A defect of the esophageal hiatus can lead to herniation of the stomach or other abdominal organs into the thoracic cavity, a condition called hiatal hernia. They constitute a rare clinical entity during infancy and childhood and their symptoms can be non-specific or subtle, making the diagnosis difficult even for experienced clinicians. In all cases, surgical treatment of the defect is necessary because of life-threatening complications.We present a rare case of a newborn with congenital paraesophageal hernia (CPEH) and microgastria, who was initially referred to our center with the diagnosis of esophageal atresia due to the inability to pass an orogastric tube beyond 15 cm from the gum margin. A contrast study revealed the CPEH. The patient underwent emergent surgery and has had no signs of recurrence until now.Although the diagnosis can be very tricky and mimic other conditions, a high level of suspicion should exist especially in patients with persistent symptoms of gastroesophageal reflux or recurrent respiratory infections. In neonates, signs and symptoms can be indicative of esophageal obstruction which should be ruled out with an upper gastrointestinal (GI) study. Hiatal hernia is one of the several known defects of the diaphragm and is well known to occur in adults. There are also a few cases of hiatal hernias described in the literature featuring younger patients. These cases present with non-specific symptoms such as vomiting, recurrent respiratory system infections, or failure to thrive but sometimes can have an acute clinical presentation with respiratory distress or gastric volvulus . CongeniHiatal hernias occur when the stomach protrudes into the thoracic cavity through a widened gap, usually due to a developmental defect of the diaphragm. There are four types of esophageal hiatal hernias ,2: type A full-term male neonate (37 weeks and two days gestation) with the prenatal diagnosis of polyhydramnios was delivered by elective cesarean section with no evidence of respiratory distress. During the first feeding attempt, the baby had a choking episode with perioral cyanosis, and due to inability to insert the feeding tube to the stomach, the patient was instantly referred to our center with the prominent diagnosis of esophageal atresia. The investigation including chest X-ray and contrast study Figure  revealedA laparotomy was decided and the patient was operated on the fourth day of life. The stomach was reduced in the abdomen and the hiatal defect was closed. A fundoplication could not be performed owing to microgastria. Thus, a gastrostomy tube was inserted to act as a gastropexy. The neonate began oral feeding the third postoperative day without incidents. Six weeks later, the gastrostomy tube was removed and since then the infant has normal growth with rare episodes of emesis.Paraesophageal hernias in children, either congenital or acquired, are uncommon. The embryologic basis of CPEH is inadequately understood but there are several hypotheses . During Patients with CPEH may surprisingly be asymptomatic or present with vague GI symptoms such as emesis and postprandial discomfort, recurrent respiratory infections, anemia, and failure to thrive. An assiduous medical history can also raise suspicion, especially in the asymptomatic child whose mother is worried about strange noises in the chest on swallowing, accompanied by chest symptoms or vomiting .Yousef et al.  found thPotentially life-threatening complications include gastric volvulus, bleeding, incarceration, obstruction, and gangrene or perforation. In a case series from Turkey , three oGiven the rare incidence and the vague nature of the symptoms, the diagnosis is often not initially suspected with the exception of the acute clinical presentation of the above-mentioned complications. The imaging evaluation begins with an anteroposterior and lateral chest X-ray (CXR). Karpelowsky et al.  confirmeUpper gastrointestinal contrast studies are the cornerstone of imaging as they can both confirm the diagnosis and also demonstrate complications. The pathognomonic findings are an 'upside down' stomach located intrathoracic while the GEJ usually remains intraabdominal . The rolAntenatal diagnosis of CPEH is described in some cases in the literature ,9. A fetThe diagnosis of CPEH is considered as an indication for surgical repair, regardless of the presence or absence of symptoms. A delay in surgery can lead to either symptom progression or complications that require emergency surgery with increased mortality ,8. A conRegardless of the approach, there are four cornerstones during the surgical repair of CPEH: 1) reduction of the hernia contents, 2) resection of the sac, 3) closure of the hiatal defect, and 4) an antireflux procedure. An antireflux procedure helps to both treat reflux disease that coexists in many cases and decreases the risk of recurrence . In our Paraesophageal hernia in children is a relatively rare entity and can occur at any age. It may be asymptomatic and be encountered incidentally or with vague symptoms such as recurrent chest infections or vomiting. In neonates, the situation can present with difficulty in inserting an orogastric feeding tube and can imitate esophageal atresia as in our case. Physicians caring for these patients should be aware of such a presentation as it has the potential for severe complications. Elective surgical treatment is mandatory shortly after the diagnosis due to the considerable morbidity and should include both hernia repair and an antireflux procedure.


Metastases to the brain from primary colorectal carcinoma are rare. Existing literature describing cranial nerve palsy from metastatic colorectal cancer is scattered. To our knowledge, we are the first to describe the combination of CN deficits V, VII, and XII as the initial presentation of colorectal malignancy. The authors present the case of a patient with no past medical history who presented with multiple cranial nerve deficits of the right trigeminal, facial, and hypoglossal nerves. MRI of the brain revealed a mass in Meckel's cave, which explained the involvement of the trigeminal nerve (CN V) but not the facial (CN VII) and hypoglossal (CN XII) nerves. Further workup revealed multiple cardioembolic strokes caused by nonbacterial thrombotic endocarditis (NBTE). Extensive workup for the cause of his NBTE and subsequent cerebrovascular events revealed colorectal adenocarcinoma. The relationship between thromboembolism and cancer was first described by Trousseau in 1865. Trousseau and colleagues reported a 15% incidence of clinically relevant thromboembolic events in patients with cancer and up to 50% in post-mortem studies . AdditioA 48-year-old man recently diagnosed with Bell’s palsy by his primary care physician presented to the emergency department with a four-week history of back pain and dysarthria, alongside right-sided facial numbness, dysgeusia, lagophthalmos, and blurred vision. He also reported intermittent nausea, a 20-lb unintentional weight loss, and intermittent subjective fevers. Upon examination, the patient had right-sided impairment of facial muscle function, including drooping of the brow and corner of the mouth, flattening of the nasolabial fold, and impaired closure of the eye and mouth. Protrusion of the tongue displayed right-sided deviation. His gag reflex, along with all other cranial nerves, was intact. Motor, sensory, and cerebellar examinations were unremarkable.Computed tomography (CT) scan without contrast, CT angiography, and CT perfusion of the head revealed no abnormalities. Magnetic resonance imaging (MRI), however, showed a 1.4 cm mass at the level of the right posterior margin of Meckel's cave Figure . MultiplMRI of the lumbar spine showed diffuse marrow replacement with multiple focal lesions throughout, and the patient was started on aspirin, clopidogrel, and high-intensity atorvastatin. A strong suspicion of the underlying metastatic process in the setting of his imaging findings and recent weight loss warranted further workup. A transesophageal echocardiogram showed a mass of approximately 1.2 x 0.5 cm on the atrial side of the anterior leaflet at the mitral valve Figure . The valOn hospital day two, the patient suddenly developed generalized left-sided weakness, a 30-second staring episode, emesis, and an ensuing change in mental status. On evaluation, the patient was hypoxic and subsequently placed on a non-rebreather. Urgent head and neck CT angiogram with perfusion revealed occlusion of the right middle cerebral artery (MCA) Figures , 2D, andThe thrombectomy tissue was thought to represent soft tissue emboli, but unfortunately, a detailed pathological examination was not performed. Due to the suspected relationship between his hypercoagulable state and ischemic stroke, the team opted for bone marrow biopsy, which revealed poorly differentiated metastatic adenocarcinoma likely secondary to colorectal cancer. Gastroenterology was consulted and discovered the primary mass via colonoscopy. The lesion was biopsied and diagnosed as poorly differentiated colonic adenocarcinoma. The evaluation of patients presenting with cranial nerve palsies is challenging as the differential diagnosis is broad. The presentation may be due to singular or multiple cranial nerve palsies, which can be sequential, discrete, unilateral, bilateral, painless, or painful. Skull base syndromes causing combinations of adjacent cranial neuropathies, such as Vernet, Schmidt, Collet-Sicard, Jackson, Tapia, and Villaret syndromes, have been well described . HoweverRegardless of the presentation, anatomic localization is the first step in evaluation. Possible locations of cranial nerve lesions include the brain stem, meninges, or base of the skull. Additionally, lesions may be extracranial, resulting in accompanied systemic symptoms . The larThere are myriad factors that may contribute to thromboembolism in cancer patients. For instance, the interaction between tumor cells and both monocytes and macrophage lineages results in the release of various pro-inflammatory cytokines, including tumor necrosis factors, interleukin-1, and interleukin-6, which damage the endothelial lining and thus convert it into a thrombogenic surface. Furthermore, the interaction between malignant cells and macrophages can activate platelets, factor XII, and factor X, thereby inducing clotting on the already hypercoagulable vascular lining. In addition, tumor cells release thrombogenic substances, including cysteine protease and tissue factor, which can activate factor X and factor VII to further stimulate the thrombogenic cascade . Along with the effects on the clotting pathway, the immobility and immunosuppressed state secondary to chemotherapy, radiotherapy, and other aggressive pharmacologic treatments often administered via intravenous access with central line placement can predispose patients with cancer to neurologic infection, which can also incite thromboembolic events . Among cNBTE, as described in the presenting case, is the most frequent cause of cerebral ischemia in patients with cancer, regardless of whether or not the stroke is the initial manifestation of cancer . Lastly,The most important issue in diagnosing cranial nerve palsy is whether it matches an expected pattern and whether it is "isolated." While it is conceivable for multiple cranial nerve palsies to have a microvascular cause, all patients with more than a single nerve palsy or with other neurologic findings must have an extensive workup (neuro-logic examination and MRI) before the diagnosis is confirmed. NBTE must be considered for patients with a background of malignancy and new onset of ischemic cerebral stroke. The diagnosis should depend on the distribution of occluded vessels, identification of thrombus, and exclusion of other possible causes. Healthcare providers must work collaboratively in these complex cases. The approach to managing NBTE should be directed at preventing systemic embolization with long-term anticoagulation and treating the underlying malignancy.


