self-limiting osteoclastic dysfunction plays the main role during bone remodelling.1 Its real prevalence remains unknown; it seems to appear in childhood, equally affecting both genders.2 Proximal long bones and pelvis are most affected; they are rarely seen associated with cranial bones.1,2Enostosis represents a slow-growing benign and highly mineralized bone lesion derived from cancellous bone.3The radiological appearance on radiograph, CT or MRI could be seen as small round and dense bone within the medullary space, sometimes with classic radiating spicules at the margins that blend with the surrounding trabeculae.4,5The main differential diagnosis consists of osteoid osteoma, osteosarcoma and osteoblastic metastasis.6The management of these lesions is usually conservative and reveals good prognosis.6 Surgical removal could be necessary in cases of refractory and function-limiting lesions, due to compression of surrounding structures.1,6 There is insufficient data regarding neurological involvement and this is to our knowledge the first reported case of enostosis-related epilepsy.Enostosis are mainly an incidentaloma; if present, symptoms are associated with their anatomic location.Enostosis is often incidental, they represent slow growing bone lesions and most of related symptoms are due to involvement of surrounding structures.Neurological involvement is rare. Those with disabling lesions require surgical treatment.


The clinical presentation of right iliac fossa pain, anorexia, and vomiting are the classic clinical features of acute appendicitis. However, a broad spectrum of manifestations may result in a similar clinical picture, including gastrointestinal, genitourinary, and gynecologic pathologies. Imaging studies are crucial to establishing the diagnosis. Here, we report the case of a 58-year-old man who presented to the emergency department with a one-week history of right lower quadrant abdominal pain. The pain was associated with nausea, vomiting, and frequent bowel motions. There was no history of fever or weight loss. The examination of the abdomen showed localized tenderness and guarding in the right iliac fossa. The basic laboratory investigation was within the reference range. The computed tomography scan demonstrated a well-circumscribed intraluminal mass lesion in the ascending colon with no evidence of complete obstruction. The mass was slightly heterogeneous but had fat attenuation. There was no evidence of invasion. There was no stranding of the adjacent fat. The radiological findings were consistent with colonic lipoma. The patient underwent laparoscopic surgery and had a segmental resection of the tumor with primary anastomosis. The appendix was also resected. Histopathological examination showed mature adipose cells along with thin-walled, capillary-sized vessels representing a benign angiolipoma. Further, the resected appendix was completely normal and showed no evidence of acute inflammation. Colonic angiolipoma is an extremely rare tumor. This case demonstrated that a large angiolipoma of the ascending colon may show a presenting clinical picture similar to that of acute appendicitis. Complete resection of the tumor is associated with an excellent outcome. Acute appendicitis is one the most frequent causes of acute abdomen and is considered one of the major indications for emergency abdominal surgeries worldwide. The classic clinical features of acute appendicitis include right lower quadrant abdominal pain, anorexia, and nausea and vomiting . HoweverA 58-year-old man presented to the emergency department with a one-week history of right lower quadrant abdominal pain. He reported that the pain had started in the periumbilical area and shifted to the current location. It had a gradual onset and had been increasing in severity. The pain was sharp in nature and was not related to food intake or posture. He attempted over-the-counter analgesic and antacid medications with no significant clinical improvement. He scored the pain as 7 out of 10 in severity and reported that had it started to disrupt his sleep. The pain was associated with nausea, vomiting, and frequent bowel motions. There was no history of fever or weight loss.The patient had a longstanding history of metabolic syndrome with hypertension, diabetes mellitus, and dyslipidemia. He had stable angina that was well-controlled with medications. He was diagnosed as having gastroesophageal reflux disease seven years ago. His medications included metformin 500 mg, captopril 25 mg, metoprolol 25 mg, atorvastatin 20 mg, and omeprazole 40 mg. He reported good compliance with his medications, and his allergy history was unremarkable. He had not undergone any surgeries. He worked as a taxi driver and had a smoking history of 18 packs a year. He consumed alcohol on a few occasions only. His family history was remarkable for colon cancer.Upon examination, the patient appeared in discomfort. His vital signs were within the normal limits with a heart rate of 88 beats per minute, a temperature of 37.0°C, a respiratory rate of 12 breaths per minute, and blood pressure of 122/84 mmHg. The examination of the abdomen showed localized tenderness and guarding in the right iliac fossa. However, there were no signs of generalized peritonitis. Bowel sounds were present with normal intensity. Digital rectal examination was normal. Other systems showed normal examination findings.The basic laboratory investigation was within the reference range. In particular, leukocytes, erythrocyte sedimentation rate, and C-reactive protein levels were normal. The liver enzymes, electrolytes, and blood urea nitrogen were also normal Table . The iniThe patient underwent laparoscopic surgery for further evaluation and management. During exploration, the previously seen mass lesion was identified. The appendix had a normal appearance