Reversed rotation of the midgut is the rarest variation of midgut malrotations, which are congenital disorders that result from aberrant rotation and fixation of the midgut during embryological development. Common complications of these disorders are small bowel obstruction by volvulus or peritoneal bands, usually occurring in early infancy.A 23-year-old Caucasian woman presented with recurrent abdominal pain. A contrast-enhanced multidetector computed tomography study revealed a novel variant of reversed rotation of the midgut. Besides the specific finding of a retro-arterial transverse colon, we also found the proximal jejunum to cross posterior to the mesenteric root, a variation that has not been reported in the literature so far. In this case, substantial symptomatic relief was achieved with conservative management.The hypothesis of a double reversed rotation of the pre-arterial segment of the umbilical loop around the superior mesenteric artery axis provides a possible explanation for this anomaly. There is no evidence-based consensus on the management of patients presenting with non-symptomatic or mildly symptomatic intestinal malrotations. In this case, radiologic and clinical presentations excluded acute small bowel obstruction, and surgical intervention was avoided. Intestinal malrotation is a rare congenital disorder, defined by an abnormal position of the bowel within the peritoneal cavity, which results from a failure in the normal course of midgut rotation and fixation during embryologic development. Depending on which stage of the physiological sequence of midgut development is stopped or disrupted, a variety of anatomic anomalies can occur, comprising non-rotation, incomplete rotation, mixed-rotation, and reversed rotation . Since tThe most common clinical manifestation of intestinal malrotation in neonates is an acute duodenal obstruction or midgut volvulus, a life-threatening condition in which the bowel and its accompanying mesenteric vessels twist around the short mesenteric pedicle resulting in bowel obstruction and ischemia. In adolescents and adult patients the incidence of volvulus declines  and clinIn this article, we present, to the best of our knowledge, the first published case of a novel variant of reversed rotation of the midgut as a cause for recurrent abdominal pain in a young adult.A 23-year-old Caucasian woman presented to our emergency department with abdominal pain and recurrent nausea of 6 days’ duration, which had progressively worsened over the past few hours, but without vomiting. The pain, she described, was rather diffuse but more intense in the epigastric region. She denied reflux, diarrhea, urinary symptoms, and fever. She reported that she had been having intermittent problems with diffuse abdominal pain throughout her adult life, but usually milder than this current episode.Regarding her medical history, she had suffered from a jejunal atresia in her left-upper abdomen which required surgical treatment in her neonatal period. It was initially treated with a Bishop–Koop side-to-side jejunojejunostomy with chimney. The stoma was reversed approximately 7 months later.A physical examination revealed a flat and soft abdomen with a big scar across her upper abdomen, without any evidence of a hernia. Abdominal palpation revealed a mild tenderness in her lower abdomen, particularly in the right lower quadrant. No abdominal masses were palpable. Bowel sounds were normal. A laboratory examination  revealed no abnormality.A contrast-enhanced computed tomography (CT) scan of her abdomen with rectal contrast enema was performed. The CT scan demonstrated a markedly altered anatomy of the midgut with a rather usual course of the hindgut Fig. –c. The nCT demonstrated no evidence of frank volvulus, bowel ischemia, or acute bowel obstruction. The patient was diagnosed as having a variant of reversed intestinal rotation.She was immediately started on intravenously administered fluids and analgesics (metamizole), which offered instant pain relief. The clinical findings did not indicate a need for immediate hospital admission or surgical intervention.In the normal sequence of intestinal development, the midgut, which extends from the entrance of the bile duct into the duodenum to the last third of the transverse colon, rotates 270 degrees counterclockwise around the axis of the SMA. This rotation occurs during the fourth through to the 12th week of gestation .Due to the rapid growth of the midgut, it initially extends into the extra-abdominal cavity and forms the umbilical loop, which is positioned sagittally. This process is known as physiological herniation of the midgut. Further growth of this umbilical loop is associated with a rotation of 90 degrees around the axis of the SMA in a counterclockwise direction, resulting in a horizontal position of the umbilical loop. The small intestine comes to lie to the right and the cecum to the left of the SMA. Between 8.5 and 9.0 weeks of development, the physiological umbilical hernia resolves as the abdominal cavity becomes sufficiently large. The loops of the small intestine return first from the umbilical stalk into the abdomen, while the cecum returns last, undergoing an additional 180 degrees counterclockwise rotation [Reversed intestinal rotation was first reported in 1883 by Tscherning . In 1923Our case differs from previously reported reversed rotations, due to the retro-arterial course of the proximal jejunum. This unusual anatomy may be explained by a variant of the retro-arterial subtype of reversed rotation. We suggest that the post-arterial segment returned first to the abdominal cavity undergoing a clockwise rotation, characteristic of reversed rotation. The following pre-arterial segment takes an additional turn of net 360 degrees in clockwise (reverse) direction, instead of the usual 90 degrees in reversed rotations, thereby resulting in a 360 degrees loop of the pre-arterial segment around the SMA axis Fig. . This vaDue to its extreme rarity, little is known about the pathophysiology of this condition and the exact incidence of complications is still difficult to determine. Reversed intestinal rotation has repeatedly been reported to be associated with impaired fixation and high mobility of the ascending colon and cecum, which is, therefore, prone to ileocecal volvulus –19. WithWhen intestinal malrotation is suspected, the most reliable radiologic sign is an intraperitoneal position of the D3-segment of the duodenum or, in other words, a retroperitoneal retromesenteric position of the D3-segment almost certainly excludes rotational anomalies of the midgut , 25. AnoThe therapeutic gold standard in the management of acute complications resulting from intestinal malrotations is a surgical approach . The staAs a matter of course, the choice of a surgical management is imperative when patients present with acute life-threatening complications. However, the dramatic rise of cross-sectional imaging and the incidental diagnosis of intestinal malrotations in asymptomatic or mildly symptomatic patients raise the question of adequate treatment in this patient group. The majority of pediatric surgeons recommend a surgical correction in any given case if intestinal malrotation is diagnosed –30. HoweWe present a novel variant of reversed midgut rotation, which may be explained by a double reversed rotation of the pre-arterial segment of the umbilical loop around the SMA axis. In this case, the radiologic and clinical presentations excluded acute small bowel obstruction, and extensive surgical intervention was avoided.


Pneumatosis Intestinalis (PI) can present with a broad range of presentations from chronic and non-specific to acute and life threatening. It is paradoxically one of the few conditions where a pneumoperitoneum found in the diagnostic workup is not necessarily an indication for laparotomy.The first case is of a 75 year old gentlemen who attended the emergency department after several weeks of worsening abdominal pain and weight loss. At laparotomy, two segments of nodular, abnormal-looking small bowel were identified with bubbles of air seen in the small bowel mesentery.The second case is of an 86 year old man with of a 5-month history of abdominal pain, weight loss, nausea and diarrhoea. A subsequent endoscopy revealed diffuse gastritis with a small antral gastric ulcer with a small amount of blood. The CT scan demonstrated free air within the bowel wall, with a follow up scan performed 3 weeks later revealing an increase in the amount of free air.PI is a rare condition characterised by the presence of subserosal and submucosal gas filled cysts occurring anywhere in the gastrointestinal tract from the oesophagus to the anus.Numerous conditions have been associated with PI including bowel obstruction, infections, ischemia and there have been reported cases suggesting as association with Crohn’s disease.PI is a condition that may manifest with a wide range of symptoms from mild abdominal pain to acute peritonitis. Its treatment is generally medical and even with radiological evidence of perforation laparotomy may not be indicated if the patient is clinically well. Its treatment depends on the acuity of the presentation and any underlying conditions and may present with a “benign pneumoperitoneum” not necessitating surgery.2These cases have been reported in line with the PROCESS criteria  research2.1A 75 year old retired electrician presented to the emergency department with a several week history of worsening abdominal pain and weight loss. He had a background of ischemic heart disease, type 2 diabetes, chronic obstructive airways disease and a duodenal ulcer diagnosed by endoscopy 2 years previously.On presentation he was dehydrated but haemodynamically stable and afebrile. His abdomen was soft, with general tenderness in the epigastrium with mild rebound but no rigidity. Full blood count, electrolytes and coagulation studies were all normal. A chest x-ray showed free air underneath the left hemi diaphragm.The diagnosis of a perforated viscous was made, thought to be due to a perforated peptic ulcer and he was resuscitated and taken to theatre for laparotomy. At laparotomy two segments of nodular, abnormal-looking small bowel were identified, the first in the proximal jejunum and the second in the distal jejunum . In the He was transferred to the high dependency unit for post-operative management where he developed acute renal failure, which responded to attention to fluid management. On the 12th post-operative day he developed increasing abdominal distension with subsequent plain films and a CT scan performed to exclude an anastomotic leak and collection. A gastograffin small bowel study showed a dilated oesophagus as well as generally dilated small bowel. He was treated conservatively and made a slow recovery. He was discharged on the 27th post operative day.He subsequently represented a month later with recurrent vomiting and was admitted for investigation. A CT scan showed the presence of locules of free air, as he did not demonstrate signs of peritonism he was treated conservatively with parental nutrition, oral oxygen therapy and intravenous antibiotics to treat the underlying pneumatosis and was then discharged 26 days later.During the first admission to hospital, histological examination was performed on sections from the jejunal resection specimen taken at laparotomy. The striking diagnostic