Surgical resection is the most effective curative management of benign rib tumors and carries an excellent prognosis. Due to complex anatomy and narrow field, higher rib resection is technically demanding and requires extensive dissection.We report a case of second rib tumor resection performed transthoracic under Da Vinci robot assistance. A 32-year-old male complained about increasing pain in the left anterior chest wall. After 3D reconstruction of CT, it showed a well-circumscribed fusiform lesion with a multi-component structure. Measured 17 × 6 × 4 cm and extended into the chest cavity to the depth below the pectoralis minor muscle. The patient underwent robotic-assisted trans-thoracic second rib resection. At four weeks of outpatient follow-up, the patient reported no pain and uncomplicated wound healing.This minimally invasive approach offers optimal visualization and tissue manipulation while dramatically decreasing the possibility of collateral damage, hence ensuring fast function recovery. To the best of our knowledge, these kinds of procedures are rarely reported in detail. Fibrous dysplasia of the rib is the most common benign chest wall tumor, it can be monostotic or polyostotic . It is uA 32-year-old male complaining of increasing pain in the left anterior chest wall and occasional numbness of fingers was referred to a local tertiary hospital for further diagnosis when a routine chest x-ray showed a large tumor in the left chest cavity. He had no notable past medical history and no smoking history. On admission, other physical examinations revealed no abnormalities. Chest CT and 3D reconstruction showed a well-circumscribed fusiform lesion with a multi-component structure, measuring 17 × 6 × 4 cm in the left second rib protruding into the chest cavity and out to deep space below pectoralis minor muscle. No pleural effusion and metastasis were noted . The camera port was placed in the mid-axillary line in the eighth intercostal space, and two robot arms were placed in the fourth intercostal space at the anterior axillary line and eighth intercostal space just below the tip of the scapula Fig. . The Da rly Fig. .Fig. 2InThe postoperative course was uncomplicated, and the chest tube was removed on the 2nd postoperative day, patient was discharged with minimal discomfort, no paresthesia, and normal function of the left arm on the third postoperative day.At four weeks of outpatient follow-up, the patient reported no pain, respiratory complaints, or uncomplicated wound healing. Clinical examination revealed no brachial plexus neuropathy, Horner’s syndrome, or winged scapula.Fibrous dysplasia (FD) of the rib is one of the more frequent pathologies of benign chest wall tumors, typically presenting as a palpable mass in the third to the fourth decade of life. Fibrotic immature bone matrix formation in the replacement of normal marrow and disorganized bony trabeculae and spindle cells are the hallmarks of pathological process , which iRib tumors are traditionally resected via a posterolateral thoracotomy. The exposure is relatively easy for the sixth and lower ribs, whereas visualizing ribs higher than the third rib requires either a deep dissection via either a trans-axillary incision, which requires deep dissection and relatively normal anatomy for visualization of costal and posterior margins, or an extended dissection via conventional posterolateral thoracotomy and adjacent structures . Minimally invasive, trans-thoracic approaches to rib resection, predominantly first rib resection for thoracic outlet syndrome (TOS), have been brought up for their superior visualization of important structures and a smaller incision . HoweverIn robot-assisted high rib resection, the first rib resection has been reported more frequently. Schmid et al.  reportedOur method can be easily reproduced and can be considered for any benign lesion of the ribs, particularly larger ones and higher ones that may benefit from the trans-thoracic approach. The reduced incision, thus postoperative pain, and quicker functional recovery are beneficial for patients. More procedures like this should be performed in order to overcome the learning curve and verify the benefit of the robotic approach.


Acute bowel diseases are responsible for more than one third of subjects who were referred to the emergency department for acute abdominal pain and gastrointestinal evaluation. Gastrointestinal ultrasound (GIUS) is often employed as the first imaging method, with a good diagnostic accuracy in the setting of acute abdomen, and it can be an optimal diagnostic strategy in young females due to the radiation exposure related to X-ray and computed tomography methods. The physician can examine the gastrointestinal system in the area with the greatest tenderness by ultrasound, thus obtaining more information and data on the pathology than the standard physical examination. In this comprehensive review, we have reported the most relevant indications and advantages to using ultrasound in the investigation of abdominal acute pain. Acute abdomen is a pathological state that requires a fast diagnosis and subsequent specific therapy. Gastrointestinal ultrasound (GIUS) or computed tomography (CT) are commonly employed to evaluate the reason for acute abdominal pain, thereby planning a specific surgical intervention if necessary or avoiding unnecessary surgery ,2,3.Acute bowel diseases are responsible for more than one third of subjects who were referred to the emergency department for acute abdominal pain ,2. UltraA general approach to GIUS consists in a systematic scanning of the gastrointestinal tract ,8,9. FirThe study of both the large and small intestine usually starts in the right iliac fossa, as the cecum, the ileocecal valve and the terminal ileum are typically located in this area, over the anatomical landmarks represented by the iliopsoas muscle and the right iliac vessels ,8,9,10.The small bowel is the most difficult GI tract to examine; the terminal ileum is the only small bowel loop and can be identified due to its fix position in the right iliac fossa and the presence of specific anatomical landmarks. Other segments of the small bowel are difficult to examine, and ultrasound can provide a general overview of the jejunal and ileal loops through a systematic scanning approach that involves parallel lines cranially and caudally through the abdomen and by exerting enough probe pressure to display the deeper part of the abdomen ,11.The small bowel is characterized by the presence of valvulae conniventes, which decrease in number and height from the proximal jejunum to the distal ileum, and they are best visualized in fluid-filled bowel loops . The smaIn order to study the large bowel, after the cecum has been identified, the probe is moved in the distal direction following the ascending colon, transverse colon, descending colon, sigmoid colon and the rectum. The colon is located along the peripheral areas of the abdomen, with the ascending and descending colon fixed to the retroperitoneum on the right and left, with a vertical course anterolaterally to the iliopsoas muscle. The location of the transverse and the sigmoid colon can significantly vary due to the different length of the mesocolon. The transverse colon usually has a horizontal course and can often be displayed just behind the gastric antrum, but it may also descend to the lower abdomen in cases of an elongated mesocolon. The sigmoid colon is usually seen into the pelvis, above the urinary bladder, but it can also cross the midline to the right iliac fossa or even extend up to the liver. The rectum may be seen behind the bladder by low-frequency probe ,13.The right colon is usually filled with stool and gas, and it allows the visualization of haustration, which gives the colon profile its typical polycyclic appearance in the longitudinal view and limits the bowel wall study to the superficial side. On the contrary, the left colon is often seen in a contracted condition, so that haustration is not usually visible and the bowel wall is better displayed .The normal diameter of the colon is up to 5 cm, but the cecum could be larger in size . The appThe bowel wall consists of five sonographic layers when examined with a high frequency transducer (above 5 MHz). The sonographic layers do not exactly correspond to the histological ones, thus representing a combination of interface echoes of the histological layers. Starting from the lumen, the first layer (hyperechoic) is the interface between the mucosa and the lumen, the second layer (hypoechoic) to the mucosa, the third layer (hyperechoic) to the submucosa, the fourth layer (hypoechoic) to the muscolaris propria and the fifth layer (hyperechic) to the interface echo between the muscolaris and the serosa  Figure .According to the European Federation of Societies for Ultrasound in Medicine and Biology (EFSUMB) Recommendations and Guidelines for Gastrointestinal Ultrasound, bowel wall thickness should be measured perpendicular to the wall, from the interface between the serosa and the proper muscle to the interface between the mucosa and the lumen. A bowel wall thickness less than 2 mm could be considered as normal in a usual filling condition, for both the small and large intestine, with exceptions represented by the duodenal bulb and the rectum .The following sections will analyze the pathological ultrasound findings divided by type of disease.According to the EFSUMB guideline, ultrasound should be employed to evaluate inflammatory bowel diseases (IBD) at first presentation, and to examine its extent, activity and possible complications . GIUS haCompared with the alternative options, namely CT and Magnetic Resonance (MR), GIUS has the advantages of being widely available, less expensive, easily repeatable and radiation free .On GIUS, the most frequent pathological findings are bowel wall thickening , changesThe presence of a thickened bowel wall is the most important US finding in CD to detect the disease and is the most frequent index considered in scores used to evaluate IBD activity ,23. In tIn the acute phase of CD, the wall thickening is caused by transmural edema: the wall layers are often well defined, and it is possible to identify a typical aspect of a “target sign” on a transverse scan. A few studies have focused on the thickening of each wall layer in CD, finding a prevalent thickening in the submucosal layer, rather than the mucosal and muscolaris layers, in active CD ,28. On tThe measurement of bowel wall thickness can be completed by a semi-quantitative evaluation of its vascularity by using color or power Doppler at the level of the most thickened segments, which is correlated with disease activity .In addition to parietal features, GIUS allows the visualization of CD extraintestinal features, such as mesenteric fat hypertrophy, mesenteric loco-regional lymph nodes and abdominal free fluid . MesenteGIUS can also detect complications of CD. Strictures are detected on US as a stretch with thickening of the wall and thinning of the lumen: there could be a dilation of the upstream tract, with a possible accumulation of liquid and/or gas. Moreover, fistulas can be observed as hypoechoic tract connecting two loops to other structures, such as the skin or bladder, and are usually characterized by vascularized walls. Abscesses could be also detected: they are represented by hypo-anechoic lesions with liquid content. Phlegmon could also be found in patients with CD, and it is characterized by hypoechoic lesions with faded edges in the absence of colliquation .In that setting, CEUS could represent a valid tool for a more specific diagnosis. Eventually, GIUS can also detect signs of perforation, characterized by the presence of free air in the subdiaphragmatic region or intraperitoneal liquid mixed with air bubbles.Concerning ulcerative colitis, the disease typically involves the colic walls, which appear continuously and concentrically thickened (over 4 mm) with an absence of haustrations . The mosAcute appendicitis is one of the most frequent abdominal emergencies worldwide, with an incidence of approximately 100 per 100,000 person-years and with a lifetime prevalence of 7–8% ,32. The Despite its high incidence, the diagnostic approach to acute appendicitis is still debated: some guidelines employ scoring systems, others suggest physician clinical assessment alone, and some guidelines include standardized imaging ,35,36. AThe role of GIUS in cases of suspected acute appendicitis is to confirm the diagnosis or to rule it out by demonstrating a normal appendix over its entire length, and to exclude an alternative cause of abdominal pain . MoreoveIn cases of suspected appendicitis, it is recommended to search the inflamed appendix at the point of the greatest abdominal pain, pointed out by the patient, using graded compression . AlternaIn patients with acute appendicitis, the most