Ovarian cancer is the most fatal gynecologic malignancy. The incidence of ovarian cancer among female-to-male transsexuals receiving treatment with testosterone is unknown, and few cases have been reported in the literature. We report a recent case in our institution, a 23-year-old female-to-male transsexual patient who received testosterone supplementation. The patient underwent a pelvic magnetic resonance imaging to study an ovarian complex cyst that revealed the presence of a bilateral ovarian tumor with imaging features of borderline serous tumor. These masses were surgically removed and the pathology report confirmed the diagnosis associated with noninvasive peritoneal implants and the presence of numerous androgen receptors in the tumor cells. Although there is still insufficient data to validate a direct correlation between hormonotherapy and ovarian cancer in these patients, this case may reinforce previous reports on this association and highlights the relevance of radiological follow-up and bilateral salpingo-oophorectomy as part of gender reassignment surgery. It has been established that the acquisition of secondary sex characteristics of the other gender, based on sex steroids treatment, is fundamental for sex reassignment in transsexuals. However, an unresolved question is whether, in the long term, the administration of cross-sex hormones is safe .Furthermore, it is believed to exist an underreporting of complications of cross-sex hormone therapy. This may occur because although the initial treatment is mainly administrated in specialized centers, complications on the long term are often seen in general practice, and they are then only occasionally reported in the scientific literature .We present a case of a female-to-male (FTM) transgender patient who developed serous borderline ovarian tumor (SBOT) while taking testosterone injections.SBOT is an intermediate grade of neoplasm between benign and malignant serous ovarian tumors . PatientCurrently, there is not enough evidence to support any recommendation to continue or stop the hormonal treatment. However, its duration probably increases the risk of development of hormone-related malignancies, especially in the case of long-term exposure.Despite this, transsexuals are often reluctant in stopping hormone administration for fear that the secondary sex characteristics of the acquired sex will diminish .We report the case of a 23-year-old FTM transgender who had been taking testosterone since 2016 in order to gender reassignment. This patient suffered from asthma, treated with budesonide/formoterol fumarate dihydrate and cetirizine, without any other personal or family medical history. Moreover, this patient had never been pregnant and had not proceeded with any surgery.On the ultrasound routine exam dated of June 2019, a complex cyst lesion was detected in the left ovary, with intracystic solid vegetation, measuring 3.0 cm. The patient was asymptomatic and did not stop the supplementation. Pelvic clinical examination was unremarkable. Blood tests showed high serum levels of CA-125 of 133 U/ml . Thereby, the patient was presented to our department of radiology in order to perform a pelvic magnetic resonance imaging (MRI) study.MRI images were acquired using a 1.5 T MRI machine. Imaging parameters are provided in the figure legends  with hypointense internal branching  and ovarian stroma preservation with a hypointense ovarian capsular margin, on T2-weighted imaging, are features strongly suggestive of this tumor . When thThe final diagnosis was confirmed by the anatomopathological examination. In immunohistochemistry, WT1 is a marker of tissues derived from the middle mesoderm inner layer, combining positivity for estrogen receptors (ER) and progesterone receptors (RP), proving to be a neoplasm derived from the adnexal structures, so excluding the diagnosis of endometrioid carcinoma. High-grade serous carcinoma typically shows a p53 mutation with positivity in 100% of cells, as well as positivity to p16 and cell proliferation index (ki-67) around 100%, which was not observed in this clinical case report, thus reinforcing the diagnosis. Low-grade serous carcinoma can be immunohistochemically identical to the borderline serous tumor, but needs to have invasion criterion, namely, invasive implants. Finally, the tumor reported in this case showed abundant expression of androgen receptors, which also reinforces the diagnosis. It should be highlighted that androgen receptors are present in almost all epithelial ovarian cancers. Their function has been proposed to be associated with a growth stimulatory effect of testosterone and a possibly larger role in tumor progression.From the above mentioned, we may consider a possible relationship between the testosterone supplementation and the development of the ovarian tumor in this patient, like few cases previously reported .Studies have stated that short- and medium-term treatment of testosterone in FTM transsexuals is safe, but long-term safety has been difficult to guarantee .Despite, in the current literature, there are no established special guidelines regarding the follow-up imaging recommendations for these patients neither defined dates for surgical procedures. These patients are, actually, advised to adhere to the routine cancer screening protocols as in nontransgender individuals, depending on the anatomic situation, according to birth-assigned sex .Regarding to the surgical procedures to prevent gynecological cancer, it remains a controversial issue, because the oncological risk in FTM transsexuals, after the initiation of testosterone therapy, is still inconclusive and lacks power . Some auOn the other hand, there is also controversy on maintaining hormonal treatment in these patients. Hage et al. suggested that hormonal therapy should be stopped after the diagnosis of an androgen receptor-positive ovarian cancer . HoweverLastly, it is important to remember that transgender individuals commonly report avoidance of medical care for being afraid of discrimination . On the Also, taking into account all these controversies, by reporting this case, we reinforce the importance of the role of imaging during the follow-up of these patients, during hormonal therapy or while waiting for surgery, allowing the early detection and diagnosis of tumors, whose greater risk cannot be excluded.This case report emphasizes that, despite the few described cases of ovarian tumors in transgender patients under testosterone therapy, we cannot underestimate its occurrence. Actually, this case was only detected by imaging studies, namely, with MRI, thus, reinforcing the importance of the role of imaging in monitoring these patients.


Uterine leiomyomas (LM) are tumors arising from the non-neoplastic proliferation of smooth muscle cells within the myometrium. Like benign tumors, LM are not generally spread through the lymphatic system, and therefore should not be associated with lymphadenopathy. Herein, we present a case of a 60-year-old female who presented to the clinic with postmenopausal bleeding in the setting of sonographically evident uterine LM and abdominal lymphadenopathy. A lymph node biopsy revealed plasma cells and an eosinophilic material presumptively diagnosed as amyloid. She then underwent an abdominal hysterectomy for definitive treatment of LM. Surgical pathology confirmed the clinical diagnosis of uterine and cervical leiomyoma. Current literature suggests that genetic and epigenetic abnormalities contribute to the pathogenesis of LM in addition to hormonal signals such as estrogen and progesterone. It is unusual for LM to occur in post-menopausal women due to reduced hormonal influence. Therefore, this case explored an alternative mechanism of tumor proliferation. This case hypothesizes that genetic mutations and epigenetic changes resulting from chronic inflammatory offenses contributed to LM growth and lymphadenopathy. Uterine leiomyomas (LM), colloquially termed uterine fibroids, are the most common benign gynecologic tumors . LM arisMED12 gene mutations have been implicated in the pathophysiology of multiple tumors affecting both sexes, including prostate cancer, and comprise one of the driving mutations involved in myocyte stem cell differentiation into leiomyoma [p53 expression leading to dysregulation of cell cycle mediators [For example, eiomyoma . Followieiomyoma . Estrogeediators .HMGA2 [In addition to genetic mutations, epigenetic changes such as hypoxia, muscle tension, or chromosomal rearrangements are key in the propagation of large fibroids via enhanced expression of several genes, including HMGA2 . HypometHMGA2 . Lymphadenopathy in the presence of gynecologic complaints is typically associated with malignancy and pelvic inflammatory disease . Rare caA 60-year-old G3P3003 female not taking hormone replacement therapy was referred to the gynecology clinic for iron deficiency anemia suspected to be secondary to postmenopausal uterine bleeding. She entered menopause at age 50 and had not had any uterine bleeding since that time. The patient reported severe bleeding with associated fatigue, presyncope, and anemia requiring blood transfusion. The patient reported this condition had been present for at least six months, over which time her systemic symptoms of fatigue and presyncope had become increasingly severe. At the time of presentation, she had not previously sought treatment. The patient had a past medical history of chronic lymphadenopathy, hypertension, and thyroid disease status post thyroidectomy and an additional past surgical history of tubal ligation. The patient reported that she was previously informed of enlarged abdominal lymph nodes found incidentally on pelvic imaging. To her recollection, there was no intervention or follow-up of that finding. Medications include levothyroxine and losartan-hydrochlorothiazide tablets.Pelvic examination revealed an enlarged uterus consistent with a 20-weeks size. Fibroids and irregular contours were also noted. There were no adnexal masses palpated. No axillary, subclavian or inguinal lymph nodes were palpable on examination. Transvaginal ultrasound relieved numerous masses consistent with fibroids; the endometrial stripe was not well visualized for measurement. Measurements of four fibroids were recorded with the largest being 7.26 cm by 5.80 cm. An endometrial biopsy was attempted; however, pathology showed no endometrial tissue was obtained. The patient was agreeable to definitive surgical management and provided consent for abdominal hysterectomy (TAH) with bilateral salpingo-oophorectomy (BSO).Preoperative abdominal and pelvic imaging revealed new retroperitoneal lymphadenopathy in addition to the existing abdominal lymphadenopathy. These lymph nodes were not palpable on examination. The patient underwent a CT-guided core needle biopsy of a solitary retroperitoneal lymph node Figure . InitialThe patient was taken to the operating room for TAH and BSO by a general obstetrician-gynecologist (OB-GYN). Given the benign appearance of the lymph node specimen, the patient did not provide consent for lymph node dissection. The pelvic cavity was accessed with a midline incision from the pubic symphysis to the umbilicus. Initial inspection of the pelvis showed an enlarged, hyper-vascular uterus with numerous hard submucosal masses Figure . The ovaHistologic examination of the cervix showed a benign tumor Figure . Plasma Two weeks postoperatively, the results of the amyloid confirmatory tests returned. The lymph node specimens were evaluated with Congo red Staining to confirm the diagnosis of amyloid deposits. However, the results were inconclusive due to the limited tissue quantity. Light chain testing revealed a polyclonal B cell population with no atypia or abnormal mitoses. Upon follow-up several days later, the patient returned to the clinic for removal of skin staples and review of pathological findings. One month post-operation the patient’s symptoms had resolved. She was hemodynamically stable and had not required additional blood transfusions. The differential diagnosis of abnormal uterine bleeding (AUB) is broad, comprising structural, and non-structural etiologies. Most causes of AUB in postmenopausal women are in part due to benign changes to the vaginal mucosa and endometrium ,11. HoweThe patient of inquiry in this report underwent appropriate initial management of her presenting symptoms of postmenopausal bleeding, fatigue, anemia requiring transfusion in the setting of a past medical history of chronic lymphadenopathy and hypothyroidism. The ultrasound identified multiple masses throughout the endometrium and cervix. While leiomyomas are the most common benign tumors of the female genital tract, these tend to regress in menopause as the driving ovarian hormones estrogen and progesterone begin to downregulate . HoweverIt is important to note the preoperative differentiation of benign LM from malignant uterine sarcoma is not reliable and care must be taken to ensure a diagnosis of malignancy is not missed especially if there is lymph node involvement . AlthougThe chronic lymphadenopathy in our patient was consistent with polyclonal B cell proliferation, not neoplastic tissue or amyloidosis. However, approximately 100 cases of benign metastasizing leiomyoma with diffuse metastasis to the soft tissue, skeletal muscle, pulmonary, and breast tissue have been identified in the literature ,17. ThesInstead, in the literature, polyclonal B cell lymphadenopathy is most consistent with a benign disease called persistent polyclonal B-cell lymphocytosis, an indolent stable disease characterized by the presence of abundant binucleated lymphocytes in the marginal zone of the lymph node . DefectsA post-menopausal woman with a history of chronic lymphadenopathy, hypothyroidism, and no current or prior use of hormonal replacement therapy, presented for evaluation of severe postmenopausal bleeding leading to iron-deficiency anemia requiring blood transfusion. Further investigation identified multiple large leiomyomas throughout her uterus and cervix. Several enlarged lymph nodes were also identified and sampled for pathology. Since the preoperative probability of malignancy was low due to definitive lymph node diagnosis, this patient was a suitable candidate for total abdominal hysterectomy rather than radical hysterectomy. The patient subsequently underwent a total abdominal hysterectomy, and histologic evaluation revealed no endometrial carcinoma. All tumors identified were benign leiomyomas, and lymph nodes revealed polyclonal B cell expansion. We propose that a similar mechanism involved in the setting of non-hormonal proliferation of uterine smooth muscle through epigenetic modification may relate to the pathogenesis of her concomitant hematological disorder. Although cancer always remains in the differential diagnosis of postmenopausal bleeding, careful consideration of alternative diagnoses makes it possible to select less invasive interventions for low-risk patients.