feature of multiple submucosal cysts appearing as large ‘empty’ spaces  lined by2.2An 86 year old man was referred for consultation in the surgical outpatients clinic regarding investigation of a 5 month history of abdominal pain, weight loss, nausea and diarrhoea.He had a CT scan prior to referral demonstrating a small amount of ascitic fluid.The patient had no past medical history of note. His last endoscopy and colonoscopy were 10 years ago, with the colonoscopy identifying a tubular adenoma and hyperplastic polyps in rectosigmoid region.On review he had observations within normal parameters and his abdominal examination was normal.A subsequent endoscopy revealed diffuse gastritis with a small antral gastric ulcer with a small amount of blood. Helicobacter pylori testing was negative. On colonoscopy there was moderate sigmoid diverticular disease. Three polyps were removed from the ascending colon, sigmoid colon and rectum. The patient was commenced of a proton pump inhibitor and review in rooms arranged for 4 weeks.On review at 4 weeks the patient reported a complete resolution of symptoms that he had prior to initial consultation. His only complaint was of slightly more flatus than usual.Abdominal examination was again unremarkable. A follow up endoscopy was arranged which was normal.A routine abdominal CT scan was arranged to assess the presence of residual free fluid that was seen on the CT scan performed prior to the patient’s referral.The CT scan demonstrated free air within the bowel wall, with a follow up scan performed 3 weeks later revealing an increase in the amount of free air. The patient had a barium swallow, which was negative for a leak.The patient was referred to a tertiary centre for management, hyperbaric therapy was considered but ultimately the patient was treated conservatively with oral oxygen therapy and antibiotics.3PI is a rare condition characterised by the presence of subserosal and submucosal gas filled cysts occurring anywhere in the gastrointestinal tract from the oesophagus to the anus.  AlthoughIn a large study of 919 cases, Jamart found that it had a peak incidence between the ages of 41 and 50 years and was more common in males with a male to female ratio of 3:1. The small intestine was found to be the most commonly affected site (42%) followed by the large intestine (36%). In 22% both the small and large intestine were affected. [The pathological findings are that of multiple gas filled cysts in a sessile or a pedunculated form usually found in a submucosal or subserosal position. Numerous conditions have been associated with PI including bowel obstruction, infections, ischemia and there have been reported cases suggesting as association with Crohn’s disease.  In additPneumatosis intestinalis can be classified into a primary or idiopathic form (15% of cases) and a secondary form. The pathogenesis of PI is unknown however several theories exit. The three most common are the mechanical, pulmonary and bacterial theories.  The mechPatients with PI may present with chronic mild non-specific symptoms or may present acutely unwell with a surgical abdomen. A large study has shown symptoms in decreasing frequency of diarrhoea, bloody stools, abdominal pain, abdominal distension, constipation, weight loss, and tenesmus with rectal involvement . PI secoThe plain radiograph is the most common way to identify pneumatosis. In a review of 919 patients with PI proven by surgery or autopsy abdominal plain films were positive in two-thirds.  CircularTreatment of PI is generally reserved for those with symptoms taking into account the whole clinical picture and any underlying conditions. Treatment with oxygen or hyperbaric oxygen has been shown to be effective. ,29 This In general, surgery is only indicated in symptomatic patients where medical therapy has failed and in patients with presenting acutely unwell with a surgical abdomen, especially those with signs of perforation, peritonitis or abdominal sepsis. Increased recognition of the benign form of PI has resulted in less unnecessary surgery, which in the past has occurred up to 27% of cases ,6. There4Pneumatosis intestinalis is a rare condition that may manifest with a wide range of symptoms from mild abdominal pain to acute peritonitis. Its treatment is generally medical and even with radiological evidence of perforation laparotomy may not be indicated if the patient is clinically well.There are no conflicts of interest including employment, consultancies, stock ownership, honoraria, paid expert testimony, patent applications/registrations, grants or other funding.There are no sources of funding for this research.This study is exempt from ethical approval in this institution.Written informed consent was obtained from the patients 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 on request.Dr Sunny Dhadlie.- study concept- data collection, analysis, interpretation- writing the paper.Contributers:Dr Daniel Mehanna- study concept- Data collection, analysis, interpretation.Mr James McCourtney- data collection, analysis, interpretation.researchregistry4407.Dr Daniel Mehanna.Not commissioned, externally peer reviewed.


We report a very rare case of acute congestive ischaemic colitis of the left colon caused by brutal decompensation of an uncommon arteriovenous malformation (AVM) in the territory of the inferior mesenteric artery (IMA) in a 45-year-old male patient. The patient presented with severe abdominal pain in the left iliac fossa and abundant mucoid stools. The diagnosis of congestive colitis was made by optical colonoscopy but the full diagnosis of the responsible AVM in the IMA territory was made by contrast-enhanced multidetector CT scan combined with colour Doppler ultrasound. Two successive attempts at selective embolization failed to resolve the symptoms and finally, extensive surgery was necessary. The complete imaging findings of the case are presented and the characteristic features of uncommon AVMs and fistulas of the IMA territory are briefly reviewed. IC has a female gender predilection and mostly affects elderly patients, with 90% of them being over 60 years of age. Patients with IC frequently present with co-morbid conditions.Ischaemic colitis (IC) is the most prevalent type of gastrointestinal ischaemia .2The aetiologies of IC are numerous and lead to a diminished perfusion of the colon, which may result in reversible mucosal injury or even full-thickness necrosis. Fortunately, most cases (80–85%) only result in non-gangrenous segmental IC and resolve with medical management, without a specific cause being found.2 Non-occlusive causes comprise numerous life-threatening situations resulting in systemic hypoperfusion, situations of colonic dilatation or obstruction, iatrogenic causes related to a long list of drugs and long-distance running.IC is frequently classified as occlusive or non-occlusive. Occlusive IC can not only be attributed to thrombosis or emboli in large vessels, numerous and various chronic diseases of small vessels, trauma, various surgical procedures and iatrogenic causes  but also to venous causes resulting from venous thrombosis or venous hypertension.We hereby report a typical but very unusual case of acute congestive IC related to the decompensation of a very uncommon arteriovenous malformation (AVM) in the inferior mesenteric artery (IMA) territory.–1 at admission to 215 mg l–1 4 days later.A 45-year-old male patient was admitted to the emergency department with a 24-h history of heavy abdominal pain. The patient had previously experienced a prolonged episode of constipation and had been treated with laxatives. He was extremely obese and had a previous history of severe ischaemic cardiopathy complicated by episodes of ventricular tachycardia and auricular fibrillation. At admission, the pain was focalized in the hypogastrium and the left iliac fossa. These areas were painful to palpation with rebound. The patient also presented a small ecchymotic umbilical hernia. He was apyretic and had recurrent episodes of abundant mucoid stools. There was no rectal bleeding or melena. Laboratory tests were unremarkable except for a mild inflammatory syndrome with a C-reactive protein protein level ranging from 92 mg lAn unenhanced abdominal multidetector CT  scan was first performed because the patient was allergic to iodinated contrast media. A marked homogeneously circumferential hypodense thickening of a very long segment of the left colon extending from the descending colon to the rectosigmoid junction was found. Prominent vessels and massive fat stranding were depicted in the mesosigmoid fat. A small amount of ascites were present. There were no colonic diverticula and the diagnosis of colonic diverticulitis was excluded. The first retained diagnosis was IC or inflammatory colonic disease.Careful optical colonoscopy  was perfA complementary contrast-enhanced abdominal MDCT  was performed 48 h later after premedicating with oral steroid antiallergic  and 3. EThese varicosities converged to a dilated vein running through the transverse mesocolon to penetrate the venous splenoportal confluence at a right angle. An accessory drainage also joined the left renal vein. The inferior mesenteric vein was constitutionally absent.Scrupulous analysis of volume rendering and maximum intensity projection clearly diagnosed an arteriovenous communication in the distal mesosigmoid . There w® 18 liquid embolic system, Micro Therapeutic Inc., Irvine, CA) was performed. ONYX® was preferred to the traditional glue or coils to allow a more distal and controlled embolization. The goal was to protect the arteries of larger caliber and avoid a dramatic necrosis of the sigmoid and, especially, the rectum.4 days after admission, a decision to first treat the patient with selective embolization was proposed. Selective arteriography  of the INevertheless, 3 days later, the important symptoms persisted and a new contrast-enhanced MDCT confirmed the persistence of an active arteriovenous communication, which was probably owing to the presence of untreated areas .A second selective embolization session was performed 2 days later (not illustrated). The patient presented a significant transitory improvement in symptoms but resurgence came after only 10 days.Because of the likelihood of permanent untreated areas and/or recurrent collateralization, the increasing risk of extensive necrosis and the complexity and length of the varicosities owing to the constitutional agenesis of the inferior mesenteric vein, it was decided to refer the patient to the surgical team.A surgical resection was performed 20 days after the initial diagnosis. The resection was difficult. The left colon, sigmoid and upper rectum appeared swollen and infiltrated, and had a bluish appearance evoking venous ischaemia. The mesocolon and omental appendages were bloated. A complete resection of the left descending and sigmoid colon, and upper rectum was performed and completed by loop ileostomy for protection of the low colorectal anastomosis.Gross anatomy of the resected specimen  confirme5 Primary mesenteric AVMs are congenital and/or idiopathic and differ from secondary or acquired AVFs that are commonly caused by blunt or penetrating trauma (bullet or knife) or have iatrogenic aetiologies.5AVMs and arteriovenous fistulas (AVFs) of the gastrointestinal tract are often grouped together in clinical studies because their physiologic