Intradural disc herniation is a rare complication that is difficult to diagnose preoperatively, despite the availability of various radiological imaging tools. We report a case of a 61-year-old man with L4-L5 lumbar disc herniation who presented with back pain radiating to both legs, difficulty in walking, and urinary incontinence. Magnetic resonance imaging showed a disc bulge at the L4-L5 level. However, fragment was not seen until perioperatively; a disc fragment was found in the intradural space. While various radiological techniques have been reported in the literature for diagnosing herniated discs, an absolute diagnosis of intradural disc herniation by a single radiological investigation is unreliable. The current case demonstrates the limitations of various diagnostic methods available. We also present a review of the literature regarding possible modalities to aid diagnosis. An intradural disc herniation (IDH) is defined as a nucleus pulposus fragment of the intervertebral disc intruding through the dural sheath into the thecal sheath. It is a rare complication of disc herniation with an incidence rate of about 0.27% to 0.33% of all cases of disc herniation and peak incidence in the fifth and sixth decades of life . The ﬁrsA 61-year-old man with diabetes and hypertension presented to the hospital complaining of chronic back pain for the past four years. The pain was radiating down both legs all the way to his feet. This pain worsened recently with the addition of a loss of urinary control for the prior month. The pain increases when the patient coughed or sneezed. He has difficulty climbing stairs and getting up from low seats. He is unable to walk without support. He also has nocturnal pain. However, he gave no history of numbness, tingling sensation, or weakness from levels L3-S2.On examination, the patient could not walk without support. The straight leg raising test revealed pain at 60 degrees bilaterally. Power was rated at 3/5 in his left leg and 4/5 in his right leg of knee flexion, knee extension, foot dorsiflexion, and plantar flexion. Perineal sensations were lost, while tingling sensation were present at posterior aspect of leg and feet. His knee reflexes were intact at grade 2+, but his ankle reflex was absent in the left leg that is grade 0 and decreased in the right leg at grade 1+. X-rays showed an osteoporotic spine only. Magnetic resonance imaging (MRI) revealed a large disc bulge at the L4-L5 level with effacement of the nerve root on the left side as seen in Figure L4/L5 decompression surgery was planned. Intraoperatively, an L4 laminectomy was performed, and the patient was found to have severe bilateral compression of nerve roots at the L4-L5 level. The dural disc was incised. Furthermore, an intradural L4-L5 disc fragment was incidentally found as felt externally and then removed. The dura was closed with primary closure and ensured the nerve root was decompressed. Postoperatively, the patient recovered with no complications.The exact etiology of IDH is unknown, but theories are proposed in the literature. The migration of a disc nucleus pulposus to the intradural site requires perforation of the dura matter, the annulus fibrosis, and posterior longitudinal ligament. This may be caused by inflammation and adhesions due to degenerative disc disease, or it may be a result of postoperative iatrogenic procedures. Other causes include congenital narrowing of the spinal canal, fineness of the dura mater, congenital thinness of the dura mater or fusion of the dura mater, and posterior longitudinal ligament .A preoperative diagnosis of IDH is difficult. Lesions might be ignored or mistaken for other findings on imaging. In almost all cases described in the literature, a definite diagnosis was made only on surgery, not via imaging along. The MRI in our case did not indicate the presence of intradural disc fragments.Hidalgo-Ovejero et al. reported the finding of epidural gas in a computed tomography (CT) scan as a diagnostic marker for IDH . The gasSome MRI findings for intradural lumbar disc herniation might aid in preoperative identification. Lidov et al. reported the case of a 53-year-old patient in whom MRI was able to reveal both the intra- and extradural parts of the lesion .Floeth and and Herdmann considered the possibility to differentiate between intraspinal/intradural sequester and an intraspinal/intradural tumorous or cystic space-occupying lesion through high-resolution MRI techniques . Choi etWasserstrom et al. made a preoperative diagnosis through gadolinium-enhanced MRI. They notice a ring enhancement in a T1-weighted image at L4-L5 after injection of contrast medium, produced by granulation tissue around the lesion ,10. HidaWe presented a case of 61-year-old male who had back pain, loss of urinary control, and difficulty in walking. His MRI showed a large disc fragment in the region of L4-L5. However, intraoperatively, the fragment was found intradurally. Therefore, the exact diagnosis of IDH was not clear until during the surgery. Preoperative markers for such cases described in the literature are detection of epidural gas in a CT scan, or air noted in the intradural space on CT. Moreover, MRI might rarely shows it as migrated fragment or irregular enhancement or the characterized "Y-sign" or “hawk-beak-sign.” Preoperative diagnosis of IDH is difficult, although gadolinium-enhanced MRI may be useful for diagnosis. The selection criterion to determine good candidates for radiology techniques is needed, which would also minimize the number of different imaging procedures performed for each patient and impart a high degree of sensitivity and specificity for diagnosing it preoperatively.


Postoperative vision loss (POVL) is a rare but devastating complication that has only recently been reported following laparoscopic surgery. We present the case of a 34-year-old gravida 6 para 4 female who experienced POVL following an uncomplicated laparoscopic hysterectomy. Operating time was 174 minutes, and EBL was 75 mL. After surgery, she complained of complete vision loss with no light perception. No cerebral hemorrhage or ischemia was detected on imaging. Funduscopic exam revealed no structural abnormalities. On postoperative day 7, she received an IV methylprednisolone taper. The following morning, she reported mild light perception. Later that night, she reported a partial return of visual acuity and was discharged home. At her 2-week postoperative visit, her vision had returned to baseline. POVL is an emergency and prompt evaluation should be initiated to optimize outcome. Perioperative vision loss (POVL) following nonocular surgery is a rare but devastating and unexpected complication with limited reports in the minimally invasive surgery literature. We present the case of a 34-year-old female who underwent an uneventful laparoscopic hysterectomy and suffered from complete blindness following the operation. We subsequently describe the most common etiologies and how they pertain to the presented case.A 34-year-old G6P4024 initially presented with symptomatic abnormal uterine bleeding and severe dysmenorrhea of three years duration. Based on her evaluation, which included a detailed history, pelvic exam, and ultrasound, endometriosis and adenomyosis were suspected. She had attempted medical management with multiple regimens including combined oral contraceptives, megestrol acetate, and tranexamic acid without resolution of symptoms. She had completed her family planning after four uncomplicated vaginal deliveries and two spontaneous abortions. Given that her quality of life was impaired to a significant degree, she opted for definitive surgical management and a laparoscopic hysterectomy was planned.2, and physical exam was remarkable for lower abdominal pain and mild tenderness to palpation. Exam and vitals were otherwise within normal limits. An endometrial biopsy was performed as part of her evaluation, and it revealed proliferative endometrium with no hyperplasia or malignancy. Preoperative laboratory values were unremarkable , she reported that she was able to perceive certain changes in light. This was confirmed on exam, where she was able to tell if a flashlight was moving in-front of her eyes. At this point, her visual acuity had improved to light perception only. During the night, the patient reported her vision had spontaneously returned. Acuity was blurry, but she was able to identify objects and movement. She was discharged later that day with instructions to follow up in a week.At her postoperative visit in clinic, she noted that her vision continued to improve after discharge and was now resolved to her baseline. Her uncorrected Snellen eye exam was 20/63 for her left eye and 20/50 for her right.POVL following nonocular surgery is a rare but devastating complication that can have significant physical and socioeconomic implications. Because of this, it should be considered a medical emergency, leading to a prompt multidisciplinary evaluation. The increasing number and complexity of cases performed via the minimally invasive approach may lead to a rise in complications related to long operating times in the Trendelenburg position . AwareneCorneal damage is one of the most common insults to the optic organs following nonocular surgery. The mechanism of injury is secondary to either direct mechanical trauma or exposure keratopathy (deficient taping of the eyelids) . IncidenCortical visual loss is another relatively common etiology of POVL. In a nationwide retrospective study spanning a 10-year period, incidence was found to be 0.38 per 10,000 discharges [ION occurs when intraoperative factors either decrease perfusion pressure to the optic nerve, or when there is an increase in intraocular pressure (IOP), which in turn augments resistance to perfusion . The typAlthough this was part of our differential diagnosis, there were several elements that decreased the likelihood of this possibility. Having bilateral ION is not a common presentation. Additionally, blood loss during surgery was minimal, and the Trendelenburg position was held for less than two hours. Fundoscopic exam by ophthalmology did not reveal edema or swelling of the optic disc, which can sometimes be witnessed in cases of anterior ION. However, in posterior ION, there are usually no changes until weeks after the insult . The patThe pathogenesis of POVL due to CRAO involves retinal damage following decreased perfusion from the central retinal artery due to external compression of the eye. This is usually caused by improper intraoperative head positioning. Vision loss is unilateral. Other findings on exam include periorbital and eyelid edema, chemosis, proptosis, ptosis, attenuated retinal vessels, and loss of eye movements . Prone pPostoperative NOVL is a diagnosis of exclusion that is made once all anatomic sources and pathways of visual impairment have been ruled out. This condition is more common in younger patients and diagnosed more frequently in females. It encompasses a wide array of conditions, from malingering to psychogenic (conversion disorder) . There aDuring the evaluation of the presented patient, she was able to sign her name and touch her fingertips together without difficulty, which made the diagnosis of malingering less likely. A psychogenic cause for vision loss was considered.Treating POVL as an emergency is of paramount importance given the severe implications the diagnosis can carry. A prompt multidisciplinary approach allows for rapid identification of potential etiologies and the development of a targeted treatment strategy.