Intra-cranial schwannomas account for less than 8% of brain tumors, among which more than 80% arise from the vestibular nerve. Intra-cerebellar schwannomas are extremely rare. Several cases have been previously reported but without remarkable degenerative changes on histology.A 61-year-old man presented with worsening disorientation, and an imaging study revealed a cystic lesion (6.5 cm in the largest diameter) in the left hemisphere of the cerebellum accompanied by a mural nodule (2.5 cm) located just inside the skull with enhancement and focal calcification, in addition to hydrocephalus. The lesion was more than 5 mm from the left acoustic nerve. The patient underwent gross total resection. Pathological examination revealed remarkable degenerative changes with various morphological features. Tumor cells were pleomorphic with rich cytoplasm containing numerous eosinophilic granules. Blood vessels and extracellular matrix showed remarkable hyalinization. Immunohistochemical staining revealed that the tumor cells were positive for S-100 protein and negative for Olig2. The tumor was diagnosed as a schwannoma with marked degenerative changes.The present case is discussed with reference to a systematic review of previous reports of intra-cerebellar schwannoma. Intra-cerebellar schwannoma should be included in the differential diagnosis of cystic lesions with heterogeneous histopathological morphology in the cerebellum. Schwannoma, also known as neurilemoma or neurinoma, is a benign nerve sheath tumor arising from differentiated Schwann cells , 2. IntrA few days after amputation of the left lower leg because of arteriosclerosis obliterans, a 61-year-old man presented with worsening disorientation and was referred to our institution. He had been undergoing treatment for hypertension and diabetes mellitus for around a year and undergoing artificial dialysis due to chronic renal failure for 4 years. He had no symptoms and no family history of neurofibromatosis. Neurological examination demonstrated disorientation in date and time, and finger-nose-finger and heel-knee-shin dysmetria on the left side but no hearing disturbance. A computed tomography scan revealed a cystic lesion (6.5 cm in the largest diameter) in the left hemisphere of the cerebellum with a mural nodule 2.5 cm) with enhancement and focal calcification, in addition to hydrocephalus   Fig. 4)4). PubliThe origin of intra-cerebellar schwannomas remains unclear. Some researchers suggest that the origin of intra-cerebellar schwannomas is Schwann cells in the perivascular plexus , and othIn this report, we presented a case of schwannoma arising within cerebellar hemisphere with remarkable degenerative changes. Although it’ rare, schwannoma should be listed in the differential diagnosis of the cystic lesion with heterogeneous histopathological morphology in the cerebellum.


According to international guidelines, recurrent inguinal hernia should be treated by a surgical approach opposing of the primary strategy (anterior–posterior or posterior–anterior). However, recent evidence demonstrates feasibility and safety of re-laparoscopic repair of recurrent inguinal hernia after primary laparoscopy. For such a strategy, correct identification of anatomical structures is challenging, but absolutely crucial for a satisfactory postoperative result. This case of an unrecognized sliding hernia of the sigmoid colon during re-laparoscopy highlights that a precise physical examination as well as an extended preoperative radiological workup  should be considered prior to re-laparoscopy of recurrent inguinal hernia. Nearly 20 million primary inguinal hernia operations are performed globally and annually. A recurrence rates of up to 12% have been reported with <50% being reoperated. Diagnosis of a recurrent hernia can be difficult, especially when clinical symptoms are equivocal. Physical examination combined with sonography represents the standard approach. In unclear cases, magnetic resonance imaging (MRI) or computed tomography (CT) may be performed additionally [The European Hernia Society and HerniaSurge Group guidelines recommend a change of the surgical strategy for reoperations, i.e. the use of posterior repair in case of primary anterior repair and vice versa , 2. HoweHerein, we demonstrate potential pitfalls of re-laparoscopic repair of recurrent inguinal hernia and highlight advantages of an extended preoperative radiological workup.A male patient (51 years) underwent bilateral transabdominal preperitoneal (TAPP) repair for a large left-sided sliding hernia (sigmoid colon) and a small right-sided medial inguinal hernia 7 months ago. A large symptomatic recurrence on the left side was clinically confirmed.Considering the patients’ comorbidities , a re-TAPP was performed to avoid possible wound complications. The recurrent hernia was presumably confirmed as a lateral inguinal hernia. After dissecting dense fibrotic scar tissue around the previous mesh preperitoneally, the hernia sac was released. A large spermatic cord lipoma was found and dissected  and 2. AHowever, the patient experienced persistent swelling in the left groin. To confirm the early re-recurrence hernia, CT of the abdomen revealed a left sliding hernia with sigmoid colon .The patient agreed to proceed with diagnostic laparoscopy for a re-re-repair of the left inguinal hernia. Intraoperatively, a loop of the sigmoid colon, which previously was hidden by colonic adhesions, was fixed into a hernia orifice lateral–caudal of the preperitoneal mesh . After rThe perioperative course was uneventful with discharge at day 3. No recurrence was seen 6 months postoperatively.Surgery of a symptomatic inguinal hernia is one of the most often performed procedures worldwide. The primary laparo-endoscopic approach ) is considered the standard procedure by many and has replaced the primary anterior technique with or without mesh  or Shouldice). Although laparo-endoscopic techniques have decreased the recurrence rates significantly , rates oBefore reoperation of a recurrent inguinal hernia, physical examination and an ultrasound of the inguinal region are recommended. In addition, MRI or CT may be added in unclear cases.There is a paucity of studies investigating the extent of preoperative radiological imaging in cases of recurrent inguinal hernia and data to support an extensive workup are scarce. A recent retrospective analysis showed 97% positive prediction of diagnosing recurrent inguinal hernia by physical examination only, suggesting that an additional ultrasound may not always be necessary . As the The literature to support diagnosis of primary and recurrent inguinal hernia remains equivocal. Low-dose CT of the inguinal region has gained importance. Advantages include evaluation of hernia content, simultaneous investigation of the contralateral side and differentiation of other causes of inguinal swelling. In this case, a preoperative CT would have shown the sliding hernia with sigmoid colon and a sufficient surgical dissection during the first re-laparoscopy may have been performed, omitting re-re-laparoscopy.Surgery for recurrent inguinal hernia can be challenging. An open anterior approach for the treatment of a recurrent inguinal hernia originally repaired by a laparoscopic technique, and vice versa, is recommended by international guidelines . NonetheOur patient suffered from obesity World Health Organization grade II in combination with metabolic comorbidities. Thus, it was reasonable to expect him to benefit from minimal invasive approach. After the dissection of the scar tissue, a large spermatic cord lipoma was detected and mistakenly identified as cause of hernia recurrence. No further dissection of the preperitoneal space was undertaken. This is not in accordance with recently published literature advising a dissection of the preperitoneal layer to at least 2 cm below the pubic symphysis . This reThe re-re-laparoscopic operation confirmed a sliding hernia of the sigmoid. A complete dissection of the preperitoneal tissue was necessary. A new mesh covering the indirect, direct and femoral triangle with overlapping of the pubic symphysis was placed.Alternative may have been a laparoscopic closure of the hernia gap, insertion of a mesh patch and preservation of the former mesh as recently proposed .Although, there is no data available, robotic approach in inguinal hernia surgery may represent a beneficial alternative as it is characterized by enhanced visibility, sole control by surgeon and improved dexterity, which may refine tissue preparation even in technically demanding situations .In summary, we present a case of a missed sliding hernia of the sigmoid during re-laparoscopy of a recurrent inguinal hernia following primary TAPP. We recommend extended radiological imaging including CT or MRI prior to revisional surgery. Re-laparoscopy is feasible and associated with all benefits of minimal invasive surgery. A thorough tissue preparation needs to be applied to recognize important anatomical landmarks. Robotic inguinal hernia surgery may have an important role in revisional surgery.None declared.