consequences are frequently exactly the same. However, there are important distinctions.7 AVMs or fistulas between the IMA and venous network are thus extremely rare.8 In an extensive review, Athanasiou et al8 recently identified only 26 primary or secondary cases described in the literature.In the splanchnic network, AVMs and AVFs are rather rare (about 200 cases reported). The involved arteries in decreasing order of incidence are the hepatic (45%), splenic (30%), superior mesenteric, gastroduodenal and inferior mesenteric, which is the rarest.7 Transfixion sutures or inadvertent ligation simultaneously passing through IMA branches and veins may cause fistulas during sigmoidectomy or left hemicolectomy.8 Similarly, the rupture of a congenital arterial aneurysm in very close anatomic relation with a vein can also result in a secondary fistula.8 There is usually a single communication between the artery and the vein in an AVF.Except for rare cases related to penetrating trauma, secondary fistulas (AVFs) in the IMA territory usually occur following colonic surgery or during arterial catheterization.3Congenital AVMs result from undifferentiated embryonic vessels tail failing to regress and interconnect the arterial and venous system. They can be distinguished by a dilated feeding artery, a large tangle of vessels representing the AVM nidus with multiple arteriovenous connections and a densely opacified early draining vein or veins.8In the reported case, the congenital origin of the AVM was the most probable cause. Indeed, the anamnesis of our patient was absolutely free of any previous surgical procedure or trauma. Moreover, embolization failed to successfully resolve the entity probably because multiple arteriovenous connections were present. The congenital nature of the AVM of our patient was also reinforced by the simultaneous presence of anatomic variants comprising agenesis of the inferior mesenteric vein and the presence of an exclusively mesenteric origin of the hepatic artery. Congenital malformations also often result in the formation of multiple fistulas .3The cause of the acute clinical decompensation of the AVM in our patient is not clear. A possible worsening of cardiac fibrillation or ischaemic cardiopathy could have promoted the degradation. The history of our patient is also consistent with the classical natural history of AVMs, which, although they are congenital, do not usually clinically reveal until adulthood.7 Less commonly, they also present with signs of congestive IC such as abdominal pain, diarrhoea and haematochezia.4 However, some patients may remain asymptomatic, with only incidental detection on imaging.3 The intensity of symptoms is extremely variable. These symptoms are flow dependant and may range from minimal signs to severe heart failure related to left-to-right shunt.8The vast majority of patients with inferior mesenteric AVMs present with symptoms of portal hypertension such as variceal bleeding or ascites.3 The classic clinical triad includes diarrhoea, haematochezia (not present in our patient) and abdominal pain.3 Guidelines for the diagnostic management of IC have recently been published.9 A combination of modalities comprising essentially MDCT—the cardinal imaging modality for patients with suspected IC—followed by colonoscopy with biopsies for a more specific diagnosis in suspected cases constitute the recommended diagnostic sequence. Contrast-enhanced MDCT assesses the distribution and phase of IC. Secondary colonoscopy with minimal insufflation follows, except in cases of gangrene, irreversible ischaemic damage or peritonitis. A recent study recently concluded that MRI, only using pre-contrast images, could be used as a valid substitute for more invasive procedures in the diagnosis and follow-up of acute IC.10Congestive IC is one of the most serious complications (50% of cases) of inferior mesenteric AVM or AVF.Wall thickening is the most common CT manifestation of IC. The wall is usually hypodense from mural oedema. Nevertheless, wall thickening is a non-specific sign and there is a wide degree of overlap with other colonic diseases such as inflammatory bowel disease and infectious colitis. Mesenteric fat stranding and ascites are also common but non-specific signs. When ischaemia progresses to infarction, free perforation, peritonitis, pneumatosis, dilatation of the colon and portal or mesenteric gas may be seen. Direct colonoscopic visualization of a cyanotic bowel wall with submucosal oedema or haemorrhage, necrotic mucosa with ulcers, or infarction can confirm the diagnosis.5 Acute congestive IC directly related to an AVM of the IMA territory as presented here is a very uncommon event.1The mechanism of congestive IC in the IMA territory may be caused by the variable combination of hypoperfusion of the mucosa—owing to a steal phenomenon during which the arterial blood flow through AVM bypasses the capillary bed of the rectosigmoid—and congestive submucosal oedema due to venous hypertension—clearly diagnosed during sigmoidoscopy and confirmed by CT in our case.8 This condition was not found in our very obese patient. Nevertheless, the sigmoid appeared extremely painful and incompressible during ultrasound examination.The congested viscera and, especially, the sigmoid colon can constitute a painful clinically palpable mass.Lower gastrointestinal bleeding can result from congestion of the bowel mucosa, direct fistula rupture within the lumen, necrosis owing to IC or bleeding haemorrhoids. Upper gastrointestinal bleeding resulting from oesophageal varices is rarer.7 Percutaneous endovascular arterial embolization of the feeding artery at the artery–venous junction is the technique of choice.6  It is extremely effective and has a low risk of complications in moderate-flow fistulae. Complications not only comprise serious IC but also recurrence, especially if there is more than one feeding artery. Embolization is not recommended in fistulas with large vessels because of the increased risk of extensive arterial thrombosis and ischaemia. Migration of the coils into the portal system has also been described for blood vessels of diameter >8 mm at high flow rates. Surgery would appear preferable in these situations. In our patient, two attempts at embolization failed to resolve the symptoms and extensive surgery was necessary.The treatment of inferior mesenteric AVMs or AVFs is complex and needs co-operation of the medical, radiological and surgical teams because the treatment needs case-specific solutions. Embolization is considered less invasive and potentially safer.The first retained diagnosis after emergency unenhanced abdominal MDCT was IC or inflammatory colonic disease. Colonic diverticulitis was immediately excluded because of the unusual length of the affected colonic segment and the absence of colonic diverticula. Drug-induced colitis was excluded by the anamnesis. Histopathology of the biopsies performed during optical colonoscopy confirmed a congestive and oedematous colonic mucosa with no trace of acute or chronic inflammatory process (ulcerative colitis or Crohn’s disease) and no sign of infection. Finally, contrast-enhanced abdominal MDCT and colour Doppler ultrasound were decisive for the diagnosis of the AVM causing congestive IC.The precise aetiology of IC remains unclear in many clinical cases. Numerous occlusive or non-occlusive causes exist. In the group of occlusive aetiologies, congestive ischaemia resulting from venous congestion owing to an AVM or fistula is extremely unusual.Only 26 cases of AVMs and fistulas in the territory of the IMA have been reported until 2014. IC is the most common complication with a prevalence of 50%. The classic clinical triad includes diarrhoea, haematochezia and abdominal pain.Fistulas essentially develop after a surgical or an interventional radiology procedure, or trauma. On the contrary, AVMs are congenital but classically remain clinically silent until adulthood.The combination of abdominal MDCT and focalized colour Doppler ultrasound was effective for the diagnosis in the reported case.The treatment of these vascular entities is complex and needs case-specific solutions. Co-operation of the medical, radiological and surgical teams is then required.


Filiform polyposis (FP) of the colon is a distinct and uncommon entity that is occasionally encountered in patients with a history or evidence of inflammatory bowel disease. It is morphologically characterized by multiple, slender, worm-like projections consisting of submucosal cores lined with normal mucosa. Here, we report a case of FP in a 43-year-old middle-aged man with a history of inflammatory bowel disease (ulcerative colitis). Filiform polyposis (FP) is most frequently secondary to a post-inflammatory reparative process and may occur in 10%-20% of the cases of inflammatory bowel disease in which chronic inflammation of the large bowel mucosa with repeated ulceration and healing may lead to the formation of worm-like polypoid projections .The term was first coined by Appleman et al. who used it to describe a syndrome involving the radiological appearance of numerous, long, slender, worm-like, or filiform defects in the colon .FP typically presents as one to hundreds of uniform, slender, arborizing, vermiform projections of the bowel mucosa and submucosa lined by normal or inflamed mucosa. These polyps are thin, straight-shaped, resembling the stalk of polyps without the heads. In rare cases, polyps coalesce and a large tumor mass, which may measure over 15 mm, known as giant filiform polyposis (GFP), is found.The patient presented with per rectum bleeding, no family history of colon polyps, colon cancer, or inflammatory bowel disease (IBD). He received steroid therapy for an unknown period on a clinical basis and remained symptomatic. Later, multiple colonic biopsies were done and revealed chronic active colitis with the possibility of IBD, most likely ulcerative colitis. The patient underwent total colectomy. The specimen showed numerous polyps, more than 100, up to 2.0 cm in size  is most frequently secondary to a post-inflammatory reparative process of the large bowel mucosa . FP is rFP typically presents as one to hundreds of uniform, slender, arborizing, vermiform projections of the bowel mucosa and submucosa lined by normal or inflamed mucosa. These polyps are thin, straight-shaped, resembling the stalk of polyps without the heads. When these polyps form tumor-like masses, as in our case, they are called giant filiform polyposis (GFP). Grossly, these giant polyps may be mistaken for malignancy. Microscopically, these polypoidal structures are lined by bland-appearing epithelium with no evidence of dysplasia. The adjacent mucosa will show changes of chronic active inflammation as a sequela of IBD. The gross identification and knowledge of this entity is essential for making a correct diagnosis.Although the presence of dysplasia or malignant transformation has never been reported in FP, in some cases, the polyps are difficult to distinguish from villous adenomas, and biopsies are needed to make the diagnosis . Our casGiant filiform polyposis can grossly mimic adenomatous polyps or colorectal carcinoma. It should be considered in the differential diagnosis of patients presenting with polyposis in IBD. A thorough sampling of the specimen is required to exclude dysplasia or invasive malignancy. A periodic, close follow-up is suggested.