accurate GIUS finding is a maximum outer diameter of >6 mm, with a sensitivity and specificity of 98% ,44. AddiSecondary US signs related to the inflammation of the surrounding tissues are described, such as peri-appendiceal fluid, mesenteric lymphadenopathy and hyperechoic peri-appendiceal tissue (mesenteric fat hypertrophy) ,47,51.In clinical practice, only the combination of different GIUS signs allows the diagnosis of acute appendicitis to be reached ,51. The The importance of complicated appendicitis (namely gangrenous appendicitis and perforated appendicitis) in the identification of signs relies on the consequences for its management, as the confirmation of complicated appendicitis usually excludes a conservative treatment .The loss of stratification of the appendix wall, and particularly the loss of the echogenic submucosal layer, is the main GIUS sign of gangrenous appendicitis, which can be associated with the lack of vascularization on color Doppler ,54.Extraluminal gas, complex fluid collection  and extraluminal fecaliths are GIUS signs associated with perforation ,51. On tColonic diverticula are a common finding in the general population, with a prevalence of <5% under the age of 40 and >65% over the age of 80 . Acute dThe clinical presentation of acute diverticulitis includes prolonged lower abdominal pain  or abdominal tenderness, changes in bowel movements, fever and increased laboratory inflammatory markers . HoweverRegarding the preferred imaging technique, CT scan is sometimes still considered the gold standard . RecentlOnce the sigmoid colon has been identified ventral to the left iliac artery, it can be searched distally and proximally to the descending colon. In cases of suspected acute diverticulitis, an alternative approach consists in starting the exam at the point of maximum tenderness, using the graded compression technique ,62.Diverticula in the colon are visualized on ultrasound as outpouchings of the wall that normally contain echogenic material, represented by gaseous interfaces, feces or fecaliths, and characterized by acoustic shadowing  Figure . In sigmThe diagnostic GIUS criteria for acute diverticulitis include at least two of the following: short-segmental bowel wall thickening (>5 mm), the presence of an inflamed diverticulum in the wall-thickened area and pericolic tissue changes. Those signs are usually present at the area of the greatest pain, induced by probe compression ,56,63.The aspect of an inflamed diverticulum may range between hypoechoic (37% of cases), predominantly hyperechoic (4%), hyperechoic with surrounding hypoechoic rim (41%) and hyperechoic with acoustic shadowing (18%)  Figure . PericolAbscess, fistulas, perforation and stenosis are common complications of acute diverticulitis. The sonographic appearance of diverticular abscesses is highly variable: the most typical aspect consists of a collection of hypoechoic fluid, but sometimes they show a prevalent hyperechoic aspect owing to echogenic solid or gaseous content . CEUS isFistulas may extend to the nearby bowel loop, urinary bladder or uterus. They can be identified as hypoechoic bands with or without central gas bubbles . The preThe typical sign of perforation is the presence of gas bubbles outside the bowel loops. In cases of free perforation, a major complication of acute diverticulitis, the free air is displayed as a hyperechoic line along the hepatic surface or the peritoneal line ,63.Bowel obstruction is a common cause of acute abdominal pain leading to emergency department admission. Small bowel obstruction (SBO) accounts for approximately 80% of cases of mechanical intestinal obstruction . SBO canThe clinical presentation of bowel obstruction depends on the location and the cause of the obstruction, and often includes abdominal pain, nausea, vomit, the abolished passage of flatus and/or stools and abdominal distension. Indeed, clinical presentation is non-specific, and imaging is mandatory to confirm the diagnosis and to distinguish between mechanical and functional SBO, to identify the site and cause of the obstruction, and to assess the risk of complications  and the appropriateness of non-surgical management ,70.Among the available imaging techniques, GIUS shows a similar accuracy than CT , and higher than X-ray in the detection of SBO .A recent meta-analysis by Lin et al. evaluating fifteen studies showed that GIUS display a sensitivity and specificity of 92% and 93%, respectively, in the diagnosis of SBO . Indeed,A three-step approach is suggested to establish the presence of bowel obstruction and define the pathological segments. Firstly, the epigastrium should be scanned to assess the stomach; a trans-lienal view can be added to display the gastric fundus . In caseSBO can be diagnosed in the presence of dilated (up to 2.5–3 cm) bowel loops detected to be at least 10 cm in length . The appIncreased peristaltic “to-and-fro” movement of the bowel loops (which tends to decrease in advanced forms) is a typical finding of acute mechanical obstruction, with the visualization of collapsed loops beyond the stenotic tract . The ideIt is relevant to consider the alteration of the peristalsis as a criterion for the diagnosis of mechanical ileus , which cIf the obstruction persists, it could enhance the endoluminal pressure with an increase in the liquid content between the mesenteric recesses (‘sign of the thong’)  and thenLBO is detected in ultrasound as a clear transition from a dilated (>4.5 cm) to a non-dilated part of the colon, often with liquid content in the right colon and solid stools in the left colon. Otherwise, it is often not possible to measure the diameter and to obtain a wide visualization due to the presence of gas in the obstructed colon.Gastrointestinal perforation is a rare cause of acute abdominal pain in emergency departments . Peptic The sudden onset of severe abdominal pain is the main symptom, but patients can report only mild symptoms, depending on the perforation site and the amount of leakage of the intestinal contents .The detection of free gas in the abdominal cavity (pneumoperitoneum) is the most relevant finding suggestive of gastrointestinal perforation . Convex US signs of pneumoperitoneum should be searched between the liver and the abdominal wall, and they are represented by an enhancement of the peritoneal stripe and hyperechoic lines with reverberation and ringdown artifacts (“dirty shadowing”). Air artifacts (gas) movement, according to patient position (shifting phenomenon), is very suggestive of pneumoperitoneum . DiffereUltrasound is characterized by a better sensitivity than abdominal X-ray (86% compared with 76%) for the diagnosis of pneumoperitoneum . GIUS isIschemic colitis can develop as a consequence of arterial or venous embolism or thrombosis ,91,92. LNon-occlusive mesenteric ischemia (NOMI) is most commonly caused by primary mesenteric arterial vasoconstriction. Most cases involve the spasm of the branches of the superior mesenteric artery (SMA), which supply the small intestine and proximal colon. In GIUS, the walls of the ischemic colon are thickened, hypoechoic and with altered stratification. In the acute phase, there are few color doppler signals on the wall, while moderate hypervascularization can be present after the reperfusion of the bowel. In CEUS, the pathological bowel segments are poorly enhanced and thus perfused .Superior mesenteric vein occlusion can induce edema and the bleeding of the mucosa. The homogeneously hypoechoic thickening of the wall, decreased peristalsis, intraluminal secretions and peri-enteric free fluid are typical sonographic findings . Bowel wIschemic colitis (IC) is the most frequent form of bowel ischemia and the second most frequent reason for lower gastrointestinal bleeding. IC may be characterized by two clinical presentations: a gangrenous form with transmural necrosis and worse prognosis, and a transient form with a segmental involvement of the mucosa or submucosa only, which is usually reversible .In ultrasound, the bowel walls are thickened circumferentially and with a hypoechoic echostructure and a loss of mural stratification. Flow signals of the bowel wall can be not present or decreased during the early phase . Those sAcute bowel diseases are often responsible for acute abdominal pain in the emergency setting. GIUS is widely available, easily accessible, low-cost, noninvasive and is characterized by good diagnostic accuracy in patients with acute abdomen. Therefore, GIUS represents a “bed-side” and non-X ray technique that could complete physical examination, giving the physicians the opportunity to integrate clinical data, signs, symptoms and immediately available imaging. More studies are needed to better define the role of GIUS in the emergency setting and to compare sonographic investigations with X-ray techniques in terms of availability, diagnostic accuracy, costs, and the time it takes to reach the diagnosis.


Fine-needle aspiration cytology (FNAC) is a safe and well-tolerated procedure with high sensitivity and specificity. Serious complications, such as large hematomas, are very rare and should be promptly identified. We present an unusual case of a 71-year-old woman with a massive cervical hematoma that developed near the thyroid capsule after the FNAC of a nodule. CT showed a hematoma measuring 38 x 34 mm, causing deviation of the laryngotracheal axis. The patient was admitted to the intensive care unit for intubation and surgical drainage. This case illustrates that FNAC, despite being considered a safe procedure with infrequent complications, carries the risk of acute life-threatening events that should be taken into account. Fine needle aspiration cytology (FNAC) is a safe and well-tolerated procedure, with a relevant role in assessing the risk of malignancy of thyroid nodules -4. Most A 71-year-old, euthyroid woman was referred for evaluation of a thyroid nodule. She presented with a 19-mm hypoechoic solid oval nodule, with normal vascularization, without microcalcifications, in the lower pole of the left lobe with a score of 4 in the European Thyroid Imaging Reporting and Data System (EU-TIRADS 4). Her medical history was significant for type 2 diabetes mellitus, hypertension, and dyslipidemia. She was chronically medicated with metformin 500 mg, olmesartan 10 mg, and simvastatin 10 mg. The patient denied taking anticoagulants or antiplatelet drugs. No pre-procedure lab testing was performed. She underwent aspiration cytology of the nodule with a 25 G needle and did not comply with the instructions, having swallowed and spoken during the puncture. The procedure was interrupted with no immediate complications. However, a few hours later, she reported sudden cervical swelling with progressive worsening and local pain and denied dyspnea, fever, or stridor.Physical examination revealed edema and stiffness in the anterior cervical region with preserved mobility. There was no subcutaneous emphysema. The oxygen saturation was 100% on room air; her pulse was 90 beats/minute and her blood pressure was 141/69 mm Hg. Hemoglobin, platelets, and coagulation were normal. CT revealed a well-defined hypodense homogeneous mass consistent with a hematoma, adjacent to the left lobe of the thyroid measuring 38 x 34 mm, causing deviation of the laryngotracheal axis contralaterally ; she was started on amoxicillin and clavulanic acid 875 mg/125 mg every 12 hours. After one week of treatment, there was no improvement in her cervical edema or change in the appearance of the hematoma on CT. Thus, surgical drainage of the hematoma was performed. No bleeding points were identified. After surgery, she progressively recovered and there was complete resorption of the hematoma. After extubation on the 13th day, neurological deficits were noticed  and a diagnosis of left lateral frontal cortico-subcortical stroke was made. The hematoma may have caused a restriction in blood flow by compressing the surrounding structures, enough to completely block the blood flow to the cerebral cortex resulting in a stroke. Our stroke unit initiated a rehabilitation program and after discharge, she recovered almost all neurological deficits. Cytology was compatible with a benign follicular nodule (Bethesda category II). Post-FNAC serious adverse events seem to be rare, but a systematic record of these does not exist in the literature . A neck The presence of factors such as hemorrhagic disorders, anticoagulant/anti-platelet drugs, and hypertension increases the susceptibility to hemorrhagic complications ,6. The rThis case intends to illustrate the adverse effects that a relatively simple procedure such as FNAC can have, with complications that, although rare, can be serious. Patient education and collaboration before the procedure are also very important. In the case of a massive cervical hematoma, airway compromise can be fatal, requiring early and appropriate intervention.