We describe a case of an 81-year-old male presenting with bitemporal visual field defects and blurry vision in the right eye. The patient was found to have a recurrent primary paraganglioma in the sellar and suprasellar region requiring a repeat transsphenoidal endoscopic resection. Immunohistochemical examination confirmed paraganglioma with the classic zellballen appearance which stained positive for chromogranin, synaptophysin, and S-100 in the periphery. Paragangliomas (PGLs) in the sella turcica are a rare entity; only 19 cases have ever been reported in the literature. PGLs in the sellar region are often misdiagnosed or diagnosed in a delayed fashion. Earlier diagnosis of this locally aggressive tumor and meticulous debulking can prevent morbidity secondary to the tumor's compressive effects. This report highlights the effectiveness of surgical interventions in treatment of paragangliomas. More research is still needed to determine the need for adjuvant therapies such as radiation. Paragangliomas (PGLs) are rare, nonepithelial neuroendocrine neoplasms (NENs)  that ariIn the head and neck, PGLs account for only 0.6% of all tumors . Of all 60–70% of carotid body PGLs present as a nontender, enlarging mass in the lateral neck often with a bruit and can lead to cranial nerve IX, XI, and XII deficits [When PGLs are intracranial, over 90% are from the temporal bone, in the jugular foramen or middle ear (termed tympanic) . JugularSellar PGL generally presents as an enlarging sellar mass most often associated with vision loss/impairment and headaches . To our An 81-year-old male with a history of atrial fibrillation on warfarin, hypertension, and glaucoma initially presented with progressively worsening blurry vision. On physical examination, he was found to have visual field defects, with no other cranial nerve sensory or motor defects. Additionally, there was no evidence of endocrinopathy or autonomic changes noted.On radiologic evaluation, the patient was found to have a sellar mass pressing on the optic chiasm, most consistent with a pituitary microadenoma. Two years earlier, he underwent a transseptal transsphenoidal resection of the pituitary tumor at another institution to decompress the optic chiasm. Histopathological evaluation of the resection had features of a paraganglioma. Postoperatively, there was no progression of his visual deficits.Several months later, the patient presented to our clinic with worsening visual problems especially blurry vision on the right side and decreased short-term memory. He also reported two episodes of epistaxis within the prior ten days. He denied having any problems with balance or episodes of headaches. On physical examination, he had decreased bitemporal fields and an increased right inferior field but can still see hand movements in both superior quadrants on the right side. Visual acuity in the left eye was good. The pupils were equal, round, and reactive to light. The extraocular movements were intact. The remainder of the physical exam was unremarkable.On imaging, MRI demonstrated a lobulated 1.9 cm × 2.1 cm × 2.3 cm T2 hyperintense homogeneous enhancing mass causing expansion of the sella turcica and extending into the suprasellar cistern compressing the optic chiasm and the right optic nerve . There wTranssphenoidal endoscopic resection with intraoperative MRI and CT stealth stereotactic navigation was used to extensively decompress the optic nerve and chiasm and resect the paraganglioma. Intraoperative MRI demonstrated a gross-total resection of the tumor Figures .On histopathological evaluation, the tumor displayed features of a paraganglioma. The tumor cells were arranged in relatively well-defined nests  Figures  surroundThe patient is currently symptom-free with no recurrent tumor on repeat imaging 2.5 years after surgery.Paragangliomas arise from neural crest cells, but the exact pathogenesis of sellar PGLs is not completely understood. A widely accepted hypothesis indicates paraganglionic cells from cranial nerve IX (tympanic or ciliary branches) migrate into the cavernous sinus that is just adjacent to the sella turcica .The differential diagnosis for sellar tumors is large and includes pituitary adenomas (85%), craniopharyngiomas (3%), Rathke cleft cysts (2%), meningiomas (1%), and metastases (0.5%) . Other rAccording to a recent literature review, sellar paragangliomas are most commonly initially diagnosed as a pituitary adenoma (77.8%) followed by meningioma, craniopharyngioma, and macroadenoma . Of the The most common presenting symptom for sellar PGLs, as seen with our patient, is visual disturbances. Of the 19 sellar PGLs, patients' symptoms on presentation were visual disturbances/vision loss , headaches , hyperhidrosis , and one patient  with each of the following: gait imbalance, amaurosis, depression, anxiety, amenorrhea, proptosis, conjunctival vessel dilation, impotence, asthenia, dysthymic disorder, temporospatial confusion, abnormal behavior, galactorrhea, confusion, short-term memory loss, progressive lower limb hyposthenia, vertical nystagmus, arrested growth and sexual development, and hypopituitarism .The typical workup, if the patient is suspected to have a PGL or pheochromocytoma, consists of an MRI or CT of the abdomen and pelvis, a serum calcitonin level, 24-hour urine collection to measure catecholamine levels, and plasma-free fractionated metanephrines . Of headSince only 1–3% of head and neck PGLs are functional, they generally do not result in increased catecholamine levels which cause the elevated blood pressures, flushing, palpitations, and so on . As descth-6th decade for carotid and jugulotympanic, 5th decade for vagal, and 4th–6th decade for laryngeal PGLs [The average age of presentation is the 5eal PGLs . Of the eal PGLs . On the eal PGLs . None ofeal PGLs . The meteal PGLs . Only 1 eal PGLs . Additioeal PGLs , 12.CT and MRI are the most common imaging studies used in diagnosis with sensitivity of 95–100% and specificity of 67–70% . PGLs en10–50% of paragangliomas are secondary to a hereditary condition such as familial paraganglioma, neurofibromatosis type I, von Hippel–Lindau disease, Carney triad, and occasionally, multiple endocrine neoplasia type 2 (MEN2) . The mosDiagnosis is made based on histopathology with the classic description of “zellballen” or nesting of cells surrounded by abundant capillaries which are found in normal paraganglia . In the After diagnosis, management of head and neck PGLs differs based on the type. For functional PGLs or pheochromocytomas, patients will require preoperative blood pressure managements with alpha-blockers  and beta-blockers (propranolol) to avoid intraoperative hypertensive crisis . PrimaryThere are limited data on recurrence and overall survival rates of these patients. Of the 20 sellar patients, all patients are living at time of follow-up (months to years); however, many patients have postoperative morbidity with visual disturbances in 15% (3/20), diabetes insipidus in 15% (3/20), adrenocortical insufficiency in 5% (1/20), diplopia in 5% (1/20), persistent hyposthenia in 5% (1/20), basal ganglion infarction in 5% (1/20), headaches in 5% (1/20), and hypopituitarism in 5% (1/20) . PatientWhile, rare, paragangliomas should be kept on the differential for a sellar lesion. If there is a high index of suspicion based on symptoms, family history, elevated catecholamines, etc., an additional workup should be considered. Surgery is the mainstay of treatment, with no clear consensus for adjuvant therapy in the literature.


Both Enteric duplication and intestinal malrotation are concerning causes for intestinal obstruction in the pediatric age group and they very rarely coexist in the same patient.We present 2 cases of previously healthy children, the first is a 4-month-old infant and the second is a 1.5-year-old boy, both presented with recurrent attacks of bilious vomiting that proved to be due to acute midgut volvulus caused by an enteric duplication cyst associated with intestinal malrotation.Enteric duplication and intestinal malrotation are two of the concerning causes of billious vomiting in the pediatric age group. They could be encountered at any level of the alimentary tract from the tongue to the anus. The term malrotation refers to all abnormalities of intestinal position. The coincidence of intestinal malrotation and an enteric duplication cyst (EDC) is very rare and has been described only in a few case reports.The concomitance of EDC and intestinal malrotation is extremely rare and should be kept in mind in a child presenting with bilious vomiting especially in a child preoperatively diagnosed with a duplication cyst. Both Here we present 2 cases of previously healthy children, the first is a 4-month-old infant and the second is a 1.5-year-old boy, both presented with recurrent attacks of bilious vomiting that proved to be due to acute midgut volvulus caused by an enteric duplication cyst associated with intestinal malrotation.22.1A 4-month-old infant presented to our emergency department with a history of repeated attacks of bilious vomiting that started 2 weeks after birth. His examination revealed lax abdomen and large abdominal cystic lesion in the right hypochondrium.Radiological assessment entailed an erect abdominal X-ray which showed 3 gas bubbles and distal gasless abdomen a and an After initial resuscitation, abdominal exploration was done which showed malrotation, midgut volvulus, and jejunal duplication cyst b. Ladd’sHistology c of the 2.2A 1.5-year-old male presented to our emergency department with a history of recurrent attacks of bilious vomiting that started since he was 6 months old. Abdominal examination revealed a palpable huge cystic mass and bloody stool per PR. Abdominal ultrasound revealed evidence of markedly dilated small bowel loops with an abnormal rotation of the bowel and the umbilical region.After adequate preoperative preparation, abdominal exploration via transverse supraumbilical incision was performed. Ladd’s procedure was performed and excision of the duplicated jejunal segment (10 cm long) 100 cm from duodenojejunal flexure a, b. ThePatient started oral intake after 3 days.Histopathological examination of the excised segment revealed a portion of jujenal wall with underlying cyst formed of intestinal wall, with preserved muscle layer and regularly scattered neural elements, with small intestinal mucosal lining. In addition, there were areas of devitalization with moderate congestion and edema. Margins were free and viable3Enteric duplication and intestinal malrotation are two of the concerning causes of bilious vomiting in the pediatric age group. Enteric duplication is defined as a cystic or tubular structure that contains a well developed smooth muscle layer and lined with gastrointestinal mucosal lining. They could be encountered at any level of the alimentary tract from the tongue to the anus . The ter75% of patients with duplication cysts present within the first year of life usually with a picture of abdominal pain, vomiting or an abdominal mass. However, it may remain asymptomatic and accidentally discovered during laparotomy ,3,6. AcuDiagnosis of EDC can be reached through abdominal ultrasound preoperatively which can reveal an anechoic mass in the absence of ulceration and bleeding. The cyst’s wall is typically 2−3 mm in thickness and the mucosa shows a characteristic echogenic signal inside the cyst. Ultrasonography can also diagnose prenatal EDC ,7.Other diagnostic modalities like plain X-ray, Computerized tomography (CT), Magnetic Resonance Imaging (MRI) can aid in the diagnosis and the localization of the duplication cyst.technetium-99 m pertechnetate scan can be helpful in diagnosing Duplication cysts containing gastric mucosa and may differentiate them from mesenteric cysts ,8.Management of cystic EDC entails laparotomy with resection of the cyst and the adjacent bowel segment with primary anastomosis . As both4The concomitance of EDC and intestinal malrotation is extremely rare and should be kept in mind in a child presenting with bilious vomiting especially in a child preoperatively diagnosed with a duplication cyst. Preoperative ultrasound seems a reliable preoperative diagnostic modality.The work has been reported in line with the SCARE criteria .We declare no conflict of interest.This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.Our study is exempted from ethical approval in Institutional Review Board of our hospital.We have written informed parental consent for the two patients involved in this report for publication.Ahamed Azzam: operated on both cases and collected pictures.Heba Taher: Consultant in charge of case 1 operated supervised the writing process and corresponding author.Ahmed Arafa: consultant in charge case 2.Ali Nabil abdulkarim: literature search and drafting manuscript.Ahmed E.M. Shehata: literature search and drafting the manuscript.Ibrahim Mahran: operated on and assisted in case one and helped with drafting.Sherif Kaddah: head of Pediatric surgical department encouraged writing the manuscript.Aliyu Ahmed Anche: pediatric surgeon Turai Umar Yar‘adua maternal and children hospital Nigeria preparation of presentation.Sherifa tawfik: pathologist.Muayad shaban: preparation of pathology slides slides.This is a case report and not a human study. It is exempt from registering.Heba Taher.Not commissioned, externally peer-reviewed.


