Post-traumatic diaphragmatic hernia is a rare but life-threatening condition resulting from a traumatic injury to the diaphragm. We present a case of a 48-year-old man with a history of fall injury and a delayed presentation of a right-sided diaphragmatic hernia with subsequent bowel obstruction and perforation in a patient with a history of trauma and tuberculosis. The patient underwent exploratory laparotomy with manual reduction of the herniated bowel, primary repair of the diaphragmatic defect, and the creation of a double-barrel ileostomy. This case highlights the importance of considering traumatic diaphragmatic hernia in patients with a history of trauma presenting with abdominal symptoms. Timely diagnosis and surgical intervention are crucial in preventing serious complications associated with this condition. HighlightsRare and life-threatening case of delayed traumatic diaphragmatic hernia complicated by bowel obstruction and perforation.A 48-year-old man with a prior history of fall injury and tuberculosis experienced a delayed presentation of a right-sided diaphragmatic hernia.Computed tomography imaging confirmed the diaphragmatic hernia and guided the surgical intervention.Traumatic diaphragmatic hernia should be considered in trauma patients, even with other comorbidities.Early recognition, accurate imaging, and prompt surgical management are crucial for improved outcomes.1. Delayed presentations of TDHs are uncommon but can lead to serious complications such as bowel obstruction and perforation2. The factors that contribute to the delay in diagnosing diaphragmatic rupture after trauma include a lack of immediate symptoms, the oversight of the diaphragmatic injury during the initial assessment, or the misinterpretation of radiological findings3.Diaphragmatic hernia is characterized by a defect in the diaphragm that allows the abdominal contents to herniate into the thoracic cavity. Traumatic diaphragmatic hernias (TDHs) can result from both blunt and penetrating trauma and pose a diagnostic and therapeutic challengeA 48-year-old man presented with a complaint of abdominal pain for 10 days with multiple episodes of vomiting with a history of not being able to pass stool and flatus for 8 days. He had a history of pulmonary tuberculosis 14 years back and a history of fall injuries 6 months back.On examination, the abdomen was distended, and guarding and rigidity were present, along with loss of liver dullness and an absent bowel sound with decreased air entry on the bilateral chest on the basal area.Hematological and biochemical parameters showed a slightly raised total leukocyte count of 11 300 with 85% neutrophils and 11% lymphocytes; however, other parameters were normal. Urine and creatinine levels were within the normal range, as were the levels of sodium, potassium, liver enzymes, amylase, lipase, total protein, and albumin.The preoperative chest radiograph revealed a suspicious opacity in the right lower zone, shown in Figure On CECT abdomen and pelvis, a defect of size 3 cm was noted in the right hemidiaphragm, through which there was herniation of distal ileal loop and mesentery shown in Figure The patient was admitted with a diagnosis of right-sided diaphragmatic hernia with herniating closed-loop ileal obstruction and ileal perforation. On exploratory laparotomy, about 1400 ml of straw-colored fluid was found in the peritoneum. There was a closed-loop ileal obstruction with a perforation of 2×2 cm, 30 cm from the ileocolic junction, and a gangrenous ileum of 5 cm, which is 20 cm from the ileocolic valves shown in Figure The patient underwent manual reduction of the ileal loop, followed by a thorough wash of the right pleural cavity, and a chest tube was placed. The right-sided diaphragmatic defect was primarily repaired. The gangrenous ileal segment was resected out, followed by double-barrel ileostomy.The patient had a successful recovery without complications and was discharged after 28 days. A follow-up after 2 months confirmed their well-being and the absence of any issues.The plan is for ileostomy reversal in 3 months, and the patient is in continuous follow-up.4. While its immediate presentation following trauma is well-documented, cases of delayed presentation with complications such as bowel obstruction and perforation are particularly rare and clinically challenging.Post-traumatic diaphragmatic hernia is defined as the displacement of intra-abdominal organs into the chest through a pathological orifice in the diaphragm resulting from trauma6. However, the incidence of delayed presentations of TDH complicated by bowel obstruction and perforation within this subset is even lower, ranging from 0.17 to 6%7. In a retrospective study conducted by Smith et al.8, out of 276 cases of TDH, only 8% presented with delayed symptoms, and among them, only a small proportion developed complications such as bowel obstruction and perforation. Blunt trauma, including falls, motor vehicle accidents, or direct blows to the chest and abdomen, is the main cause of TDH (81%)9. The left hemidiaphragm is the most frequently affected site, accounting for 80–90% of cases6.The incidence of TDH is estimated to be 0.8–5% among all thoracoabdominal injuries10.Diagnosis of TDH can be challenging due to its subtle or nonspecific symptoms, which can be masked by other underlying conditions. However, symptoms such as shoulder and/or epigastric pain, respiratory distress, and intrathoracic bowel sounds can indicate diaphragmatic defects. In cases where the defect is detected later on and becomes chronic, patients may present with symptoms suggestive of partial or complete intestinal obstructionIn this case, the patient had a prior history of trauma 6 months prior to presentation, raising suspicion for the delayed manifestation of TDH. While the presence of a past history of tuberculosis added complexity to the differential diagnosis, as it could have potentially led to intestinal obstruction. However, the subsequent development of intestinal obstruction symptoms and radiological findings confirmed the diagnosis of a rare right-sided TDH.11, which can be asymptomatic and unnoticed sometimes10. An initial radiograph of the chest and abdomen can be performed to assess for any signs, such as an elevated hemidiaphragm, bowel gas in the chest cavity, or abnormal bowel gas patterns, which can raise suspicion of a diaphragmatic hernia11. Computed tomography (CT) is the preferred imaging modality for diagnosing diaphragmatic injuries in a clinically or radiologically suspected patient12, offering valuable information for surgical planning.Radiological diagnosis is the mainstay for the diagnosis of diaphragmatic hernias13:TDHs are classified into three types based on the time interval for diagnosisType 1 hernia – when the diagnosis is made immediately following trauma.Type 2 hernia – when the diagnosis is made within the recovery period.Type 3 hernia – when the diagnosis is made when the patient presents with ischemia or perforation of herniated organs.Our case falls into the Type 3 category, as the patient only presented after ischemia and perforation of a herniated closed ileal loop.14. In our case, CT findings confirmed the presence of a diaphragmatic hernia and guided the subsequent surgical intervention, which involved exploratory laparotomy with manual reduction of the ileal loop. Primary repair of the right-sided diaphragmatic defect was performed, and a double-barrel ileostomy was created. This comprehensive surgical approach aimed to relieve bowel obstruction, restore normal anatomy, and prevent further complications.The surgical management of TDHs involves the reduction of herniated organs, repair of the diaphragmatic defect, and appropriate drainageThis case report highlights several noteworthy aspects. Firstly, the delayed presentation of TDH complicated by bowel obstruction and perforation is an uncommon and challenging scenario. The presence of a prior history of trauma, despite a significant time lapse, underscores the significance of considering TDH in the differential diagnosis. Secondly, the radiological findings, both the initial radiograph and subsequent CT scan, played a pivotal role in confirming the diagnosis and guiding the surgical intervention. These imaging modalities can aid in detecting diaphragmatic, even in cases where symptoms may be absent or overlooked.Timely recognition and effective management of TDHs play a vital role in preventing complications and improving patient outcomes. The rarity of this case underscores the importance of increased clinical awareness and multidisciplinary collaboration involving radiologists, surgeons, and critical care physicians. Furthermore, this case report contributes to the existing literature on TDHs, particularly the delayed presentation with bowel obstruction and perforation. Given the rarity and intricacy of such cases, there is a need for further documentation and research to expand our understanding and enhance clinical approaches.Delayed TDHs are rare but potentially life-threatening conditions that require prompt recognition and intervention. This case emphasizes the importance of considering diaphragmatic hernias in patients with a history of trauma, even if there is a delay in presentation. CT plays a vital role in diagnosing diaphragmatic injuries, and surgical intervention, including reduction of herniated organs and repair of the diaphragmatic defect, remains the mainstay of treatment. Early diagnosis and timely surgical intervention are essential to prevent serious complications associated with TDHs.Ethical approval was not necessary for this case report as it involves the presentation of a single patient’s medical history and treatment. The case report does not involve experimental interventions or research on human subjects. It focuses on the clinical management and challenges associated with post-traumatic diaphragmatic hernia. Therefore, ethical approval for this particular case report was not required.Written informed consent was obtained from the parents for the publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.No funding was received for the study.K.K.Y.: had taken the history and was involved in the active management of the case; R.G., R.R., R.K., A.B.L., L.K., and R.K.Y.: were involved in the writing of the manuscript; K.K.Y. and P.Y.: edited and revised the manuscript. All authors have read and approved the final version of the manuscript.The authors declare no conflicts of interest.Name of the registry: none.Unique identifying number or registration ID: none.Hyperlink to your specific registration (must be publicly accessible and will be checked): none.krishna321ydv@gmail.comKrishna Kumar Yadav, Department of General Surgery, Tribhuvan University, Institute of Medicine, Maharajgunj, Kathmandu, Nepal, E-mail: All available data are within the manuscript itself.Not commissioned, externally peer-reviewed.