Intraventricular schwannomas are rarely encountered in neurosurgical practice. The development and progression of a schwannoma within the ventricular system is still a hypothesised theory. Here, we present a case of a 59-year-old female who presented with a three-week history of headaches. Her symptoms progressively worsened, with resultant altered mental status within the last week. Imaging scans of the brain demonstrated a well-defined mass within the right lateral ventricle with associated midline shift and obstructive hydrocephalus. A parietal craniotomy and resection of the intraventricular mass was performed. Her postoperative course was uneventful. Histopathological assessment depicted a biphasic pattern of Antoni A and B, with a strongly positive S100. This was in keeping with an intraventricular schwannoma. The diagnosis of an intraventricular schwannoma does not fit within the classical differential framework for ventricular masses. These tumours are extremely uncommon but fortunately, they are typically benign. Therefore, adequate surgical resection remains the gold standard of care for these unfamiliar masses. Schwannomas represent approximately 5%-8% of intracranial tumours . These bA 59-year-old female, known hypertensive, presented to the emergency department with a three-week history of progressive generalized weakness and headaches. Within the last week, bowel and bladder incontinence was also noted. Her social history showed recreational use of cigarettes and marijuana. On presentation, she was in an acute confusional state with slurred speech. Her Glasgow coma scale (GCS) was assessed as 14 (fourteen) and there was normal power and tone in all limbs. However, she had appreciable left dysdiadochokinesia and past pointing. Cranial nerve function remained intact.Computed tomography (CT) of the brain with contrast showed a 4.3cm x 4.6cm x 5.2cm well-defined mass arising from the posterior horn of the right lateral ventricle. This was associated with significant midline shift and effacement of the quadrigeminal cistern Figure . MagnetiHigh-dose intravenous corticosteroids were commenced, and tumour resection was scheduled urgently. A parietal craniotomy with trans-cortical access to the lateral ventricle was performed under general anaesthesia. Complete resection of the intraventricular tumour was achieved. The post-surgical course was uneventful, and her GCS returned to 15 (fifteen) on day one following surgery and thereafter. Her Karnofsky Performance Status score is 100% on follow-up. Repeat radiologic imaging demonstrated no gross evidence of residual tumour, with resolving hydrocephalus and midline shift Figure . A CSF dIntraventricular schwannomas are exceedingly rare intracranial tumours . When enApproximately 90% of these nerve sheath tumours occur sporadically, while the remainder is associated with familial tumour syndromes. Neurofibromatosis type 2 (NF 2) and the inactivation of the tumour suppressor gene, merlin, is one of the primary familial syndromes . InteresMacroscopically, these lesions are usually well defined, which can displace surrounding structures without direct invasion. They may also show calcification, cystic degeneration, or haemorrhage. Microscopically, these tumours are described as biphasic, composed of spindle-shaped elongated cells with abundant eosinophilic cytoplasm. Hypercellular (Antoni A) and loose hypocellular (Antoni B) areas are noted ,2. ImmunRadiological imaging usually demonstrates a well-defined lesion, with or without evidence of cystic degeneration and calcification ,9. HowevNeoplasms within the lateral ventricle in the adult neurosurgical patient are typically diagnosed as meningiomas, gliomas, choroid plexus tumours or possible metastasis. Intracerebral schwannomas, especially intraventricular schwannomas, are extremely uncommon. Based simply on radiological assessment, the rate of misdiagnosis will be high. This is case demonstrated the extraordinary diagnosis of an intraventricular schwannoma in the lateral ventricle of a middle-aged female, which will add to the limited published data available. Although this tumour is infrequently encountered in clinical practice, a clear histopathological diagnosis with or without immunohistochemistry is essential. These tumours are usually benign, and a complete surgical resection will result in an overall good prognostic outcome, as in this case.


There is sparse literature about lower ureteric obstruction due to aberrant blood vessels. We report a case of a patient who was referred to our hospital due to left sided flank pain caused by external compression of the distal ureter.A 47-year-old male patient presented with left sided flank pain. A computed tomography scan revealed external compression of the lower ureter. Hypertrophy of the psoas muscle due to extensive cycling for 20 years lead to concomitant kinking and elongation of the iliacal vessels which caused the distal ureteric obstruction. Robotic-assisted laparoscopic ureterocystoneostomy with psoas hitch technique was performed.Lower ureteric obstruction, mostly seen in children, is mostly caused by vascular anomalies such as a persistent umbilical artery. After literature review, we presume it to be the first reported case of distal ureteric obstruction caused by external vascular elongation.The external elongation of pelvic vessels due to excessive cycling and the concomitant extrinsic compression of the distal ureter should be considered as rare but possible cause of lower uretic obstructions. •A 47-year-old cyclist was admitted with left sided flank pain•Excessive cycling lead to elongation of the iliacal vessels and concomitant hydronephrosis•External compression of distal ureter due to pelvic elongation is a rare differential diagnosis of lower ureteric obstruction•Robotic assisted ureterocystoneostomy as a treatment option was performed Ureter stones and malignancies are common reasons for obstruction. Undetected injuries after abdominal surgery as well as traumatic causes are also known etiologies for ureteric stricture. Rare causes are infectious diseases such as tuberculosis or ureteric lymphoma . Lower u2A 47-year-old male patient was referred to our department from the general practitioner with recurrent left sided flank pain for 3 months. He had no urological history and his serum creatinine level was normal. A previous abdominal ultrasound revealed left sided secondary hydronephrosis, kinking of the iliac artery and probably a neoplasm of the ureter. Hence, a contrast-enhanced computed tomography scan was performed, which showed an extrinsic compression of the left ureter at the level of the bifurcation of the common iliac artery . No intrAfterwards the patient underwent retrograde pyelography and primary ureterorenoscopy with double-J stenting, which revealed the ureteric stricture at the level of the bifurcation of the common iliac artery . During Afterwards, the patient was presented to the colleagues from the vascular surgery in our hospital. The patient had no symptoms concerning angiopathy especially no signs of intermittent claudication. He has been a cyclist for over 20 years with about 10,000 driven kilometers per year.In the clinical examination all pulses  were well palpated. After re-evaluation of the computed tomography scan, hypertrophy of the psoas muscle as well as extension of the internal iliac artery (diameter 1.0 × 1.1 cm) could be shown. In men, the average diameter of the iliac artery is 1.2 ± 0.2 cm.In our view, the muscular hypertrophy could be explained by the strong hip flexion due to extensive cycling for several years and the hypertrophy caused the concomitant enlargement and kinking of the iliacal vessels. Especially the artery was made responsible for the lower ureteral obstruction. After interdisciplinary discussion with the vascular surgeons, no vessel reconstruction was recommended. The patient was admitted to robotic-assisted laparoscopic ureterocystoneostomy. The stenotic area, which could be seen directly next to the artery, was resected and the patient underwent ureterocystoneostomy with psoas hitch technique. The histopathological samples showed no malignancy . The pat3The etiologies of ureteric strictures are heterogeneous. Upper ureteric obstruction by aberrant vessels is a well-documented pathology, whereas literature about lower ureteric compression by blood vessels is rare. In 1929, Hyams reported the first time of an extrinsic obstruction of the lower ureter due to an aberrant vessel .Five cases of anomalous arteries leading to ureter compression were presented by Young and Kiser in 1965 . In thesObstruction of the distal ureter occurs more often during childhood. Read and Devine reported of 7 distal ureter obstructions in children up to 6 years of age caused by vascular anomalies . Four ofIn 1992 Grifoni et al. summarized 11 cases of ureteric obstruction caused by anatomical variants of the umbilical artery. According to the authors anomalies of the umbilical artery are rare but possible causes of lower ureteral obstruction .Gupta et al. presented a case of a 32-year-old woman with persistent umbilical artery leading to obstruction of the ureter. Interestingly, the patient was treated with ureterocystoneostomy and resection of the vessel .The latest report to our knowledge is also the first published case, where distal ureteric obstruction was caused by an aberrant gonadal vein and not by an artery. Raghavendran et al. resected the stenotic ureteric segment and performed ureterocystoneostomy. No clinical symptoms were reported in the one-year follow-up .4The external elongation of pelvic vessels due to excessive cycling and the concomitant extrinsic compression of the distal ureter should be considered as rare but possible cause of lower uretic obstructions. Ureterocystoneostomy may be an option for definitive treatment.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.Not commissioned, externally peer-reviewedNot applicableThis study has not received funding from any person or institution.Christian Ramesmayer is the guarantor and accepts full responsibility for the work and/or the conduct of the study, had access to the data, and controlled the decision to publish.Not applicable.Christian Ramesmayer, Michael Mitterberger and Lukas Lusuardi designed and directed the project. Christian Ramesmayer took the lead in writing the manuscript. All authors provided critical feedback and helped shape the research, analysis and manuscript.The authors report no declaration of interest.