We are presenting a unique case of a sub-periosteal osteoid osteoma involving coronoid fossa in a 25-year-old male. He was symptomatic for 2 years and his presentation mimicked mono-articular inflammatory arthritis. His plain radiographs were normal and the computed tomogram confirmed features of a sub-periosteal osteoid osteoma. He was treated with arthroscopic excision of the lesion. Pain relief was noticed immediately after the surgery and maintained at latest follow up of 1 year. Osteoid osteoma is a common benign lesion of bone. It is a bone-forming tumour and is most commonly located in the diaphysis of long bones. About half of them occur in bones of lower limb. Osteoid osteoma involving humerus is relatively rare . TypicalA 25-year-old male, right hand dominant and manual labourer by occupation came to our hospital with history of pain and stiffness of right elbow for last 2 years. Pain was mainly worse after periods of rest and after waking up in the morning, with no history of nocturnal exacerbations. Pain relived only partially with salicylates and other non-steroidal anti-inflammatory medications. He had no other musculoskeletal complaints or any other significant past medical history. He had previously sought consultation in other places and various diagnoses such as lateral epicondylitis, mono-articular rheumatoid involvement.°. Supination and pronation were normal. Plain radiographs were normal. Computed tomography (CT) with 3D reconstruction revealed a radio-dense ring measuring about 4 mm in the coronoid fossa of humerus. It had a radiolucent nidus measuring about 2 mm in diameter. There was a thin bony shell in the anterior aspect of the lesion. These findings were consistent with a diagnosis of a sub-periosteal osteoid osteoma. A decision of arthroscopic excision was made Physical examination revealed no swelling or fullness around the elbow. There was mild tenderness along the anterior aspect of elbow. There was restriction of both flexion and extension with elbow range of motion from 30 to 90Elbow arthroscopy was carried out under general anesthesia in lateral decubitus position. Standard proximal anteromedial viewing  and proximal anterolateral instrumentation portals  were used for elbow arthroscopy. A 2.7-mm, 30° arthroscope was used. The lesion was localised in the coronoid fossa. It appeared as a hyperaemic bony protuberance covered with hypertrophied synovium. Synovium was shaved with an arthroscopic shaver. Thin cortical shell over the lesion was removed with an arthroscopic shaver. The nidus was exposed and it shelled out easily upon manipulation with a curette. It was delivered through the anteromedial arthroscopic portal and sent for histopathology. The base of the lesion was cleaned with curette and burr. Normal bone deeper to the base of the lesion was not removed and posterior cortex was not perforated. No other arthroscopic releases were done. Intraoperative imaging was net required as the lesion was large and easily visible upon insertion of arthroscope.Histopathology findings of the nidus were consistent with an osteoid osteoma. There was an interlacing network of osteoid and bony trabeculae with some mineralization. Histopathology of the synovium only showed features of non-specific chronic inflammation. Gram stain and culture results were negative. His complete blood counts, erythrocyte sedimentation rate and C-reactive protein were within normal limits.Pain relief after the procedure was remarkable. He had no pain after 3 days and could resume his work. At latest follow up of 1 year patient is completely pain-free. He has full range of motion of the elbow 0–150°, . Osteoid osteomas may be frequently missed on a plain radiograph as approximately 25% of them may not be seen . This maClinically differential diagnosis could be mono-articular rheumatoid arthritis, tubercular arthritis or osteochondritis dissecans. CT scan was suggestive of an osteoid osteoma, but a possibility of chronic osteomyelitis should also be considered . IntraopSome aspects of arthroscopic technique may merit discussion. There were florid intra-articular adhesions in this case as described by Bhatia . We usedIn elbow joint osteoma has been described in olecranon fossa ,4, trochThus this case highlights the difficulty in diagnosis of a case of mono-articular elbow pain and stiffness. Due to absence of specific features of osteoid osteoma, diagnosis may be delayed in juxta-articular lesions. A neoplastic aetiology should always be considered in differential diagnosis of a mono-articular joint pain, stiffness and synovitis. Evaluation with a CT scan or a MRI should be carried out. Relief of symptoms is generally very dramatic after resection of osteoid osteoma.The authors declare that they have no conflicts of interest in relation to this article.


T1 and high intensity on T2 weighted images; arterial enhancement, which became more homogeneous in the later phases; and a relative hypointensity on the delayed phase images. Because a high level of awareness is necessary for making a correct diagnosis of this condition, one should consider the possibility of intrahepatic splenosis in a patient with a history of abdominal trauma or splenic surgery.Splenosis refers to the autotransplantation of splenic tissue throughout different anatomic compartments secondary to trauma or splenic surgery. The liver is an uncommon location for splenic implants and imaging findings are often described as non-specific. We report MRI findings of a patient with a large liver mass that was first diagnosed as a malignant tumour but histopathology revealed that it was actually intrahepatic splenosis. The signal characteristics of this mass were low intensity on  Splenosis refers to the autotransplantation of splenic tissue throughout different anatomic compartments. It is thought to result from a capsular tear, either traumatic or iatrogenic, occurring during splenic surgery, with subsequent spillage and seeding of splenic pulp that later grows into a mass.4 The incidence of splenosis may be underreported, as most patients are asymptomatic. The common locations for splenosis include the mesentery, omentum, peritoneum and peritoneal cavity.6 However, uncommon sites such as the thoracic cavity, gallbladder, skin and brain have also been reported.Regarding the mode of splenic tissue autotransplantation, there are two possible routes: direct implantation and haematogenous spreading. The first accounts for the majority of cases, while the latter is believed to be responsible for the implantation of splenic tissue in distant compartments.12 The reported imaging findings, especially MRI, are scarce and often non-specific.12 Here, we report the MRI findings of a patient with an isolated intrahepatic splenosis that was mistaken for hepatocellular carcinoma and discuss the possible helpful features for making the correct diagnosis.Intrahepatic splenosis is referred to as the autotransplantation of splenic tissue in the liver. It is an uncommon condition and, when present, is usually mistaken for a neoplasm.A 51-year-old male who had undergone a splenectomy 20 years earlier, supposedly owing to thalassaemia, presented to another hospital with intermittent abdominal pain at the left upper quadrant. There was no history of weight loss or any other abnormal symptoms. A liver mass was found during the initial work-up and he was then referred to our hospital for further investigation and management.The liver function test revealed slightly elevated aspartate transaminase, alanine transaminase and total bilirubin levels. The details were as follows: alanine transaminase 64; aspartate transaminase 75; alkaline phosphatase 64; total bilirubin 1.7 and direct bilirubin 0.3; albumin 3.8; globulin 2.3. The hepatitis B and C profiles were negative. Tumour markers, including cancer antigen 125, alpha-fetoprotein, cancer antigen 19-9 and carcinoembryonic antigen, were all negative.Ultrasound images from other hospitals were not available for interpretation.T1 and T2 weighted images is shown in MRI showed a large, slightly lobulated exophytic mass at the left lobe of the liver without a separable fat plane between the mass and the liver parenchyma. The signal intensity of this mass on Owing to its large size, the mass was presumed to be malignant. The pre-operative diagnosis favoured hepatocellular carcinoma because of the arterial-phase enhancement and the delayed-phase hypointensity. The patient subsequently underwent a left lateral segmentectomy, from which the gross specimen revealed a mass within the left hepatic lobe. However, the cut surface of this mass looked similar to that of the spleen  and the Isolated intrahepatic splenosis is rare. Although it is a benign condition, diagnosing it can be difficult in clinical practice because it can mimic a liver tumour, which frequently leads to unnecessary surgery.14Currently, there are a number of hypotheses regarding the mechanism of hepatic implantation of splenic tissue, including invagination or exophytic growth of splenic tissue that is directly seeded into the liver capsule, and haematogenous spreading due to entry of an erythrocyte progenitor cell into the portal venous system.T1 and iso- to high signal intensity on T2 weighted images.11,15 Because of the difference of blood flow in the red and white pulp, the splenic implants were shown to have heterogeneous enhancement on the arterial phase and became homogeneous in the later phases of dynamic MRI.15 On the delayed phase, the signal intensity of intrahepatic splenosis may be lower than that of the liver parenchyma.9Imaging findings of splenosis have been often described as non-specific. Most authors described the MRI characteristics of splenosis as low signal intensity on 16 As in a normal spleen, restricted diffusion has also been demonstrated in the case of splenosis.17A previous study on the apparent diffusion coefficient value of intra-abdominal organs has shown that the value was lowest in the spleen.T2 and hypointense on T1 weighted images, with heterogeneous enhancement on the arterial phase and becoming more homogeneous in the later phases. Diminished enhancement was also observed on delayed images. Diffusion-weighted images showed a slightly high signal intensity of the lesion.The signal characteristics and enhancement pattern of intrahepatic splenosis in our case are similar to those previously reported—that is, being hyperintense on 8 reported a signal dropout on in-phase T1 weighted image when compared with the signal on the opposed-phase images, which was indicative of haemosiderin deposition in splenic tissue. Likewise, this finding was also observed in our study. Thus, we believe the presence of a signal change in chemical-shift imaging to be a helpful feature for the diagnosis of splenosis.Inchingolo et alMoreover, the demonstration of extrahepatic venous drainage is unusual for a primary liver tumour. The presence of such vessels should thus raise the suspicion of another possible diagnosis.10As described in a previous report, a thin rim of fat at the border of the lesion was not visualized in our study.99mTc denatured red blood cell scintigraphy or liver MRI using superparamagnetic iron oxide-based contrast agents, and avoid unnecessary surgery.15 Therefore, in the case of patients with a history of splenic surgery or abdominal trauma, one should consider splenosis in the differential diagnosis.Above all, a high level of awareness remains the most crucial factor in the diagnosis of splenosis. If recognized preoperatively, one can opt for non-invasive imaging confirmation such as In the case of patients with a history of splenectomy or splenic trauma, the presence of an arterially enhancing liver mass that becomes more homogeneous in the later phases should raise the clinical suspicion of splenosis.The presence of a signal dropout on in-phase images and extrahepatic venous drainage are helpful features for the diagnosis of this pathology.If splenosis is suspected, there are non-invasive imaging modalities available for the confirmation of this pathology.This retrospective case report was approved by the Ethic Committee for Human Research based on the declaration of Helsinki and the International Conference on Harmonization good clinical practice guidelines. Clinical data were obtained by reviewing medical records. MRI findings were reviewed and consented to by two radiologists specializing in gastrointestinal and hepatobiliary imaging.Informed consent for the case to be published was obtained for publication of this case report, including accompanying images.