Hepatic arteriovenous malformations (HAVMs) are a rare disorder reported in association with hereditary hemorrhagic telangiectasia (HHT), known as Rendu-Osler-Weber syndrome. HAVMs are usually detected in adulthood.A 29-year-old pregnant woman underwent a routine prenatal examination at 37 weeks of pregnancy.There were fetal liver anomalies detected by prenatal ultrasonography and were managed. Furthermore, a hepatic mass was detected and was subsequently analyzed by fetal magnetic resonance imaging. There were no typical imaging findings in this case which was once misdiagnosed as a hepatoblastoma.Considering the massive hepatic lesion, labor induction was performed on a pregnant woman to avoid adverse maternal and fetal outcomes. Histopathological examination confirmed the diagnosis of HAVMs. Lesions detected by imaging were determined to be hemorrhagic and necrotic.Prenatal hepatic hemorrhage and necrosis due to an arteriovenous malformation are rare. The authors describe their observations and results. Thus, this presentation aims to improve the understanding of this disease for sonographers and radiologists to better understand the prenatal diagnosis. Timely and correct diagnosis would allow better prenatal counseling and management decisions.th week of pregnancy. Real-time ultrasonography revealed an irregular abdominal shape and an unclear margin mass, measuring 4.4 cm × 3.2 cm × 4 cm, located in the right lobe of the fetal liver. The mass had a mixed echogenicity (hyperechoic and or anechoic pattern)  Fig. A. Color rn) Fig. B.T1 hypointense  sequence revealed empty vascular around the mass and deformation of the inferior vena cava next to the mass  for further evaluation. MRI revealed a relatively clear well-circumscribed mass with mixed signals measuring 4.6 cm × 3.3 cm × 3.5 cm segments IVa and VIII of the liver. The lesion was of heterogeneous signal intensity, The parents of fetus opted for pregnancy termination and agreed to an autopsy because of fear of a bad prognosis. Gross fetus samples showed hepatomegaly with a size of 12 cm × 8 cm × 4 cm Fig. .,4 but no MRI imaging data were provided. HAVMs are reported to be associated with HHT, which is inherited as an autosomal dominant disease caused by mutations in ENG, ACVRL1, or SMAD-4 genes. HHT comprised a triad of epistaxis, telangiectasia, and suitable family history involving the mucocutaneous tissues, brain, lungs, and liver.,6 In our case, the fetus had no family history of inherited diseases through medical record review. Although the gene chip assay was normal in our case, whole-exome sequencing should be recommended because of insufficient deception. Mucosal bleeding points and vascular malformations of other organs had not been detected.HAVMs are rare congenital lesions consisting of hepatic artery-portal vein fistula and hepatic artery-hepatic venous malformation. There have been only 8 reports of HAVMs in fetuses with typical imaging in ultrasonography in the previous literature, In our study, the fetus presented with significantly larger hepatomegaly than observed at the same gestational age. In adults, like other arteriovenous malformations, HAVMs show that arteries and veins communicate directly with high-pressure arteries feeding into low-pressure veins via malformed vascular structures without a capillary. However, the blood supply of fetal liver, different from that of the adult, is dominantly supplied by the umbilical vein with a higher pressure than hepatic artery and vein. Doppler ultrasonography plays a key role in detecting hemodynamic changes. Enhanced CT or MRI examinations are important for detecting arteriovenous malformations, including feeding arteries, intra-tumoral vasculature, and draining veins. Because of radiation protection and disabling of contrast agents for the fetus, indirect signs such as hemorrhage, necrosis mass, and disordered vessels around the lesion were detected instead of typical ultrasonography and MRI imaging signs. The imaging findings of this case are similar to the cerebral arteriovenous malformations that were finally diagnosed using DSA reported by Eguchi et al High-pressure blood flow directly into vascular structures can disrupt the thin vessel with defects in smooth muscle and can ultimately cause hemorrhage. The steal phenomenon caused by arteriovenous malformations leads to insufficient blood supply to other liver parts and causes necrosis. In our case, ultrasound hypoechoic areas, T1WI hypointensity, and T2WI hyperintensity suggest old hemorrhagic necrosis, whereas ultrasound hyperechoic areas and T2WI patchy hypointensity suggest hemorrhage or hemosiderin deposition, consistent with gross specimens. The short bar blood flow signal around the nodule in color doppler ultrasonography and empty vascular around the mass on T2WI may suggest the presence of vascular malformations.In HAVMs, abnormal blood circulation in the liver causes hepatomegaly and portal hypertension.T2-hyperintense and T1-hypointense with heterogeneity areas due to necrosis, hemorrhage, or calcification in MRI imaging. However, hepatoblastoma was typically a hyperechoic solid mass with aggressive features such as rapid growth or metastases, which are helpful for differential diagnosis.,13 Diffusion restriction (high in diffusion-weighted imaging) may also contribute to misdiagnosis. Malignant tumors like hepatoblastoma have highly cellular tissue and several cell membranes per unit volume, limiting water molecules diffusion into the extracellular space. However, the diffusion restriction may indicate edematous areas of lesion or hemorrhage in the acute or subacute phase. The deformation of inferior vena cava in T2-weighted imaging that was misidentified as an invasion of hepatoblastoma tumor was caused by compression of hemorrhage and necrosis. In addition to hemorrhage and necrosis, HAVMs can result in high-output heart failure. In this case, cardiac abnormalities have not yet been described by ultrasonography. Additionally, multiple or large lesions of hepatic arteriovenous malformations can lead to significant complications, including hepatic encephalopathy, biliary ischemia, liver failure, and so on.Fetal liver lesions are rare, and differential diagnoses of hepatic arteriovenous malformations mainly include hepatic hemangioma, hepatoblastoma, and metastatic malignant tumors. Similar to our case, hepatoblastoma was predominantly ,19 The best way is liver transplantation, which increases the family’s financial burden. preoperative diagnosis in our case seems to be very difficult because of the limitations of fetal ultrasonography and magnetic resonance imaging. The confirmed diagnosis of hepatic arteriovenous malformations in a fetus should rely on the common decision of imaging examinations, gross sample observation, and pathological examinations. Besides typical signs in imaging, sonographers and radiologists should improve the understanding of HAVMs and consider the possibility of tissue hemorrhage and necrosis caused by HAVMs when discovering liver disease during prenatal examinations.HAVM treatment mainly includes interventional treatment and surgical resection. Several molecular targeted drugs, such as the anti-VEGF inhibitor bevacizumab, are reported to improve the increased cardiac output due to HAVMs.preoperative diagnosis in our case seems to be very difficult because of the limitations of fetal ultrasonography and magnetic resonance imaging. The confirmed diagnosis of hepatic arteriovenous malformations in a fetus should rely on the common decision of imaging examinations, gross sample observation, and pathological examinations. Besides typical signs in imaging, sonographers and radiologists should improve the understanding of HAVMs and consider the possibility of tissue hemorrhage and necrosis caused by HAVMs when discovering liver disease during prenatal examinations.It is common to misdiagnose HAVMs due to their nonspecific presentation. This case highlights the importance of having a high index of suspicion when diagnosing HAVMs.Conceptualization: Wei BianData curation: Wei Bian, Jun Yuan.Writing – original draft: Wei Bian.Writing – review & editing: Yuan Yao.


Lymphangiomas are lesions attributed to congenital malformations of the lymphatic system, or acquired chronic obstruction of the lymphatic network due to trauma, radiation, surgical manipulation, inflammation, or infection. Overall, lymaphangiomas are rare, and particularly, retroperitoneal lymphangiomas are far more uncommon per reported cases.A 49-year-old Iranian woman presented with a progressive abdominal pain since approximately 1 month before admission. She was found to have a retroperitoneal lymphangioma after a precise radiological and surgical workup.Retroperitoneal lymphangiomas are rare lesions, sometimes indistinguishable from malignant lesions originating from pancreas and adjacent organs. Complete surgical removal and histologic evaluation of the lesion is the gold standard of treatment and diagnosis. Lymphangiomas are rare benign lesions originating from lymphatic system . They ca3, hemoglobin (Hb) 11.2 g/dl, and platelets (plt) 210,000/µl. Further evaluations for anemia demonstrated a microcytic and hypochromic anemia with a serum iron profile compatible with iron-deficient anemia. Erythrocyte sedimentation rate (ESR), renal function tests, liver function tests, pancreatic enzymes, coagulation tests, and serum bilirubin level were all within normal limits. Tumor markers such as cancer antigen 19-9 (CA19-9), α-fetoprotein, and carcinoembryonic antigen (CEA) were all within normal ranges. Abdominopelvic ultrasound revealed a heterogeneous hypo- and hyperechoic lesion about 48 × 40 mm2 at the head of the pancreas, adjacent to the liver hilum. Color Doppler study of major abdominal vessels was unremarkable. Spiral chest CT scan was normal. Abdominopelvic CT scan showed a well-defined hypodense lesion measuring about 45 × 46 mm2 in size, which resembled a soft-tissue mass of unknown origin, without clear enhancement, near celiac trunk with abutment of left gastric artery. Adjacent mesenteric vessels were severely engorged. An increased gastric wall thickness was noted and porta hepatis lymph nodes  were also detected  and common bile duct (CBD) was against the diagnosis of adenocarcinoma. Elastography strain ratio of the lesion was estimated to be 22  5100 cells/mmT1 and T2 images [Lymphangiomas are slow-progressing tumors that are not reported to harbor malignant potential . The kno2 images . Recurre2 images . One-yeaRetroperitoneal lymphangiomas are rare lesions with nonspecific symptoms and indistinct imaging clues, which make the diagnosis challenging. When located adjacent to the pancreas or peripancreatic major vessels, differentiation of pancreatic adenocarcinoma or pancreatic cystic lesions, which harbor a malignant potential, is of clinical importance. Histologic evaluation of the lesion maybe the gold standard of definite diagnosis. Surgical removal of the lesion is the standard method of therapy for symptomatic lesions.