Case Presentation. We report a case of accidental ingestion of a razor blade in a chronic alcoholic who presented with hematemesis after an esophageal perforation, the diagnosis of which was confirmed by radiology.  Ingestion of sharp foreign bodies is uncommon and often underreported. It can present with esophageal perforation which is a life-threatening complication requiring prompt diagnosis and management.  Early recognition of esophageal perforation is crucial for early intervention. Proper history taking and radiological investigations are a key to reaching a diagnosis. Ingestion of foreign bodies (FB) is common in children, while in adults it is commonly seen among those with psychiatric disorders, mental retardation, prisoners, and alcoholics . The ingReports of ingestion of sharp objects, more specifically razor blades, are rare in the literature. Herein, we report a case of a chronic alcoholic who accidentally ingested a razor blade and presented with esophageal perforation.A 58-year-old male was referred to our center with a two-day history of acute anterior chest pain radiating to the shoulder, dysphagia, and multiple episodes of hematemesis following probable ingestion of a foreign body under the influence of alcohol. The patient was apparently unaware of the incident and timing of the ingestion. There was no history of fever, loss of consciousness, body discoloration, significant weight loss, dysphagia, and similar events in the past. He had been consuming approximately 1.5 liters of homemade alcohol every day for the last 40 years. There is no history of intake of psychoactive substances and of any documented psychiatric illness.2 of 82% in room air with right intercostal drain and nasogastric tube in situ draining blood mixed contents. On chest auscultation, air entry was reduced bilaterally without any added sound. The abdomen was soft, and examination of other systems was unremarkable.On examination, the patient was ill-looking, alert, and afebrile with a blood pressure of 110/80 mmHg, pulse rate of 100 beats per minute, respiratory rate of 28 per minute, and SpO6-8 level with extensive pneumomediastinum and surgical emphysema in the neck along with bilateral pleural effusions  of the chest and abdomen revealed a 5 cm × 2 cm double-edged shaving razor blade in the midesophagus causing a 45 mm perforation at its lateral aspect at Tffusions .With a diagnosis of razor blade ingestion causing thoracic esophageal perforation, a decision was taken to pursue operative management. The patient underwent a right-sided posterolateral thoracotomy. The blade was removed intact, the pleural cavity was cleared of pus and debris . The medForeign body ingestion is not an uncommon entity. Individuals under the influence of drugs and/or alcohol often present to the emergency after ingesting multiple foreign bodies. The ingestion tends to be spontaneous, and frequently, patients do not remember swallowing the object . Our patOne-third of foreign bodies retained in the gastrointestinal tract are present in the esophagus . The duoThe common presenting complaint in esophageal perforation is pain in the neck, chest, abdomen, or shoulder, depending upon the location of the perforation. Episodes of forceful or repeated vomiting may precede pain. In about 25% of the patients, pain is followed by vomiting and dyspnea. Dyspnea is more common in thoracic esophageal perforations. The presence of the Mackler's triad, a history of forceful emesis, subxiphoid chest pain, and subcutaneous emphysema often suggests acute esophageal rupture . Our patFor those who ingested FB accidentally under the influence of alcohol as in our case, medical evaluation can be difficult as they often cannot provide a reliable history . BecauseChest and abdominal X-rays can elicit a radiopaque FB-like metallic material among suspected esophageal FB . HoweverOf all the FB ingested, about 20% need endoscopic extraction and surgical interventions are required in only less than 1% of presentations while the remaining pass through the gastrointestinal tract uneventfully . HoweverIn addition to the medical and surgical treatment, psychiatric evaluations are necessary in cases of intentional and/or repeat foreign body ingestion . Our patIngestion of sharp objects causing esophageal perforation is a life-threatening condition. Prompt diagnosis is imperative for favorable results.


Nowadays, colorectal cancer (CRC) is the third most frequent cancer, and about a third of patients with CRC presents themselves with symptoms of large bowel obstruction. Historically, surgical resection was the treatment of choice for colonic obstruction, but this kind of approach is burdened by a high risk of postoperative morbidity and mortality. In recent times, the use of a colonic stent has been proposed to overcome the obstruction and transform an emergency surgical case into an elective one to avoid emergency surgery complications. Endoscopic stenting is the first-line treatment option in the palliative management of colonic obstruction, and there is sufficient scientific evidence to support this approach. However, endoscopic stent used as a bridge to surgery is not yet widely adopted because the concern was raised about the long-term survival and cancer safety of this approach. The recent scientific evidence has shown that this approach improves the short-term outcomes, such as postoperative complications and the stoma rate, without differences in long-term outcomes compared to emergency surgery. Therefore, the European Society for Gastrointestinal Endoscopy in 2020 has reconsidered stenting as a bridge to surgery as a valid alternative to emergency surgery. Globally, colorectal cancer (CRC) is the third most common cancer in males and the second in females, as reported by Globocan in 2018. The incidence decreased in patients older than 50 years, whereas it increased in patients younger than 50. Males are affected more often than females by about 25% . AccordiScreening programs for CRC indeed reduced the incidence of advanced diseases. Nevertheless, about 30% of patients affected by colorectal cancer report symptoms of large bowel obstruction. This is notably reported when the lesion is located at the upper rectum or distal colon. This is supposed to be related to the smaller size of the lumen and the increased consistency of the stools . In thisThe present review aims to assess the role of colonic stenting in the emergency setting according to the latest evidence of the literature and international guidelines.Patients with bowel obstruction can present with acute abdominal pain, typically cramping with paroxystic episodes every 20–30 min, bloating, obstipation, or chronic and progressive change in bowel habits until failure of passage of the flatus and stools. Nausea and vomiting are more commonly associated with proximal bowel obstruction, provided that the ileocecal valve is incompetent. Not rarely, the competence of the ileocecal valve prevents distension of the ileal tract if not in the late stages. Acute pain in the pelvis with tenesmus is a typical sign of rectal obstruction. Focal pain is usually due to necrosis or ischemia that determine peritoneal irritation. This is generally located in the lower right quadrant and corresponds to the significant dilation of the caecum, particularly in cases of the competent ileocecal valve. In case of considerable distension, the caecum, having the thinner wall of the entire large bowel, is subject to diastasis of the muscular fibers, preluding to perforation. An abdominal computer tomography (CT) scan with i.v. contrast completes the patient assessment. This aims to achieve a diagnosis, and in case a neoplasm is assessed, to stage it in order to provide sufficient information for the therapeutic algorithm. In this case, the CT scan should be extended to the examen of the thorax. CT scan with contrast is the standard to confirm the diagnosis because of the high sensitivity and specificity in detecting colorectal cancer . MoreoveDespite the high sensitivity, it may be argued that a CT scan does not offer high specificity as several misdiagnoses are reported. These entail as high as 15% of the cases, being in about half of the cases diverticular pseudotumors. While no-one would object to a surgical indication in these cases, different is the case of misdiagnoses consisting of Clostridium Difficilis colitis, ischemic colitis, or stool impaction as recently reported. Therefore, it has been questioned if, in all cases, a flexible endoscopy exam should follow a CT scan for confirmation of the diagnosis. However, an agreement on this has not reached.First of all, it is necessary to assess if the intent is curative or palliative. According to European Society for Medical Oncology (ESMO) guidelines 2020, it is essential to distinguish among patients with localized colon cancer, patients with oligometastatic disease, and diffuse metastatic disease. For localized colon cancer, TNM staging remains the most relevant criteria for risk assessment. The oligometastatic disease is characterized by metastasis at up to three different sites, with five or more lesions . Liver metastases could be resected at once or at a second stage, if less than five. An R0 resection should be performed in these cases, provided that more than 30% of liver is left and there is no other concomitant extrahepatic unresectable disease. According to this, any patient with limited liver and/or lung metastases should be considered for potential secondary resection with curative intent. For a patient with diffuse metastatic disease or unresectable disease, the best supportive care, such as palliation, is indicated.Curative intent can be pursued either by stent bridge-to-surgery (SBTS) strategy followed by elective surgical resection in one stage or two stages, just as palliative intent may be offered more likely with stent placement, if feasible, this way offering the chance of a quick onset of oncologic therapies ,11. In bAvailable stents are fully covered, partially covered, and uncovered. The first ones hypothetically reduce tumor ingrowth, but they do not have an excellent grip on the intestinal wall, so they are more at risk of migration. A higher grip is offered by uncovered stents, although they are subjected to tumor ingrowth and, therefore, stent occlusion . MoreoveOther stent characteristics are still the object of debate, although there is very limited evidence for a recommendation. Regarding stent diameter, smaller caliber stents have shown less mechanical stress but more risk of migration ,18. It iAlthough not a standard one, even endoscopic stent positioning requires some bowel preparation. This consists of one or more enemas, which are sufficient to prepare the bowel distal to the obstruction and facilitate the procedure . The belAntibiotic prophylaxis is generally unnecessary, although in selected cases, this is suggested . In factThe severity’s grade of the obstruction might be challenging to assess. Several studies tried to determine it but reported inconsistent outcomes ,23. ThisInstead, it is a good practice to rinse the stenosis with hydrosoluble contrast enema at the end of the positioning maneuver to detect under fluoroscopy both the patency of the lumen after stenting and the absence of leaks. In any case, it is recommended to not perform stricture dilation due to the increased risk of colonic perforation ,25. Stents in the colon and rectal tracts can be positioned using either a through the scope or an over the wire technique, although the first one is much more in use as relatively easier. In fact, when the stent is advanced though the scope, this is pushed through the working channel, which makes the handling of the device easier to direct through the stenosis. In both cases, the stent advances over a stiff guidewire with a technical and clinical success rate of 83–100% and 77–100%, respectively ,29,30,31In the past, the use of stents in colorectal occlusions had been pointed out as responsible for a worse survival, both overall and disease-free . This wap = 0.03). Differently, considering those studies in which the occurrence of perforation was >8%, the 3-years survival did not differ compared to ES  [Today, the only real contraindication for stenting is the risk of perforation. In a critical appraisal of oncological safety of SBTS in left-sided colon cancer obstruction, Amelung et al. observed that in those studies in which the occurrence of perforation was <8%, the 3-years survival was significantly lower compared to emergency surgery (ES)  .This obviously influences the indication to place a stent in the colon, reserved for obstructed patients. ESGE recommends the placement of a stent only in patients with clinical symptoms and radiological signs of malignant large bowel obstruction, whilst prophylactic treatment is not recommended. At the same time, not all the locations of obstruction may be easy to stent. While some sites are relatively easy to reach and stent, some others are challenging. In general, all the junctions between straight tracts of the large bowel may be challenging to approach, particularly in indeed obstructed patients, due to the distention above, which might increase the angle of the lumen bend. This is particularly true at the splenic flexure, which is per se the most angulated tract of the large bowel. Here, it is reported the higher risk of perforation any time the stent has to cross the flexure. Despite the excellent flexibility of the new Nitinol materials, provided they have sufficient expanding force, the stent, which is continuously an object of a straightening force, tend to erode either at the proximal or distal edge of the stent cylinder. Moreover, despite using the most atraumatic hydrophilic guidewires and cannulas, the passage through real tight stenosis located at a natural flexure may put the delivery mechanism with its anvil at increased risk lumen perforation.A further but relative contraindication is represented by the short distance from the anal verge. Stent placement within 5 cm of the anal verge is usually avoided because of the possibility of severe pain, tenesmus, and rectal bleeding. However, some patients who wish to prevent an ostomy can undergo stent placement very low in the rectum with good tolerance. In these cases, always with palliative intent, the maneuver may be attempted as it would be easily reversible in case of intolerance.The worst complication it can occur while stenting an obstructed colon is perforation. A systematic review shows a median perforation rate of 4.5% . To reduMigration happens in approximately 10% of stent positioning maneuvers , althougRe-obstruction happens almost exclusively in the palliative setting. It helps position the stent exceeding 2 cm from the tumor proximal and distal margin to reduce this risk. Two different strategies are possible in the emergency setting to manage malignant colonic obstruction if a curative intent might be pursued. The first is represented by ES, including various options, from the simple diverting stoma to the subtotal colectomy, including Hartmann procedure (HP) and intraoperative wash-out with primary anastomosis with or without diverting stoma. Some of them require a second treatment to close a stoma or resect the primary tumor if a simple stoma had been performed initially. In a few cases, even a third surgical procedure is required. It is difficult to restrict the options available in the emergency setting and design a prospective randomized trial that would be very much awaited to clarify the surgical outcomes of the different procedures and the functional outcomes, less critical but still absolutely relevant in this context. Indeed, the patient’s age, his/her willing, and his/her life expectations are of paramount importance to balance the benefits of the different options. In this scenario, the possibility to return to an elective treatment condition would play a significant role. This is now again offered by the chance of stenting malignancies resolving the obstruction, delaying the treatment to election. It is, therefore, quite challenging to drive the indisputable and incontrovertible conclusion. In recent times, the results of two randomized controlled trial studies comparing SBTS versus ES became available: ESCO study  and CRESIn a meta-analysis of RCT’s of our conception , SBTS seNevertheless, at the time of the embargo for colonic stents, particularly in the Netherlands, the policy of performing a simple diverting stoma in an emergency became popular. This had the advantage to delay the proper colorectal resection to a later time in the hands of a more expert and dedicated colorectal surgeon. So far, only a few studies have compared SBTS with diverting stoma , and none of them are randomized. Amelung et al.  show eviSensitivity analysis has shown that experience, techniques, and case volume might influence long-term outcomes and treatment decisions . ESGE guPalliative stenting for malignant colonic obstruction has been proven effective and safe as a first-line treatment when not contraindicated. In this setting emerged that stenting, compared with ES, results in shorter hospitalization, lower intensive care unit admission rate, shorter time to start chemotherapy, significant lower incidence of stoma formation, resulting in an increased quality of life, even if the clinical relief of the obstruction is greater in the patients treated by emergency surgery ,44,45,46A systematic review published by Amelung et al.  comparedA more recent systematic review published by Boeding et al. , publishColorectal stenting, with endoscopic deployment, is a relatively new technology that might be useful in an emergency, such as colonic obstruction caused by primary colorectal cancer. It can quickly resolve the occlusion by allowing the passage of stools and gases, improving the clinical condition of the patient. Therefore, stenting may be used as an alternative to emergency surgery in the palliative setting. At the same time, in the curative setting, it can be used in a sequential bridge to surgery policy. While there have been concerns about long-term oncological outcomes of SBTS, the more recent scientific evidence suggests that this kind of approach is comparable with ES in terms of oncological results.