Nontraumatic haematoma of parapharyngeal space is very rare and may cause dysphagia and dyspnea. The authors present a case report of a 74-year-old woman with sudden nontraumatic neck swelling without dyspnea and with left pharyngeal bulging and endolaryngeal displacement. Parathyroid hormone elevation and imaging exams confirmed bleeding from a parathyroid adenoma. Symptoms and signs resolved after one week of conservative treatment. There are few cases of parapharyngeal haematomas caused by parathyroid adenomas. Most patients can be managed without emergent surgery, but close airway monitoring is fundamental. Lesions of parapharyngeal space are rare, comprising only 0.5% of head and neck neoplasms . They maMost haematomas resolve spontaneously, so conservative management is often appropriate except in the presence of airway compromise or compression symptoms, when surgical treatment should be considered , 3.A 74-year-old woman presented to the emergency department with sudden onset (24 hours) of painful neck swelling and concurrent dysphonia and solid dysphagia. She denied neck trauma. On physical examination, there was a tough and painful mass and ecchymosis in the thyroid bed. Nasofiberoscopy showed bulging of the left lateral pharyngeal wall leading to right displacement of the endolarynx. Left ventricular fold and ventricle exhibited a violaceous coloration. Computed tomography revealed a nonenhancing collection in the left parapharyngeal space . MagnetiThis is a very rare clinical situation: until 2009, only 28 cases of spontaneous cervical haematoma caused by extracapsular rupture of the parathyroid gland were reported . ExpansiMany conditions can be responsible for sudden neck swelling. After excluding trauma, blood test results and imaging are crucial for an accurate etiological diagnosis. Close monitoring of the airway is essential, in order to do emergent treatment in life-threatening cases.


Pierre Robin sequence (PRS) is a set of congenital abnormalities commonly comprising small jaw (micrognathia), posteriorly placed tongue (glossoptosis), and airway obstruction; however, other abnormalities may also be associated with PRS. Here we report the case of a 5-year-old girl with short stature, polysyndactyly, and an ectopic kidney who presented with PRS features. Pierre Robin sequence (PRS) is a combination of congenital anomalies comprising fetal gnatho-glosso-palatoschisis. It was first described by Hilaire in 1822 and included micrognathia, cleft palate, and airway obstruction; this description was followed by that given by Fairbain in 1846 and by Shukowsky in 1911 . The incA 5-year-old Yemeni girl with a known diagnosis of iPRS was delivered at full term via cesarean section. Her parents were first-degree cousins. Her antenatal history was normal; however, her mother had mild preeclampsia, which needed to be treated with antenatal vitamin supplements. She had a low birth weight of 1800 g and a height of 48 cm, which were both below the third percentile for her age and sex. Her parents presented her to the Pediatric Endocrine Clinic with complaints of short stature and poor weight gain, in comparison to her three siblings who were growing normally. Her past medical history revealed symptoms of feeding difficulties, repeated milk regurgitation with shocking attacks, respiratory distress, snoring, and sleep apnea, all due to her initial congenital anomalies of micro-retrognathia and cleft palate. She had no history of glossoptosis as she had congenital tongue tie. Her developmental history revealed dysarthria with delayed language skill without nasal resonance, normal vision, and normal hearing. There was no family history of a similar condition. She had undergone three surgeries previously; the first was for cleft palate repair at the age of one year Figure , and theImaging modalities were used to rule out other associated abnormalities; her bone mineral density was 2.4 standard deviations below the expected normal range for her age. Renal ultrasound showed an ectopic horseshoe kidney at the superior midline and a renogram scintigraph demonstrated adequate flow to the ectopic kidney, with no evidence of obstruction. Results of other systematic investigations were normal. Growth hormone therapy was started because she was small for gestational age and owing to failure to catch up in terms of growth in the first three years, as indicated by the Food and Drug American Association. One year after receiving growth hormone therapy, her height improved to 104 cm Figure .Here we report a case of a 5-year-old girl with short stature, polysyndactyly, and an ectopic kidney who presented with PRS features.The name PRS was derived from one known or presumed single anomaly or mechanical factor, but it can cause multiple anomalies . PRS preInfants and children with isolated or syndromic type of PRS should be evaluated for the presence of renal anomalies during early assessment and follow-up to prevent further kidney injury. Patient stature evaluation should also be considered for any abnormalities to administer growth hormone therapy when indicated.


Primary sarcoma of the lung is a very rare malignant tumor accounting for less than 0.5% of all lung tumors and presenting diagnostic and treatment challenge. We describe a case of a patient diagnosed with primary lung undifferentiated pleomorphic sarcoma developing subsequent peritoneal and small bowel metastases, which stand for highly unusual disease presentation.A 57-year-old male presented with extensive partially necrotic tumor in the left upper lobe (LUL) of the lung that involved LUL bronchus and extended to the visceral pleura. There was no evidence of nodal or visceral dissemination. After initial presentation, the patient was admitted to the hospital’s pulmonology department for further workup. The most likely diagnosis based on biopsy specimen was poorly differentiated sarcoma. Left pneumonectomy with mediastinal lymph node dissection was performed. The final pathohistological diagnosis (PHD) was undifferentiated pleomorphic sarcoma (UPS). Three months after lung surgery, a follow-up CT scan was done which showed a 60-mm obstructive metastatic intraabdominal lesion with small bowel infiltration and further separate peritoneal deposits. Unfortunately, an urgent surgery had to be performed as the patient developed signs of acute abdomen due to bowel perforation. Only 2 months later, the patient passed away at home.Treatment options of UPS are based on algorithms used in treatment of extremity lesions with well-established role of surgery. However, the role of perioperative chemotherapy remains equivocal with no strong evidence-based data due to the rarity of the disease. Small bowel is an unexpected metastatic site, but of significant clinical relevance. The undifferentiated pleomorphic sarcoma (UPS) is a rare tumor accounting for less than 0.2% of all lung tumors –3 and thA 57-year-old male was admitted to the Pulmonary Oncology Department of University Hospital Centre Zagreb in November 2017 with cough and hemoptysis. He smoked 27 packs/year, had an alcohol abuse history, and displayed no evidence of present or past soft tissue neoplasms or history of radiation exposure. His Karnofsky performance score was 80. Subsequent physical examination revealed reduced air entry in the left lung. His complete blood count and biochemical parameters were within the normal range. Initial chest x-ray showed an extensive consolidation in the left lung suggesting possible malignancy; CT of chest and abdomen was recommended. The CT scan showed an extensive partially necrotic tumor in the left upper lobe (LUL) extending from the left hilum with infiltration of the LUL bronchus to the visceral pleura, measuring 74 mm in the largest diameter , CK7, and S100. The most likely diagnosis was poorly differentiated sarcoma.For final staging, PET/CT scan was performed, showing no extra thoracic spread of the disease. The multidisciplinary tumor board deemed that lung surgery was the best treatment alternative, and several weeks later, left pneumonectomy with mediastinal lymph node dissection was done. Final pathohistological analysis noted a tumor size of 100 × 85 × 71 mm, more than 80% of which was necrotic. The pleomorphic tumor cells showed the same immunohistochemical profile as in biopsy specimen. Synovial sarcoma-associated translocation was negative. All surgical margins and dissected mediastinal lymph nodes were tumor free. Based on this, the final diagnosis of undifferentiated pleomorphic sarcoma (grade 3) was made Fig. .Fig. 2IThe patient recovered well from the surgery, and follow-up was planned. At the first follow-up visit 7 weeks after lung surgery, the patient was free of malignant disease as per physical examination and chest x-ray. Three months after the lung surgery, a follow-up CT scan was done which showed a 60-mm metastatic intraabdominal lesion with an evident small bowel involvement, as well as further separate malignant peritoneal deposits. The scan also showed mild upstream dilatation of the small bowel suggestive of partial obstruction Fig. .Fig. 3CTherefore, an abdominal surgery was planned. However, just a couple of days later, the patient presented to the Emergency Department with clinical signs of acute abdomen due to bowel perforation. Urgent abdominal surgery was performed with resection of perforated segment of the ileum, which was infiltrated with tumor. A unipolar ileostomy was then fashioned. The histopathological analysis confirmed metastasis of undifferentiated sarcoma Fig. .Fig. 4HThe patient was discharged from the hospital 9 days later with the Karnofsky performance score of 40; thus, the best supportive care was indicated. The patient died at home 2 months later.Primary pulmonary sarcomas are extremely rare, and can be found in 0.1 to 0.5% of all pulmonary neoplasm cases. The most common sarcomas include leiomyosarcoma, malignant fibrous histiocytoma, and synovial sarcoma , 7. SympImmunohistochemistry has an important role for accurate diagnosis and classification of sarcoma type. It is often positive for keratin, actin, desmin, EMA, CD99, and CD34, but positive stains usually do not help diagnosis, so UPS is a diagnosis of exclusion . In our Treatment options are based on algorithms used in treatment of extremity lesions with the well-established role of wide surgical resection aiming for tumor-free margins as the primary therapeutic modality. Some authors found that completeness of resection was correlated with significantly increased survival, but size and grade of tumor were not . RadicalThe role of adjuvant radiation therapy still remains equivocal. It can provide acceptable local control of some soft tissue sarcomas after surgical resection , but theThese neoplasms have an aggressive clinical course with a high potential for recurrence and metastasis. In an advanced stage, a combination of chemotherapy or radiotherapy may be used as a palliative approach, although the tumor seems insensitive to both chemotherapy and radiotherapy . The oveDuring the short follow-up period, our patient developed unexpected peritoneal dissemination, which, to the best of our knowledge, has not been reported as a site of metastatic spread for primary pulmonary sarcoma yet. There has been one case report of cardiac metastasis from an undifferentiated pleomorphic sarcoma of the lung presenting with symptomatic right heart failure described in the literature , and treHigh-risk features in our case were poorly differentiated histology and tumor size; thus, despite radical surgery with completeness of resection, clear margins, and no nodal involvement, the patient passed away in less than a year after diagnosis was made.Primary undifferentiated pleomorphic lung sarcoma remains an extremely rare malignancy without standardized treatment and poor prognosis. An optimal treatment strategy has not yet been elucidated due to limited data available, although complete surgical excision remains the preferable treatment option. Further investigation and data collection from clinical practice are needed to improve the outcomes, optimize treatments, and define the follow-up approach to this aggressive malignancy.