This case report is intended to present an unusual cause of urinary retention by a congenital vaginal obstruction, such as hematocolpos. Hematocolpos is an obstruction of the menstrual flow due to an anomaly of the genital tract, with imperforate hymen being the most common one.A 12-year-old female patient, was referred to our emergency department for recurrent abdominal pain lasting approximately 90 days. She presented with intermittent dysuria and supra pubic fullness. Ultrasonography confirmed acute urinary retention and features of hematocolpos .High index of suspicion is necessary to diagnose and treat such patients promptly. Patients usually present with cyclic pain, primary amenorrhea, incomplete emptying of urine, chronic constipation, back pain and recurrent urinary tract infections. Our patient presented with acute urinary retention.This case report highlights the importance of early recognition of hematocolpos as a potential cause of cyclic pain, and urinary symptoms. Prompt and accurate diagnosis with the appropriate surgical managements, to resolve their symptoms with successful outcomes. •Hematocolpos is relatively uncommon condition leading to devastation outcomes.•High level of suspension is warranted to diagnose and manage those patients.•This case report presents a unique instance of hematocolpos in an adolescent girl, emphasizing the diagnostic challenges, clinical management, and successful outcomes.•Diagnosis is primarily based on clinical presentation and imaging modalities, such as ultrasound and MRI.•Treatment options include surgical hymenotomy or hymenectomy, aiming a patent vaginal outlet and relieve obstruction. In the pubertal age group, congenital anomalies from developmental failure of the Müllerian ducts begin to manifest as pelvi-abdominal pain, and primary amenorrhea caused by hematocolpos, which is defined as fluid accumulation in the cervix . AnotherThe rarity of this condition causes it to be undiagnosed until menarche in many cases. Patients commonly present with abdominal, pelvic, or perineal pain. As well as constipation and lower urinary tract symptoms secondary to mechanical obstruction. Urinary retention in the pediatric age group is considered a rare event making is unlikely to be diagnosed early, thus, delaying its treatment similarly our patient. Regardless of the age group, urine retention is a urologic emergency and must be managed immediately. Our patient presented to our community hospital where she was later diagnosed and managed. This case report has been reported in line with SCARE 2020 criteria .2A 12-year-old young girl, 149 cm in height, weighing 55 kg known to have recurrent urinary tract infection (UTI) since childhood with no investigations done. The patient had a history of recurrent visits to the emergency department 3 months prior to admission. She complained of intermittent dysuria and lower abdominal pain.She was treated as Urinary tract infection and was discharged home.The patient had dysfunctional voiding. Her mother reported that she holds her urine for long periods of time, specifically in public areas. She usually double voids to empty her bladder. She presented to the emergency room with 90 days history of intermittent dysuria, lower abdomen and suprapubic cyclic pain for the past 2 months. She denied any trauma, constipation or back pain.2.1The patient had an unremarkable antenatal, postnatal history and past medical history. Initially the Patient was tachycardic (110 beats per minute). She was afebrile with a normal blood pressure, oxygen saturation, and respiratory rate. A full examination was showed distended abdomen up to the umbilicus with tenderness mainly in the suprapubic area. Patient's sexual characteristics were of Tanner stage 3.Pelvic examination was then conducted by labial separation in a supine frog-leg position. A bluish bulging hymen obstructing the urethral meatus was seen that was tender to touch.Laboratory investigations were done and were mostly unremarkable except white blood cell count (11.300/μL).Renal function test and urine routine were also unremarkable. Urine culture showed no growth.A decision was made to perform a bedside ultrasonography that showed bladder filled with anechoic fluid with a dilated vagina filled with low level echoes that could be blood products. A 14 French catheter was inserted and drained 750 ml clear urine which relieved her acute pelvic and abdominal pain.She was then admitted to the urological ward, and an MRI was done  and reveThe uterus was seen displaced superiorly showing its cavity mildly distended with minimal amount of fluid showing signs of hematocolpos . A multiAfter counseling the parents about the risks and benefits of hymenotomy, the parents signed a consent forum for surgical drainage.2.2An urgent hymenotomy was done on an urgent basis. Two consultants scrubbed in, a urologist as well as a gynecologist both with high level of training. A surgical incision to the hymen was made in the centre of the imperforate hymen which was in a cruciate manner. The hymen was thick and tough which made the incision difficult. Approximately 500 ml of thick tarry colored old menstrual blood with clots were drained with the help of suprapubic manual pressure application. Redundant flaps were later cut, and the edges of the margins were everted by suturing the inner vaginal mucosa to the exterior vestibular mucosa. Vicryl 2–0 braided absorbable sutures were used. The enlargement of the abdomen almost immediately subsided as the vagina emptied.A medical-legal certificate was given to the family for the medical intervention done causing her loss of virginity.2.3No future surveillance will be needed for this diagnosis. Although she had a 1 week follow up post intervention to check on the wound and proper dressing.A later follow up was also done with a uroflow and post residual volume to make sure proper voiding has been achieved. An ultrasound was also done to check for vaginal patency. She was also advised to come to the Emergency Room, if she was unable to void, faced intractable pain, hematuria, or fever. The patient had high satisfaction after the surgical intervention which relieved her from a long duration of pelvic pain.3Hematocolpos is a medical condition characterized by the accumulation of menstrual blood in the vagina. It typically results from a blockage in the reproductive tract, such as a vaginal septum or an imperforate hymen . During CA-125 and CA-19-9 are elevated in imperforate hymen, although these markers are not really necessary for the diagnosis and would delay prompt treatment [An imperforate hymen can be diagnosed by clinical examination, showing a tense, bluish bulging membrane. Tumor markers such as reatment .The use of ultrasound is usually the preliminary imaging tool in the diagnosis of Müllerian duct anomalies, and adequate enough to detect blood clots in the vagina . HoweverThere are several surgical interventions for hematocolpos. One of them is Hymenotomy which is commonly done. An incision is created in the hymen to create an opening to expel the menstrual blood relieving the patient. A special technique such as Z-plasty, may be performed in an attempt to reduce the risk of restenosis . It is d4In summary, hematocolpos is a serious condition that can lead to complications if left untreated. Prompt diagnosis and management are essential to relieve acute symptoms, prevent complications, and restore normal menstrual function. Treatment typically involves a minor surgical procedure to create an opening in the hymen and allow the accumulated menstrual blood to drain. Keeping a high index of suspension is key to diagnosing such rare conditions.A written consent was obtained from the patient and legal guardians for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this Journal in request.Ethical approval is exempted at our institution.No funding sources to be declared.Noor Al-Buloushi: literature review, writing, paper editing, manuscript drafting.Shahad alBusari: literature review, writing.Abdullah Alenezi: paper editing.Mohammed Zahir: primary physician, critical review, supervision, final approval.Corresponding author: Noor Al-Buloushi.1. Researchregistry.2. researchregistry9374.https://www.researchregistry.com/browse-the-registry#home/.3. The authors report no declaration of interest.


Non-operative management has been suggested as a therapy for uncomplicated appendicitis. Notwithstanding, the risk of missing an appendiceal tumor must be considered, being the surgical piece crucial to rule out neoplasms. Therefore, we aim to determine the incidence of appendiceal neoplasms in patients with acute appendicitis, tumor types and the importance of the anatomopathological study of the surgical piece.Retrospective study in which we described patients who underwent emergent appendectomy with histopathological findings of appendiceal neoplasms from January 2012 to September 2018. Descriptive analysis included demographic variables, diagnostic methods, and surgical techniques.2993 patients diagnosed with acute appendicitis who underwent an emergency appendectomy. 64 neoplasms of the appendix were found with an incidence of 2,14%. 67.2% were women, the mean age was 46,4 years (± 19.5). The most frequent appendiceal neoplasms were neuroendocrine tumors , followed by appendiceal mucinous neoplasms , sessile serrated adenomas , and adenocarcinomas . In 89,1% of the cases, acute appendicitis was determined by imaging, and 14% of cases were suspected intraoperatively. Appendectomy was performed in 78,1% without additional procedures.Appendiceal tumors are rare and must be ruled out in patients with suspected acute appendicitis. The incidence of incidental neoplasms is higher in this study than in the previously reported series. This information must be included in decision-making when considering treatment options for acute appendicitis.The online version contains supplementary material available at 10.1186/s12893-023-02183-4. Appendectomy has been widely accepted as first-line management for acute appendicitis in the absence of abscess formation or peritonitis –4. HowevPseudomyxoma peritoneum) [Tumors of the appendix are unusual entities, mainly diagnosed incidentally in the anatomopathological study of appendectomy pieces, and rarely suspected before or during a surgical procedure , 11–15. itoneum) , 21. Desitoneum) .These neoplasms correspond to less than 0,5% of all tumors of the gastrointestinal tract , 24, andAfter Institutional Review Board’s approval, a retrospective study composed of patients with diagnosed acute appendicitis who underwent emergency appendectomies at a tertiary referral hospital was conducted. Over 7,5 years between January 2012 and September 2018, 2993 clinical records were reviewed. Patients over 18 years old, and those who underwent appendectomy for acute appendicitis, were included. Patients with previously known diagnoses of an appendiceal tumor, those undergoing right hemicolectomy (RHC), and patients who underwent