This case report explores the interesting case of a 71-year-old gentleman who presented with a testicular lump following trauma. Ultrasound imaging of the testicle demonstrated malignancy and subsequently orchidectomy was listed. Due to a scheduling difficulty, this was prioritized ahead of his whole-body computed tomography scan. Intraoperatively, he developed electrocardiogram changes suggestive of a non-ST elevated myocardial infarction. Post-operative imaging demonstrated a diffuse large B-cell lymphoma encroaching the heart and greater vessels. This case report highlights the importance of preoperative imaging, even where it may prove challenging. We assess the adequacy of current guidelines within the UK on imaging for new testicular malignancies. At present, guidance for imaging in patients with a new diagnosis of testicular malignancy on ultrasound imaging do not uniformly outline the chronology of further imaging and orchidectomy. Typically, a full-body computed tomography (CT) scan is sought prior to operation to explore the extent of further disease, particularly because post-operative survival rates are much lower in the presence of metastasis , and pos).Current guidelines outline conflicting orders for investigating new malignancy .In this case, scheduling challenges in the weeks pre-COVID meant that the operation went ahead prior to further imaging. Here, we explore details of the case and the consequences of this decision.). Tumour marker results demonstrated a raised lactate dehydrogenase at 426 but normal beta-human chorionic gonadotrophin and alpha-fetoprotein levels. His liver function tests revealed raised aspartate transaminase (62) and alkaline phosphatase (252) but normal bilirubin levels. The impression was a testicular lymphoma.A 71-year-old man presented to the emergency department 3 days after falling from a ladder, having noticed a hard lump on his testicles. His past medical history included: osteoarthritis, hypercholesterolaemia and ‘prediabetes’; he had a family history of ischaemic heart disease. Examination revealed firm non-tender lumps on the right testicle, with no signs of testicular rupture. A subsequent ultrasound scan demonstrated ‘multiple hypoechoic lesions in the right testis (up to 3 cm) suspicious of malignancy’ . Tumour He was discussed at the multidisciplinary team (MDT) meeting, and due to scheduling clashes over the Christmas period, his operation was booked prior to CT staging. The following week, he underwent a right orchidectomy via inguinal approach as a day case and follow-up was planned with histology results and oncology MDT outcomes.).Intraoperatively, he developed new t-wave inversion on lead II of the 3-lead electrocardiogram (ECG). This resolved post-operatively, and he denied chest pain. He was kept in overnight for observation. The next morning, during the round, he reported central crushing chest pain the previous day that he had not disclosed. His ECG was repeated and demonstrated the t-wave inversion in lead II—indicating dynamic ECG changes. A troponin I level measured 27. As such, management for a non-ST elevation myocardial infarction was initiated and a cardiology consult organized, who advised continued acute coronary syndrome (ACS) treatment and an echocardiogram. This demonstrated a ‘pericardial mass  encroaching and extending outwards on both sides of the right atrioventricular groove’. ACS protocol was stopped and a cardiac magnetic resonance imaging (MRI) was organized  and 3.3This demonstrated a bulky mediastinal tissue (measuring 50% of the heart) that encased all the aorta and pulmonary artery as well as the coronary artery. It has malignant behaviour with multiple foci that breached fascial planes. It restricted the long axis of the heart but was not causing haemodynamic occlusion. It was non-resectable.The differential for this lesion included: primary tumour , metastatic tumour or (rarely) histiocytosis-type disease (Ehrdheim Chester/Rosai Dorfman). The working diagnosis remained lymphoma.Further staging imaging was recommended by the radiology team. A CT head scan demonstrated no abnormalities, whereas CT chest–abdomen–pelvis (CAP) found significantly enlarged necrotic mediastinal lymph nodes with invasion into the pericardium and heart; bilateral adrenal nodules (suspicious for metastases) and appearances consistent with a lymphoproliferative disorder. The testicular biopsy found large lymphocytes with abundant cytoplasm and prominent nucleoli consistent with a diffuse large B-cell lymphoma (DLBCL). A positron emission tomography (PET) scan was organized at the request of the oncology team  and 5.He was subsequently diagnosed with stage IV (Lugano) DLBCL and underwent the R-CHOP chemotherapy regimen. He has responded well to his oncological treatment.In men who present with a suspicious testicular lesion, it is important to perform a orchidectomy for a histological diagnosis . PrimaryThe urology team considered whether preoperative imaging and tissue biopsy would have altered this patient’s outcome; however, orchidectomy is the first-line management of patients with DLBCL presenting with primary or secondary testicular lesions . TherefoThis case demonstrates that, considering the increased incidence of DCBCL in patients over the age of 60, there can be significant clinical value in performing a CT scan before orchidectomy—in keeping with European guidance . This is