Abdominal apoplexy, otherwise known as intraperitoneal idiopathic spontaneous haemorrhage, is a rare condition that presents as a diagnostic dilemma and is associated with high mortality. Symptoms and signs typically are similar to other conditions presenting with upper abdominal peritonitis. Intraabdominal haemorrhage can occur from many different causes, including trauma, iatrogenic, ruptured aneurysm, gynaecological conditions, malignancy, and inflammatory or autoimmune processes. Spontaneous or idiopathic causes are much rarer. Prompt diagnosis and ligation of the bleeding vessel usually result in a good outcome. Most cases described involve males in the fifth and sixth decade of life who present in the setting of hypertension and known atherosclerotic disease and are managed with laparotomy or are diagnosed at autopsy. We present a case of abdominal apoplexy managed laparoscopically in a healthy 20-year-old male with no pre-existing medical conditions. This case highlights the importance to consider abdominal apoplexy in any demographic. Abdominal apoplexy, otherwise known as intraperitoneal idiopathic spontaneous haemorrhage, is a rare condition that usually presents as a diagnostic dilemma and is associated with high mortality. Symptoms and signs typically are similar to other conditions presenting with upper abdominal peritonitis. The term abdominal apoplexy was first used in 1931 by Green and Powers to describe a case series of spontaneous haemoperitoneum resulting from the spontaneous rupture of an intraabdominal vessel . The patA 20-year-old male presented to our emergency department with a one-day history of worsening central and epigastric abdominal pain associated with multiple instances of vomiting. He denied any intake, recent trauma, or any recent infective symptoms. He also complained of chest pain, shortness of breath, and lightheadedness. Our patient was a fit and well 20-year-old male with no significant medical or family history. He had a systolic blood pressure between 100 - 140 mmHg, was tachycardic between 100 - 115 beats per minute (bpm), and required increasing amounts of opiate analgesia. His haemoglobin was 129 g/l and the lipase level was within normal limits. Computed tomography (CT) scan showed moderate free fluid throughout the abdomen, particularly within the pelvis, of an intermediate density of 35 - 40 Hounsfield units and a lobulated heterogenous mass extending from the greater curvature of the stomach into the greater omentum measuring 11.7 x 6.3 x 13.7 cm, in keeping with a neoplasm, such as a gastrointestinal stromal tumour (Figures Intraabdominal haemorrhage can occur from many different causes, including trauma, iatrogenic, ruptured aneurysm, gynaecological conditions, malignancy, and inflammatory or autoimmune processes. Spontaneous or idiopathic causes are much rarer. Cases of abdominal apoplexy are usually described in the context of underlying vascular disease, most commonly atherosclerotic disease and hypertension . CoaguloThere is a 2 to 3:1 male preponderance, typically presenting between 50 - 70 years of age. The presentation usually includes abdominal pain, which may be associated with nausea, vomiting, and diarrhoea. The condition is clinically difficult to differentiate from peptic ulcer disease or acute pancreatitis .When initially described, the diagnosis was made during laparotomy. Diagnostic methods have evolved with easier access to imaging. CT is considered the most important imaging technique with CT angiography proving useful to identify the sites of active bleeding . FocusedOur case report presents a unique case of abdominal apoplexy. While the typical patient is in their fifth or sixth decade of life with known systemic conditions associated with vasculopathy or coagulopathy, our patient was a young and healthy person with no known medical conditions or genetic predispositions for vasculopathy. While the patient showed signs of deterioration, conditions were deemed appropriate for an exploratory laparoscopy and evacuation of the haematoma, with a low threshold for converting to laparotomy. While no bleeding source was found, the absence of an ongoing bleed was excluded with a period of observation and follow-up CT angiogram two days postoperatively. Furthermore, the diagnosis was made more challenging due to the ambiguous findings on CT suggestive of a haemorrhage from a ruptured neoplastic mass. We recognise that this was a rare case of spontaneous intraperitoneal haemorrhage and have consulted vascular medicine to exclude underlying conditions that may predispose our patient from subsequent episodes.Abdominal apoplexy is a fatal condition which can progress rapidly. Better awareness of the condition may help to improve survival by promoting early diagnosis and intervention. Our case report highlights that, while most cases of abdominal apoplexy occur in patients in the fifth or sixth decade of life with systemic conditions that can precipitate spontaneous vascular injury, it can also occur in young patients who have no preexisting conditions. This case also shows that in the appropriate clinical setting, exploratory laparoscopy and evacuation of the haematoma is a safe and viable option in the management of abdominal apoplexy.


Tumoral calcinosis is an uncommon clinicopathological condition which is characterized by the formation of calcium salt deposition in intra-articular or peri-articular soft tissues. It usually presents as a focal growth of hard tissue, either solitary or multiple, beneath the skin and connective tissue. Diagnostic techniques mainly include clinical and radiographic evaluation. The most commonly involved locations include the hip, elbow, shoulder and knee. Involvement of the head and neck regions are far less common. There have been 5 case reports of temporomandibular joint involvement in the literature so far.We present a case report which describes the diagnosis and management of a 59 year old female patient with chronic right temporomandibular joint pain and localized bony hard swelling over the right pre-auricular region. Patient retained normal range of motion and mouth opening. Computed tomography taken showed a radio-opaque juxta-articular ovoid mass over the right pre-auricular region in close proximity but not fused to the mandibular condyle. Surgical excision was performed for this swelling via a pre-auricular approach under general anaesthesia. Histological examination performed confirmed the diagnosis of tumoral calcinosis. Pain at the right temporomandibular joint was resolved after the surgery. Serum calcium and phosphate levels were normal in this patient.Surgical excision is the primary treatment modality for tumoral calcinosis. Tumoral calcinosis may be associated with elevated serum calcium and phosphate levels. In patients with elevated serum electrolytes, it is important to consider the overall systemic health in management of this condition. Management of serum electrolytes levels plays a role in reducing recurrence rates. This case report and review aims to discuss the diagnosis, treatment and overall systemic management of this rare condition. In 1899, Duret first described a clinical case of calcifying cystic masses in the connective tissue adjacent to a joint space . Since tThe most commonly reported sites affected by TC are hip, elbow, shoulder and knee –5, 10. IThe first case of TC involving the temporomandibular joint (TMJ) was reported by K. Shirasuna in 1991 in a male Japanese patient . Since tA 57-year-old Chinese female was referred to National University Hospital, Singapore in February 2016 for management of a hard swelling over her right TMJ. This patient suffered from chronic right TMJ pain for more than 5 years. She had consulted several physicians over the past few years, but could not obtain a definitive diagnosis. There was no prior treatment provided. Her past medical history includes surgical excision of left breast fibro-adenoma, recurrent upper urinary tract infection, stress urinary incontinence and infective colitis. She was on active follow up with the Otolaryngology department for hoarseness of voice. She was a non-smoker and non-drinker. There was no known history of any genetic disorder. The patient denied any history of trauma or infection to the jaw area. There were no other family members with a history of temporomandibular joint or jaw swelling.On examination, a localized swelling was palpable over the right pre-auricular region which measured 15 mm by 20 mm. It was tender upon palpation and had a bony hard texture. The swelling appeared distinct from the TMJ capsule as it did not move when the right mandibular condyle translated anteriorly during mouth opening. On maximal mouth opening, the condylar translation at both TMJs was similar. The maximal mouth opening was normal. The overlying skin was normal in consistency and was not fixed to the swelling.A series of lab tests were ordered, including liver function test, renal function test and complete blood count. All results were being found to be within normal ranges. A dental panoramic tomogram (DPT) was taken Fig. , but no The provisional diagnosis was tumoral calcinosis (TC) of the right TMJ. This was because the mass appeared distinct and was not contiguous with the right mandibular condyle. There was no erosion of the mandibular condyle. The joint space and capsule appeared preserved on CT. Other less likely differential diagnoses included synovial osteochondromatosis, synovial chondrosarcoma and osteochondroma.In view of the swelling and chronic pain of more than 5 years, she was advised to undergo surgical excision of the calcified mass. Subsequently, surgical excision of the calcified mass was performed through a pre-auricular approach under general anaesthesia in March 2016 Fig. a-b. IntrHistological examination of the specimen reported nodular fragments of amorphous pink matrix and fibrous tissue with calcified areas. There were areas of foreign body reaction. Figure The post-operative recovery was uneventful. Pain at the right pre-auricular region was resolved after surgical excision of the swelling. There was no limitation of function of the right TMJ. Maximal mouth opening was normal and the patient could eat well. Upon histological confirmation of TC, a baseline serum electrolyte test was ordered for this patient. The baseline serum calcium and phosphate levels were normal.The patient was reviewed regularly for 3 months. However, she subsequently defaulted on her follow-up appointments after the review in April 2016. She eventually returned for a review on September 2017, 17 months after surgical excision. There was no clinical recurrence noted. The pre-auricular skin wound healed with minimal scarring. She reported minor sensory deficit in the right pre-auricular region with a Visual Analog Scale score of 1/10. Other than the minor sensory deficit, there were no other adverse outcomes. She was able to functional well without any limitation or pain. Serum electrolytes were tested again and levels were normal. Further follow-up appointments were arranged. However, the patient defaulted on the subsequent appointments.Tumoral calcinosis is a rare disease entity