Seventy-five percent of the tumors are benign and 25 % are malignant.•Primary cardiac sarcoma is a rare malignant cardiac neoplasm with a poor prognosis.•No evidence-based guidelines exist regarding optimal surgical treatment of primary cardiac sarcomas.•Systemic chemotherapy is indicated for patients with widespread or unresectable malignancies. Primary cardiac sarcoma is a rare malignant cardiac neoplasm with a poor prognosis. No evidence-based guidelines exist regarding surgical treatment management, although some case reports have been published. We report the outcome of a patient with spindle cell sarcoma of the heart treated postoperatively with carbon-ion radiotherapy and chemotherapy.A 16-year-old female patient was noted to have abnormal electrocardiogram results for 2 consecutive years at a high school medical check. She was referred to our hospital and a cardiac tumor in the left atrium was suspected. The preoperative diagnosis was myxoma, but abnormalities were also observed macroscopically during surgery, and a diagnosis of spindle cell sarcoma was made by rapid intraoperative pathological analysis. We resected the tumor as much as possible with surgery, but the tumor in the left lower pulmonary vein could not be respected. We performed carbon-ion radiotherapy postoperatively. She experienced recurrence 1 year later and tumor growth 2 years later. We considered additional surgical treatment, but eventually selected chemotherapy. She died 4 years and 7 months after initial surgery.Spindle cell sarcoma is a rare disease with a poor prognosis. Although no optimal treatment method has been established, in the present case, incomplete surgical resection and postoperative carbon-ion radiotherapy were performed, yielding a relatively long survival period. About 25 % of these tumors are malignant, 34 % are angiosarcomas, and 24 % are undifferentiated sarcomas . Herein 2A 16-year-old female diagnosed with a left atrial (LA) tumor, suggestive of myxoma, was referred to our hospital. Electrocardiography conducted at high school enrollment revealed premature ventricular contraction. She was diagnosed with a LA tumor by transthoracic echocardiography (TTE) and was referred to us from a peripheral hospital. TTE showed a 16 × 20 mm mass arising from the anterior wall of the left atrium extending to the anterior mitral annulus. We diagnosed the patient with myxoma. Further assessment by enhanced computed tomography (CT) showed a large low-density mass (25 mm) in the left atrium. She underwent surgery 2 months later. TTE showed that the mass had increased to 19 × 28 mm.Tumor resection was performed. During surgery, the tumor was found to have an irregular LA surface of 30 × 30 mm, which invaded the left inferior pulmonary vein from the atrial septum a. We susAt 1 year after surgery, PET showed FDG accumulation in the left atrium and right clavicle outside the irradiation field, and she was diagnosed with recurrence and metastasis. CT showed tumor expansion a. The maAt 2 years after surgery, PET and CT showed that the LA tumor within the irradiation field had grown in size, with increased FDG accumulation compared with the previous examination b. BecausAt 4 years, 4 months after surgery, she developed palpitations and dyspnea, and the tumor had spread rapidly. Two months later, the tumor had grown to 55 × 33 mm c, althou3No established treatments for primary cardiac sarcomas are currently available. Therefore, systemic chemotherapy is indicated for patients with widespread or unresectable malignancies, and combined chemotherapy and radiation therapy is indicated for patients with primary cardiac lymphoma .Simpson et al. reported that this disease significantly differs from other malignant tumors and requires amelioration of circulatory dysfunction caused by the tumor before any type of tumor control . These investigators recommend tumor resection as first-line treatment. They observed that the median survival of patients with primary cardiac tumors was 17 months versus 6 months after complete versus incomplete resection, respectively, suggesting that the extent of surgical resection contributes significantly to survival. However, 73 % of patients experienced recurrence despite complete resection, and 83 % of patients with incomplete resection experienced recurrence. Furthermore, death has been reported to occur due to obstruction of intracardiac blood flow because of local recurrence prior to observation of metastases [Ramlawi et al. reported no significant differences in outcomes of cardiac sarcoma by side. Left-sided cardiac sarcomas are localized and less invasive, but may be associated with heart failure at an early stage. For cardiac sarcomas on the right side, survival rates were lower than those on the left side, because tumors were more likely to be extravasated, highly invasive, and metastatic . The autThe role of adjuvant therapy following surgery in this setting has not yet been defined. Hendriksen et al. demonstrated that median survival was more than 2-fold longer in patients after postoperative therapy compared with resection alone . In addition, multivariate analysis showed a significant association between an improved survival rate and postoperative therapy (p = 0.009), although no significant difference in 5-year overall survival rates was observed [Few reports about the use of CIRT to treat cardiac sarcomas have been published. In the present case, complete resection with an appropriate resection margin was not possible, so postoperative CIRT treatment was performed. Our patient developed LA metastases 1 year after CIRT. However, she was treated again with CIRT and chemotherapy and survived 4 years and 7 months. It is unclear whether CIRT would be effective for other patients with high-grade spindle cell sarcoma, because we have treated only one case. However, the patient experienced relatively long survival compared with previously reported survival rates for incomplete resection. Thus, CIRT may be an effective treatment for primary cardiovascular sarcoma.4No established cures for spindle cell sarcoma are currently available. Relatively good results have been obtained with surgery and postoperative treatment. In the present case, incomplete surgical resection and postoperative carbon-ion radiotherapy were performed, resulting in relatively long survival.There are no conflicts of interest to report.None.On the basis of this being a case report, the Institutional Review Board of the Dokkyo University does not mandate that ethical approval is required. Thus, this case report is exempt from the Institutional Review Board Approval process.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.Study concept, design, final proofreading: Ikuko Shibasaki.Data collection: Shigeru Toyoda, Yusuke Takei.Advised and designed the report: Masayuki Chida, Hirotsugu Fukuda.Not Applicable.Ikuko Shibasaki.Not commissioned, externally peer-reviewed.


A cardiac myxoma in a young person may pose a diagnostic challenge as symptoms may be variable and the differential diagnosis is wide. The differential diagnosis can include rheumatic mitral valve disease, pulmonary hypertension, endocarditis, myocarditis and vasculitis.This case report involves a 49 years old female with a 2.8 cm × 3.4 cm myxoma in the left atrium causing mitral valve obstruction. She presented with fatigue, fever of unknown origin, transient ischemic attack and shortness of breath. Prompt surgery is often recommended due to the risk of embolic complications or complete obstruction. Due to her symptoms, patient underwent successful cardiothoracic surgery to excise the myxoma within 2 weeks of confirmation by cardiac echocardiography.This case also emphasizes the diagnostic challenge as symptoms may be variable, ranging from fatigue, fever and shortness of breath to transient ischemic attack and at worst, sudden cardiac death. In conclusion, if a cardiac mass is suspected, echocardiography should be performed early. Surgical resection is curative and recurrence rate is very rare in sporadic isolated myxomas, however, recurrence can be higher in genetic diseases associated with increased frequency of myxomas such as Carney complex. This subpopulation of patients may present further research opportunity to learn more about the perioperative management of patients with myxomas such as determining the optimal time to surgical intervention and decision to anticoagulate. This case report involves a 49 years old female with a myxoma in the left atrium causing mitral valve obstruction. She presented with fatigue, fever of unknown origin, transient ischemic attack and shortness of breath. Prompt surgery is often recommended due to the risk of embolic complications or complete obstruction. Due to her symptoms, patient underwent successful cardiothoracic surgery to excise the myxoma. Although the surgery was a success, this case emphasizes 3 main points. First, a myxoma in a young person may pose a diagnostic challenge as symptoms may be variable and the differential diagnosis is wide. The second point is that the cardiac mass in her case was unique in terms of size. The excised specimen was measured as 6.2 × 4.3 × 2.9 cm which would cause significant adverse hemodynamic effects on the heart. The third point is the emphasis on diagnostic echocardiography when a myxoma is suspected or if other diagnostic workup has been negative.Primary cardiac tumors are rare, with an incidence of 1.38 per 100,000 people per year . About 7The patient is a 49 years old female, with no significant medical history, who started experiencing symptoms of reduced exercise tolerance and easy fatigability along with increased vulnerability to viral illnesses that started about 1 year ago. She started having intermittent fevers of 100.5 °F with cough and rhinorrhea which was diagnosed as bronchitis and she was prescribed azithromycin. She also developed abdominal cramping and diarrhea and her CRP at that time was found to be elevated to 40.6 mg/L . Abdominal CT did not show any abnormalities. The gastrointestinal symptoms and viral illness gradually resolved over the next month. A few months later, however, she suffered stroke like symptoms while at work and she was admitted for vertigo and presyncope. Her National Institutes of Health stroke scale score was 0 and Computed Tomography (CT) scan of her brain was negative for acute pathology. She was diagnosed with peripheral vertigo. Throughout the year, she continued to have fatigue and reported a 10 lbs. weight loss. Her CRP also continued to be elevated, reaching a zenith of 129 mg/L . At this time, her fatigue, shortness of breath and heat intolerance became more pronounced. She continued to experience intermittent fevers with night sweats for about 3 weeks, prompting an infectious disease workup. The Infectious Disease specialist started her on Cefuroxime for 5 days, which did not help, and then switched her to Doxycycline for 10 days with inconclusive results. CT chest with contrast was ordered but unfortunately, she was unable to obtain the imaging due to delays with her health insurance. She was then referred to a pulmonologist to evaluate for possible pulmonary etiology. The pulmonologist was able to order the CT chest but imaging was obtained without contrast which did not identify any pathology such as pneumonia  was performed which did not find a defect in the septum but did confirm a thin septum and presence of mild mitral valve regurgitation. Cardiac myxomas are a risk factor for valvular heart disease as the myxoma can push violently past the mitral valve during the cardiac cycle . UnfortuThe diagnostic modality of choice is an echocardiography, which identifies the size, site, attachment, mobility, and also grossly differentiates the myxoma from vegetation or a thrombus, with transesophageal echocardiography as the preferred choice . CT imagMyxomatous tumors are generally divided into solid or papillary groups based upon gross external features . Tumors Embolization commonly occurs in about 35% of all left atrial myxomas, whereby it is actually the thrombus on the surface that embolizes more than the tumor itself and a smaller tumor of < 4.5 cm poses an increased risk. Embolization can occur in the lower extremities such as the iliac and femoropopliteal, viscera, spleen, adrenals, kidneys, and even the abdominal aorta [In terms of treatment, organized and small thrombus can be treated medically with anticoagulants, thrombolysis or thrombectomy . AnticoaIn terms of myxomas, despite the fact that the majority of such tumors are benign, surgical resection is highly suggested to prevent complications including sudden death . SurgicaAlthough it is generally agreed that an intracardiac mass requires surgical intervention, there is no consensus in terms of the timing for the excisional surgery. For instance, in the case of the patient with the left atrial myxoma Fig. a and b pThe topic of Carney complex is also interesting in this case. Carney complex (CNC) is an autosomal dominant multiple neoplasia syndrome that includes cardiac myxoma, endocrine, cutaneous, and neural tumors . Extra-cAdditional past medical history from the patient includes two right knee effusions performed 20 years ago, roughly age 30, and diagnosed with Pigmented villo-nodular synovitis (PVNS). The incidence of PVNS ranges around 1.8 per million, usually monoarticular, affecting large joints, with the knee being the most common site. Microscopically, the synovial membrane is characterized by inflammation and presence of hemosiderin deposits, lipid-laden macrophages, multinucleated giant cells and proliferation of fibroblasts and stromal cells [The patient also had basal cell carcinoma of the right upper lip with surgery 8 years ago, at around age 40. She also had squamous cell carcinoma of the right lower abdomen also requiring excision this year, at age 49. Incidentally, patient also recalls she had a dark lentigines (liver spot) on her left upper arm as a child which resolved in adulthood.Although PVNS, basal cell carcinoma and squamous cell carcinoma are not necessarily associated with cardiac myxomas, perhaps the patient may have a variant of Carney complex that involves dermatologic and soft tissue proliferation. Furthermore, her upper lip skin lesion may have been a cutaneous myxoma that was misdiagnosed as a basal cell carcinoma since they are both flesh colored. The squamous cell carcinoma may have been a pigmented nevus common in CNC. Given the rarity of sporadic primary cardiac myxoma, incidence of 0.5 per million population per year , and theThis case report illustrates the finding of a cardiac myxoma in a young patient causing intermitent fevers, dyspnea on exertion, and extreme fatigue. Although the patient initially presented with bronchitis and URI symptoms, further diagnostic steps eventually revealed the finding of the intracardiac mass. Clinical features of myxomas encompass a triad of arterial embolism, obstruction of intracardiac blood flow, and constitutional signs often include fatigue, fever, weight loss, and elevated CRP. This array of symptoms poses a diagnostic challenge as the differential diagnosis can include rheumatic mitral valve disease, pulmonary hypertension, pulmonary embolism, endocarditis, myocarditis and vasculitis. This patient’s episode of vertigo and presyncope may have been a transient ischemic attack associated with micro emboli from the myxoma. Similarly, the intermittent fevers were likely due to associated inflammatory cytokines such as interleukin-6 and other acute phase reactants such as CRP. In cases of diagnostic uncertainty involving symptoms of dyspnea on exertion, fatigue and fevers, a chest CT with contrast enhancement has clear benefits over chest CT without contrast. If the patient’s renal function presents a limitation in using contrast, then a nephrology consult may be appropriate. Cardiac echocardiography can then be used to confirm the diagnosis with subsequent cardiothoracic surgical evaluation. Although surgical resection is curative and recurrence rate is low, patients may benefit from serial echocardiograms in the outpatient setting.