Paranasal sinus mucocoeles commonly involve the frontoethmoidal sinuses and can rarely present with vision changes due to expansion and invasion through the orbit. A 50-year-old female presented out of hours with an acute, 16-h history of complete left sided visual loss, on a background of 3 days of visual changes. A left ethmoid mucocoele extending into the left orbital apex causing compression of the optic nerve was diagnosed on imaging. Emergency endoscopic sinus surgery with decompression and marsupialization of the ethmoid mucocoele was performed, which resulted in improvement of vision. This case is unusual due to improvement in vision despite the length of visual loss prior to surgical intervention. This case demonstrates the importance of considering rhinological causes for vision loss, and how critical early identification and surgical intervention can be to prevent serious complications such as permanent vision loss. The man4 h or pe2Staphylococcus aureus and directed an antibiotic switch to a 10-day course of intravenous Flucloxacillin.An otherwise well and fully independent 50-year-old Caucasian female, who reported previously normal visual acuity , presented with a 16-h history of complete left sided visual loss on a background of blurry vision for the preceding 3 days. When her left eye became blurry, she sought an opinion from her local Ophthalmology department, who could not find a cause. She denied any sinonasal or other symptoms. On examination, she had full, painless eye movements, no proptosis and aside from the visual disturbance in the left eye, there was only a small bruise at the left medial canthus and some mild puffiness of the medial aspect of the left upper eyelid. Flexible nasendoscopy revealed a large septal perforation and bulging left ethmoid bulla without evidence of nasal polyps or discharge. Urgent CT imaging showed a large left ethmoid mucocoele with associated pressure erosion of the medial bony wall of the left optic canal and compromise of the left optic nerve  a and b.As immediate pre-operative formal ophthalmology assessment was not available out of hours, the first formal visual assessment was day 1 post-operatively where the visual acuity of her left eye was 6/60 and subjectively the patient self-reported a 50–60% improvement in her vision, particularly in her peripheral vision. Day 3 post-operatively, she underwent repeat CT imaging. This showed a small bony fragment and some residual ethmoidal disease, which required revision FESS and orbital decompression jointly with the specialist Rhinologist. This resulted in further improvement of the patient's visual acuity to 6/36 by the next day.3Four months later she was discharged from ophthalmology follow-up with a stable visual acuity of 6/36 in the left eye. 6/36 means that she can see an object that is 6 feet away clearly, whereas somebody with normal vision could clearly see this object from 36 feet away. As the vision in her right eye was normal, this caused her to have distorted depth perception, which she was advised would further improve over the next 3–4 months as she adapted.4Ethmoid sinuses are paired sinuses that vary in size and number. They lie between the nasal septum and orbit divided by the thin lamina papyracea. Permanent obstruction of a sinus ostium leads to the trapping and accumulation of sinus secretions . A mucoc5-Always consider rhinological causes for unilateral acute vision loss.-Emergency flexible nasendoscopy and imaging is required to rule out sinonasal pathology. CT provides anatomical detail and bony delineation to plan surgery, but MRI is superior for demonstrating soft tissue relationships and differentials.-Emergency surgical intervention should be considered as soon as possible in Compressive Optic Neuropathy, even if visual loss has been sustained for longer than 2 h, to prevent further visual compromise and spreading infection.-The endoscopic approach is preferred to the open approach as there is less risk of mucosal injury.-In emergency cases, have frank conversations with patients regarding the chances of surgical success and potential complications.-In emergency cases, do not wait for formal Ophthalmology assessment if it is not immediately available, but we suggest performing a non-specialist bedside clinical assessment of vision, e.g. Using a Snellen chart, testing visual fields, examining the fundi with an Ophthalmoscope, assessing colour vision and for relative afferent pupillary defect. Equipment needed to make these assessments should be available in most A&E departments .6A discussion with the patient after discharge revealed that for the preceding couple of years she suffered with discomfort in her head when running on hard surfaces. Fortunately, she has noticed that since the surgical intervention this has never happened again. She was appreciative of receiving prompt out of hours, emergency intervention and subjectively reported some gradual improvement in her vision in regard to depth perception post-discharge over time.There were no conflicts of interest during the writing or submission of this article.No funding was required.Ethical approval was not sought for this case report as it was not required due to the nature of the case report.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.All authors were involved in the care of this patient and made substantial contributions. Additionally, Amit Raithatha was involved in the writing of the draft manuscript, which was reviewed and edited by Paula Coyle and Rishi Talwar.Not applicable.Amit Raithatha.Not commissioned, externally peer-reviewed.


Cerebral fat embolism (CFE) is a subtype of fat embolism syndrome which tends to cause ischemic cerebral infarction. Fat embolism in the cerebral venous system have not been reported. We hereby present a rare case of fat embolus formed in the cerebral venous system 10 days after cosmetic surgery, and describe our management of this patient.A 26-year-old woman with the disturbance of consciousness and recurrent convulsions of the right upper extremity over a 21-h period was admitted to our hospital. The patient was initially diagnosed with haemorrhagic infarction, and cerebral venous thrombosis (CVT) was suspected based on computed tomography (CT). A diagnosis of CFE was confirmed based on surgical findings. Breast and hip augmentation performed 10 days ago was considered the underlying cause. Drug-induced hypothermia, low molecular weight heparin, atorvastatin, dexamethasone, piperacillin/tazobactam, valproic acid, and mannitol were applied. On hospital day 30, she was discharged with a Montreal Cognitive Assessment score of 25.Fat embolism can occur in the cerebral venous system, and may mimic CVT symptoms rather than CFE symptoms. Early identification of the nature of the embolus is essential. The use of heparin may prevent secondary thrombus formation, and accelerate fat embolus decomposition. Cerebral fat embolism (CFE) is a rare manifestation of fat embolism, with an incidence rate of 0.9–2.2% and a mortality rate of up to 10% . Long boTypical fat graft procedures include three main stages: fat harvesting, fat processing, and fat injection. Autologous fat is usually harvested through techniques such as vacuum aspiration, syringe aspiration, and surgical excision . The abdLipid droplets with diameters < 20 μm can pass through the pulmonary capillaries and embolize in the cerebral arteries over a 24–72-h period. Circulating particles may lead to a classic triad of respiratory disorders, rashes, and changes in mental status –14. The Over the past decades, the diagnosis and treatment of CFE have been recognized as a clinical challenge , 13. AvaWe hereby report a rare case of CFE in the cerebral venous system in a 26-year-old woman, in whom pharmacological hypothermia was applied. Our paper aims to highlight 1) the uncertain mechanisms to which the fat embolus led to cerebral venous thrombosis (CVT) symptoms, and 2) the potential role of heparin in CFE treatment.A 26-year-old woman with the disturbance of consciousness and recurrent convulsions of the right upper extremity over a 21-h period was admitted to our hospital. The patient interview revealed a history of breast and hip augmentation 10 days ago. On physical examination, she was confused and uncooperative, but showed no signs of rash or dyspnoea. Her Glasgow Coma Scale (GCS) score was E1V1M3, while her vital signs and routine blood test results were normal. No pathological abnormalities were observed on chest computed tomography (CT), transthoracic echocardiography, or transoesophageal echocardiogram. No pulmonary arterial hypertension was demonstrated on lung ultrasonography. A low-density circular structure surrounding an irregular light zone was identified in the left parietal lobe on CT. A haemorrhagic lesion was also detected in the right frontal tip  was administered intravenously after dissolution in 50 mL normal saline. The flow rate was set at 8 mL/h, and was adjusted every hour according to the measured core temperature. The cooling blanket was removed on HD 8, and the lytic cocktail was gradually reduced at a rate of 0.5 °C /h during the rewarming process. On HD 2, after careful evaluation of the haemorrhage risk, subcutaneous low molecular weight heparin (LMWH) was initiated at a daily dose of 6150 AXaIU. Her serum triglyceride levels were well controlled (0.88 mmol/L) during the administration. The drug was discontinued on HD 8, but her cholesterol levels rose to 6.96 mmol/L 3 days later. Atorvastatin was administered on HD 13 to maintain an average serum lipid content. Other symptomatic treatments, including dexamethasone, piperacillin/tazobactam, valproic acid, and mannitol, were administered under regular guidance.The midline returned to normal after the surgery, and only slight improvement in neurological function was observed (GCS score: E1V1M2). Brain CT on HD 4 revealed mild recovery of the infarcted area Fig. c. MagnetCFE following cosmetic surgery is usually induced by facial fat grafting , 17. FolA fat embolus was observed in the superficial vein during surgery. Magnetic resonance venography performed on HD 4 revealed stenosis in the superior sagittal, left transverse, and sigmoid sinuses. At these locations, venous drainage was blocked by the embolus, leading to haemorrhagic infarction. Based on the nature of the embolus, we speculated that the fat graft surgery performed 10 days prior was the underlying cause. Nevertheless, we found it challenging to explain not only the formation of the embolus, but also the absence of CFE symptoms such as dyspnoea [The retrograding pathway was considered the most promising mechanism. To examine this possibility, the injection pressure exerted during the cosmetic surgery would be needed. However, there are no published studies on the appropriate pressure needed for fat reinjection in humans. Since hyaluronic acid can serve as a substitute for autologous fat in clinical settings due to their similar physical properties, an estimation based on the relevant data of hyaluronic acid was made . In a reWe also considered the conventional venous pathway, whereby fat emboli pass through the capillary networks twice — once in the lungs and once in the brain. However, the abundant lipids (100–200 mL) used for fat grafting should have provoked dyspnoea when passing through the pulmonary capillaries, but this was absent during physical examination. Furthermore, no pulmonary arterial hypertension was revealed on lung ultrasonography. In addition, the embolus is less likely to have passed through the cerebral capillaries to subsequently embolize in the cerebral venous system.The crossed pathway was also discussed and later rejected. Provided this assumption holds, it does not explain how the fat embolus escaped the cerebral capillary sieve. In addition, pathological structures such as patent foramen ovale and signs of left-to-right shunt should have been identified on either transthoracic or transoesophageal echocardiography, yet these were not detected in our case.The three aforementioned pathways are predominantly based on mechanical theories. Another possible hypothesis conforms to the biochemical theory , which p(Table 1) [CFE has been recognized as a diagnostic challenge over the past few years , 11. A mTable 1) , 24. GurTable 1) , 26. ObjTable 1) . SchonfeTable 1) , wherebyTable 1) . Among tIn clinical practice, CFE diagnosis is mainly based on radiological examination. Due to the poor ability of CT in distinguishing soft tissue, it is seldom used to diagnose CFE , 11. LunTo summarize, there are currently no diagnostic criteria with satisfying sensitivities or specificities. The lack of specific symptoms renders it difficult to diagnose CFE. MRI can merely reveal non-specific findings, and brain CT can only reveal haemorrhagic infarction, which is also non-specific. While CFE was initially suspected, typical signs such as rash or dyspnoea were absent in our patient. The negative lung ultrasonography findings further contributed to the inaccurate initial diagnosis. It was surgical findings that eventually led to our diagnosis of CFE.CFE is often self-limiting, but can also be fatal if left untreated . SimilarDexamethasone, LMWH, mild hypothermal therapy, mannitol, valproic acid, atorvastatin, and piperacillin/tazobactam were employed in our case Fig. . DexametThe management of this case had several limitations. From an ex post perspective, MRI should have been performed during the acute phase . This waThis rare case of cerebral venous fat embolism adds to the current understanding of CFE and CVT. To the best of our knowledge, this is the first report of fat embolism in the cerebral venous system. While the retrograde pathway was proposed as the underlying cause, further explorations are warranted. Dexamethasone, LMWH, mild hypothermal therapy, mannitol, valproic acid, atorvastatin, and piperacillin/tazobactam were administered, and resulted in a positive outcome. Heparin was tentatively used in our case, and subsequent lysis of the fat embolus was confirmed on magnetic resonance venography. We therefore speculate that heparin may prevent secondary thrombus formation as well as DIC, and accelerate fat embolus decomposition.