appendectomy for a different indication than acute appendicitis, were excluded. To clarify, individuals with an Alvarado rating ranging from 1 to 4 were discharged. Alvarado scoring ranging from 5 to 6, were sent to ultrasound examination and abdominal CT scans if needed. Individuals with an Alvarado rating of 7 to 10 were directly diagnosed with acute appendicitis and underwent appendectomy. Demographic variables (such as the age of presentation and gender), as well as diagnostic methods, surgical techniques (including the extension of the resection), and the histological type described in the pathology report, were analyzed using descriptive statistics. The follow-up on these patients was continued until the first postoperative control.Of the 2993 samples of appendectomies analyzed, 64 neoplasms of the appendix were found  reported in 27 patients , of which 62,9% of the cases were women Fig. , with anThe second most frequent tumor was mucinous neoplasm of the appendix (AMN) reported in 23 samples , of which 69,6% were women Fig. . The aveThe sessile serrated adenoma (SSA), considered a pre-malignant lesion, was the third most frequent appendiceal tumor in this series, reported in 12 cases . The mean age was 46 (± 15.6) years. Of the 12 SSAs, 9 cases (75%) were women . The ratio of women to men was 1:1, with an average age of presentation of 54 years. One of the cases was reported as a well-differentiated neoplasm, and the other was poorly differentiated. Neither of the tumors was suspected before the surgical procedure. In one case, non-tumor-free resection margins were reported. Table Our population shows that appendiceal tumors incidentally found in emergent appendectomies are significant, with an incidence of 2,14%, similar to those reported worldwide, between 0,2% and 2,5% , 25. FroCurrently, the treatment of acute appendicitis is controversial since most recent literature has shown that the conservative management of these patients with the use of antibiotics in selected cases, is a viable and promising non-invasive option , 5, 6. NDifferent studies evaluating the non-operative management of uncomplicated acute appendicitis, such as the APPAC  and the M. Enblad et al.  showed tAppendicular inflammatory masses can be presented in 2 to 6% of cases, with a higher risk of appendiceal neoplasms that may vary between 5,2 and 12% , 29. MorAmong the limitations of this study are the relatively small number of patients, its retrospective nature, and the lack of longer follow-up. Further prospective studies are needed to evaluate the impact of non-surgical management of appendiceal inflammatory processes in terms of morbidity and mortality of appendicular neoplasms.Tumors of the cecal appendix must be considered as differential diagnoses to be ruled out in patients with suspected acute appendicitis. Unfortunately, they do not present specific signs or symptoms and are not suspected or evidenced before, or during surgical intervention, so their diagnosis remains incidental after the anatomopathological analysis of the surgical samples obtained from appendectomies. Appendiceal tumors are infrequent. However, the increasing incidence reported in recent international series, including the current one, enhances the need for the surgeon to discard this pathology as a cause of acute appendicitis. The possibility of incidentally diagnosing appendiceal neoplasms should be considered when making decisions about the appropriate management for acute appendicitis, whether surgical or not, considering that the histopathological analysis of the surgical specimen should be used for diagnostic purposes and to determine the requirement for additional treatment.Below is the link to the electronic supplementary material.Supplementary Material 1


Orbital lymphoma is a rare tumor with an incidence of 2.02 per million per year. It can occur as a part of systemic lymphoma or spontaneously arise primarily in orbit. The most commonly reported malignant lymphoma is non-Hodgkin’s lymphoma. The typical clinical finding includes exophthalmos, swelling, and limited eye movement. Our patient presented with dryness and irritation of the eye without signs of proptosis until months later. The definitive diagnosis of orbital lymphoma requires a biopsy. Still, imaging studies such as MRI and CT scans play a critical role in distinguishing benign from malignant lesions and invasion of local tissues. The treatment of orbital lymphoma is multidisciplinary, involving surgical resection, radiotherapy, and chemotherapy depending on the histological type of tumor and the presence of metastasis. Orbital masses are rare clinical entities typically caused by infectious, inflammatory, and neoplastic etiologies. They can be either primary lesions stemming from the orbit itself, including the lacrimal gland, or secondary due to lesions involving the orbit by contiguity . AdjacenLymphoproliferative lesions may range from benign lesions such as reactive lymphoid hyperplasia or malignant lesions including lymphomas. Histopathology is required to differentiate benign lesions from malignant and other mimics such as orbital pseudotumors . Benign A 54-year-old female with a past medical history of hypothyroidism and type 2 diabetes mellitus presented to the primary care clinic with complaints of dryness and irritation of her right eye. A basic eye exam performed  revealed no significant findings. The patient was sent home with recommendations for an orbital warm compress. Two months later, she complained of a bump on her right eyelid along with dryness and was referred to an ophthalmologist. On examination, her extraocular muscles were functioning normally, and there was no appreciable proptosis. The patient revisited her ophthalmologist four months after the initial presentation with similar complaints of dryness and irritation, this time with mild 4.4mm proptosis on examination. A computed tomography (CT) scan of the brain and orbit showed a lobulated enhancing soft tissue mass lesion in the superomedial aspect of the right orbit involving the intraorbital portion of the right optic nerve and the medial rectus muscle which was causing the mild proptosis Figure . A magneThe imaging studies were inconclusive in making a diagnosis and tissue samples were needed for further evaluation. She underwent an orbitotomy and initial histopathology showed signs of a lymphoproliferative disorder versus granulomatous process. The further immunohistochemical evaluation showed a lymphoid infiltrate mostly consisting of CD20+ atypical B cells which were small to medium-sized with follicular expansion. The cells expressed Bcl2 but were negative for CD3, CD5, CD10, CyclinD1, and Bcl6. The findings were conclusive of low-grade EMZL. A positron emission tomography (PET) scan was performed which showed focal tracer uptake in the right half of the hard palate. There was also tracer uptake in the right anterior cervical lymph nodes making this a stage II EMZL. A plan was made in concurrence with the surgical and oncology teams to treat the patient with 20 sessions of targeted radiotherapy over the course of four weeks. A repeat PET was performed two months later and a repeat MRI six months later showed complete resolution of the EMZL.Orbital lymphomas are rare and occur at an estimated incidence rate of 2.02 per million person-years, with only two thousand cases reported over the last 24 years ,3. ThereThe most commonly reported malignant orbital lymphoma is non-Hodgkin lymphoma (NHL), specifically the mucosa-associated lymphoid tissue (MALT) subtype of B-cell origin (97%), followed by a mixed T/NK-cell origin 64%), with only 3% of cases reported being of T-cell origin [%, with oThe low-grade EMZL is associated with several chromosomal abnormalities such as trisomies 3, 7, 12, and 18, somatic deletions or mutations of the A20 gene, and several chromosomal translocations [Helicobacter pylori in gastric EMZL, Borrelia burgdorferi in skin EMZL, Chlamydia psittaci (C. psittaci) in orbital cell lymphoma, and hepatitis C virus in ocular EMZL [C. psittaci is prevalent. A recent study revealed that in those with ocular lymphomas, the prevalence of C. psittaci is approximately 50% in Italy and Korea; 33% in Austria; 5-15% in China, Germany, the United Kingdom, France, India, the Netherlands, and Cuba; less than 5% in the United States; and 0% in Japan and Kenya [C. psittaci in 80% of cases of orbital lymphoma [There is an association of EMZL with autoimmune disorders and other sources of chronic antigenic stimulation . Severallar EMZL ,5-7. PreWhen orbital lesions are suspected, it is crucial to obtain imaging using both diffusion-weighted and dynamic contrast-enhanced MRI to distinguish benign lesions, such as inflammation, from malignant lesions . AdditioThere are several variations of how different tumors and lymphomas can morphologically appear on imaging, making the identification of any orbital lesion challenging. The differential diagnosis of thrombosed varix cannot be ruled out with CT imaging but by having the patient perform the Valsalva maneuver during MRI. This would cause thrombosed varix to change size but not a mass lesion. A biopsy is the best way to identify the lesion and the cellular origins of any orbital mass. Lymphomas are generally small, and an open biopsy is typically the standard procedural protocol, as fine-needle aspiration is usually insufficient. In addition to biopsy, immunohistochemical identification is the preferred way to classify an orbital NHL. Testing for antibodies against CD3, CD5, CD20, and CD79α to identify a lymphoma is either of B-cell or T-cell origin . A PET sThe treatment of orbital NHL is multidisciplinary and depends on the histological type of the tumor and potential metastasis. For symptomatic lesions or those not metastatic, as in our patient, either surgical excision alone or in conjunction with radiotherapy is appropriate . TypicalOrbital masses are exceedingly rare and can be classified as either primary, stemming from the orbit, or secondary. A typical initial presentation of an orbital mass can include proptosis, eyelid swelling, visual changes, and deficits in extraocular eye movement. However, in this case, the initial symptom leading to diagnosis was dry eye disease alone without associated visual changes or extraocular eye movement weakness, which subsequently progressed to the development of mild right eye proptosis. This unusual presentation of unilateral dry eye disease as an indicator of a rare disease highlights the importance of investigation into orbital masses in those with a higher risk of developing lymphoproliferative lesions of the orbit, especially when unilateral dry eye or exophthalmos is present. This includes patients over 60 years of age, those with autoimmune diseases, or those who reside in an area where specific bacterial infections are common. Earlier diagnosis of these patients can expedite treatment and further improve the patient prognosis for orbital lymphoproliferative lesions.