Moyamoya disease is a rare idiopathic disease characterized by progressive stenosis and collateral development of the distal internal carotid arteries. It is mainly seen in East Asia and is the most common cause of stroke in Asian children. However, it is rare in the Indian subcontinent. We present three exciting cases of moyamoya disease with varied clinical presentations in one pediatric, one young adult, and one older patient. Moyamoya disease (MMD) is a rare progressive idiopathic cerebrovascular disorder characterized by progressive narrowing of the distal internal carotid arteries (ICA) or arteries around the circle of Willis . The devCase 1A 15-year-old boy presented to the hospital with three episodes of generalized tonic-clonic seizure for one day. The MRI brain showed no evidence of infarct or hemorrhage . Old imaging reports were unavailable.An MRI brain was done, which showed a large area of chronic infarct with gliosis and encephalomalacia involving the right frontal lobe, right insular region, and right-sided genu of the corpus callosum Figure  A and B,A CT Angiogram revealed occlusion of the M1 and M2 segments of the bilateral MCAs with multiple collateral formations around the occluded M1 and M2 segments, particularly on the left side  score of 3. She was a known hypertensive and diabetic on medication.On the CT brain, acute intraparenchymal hemorrhage was seen in the anterior aspect of the left thalamus with extensive intraventricular hemorrhage extension into all the ventricles Figure  A. On CTThe patient was intubated and kept on mechanical ventilation. Craniotomy was done and an external ventricular drain was placed in the right lateral ventricle. Follow-up CT Brain after four months showed complete resolution of the hemorrhage with residual gliosis and no hydrocephalus, and clinically there was no focal neurological deficit.Moyamoya disease is primarily an idiopathic disease. It is seen mainly in East Asian countries. Around 10% to 15% of MMD cases may be familial. Male-to-female predominance is 1:1.8, while it is 1:5 for familial cases . It is oThe MMD is characterized by irregular hyperplasia of tunica intima with waving of the internal elastic lamina of affected vessels on histopathology. Thinning of tunica media may result in pseudoaneurysms, later causing hemorrhage. The lumen may collapse resulting in thrombosis leading to ischemic stroke. Although classically described as affecting anterior circulation, over 50% of cases also have involvement in the posterior circulation. This results in diffuse cerebral atrophy, but it tends to mainly affect the regions supplied by anterior circulation. It can be demonstrated on MRI perfusion-weighted imaging where MMD will show reduced perfusion in hemispheric deep white matter with relatively increased perfusion in posterior circulation territory -5.The MMD is commonly seen with bimodal age distribution, with a peak around five to 10 years in children and another peak in the fourth decade in adults. However, clinical presentation differs: whereas adults commonly present with infarcts or intracranial hemorrhage, children mainly will have migraine, seizures, or a history of transient ischemic attack (TIA), as seen in our cases. Moyamoya disease in children can also manifest with poor feeding, developmental delay, and chorea due to brain ischemia but intracranial hemorrhage is rare in children. It has been seen that seizures and epileptiform electroencephalogram (EEG) changes are common in patients with MMD, as seen in our cases. In children, the re-build-up of slow waves at the end of hyperventilation has been said to be characteristic of MMD ,6. HyperThe MRI brain with contrast and neck and cerebral angiography (MRA) along with a CT angiogram is the common diagnostic tool used to diagnose MMD. The MRA will show classic narrowing of the distal internal carotid artery and/or arteries around the circle of Willis with collaterals. Multiple tiny dot-like flow voids can be noted in the basal ganglia region  on T2WI and T1WI that enhance on contrast administration . Bright An MRI vessel wall imaging can distinguish MMD from moyamoya syndrome. Moyamoya disease typically shows stenosed distal ICA with no significant contrast enhancement without outer wall remodeling as compared to atherosclerosis  or vasculitis  .Digital subtraction angiography of cerebral arteries is considered the gold standard -7. MoyamBased on angiography, Suzuki staging was developed in 1969 and is still in use today . HoweverThe CT brain usually shows diffuse cerebral atrophy (predominantly involving the anterior circulation territory) or may demonstrate the presence of intracranial hemorrhage. The CT angiography demonstrates similar findings as seen on MRA. A Xe-133 CT may show reduced cerebral reserve with acetazolamide challenge Before making the diagnosis of MMD, it is important to exclude conditions that may lead to moyamoya syndrome. No effective medical treatment for MMD exists yet. Anticoagulation can help to correct/control prothrombotic states.Surgical intervention is usually required for MMD treatment. Indirect bypass surgery  is more commonly performed in children , whereas direct bypassing like superficial temporal artery and middle cerebral artery (STA-MCA) is more commonly done in adults ,8.Moyamoya disease has bimodal presentation, commonly presenting with ischemia and stroke in pediatric patients and with hemorrhagic manifestations in adults. It is a chronic pathology and it is important for the neurologist and radiologist to be well-versed in clinical manifestations and imaging features of this condition for early diagnosis and management.


Plantar fasciitis is a common cause of heel pain, and the disorder is generally self-limiting after adequate conservative treatment. When conservative treatment is unsuccessful, surgical release is an effective treatment option. Here we report a case of iatrogenic plantar fascia rupture after surgical release for treatment of recalcitrant plantar fasciitis. Preoperative MRI revealed a 4.2 cm gap between the distal fascia stump and the calcaneal tuberosity in the sagittal view at 8 months post-injury. To circumvent the possibility of rupture site retear or poor tissue healing by direct repair, we used tendon allografting for the reconstruction of the chronic plantar fascia rupture. The patient gradually recovered after the surgery. Complications of plantar fascia rupture after surgical release is a potential risk but rarely observed. Chronic plantar fascia rupture with medial arch collapse is difficult to treat. We used a tendon allograft to reconstruct the plantar fascia, restoring its function and mechanical strength. After 5 years of follow-up, no complications were reported, and magnetic resonance imaging indicated the reconstructed plantar fascia tissue to be in good condition. Plantar fasciitis is a highly common cause of heel pain. The proposed etiologies are overload and overuse. Conservative treatments include physical therapy, use of a heel cushion or insole, and night splinting. Local steroid injection and prolotherapy are also indicated as alternative treatments ,2. SurgiHowever, excessive release may lead to complications such as lateral column syndrome, medial arch collapse, and even iatrogenic plantar fascia rupture ,4. PlantHerein, we report an iatrogenic total plantar fascia rupture reconstructed with tendon allografting. After a 5-year follow-up, the patient’s American Orthopedic Foot and Ankle Society (AOFAS) score was 85 with obvious improvement, and magnetic resonance imaging (MRI) findings indicated that his reconstructed plantar fascia was in good condition .2) with hypertension under regular control with Amlodipine but otherwise healthy presented to his primary care physician with right heel pain. The pain was located on the bottom of the foot near the posteromedial heel. The patient reported the particular pain when stepping out of bed in the morning or after sitting for a long period. After the diagnosis of plantar fasciitis, he received conservative treatments including physical therapy, shoe modification, and heat pack application. Following the failure of conservative treatments, he underwent open release of the right plantar fascia at a local hospital.A 61-year-old obese man  see .A plantar-flexed short leg splint was applied thereafter. Splinting protection was removed 4 weeks postoperatively, and a walking boot was utilized for assistance with walking and partial weight bearing. Full weight bearing was permitted 2 months postoperatively. No complications such as infection were found. Two years after the operation, the patient’s AOFAS score was 85. The allograft was not rejected, and MRI showed good thickness and continuity of the repaired plantar fascia see .Plantar fasciitis is a common disorder and is the cause of 11% to 15% of cases of foot and ankle discomfort in daily practice . PlantarPlantar fascia rupture is a rare disorder that can be distinguished into acute and acute-on-chronic rupture . The risHowever, this type of repair should be undertaken on the reparable fascia tissue of the distal stump with no obvious fascial gap between the calcaneal tuberosity and distal fascia stump. In this case, however, we noted a 3 cm gap between the ruptured end and the calcaneal tuberosity even after plantarflexion. This condition made repair challenging. Overtightening of a ruptured fascia end to ensure direct contact of the stump with the calcaneal insertional site can increase the risk of fascia re-rupture or poor tissue healing. Because the result was unpredictable, we chose a tendon allograft for the plantar fascia reconstruction. Although no study has reported on the reconstruction of a plantar fascia rupture with a tendon allograft, studies have reported that allograft reconstructions in chronic Achilles tendon ruptures and quadriceps tendon ruptures yield good clinical outcomes ,18. An a