Sinonasal mucosa is an area of high melanocyte density compared to other mucosa-lined sites. Sinonasal mucosal melanomas (SNMM) most commonly arise from the nasal cavity and the paranasal sinuses. Due to their obscure anatomic location and lack of early symptomatology, SNMM are often diagnosed in an advanced stage. The majority of patients who present with symptoms complain of unilateral nasal dysfunction, such as obstruction and epistaxis.We hereby report a case of an 86-year-old female, who presented with a three-year history of progressive right-sided nasal obstruction and recurrent epistaxis. Posterior rhinoscopy and endoscopy revealed a polypoid, fleshy lesion whose coloration varied from mildly pigmented to amelanotic. Inverted sinonasal papilloma was included in the differential diagnosis due to MRI findings. Post-resection histopathology indicated a mucosal melanoma. Typically, amelanotic lesions are rare, more difficult to diagnose and associated with worse prognosis due to both their aggressiveness and delayed diagnosis. Mucosal melanomas (MM) are rare malignancies that account for 1-4% of all melanomas and present a female predilection (1.2 vs 1 per million). The sinonasal mucosa is an area of high melanocyte density compared to other mucosal surfaces and sinonasal mucosal melanomas (SNMM) most commonly arise in the nasal cavity rather than the paranasal sinuses [We report the case of an 86-year-old patient with septal MM presenting with chronic unilateral nasal dysfunction. An 86-year-old female presented to the ENT Department due to a three-year history of progressively worsening right-sided nasal obstruction and recurrent episodes of epistaxis. Anterior rhinoscopy and fiberoptic nasoendoscopy revealed a large mass with macroscopically heterogenous pigmentation, ranging from completely amelanotic to mildly pigmented areas. The mass was totally obstructing the right nasal cavity and extended to the nasopharynx. Lymphadenopathy was not detected. The patient had been receiving medical therapy for hypertension and osteoporosis and had no history of smoking, alcohol abuse or exposure to chemicals, such as formaldehyde.Computed tomography (CT) of the facial sinuses demonstrated a large mass of the right nasal cavity, measuring 4.5 x 2.5 x 1.8 cm along its largest axis, originating from the nasal septum and totally obstructing the right nasal cavity. The mass expanded in the nasal cavity and caused bone erosion to the right inferior turbinates Figure . FindingAn extended biopsy was performed along with functional endoscopic sinus surgery (FESS) to restore sinus ventilation and normal function. Microscopy revealed a malignant neoplasm that exhibited immunohistochemical positivity against SOX10, Melan-A, HMB45, MiTF and CD117/C-KIT, whereas the neoplastic cells were negative for CKAE1/AE3. Both histologic and immunohistochemical findings were consistent with a malignant mucosal melanoma  according to the American Joint Committee on Cancer (8th edition) staging system for MM [N0M0 accoMucosal melanomas are rare malignancies that constitute less than 4% of all head and neck cancers, with a higher incidence in women, usually between 60 and 80 years of age . The sinonasal mucosa is characterized by high melanocyte density and is the most common location for exposure to carcinogens like formaldehyde which was for decades considered to be a risk factor for MM development. However, many studies have failed to confirm this hypothesis . SunlighThe majority of sinonasal mucosal melanomas (SNMM) arise from the nasal cavity (80% of cases) while the remainder originates from the paranasal sinuses (20% of cases). Malignancy usually derives from the lateral nasal wall, followed by the nasal septum, maxillary sinus, and ethmoid sinus. Except for the typical complaint of unilateral nasal dysfunction, facial pain, V2 hypoesthesia and ophthalmologic signs  may rarely be observed and are associated with advanced-stage disease .In the present case, the color of the MM varied from minimally pigmented to amelanotic. Interestingly, amelanotic lesions, in spite of their rarity, are linked to poor prognosis, due to their aggressive behavior, as well as their typically impeded identification and management. Previous cases of masses with different degrees of pigmentation within the same mass as well as satellite lesions surrounding the main tumor have been reported .MRI with contrast agent is regarded as the gold standard for diagnosing unilateral sinonasal pathology as it is capable of distinguishing solid lesions from inflammation. The hallmarks of radiological findings differentiating malignant sinonasal tumors from SNIP are bone erosion on CT, involvement of adjacent structures, and intermediate signal intensity on T2-weighted MRI sequences. Both SNIP and SNIP-SCC have different degrees of enhancement on MRI. Especially in dynamic MRI enhancement studies, the inflow and outflow velocities of the contrast agents differ due to variability of blood vessel constriction, which leads to differences in the dynamic enhancement curves. This difference is an important index for the diagnosis and differentiation of malignant transformation. Both malignant and non-malignant inverted papilloma can cause bone hyperplasia and calcification . MMs, onAccordingly, our patient’s MRI demonstrated low signal intensity of the lesion on T1- and heterogeneous intermediate to high signal intensity on T2-weighted sequences, a finding which posed inverted papilloma high in the differential diagnosis . In a siRegarding the treatment modalities available, surgical wide excision still remains the first line of treatment for this type of melanomas. Meanwhile therapeutic strategies include controlling local residual disease and preventing distant metastasis. Selective neck dissection and sentinel node biopsy (SLNB) in lymph node negative patients are controversial, whereas the role of post-surgical radiotherapy as adjuvant needs further elucidation .In our case, due to the long course of our patient’s disease and the lack of severe functional impairment, an extended excisional biopsy was endoscopically performed, as FESS produces similar oncologic outcomes and carries smaller risk when compared to traditional open surgical approaches . Clear sImmune checkpoint inhibitors (ICIs) have been an advance in the management of melanoma; however, mechanisms of response and resistance remain poorly understood. Systemic adjuvant therapy with ICIs or targeted therapies have begun to demonstrate a benefit on mortality rates after complete resection of high-risk melanoma (stages III and IV), whereas outcomes remain poor for advanced (metastatic) disease . CombinaDespite these advances, patients with SNMM show limited benefit from current therapeutic strategies and complete excision of the tumor is widely regarded as the sole, proven efficient treatment modality for SNMM ,13. BaseMM is a very rare and aggressive neoplasm that requires vigilance. In the present case, adjuvant immunotherapy was implemented post-surgically for an optimal outcome aiming to eradicate any residual MM, as given the bulk of the tumor, clear surgical margins were impossible to obtain. The slow growth rate of this mildly colored tumor, the complexity of the nasal and septal anatomy and limited accessibility, as well as the longstanding non-characteristic symptomatology, highlight that high clinical suspicion is required, especially in the case of hypomelanotic/amelanotic MM. Moreover, while MRI is the gold standard of imaging for diagnosis, it may occasionally lead to diagnostic pitfalls. Lastly, as with this case, therapeutic options may be limited due to the patient’s age and clinical profile, rendering SNMM survival and prognosis improvement rather problematic.


It can present anywhere throughout the body; however, its onset in the maxillary sinus is an extremely rare condition, making it hard to diagnose. This tumor occurs equally, without predilection for males or females, and the incidence peaks in the age of 35. The diagnosis is confirmed by histopathological study, and the main treatment is complete surgical excision.We are reporting a case of a 53-year-old male with a left sided hearing loss accompanied by a left sided nasal block and a vague facial and dental pain.Magnetic Resonance Imaging (MRI) showed a heterogeneously enhancing tumor in the maxillary sinus that extended to the pterygopalatine fossa and other surrounding structures, and a biopsy showed the tumor to be a synovial sarcoma. The tumor was managed with a less aggressive curative surgery, and was put on an adjuvant radiotherapy, and is being followed regularly; with no recurrence 5 months after therapy.In conclusion, we are writing this report to introduce a case of synovial sarcoma in a rare location that was managed by a “cosmetically oriented” curative surgery, satisfactory results and prognosis. •Synovial sarcoma is a rare type of spindle cell tumors.•Synovial sarcoma of the maxillary sinus is a quite rare condition.•The term “synovial sarcoma” is an old misnomer.•The cellular origin of Synovial sarcoma is still unknown.•The main treatment for synovial sarcoma is surgical excision. The term “synovial sarcoma” was used in early literature due to the microscopic appearance of this sarcoma that is similar to a developing synovium, however, the origin of this tumor is still unknown [Although synovial sarcoma can arise in various sites throughout the body , its ons2A 53-year-old male complained of a left sided hearing loss for few months as well as a left sided nasal block, then developed a vague facial and dental pain few weeks prior to his visit. Physical examination showed the hearing loss to be of conductive nature, reduced sensation in the area of skin innervated by the infraorbital nerve, and no lymphadenopathy was noted. MRI showed a heterogeneously enhancing tumor measuring 38 mm × 28 mm × 42 mm in the maxillary sinus that eroded the medial wall of the sinus, extended to the posterior aspect of the nasal cavity eroding the posterior aspect of the hard palate, and extended posteriorly thereafter closing the pharyngeal aspect of the Eustachian tube causing otitis media with effusion, and reaching the pterygopalatine fossa eventually . No signAll information was pointing towards a possible malignant tumor; Therefore, a biopsy was endoscopically obtained from the tumor in the maxillary sinus. Microscopic examination of the biopsy showed small ovoid to spindle-shaped cells with scanty cytoplasm, some of which demonstrated signs of atypical mitosis. The cells were arranged in short interlacing sheets, with no epithelial components to be found; all of which pointed towards a monophasic spindle cell tumor. Immunohistochemically, the tumor was positive for Vimentin, Bcl-2, and CD99 , and neg3Although synovial sarcoma is considered a quite common soft tissue tumor in the limbs, it can present anywhere in the body with the maxillary sinus being an extremely rare location . The ter4We are reporting a case of maxillary sinus synovial sarcoma that was managed with partial maxillectomy with alveolar arch and anterior sinus wall preservation after most prognostic factors were found to be on the safe side, and the surgical margins were clear on microscopic examination; with satisfactory prognosis and a 5-months disease free period.All the authors declare that they have no conflicts of interest.