Superior mesenteric artery syndrome also known as Wilkie's syndrome (WS) and Nutcracker syndrome (NCS) are 2 rare vascular syndromes characterized by the reduction of the aortomesenteric space. In the WS the reduction of the aortomesenteric angle leads to compression of the third portion of the duodenum. In the NCS the reduced aortomesenteric space usually causes a left renal vein (LVR) entrapment and the clinical presentation is a left flank pain, micro/macrohematuria and proteinuria. Arterial hypertension can be an unusual manifestation of the NCS. Herein, we describe the case of a 37-year-old woman with a history of breast cancer and abdominal subocclusion, with a recent onset of arterial hypertension whose enhanced computed tomography (CT) showed a reduced angle between the abdominal aorta and superior mesenteric artery with the CT findings of both the WS and NCS. Superior mesenteric artery syndrome, also known as Wilkie's syndrome (WS), is a rare cause of acute abdomen in the emergency setting presenting with abdominal obstruction A 37-year-old woman, who had undergone a bilateral mastectomy for breast cancer 2 years earlier, came to our Institution complaining of frequent abdominal pain with subocclusive episodes, nausea, and vomiting and for regular follow-up for the previous surgery. Her mother had died from colic and pancreatic cancer. She had a history of 3 cesarean deliveries, the last one in 2016. Following the last cesarean, she began to suffer from abdominal pain and rapid weight loss (BMI 16.4). In 2019, the patient was admitted to the emergency for a subocclusive episode where a reduction of the aortomesenteric angle was described. However, no other alterations were reported. The patient was treated conservatively with periodic insertion of a nasogastric tube to decompress the stomach, a high-protein diet and parenteral nutritional supplements. However, the patient reported recent episodes of hypertension (PA 140/100 mm Hg) with frequent headaches. The clinicians who hypothesized a psychiatric condition did not consider these symptoms with due attention. The patient had no family history of hypertension. Her family doctor considered prescribing laboratory examinations. The results of the urine screening test showed the presence of microhematuria with an increased value of the protein/creatinine ratio (300 mg/gr creatinine), an increased value of albumin (80 mg/L), and an increased value of albumin/creatinine ratio (150 mg/gr creatinine). The other laboratory results were in the normal range. The enhanced thoracic abdominal computed tomography (CT), confirmed the reduction of the aortomesenteric angle (about 11°)  with compression of the third portion of the duodenum and a mild distended stomach but with no sign of intestinal occlusion , and 2. There was, however, evidence of entrapment of the left renal vein (LVR) , and 4 wFollowing the imaging results, a diagnosis of combined WS with NCS was made. Subsequently, the patient was referred to a nephrologist in consideration of a possible association of the recent onset of her arterial hypertension with the Nutcracker syndrome which resulted from the imaging, and also for correct management. A follow-up with a renal Doppler ultrasound was also planned.The WS and the NCS are 2 rare vascular syndromes characterized by the reduction of the aortic-mesenteric space Radiologists play a pivotal role in the diagnosis of vascular syndromes because they can address a correct diagnosis. In fact, the WS, although is rare, can be a cause of abdominal occlusion in young women. Doppler ultrasound (US) is considered the first-level examination for the evaluation of vascular compression syndromes Since WS and NCS are rare vascular conditions, clinicians may not consider the symptoms with due attention. However, these syndromes can be the root cause of abdominal pain in young women and this phenomenon should not be overlooked. Radiologists should pay attention to the symptoms and consider the possible association of the WS to the NCS. A first clinical presentation of the NCS in young patients may consist of arterial hypertension with headache, thus these conditions should always be further investigated.All listed authors must have made a significant scientific contribution to the research in the manuscriptWritten informed consent of the patient has been obtained to publish this case report and it will be available on Journal Request


Up to 90% of tumors in the head and neck region are squamous cell carcinomas (SCC) . SCC canThe main risk factors for SCC in the oral cavity are chronic alcohol abuse and smoking . FurtherSCC can manifest itself through various symptoms such as burning, pain and changes in the mucous membranes . A prosthesis that no longer fits as well as loose teeth can also indicate for SCC. However these symptoms may vary. Within the oral cavity, SCCs often occur in the area of the tongue, the floor of the mouth, as well as the jaw ridge and the palate .Radiological imaging (computed tomography (CT) of the head, neck, thorax (abdomen) region and abdominal and cervical sonography, in special cases PET-CT) are linked to the tumor biopsy with subsequent histopathological confirmation of the findings . The tumThe 5-year survival of isolated tongue carcinomas without evidence of lymphatic and hematogenous metastasis is >70% , 5. SimiHematogenous metastases  in particular are rarely possible in advanced stages and/or recurrent SCC . HoweverThe therapy of SCC of the oral cavity consists of different pillars. Therapy usually begins with surgical removal of the tumor and, in particular because of the lymphatic metastasis behavior, is supplemented by selective neck dissection , 4. In tSCC recurrences occur primarily within the first two postoperative years . ConsequHere, the follow-up care focuses in particular on the careful examination of the oral cavity and the throat to identify tumor recurrences in time. A retrospective study impressively showed that only 60% of all recurrent SCC of the oral cavity were symptomatic . In partHowever, depending on the initial tumor stage and the size of the recurrence, (re)operative therapy is a crucial pillar. In rare cases, SCCs in the oral cavity also tend to spread distant hematogenous metastases , 6. As aIn a recently published case report, we were able to associate a symptomatic distant osseous metastasis in the area of the femur with late recurrent SCC in the oral cavity . Even if


Endometrioid adenocarcinoma is usually diagnosed by endometrial curettage with a positive rate of 94%, while a hysteroscopic examination can increase the positive rate. Differently, endometrioid adenocarcinoma arising from adenomyosis (EAAFA) is always misdiagnosed, even after endometrial curettage or hysteroscopy. EAAFA is rarely reported.We reported two cases of EAAFA with long-term follow-ups of 9–10 years. The two cases were misdiagnosed even if endometrial curettage or hysteroscopy was performed. One case complained of postmenopausal vaginal bleeding with elevated CA199, and she was diagnosed by fast-frozen pathology during hysterectomy, followed by bilateral salpingo-oophorectomy and pelvic lymphadenectomy. In another case, a premenopausal woman with acquired progressive dysmenorrhea was diagnosed as EAAFA by the histopathological result after a transvaginal hysterectomy and had to undergo reoperation. The FIGO stage was IB in both cases. Chemotherapy or radiotherapy was performed after the operation. Patients were followed up for 9–10 years, with no metastasis or recurrence being observed.The diagnosis of EAAFA is always delayed because of tumor-free eutopic endometrium and negative results of the endometrial curettage or hysteroscopy examination. Fast-frozen pathology of the whole uterus helps diagnose EAAFA precisely and avoids reoperation. Adenocarcinoma foci of EAAFA usually involve the myometrium deeply. A better prognosis of EAAFA should be expected due to good differentiation and negative lymphovascular space invasion. Endometrioid adenocarcinoma is usually diagnosed by endometrial curettage with a positive rate of 94%, while a hysteroscopic examination can increase the positive rate. Differently, endometrioid adenocarcinoma arising from adenomyosis (EAAFA) is always misdiagnosed, even after curettage or hysteroscopy. EAAFA is rarely reported, and its long-term follow-up is extremely rare , 2. EAAF2. An ultrasound scan revealed a solid mass with a clear margin measuring 3.9 cm × 2.2 cm in the uterine cavity, which included a blood flow signal. The serum carbohydrate antigen (CA) 125 and CA19-9 levels were 70.1 U/ml and 455.93 U/ml, respectively. Hysteroscopy was performed on February 29, 2012, and it revealed that the endometrium was thin and tumor-free. A little endometrium was removed. At the same time, an ultrasound examination was done again and revealed that the anterior uterine myometrium was much thicker than the posterior myometrium (2.4 cm vs. 1.2 cm); a solid mass measuring 3.4 cm × 3.3 cm × 2.6 cm was found in the anterior uterine myometrium. Pathology of the endometrium revealed normal glandular cells, partially papillary without atypia. Because CA199 may be elevated in gastrointestinal tumors, gastroscopy and colonoscopy were performed, and normal results were obtained. Diagnosis of sarcoma of the uterus was suspected, while endometrial cancer was excluded.Case 1 was of a 67-year-old woman presenting with postmenopausal vaginal bleeding for 1 month and was referred to our hospital. She had a history of acquired progressive dysmenorrhea. She denied any history of hypertension or diabetes mellitus. Here body mass index (BMI) was 23 kg/mA transabdominal hysterectomy with bilateral salpingo-oophorectomy was performed on March 15, 2012. On gross inspection, the endometrial surface was smooth and tumor-free. On the cut surface, the anterior uterine myometrium was much thicker and harder, with several small scattered lesions. The tumor was clearly apart from the endometrial cavity. Fast-frozen pathology revealed multifocal infiltrating adenocarcinoma tissues within the myometrium. Endometrioid adenocarcinoma was diagnosed. Then, a pelvic lymphadenectomy was performed. The paraffin pathological examination  revealed2. A transvaginal ultrasound scan revealed an enlarged uterus, uneven echoes in the uterine myometrium, a much thicker anterior wall than the posterior wall of the uterus (5.0 cm vs. 1.7 cm), and a depth of the endometrium of 0.6 cm. The serum CA125 and CA19-9 levels were within normal limits. A gynecologic examination found nothing but an enlarged uterus, the same as 8 weeks of gestation. Endometrial curettage and transvaginal hysterectomy were performed on November 06, 2013. Fast-frozen pathology of endometrial tissue revealed the hyperplasia phase of the endometrium. On gross inspection, the uterus was slightly enlarged like 8 weeks of gestation, the uterine wall was thick, dense, and hard, and multiple coffee-colored bleeding foci were scattered in the myometrium. Histopathological results  level prompted the patient to undergo surgery. Tumor markers, CA125/CA199, were within normal limits or slightly elevated in almost all cases of adenomyosis. If the value of CA125 or CA199 increases in postmenopausal women diagnosed with adenomyosis, malignant changes of adenomyosis should be taken into account. We should be reminded that a regular test of serum CA125 or CA199 is needed for postmenopausal women diagnosed with adenomyosis. CA125 or CA199 is useless in early diagnosis of eutopic endometrium adenocarcinoma, while it may be helpful in following up and evaluating prognosis for the advanced stage.Magnetic resonance imaging (MRI) may be useful for the diagnosis of EAAFA in the absence of positive pathological results of the endometrium . A 41-yeAdenomyosis commonly undergoes a malignant transformation into endometrioid adenocarcinoma, seldom into clear cell, serous, and poorly differentiated adenocarcinoma , 15, 16.EAAFA must be distinguished from endometrium adenocarcinoma coexisting with adenomyosis (EACA). EAAFA is rarely reported. As a result of malignant transformation from uterine adenomyosis, EAAFA usually involves the myometrium deeply with the tumor-free endometrium. In the early stage of EAAFA, the histological examination revealed endometrioid adenocarcinoma located at least 0.5 cm from the endometrium, and the endometrium was without any tumor . At the Because of the rarity of the disease, the standardized therapy of EAAFA is not well characterized yet. EAAFA is commonly treated according to the endometrial adenocarcinoma guidelines. Arising from adenomyosis within the uterine muscle layer, adenocarcinoma foci of EAAFA usually involve the myometrium deeply. Myometrial invasion of EAAFA occurs at a greater depth than endometrial adenocarcinoma, so the FIGO stage is often IB . MeanwhiTo be emphasized, the diagnosis of EAAFA is always delayed because of a tumor-free eutopic endometrium and a negative result of endometrial curettage or hysteroscopy. It is difficult to diagnose EAAFA preoperatively, so reoperation is often performed, which may be avoided by fast-frozen pathology of the whole uterus during surgery. Despite the deep myometrial invasion of EAAFA, a better prognosis of EAAFA should be expected due to good differentiation and negative lymphovascular space invasion.