Spontaneous regression of pituitary tumours are rare and can be due to tumour ischaemia, pituitary apoplexy, or lymphocytic hypophysitis. We report a case of a 32-year-old female, who presented with symptoms and signs of extrasellar pituitary enlargement and hypothyroidism. MRI revealed a pituitary mass that spontaneously regressed after a month, with complete resolution of symptoms. Not all pituitary tumours require surgical intervention especially in the case of autoimmune lymphocytic hypophysitis. Pituitary tumours are common intracranial neoplasms, they account for 10% to 15% of all intracranial tumours of which 90% of them are adenomas . SpontanA 32-year-old female presented with three months history of headache, amenorrhea, and lethargy, followed by worsening of nausea and vomiting. Apart from that, there was no visual defect, weight loss, or symptoms suggestive of diabetes insipidus. She is single and denied any personal or family history of autoimmune disorders. Her body mass index was 23 and had no neurological deficit. Initial MRI showed a pituitary mass with thickened stalk, extending into the cavernous sinus with a small gap from optic chiasm Figure .She was hypothyroid at presentation, thus started on levothyroxine 50 ug daily. Other pituitary hormone profiles were within the normal range. Liver function tests were also normal. However, her fasting lipid profiles showed high total cholesterol 8.16 mmol/L (<5.18), with low-density lipoprotein (LDL)-cholesterol of 6.24 mmol/L (<3.80) and triglycerides of 2.14 mmol/L (<1.7). She was initially referred to the neurosurgical team surgery, however, a repeat MRI one month later showed marked shrinkage of the pituitary tumour . Of note, hyperprolactinaemia can also cause amenorrhea, but her serum prolactin was normal. Her initial MRI showed pituitary macroadenoma and she was planned for surgery. However, a repeat MRI scan prior to surgery showed tumour shrinkage.Lymphocytic hypophysitis is an autoimmune disease in which the pituitary gland is infiltrated by lymphocytes, plasma cells, and macrophages, causing impairment of its function. The pathogenesis is still unclear. An autoimmune pathogenesis as well as a viral origin has been suggested . Prior tMRI scan is the best imaging diagnostic tool in differentiating lymphocytic hypophysitis from pituitary adenomas. MRI scan features suggestive of lymphocytic hypophysitis include symmetrical enlargement of the pituitary gland, thickened pituitary stalk, and an intact sellar floor. In contrast, pituitary adenomas are usually asymmetric, often displacing the infundibulum, rarely involved the stalk or erode the sellar floor ,5. They In this particular case, our patient was diagnosed with lymphocytic hypophysitis based on her clinical presentation and MRI scan features of thickened pituitary stalk which favours the diagnosis of autoimmune hypophysitis. Even though it commonly affects women in their late pregnancy or in the early postpartum period, cases occurring outside pregnancy is not unusual. It also usually affects those with autoimmune diseases. Interestingly, our patient was nulliparous and had no autoimmune disease or family history as such.Although it is rare, pituitary apoplexy regression, which is clinically silent and radiologically undetectable tumour ischaemia, should be considered in the differential diagnosis. Pituitary apoplexy occurs when the large tumour compresses or outgrows the blood supply resulting in ischaemic necrosis and haemorrhage into the necrotic tumour ,6,7. It This patient was treated with oral levothyroxine for her hypothyroidism. Subsequent follow-up showed normalization of her thyroid function tests and complete resolution of her symptoms. As the pituitary gland had significantly decreased in size, surgery was no longer indicated.Autoimmune lymphocytic hypophysitis should be considered in cases of spontaneous regression of pituitary tumours. We also conclude that in the absence of visual field defects, observation alone is a safe alternative for surgery, especially in patients without compromised pituitary function and without compression of the optic chiasm.


Hypercalcemia of malignancy is not uncommon in patients with advanced stage cancer. In rare cases the cause of the hypercalcemia is excessive production of calcitriol, the active form of vitamin D. Although inappropriate tumoral secretion of calcitriol is typically associated with lymphomas and some ovarian germ cell tumors, we present a case of calcitriol overproduction-induced hypercalcemia due to a pancreatic neuroendocrine tumor. The high expression of somatostatin receptors on this neuroendocrine neoplasm opened up the opportunity to treat the patient with radiolabelled somatostatin analogs, which successfully controlled the refractory hypercalcaemia and calcitriol levels. This case documents a rare finding of refractory hypercalcaemia of underlying malignancy due to a calcitriol-producing pancreatic neuroendocrine tumor, responding to peptide receptor radionuclide therapy (PRRT).177Lu-DOTATATE PRRT resulted in a morphological response and a normalization of serum calcium levels, confirming the hypothesis of a calcitriol producing pancreatic neuroendocrine tumor. Progression of liver metastases warranted further therapy and temozolomide-capecitabine was started with morphological and biochemical  stabilization.A 57 years-old patient presented with back pain, general discomfort, polydipsia, polyuria, fatigue and recent weight loss of 10 kg. Clinical examination was normal and there was no relevant medical history. Biochemical evaluation showed hypercalcemia with markedly increased calcitriol levels. CT-thorax-abdomen and ultrasound guided biopsy revealed a pancreatic neuroendocrine tumor with multifocal liver metastases, suggesting that excessive overproduction of calcitriol by this neuroendocrine tumor was the cause of the refractory hypercalcemia. The patient was eligible for PRRT. Four cycles of Although up to 30–40% of gastroenteropancreatic neuroendocrine tumors are known to be functional (i.e. producing symptoms associated with the predominant hormone/peptide secreted), calcitriol secreting pancreatic neuroendocrine tumors are very rare. However, treatment with PRRT resulted in normalization of calcium and calcitriol levels, strongly supporting the hypothesis of a calcitriol-producing pancreatic neuroendocrine tumor. Hypercalcemia without elevated parathyroid hormone (PTH) level is most frequently due to an underlying neoplasm , 2. This68Ga-DOTATATE) or targeted radionuclide therapy (eg. 177Lu-DOTATATE). In case of inoperable/metastatic well-differentiated GEP-NETs, this so-called Peptide Receptor Radionuclide Therapy (PRRT) is an established treatment modality [The high incidence of somatostatin receptors (SSR) on gastroenteropancreatic neuroendocrine tumors (GEP-NETs) provides imaging and therapeutic options. Somatostatin receptors overexpression is the basis of pharmacological treatment with non-radioactive somatostatin analogs (SSAs) (eg. Octreotide) . SSAs camodality  whose romodality . Furthermodality , and evimodality . This caA 57 years-old male patient presented with back pain, general discomfort, polydipsia, polyuria, fatigue and recent weight loss of 10 kg. Clinical examination was normal and there was no relevant medical history. Biochemical evaluation showed hypercalcemia  with slightly lowered PTH levels  excluding hyperparathyroidism. CT-thorax-abdomen and ultrasound guided biopsy revealed a pNET with multifocal liver metastases as well as some small bone lesions. The Ki-67 index was 15 to 20% compatible with a grade 2 tumor. PTHrP was normal and although our patient had some osteodense skeletal metastases, these bone lesions alone could not explain his marked hypercalcemia. However, markedly increased calcitriol levels up to 134.3 ng/L  were detected. We hypothesized that overproduction of this active form of vitamin D by the pNET was the cause of the HCM.68Ga-DOTATATE scan revealed intense SSR expression in the pancreatic lesion as well as strong uptake in the liver metastases and the skeletal metastases. All malignant lesions had an uptake intensity above the spleen (Krenning score grade 4) [18F-FDG-PET/CT showed strong hypermetabolism in some of the liver metastases (metabolic grade 3) [18F-FDG + / SSR - mismatched lesions. Evaluation of the renal function showed no contraindication for therapy. Four cycles of PRRT with 177Lu-DOTATATE were given, with a treatment interval of 8 weeks up to a cumulative activity of 29.6 GBq. Three months after the final cycle, the initially refractory serum calcium levels had normalized and the associated symptoms disappeared , resulted in morphologically stable disease, but there was no effect on the hypercalcemia nor on the associated symptoms. The patient was evaluated for treatment with PRRT. In the meantime, therapy with FOLFOX chemotherapy was started. grade 4) . 18F-FDGgrade 3) . There wHypercalcemia without elevated PTH level is most frequently due to HCM. However, the normal PTHrP values and the absence of osteolytic bone metastases, ruled out PTHrP production and skeletal osteolysis respectively. Instead, the strongly elevated calcitriol levels suggested a third, more rare cause of HCM: calcitriol overproduction-induced hypercalcemia due to a pNET. To the best of our knowledge, only 2 other cases have described calcitriol-producing pNETs , 26, bot68Ga-DOTATATE scan revealed intense SSR expression in the pancreatic lesion, the liver metastases and the rare skeletal metastases. Strong hypermetabolism in some of the liver metastases, suggested increased aggressiveness in those lesions [18F-FDG + / SSR -) lesions were detected, so there was no significant dedifferentiation which would be a contra-indication for treatment with PRRT [ lesions . Howeverith PRRT . Since PNeuroendocrine neoplasms are known to be functional in up to 30–40% of the cases, but calcitriol secretion by a pNET has only rarely been described. However, treatment with PRRT resulted in normalization of calcium and calcitriol levels, strongly supporting the hypothesis of a calcitriol-producing pancreatic neuroendocrine tumor.