Ependymomas are commonly reported at an intradural intramedullary location and more frequently at the conus medullaris or filum terminale. In comparison to this, the incidence of spinal tumors being reported at an intradural extramedullary site is less. We describe a young patient who presented with urinary retention and a long-standing history of back pain radiating to the right lower limb. Imaging revealed an intradural ependymoma extending from D11 to S1 and measuring 21 cm in length. The patient underwent D10 to S1 laminectomy. Although the tumor originated from the conus medullaris, the histological evaluation revealed a WHO grade II ependymoma, which is rare, as only 30% of tumors in this location are non-myxopapillary. Ependymomas are tumors that originate from the ependymal cells lining the ventricles and central canal of the spinal cord. These are slow-growing tumors, with the majority being intramedullary in location. Only 30% of the spinal tumors are reported as intradural extramedullary (IDEM) -2. This A 27-year-old female visited the hospital with a complaint of retention of urine for one day. The patient also had a history of low back pain radiating to the right leg, which developed gradually over eight years. She also noted this pain at rest and developed lower limb weakness during the same period. The pain significantly reduced the walking distance. Her past medical history was unremarkable. Neurologic examination revealed decreased strength in the right leg (MRC grade - 3/5). The tone of the anal sphincter was normal. No significant finding was noted in the blood tests. Magnetic resonance imagining (MRI) of the whole spine revealed a large, space-occupying, intradural spinal lesion extending from the D11 to S1 segments  and preserving the healthy tissue. The removal depends on the location, grade, histology, and presence of a tumor capsule. Since spinal cord ependymomas rarely infiltrate the spinal cord, the rate of GTR in these is as high as 84%-93% -16. In aIn the case presented here, the patient underwent laminectomy from D10 to S1, and careful surgical resection was performed to separate it from the conus medullaris. Postoperative MRI confirmed the removal of the tumor. On a six-months follow-up, there was no sign of recurrence and the patient had no neurological deterioration. The patient will be followed long-term with an enhanced MRI to exclude relapses .We report a rare case of multisegment IDEM ependymoma measuring 21 cm originating from conus medullaris and extending over the dorso-lumbar and sacral regions. Even though ependymomas situated in this region are commonly reported as myxopapillary, the tumor in the current case revealed a non-myxopapillary variant (WHO grade II) on histological examination. The tumor was resected via D10 To S1 laminectomy without any deterioration of neurological functions. There have been no recurrences on a six-month follow-up.


Congenital disorder of glycosylation-Ia (CDG-Ia) is a rare autosomal recessive genetic disorder, characterized by systemic and ophthalmological abnormalities. Here, we report multiple retinal astrocytic hamartomas as a new retinal finding in an adolescent affected by congenital disorder of CDG-Ia. A 15-year-old boy affected by CDG-Ia underwent full ophthalmic examination, full field electroretinography (ERG) evaluation and retinal multimodal imaging, including: fundus photography, spectral domain optical coherence tomography (SD-OCT) and blue fundus autofluorescence (FAF). Blue FAF showed multiple papillary and iuxtapapillary bilateral hyper-FAF lesions, corresponding to hyperreflective thickening of the retinal nerve fiber layer, with internal optical empty spaces and posterior dense optical shadowing at SD-OCT. These imaging findings were consistent with retinal astrocytic hamartomas. Scotopic ERG response was significantly reduced in both eyes. Macular edema and absence of the retinal outer segments layer were also detectable. Retinal multi-modal imaging provides additional insights about retinal involvement of patients affected by CDG-Ia. In particular, this case shows the presence of multiple retinal astrocytic hamartomas. Congenital disorder of glycosylation (CDG) Ia, also known as phosphomannomutase 2 (PMM2)-CDG, is a rare autosomal recessive genetic disorder, characterized by neurometabolic abnormalities –3. Main A 15-year-old boy affected by, genetically confirmed, CDG-Ia complaining progressive high myopia, visual acuity impairment and night blindness, was referred to our pediatric low vision unit. His systemic history was characterized by: hypotonia, abnormal fat distribution, joint contracture, developmental delay and feeding difficulties. Eye examination showed: bilateral high myopia (−8.0 diopters), best-corrected visual acuity of 20/40 in both eyes, exotropia and hypertropia. Fundus biomicroscopy of the right eye revealed vitreous disorganization and, only in the right eye, a iuxtapapillary, white, gliotic tissue. Optic disc of the left eye appeared normal. Blue fundus autofluorescence (FAF) of the right eye showed multiple intensely hyper-FAF spots corresponding to the gliotic tissue, and an isolated hyper-FAF spot on the optic nerve head. Blue FAF of the left eye showed a mild hyper-FAF spot on the optic nerve head. Spectral domain optical coherence tomography (SD-OCT) of these lesions showed hyperreflective thickening of the retinal nerve fiber layer (RNFL) with internal moth-eaten optical empty spaces and posterior dense optical shadowing. The combined imaging techniques were diagnostic for multiple, bilateral, calcific retinal astrocytic hamartomas . In partCDG-Ia is the most common congenital disorder of N-glycosylation. Mutations in the PMM2 gene, located on chromosome 16p13, cause a deficiency of PMM. This cytoplasmic enzyme has an essential role in the N-glycosylation process and in the synthesis of glycosylphosphati-dylinositol, which is used to anchor proteins to the cell membrane . ImmunohIn conclusion, the present case confirms the essential role of retinal multimodal imaging which provides, in a non-invasive way, new insights in the retinal involvement of patients affected by CDG-Ia. In particular, retinal multimodal imaging was instrumental to document the association, never reported before, between CDG-Ia and multiple, bilateral, calcific retinal astrocytic hamartomas. These new findings should be considered in the multidisciplinary evaluation of children within CDG, and retinal and/or brain astrocytic hamartoma, which share the same originating cell, should be excluded.The original contributions presented in the study are included in the article/supplementary material, further inquiries can be directed to the corresponding author.Written informed consent was obtained from the minor(s)' legal guardian/next of kin for the publication of any potentially identifiable images or data included in this article.GM and EP: data research, case design, drafting and revising, final approval, and agreement to be accountable for all aspects of the work.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article, or claim that may be made by its manufacturer, is not guaranteed or endorsed by the publisher.


We report a rare case of non-communicating rudimentary horn pregnancy (RHP). The patient presented with lower abdominal pain and underwent laparoscopic surgery in which the gestational tissue was removed without excision of the rudimentary horn and ipsilateral fallopian tube. Unicornuate uteri often coexist with rudimentary horns, most of which are non-communicating. RHP is rare, and symptomatic women tend to complain of abdominal pain. Once RHP is suspected, the clinician should monitor the patient for signs of hypovolemic shock, such as hypotension, because the RHP can rupture owing to the poorly developed musculature. Early surgical intervention with removal of the rudimentary horn along with the ipsilateral fallopian tube is generally suggested to prevent future ectopic pregnancy. The theory of sperm transmigration from the contralateral oviduct has been hypothesized in non-communicating RHP. The incidence of unicornuate uterus is 0.4%, with approximately 84% of the women having a contralateral rudimentary horn . In one A 37-year-old woman  presented with sudden onset lower abdominal cramping pain associated with cold sweat and diarrhea. She gave birth to her first baby through caesarian section owing to breech presentation at a gestational age of 38 5/7 weeks. According to the patient, no structural Müllerian abnormality was recalled at that time. Fifteen days before admission, the patient noted that she had missed her expected menstrual period. Pregnancy was confirmed by the doctor. Transabdominal sonography did not reveal any intrauterine gestational sac at the time, and neither was an intrauterine sac found ten days later, with a serum β-HCG level of 8487 mIU/mL. Under the suspicion of ectopic pregnancy, she was referred to our outpatient department at a gestational age of 7 1/7 weeks in accordance with her last menstrual period. Transabdominal sonography revealed a right adnexal mass adjacent to the uterus, with a size of 2 cm and a hypoechoic sac-like structure . Her serA single intramuscular injection of MTX (50 mg) was administered. The patient returned two days later because of another episode of lower abdominal cramping pain. Transabdominal sonography revealed scant fluid collection over the cul-de-sac. Her serum β-HCG level was 27,472 mIU/mL. The patient was admitted for laparoscopic intervention. Whole abdominal computed tomography (CT) without contrast was performed before surgery, which showed a right adnexal mass in compliance with ectopic pregnancy .Upon entering the pelvic cavity, a unicornuate uterus with a right rudimentary horn was identified, with the attachment of round ligaments as well as ovarian ligaments and the fallopian tube to the horn . There wThe patient was discharged uneventfully, and her serial serum β-HCG levels were followed up until normal. The final pathology report indicated gestational products. An office hysteroscopy was performed after the commencement of her first menses, with no opening found between the uterine cavity and the horn. A left unicornuate uterus with non-communicating right RHP, probably conceived through transmigration of the sperm via the left oviduct, was the final diagnosis.Mullerian abnormalities are reported in 0.17% of fertile women and 3.5% of infertile women, and the unicornuate uterus is found in 0.4% of all women . Non-comThe other female genital tract anomaly classification is the ESHRE/ESGE system based on the main uterine findings (U0–U6) and cervical/vaginal anomalies ; the present case was categorized as U4 C0 V0 .The overall sensitivity of ultrasonography for a definite diagnosis of rudimentary horn was 26%, and only 14%  could be diagnosed before symptom presentation [RHP is rare, with a reported incidence ranging from 1 in 76,000 to 1 in 150,000 . MassiveThe theory of transmigration of sperm from the contralateral oviduct has been advocated in an article by Ansari et al. , with co