FN). These soft tissue injuries usually appear on the shins, thighs, breasts, arms, and buttocks. FN is mainly caused by trauma and may be associated with other conditions such as pancreatic disease. FN arising after intramuscular injections is uncommon and usually appears as firm, encapsulated, mobile, nontender, and solitary or multiple subcutaneous nodules. We report an interesting case of FN caused by intramuscular injections of cefazolin and meglumine antimoniate (MA) in a 38‐year‐old female patient. MA is regarded as the first‐line systemic treatment for cutaneous leishmaniasis (CL). However, these drugs are not devoid of various potentially adverse reactions.Panniculitis has various etiologies. One of the less common causes is trauma and hence traumatic fat necrosis ( Intramuscular injection (IM) into the gluteal muscles remains a common route of medication but may lead to complications. Although rare, and with very few published similar case reports, it remains possible that FN resulting from meglumine antimoniate (MA) injections is underrecognized and underreported. It is important for healthcare professionals to be careful of fat necrosis as a possible side effect of MA injections and to elaborate adequate imaging investigations and surgical management in those patients with localized and persistent superficial nodular pain and tenderness at injection sites. We report an interesting case of subcutaneous FN caused by repetitive IM injection in a 38‐year‐old female patient referred to our department for the management of cutaneous leishmaniasis. Most cases are caused by trauma, although some have been described following injection therapy. It is a benign nonsuppurative inflammatory disease of adipose tissue. It is a rare condition, affecting all ages.Herein, we present a distinctive case of FN occurring after intramuscular injection of meglumine antimoniate and cefazolin.2A 38‐year‐old woman was admitted to our department with asymptomatic multiple ulcerative nodules of 1‐month duration. Her medical history was unremarkable. Dermatological examination revealed an ulcerocrusted nodule on the right leg and the left forearm and multiple subcutaneous nodules extending along the line of lymphatic vessels. The patient also had a paronychia of the big toe Figure  and an i3First described on the breast in 1920, FN is a benign nonsuppurative inflammatory disease of adipose tissue. It is an uncommon, self‐limited panniculitis of traumatic origin in newborns, while it mainly affects women after a breast surgery.In fact, the fat is composed of microlobules of fat cells; each microlobule is serviced by a blood vessel. In situations where there is pressure on the fat compartments, they burst and the surrounding septa and vessels rupture, leading to the damage of the fat cells. The damaged fat cells are transformed into glycerol and fatty acids.Clinically, FN presents as indurate painful deep nodules of the subcutaneous tissue covered by a normal skin or an “orange‐peel” appearance, local depression, or discoloration of the skin.FN has a similar imaging appearance as mature adipose tissue. Ultrasonographic image displays a hypoechoic appearance in most cases (like ours). However, FN of the trunk and in extremities are not fully characterized.Intramuscular injection (IM) into the gluteal muscles represents a common route of medication, and it may lead to various complications. A retrospective study including 32 patients who required surgical treatment for local complications of buttock injections in children was made at the Taegu Fatima Hospital during a 7‐year 9‐month period. They observed local complications such as acute inflammation, cellulitis and abscess (71.9%), FN (21.9%), and injection granuloma (6.2%).Chemical agents such as vasopressin, human growth hormone, triamcinolone, and insulin are also mostly reported.In our patient, FN was induced by IM injections of cefazolin and compounded by IMMA. Several cutaneous side effects of these molecules were previously reported. First, MA is known for its different adverse drug effects: either general reactions (including rash and urticaria) or local ones . According to the large series of Beheshti et al.,The diagnosis of traumatic panniculitis was first proposed in our patient, hence the prescription of colchicine. Given the resistance, a cutaneous calcinosis or foreign body granuloma was suspected. An excision confirmed the diagnosis of FN. To the best of our knowledge, there were no previous case reports of FN post‐cutaneous leishmaniasis treatment.Histopathology findings in FN are polymorph and depend on the stage of the disease. At early stages, FN with cystic spaces and numerous neutrophils in the adjacent fat is seen. Later on, predominant cells become lymphocytes, histiocytes, and multinucleated giant cells with lipid vacuoles in some cells. Finally, fibrosis prevails in histology.Since FN is a self‐limiting condition, a conservative approach can be followed. Surgical intervention is required only in patients with intractable pain and complications4We report an original case of FN. It is the first one described after IM cefazolin injections and compounded by repetitive IMMA. We believe that the injections on the superficial fat tissue in an obese patient compromised local vascularization and caused a FN with a cystic degeneration and a foreign body reaction. Although cutaneous leishmaniasis is a benign parasitic infection, lesions can lead to disability and disfiguring scars. Systemic treatment remains the main treatment in these cases. Follow‐up and early detection of adverse drug reaction of IMG is of a good help for better management.Dr Kouki Chaima wrote the manuscript and is the guarantor of the content of the manuscript, including the data and analysis. Dr Amouri Mariem analyzed, interpreted, and critically revised the data. Dr Hammami Fatma and Bahloul Emna contributed to interpretation of data and revision of the manuscript. Dr Slim Charfi provided the anatomopathological figures. Dr Hamida Tuki and Dr Boudawara Tahya contributed to the final approval of the version of the manuscript to be submitted.None.The ethics statement was approved.The consent statement was approved by all authors. Written informed consent was also obtained from the patient to publish this report in accordance with the journal's “patient consent policy.”


Although postoperative chylothorax following lung cancer surgery is rare, it is a recognized complication in 0.25–3% of patients. However, cases of cardiac tamponade caused by chylopericardium after lung cancer surgery are extremely rare.We describe hitherto unreported sequelae of chyle leak following lobectomy and systematic mediastinal lymph node dissection (SLND) causing pericardial tamponade and cardiovascular compromise. The patient was successfully treated with minimally invasive surgical repair and ligation. We also discuss the development of chylopericardium as a potential complication of lobectomy and SLND.The anatomical characteristics of the thoracic duct warrant special attention in postoperative chyle leak management in patients who undergo definitive mediastinal lymph node dissection. Surgeons should be aware that chylopericardium is a rare but potential complication of lobectomy and SLND as it may help with early diagnosis, management, and prevention of cardiac tamponade.The online version contains supplementary material available at 10.1186/s13019-023-02126-3. While postoperative chylothorax after lung cancer surgery is rare, postoperative chylopericardium is an extremely rare but fatal complication that usually occurs after various cardiothoracic procedures –3. Moreoth edition of the TNM lung cancer staging system.A 54-year-old man presented with a persistent cough for two months without hemoptysis. He was overall healthy but had a smoking history of 30 pack-years. On physical examination, no abnormalities were observed. Chest computed tomography (CT) revealed a 2.5 cm × 2.0 cm mass in his right lower lobe, suggesting malignancy. Bronchoscopy revealed a tumor infiltrating the distal end of the right bronchus intermedius. A pathological diagnosis of squamous cell carcinoma was made based on the biopsy specimen. Abdominal CT, cranial magnetic resonance imaging, and whole-body bone emission CT were performed to rule out distant metastases. Subsequently, the tumor was staged as cT1cN0M0 (IA2) according to the 8The patient underwent minimally invasive thoracic surgery. Right lower and middle bilobectomies with mediastinal lymph node dissection were performed using uniportal video-assisted thoracoscopic surgery (VATS) Fig. A. HistopOn the sixth postoperative day, the patient suddenly developed sinus tachycardia and hypotension . A chest radiograph did not showany obvious abnormality; however, a bedside echocardiogram revealed significant diastolic collapse caused by a large pericardial effusion. An emergency pericardiocentesis drained 400 mL of chylous fluid, resulting in dramatic clinical improvement. As the characteristics of the pericardial drainage fluid were like those of the previous pleural drainage fluid, a diagnosis of cardiac tamponade caused by chylopericardium was considered. The pericardial fluid continued to drain at a rate of 600 mL/24 h or more for the next 7 days, while the pleural drainage was less than 100 mL/24 h.Subsequently, a uniportal VATS examination was performed under general anesthesia through the original incision  at the first operation and surgical intervention for chylopericardium at the second operation. VATS, Video-assisted thoracoscopic surgery.