Rare disease like primary spinal epidural diffuse large B‐cell lymphoma should be considered as a differential diagnosis in patients presenting with back pain and rapid neurological deterioration in the lower extremities. Rare diseases like primary spinal epidural diffuse large B‐cell lymphoma should be considered as a differential diagnosis in patients presenting with back pain and rapid neurological deterioration in the lower extremities. MRI revealed a spinal epidural tumor at thoracolumbar junction. He underwent decompression and excisional biopsy. Histopathology and immunohistochemistry identified it as diffuse large B‐cell lymphoma. He received chemotherapy and is asymptomatic at 1‐year follow‐up.Primary spinal epidural lymphomas (PSELs) comprise a group of tumors, which are present only in the spinal epidural space, with a histopathological picture of lymphoma and negative diagnostic workup for lymphoma at other sites.This report describes the clinical features, imaging characteristics, and histopathological features of the rare case of primary spinal epidural diffuse large B‐cell lymphoma (DLBCL). To the best of our knowledge, this is the first reported case of primary spinal epidural DLBCL from Nepal. It also emphasizes the importance of a multidisciplinary approach for the successful treatment of this disease.2A 24‐year‐old man presented with a 3‐month history of low back pain associated with the burning sensation of bilateral lower limbs. For the last 12 days, he was unable to walk due to gradually progressive weakness in bilateral lower limbs. These symptoms were not accompanied by fever, headache, and night sweats. He could not recall any history of trauma in the past. His past medical and surgical history was not significant.On examination, there was a localized tenderness over the thoracolumbar region. Lower limb motor power varied across muscle groups: hip flexors , knee extensors , ankle dorsiflexors , long toe extensors , and ankle plantar flexors . The sensation of lower limbs was altered bilaterally, but bowel and bladder habits were normal. Deep tendon reflexes in the knee and the ankle joints were absent. Plantar reflexes were downgoing bilaterally.With the clinical diagnosis of a space‐occupying lesion in the thoracolumbar spine region, an X‐ray of dorsolumbar spine was done. Apart from the loss of lumbar lordosis, the rest of the X‐ray findings were normal. Magnetic resonance imaging (MRI) of the thoracolumbar spine revealed extradural lesion extending from T10 to L2 vertebral level. T1W image showed homogenous lesion, which was iso to hypointense and the T2W image showed heterogeneous lesion, which was iso to hyperintense, compressing over the spinal cord dorsally Figures . Chest X−, CD20+, CD30+, CD10+, ALK‐1−, BCL‐6+, MUM‐1−, and Ki‐67+. No other lesion was detected on staging evaluation, which included contrast‐enhanced computed tomography (CECT) of chest, abdomen, and pelvis, and bone marrow aspiration and biopsy.Decompression (laminectomy) and excision biopsy of the mass were planned. The thoracolumbar spine (T9 to L3) was approached midline posteriorly. After laminectomy, a dorsally located greyish red extradural tumor extending from T10 to L2 was removed in strips. One of the long strips of the excised mass (approximately 15 × 1 cm) was sent for histopathological examination Figure . HistopaHis postoperative period was uneventful with successful recovery in his neurological symptoms. He was referred to a cancer center after 16 days of hospital stay where he obtained six cycles of chemotherapy with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone (R‐CHOP) regimen. The interval between each cycle of chemotherapy was 3 weeks. Hematological parameters during and after the completion of chemotherapy were within normal limits. After the treatment, the tumor regressed, and he fully regained his muscle power. At the latest, 1‐year follow‐up, he is asymptomatic and is performing his routine daily activities.3Lymphomas are malignant tumors arising from lymphoid tissue. It can be Hodgkin's or non‐Hodgkin's type, later being more common, and their primary epidural occurrence is very rare.The pathogenesis of primary central nervous system lymphomas (PCNSLs) has been a matter of considerable debate among experts. Some authors mention that PCNSL arise de novo in nervous system tissues due to the presence of lymphoid precursor cells in this location.There is a male preponderance (69% vs 31%) of PSEL matching exactly with our case.The thoracic spine is the most commonly involved site, followed by the lumbosacral and cervical spine.The onset of symptoms is usually subacute, occurring over a few days to weeks. The symptoms and signs of the disease depend on the location and size of the tumor. Localized back pain followed by lower limb weakness is the most common symptom.B symptoms  were rare, and none had hepatomegaly or splenomegaly in one of the largest surveys, which is consistent with our case. As most of the patients presented in the earlier stage, B symptoms were probably absent.MRI is the investigation of choice in the preoperative and postoperative evaluations.The whole‐body scan should be performed to identify lungs, pleural, splenic, gastric, intestinal, pancreatic, and renal involvement. Bone marrow biopsy from the sternum or iliac crest is required to rule out lymphoreticular involvement. CBC, LDH, and chest X‐ray should be performed as well. In our case, we could not find any positive reports. Lumbar puncture is not recommended, because it is potentially hazardous because of the coning phenomenon and it is also of limited diagnostic value as it shows a non‐specific increase in protein levels; neoplastic cells are rarely found.Monnard et al+, CD10+, BCL6+, and MUM‐1− further supported our diagnosis of DLBCL .Histopathological examination of the biopsy specimen usually shows diffuse proliferation of atypical lymphoid cells,Combined modality treatment, including radiotherapy and chemotherapy, has been recommended to be the most efficient treatment because lymphomas are very chemo‐ and radiosensitive tumors.4Primary spinal epidural DLBCL is one of the rarest tumors due to which its diagnosis is challenging. Signs and symptoms are similar to those of any other epidural space‐occupying lesions and, thus, can be frequently misdiagnosed. Back pain with sudden neurological deterioration in lower limbs should raise the suspicion of rarer diseases like primary spinal epidural DLBCL. A high degree of suspicion and a team approach involving multiple specialties  is vital for diagnosis and management.None declared.NBP, RP, and SP: wrote the initial draft, edited it, and reshaped into this manuscript. DK and RKP: reviewed the manuscript. All authors: approved the final version of the manuscript and agreed to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.Need for ethical approval waived. Consent from the patient deemed to be enough.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the editor‐in‐chief of this journal.


Teratomas are one of the most common germ cell tumors, and they usually occur in ovaries. Extragonadal teratomas are rare, especially immature ones. Only several cases of primary teratomas of the uterus have been reported since 1929. Here, the case of an 11‐year‐old patient who had a 6‐month history of sustained abnormal vaginal discharge is presented. Transabdominal ultrasonography revealed a solid mass in her uterus, resulting in the patient undergoing surgery. Examination of PET‐CT scans revealed a mass in the right ovary of the patient 20 days after surgery. The patient underwent a second surgery followed by chemotherapy. This is the youngest case among reported patients of primary immature uterine teratoma, and this patient showed no evidence of recurrence during 2 years of follow‐up. Teratomas are one of the most common germ cell tumors classifiable as mature or immature.An 11‐year‐old girl with no special medical history went to the hospital in February 2018 with a 6‐month history of sustained abnormal vaginal discharge. A mass could be seen in the orifice of her vagina through a gynecologic examination. Transabdominal ultrasonography revealed a solid mass measuring 11 × 6.5 cm from the uterus to the vagina with heterogeneous internal echo Figure . Tumor m2, days 1–2) and cisplatin  every 3 weeks. The patient was well after 2 years of follow‐up post‐surgery. No residual tumors were found through regular examinations of ultrasound and CT scans. As well, the patient no longer has any abnormal symptoms.Examination of ultrasound and CT scans showed no residual teratoma after the operation, but a mass was present in the right ovary of the patient 20 days after surgery. It measured about 3.1 × 3.2 cm in size, with fat density and dot‐strip calcification, suggesting that it may be a teratoma. The mass was not noticed before. Inevitably, the patient underwent a second operation via a laparoscope in March 2018. During the operation, a 3.1 × 3.2 cm mass was removed from the right ovary. Microscopically, the mass was revealed to be mature teratoma Figure . The folExtragonadal germ cell tumors can arise anywhere along germ cell migration routes.Two main theories about uterine teratoma origins can be discussed. The first theory suggests that they arise from primordial fetal germ cells when migrating abnormally from the fetal yolk sac endoderm to the gonadal ridge during early embryogenesis. These tumors are considered to arise in middle structures like the uterus.In uterine teratoma reports, patient ages ranged from 15 to 90. In this case, the age of the patient, 11‐year‐old, was the youngest in all reports since 1992. Usually, no special symptoms occur with uterine teratoma, including vaginal bleeding, lower abdominal pain, pelvic inflammation and other single or combined symptoms.No uniform consensus exists regarding chemotherapy and radiotherapy of uterine immature teratomas.None declared.