and its involvement at the TMJ region remains scarce. Common differential diagnosis for a hard tissue growth around the pre-auricular region includes synovial osteochondromatosis, synovial chondrosarcoma, osteochondroma, and osteochondrosarcoma. Synovial chondromatosis is a benign condition characterized by synovial membrane proliferation and metaplasia. It presents as calcifying nodules of cartilage within the synovial membrane of the TMJ and usually affects a unilateral joint space. It is often associated with trauma. The loose calcified nodules may increase in numbers with time and can eventually occupy the entire joint space. Like TC, synovial chondromatosis can result in pain, swelling and limitation of range of motion of the TMJ. Synovial chondrosarcoma is a very rare malignancy that can arise from synovial chondromatosis or de novo within the synovial membrane. Synovial chondrosarcoma involvement of the TMJ is rare.Osteochondroma is more commonly associated with the axial skeleton and less commonly associated with the facial bones. It is a slow-growing benign tumour which causes enlargement of the mandibular condyle with time. It results in limitation of mouth opening, malocclusion and TMJ dysfunction. Osteochondroma, despite a similar presentation on plain radiographs, exhibit a characteristic cartilage-capping over the bony lesion which appears to be contiguous with periosteum of the affected bone.On the contrary, TC is not contiguous with the periosteum of the affected bone. It may present as intra-articular or juxta-articular lesions. Of the 5 reported cases of TMJ involvement, 3 of them were intra-articular –29 and 2The diagnosis of TC is primarily based on imaging. Plain radiographs depict amorphous, multi-lobulated radio-opaque masses adjacent to the mandibular condyle. In this patient, the DPT did not demonstrate the presence of the lesion. This could be attributed to the fact that the lesion was not as radio-opaque when compared to its surrounding bone, owing to its lower density. In addition, its presence on the DPT may be obscured by the skull base due to its location. This case report highlights the difficulty in diagnosing the lesion using the DPT. In patients where the swelling is not highly noticeable during clinical examination, it is possible that such a lesion can be missed or undiagnosed.This highlights the need for CT for proper evaluation of such lesions. CT scan is critical for diagnosis as it provides the precise location, size and relations to the surrounding tissues. Of the 5 reported cases of TMJ involvement, 4 were diagnosed using CT scans , 28, 29.Of the 5 reported cases of TMJ involvement, only 1 was diagnosed using magnetic resonance imaging . MagnetiHistological features of TC are distinctively different from the rest. Idiopathic calcifications are evident with positive calcium stains using von Kossa’s method, without involvement of cartilage transformation , 4. CalcThe exact pathogenesis of tumoral calcinosis remains unconfirmed till today. Several theories have been proposed in the literature, alluding to its possible association with systemic hyperphosphatemia in patients receiving chronic haemodialysis and patients with potential underlying familial traits that lead to mutation of genes involved in phosphate metabolism, e.g.FGF-23 (Fibroblast Growth Factor - 23) and GALNT-3 , with presence of associated mechanical injury , 13–17.A commonly recognized classification for TC was introduced by Smack et al., who proposed a classification based on possible pathogenesis after retrospectively reviewing 122 cases of TC  , the primary treatment modality is surgical excision . This waFor secondary TC sub-type patients with chronic renal failure and haemodialysis, surgery may not be the first option due to the presence of multiple co-morbidities and higher risk of recurrence. In such patients, medical treatment may have a role. Treatment modalities such as calcium-depleted electrolytic bath , phosphaAccording to Smack et al., the risk of recurrence after surgical excision for patients in the Primary Normophosphatemic TC sub-type is low . HoweverTumoral calcinosis associated with the temporomandibular joint is a rare condition. Proper diagnosis requires detailed history taking, clinical examination, radiographic investigations and baseline serum electrolytes. Computed tomography is preferred over Dental Panoramic Tomogram as such calcifying lesions may not be clearly seen in DPT images as exemplified by this case. Computed tomography provides a detailed assessment of the location, size and relations to surrounding tissues. Surgical excision is usually the treatment of choice for Primary Normophosphatemic TC and Primary Hyperphosphatemic TC sub-types. Medical therapy such as phosphate binders, calcitonin can play a role in management of Secondary TC sub-type. It is important to consider the overall systemic health in patients belonging to Primary Hyperphosphatemic TC and Secondary TC sub-types, due to raised serum electrolytes. Correction of the serum electrolytes plays a crucial role in reducing the risk of recurrence of these lesions. It is advisable to refer patients belonging to the Primary Hyperphosphatemic TC or Secondary TC sub-types to the appropriate physician for evaluation of underlying causes in relation to raised serum electrolytes. Monitoring for recurrence of lesions is important for the Primary Hyperphosphatemic TC or Secondary TC sub-types, due to the higher recurrence rates. However, there is no commonly accepted protocol for long term follow-up of TC due to the rarity of this condition. We suggest a yearly follow-up for all three sub-types of TC. In particular, there should be longer follow-up periods for the Primary Hyperphosphatemic TC and Secondary TC sub-types.


Lymphatic malformation is a rare disorder caused by expansion and proliferation of the lymphatic vessels. About 50% of lymphatic malformations are present at birth, and 90% are diagnosed before the age of 2 years. The most common site of lymphatic malformation in the oral cavity is the anterior two‐thirds of the tongue, and involvement of the buccal mucosa is relatively unusual. Treatment of lymphatic malformation includes surgical excision, sclerotherapy, laser therapy, radiofrequency ablation, or a combination of these interventions. In many cases, surgery or sclerotherapy is the treatment of choice. However, there is currently no consensus on an optimal treatment approach. Here, we report an unusual case of lymphatic malformation in the buccal mucosa of an older adult that was surgically excised. The patient had no congenital swelling of the left buccal mucosa at birth and no history of systemic disease or relevant family history. The patient had undergone maxillary molar restoration treatment 2 years earlier, after which she reported biting regularly on her buccal mucosa. On most occasions, the wound had healed within a week, so she had not sought medical treatment. An extraoral examination revealed no facial swelling or asymmetry. However, an intraoral examination revealed an area of diffuse swelling on the left buccal mucosa measuring about 15 mm × 30 mm and containing a papillary lesion with multiple red, blue, and clear pebble‐like vesicles Figure , 5. On f3Lymphatic malformation is caused by failure of the lymphatic communication, lymphatic obstruction, fluid retention and cell proliferation. Although lymphatic malformation can occur at any age, cases in adults are very rare.The present case of lymphatic malformation in a 68‐year‐old woman is exceptionally unusual. Lymphatic malformation was not present at birth in our patient, but its development coincided with the patient regularly biting her buccal mucosa subsequent to a maxillary dental restoration. The etiology of acquired lymphatic malformation is unknown, but inflammation and trauma are thought to be likely causes. We found a total of five reported cases of lymphatic malformation of buccal mucosa by surgical treatment in an adult.There are several stages in the pathogenesis of vessel proliferation in acquired lymphatic malformation. First, the accumulation of interstitial fluid leads to swelling of the extracellular matrix, to which the endothelial cells lining the lymphatic capillaries become attached. Next, the endothelial cells lining the lymph ducts become elongated at the capillary level, β‐1‐integrin is activated, and phosphorylation of vascular endothelial growth factor receptor 3 occurs. This process results in the proliferation of endothelial cells in the lymph ducts, which is increased by edema and mechanical pressure.In our patient, the lesion involved the buccal mucosa but did not infiltrate the buccinator muscle. Therefore, it was possible to treat the patient surgically without damaging the parotid duct. Second, the lesion could be removed via the fascia of the buccinator muscle and the surgical wound completely closed by sutures.When considering excision of a lymphatic malformation, five factors need to be taken into account, that is, the mortality risk, resectability of the malformation, functional effects, complications, and the likelihood of recurrence. In this case, the lesion was completely removed surgically without any residual dysfunction, such as trismus, or recurrence.Spontaneous regression of a lymphatic malformation is rare, and various treatment strategies have been reported in the literature. In many reports, surgery or sclerotherapy was performedLymphatic malformations are classified into three pathologic types, that is, macrocystic, microcystic, and mixed. The macrocystic type is usually lined by many layers and typically occurs in the neck, whereas the microcystic type has a single endothelial layer and typically occurs in the oral cavity. The lesion in our patient was of the typical microcystic type with an expanded vascular lumen that was stained by podoplanin. Sclerotherapy has often been reported to be of benefit in the treatment of lymphatic malformation of the microcystic type, albeit with wide differences in the rate of contraction of lesions.Some authors do not recommend surgery as a first‐line treatment for lymphatic malformation because of relatively high rates of nerve injury, surgical site infection, and recurrence. Diffuse lesions have a high growth rate and may involve extensive amounts of surrounding tissue, which makes resection particularly difficult.4Patient age, type of pathology, anatomic location, growth rate, depth, dysfunction, and deformity must be considered when planning treatment for a lymphatic malformation. When choosing surgery, the type of pathology, anatomic location, and depth are particularly important considerations. Acquired lymphatic malformation in an adult may proliferate in response to repeated irritation as a result of contact with the teeth. In our patient, the lesion was confined and did not involve adjacent vital structure. Surgery was chosen because the risk of complications was deemed to be minimal.None declared.AA: developed the concept and design of the study. KK: critically revised the manuscript for important intellectual content and gave the final approval of the version to be submitted. YI: drafted the article.