A rare pathology, the solid pseudopapillary epithelial neoplasm (SPEN) of the pancreas accounts for approximately 1% of pancreatic neoplasms. Initially called ‘Frantz’s tumour’, it has now been renamed to SPEN by the World Health Organization (WHO). This tumour has a predilection for females and a good prognosis with surgical excision being the treatment of choice.Palpable abdominal masses in children are of significant clinical importance. Identifying cystic lesions in the pancreas from CT or MRI scans always warrant further investigations. Primary pancreatic neoplasms account for 0.1% of pancreatic tumours in the paediatric population; an extremely rare circumstance constituting a diagnostic and therapeutic challenge to surgeons. This article comprises two paediatric cases of SPEN in 14- and 11-year-old females, respectively, and a literature review on current management. A palpable mass in the abdomen of a child is a serious finding. Abdominal masses are most common in children under the age of five years, the majority arising from the kidney. When an older child presents with an abdominal mass it is more likely to be a malignancy. The most common tumours presenting as a large abdominal mass in an older child are Wilms’ tumour and neuroblastoma. The appearance of a cystic lesion in the pancreas on a computed tomography (CT) and magnetic resonance imaging (MRI) abdomen of a child being investigated for a symptomatic abdominal mass can cause a diagnostic dilemma.The occurrence of primary pancreatic neoplasms in the paediatric population is exceedingly rare, comprising only 0.1% of pancreatic tumours from all age groups, and thus constitutes a diagnostic and therapeutic challenge to surgeons [The solid pseudopapillary epithelial neoplasm (SPEN) of the pancreas is one of the rarest pathologies of the exocrine pancreas. Virginia Kneeland Frantz discovered this tumour and published her work in 1959 in a paper detailing its pathologic characteristics in three cases . This tuIn the literature, there is a paucity of cases of SPEN in children compared to adults and thus we present two cases, a 14-year-old and an 11-year-old female, respectively, and a literature review on the current management with the emphasis on paediatric cases.Case report 1A 14-year-old female with no prior medical conditions described intense abdominal pain after sustaining a fall during a sporting event. She described a central vague abdominal pain that was not relieved with the over-the-counter medication that her parents administered. Upon arrival at the emergency department, she had five episodes of bilious vomiting that had not happened before her admission to the hospital. The clinical examination revealed tenderness at the epigastrium and the right upper quadrant and had no evidence of peritonism.A round mass, firm in texture and fixed to the surrounding was present in the upper abdomen, measured approximately 8 cm x 8 cm, correlating with the tenderness found on clinical examination. Initial laboratory results were unremarkable, with adequate renal function and haematological parameters. Initial plain radiographs demonstrated no evidence of a pneumoperitoneum, nor was there any feature of intestinal obstruction. However, there was an unusual finding of bowel shadows isolated to the left side of the abdomen. Contrast-enhanced computed tomographic (CECT) imaging of the abdomen and pelvis revealed a pancreatic mass involving the body, neck, and head of the pancreas Figure .This lesion on CECT was heterogeneous, with multiple cystic components and areas of necrosis within a seemingly well-developed capsule. There was a definite mass effect from the tumour, with the displacement of the small bowel to the left lower abdomen. There was compression of the transverse colon, and the inferior vena cava (IVC) flattened in appearance. The lesion measured 11 cm (anteroposterior plane) x 14 cm , and we noted an incidental finding of a replaced left hepatic artery. The discovery of this mass prompted a focused history of specific pancreatic exocrine and endocrine symptomatology. Our patient had no evidence of a functional pancreatic tumour on history or physical examination.A multidisciplinary team meeting determined the next step in the management of the tumour, inclusive of a paediatric gastroenterologist, radiologists, oncologists, and senior surgical staff. During this discussion, preoperative histological sampling was a consideration, to confirm the diagnosis, however, the technology of endoscopic ultrasound (EUS) biopsy was not available. Additionally, the option of neoadjuvant chemotherapy was raised, in view to reduce the mass' size and reduce the possibility of anatomically contiguous 'en bloc' resections. The characteristics were, however, radiologically textbook for the identification of a SPEN. Most of the major vascular structures considered in pancreatic resections were all clear of the mass. There was some concern, however, about the relationship between the lesion, the splenic vein, the gastroduodenal artery, and the IVC.Interestingly, our patient also had a replaced left hepatic artery that originated from the left gastric artery before its anastomosing branches with the right gastric artery Figure . The finTeam members were confident that radiologically, this was, in fact, a resectable SPEN, in correlation with its history and clinical behaviour. We chose a total pancreatectomy due to the prediction that the remnant pancreas, postoperatively, would not be sufficient to support physiological function. The possible involvement of the inferior mesenteric and splenic vein also lowered our threshold for a splenectomy. Her parents were counselled extensively on the decision made, and all this included the essential general and specific risks that may occur perioperatively. We chose an open approach, and intraoperatively, a detailed search for metastatic disease was performed. Our findings correlated with the CT findings of no gross haematogenous dissemination.The pancreatic mass involved the majority of the pancreas, and the remnant pancreas, if enucleation were chosen, would be technically challenging to reconstruct. It was inseparable from the right aspect of the mesocolon, splenic vein, and the inferior mesenteric vein Figure . These fDuring the final stages of specimen removal, the tumour abutted the superior mesenteric vein, but not invaded by it. After the specimen was removed Figure , the recHer recovery was uneventful and progressed without any degree of complications, and we discharged her to outpatient care. We received histological confirmation of a SPEN with no evidence of involved margins or capsular rupture. Additionally, there were no lymphatic metastases on the nodes harvested in the resection and no evidence of lymphovascular invasion. The specimen’s immunohistochemical panel was positive for neuron specific enolase as well as progesterone receptors , in keeping with the diagnosis of a SPEN. She continued follow-up with the surgical team postoperatively in addition to a paediatric gastroenterologist as well as a paediatric endocrinologist. She is on pancreatic enzyme replacement currently and demonstrates no evidence of malabsorption such as diarrhoeal episodes, steatorrhea or nutritional deficiencies on serum evaluations. Initially, her glycaemic control postoperatively was challenging with both hyper- and hypoglycaemic episodes; however, this has been corrected with adjustments made by her endocrinologist.Case report 2An 11-year-old female was taken by her mother to a general hospital in south Trinidad, the San Fernando General Hospital (SFGH), for abdominal pain and abdominal distension occurring over six months. The abdominal distension was progressive, her abdomen became protuberant which prompted her mother to seek medical attention at SFGH. The abdominal pain was in the epigastrium, unrelenting, and had no radiation to other areas. It was associated with anorexia but no vomiting or constipation. She had no weight loss and there was no prior history of pancreatitis. An ultrasound was done in the accident and the emergency department showed an 8 cm intrabdominal mass that appeared to be arising from the liver.The child was admitted to the paediatric ward for further investigation. A CT of the abdomen and pelvis with intravenous (IV) contrast was done which showed a 9 x 9 x 8 cm cystic mass in the distal pancreas. Subsequently, an open biopsy was done because the technology of EUS biopsy was not available. The histology reported a solid pseudopapillary neoplasm of the pancreas and the patient was transferred to a specialized paediatric oncology hospital.A CT abdomen and pelvis were repeated and demonstrated an 8.7 x 8.4 x 7.2 cm cystic mass of mixed density Figure , arisingThe resection was performed using an antegrade approach. The pancreatic neck was transected with a linear stapler and the specimen was removed intact. Two 19 French abdominal drains were placed in the pancreatic bed and left upper quadrant respectively. A nasojejunal tube (NJT) was inserted and secured before abdominal closure. Postoperatively, the patient was diagnosed with a type B pancreatic fistula .Elemental NJT feeds were administered, by day 10 post-op the child was ambulating and passing stool however a milky substance was seen emerging from the left upper quadrant drain. Further biochemical analysis confirmed there was a chyle leak which was managed conservatively. An abdominal US revealed no intra-abdominal fluid collections. Streptococcus pneumoniae, haemophilus influenzae type B and neisseria meningitidis vaccines were administered approximately 14 days postoperatively. On day 22, the pancreatic fistula resolved, and the patient was discharged to the outpatient clinic. She had an abdominal ultrasound 30 days after the resection of the SPEN which reported the proximal pancreas appeared normal with no peripancreatic fluid collection.The final histopathology report stated the specimen was an 8.5 cm solid pseudopapillary neoplasm in the tail of the pancreas. The surgical margins were negative for tumour and three lymph nodes resected did not have tumour involvement. The cut surface of the tumour had a variegated solid and cystic appearance with thromboses and haemorrhagic regions Figures , 7B. TheThe SPEN of the pancreas is an uncommon tumour of its exocrine component. Historically, Moynihan and Robson documented cystic neoplasms of the pancreas in separate instances in 1903, before Dr. Frantz's description . KloppelThe name encompasses two distinctive features seen in histology, solid and pseudopapillary areas. On gross examination, large cystic or cystic and solid tumours with necrotic and haemorrhagic zones are seen. The origin of these solid pseudopapillary tumours is not clear, one theory is that they arise from the multipotent primordial cell while another suggests it originates from genital ridge angle-related cells .Currently, the literature comprises numbers over 2,500 cases, evidenced by an extensive systematic review done by Law et al. at the Johns Hopkins School of Medicine . LocallyThe most common clinical finding of these lesions includes vague abdominal pain, with one review demonstrating 63% of patients describing this as the only symptom present at the time of diagnosis . AlternaDifferentiation from other cystic neoplasms, in the paediatric population, is necessary. These include pathologies such as a pancreatoblastoma in younger patients, a pancreatic pseudocyst, and secondary pancreatic tumours, for example, in lymphoproliferative disorders and neuroblastoma . VariousThe CT features suggestive of a SPEN are a well-encapsulated or partially encapsulated heterogeneous lesion in the pancreas with scattered areas of haemorrhage, necrosis, and cystic degeneration. Enhancement on the CT usually remains isodense amongst the arterial and venous phases, which is a differentiating feature from pancreatic neuroendocrine tumours which enhance significantly in the arterial phase and wash out quickly in the venous phase . CT deliThe use of an MRI abdomen can aid with differentiating a SPEN from other tumours with its high degree of soft tissue characterization, assessing areas of haemorrhage and necrosis, for example. The apparent diffusion coefficient is another method on MRI to help distinguish the SPEN from other differentials, as this value is much higher than other cystic lesions of the pancreas. This measurement depends on the extent of tissue cellularity and water diffusion . AngiogrA pre-operative diagnosis can be made with a biopsy; however, CT-guided biopsy is not recommended because it can cause rupture of the tumour, haemorrhage and seeding of the tumour by peritoneal or cutaneous contamination during sampling . A laparEUS also provides additional information necessary in planning a surgical resection such as the exact size of the tumour, local invasion, associated lymph nodes and hepatic metastases. The location of the lesion will determine the route of FNA, a trans gastric approach is adopted for lesions in the body or tail of the pancreas whereas tumours in the head or uncinate process are aspirated via the duodenum, this facilitates a short needle tract. On EUS, a solid pseudopapillary tumour will have the characteristic appearance of a heterogeneous solid, mixed solid, or cystic hypoechoic lesion . The potA systematic review has reported that cytology of EUS aspirate has good specificity for pancreatic cancer of 90.6%, but a low sensitivity of 64.8% . One reaThe diagnosis can be confirmed by performing immunohistochemistry. Solid pseudopapillary neoplasms show immunoreactivity to vimentin, alpha-1-antitrypsin, phospholipase