Background: The term congenital diaphragmatic eventration (CDE) refers to an anatomical abnormality of the diaphragm. It is a very rare condition; however, early and prompt diagnosis is of very great importance due to possible life-threatening complications. Most severely affected patients are neonates, usually presented with respiratory distress symptoms. The aim of this study was to systematically review the existing literature and to consolidate data on CDE in neonates as well as to report a case of a neonate with congenital diaphragmatic eventration of the left hemidiaphragm and clinical signs and symptoms of the gastrointestinal tract. Methods: An electronic search of the PubMed and Scopus databases was performed regarding studies evaluating the clinical presentation, diagnosis methods, treatments, and outcomes of CDE in the neonatal population. Results: Data from 93 studies were integrated into our review, reporting 204 CDE cases, and according to them, the male/female ratio was 1/1 with a predominance of right-sided eventration. The diagnosis was primarily established by chest X-ray; surgical intervention was the most frequent treatment. The recurrence rate was 8.3% (9/109 cases). Conclusions: Early and accurate diagnosis of CDE and repair of the diaphragm can prevent complications, reduce morbidity, and improve the quality of patient’s life. Diaphragmatic eventration is the elevation of an intact diaphragm due to the replacement of the diaphragmatic muscle by fibroelastic tissue, either partially or completely, accounting for 5% of all diaphragmatic malformations . It may CDE could also present secondarily to congenital viral infections such as rubella and cytomegalovirus infection that cause destruction of the muscular tissue and replacement with membranous diaphragm ,4. EventThe left hemidiaphragm is more commonly affected. According to Cutler and Cooper , this prMost patients are asymptomatic at birth and through adult life, and diagnosis is made by a random chest X-ray; however, some of them present with significant respiratory distress or gastrointestinal symptoms. The most severely affected patients present as neonates, with signs of respiratory compromise such as mild or severe respiratory distress requiring mechanical ventilation, apnea, or a weak cry. It is of note that in some cases, the predominant signs refer to the gastrointestinal tract, including tiring during feedings and vomiting ,9,10. ElThe antenatal diagnosis of CDE is very difficult as, in most cases, CDE may be confused with congenital diaphragmatic hernia (CDH). Sallout et al.  proposedTreatment depends on the severity of the symptoms, with many surgical techniques used for the correction of the defect .This study aimed at systematically reviewing the existing literature and consolidating data on CDE in the neonatal population, as well as reporting a case of a neonate with congenital diaphragmatic eventration of the left hemidiaphragm and clinical signs and symptoms of the gastrointestinal tract.A systematic review of the literature was conducted. The keyword combination: “diaphragmatic eventration”, “congenital diaphragmatic eventration”, “eventration of diaphragm”, “neonate*”, “ newborn*”, and “fetal” with Boolean logical operators  was used to search PubMed and Scopus databases until May 2023 following the guidelines of the Preferred Reporting of Systematic Reviews and Meta-Analyses .Furthermore, a manual electronic search and review of the references of extracted studies was performed to minimize the risk of missing studies. References of published relevant reviews were also evaluated.The review included all studies and scientific articles reporting data related to CDE diagnosis during the neonatal period. We focused on studies reporting cases of neonates with CDE and especially those that describe clinical features, diagnosis methods, comorbidities, treatments, and outcomes. Studies enrolling adults and children were included if they provided relative information regarding the neonatal population separately.Narrative reviews, systematic reviews, meta-analyses, editorials, conference papers, and studies that did not report data on neonates, as well as those published in languages other than English, were excluded. One author (R.S.) conducted the literature search and uploaded all retrieved abstracts onto the software. Three authors  independently screened abstracts to identify eligible studies and reviewed the selected articles in full text, while another author (A.K.) resolved any disagreement.A total of 463 studies were extracted from the initial search of the literature databases; 133 of them were duplicates and were removed. After reviewing the title and the abstract, 204 studies were excluded. After thoroughly reading and evaluating the full text of the remaining 126 articles, 93 studies met the inclusion criteria and were included in the review  were term neonates and 36 (26.1%) were preterm neonates. Based on available data (177 cases), the male/female ratio was 1/1. Left-sided eventration was reported in 54 (28.9%) cases, right-sided in 92 (49.2%) cases, and bilateral in 39 (20.9%) cases, while in 2 (1%) cases, the eventration was central. In 54% of cases, the symptoms appeared during the first 3 days of life. Regarding the clinical presentation, 188 neonates presented respiratory distress, 6 neonates had symptoms from the gastrointestinal tract, 11 neonates were asymptomatic, and in 22 cases data were not provided. In 133 out of 163 cases with available data, comorbidities were reported with a predominance of congenital heart diseases and congenital malformations. Only in 24 cases was the diagnosis of CDE suspected prenatally; chest X-ray and CT were the most used diagnosis methods. Regarding the therapeutic choices, surgical intervention was the most frequent treatment; only in 25 cases the patients were treated conservatively. In 142 (72%) cases out of 197 (in 7 cases data were not reported) the outcome was good, while the mortality rate was 28% (55 cases). The recurrence rate, according to retrieved data, was 8.3% (9/109 cases).+1-weeks was born to a 28-year-old secondi gravida mother via vaginal delivery, with a birth weight of 3.420 g. The antenatal period was uneventful, as was confirmed by the ultrasound screening tests and another workup. The newborn cried immediately after birth, and there was no need for special resuscitation, so he was given to his mother to breastfeed. The postpartum period during the hospital stay was uneventful, except for a brachial plexus paresis due to a right clavicle fracture as a result of shoulder dystocia. The neonate was discharged from the hospital at 4 days of life. The following days at home, the neonate presented episodes of intermitted vomiting after feeding whenever the milk feed volumes exceeded 35 mL. The abdominal ultrasound suggested by the pediatrician showed no findings for intestinal torsion or a typical picture of pyloric stenosis, although incomplete gastric torsion could not be ruled out.A male neonate with gestational age of 40At 8 days of life, the mother was referred to our neonatal intensive care unit (NICU) for further investigation as vomiting persisted. Clinical examination revealed a fixed right clavicle fracture and brachial plexus paresis, while the vital signs were normal, and there were no signs of respiratory difficulty. The laboratory assessment showed: hemoglobin 14.4 gm%, hematocrit 40.3%, total leucocyte count 12,890 k/μL, polymorphs 21.3%, monocytes 0.5%, lymphocytes 54.8%, platelets 449.000 k/μL, sodium/potassium 139/5 mmol/l, calcium 10.2 mg/dl, total bilirubin 6.3 mg/dl, C-reactive protein negative, and arterial blood gases were normal. Both chest and abdominal X-rays showed elevation of the left hemidiaphragm with the possible existence of a hernia sac and the presence of intestinal helices in the left hemithorax .Although the infant did not present with respiratory distress at that time, diaphragmatic palsy, congenital diaphragmatic hernia, or diaphragmatic eventration were considered possible diagnoses. The pediatric surgery team conducted a passage with a radiographic contrast agent that revealed the presence of stomach and left colic bend in the left hemithorax with an elevation of the left hemidiaphragm; surgery was decided. Cranial and abdominal ultrasounds performed before surgery were without pathological findings. Enteral feeding was discontinued, and intravenous parenteral fluids were administered along with antibiotic therapy with cefoxitin, gentamicin, and metronidazole as a preparation for the surgical intervention.A transabdominal repair was performed. During surgery, a left supra-umbilical transverse incision was made, and a high-grade atrophy of the central portion of the diaphragm with significant elevation showed up. The left lobe of the lung appeared to be compressed. The sac contained the spleen, stomach, left colic bend, left lobe of the liver, and a part of the right lobe of the liver. After reducing the herniated organs into the abdominal cavity, diaphragmatic plication was performed .After surgery, the neonate was transferred to the NICU. The postoperative period was uncomplicated, and the neonate was extubated on the 3rd postoperative day without the need for oxygen supplementation. Continuous enteral feeding started on the 4th postoperative day with a gradual increase in the amount of milk given. Cranial, kidney, and abdominal ultrasounds were normal. Full enteral feeding without any problem was achieved on the 12th postoperative day, and the infant was discharged from the NICU on the 23rd day of life, weighing 3640 g. Instructions were given to the parents to perform chest and abdominal X-rays at the age of 3, 6, and 12 months of life, abdominal ultrasound after 1 month and at the age of 3 months, as well as cardiological evaluation.The abdominal ultrasound performed 1 month later was without pathological findings. The chest/abdominal X-rays at the age of 3 and 6 months were normal, and there were no signs indicating a rebound of the eventration of the diaphragm . The rigCDE as an anomaly of the diaphragm was initially described by Jean Louis Petit in 1774 as “a special form of thoracic hernia” during a postmortem examination, but the term diaphragmatic eventration appeared in the literature for the first time by Beclard and Cruveilhier in 1829 and became generally accepted instead of definitions such as diaphragmatic relaxation, phrenic insufficiency, and hypertrophied diaphragm ,15,16,17Embryologically, the diaphragm is derived from muscular components and three more elements: the septum transversum, the pleuroperitoneal membranes, and the mesentery of the esophagus. Eventration of the diaphragm can be congenital or acquired ,15. CDE The inadequate muscularization of part of or the entire hemidiaphragm is presented as diaphragmatic eventration, and the most acceptable theory for the etiology of this condition is the altered myoblast migration to the septum transversum and the pleuroperitoneal membrane . The musThe thinned, weakened hemidiaphragm is insufficient to hold the abdominal organs; the diaphragm goes up and drags them into the thoracic cavity. Although in the general population, left-sided eventration appears to be most frequent (9:1) with a two-fold predominance of males , accordiClinical presentation of eventration of the diaphragm varies in severity, and the patients often appear to be asymptomatic. In most of the reported cases in the literature, clinical presentation occurred mainly with respiratory distress , cyanosis, cardiac symptoms, and gastrointestinal symptoms such as vomiting, anorexia, nausea, bowel sounds into the thoracic cavity, scaphoid abdomen, and abdominal pain ,11,13,15CDE must be differentiated from other situations like CDH with sac, phrenic nerve injury, myotonic dystrophy, and post-GBS infection . MaternaUsually, the diagnosis of CDE is not suspected prenatally, and even after birth, it may be delayed due to the absence of symptoms ,32. Our Postnatally, chest radiographs, frontal and lateral, can set the diagnosis of CDE by displaying characteristic elevation of the affected hemidiaphragm for at least two intercostal spaces higher than the unaffected side, and they may highlight bowel helices into the thoracic cavity . NeverthTreatment of CDE is mainly surgical, especially when severe respiratory distress with the need for mechanical ventilation or failure-to-thrive is present ,29. The Complications of the surgical interventions include wound infection, adhesive intestinal obstruction, partial wound dehiscence, recurrence and reoperation, pneumothorax, and empyema .Early and accurate diagnosis of CDE and repair of the diaphragm can prevent complications from gastrointestinal and respiratory tracts and improve the quality of a patient’s life ,35.The patients who went through diaphragm plication surgery should be monitored for possible complications or recurrence of the eventration . Based oThe management and the prognosis of diaphragmatic eventration depend on the severity of clinical status and the underlying etiology; acquired elevation of the diaphragm due to birth trauma has the potential to resolve automatically .Diagnosis of CDE is challenging, and it can be delayed due to the variability of its clinical presentation; however, neonatologists should suspect CDE in every infant with respiratory distress and an X-ray suggestive of lower lobe opacity. The surgery team should be in collaboration with neonatologists to manage the situation and ensure the best outcome for the patient. Follow-up is essential for these patients as diaphragmatic eventration has the tendency to relapse.