A five-year-old male presenting with progressive right facial swelling underwent multiple biopsies before being diagnosed with a polyostotic frontal-zygomatic primary intraosseous hemangioma. Intraosseous hemangiomas are rare, more frequently afflict adult females, and very rarely involve the orbit. Our case with bony destruction and surrounding soft tissue mass measured 5.3 cm in a child mimicked a more ominous malignancy. This case is unique with its rapid progression and largest reported size, leading to additional challenges such as difficulty in achieving an adequate tissue sample and in the surgical management with respect to significant blood loss in a small child. Primary orbital intraosseous hemangiomas in children are exceedingly rare –5. IntraA five-year-old male with no medical or ophthalmologic history presented with one month of a progressively enlarging right facial mass. There was no prior trauma. This painless mass was firm to palpation and measured 3 cm in greatest dimension on initial presentation. Computed tomography (CT) of the orbits revealed a lytic osseous-based mass arising within the right zygoma Figures . The lesBased on these results, the patient was referred to our oculoplastic service for further management. Ophthalmic examination revealed best corrected visual acuity of 20/25 in the right eye and 20/20 in the left eye with normal stereopsis. The right bony orbital mass was noted  with relIn children, a number of malignant orbital lesions can present with bone destruction such as Ewing's sarcoma, metastasis , Langerhans cell histiocytosis, leukemia, and rhabdomyosarcoma –5. AlthoPrimary orbital intraosseous hemangiomas in children are extremely rare. A recent case report and literature review identified 49 reported cases of zygomatic intraosseous hemangioma; only five of the patients were under the age of 18 at diagnosis and only seven were noted to have ocular findings . None ofClinically, these tumors often present as a subacute to chronic enlarging, firm, mass which may or may not be painful . OccasioFine-needle aspiration is technically difficult in these bony tumors and may lack sufficient diagnostic accuracy as our case required excisional biopsy for diagnosis. Needle biopsy can disrupt the thin-walled blood vessels, so the specimen is nonspecific. Biopsy specimens without incorporating the bony component can also be nondiagnostic. Histologic subtypes have been classified as either cavernous, capillary, mixed, or scirrhous on the basis of the size of the vascular spaces and amount of connective tissue within the lesion , 18. WhiTreatment is typically total surgical excision with or without preoperative embolization , 21–25.


Ophthalmologists and retina specialists may consider choroidal detachment if patients with rhegmatogenous retinal detachment present with choroidal elevation. That misdiagnosis may lead to inappropriate treatments, development of tumor cell dissemination, and eventual promotion of patient death. We report a case of a patient with rhegmatogenous retinal detachment associated with choroidal melanoma simulating choroidal detachment according to fundus findings.A 78-year-old Japanese woman with blurred vision in her right eye was referred to our hospital because of rhegmatogenous retinal detachment with complicated atypical choroidal detachment. Her intraocular pressure was normal with clear anterior chamber. Retinal detachment involving the inferior and nasal retina was observed, and a retinal hole was noted in the same quadrant. A small yellowish choroidal elevation was located in the inferonasal site. Gadolinium-enhanced magnetic resonance imaging revealed enhancement corresponding to the elevation, leading to the identification of a choroidal tumor. Enucleation of the patient’s right eye was eventually performed. The enucleated eye histologically demonstrated malignant melanoma.If hypotony or an inflammatory sign is absent, ophthalmologists should pay attention to the differential diagnosis of choroidal elevations observed in such patients. Coincidence of rhegmatogenous retinal detachment (RRD) with choroidal melanoma (CM) is a rare phenomenon, accounting for less than 1% of total CM cases . The pheDiagnosis with CM in RRD may be challenging in selected cases. A variety of publications regarding their rare coincidence have shown choroidal tumors to be relatively easy to identify, which were classified as medium- or large-sized tumors , 3. UnleA 78-year-old Japanese woman who complained of blurred vision in her right eye visited an ophthalmology clinic. Because RRD was suspected, she was referred to a nearby vitreoretinal surgery center. When RRD with choroidal elevation was noted in the center, the ophthalmologist evaluated CD as atypical. She was eventually referred to our hospital in June 2015. Her medical history revealed nothing of note. Ophthalmological findings demonstrated visual acuity of 4/20 in the right eye (OD) and 10/20 in the left eye (OS). The patient’s intraocular pressure was 12 mmHg OD and 16 mmHg OS. Slit-lamp examination demonstrated clear anterior chambers and senile cortical cataracts in both eyes. Mild pigmented anterior vitreous cells were noted OD. Fundus examination displayed retinal detachment involving the inferior and nasal retina, where a round retinal hole was noted OD  in a nearby hospital because she refused enucleation and other possible treatments. However, because she had severe ocular pain due to a persistent corneal ulcer, she eventually underwent enucleation in October 2015. Histological findings demonstrated typical mixed-type malignant melanoma with a low Ki-67-positive rate. However, liver metastasis developed about 1½ years after the enucleation. The patient was fine without systemic treatments based on her will in December 2017, and she is being observed in a nearby palliative care center.CD, which is involved with inflammatory pathogenesis , manifesClinical features in CM and CD coincident with RRD include their different frequency. Moreover, RRD associated with CD reveals hypotony with inflammatory signs such as increased flare levels in the anterior chamber and vitreous opacity in most cases. Hypotony is a useful finding to consider in the differential diagnosis; however, the findings of increased flare levels in the anterior chamber are not helpful if the tumor volume/size are medium or large in CM . FurtherWhen RRD complicating choroidal elevation without inflammatory signs or hypotony is seen, further testing with imaging modalities such as B-mode echography and Gd-enhanced MRI is needed to exclude CM. Those imaging findings clarify whether malignant solid tumor or CD causes choroidal elevations in patients with RRD.


Reactive lymphoid hyperplasia (RLH) is a rare and benign lesion found in organs of the gastrointestinal tract, skin, lung, orbit, and more rarely in the liver. Due to its similar appearance on imaging, it is hard to differentiate from primary liver malignancies. The following is a case report of a patient presenting with a suspicious liver lesion found to be RLH associated with primary biliary cirrhosis (PBC), after surgical resection.  A 54-year-old woman presented with nonspecific abdominal pain, and her workup included axial imaging of the abdomen that showed a suspicious lesion in her liver. After an extensive workup, which included a percutaneous biopsy, failed to confirm a diagnosis, a laparoscopic surgical resection was recommended.  RLH is a rare condition of the liver, presenting in a suspicious fashion and raising concerns for a primary liver malignancy. RLH should be considered in the differential diagnosis of small hepatic lesions in middle-age females in the absence of any significant risk factors for hepatocellular carcinoma (HCC). RLH tends to be associated with PBC of the liver.  RLH of the liver is a rare, hard to diagnose, benign lesion. When intrahepatic, it cannot be easily differentiated from primary liver tumors and frequently requires surgical resection for pathological diagnostic confirmation. Reactive lymphoid hyperplasia, a proliferation of benign, polyclonal lymphocytes, forming localized mass-like lesions, can present in many organs of the body and mimic primary or secondary malignancies . RLH, orWe present here a case of RLH in the left lateral section of the liver in a 54-year-old woman, requiring a laparoscopic left lateral sectionectomy for final diagnosis.A 54-year-old Asian American woman presented to her family physician with right side flank pain. She had no other symptoms, and her physical exam was normal. Laboratory testing showed elevated liver function tests including alkaline phosphatase and aspartate aminotransferase. An abdominal ultrasound showed a 1.5 cm hypodense lesion in the left lobe of the liver with associated porta hepatis lymphadenopathy. Computed tomographic (CT) scan revealed a 1.8 × 1.4 cm hypodense mass located in the left lateral section of the liver with minimal peripheral enhancement . MagnetiBased on her clinical presentation, imaging, and an indeterminate pathology report, she was seen at the hepatobiliary high-risk clinic and a laparoscopic left lateral sectionectomy of the liver was recommended. The patient had an uneventful hospital stay postoperatively and was discharged home on postoperative day 3.Her final pathology revealed nodular reactive lymphoid follicular hyperplasia (RLH) and evidence of primary biliary cholangitis (PBC), which was not diagnosed until final pathology was obtained.First reported by Snover et al., reactive lymphoid hyperplasia has been reported in many organs in the body and rarely in the liver , 7. MoreThe pathophysiology of RLH of the liver is not well understood. It was thought that reactive immune phenomena to various immune stimulants were implicated , 9. As mRLH is a histopathological diagnosis after surgical resection. The inaccuracy in preoperative diagnosis of RLH stems from the fact that these lesions are incidentally found on imaging studies done for other reasons, there is an absence of specific tumor markers, and last, a percutaneous biopsy of the inflammatory component would be nonspecific and the nature of the lesion is best determined through review of the overall morphology in the context of a resection specimen. Lesions are small and often misdiagnosed as malignant since they share features with hepatocellular carcinoma, specifically, being hypoechoic on ultrasound, hypodense on CT with variable enhancement characteristics, hypointense on T1-weighted and hyperintense on T2-weighted MRI .These similarities on imaging to HCC have sparked interest in further research into discovering any distinguishing features to help differentiate the two. There have been some reports of using “perinodular enhancement” as seen on RLH as a unique characteristic on CT, but this needs further investigation. In general clinical practice, a liver lesion workup is performed differently in different clinical backgrounds. Thus, in patients with no background liver dysfunction or cirrhosis or chronic viral hepatitis infection, RLH of the liver has to be indicated in the differential diagnosis, specifically when tumor markers like AFP are normal , 9.These lesions can also be PET avid lesions raising the suspicion for metastatic disease to the liver; thus, appropriate metastatic workup has to be performed for tumors that most commonly spread to the liver .On pathology examination, Faris and Saltztein defined RLH as propagating lymphoid cell follicles without atypia, having reactive germinal centers . It was Finally, it is known from the literature that no cases of recurrence after resection were found on follow-up imaging, and no cases have been treated with anything additional to a surgical resection; thus, its natural history has yet to be defined , 17. HowReactive lymphoid hyperplasia of the liver is a very rare disease of the liver, often found incidentally and often incorrectly diagnosed as hepatocellular carcinoma on preoperative workup. It has a predilection to middle-aged females, often associated with autoimmune diseases, specifically primary biliary cholangitis, or other malignancies. The definitive diagnosis, despite major advances in percutaneous biopsies and axial imaging techniques, remains a surgical resection diagnosis for appropriate morphological, immunohistochemical, and molecular studies. Patients with RLH of the liver have to be followed up closely after resection due to the rare incidence of malignant transformation.