A2, beta-catenin, alpha-1-antichymotrypsin, CD10 and neuron-specific enolase ,29,32,33Aggressive resection of SPEN tumours results in loss of pancreatic parenchyma and may lead to pancreatic insufficiency in children. This has been a major concern of pancreatic resections and a more conservative approach has been advocated by performing enucleation of the tumour instead. Solid pseudopapillary neoplasms are surrounded by a fibrous capsule that can be excised from the adjacent parenchyma without removing pancreatic tissue. Tumour involvement of the main pancreatic duct, local invasion into the pancreas or surrounding organs and metastases are contraindications to the use of this technique. The use of intraoperative frozen section should be employed to ensure the surgical margin is free of tumour and thus confirm a complete resection. The presence of positive margins and suspicion of malignancy mandates surgical resection and removal of the distal or proximal pancreas depending on tumour location. Wang et al. published the retrospective series with the largest number of SPEN cases that were treated with enucleation . It inclDespite the potential for preservation of pancreatic function and the lower morbidity associated with enucleation, the majority of cases of SPEN reported have been treated with pancreatic resection. The proponents of aggressive surgical resection have stated that the tumour has malignant potential and thus should be treated with wide resection. Other arguments include, it is not rare that the tumour partially lacks a capsule in areas of local infiltration, the tumour occasionally incorporates the main pancreatic duct into the capsule wall and metastasizing SPEN shows a low growth fraction . In addiA meta-analysis done in 2015 by Hüttner et al. collected data from 22 observational studies, nine of which were prospectively executed, including over 1,100 patients. The startling difference between these resections was the higher rate of postoperative pancreatic fistulae (POPF) that occurred with enucleation, 25.5% versus 19.7%. These leaks were not found to impact overall morbidity or mortality. Additionally, this meta-analysis included cystic neoplasms of the pancreas in addition to the SPEN; hence, it may be a confounding variable in the assessment of enucleation strictly in cases of SPEN. However, the benefits of less functional disruption, shorter operative times, and less intraoperative blood loss were retained, with no differences in overall mortality calculated. One of the major critiques in this study consisted of the failure of addressing the comparative results of disease-free survival between the arms of treatment, as most of the studies covered short-term parameters .Surgical options, concerning anatomical resections, depend on the location of the tumour, as well as its relation to important vaso-biliary structures related to the pancreas. Lesions in the head or uncinate regions of the pancreas benefit from pancreaticoduodenectomy with pancreatic reconstruction via jejunal or gastric anastomotic outlets. Tumours at the neck of the pancreas are dealt with via central pancreatectomy, which includes a distal pancreaticogastrostomy and over-sewing of the proximal remnant. Its drawback comes from two sources of pancreatic leakage postoperatively and is advised to be done only by extensively trained and experienced in pancreatic resections .Body and tail lesions are effectively resected via distal pancreatectomy. Splenic preservation has been debated in these tumours as the argument of adequate lymphadenectomy holds little support in SPEN cases. Prophylactic lymphadenectomy is not usually required, considering the incidence of lymph node involvement is rare . ExceptiThe guidelines for the management of SPEN were produced mainly from adult cases of SPEN. Bender et al. published the only systematic review of SPEN in the paediatric population in 2018 . They reThe Whipple’s procedure and distal pancreatectomy were the most frequent operations performed at 28.6% and 26.8%, respectively. A smaller group of patients had distal pancreatectomy and splenectomy (14.2%), the lesser common operations were enucleation (6.5%), central pancreatectomy (6.3%) and head resection (4.1%). In some patients the operative procedure performed was not specified (13.4%). The development of a pancreatic fistula was the most frequent complication reported. The other complications were delayed gastric emptying, pancreatic leak, pseudocyst formation, bleeding and pancreatitis. The prognosis of SPEN in children is good. Four hundred (96%) of the patients were successfully followed up for 46.6 months. The majority, 93.3%, remained disease free and only 3.8% required adjuvant therapy. There were no deaths recorded in any of the studies, however, 6.7% had recurrent disease. In comparison with the data from adult studies, SPEN is still more prevalent in the female sex and more frequently located in the tail. In addition, surgery was also the mainstay of therapy. Laparoscopic distal pancreatectomy (LDP) has been performed safely in several case series of SPEN in paediatric patients -43. RelaClinicopathological features that may be predictive of malignant change and disseminated disease include male gender, tumours over 5cm, capsular rupture, and high mitotic counts . Even inButte and colleagues outlined that SPEN may be defined as malignant in cases of recurrence, distant metastases, or an unresectable tumour with macrovascular invasion ,50. ChemSPEN of the pancreas is a rare constituent amongst cystic pancreatic neoplasms with a definite predilection for younger females. Clinical suspicion of this tumour in an adolescent female with an upper abdominal mass devoid of exo- or endocrinopathy is paramount. Its indolent nature and its low metastatic potential make it a candidate for aggressive surgical resection, which results in very favourable disease-free survival and overall survival rates.


Malherbe and Chenantais were the first to report a pilomatrixoma in 1880. They described a calcifying epithelioma, believing it to be a sebaceous gland tumor. Since 1905, this uncommon neoplasia has been called Malherbe's calcifying epithelioma. Numerous studies suggest that this tumor stems from the external sheath of hair follicles. In 1961, Forbis and Helwig proposed the term pilomatrixoma, which is more etymologically correctA 39 year old male patient, with a painless, slow growth left pre-auricular nodule evolving for one year. On physical exam we noticed a tumor in the left pre-auricular region, measuring 2.5 × 1.5cm, hard, somewhat fix. A face CT scan showed a 4cm nodular tumor located in the subcutaneous tissue above the parotid gland, in close contact with it, highlighted by endovenous contrast . A fine An 18 year old male had had a painless and slow growth neck nodule for two years. On physical exam, he had a level V tumor on the right side, of 2 × 1cm in size, hard, somewhat fixed and painless . FNA shoThe pilomatrixoma is a relatively rare skin neoplasia. It may affect individuals at any age, with incidence peaks on the first and sixth decades of life, and it is more common in women (1.5 to 2.5:1). Among young people, 40% happen before 10 years of age and 60% before 201,4. In the cases hereby presented, the patients had 39 and 18 years of age and were males.New hair follicles are not formed after birth, only some are activated during puberty. If they are located in very deep layers, differentiation induction agents will not act properly on them. These partially differentiated follicles would form the pilomatrixomasThe most frequent location is on the neck, followed by the frontal, periorbital and pre-auricular regions. Clinically it manifests as a subcutaneous or intradermal, hard and slow growth tumorThe pre-surgical diagnosis is almost always difficult, especially when the elderly are concerned. The finding of a keratinous material in the FNA can be misinterpreted as carcinomaHistological characteristics include ghost cells in the center with basophilic nucleated cells in the periphery. Calcification is present in 70-95%The CT scan has little value to approach pilomatrixomas. It is mainly used to differentiate pre-auricular from parotid tumors and to assess large and aggressive tumors. The characteristic is a well outlined and calcified subcutaneous lesionStandard treatment involves tumor resection. It is recommended to remove the tumor with safety margins in order to minimize the risk of recurrence of the malignant variants. Adjacent skin must be occasionally resected when adhered to the dermis. Recurrences are rare, occurring in 0 a 3%Although being a benign neoplasia, pilomatrixomas can be misdiagnosed as carcinoma. It is important that the otorhinolaryngologist be familiarized with this neoplasia, and considers it in the differential diagnosis of a superficial tumor in the head and neck region, thus avoiding unnecessary aggressive resections.


Eccrine angiomatous hamartoma (EAH) is a rare, benign, slow-growing cutaneous lesion characterized by hamartomatous proliferation of the eccrine glands and vascular structures. It usually arises in early childhood; however, cases in adults have also been reported. It is diagnosed based on the clinical features of the lesion as well as the histopathological findings of the excised tissue. As the name indicates, EAH shows a close association with mature eccrine elements and capillary-sized blood vessels at the histopathological level. In rare instances, the vascular component can show the features of arteriovenous malformations. Here, we report a rare case of EAH with a component of arteriovenous malformation in a 39-year-old woman who presented with a foot lesion. Eccrine angiomatous hamartoma (EAH) is an uncommon benign cutaneous lesion histologically characterized by peculiar hamartomatous proliferation of mature eccrine units and vascular structures in the mid and deep dermis. Typically, it presents as a solitary nodule or plaque with variable discoloration during early childhood . HoweverA 39-year-old healthy woman presented to the general surgery clinic complaining of a non-progressing lesion on the right foot for two years. The lesion was discovered incidentally by the patient and was associated with mild pain. She had no history of hyperhidrosis, hypertrichosis, or trauma. The patient’s medical, surgical, and family history were unremarkable. On examination, a single, well-circumscribed, 1 × 1 cm firm lesion on the right foot inferior to the lateral malleolus, associated with hardening and brown discoloration of the overlying skin, was revealed. The clinical diagnosis was callus, and the lesion was surgically excised under local anesthesia.Grossly, the specimen consisted of an ellipse of tan unremarkable skin, measuring 2 × 0.3 × 0.2 cm. A separate irregular piece of yellow-brown soft tissue, measuring 1 × 0.5 × 0.4 cm was also identified. Histologically, the epidermis showed compacted orthokeratosis with mild focal acanthosis (Figure EAH was first described by Lotzbeck in 1859 when he noticed an angiomatous-appearing lesion on a child’s face. The term EAH was coined by Hyman et al. in 1968 . It is aEAH is generally diagnosed by histopathological examination of the resected specimen. The overlying epidermis often shows none to minimal alterations, including mild hyperkeratosis, acanthosis, papillomatosis, and ulcerations. The lesion is often based in the mid or deep dermis and appears well demarcated but not encapsulated. It comprises lobules of proliferating mature eccrine ductal and glandular structures that either appear normal or exhibit hypertrophic to hyperplastic changes with dilation of the glandular lumina and, occasionally, hyperplasia of the lining epithelium with some luminal tufting or papillary fronds. The eccrine glandular structures are generally intermingled with thin-walled capillary-sized blood vessels. This is associated with variable proportions of mature adipose tissue, lymphatics, smooth muscle, pilar structures, apocrine glands, neural elements, and, rarely, bony tissue. The stroma is often loose and collagenous, with occasional cases showing myxoid changes or mucin deposition -4. OftenOur thorough literature search using the PubMed search engine revealed only two reported cases of EAH with a component of arteriovenous malformation. The first described case of this variant was in a 54-year-old man who had a red-brown nodule on his left hand that was associated with occasional pain and hyperhidrosis. Histopathological examination of the excised specimen revealed a dermal lesion with features of EAH. In addition, the proliferation of variably sized blood vessels with hybrid features of arterioles and venules was noted throughout the lesion. Of this vascular proliferation, some enlarged vessels with unevenly thickened muscular walls and partially developed internal elastic lamina were observed. Intimal proliferation with partial obliteration of the vascular lumina was also reported . In theiThe etiology of EAH is still unknown; however, congenital cases are thought to involve a defective biochemical interaction between the differentiating epithelium and subjacent mesenchyme, which leads to abnormal proliferation of adnexal and vascular structures ,3. In coThe clinical course of EAH is virtually always benign, with no reported malignant potential, and a very rare tendency for spontaneous regression ,3,8. TheOur case report describes the third case of EAH with an arteriovenous malformation component, which adds to the wide spectrum of histopathological findings seen in association with this lesion. Furthermore, this finding emphasizes the hamartomatous nature of this rare cutaneous lesion.