with no congestion to suggest acute appendicitis. Segmental resection of the tumor was performed with primary anastomosis. The appendix was also resected. The recovery of the patient was uneventful. Postoperatively, he reported improvement in his pain. He started oral feeding on the second postoperative day and was tolerating it well. He was discharged on the sixth postoperative day. Histopathological examination showed mature adipose cells along with thin-walled, capillary-sized vessels representing a benign angiolipoma Figure . FurtherWe reported the case of a colonic angiolipoma that had a classic presentation of acute appendicitis. Angiolipoma is a rare benign tumor of mature adipose tissue and blood vessels. It typically occurs in the subcutaneous tissue in the trunk and upper extremities in young adults and presents as a tender small nodule. However, colonic angiolipoma is an extremely rare entity with few reported cases in the literature -10. ThisA recent review of the literature by Kimura et al.  in 2021 The diagnosis of angiolipoma cannot be made confidently by imaging studies. In our case, the radiological diagnosis was a lipoma. However, it has been reported that on magnetic resonance imaging the tumor shows the signal intensity of adipose tissue along with low T1 signal intensity and high T2 signal intensity in the vascular element that exhibits vivid contrast enhancement . The accRegarding the management of colonic angiolipoma, the method of surgical resection largely depends on the location of the tumor, its type, and size. Endoscopic resection can be performed for small-sized pedunculated tumors . HoweverColonic angiolipoma is an extremely rare tumor. Our case demonstrated that a large angiolipoma of the ascending colon may show a presenting clinical picture similar to that of acute appendicitis. A CT scan may suggest the benign lipomatous nature of the lesion, but the accurate diagnosis of angiolipoma is often made by histopathology. Complete resection of the tumor is associated with an excellent prognosis which can be achieved by laparoscopic procedures.


Case Report. We report the first case of a successful vaginal delivery after induction of labour in a woman recovered from a primary large B-cell lymphoma of the cervix and a literature review.  Primary non-Hodgkin's lymphomas of the cervix are rare; they represent about 1% of all cases. There are no available guidelines regarding the safest mode of delivery after treatment and resolution of a cervical lymphoma.  In carefully selected patients with fully treated non-Hodgkin's lymphoma of the cervix with no residual disease, induction of labour via prostaglandins pessary may be a safe option if indicated. Primary non-Hodgkin's lymphomas of the cervix are a rare finding, representing about 1% of all cases of non-Hodgkin's lymphoma , 3. Ther3/μl. The last smear test, performed 24 months before, was negative for neoplasia. At the transvaginal evaluation, the cervix appeared to be irregular due to the presence of a 5 cm diameter mass. The patient underwent hysteroscopy, with a fractional curettage and cervical biopsies. Endometrial and endocervical curettage revealed benign glandular tissue, but cervical biopsies demonstrated a diffuse large B-cell lymphoma (LBCL). The immunohistochemical evaluation of the cervical lesion biopsy was positive for B-cell markers , as shown in A 30-year-old nulliparous Caucasian woman presented with a history of dysfunctional uterine bleeding lasting for six months. The patient did not report any past or present significant medical history. Blood analysis was within the normal range, with white blood cells 6000 U/L, haemoglobin concentration 13 g/dL, haematocrit of 33%, and platelet count 165 × 10Both a computed tomography (CT) and a magnetic resonance image (MRI) demonstrated the presence of a cervical mass without extra pelvic lesions. A positron emission tomography (PET) revealed mild uptake of the radioactive tracer at the low uterus level. Following completion of staging studies, the woman was referred to the oncology team, who staged the patient's tumour as IE-B, one extra lymphatic site without fever, night sweats, or weight loss (Ann Arbor staging system). Eight cycle courses of chemotherapy and immunotherapy were recommended. The treatment protocol was based on the combination of: CHOP+Rituximab . Before chemotherapy, depot Leuprolide 11.25 mg intramuscular injection was given every 12 weeks to preserve future fertility against potential chemotherapy's side effects. Six months after completion of medical treatment, a transvaginal ultrasound revealed resolution of the cervical lesion, and follow-up cervical biopsies did not reveal residual disease. PET, CT, and MRI revealed a complete resolution of the cervical lesion. Pre and posttreatment MRI are available in Primary non-Hodgkin's lymphomas of the cervical stroma are localized at the level of the cervix without any involvement of the myometrium or evidence for leukaemia at the time of diagnosis .A primary cervical involvement by a lymphoma is a rare finding. Among subtypes, large B-cell is the most common non-Hodgkin's lymphoma of the cervix, representing approximately 31% of cases . AffecteManagement with chemotherapy and immunotherapy reduces the need for radiotherapy or surgical resection, particularly for women during childbirth age , 15. CurThere are no available guidelines regarding the safest mode of delivery after treatment for a cervical B-cell lymphoma. There is occurrence of potential complications such as pelvic outlet obstruction or potential lower uterine segment rupture  –8. IndeeIn carefully selected patients with fully treated non-Hodgkin's lymphoma of the lower uterine segment and cervix with no apparent disease, induction of labour via prostaglandins pessary may be a safe option in case of indication.