Fecal impaction is common in elderly, bed ridden, schizoaffective patients on antipsychotics. Intestinal obstruction due to distal colonic fecaliths is rare as it is amenable to digital manual evacuation and enemas. Our patient presented with abdominal distention, with last bowel evacuation reported 3 months ago. Computed tomography (CT) abdomen demonstrated a huge sigmoid fecalith causing bilateral hydronephrosis. He was managed through laparotomy with sigmoid colon resection and end colostomy. We present the case of a fecaloma presenting as a huge abdominal mass. Fecal impaction is common in bedridden and debilitated elderly patients. Intestinal obstruction due to prolonged fecal impaction in the distal colon is rare as it is amenable to manual evacuation and enemas.A 48‐year‐male, schizophrenic on clozapine, presented with complaints of progressive abdominal distention, mild abdominal discomfort, and recurrent episodes of vomiting for 10 days. He had not passed stool for the last 3 months. Previously, the patient used to move bowel after a week and occasionally used enemas for constipation. During these 3 months, the patient took laxatives and enemas but did not pass stool. Clinical examination demonstrated a pulse of 89 beats/min, blood pressure (BP) 110/80 mmHg, and respiratory rate of 14 cycles/min. Examination showed a hugely distended abdomen with a firm irregular mass occupying the left iliac, hypogastrium, right Iliac fossa, and right lumbar quadrant of the abdomen. Bowel sounds were absent, and no bruit was heard. On rectal examination, anal tone was normal, and a hard mass was felt in the rectum.Complete blood count was within normal limits. Serum chemistry demonstrated urea, 80 mg/dL; creatinine, 2.37 mg/dL; and serum albumin, 2.82 gm/dL. Abdomen radiograph showed a huge mass occupying almost the whole abdomen with no gas in the bowel loops. Computed tomography (CT) abdomen showed grossly distended rectosigmoid colon filled with hyperdense material suggestive of fecal matter and dilated descending colon Fig. a,b.In view of the marked distension of the sigmoid colon with impending perforation, the patient was subjected to laparotomy. Intraoperative findings indicated a markedly distended sigmoid colon approximately 30 cm in diameter containing hard fecal matter Fig. c,d. The Fecal impaction is defined as hard stool in the intestinal tract not evacuated spontaneously.


This article reports a case of a 7-year-old girl with Turner syndrome, treated with growth hormone (GH), who developed ovarian dysgerminoma. The patient karyotype was mosaic for chromosome Xq deletion: 46,X,del(X)(q22)/45,X. No Y chromosome sequences were present. Molecular studies revealed the presence of a driving mutation in exon 17 of the KIT gene in the neoplastic tissue, as well as Sonic-hedgehog (SHH) pathway activation at the protein level. The patient responded well to chemotherapy and remained in complete remission. This is the first case of dysgerminoma in a Turner syndrome patient with such oncogenic pathway. Turner syndrome  is a chThe pathogenicity of ovarian GCT is largely heterogenous with significant age-dependency. Several theories of GCT have been proposed, such as the arrest of gonocyte migration and maturation, blockade of apoptosis, mitosis–meiosis switch alterations, miRNA dysregulation, neoplastic niches, and signaling pathway changes. Several pathways are involved in gonadogenesis and intragonadal germ cell–stroma interactions. One of them is crucial in embryogenesis—Hedgehog (Hh) signaling, whose misactivation occurs in different human tumors. Moreover, few cases of mutations in Hh coding genes have been reported to play a role in gonadal dysgenesis and are associated with GCT formation ,9,10,11.KIT. Two distinct but overlapping pathways of ovarian GCT development exist. In the first pathway, the triggering factor is the presence of constitutional Y chromosome material in individuals with defects in their sexual development  who present clinically as phenotypic females. The second results from spontaneous KIT activation leading to increased survival and the proliferation of undifferentiated oogonia /45,X[19] karyotype leading to a clinical diagnosis of TS. GH treatment with 5 mg somatropin  once daily was initiated 4 months after the diagnosis of TS. The GH dose corresponded to the levels of insulin-like growth factor 1 (IGF-1) and insulin-like growth factor binding protein 3 (IGFBP3) in the patient’s serum. During GH-therapy, the IGF-1 and IGFBP3 levels were always in the normal range for the patient’s age and gender. A good response and accelerated growth were observed.Two years and 8 months after the initiation of GH therapy, the patient was admitted to a hospital due to fever and abdominal pain lasting for three days. Physical examination revealed a palpable mass in the lower abdomen. Upon ultrasonography, the presence of a heterogeneous tumor of 6 × 4 × 9 cm with calcifications was confirmed. Laboratory tests showed increased levels of lactate dehydrogenase (LDH) (568 U/L), Ca125 (39.7 U/mL), fibrinogen (5.61 G/L), D-dimer (5198 µg/L FEU), and C-reactive protein (CRP) (13.87 mg/L). The serum beta-hCG, alpha-fetoprotein (AFP), Ca15.3, and Ca19.9 were within normal limits. The contrast MRI demonstrated a well-defined 65 × 69 × 95 mm solid and cystic mass likely originating from the ovary and infiltrating the peritoneum, accompanied by enlarged retroperitoneal and iliac lymph nodes. The uterus was relatively small, and evident ovaries were not identified bilaterally. GH therapy was then stopped.A laparoscopic biopsy of the neoplastic mass was then performed. Microscopically, the tumor consisted of nests and sheets of uniform large oval cells with prominent nucleoli and low mitotic activity, surrounded by lymphocytic infiltrate. The histology and immunophenotype , as well as negativity for CD30, Glypican 3, and cytokeratins) were consistent with a diagnosis of dysgerminoma .Subsequently, the patient received three cycles of chemotherapy with vinblastine, bleomycin, and cisplatin according to the French protocol TGM 95—Strategie therapeutique des tumeurs germinales malignes extra-cerebrales de l’enfant. A partial radiological response to the treatment was observed. An ultrasound exam after the third chemotherapy cycle showed a partial reduction in the tumor. The patient underwent a bilateral salpingo-oophorectomy and an iliac lymph node resection. Histopathological examination revealed complete regressive changes within the tumor and reactive lymph nodes without metastases. The opposite gonads with diameters 13 × 9 × 6 mm contained fibrous tissue and ovarian stroma with very few primary follicles corresponding to a streak gonad.KIT NM_000222.2: c.2466T>A  resulting in the amino-acid substitution of asparagine with lysine in the codon 822 at the hot-spot exon 17 of the gene was present with a 15% variant allele frequency. In parallel, analysis of the 23 STR loci ruled out the presence of chromosome Y  in DNA extracted from the tumor sample.DNA was extracted from the paraffin embedded tumor sample and subject to a mutational analysis of the 25 genes associated with GCT pathogenesis and/or encoding key proteins of the Hh pathway  using thIn addition, SHH pathway protein expression was examined with a panel of antibodies by Abcam  using the routine immunohistochemistry technology DAKO Agilent. The neoplastic cells showed SHH cytoplasmic staining, nuclear GLI1, PTCH1, SUFU expression, as well as a membranous SMO reaction in parallel with GLI2 negativity.The study was conducted in accordance with the Declaration of Helsinki, and the protocol was approved by the Ethics Committee of the Medical University of Gdansk, Poland . The parents of a child gave their informed consent for inclusion before they participated in the study.The etiology of TS involves the dysregulation in meiosis signaling to germ cells, which may result in nondisjunction and monosomy X due to separation failure of the chromosomes in either the parental gamete or during early embryonic divisions. Growth failure affected almost all TS patients, and their final height was approximately 20 cm shorter than the mean adult height. The initiation of GH therapy in TS is recommended early, at approximately 4–6 years of age ,16. An SApproximately 10% of TS patients are mosaic for Y-chromosome material in their genomes . DetectiKIT gene ) encoding the growth factor receptor important for normal germ cell migration and development. As the result of a somatic mutation leading to the activation of KIT, sustained survival, proliferation, adhesion, and motility were observed. The detected pathogenic variant resulted in a gain-of-function alteration of the c-KIT receptor. A recent study of 87 ovarian GCTs revealed a homogeneous profile of this group of tumors, with recurrent somatic exon 17 KIT mutations in 16.7% of all samples including 44.4% of dysgerminomas and mixed forms with a dysgerminoma component [KIT mutations are considered the primary recognized driving mutation behind ovarian GCT development [Conversely, comprehensive genetic studies of the neoplastic tissue revealed the presence of a somatic pathogenic variant in exon 17 of the elopment . In the KIT activation as the driver of oncogenesis. Previously, Gravholt et al. reported a single case of a metastatic embryonal carcinoma in a 40-year-old female with a 45,X chromosomal constitution without signs of the Y chromosome. Remarkably, whole exome sequencing revealed that the tumor harbored a gain-of-function pathogenic variant in exon 13 of the KIT gene  and additional putative somatic pathogenic variants in the AKT1 and ZNF358 genes, as well as a few genomic copy-number variations of uncertain importance [Our patient appears to be the first proven pre-pubertal case of TS featuring no Y chromosome material but somatic portance . The patportance ,27,28.DHH, IHH, SHH, PTCH1, SMO, SUFU, GLI1, GLI2, and GLI3) were detected in the molecular analysis. SHH expression was present within the cytoplasm of the neoplastic cells, which suggests an autocrine function in signaling [KIT expression, irrespective of KIT/PDGFRA mutational status [Gonadal dysgenesis in TS cases may be due to the impairment of theca cell–oogonia interactions during gonadal development and differentiation during early fetal development, when female germ cells proceed into meiosis and begin folliculogenesis. In neonates, theca cell lineage requires both granulosa cells and oocytes through multicellular interactions via GDF9 and Hh signaling. In the absence of the Hh ligand, theca progenitor cells fail to differentiate into androgen-producing theca cells, and ovarian folliculogenesis is disrupted. The studied tumor showed the expression of Hh signaling pathway proteins with features of this pathway’s activation. The nuclear expression of GLI1 is also evidence of pathway activation . No pathignaling . The obsignaling . Interesl status .In summary, even in the absence of chromosome Y-fragments, GTCs may develop in otherwise streaky gonads in TS patients through somatic KIT gene activation with probable supporting SHH pathway activation. Such molecular background constitutes a therapeutic target in disseminated high risk tumors.