NF1 (17q11.2), predisposing affected subjects to a variety of benign (neurofibromas and plexiform neurofibromas) and malignant tumors. The lack of the NF1-neurofibromin gene product can cause uncontrolled cell proliferation in the central or peripheral nervous system and multisystemic involvement, and so the disease includes a heterogeneous group of clinical manifestations. Ganglioneuromas are benign tumors developing from the neural crest cells of the autonomic nervous system, considered to be part of neuroblastic tumors. Bladder localization is extremely rare in adults, and only three such cases were reported in children so far. The aim of our study, in addition to a brief review of the literature of these pathologies, is to bring to your attention the case of a sixteen year old patient with a very rare association of NF1 and bladder ganglioneuroma, who presented at the hospital with gross hematuria. Since bladder ganglioneuroma is a rare pathological condition, the differential diagnosis is difficult and imaging investigations and pathological investigations are the ones that elucidate this disease. The clinical approach of the medical multidisciplinary team involved should help the patient in managing her medical and surgical situation.Neurofibromatosis type 1 (NF1) is a genetic disease, with autosomal dominant transmission, related to pathogenic variant of the tumor suppressor gene  NF1 (located on chromosome 17q11.2), predisposing affected subjects to a variety of benign (neurofibromas and plexiform neurofibromas) and malignant tumors. The incidence is around 1:3500 newborns [Neurofibromatosis type 1  or Von Recklinghausen disease is an autosomal dominant genetic disease related to pathogenic variant of the tumor suppressor gene The lack of the NF1-neurofibromin product, a peptide with tumor-suppressor action, can cause uncontrolled cell proliferation in the central or peripheral nervous system and multisystemic involvement , and so the disease includes a heterogeneous group of clinical manifestations. To confirm the diagnosis of type 1 neurofibromatosis in a patient without a parent with NF1, the presence of at least 2 of the following standardized clinical criteria is required: 1. Six or more café-au-lait macules over 5 mm in diameter in prepubertal persons or over 15 mm in post pubertal individuals; 2. Two or more typical neurofibromas or a plexiform neurofibroma; 3. Axillary or inguinal freckles ; 4. Distinct bone lesions; 5. Glioma of the optic nerve; 6. Two or more hamartomas of the iris (Lisch nodules) or two or more choroidal abnormalities; 7. Heterozygous pathogenic NF1 variant with a variant allele fraction of 50% in apparently normal tissue. If one parent is already diagnosed with neurofibromatosis, the child only needs one or more of the above criteria for being diagnosed with NF1. The recently revised criteria for NF1 incorporated new clinical features  and genetic testing [Ganglioneuromas are benign tumors developing from the neural crest cells of the autonomic nervous system, and are considered to be part of neuroblastic tumors . They can develop anywhere along the sympathetic nerve chain and are fully differentiated and benign . BladderThe aim of our study, in addition to a brief review of the literature of these pathologies, is to bring to your attention the case of a patient with a very rare association of neurofibromatosis type 1 and bladder ganglioneuroma.Enterococcus faecalis and Proteus mirabilis. The patient received hemostatic treatment, antibiotics, analgesics, with a favorable evolution. After one month, she came back with abdominal pain and urethrocystoscopy was performed, followed by open tumor biopsy and partial excision of the bladder tumor. The pathological examination established the diagnosis of ganglioneuroma and then the patient received antibiotics for preventing urinary tract infection and analgesics. In this case, a major role in the management of her condition was also the psychological counseling at the periodic reassessments because she previously had suicidal attempts. At the last follow up, the patient was asymptomatic, the biological investigations and imaging investigations were showing improvement in her state, and she declared an improvement in the quality of life.We present a female patient, S.M.C., diagnosed at the age of 16 with type 1 neurofibromatosis, meeting the 2021 revised NF1 diagnostic criteria: café au lait spots, axillary and inguinal freckling, dystrophic scoliosis, labial plexiform neurofibroma; she also had slight intellectual disability (learning problems). At the initial presentation she came with gross hematuria, dysuria, and abdominal pain. An abdominal ultrasound was performed and detected a tumor in the bladder; the imaging investigation was completed with an uro-computed tomography, cranial and spine magnetic resonance imaging (MRI). Subsequently, the patient was multidisciplinary investigated , performing a complex clinical-paraclinical assessment. Laboratory analyses showed anemia and urinary tract infection with A 16-year-old girl was hospitalized in our clinic for hematuria, dysuria, and urinary frequency symptoms that lasted for three months. Her medical history included multiple episodes of depression and a suicide attempts, having multiple autolytical scars on the anterior face of the left arm. The socio-economic environment from which the patient came was a disadvantaged one, the teenager being raised by an uncle, while the mother worked abroad. Although not investigated or diagnosed with NF1 before, the patient’s mother had multiple café-au-lait spots and subcutaneous neurofibromas. Physical examination of the patient revealed short stature for age, axillary and inguinal freckles, and multiple café-au-lait spots around her body; she also had more than two subcutaneous nodules and hypertrophy of the labia majora (plexiform neurofibroma) . No neurThe psychological examination revealed emotional disorders and borderline intellectual and learning difficulties.Abdominal ultrasound: a large tumor with wide implantation base (84.8/38 mm) was seen in the urinary bladder at the level of the posterior wall, with intense Doppler signal and irregular and pseudo polypoid internal contour. Computerized tomography with urography (Uro-CT) showed infiltrative, moderate increases in density with enhanced CT scan mass located at the level of the posterior bladder wall, dimensions 22.2/87.4/95 mm; thickening of the lower bladder wall up to 9.2 mm and infiltration of the right uretero-vesical junction; bilateral grade II ureterohydronephrosis . CranialOne year after surgical intervention, micturition urethrocystography was performed, which visualized the bladder with an irregular outline due to the presence of addition images  with variable sizes at the side walls of the bladder; absence of vesicoureteral reflux .Urethrocystoscopy revealed the presence of the bladder tumor as well as the “fighting” appearance of the bladder wall with “cells and columns” and diverticula on the lateral walls . Tissue Histological assessment: the surgically removed specimens underwent standard processing at the Pathology Laboratory. The samples were fixed in 10% neutral buffered formalin, embedded in paraffin, and sectioned at 3–5 μm. Microscopic examination was performed after tissue staining with Hematoxylin–Eosin (HE) stain and revealed a proliferation of fusiform tumor cells arranged in fascicles with variable orientations associated with mature ganglion cells . The finAt the two-year follow-up, the ultrasound and micturition urethrocystography showed no evidence of the progression of the disease, laboratory analyses showed the absence of anemia and urinary tract infection, and the psychological evaluation also highlights a favorable evolution.We used Medical Subject Headings MeSH term “neurofibromatosis type 1” and “bladder ganglioneuroma” and performed a PubMed literature search for systematic reviews, randomized controlled trials, observational studies, and series of cases studies and case reports from the earliest possible date to September 2022. Articles published in English and their reference lists were analyzed for other relevant cases. Searching PubMed for the term “neurofibromatosis type 1”, 13,421 articles were found, and searching for “bladder ganglioneuroma” retrieved 41 articles, but when the terms were combined, only five articles were returned. We reviewed the title and the abstract of the papers and the full text of 51 articles, to extract data on incidence, diagnosis, etiology, associated abnormalities and treatment. Twenty-two of all these articles are found in our reference list.We presented a case of neurofibromatosis type 1 in an adolescent girl with urinary symptoms associated with a very rare tumor called a bladder ganglioneuroma. The diagnosis of NF1 in the case of our patient was relatively simple, the teenager ticking five of the seven diagnostic criteria. The case was investigated and managed by a multidisciplinary medical team, and the evolution after two years was favorable.Ganglioneuromas are rare benign tumors which arise from the neural crest and are composed of well-differentiated ganglion cells, nerve fibers, and a large number of Schwann cells. They usually develop in the posterior mediastinum, retroperitoneal space, adrenals and brain, sometimes in the spine, and very rarely in the pelvis. Bladder ganglioneuroma is extremely rare in adults, and only three such cases were reported in children so far ,5,6. GanIn addition to the clinical examination, imaging investigations are to be used to assess the manifestations of neurofibromatosis in the spine, abdominopelvic, and cranial regions . In radiDue to the rarity and the histological similarities of each disease, clinical and radiological diagnosis of bladder neurogenic tumors is difficult, especially if needle biopsy is performed . For exaUrogenital involvement (neurofibroma or plexiform neurofibromas) is also very rare in NF1 and only 80 cases have been reported to date in the world literature ,12. Our Another important problem in patients with NF1 is the risk of developing malignant peripheral nerve sheath tumor later in life of 5 to 10–13%, while it is 0.001% in the general population ,17. The Due to the great variability of clinical signs, symptoms, and organ involvement, the patients with NF1 need an individualized treatment and periodic reassessment. We demonstrated in our article this variability of the severity of the disease and the phenotypic aspect, by presenting the case of the sixteen-year-old teenager. Considering the fact that the disease is autosomal dominant and the risk of having offspring with the same condition is 50%, genetic counseling is extremely important in monitoring and preventing the occurrence of serious conditions .Adolescents with unusual diseases may face challenges different from those with frequent medical disorders, especially if they come from disadvantaged social backgrounds or disorganized families ,21. The Due to the variability of clinical manifestations, NF1 represents a therapeutic challenge, most of the times necessitating a multidisciplinary team to develop strategies for diagnosis of multisystem involvement, risk assessment, initiation of targeted therapy, surgery, counseling, and support. Bladder ganglioneuroma is a very rare neurogenic tumor and there are few cases associated with NF1. The majority of cases reported in the literature were treated with local excisions, as in our case.