Favorable evolution of neuromotor symptoms was observed after rehabilitation, with less residual neurological impairment in the case of higher-grade spondylolisthesis. This case series aims to emphasize the importance of recognizing this pathology in athletes as well as its correct management. Early surgical intervention can prevent the onset and progression of neurological deficits.Isthmic spondylolisthesis is a condition characterized by the subluxation of one vertebral body over the adjacent inferior vertebral body, secondary to a defect in the pars interarticularis. This paper reports two cases of young acrobatic gymnasts who presented with long-term, progressive lumbosciatic pain and lower limb dysesthesia. One gymnast presented with additional neurogenic bladder dysfunction. Diagnostic testing revealed L5-S1 spondylolisthesis due to bilateral isthmic lysis in both gymnasts. Both underwent L4-S2 fixation followed by L5-S1 transdiscal arthrodesis with Rialto sacroiliac fusion system (Medtronic Spondylolisthesis results from an anterior, posterior, or lateral translation of a vertebral body relative to its caudal vertebral body . IsthmicThe degree of the subluxation is categorized according to Meyerding’s classification . This scale allows the distinction between low-grade spondylolisthesis (grades I and II), high-grade spondylolisthesis (grades III and IV), and spondyloptosis (grade V) .Clinical presentation includes lower back pain with or without radicular symptoms and, rarely, bladder and/or bowel dysfunction -3. ThereCase oneAn 18-year-old female acrobatic gymnast presented with chronic low back pain radiating to the lower limbs  and dysesthesia in the L5 territory for four years. She had normal osteotendinous reflexes, absence of motor deficits, and positive Lasègue’s sign on the left lower limb. Dynamic radiography (DR) revealed grade V spondylolisthesis at L5-S1 and the presence of instability Figure . ElectroCase twoA 19-year-old female acrobatic gymnast presented with left lumbosciatic pain associated with dysesthesia in L5 and S1 territories as well as urinary urgency episodes for the past three years. She presented a positive Lasègue’s sign on the left lower limb and sensory deficits in the corresponding regions. DR Figure  and MRI IS affects 4-8% of the general population ,10, withThe most common clinical symptom is mechanical lower back pain -3,10. AcDiagnosis is generally performed with DR, allowing for assessment of spondylolisthesis severity, but MRI is the gold standard in patients with neurological symptoms, possibly requiring surgical intervention . The decSeveral surgical techniques are available for the treatment of spondylolisthesis, but there is still no consensus regarding which is most effective ,13. In tCuriously, the patient with greater neurological impairment (radicular symptoms and neurogenic bladder) had a less severe anatomical lesion (grade III vs. grade V). Both patients participated in a rehabilitation program integrating physiotherapy and hydrotherapy, with significant improvement in pain and sensory deficits. They performed daily physiotherapy for four weeks, which consisted of neurosensory and proprioceptive stimulation of the lower limbs, strengthening of the dorsolumbar muscles, postural re-education exercises, and desensitization massage of the surgical scar. In the following three weeks, they started hydrotherapy every two days. It included water gait training with progressive loads for strengthening the core and lower limb muscles, proprioceptive training, and effort reconditioning.Sports-related IS can result in serious clinical sequelae. This case series intends to highlight the importance of early diagnosis in the management of IS in gymnasts. Early intervention may prevent the onset and progression of neurological deficits, as well as promote functional recovery. Additionally, further evidence is required to determine the best approach for the management of IS, and the novel surgical technique used in this case series, using the Rialto system to promote healing, should be investigated as an alternative surgical strategy.


Water-clear-cell parathyroid adenomas are extremely rare tumors characterized by cells that contain clear, foamy cytoplasm. Here we report a case of a large water-clear-cell parathyroid adenoma in a 70-year-old male. The patient was presented to an outside hospital with severe abdominal pain and supporting CT imaging confirming a small bowel obstruction. Initial laboratory studies revealed hypercalcemia and elevated parathyroid hormone levels. Subsequent ultrasound imaging revealed a 2.7 × 2.1 cm neck mass suspicious for a parathyroid adenoma. A parathyroidectomy was performed, and microscopic evaluation revealed an expansile proliferation of cells with characteristic water-clear cell features. Although rare, water-clear-cell parathyroid adenomas are clinically indistinguishable from more common subtypes and should be considered in the differential diagnosis of an anterior neck mass.  Parathyroid adenoma is the most common cause of primary hyperparathyroidism, a condition that affects nearly 1% of adults ,3. An adA 70-year-old male initially presented to an outside hospital with symptoms of sharp abdominal pain, nausea, and vomiting. He was vitally stable upon admission, and supporting CT imaging revealed a small bowel obstruction. Initial lab results revealed hypercalcemia, with a total serum calcium of 12.1 mg/dL. Further workup revealed a PTH level of 119.3 pg/mL, and he was diagnosed with primary hyperparathyroidism. The patient’s hypercalcemia improved after fluid administration, and his bowel obstruction was resolved with conservative management. He was discharged on the third day of hospitalization. Subsequent ultrasound imaging revealed a 2.7 × 2.1 cm lesion lateral and inferior to the left thyroid gland, and a technetium-99m sestamibi (MIBI) scan was significant for uptake at 3 hours in the left neck, suggesting a possible parathyroid adenoma  and an intact PTH of 110.4 pg/mL (13.8-85.0 pg/mL). After appropriate time-out and anesthesia, the patient was placed in a supine position with neck extension and neuromonitoring, and the left lateral thyroid was mobilized, providing a view to the left superior parathyroid tissue. The tissue was excised and passed off to pathology for frozen section evaluation. Intraoperatively, frozen section results confirmed the presence of hypercellular parathyroid tissue, and the specimen was placed in formalin for permanent. The recurrent laryngeal nerve was identified and preserved via neuromonitoring during the operation. There were no intraoperative or postoperative complications. At the two-week follow-up appointment, the patient was feeling well and denied any symptoms of hypocalcemia. Laboratory values demonstrated a correction of total serum calcium at 9.7 mg/dL and a lower intact PTH level of 11.4 pg/mL, which can occur after the removal of a large parathyroid adenoma. The patient was advised to return to the clinic in two months for an additional follow-up appointment.Pathological examinationA gross examination of the resected specimen revealed a 2.886 g, 3.2 x 1.5 x 0.6 cm, tan-encapsulated tissue fragment covered by adipose tissue. Upon sectioning, the cut surfaces demonstrated a cystic area filled with brown serous fluid. Microscopically, the tissue revealed an expansile proliferation of predominately WCCs with no significant atypia or mitotic activity (Figures Primary hyperparathyroidism is one of the most common endocrine disorders, and it is an important cause of hypercalcemia. Notably, adenomas are the most frequently cited etiology, comprising up to 75-80% of cases, followed by primary hyperplasia and carcinoma [Clinically, hyperparathyroidism can be divided into two categories: symptomatic and asymptomatic. "Painful bones, renal stones, abdominal groans, and psychic moans" are terms that have been used to describe the spectrum of symptoms. Biochemically, the most common manifestation of primary hyperparathyroidism is an increase in the level of serum ionized calcium . The serMost adenomas are solitary masses that are grossly oval to slightly lobulated with a thin connective tissue capsule. On the cut section, they are often grayish brown with possible foci of hemorrhage, calcification, or cystic change, with an increased predilection to involve one of the lower glands. Microscopically, adenomas are encapsulated and highly cellular. An adjacent rim of compressed non-neoplastic tissue can be identified in about 60% of cases. Various cell types may comprise the adenoma, but chief cells usually predominate. Mitotic figures are usually absent, and the pattern of growth is diffuse, or to a lesser extent, nesting, follicular, or pseudopapillary . In our case, WCCs predominated. The cells are large, optically clear, and have sharply outlined cell membranes. Grossly, water-clear variant adenomas have a predilection to involve the superior glands, are soft, and are typically chocolate brown in color. Cysts and hemorrhages have been observed. Interestingly, a strong association with blood group O has been observed .The WCC adenoma is an extremely rare subtype of parathyroid adenoma. A recent literature review claims that only 37 cases have been reported in the English literature between 1985 and 1981, with most occurring in middle-aged women . WCCs arHistologically, WCC adenomas are composed of large polygonal cells with abundant clear cytoplasm that contain vacuoles with or without granular material. This composition may cause them to appear isoechoic on ultrasound imaging and have reduced scintigraphy sensitivity . ImmunohIn summary, we report a rare case of large WCC parathyroid adenoma successfully treated with surgical resection without residual signs of hypercalcemia. Our patient initially presented with abdominal pain secondary to small bowel obstruction in the setting of PTH-dependent hypercalcemia. Further work-up revealed a parathyroid adenoma with histopathologic confirmation of a WCC variant. Although less aggressive, WCC adenomas can present with a myriad of symptoms. This is a rare entity and, to our knowledge, the first reported case in the state of Alabama.