Cavernous hemangioma is an encapsulated nodular mass composed of dilated, cavernous vascular space separated by connective tissue stroma. Flattened endothelial cells line the vascular spaces, which were filled with blood. Though hemangiomas are the mast common benign neoplasms seen in children, they rarely occur in adults. In the head and neck region, the masseter and trapezius muscles are most commonly involved. Herein, the case is a 64 years old male who presented with a round, painless mass in the right temporal fossa with extension to infratemporal fossa. The lesion was surgically excised and histopathology confirmed the diagnosis of cavernous hemangioma. Physical examination showed 4×5 cm round and painless mass situated lateral to orbital wall. His problem was associated with right infraorbital edema. Fine needle aspiration was not diagnostic . MRI shoSurgical excision was performed through coronal incision. The mass was found within the temporal muscle and was excised with loupe magnification to protect right frontal branch of facial nerve. Blood loss during operation was trace. Postoperative recovery was uneventful and functional and cosmetic results were excellent . Gross e6 The masseter (36%), followed by the trapezious (24%) are the muscles most frequently affected, whereas temporalis muscle hemangioma are extremely rare.7Hemangiomas are benign vascular proliferations and one of the commonest benign soft tissue tumors consisting 7% of all soft tissue tumors. They are usually present in infants and children, while females are more affected than males. The etiology is unclear. Intramuscular hemangiomas are rare tumors accounting for 0.8% of all hemangiomas and approximately 14% of cases are localized in the musculature of the head and neck.1 The most common presentation is a smooth, non-tender and mobile mass. The lesion usually does not show any of the vascular signs such as thrill or skin discoloration like superficial hemangioma.8 Plain x-ray films, CT scan, angiography may not be specific for this tumor. CT scan is helpful for evaluation of bone involvement. MRI is the method of choice. On MRI, the tumor is isointense on T1 and hyperintense on T2 weighted sequences.9In 1972, Allen and Enzinger classified intramuscular hemangioma according to the vessel size as (i) capillary type , (ii) large vessel or cavernous type, and (iii) mixed type (consisting of both).10 Preoperative embolization can help to reduce intra-operative bleeding.11 Sclerosing agents, corticosteroids and radiotherapy are another modalities for treatment.12 But, in this case; embolization was not performed due to relatively slow and small amount of blood pooling. Surgical excision is the treatment of choice. Coronal incision is the best way to approach this tumor and the tumor should be excised with loupe magnification to protect frontal branch of facial nerve and securely ligate feeding vessels. Long term clinical and radiological follow up are necessary to detect any relapse.13Treatment options are variable depending on the size, vascularization and location.Hemangiomas are benign vascular tumors and are rarely seen in the temporalis muscle. This case showed a cavernous intramuscular hemangioma involving temporalis muscle in old adult. Surgery is the treatment of choice to exclude malignancy and for adequate treatment of these lesions. Coronal incision is the best way to approach this tumor and the tumor should be excised with loupe magnification to protect frontal branch of facial nerve and securely ligate feeding vessels. Long term clinical and radiological follow up are necessary to detect any relapse.


The use of 5-ALA in WHO grade I lesions is still controversial. A 49-year-old lady was diagnosed in 2004 with a left temporal lobe lesion as an incidental finding; she was followed up clinically and radiologically. In 2016, the lesion showed contrast enhancement and she was offered surgical resection but given she is asymptomatic, she refused. In 2018, the lesion showed signs of transformation with ring contrast enhancement, increased vasogenic oedema and perfusion; the patient accepted surgery at that point. She had preoperative mapping by navigated transcranial magnetic stimulation and she had operative resection with 5-ALA. The tumour was bright fluorescent under Blue 400 filter—Zeiss Pentero 900©(Carl Zeiss Meditec)—and both bright fluorescence and pale fluorescence were resected. Postoperative MRI showed complete resection and histopathology revealed WHO grade I papillary glioneuronal tumour, negative for BRAF V600 mutation. WHO grade I papillary glioneuronal tumour may present as 5-ALA fluorescent lesions. From a clinical perspective, 5-ALA can be used to achieve complete resections in these lesions which, in most cases, can be curative.The online version of this article (10.1007/s00701-020-04223-x) contains supplementary material, which is available to authorized users. In the last decades, clinical and imagiological follow-up of asymptomatic low-grade lesions was common practice. In 2012, Jakola et al.  publisheThe current literature supports the continuous diametric expansion of these tumours  and the This conservative approach is rarely responsible for uncommon histological findings allowing for a better understanding of the natural history of rare lesions. In this report, the authors present a rare case of a clinical and imagiological natural history of a papillary glioneuronal tumour (PGNT) that was treated with a presumptive diagnosis of a transforming low-grade glioma/high-grade glioma.A 49-year-old right-handed bilingual lady  was referred to our neuro-oncology department for follow-up of a lesion located in the posterior aspect of the left temporal lobe . She was incidentally diagnosed in 2004 (from image investigations for headaches) with a left temporal lobe lesion, and it was decided to monitor the lesion with regular clinical and imaging follow-up Fig. a–c. The Two years later, she remained asymptomatic, but imaging of the lesion revealed further signs of transforming lesion with ring contrast enhancement, increased vasogenic oedema and perfusion Fig. m–u. At tPreoperative brain mapping with navigated transcranial magnetic stimulation was performed—one site of speech arrest was found in the posterior frontal gyrus Fig. , video. The patient experienced mild dysphasia postoperatively  that recovered to her baseline after 2 weeks. The postoperative MRI documented completed resection Fig. .Fig. 4PoThe histopathological result revealed an unexpected WHO grade I papillary glioneuronal tumour, negative for BRAF V600 mutation Fig. .Fig. 5HiGiven these diagnoses and the complete resection achieved with the surgical resection, the multidisciplinary team decision was to follow-up this patient with no adjuvant treatment.WHO grade I papillary glioneuronal tumour is an uncommon diagnosis in the adult population. A recent review of the literature documented less than one hundred fifty cases reported in the literature . The hisAs far as the authors are aware, this is the first report of a 5-ALA fluorescent PGNT. Our group has reported other cases as well, of benign lesions that were fluorescent with 5-ALA . Other cWhen diffuse WHO grade II tumours are considered, the rates of 5-ALA-induced fluorescence found in the literature are quite heterogeneous  . The surWHO grade I papillary glioneuronal tumour may present as 5-ALA fluorescent lesions. From a clinical perspective, 5-ALA can be used to achieve complete resections in these lesions which, in most cases, can be curative.ESM 1.Resection of the tumour with 5-ALA. From second 20 onwards, shows fluorescene with Blue 400, showing the evidence for the use of Gliolan in low grade tumours. (MP4 15224 kb)