Acute appendicitis is a very common event. Migration of hernia mesh is rare, especially intraluminal migrations. We aim to report a case of a migrated inguinal mesh presenting as an acute appendicitis. A 58-year-old male previously submitted to ONSTEP right inguinal hernia repair with a PolySoft™ hernia patch eight years before, was admitted in the emergency department with acute appendicitis, and submitted to laparoscopic appendectomy. Intraoperatively, the “recoil ring” from the inguinal hernia patch was extended from the anterior abdominal wall to the appendix, perforating it and progressing intraluminally. Appendectomy was performed, as well as removal of the mesh by an anterior approach. Hernia mesh migration to an intraluminally position is extremely rare with only a few cases described in literature. Pathogenesis of migration is still poorly understood. Clinicians should consider hernia mesh migration in their differential diagnosis for causes of acute appendicitis, in the right clinical setting, when a previous hernia defect correction is present. To the best of our knowledge, this is the first reported case of inguinal hernia mesh migration to the appendix, presenting as acute appendicitis. Acute appendicitis (AA) is one of the most common conditions presenting at the emergency department in daily practice. Prompt diagnosis is mandatory and, in the majority of the cases a surgical procedure is required. Although several causes of AA are possible, it is not always clear why certain patients develop this condition  and someWe report a case of a migrated inguinal mesh presenting as AA. This report has received approval by the local Ethics Committee.A 58-year-old male was admitted in the emergency department of our Institution complaining of diffuse, intense and worsening abdominal pain migrating to the right iliac fossa, with eight hours of duration. The patient denied nausea, vomiting, or altered bowel movements. The patient had a relevant medical history of hypertension (medicated with Perindopril 4 mg 1id). He had been previously submitted to ONSTEP right inguinal hernia repair with a PolySoft™ hernia patch  eight yeOn physical examination the patient presented an auricular temperature of 37.8°C and was hemodynamically normal. He experienced pain in the right inferior quadrant of the abdomen on palpation, with a Blumberg and Rovsing positive signs. No hernias were noted. Blood tests showed elevated white blood count with increased neutrophils, along with elevated C reactive protein, without other relevant alterations. Urinalysis was unremarkable. A diagnosis of acute appendicitis was suspected, and an abdominal ultrasound was done, which revealed a luminal dilation of the appendix with 10 mm diameter, which was non-compressible, along with echogenic, prominent peri-appendiceal fat, and a thin layer of peri-appendiceal fluid, suggestive of acute appendicitis. Intravenous antibiotics and a subsequent laparoscopic appendectomy were proposed.Intraoperatively, the appendix was highly adherent to the anterior abdominal wall, reddish, dilated and with evidence of suppuration with small amount of free fluid. During the dissection, a foreign body was detected, clearly originating from the anterior abdominal wall. This foreign body, unequivocally perforated the appendix and progressed intraluminally. The material was noticed to be the recoil “ring” from the inguinal hernia patch previously applied to the patient for treating his inguinal hernia . AppendeHistologically, acute appendicitis with extensive serositis was confirmed. The drain was removed at day two post-operatively, the post-operative course was unremarkable and the patient was discharged three days after the procedure.Surgical repair of abdominal wall hernia is amongst the most common surgical procedures performed worldwide in Surgical Departments, with around 20,000,000 groin hernia repairs performed annually . DespiteAs mesh migration can occur with a wide variety of organs involved, including colon, small bowel or bladder, clinical presentation of these patients varies according to the site affected . To the The event of migrating hernia mesh is still not completely understood. Literature suggests that mesh fixation may interfere with this event but no sufficient and reliable data is available that allows a strong conclusion. On the other hand, mesh fixation may related to postoperative pain and nerve entrapment. For this reason it is still not clear what the best approach is to avoid this complication.Removal of the mesh was challenging. The presence of suppuration in the abdominal wall determined the option to go for an anterior approach to the right inguinal area, leading to a complete removal of the prothesis. The patient is currently in follow-up for prompt identification of hernia recurrence, but after 2 years has not shown evidence of this.With the increasing popularity of mesh repair of abdominal hernias, even though mesh migration is an uncommon complication, we could expect for an increasing number of patients presenting with such scenario. Given the non-specific symptoms and wide array of possible clinical presentations, the key for early identification of this potentially severe complication is simply the awareness of its existence.Hence, clinicians should consider this in their differential diagnosis for causes of AA, in the right clinical setting, when a previous hernia defect correction is present.