The most common imaging findings in idiopathic normal pressure hydrocephalus (iNPH) are disproportionately enlarged subarachnoid space hydrocephalus, i.e., enlarged ventricles (Evans index >0.3), narrowing of the superior arcuate and median sulci, widening of the Sylvian fissure, and focal widening of the sulci of the brain. In the present study, we encountered an interesting case of a 73-year-old woman with iNPH with characteristic imaging findings of cerebral atrophy-like features and no prominent ventricular enlargement. Many cases might be difficult to diagnose as iNPH because of atypical imaging findings such as no prominent ventricular enlargement and so on. Even in cases with multiple atrophic sulcus openings without any prominent ventricular enlargement, the callosal angle and posterior commissure-level brain-ventricle ratio (BVR) could be helpful in the diagnosis, and bilateral opening of the occipitoparietal sulcus might be a key imaging finding. Idiopathic normal pressure hydrocephalus (iNPH) is characterized on imaging by disproportionately enlarged subarachnoid-space hydrocephalus (DESH) , i.e., tA 73-year-old woman developed postural maintenance function deterioration. Six years before visiting our institution, the protrusion phenomenon appeared and progressed. Specifically, symptoms such as leaning forward when standing or running too fast and not being able to stop appeared and progressed. Although she visited several hospitals and clinics, doctors explained that it was caused by brain atrophy. So, she was saddened by the progression of her gait disturbance and at a loss, and she had no choice but to wait and see what would happen. She developed a tendency to fall five years before presentation, and consequently, she experienced a left shoulder dislocation fracture four years before presentation and a right humerus fracture three years before presentation. Her gait disturbance progressed gradually, and she required a cane to walk. Specifically, she was no longer able to walk without a cane and slow, careful steps. She visited the neurologist at our hospital. The Mini-Mental State Examination (MMSE) score was 30 points, and no dysuria was detected. Her gait disturbance improved with a tap test, and she was referred to our department. She did not smoke or drink alcohol, and she had no allergies. She was married after graduating from high school, had a child, and lived peacefully as a housewife of gentle disposition. Her medical history included uterine fibroid surgery at age 42, stage Ⅲb right breast cancer that went into remission after surgery followed by chemotherapy at age 59, and dyslipidemia requiring statin therapy at age 70. Her family history was not significant.Physical examination revealed a small stature with a height of 148 cm, a weight of 51 kg, and a body mass index of 23.3. Regarding awareness and higher functions, her score was 15 (4-5-6) points on the Glasgow Coma Scale and 30/30 points on the MMSE. In the cranial nervous system, there were no visual field abnormalities, oculomotor disturbances, contra lesional reflexes (+/+), facial paralysis, or dysarthria. Assessment of the motor system revealed a negative Barre’s sign in both the upper and lower limbs, a grade of 5 on the Manual Motor Test in both the upper and lower limbs, and grip strengths of 25 and 23 kg in the right and left hands, respectively. No obvious sensory disturbances were observed in the whole body. The coordination of hand rotation was smooth. Blood testing revealed no obvious abnormality.Head computed tomography . The cutoff of 0.95 had the highest sensitivity (96.8%) and specificity (83.3%) . HoweverWhy was there no ventricular enlargement and why did iNPH symptoms still occur?Although the fourth ventricle and subarachnoid space of the alveolar region are connected by the foramina of Luschka and Magendie in healthy subjects, it was recently suggested that the fourth ventricle and subarachnoid space are connected by the choroid plexus ; the mosEven in cases without ventricular enlargement and with a cerebral atrophy-like appearance and multiple sulcus openings, the tendency of the median sulcus and subarachnoid space to narrow is a reliable indicator of iNPH, and the callosal angle and PC-level BVR might be helpful. In addition, the bilateral opening of the occipitoparietal sulcus could provide a clue to the imaging findings. We believe that these findings might help undiagnosed iNPH without ventricular enlargement to be performed by a tap test.


A 27-year-old woman presented with an epigastric mass, accompanied by emesis and weight loss. An abdominal computed tomography (CT) scan showed a pancreatic head-body mass and liver metastasis. Biopsy revealed well-differentiated pancreatic neuroendocrine tumor, grade 3. Chromogranin and synaptophysin stains were positive, with a Ki-67 index of 33.6%. Increased frequency of episodic emesis with incomprehensible speech and right-sided weakness prompted admission. A cranial magnetic resonance imaging (MRI) scan showed a subacute cerebrovascular infarct. Serum glucagon was markedly elevated. On the third hospital day, neurologic symptoms progressed, with repeat cranial MRI demonstrating acute lacunar infarcts. Two-dimensional echocardiography was consistent with dilated cardiomyopathy. Because of her fragile condition, she was not a candidate for surgery and chemotherapy. She was treated with long-acting octreotide every 4 weeks for a total of 10 doses. Posttreatment, a 2-dimensional echocardiogram and an abdominal CT scan showed no significant change. We report a rare case of glucagonoma with associated prominently dilated cardiomyopathy and ischemic stroke. Glucagonoma is a rare pancreatic neuroendocrine tumor with an incidence rate of 2.6 per 100 000 000 persons [A 27-year-old Filipino woman with no known comorbidities was referred because of a 2-year history of a gradually enlarging abdominal mass. This was accompanied by nausea, nonbilious vomiting, abdominal pain, diarrhea, and undocumented weight loss. She also presented with intermittent oozing excoriations on both lower extremities. She had a family history of breast and liver malignancy, but there were no known premature cardiovascular diseases, coagulopathies, or endocrinopathies in the family. She denied smoking, alcoholic beverage intake, and use of illicit drugs.Abdominal computed tomography (CT) scan showed a heterogeneously enhancing, lobulated pancreatic head-body mass measuring 6.7 × 6.1 × 9.9 cm . A hypo-Endoscopic-guided fine needle aspiration biopsy of the pancreatic mass was done. Immunohistochemical stains were negative for CA19-9 and SMAD-4, but positive for chromogranin and synaptophysin . Ki-67 i2). Her heart rate was elevated at 101 beats/min. Her skin was dry with hyperpigmented patches on both legs, which were consistent with the clinical picture of necrotizing migratory erythema  and abdominal discomfort, along with a 2-day history of incomprehensible speech and right-sided weakness. On physical examination, the patient was underweight  scans revealed an acute infarct in the left lentiform and caudate nuclei, insular cortex, and frontal coronal radiata. She was started on aspirin and atorvastatin. On the second hospital day, she started having diarrheal episodes, which were managed supportively.Serologic workup for functional pancreatic neuroendocrine tumor showed the following: serum glucagon was markedly elevated , serum pancreatic polypeptide , and gastrin  were increased, and serum vasoactive intestinal peptide  was within normal limits. Other pertinent findings were prediabetes , normocytic, normochromic anemia , and hypoalbuminemia (3.2 g/dL). Screening for MEN1 was unremarkable. The patient was referred to clinical nutritional support for nutritional assessment. No further workup for nutritional deficiencies were done because of limited funds.On the third hospital day, her slurred speech and right-sided weakness progressed. A repeat cranial MRI scan revealed a new infarct in the precentral gyrus of the right frontal lobe. Aspirin was changed to enoxaparin. Workup for hypercoagulability was pursued. Two-dimensional echocardiography showed depressed ejection fraction (20.9%), a dilated left ventricle with normal relative wall thickness, and global hypokinesia of the left ventricular wall . Twelve-Because of the patient's fragile condition, she was not a candidate for surgery and chemotherapy. Instead, she underwent therapy with octreotide long-acting repeatable (LAR) 30 mg IM every 4 weeks. As part of her heart failure regimen, initiation of sacubitril + valsartan was attempted but was eventually discontinued because of hypotension. She was started on captopril, spironolactone, and carvedilol. Oral nutrition supplementation was included as part of her nutritional support.Ten doses of 30 mg octreotide LAR were given. This was based on the placebo-controlled, double-blind, prospective, randomized (PROMID) study, and no uptitrations were made [The manifestations of glucagonoma syndrome result from supraphysiologic levels of the hormone glucagon. Glucagon promotes gluconeogenesis and glycogenolysis, leading to elevated blood glucose levels. Amino acids are mobilized and protein degradation is increased. As a consequence, patients are at risk for malnutrition and severe nutritional deficiencies manifesting as glossitis, stomatitis, or cheilitis. Moreover, necrotizing migratory erythema (NME) is believed to result from hypoaminoacidemia and zinc deficiency .There are few case reports on glucagonoma-induced dilated cardiomyopathy. Chang-Chretien et al described a 54-year-old woman with a 7.0-cm pancreatic mass with lymph node metastasis and elevated glucagon concentration at 1261 pg/mL . She initially presented with glossitis, angular cheilitis, necrotizing migratory erythema, resting tachycardia, and depressed ejection fraction (15%). Surgical resection of the mass led to the resolution of symptoms and normalization of glucagon (26 pg/mL) and ejection fraction (55%) [In contrast to our case, the symptoms of the patients in the previous case reports manifested during the 5th to 7th decade of life. Similarly, all of these patients had reduced ejection fractions ranging from 10% to 38% secondary to a nonischemic dilated cardiomyopathy as documented by an angiogram or cardiac MRI scan. In our patient's case however, because of her fragile condition as well as financial constraints, advanced cardiac workup was not pursued at this time. The patient's clinical profile and 2-dimensional echocardiogram findings clearly suggest that the dilated cardiomyopathy was induced by the glucagonoma.Cardiomyopathy is unusual in glucagonoma, and the mechanism of action is still not well understood. One proposed mechanism is that glucagon causes an increase in myocardial cAMP, resulting in increased heart rate and cardiac output . This elAs mentioned, surgery in the previously reported cases resulted in significant improvement of left ventricular function. However, our patient's poor cardiac function and severe malnutrition made surgery and chemotherapy prohibitive. Hence, she underwent somatostatin analogue therapy with octreotide LAR because it has both antisecretory and antiproliferative effects. Previous studies noted a decrease in glucagon secretion and reduction of NME, glossitis, and diarrhea after treatment. In addition, the PROMID study revealed that octreotide LAR has a significantly longer median time to tumor progression compared with placebo . With reInterestingly, our patient also manifested with ischemic stroke. Glucagonomas are associated with venous thromboembolic events as reported in 30% to 80% of cases . IncreasIt is recommended to perform cardiovascular evaluation at the time of glucagonoma diagnosis to institute timely and appropriate management of possible cardiomyopathy and thromboembolism.Further studies regarding the pathophysiology of cardiomyopathy and thromboembolism in glucagonoma are needed to allow the development of alternative therapies.Communication among the multidisciplinary team is of utmost importance to provide the best patient care.