Undifferentiated abdominal pain is a common presentation often requiring immediate medical or surgical intervention. Providing an accurate diagnosis involves a detailed patient history and thorough physical exam. Point of care ultrasound is gaining acceptance as a rapid diagnostic tool that can be used to accurately detect life-threatening conditions while potentially avoiding unnecessary radiation exposure and facilitating rapid treatment. Detection of pneumoperitoneum with point-of-care ultrasound is a simple procedure that relies heavily on the experience of the investigating practitioner. Standard technique involves placing a high-frequency linear-array transducer in the right upper quadrant, where abdominal free air is most likely to accumulate. Detection of the ‘gut point’, which is the transition of abdominal wall sliding to lack thereof in a single image, is the pathognomonic finding of pneumoperitoneum. If visualization is difficult, moving the patient to the left lateral decubitus position or using the scissors technique can provide additional image views. This representative case report and review highlights the use of abdominal POCUS for the diagnosis of pneumoperitoneum. Ultrasound should continue to be explored by clinicians to narrow the differential diagnosis of acute abdominal pain. Medical diagnosis, and undifferentiated abdominal pain in particular, is a complex, intricate, and multifactorial process that relies on years of experience and training. Physicians are often challenged to analyze a series of complicated, nonspecific symptoms, and expected to make a diagnostic conclusion. Undifferentiated abdominal pain is a symptom that points to a wide array of pathologies, ranging from benign to life-threatening conditions . A recenIn patients with severe, acute abdominal pain, the preferred diagnostic tools are primarily multidetector Computed Tomography (MDCT) and abdominal radiography . HoweverOne potential etiology of undifferentiated abdominal pain is post-operative pneumoperitoneum, an often life-threatening condition that requires immediate surgical care to abrogate its high risk of mortality . This caAn 81-year-old woman presented to the emergency department with hematochezia. Although she had chronic nonspecific abdominal pain, she denied significant pain on presentation. She had no prior gastrointestinal bleeds, NSAID use, vaginal bleeding, or vaginal discharge. Remaining history was noncontributory. Physical examination was significant for diffuse abdominal tenderness and gross blood on digital rectal exam. Vital signs were unremarkable. Her hemoglobin was 9.9 g/dL, decreased relative to a known baseline of 14 g/dL. The patient was admitted to the hospitalist service for further evaluation of lower gastrointestinal bleeding.The initial CT of the abdomen and pelvis only showed colonic diverticulosis without any findings of inflammation or free intraperitoneal air. During the first 24 h after admission, the patient required multiple transfusions of packed red blood cells. The gastroenterology team was consulted and ordered nuclear imaging given the large volume blood loss, which revealed no scintigraphic evidence of active GI bleeding. Esophagogastroduodenoscopy (EGD) was subsequently performed and was grossly unremarkable. A colonoscopy was then performed on hospital day 2, noting numerous diverticula, none of which appeared to be bleeding. The plan at that time was to monitor the patient given that she was hemodynamically stable, and her hemoglobin had stabilized without additional evidence of active bleeding.The following day, the rounding hospitalist noted the patient had become acutely confused and complained of worsening abdominal pain. The hospitalist, who has received extensive POCUS training through completion of a primary care ultrasound fellowship , performOver the next hospital day, the patient had frequent episodes of hypotension and hypothermia and was transferred to the medical ICU, where she was started on vasopressors. She later developed persistent atrial fibrillation with rapid ventricular rate. The palliative care team was consulted, and comfort care was initiated. The patient died on hospital day 6.A tissue–air interface acts as a strong reflector of ultrasound waves. Its strong acoustic reverberations are detected as bright repeating horizontal artifacts. In lung ultrasound, these repeating echogenic artifacts below the pleural line are often termed A-lines –13  linear transducer is preferred. This will produce a shallow, but high-resolution image of anterior abdominal gas pockets while reducing interference of deep intestinal air . Right uFor ultrasound operators familiar with lung ultrasound, a “gut point” may be thought of as analogous to a “lung point” found in pneumothorax . In normThe scissors maneuver can also be used to help detect intraperitoneal free air. It involves lightly placing a linear-array transducer parasagitally in the right epigastric region with the patient lying supine. The transducer should be lightly placed on the abdomen with careful consideration to not compress the skin surface. If reverberation artifacts are seen with a suspicion of abdominal free air, gently press the caudal end of the probe onto the abdomen. This should press the free air away from the anterior liver. The reverberation artifacts that were previously obstructing the liver should now be less prominent. Releasing the pressure from the probe should allow the free gas to return and make the reverberation artifacts more visible .Pneumoperitoneum arises from a variety of causes in both operative and non-operative settings. Exact pathophysiology varies, but generally pneumoperitoneum is caused by a perforated hollow viscus in 85–90% of cases . RecentlMost cases of suspected pneumoperitoneum are evaluated with CT or radiography . CT is tThe major downfall of POCUS is its heavy reliance on the technical skills of the operator. A prospective observational study asked four senior physicians with ultrasound experience and two internal medicine residents with no ultrasound experience to blindly interpret both ultrasound and radiographic images of patients with and without pneumoperitoneum. The study concluded that ultrasound had a higher sensitivity for detecting pneumoperitoneum compared to radiography (95.5% vs. 72.2%), but had a lower specificity (81.8% vs. 92.5%) . The diaMany patients with risk factors for pneumoperitoneum may fall under medical, rather than surgical, care. This highlights the importance of not limiting pneumoperitoneum as a diagnostic consideration to surgical floors and emergency departments. Hospitalists and intensivists should consider pneumoperitoneum in patients presenting with undifferentiated abdominal pain with an additional history of abdominal pathology. To account for its low prevalence, ultrasound can be used as a rapid diagnostic tool to differentiate life-threatening pneumoperitoneum while also preventing unnecessary radiation exposure . Such usUndifferentiated abdominal pain is a common diagnostic dilemma that may not always present with a clear answer. Understanding the basic technique of the abdominal ultrasound exam, along with the ability to differentiate between intestinal and free abdominal air provides clinicians with an additional imaging modality to rapidly detect life-threatening cases of acute pneumoperitoneum. Due to its high sensitivity, low cost, and safety profile, abdominal ultrasound should be considered a first-line imaging modality for diagnosing pneumoperitoneum, as demonstrated in the previously described case presentation. While already widely embraced by emergency medicine, improved ultrasound education in the primary care and surgical specialties will enable the confidence and proficiency to fully realize the scope of ultrasound as a powerful diagnostic tool.Additional file 1: Video S1. The “gut point” is the transition zone between normal bowel artifact, that may normally contain A-lines, and the abnormal A-line pattern without sliding. Similar to pneumothorax, an absence of sliding with the presence of A-lines is a diagnostic indicator of pathologic free air.


Unilateral facial paralysis (FP) in the pediatric population is a rare entity secondary to multiple etiologies including infectious, vascular, and neoplastic. In persistent or recurrent FP, imaging can demonstrate a peripheral facial nerve (FN) lesion. Given the rarity of FN lesions, however, there is limited literature regarding optimal management. In this case series, we describe the presentation, evaluation, and management of unilateral FP in three pediatric patients along with a review of the literature. All patients presented with complete FP due to a peripheral FN lesion or compression of the FN. A combined mastoid and middle cranial fossa approach was utilized for excision in two cases, and the other child underwent a translabyrinthine approach. The pathology of the lesions revealed a meningioma, an arachnoid cyst, and a hemangioma. Presentation, evaluation, post-operative outcomes, as well as final pathologies are discussed. Pediatric facial paralysis (FP) is a rare entity. It affects approximately 5-21/100,000 children per year, with a mean age between five and 11 years and no gender predilection . AlthougIn this paper, we present three pediatric patients with unilateral FP caused by lesions in the IAC who underwent surgery. The first patient is a 12-year-old male with progressive FN palsy, the second is a three-year-old male with recurrent FN palsy, and the third is a five-year-old male with acute-onset FN palsy. The presentation, diagnosis, and medical as well as surgical management of three pediatric patients with unilateral FP are presented in this case series.Case 1A 12-year-old boy presented to the neurologist with frequent migraine headaches and right-sided progressive FN palsy over three years, with complete paralysis occurring in the last year. Family history was significant for neurofibromatosis type 1 (NF1). Right-sided FP was the only significant finding on physical examination, and no cutaneous manifestations  of NF1 were visualized. An MRI revealed a right-sided FN mass involving the geniculate ganglion (GG) and tympanic and labyrinthine segments. The lesion was isointense on T1 and T2 with enhancement on T1 post-contrast  grade I surgical margins. His three-month post-operative visit was notable for subjective improvements in his headaches and dizziness. His post-operative audiogram revealed normal hearing in all the frequencies except for 8,000 Hz, which showed moderate sensorineural hearing loss (SNHL). Subjectively he has no hearing or vestibular concerns. His six-month post-operative MRI does not show recurrence and the one-year post-operative audiogram is stable. Case 2A three-year-old boy presented to his pediatrician with a sudden onset of left facial paresis progressing to FP, over the course of 48 hours. He was diagnosed with Bell’s palsy and started on oral prednisone. Within four months he had complete resolution of the paresis. However, one month later, the left-side palsy recurred. Due to the recurrence an MRI was performed, which demonstrated asymmetric increased contrast enhancement in the left GG. He was treated with steroids and acyclovir with no improvement. He underwent further work-up with genetic testing for NF2 and a repeat MRI. Test was negative for NF2. The follow-up MRI showed increased enhancement of the FN as well as a more notable asymmetric enlargement in the mastoid segment Figure . An FN sAt his three-month follow-up, parents did not report any concerns regarding his hearing. Pathology showed hemangioma of the FN. The post-operative audiogram revealed mild-to-moderate hearing loss at 750-1,000 Hz, otherwise unremarkable. Case 3A five-year-old white male presented to the emergency department with one-week history of headache, dizziness, vomiting, and acute onset of FP. Upon presentation, he denied otalgia, tinnitus, or hearing changes. He also denied recent illness, exposure to sick contacts, out-of-country travel, and exposure to ticks.Examination was normal with the exception of a complete right FP. Initially, a computed tomography (CT) angiogram was negative for stroke, but showed a dilated right IAC. An MRI revealed mild asymmetric enlargement of the right IAC secondary to a small cystic mass measuring 0.7 x 1.0 x 0.6 cm and exerting mass effect over the seventh and eighth cranial nerves Figure . Oral prGiven the location of the lesion and no serviceable hearing, he underwent a translabyrinthine approach for tumor excision at an outside institution, with the goal of FN preservation. Pathological examination of the specimen revealed an arachnoid cyst. He developed a cerebrospinal fluid (CSF) leak on POD 2, for which he underwent post-auricular exploration and plugging of Eustachian tube with fat graft. At his six-month post-operative visit, his FN function had improved to a House Brackman Grade II/VI, from complete paralysis. The follow-up MRI did not show any cyst re-accumulation. Children infrequently present with facial palsy. The most common etiologies are traumatic or an infective process, with tumors accounting for approximately 2%. It is important for these patients to undergo a thorough work-up. This will direct treatment options ranging from antibiotics and steroids for infectious processes, surgical treatment for traumatic or solid malignancies, or even chemotherapy for leukemia patients . Accordingly, the work-up for FP in a pediatric patient should include a detailed history and a physical examination to determine the severity and extent of symptoms. Inquisition into trauma, recurrent ear infections, surgical history, travel, radiation exposure, and family history may help tailor diagnostic work-up. Physical examination of the head and neck, with specific attention to the middle ear, can provide a quick and easily treatable diagnosis with consideration to the high rates of traumatic or infectious causes of FP. Further evaluation looking for cutaneous abnormalities associated with NF1, visual and auditory disturbances, and imbalance issues may lend support to an IAC lesion . In caseIn our first case, the patient was diagnosed with FN meningioma. Primary extradural meningiomas represent 1%-2% of all meningiomas, and those located in the middle ear can cause FP ,8. A majIn the second case, the child was initially thought to have Bell's palsy; however, due to the atypical course, imaging was obtained. Imaging initially showed some enhancement in the location of the GG, which progressed to include the mastoid segment of the FN on subsequent MRI. Given the clinical course and MRI findings, we proceeded with surgery. Using this approach, we were able to carefully remove the diseased FN segment and perform an FN graft. The final pathology revealed hemangioma, which rarely involves the GG of the FN, accounting for 0.7% of intratemporal tumors ,15. The The third child with FN paralysis underwent a translabrynthine approach at an outside institution. This is the best approach to remove the lesion and decompress the FN in the setting of no serviceable hearing. The final pathology returned as arachnoid cyst and the patient’s FN recovered to a House Brackman grade II. Arachnoid cysts account for 0.5% of all tumors found in the IAC . DependiFP is a rare occurrence in the pediatric population and more often warrants inquisition into traumatic or infectious causes though tumors have been recorded to present as FP. In most cases, surgical treatment is not the initial consideration, and children are either observed or managed medically. However, it is important to identify cases that would benefit from surgical resection and understand proper techniques to preserve hearing, balance, and FN function. In cases demonstrating an FN mass or enlargement of the FN canal, rare tumors, such as schwannomas, meningiomas, hemangiomas, and arachnoid cysts, must be considered. Management often requires a multidisciplinary approach with a role for shared decision making between the physicians and the caregivers. Given the potential growth of these lesions, surgical management should be considered with emphasis on preservation of hearing and vestibular function. In select cases, a combined transmastoid and middle cranial fossa approach has led to hearing preservation and the opportunity for FN grafting without complications. These cases highlight the importance of evaluation of neoplastic causes of FP in pediatric patients.