Introduction: Spontaneous iliac vein rupture (SIVR) is an uncommon disease with less than sixty cases reported before. This disease often requires surgical intervention. SIVR has never been imaged in the literature. This report shows how to image this diagnosis. Case report: A 71-year-old female was diagnosed with SIVR with the use of CT venography. Endovascular repair with 2 endografts and a sinus XL stent was performed. Postoperatively, the patient developed abdominal compartment syndrome and a large part of the intestines had to be removed because of ischemia. Discussion: This is the first report that shows SIVR before and after endovascular treatment with the use of CT venography by injecting a contrast bolus in the femoral vein. This information is of high interest for a broad range of clinicians to show or exclude a venous abdominal bleeding in an early stage. Spontaneous iliac vein rupture (SIVR) is a rare disease associated with high mortality. Until now, less than 60 cases having been reported in literature since the first reported case in the 1960′s This study was conducted in accordance with the modified PROCESS criteria, which provides a structure for reporting surgical case series A 71-year-old female presented to the emergency department with hypovolemic shock. Earlier that day she had acute pain in her left hip and lower part of her abdomen. She had no history of trauma. A contrast-enhanced computed tomography (CT) was obtained. This revealed a retroperitoneal hematoma on the left side, see video 1. For many hours, the diagnosis was unclear. There was no evidence of active contrast leakage on CT, but an ectatic external iliac vein was observed. Then a central venous line was put into the left femoral vein. A bolus of 50cc contrast (iodine) was injected to make a CT venography. This showed SIVR (SIVR is a very uncommon event. To our knowledge this is the first time SIVR has been diagnosed with CT venography by injecting a contrast bolus in the femoral vein. Not to mention the fact that this has never been imaged in the literature after endovascular repair. In this case, the diagnosis was unclear for a relative long period. This report is of high relevance for a broad range of clinicians due to the fact that this imaging technique can be used in daily practice to early exclude or show a venous abdominal bleeding.In conclusion, CT venography by injecting a contrast of bolus in the femoral vein makes it possible for early diagnosis of SIVR. This imaging technique is especially useful when a normal CT-scan shows a retroperitoneal hematoma.This research did not receive any specific grant from funding agencies in the public, commercial or not-for-profit sectors.Waived by the ethical committee of Amsterdam UMC, location VUmc.A.A.J. Grüter wrote the paper. S.F. Oudkerk gave his feedback on the paper and performed the procedure and the CT venography.Patient signed informed consent for the use of clinical data and images and videos of CT scan. The authors confirm that informed consent of the patient for publication has been obtained. Appropriate consent has been obtained to include case details and medical images.


Kimura disease (KD) is a rare, chronic inflammatory disease characterized by painless subcutaneous nodules predominantly located in the head and neck regions. Orbital KD, which intrudes into the intraconal space and results in compressive optic neuropathy, is rare and has not been previously reported.A 68-year-old man presented with blurred vision and progressive proptosis in the left eye that had been present for 2 years.Magnetic resonance imaging of the brain revealed soft tissue lesions with contrast enhancement and restricted diffusion involving the bilateral eyelids, orbits, and intraconal region; those on the left side were more prominent than those on the right side. The lesion encased the left optic nerve. Laboratory test results revealed elevated serum immunoglobulin E level and peripheral eosinophilia. An orbital mass biopsy demonstrated hyperplastic lymphoid follicles with germinal centers in the subcutaneous area and abundant mononuclear and binuclear eosinophils infiltrating the interfollicular area. A pathological diagnosis of KD was made based on the blood test results.Orbital decompression and debulking surgery of the orbital tumor in the left eye were performed to treat the compressive optic neuropathy.After systemic oral steroid and immunosuppressive agent therapies, the patient’s visual acuity in the left eye improved, and the KD activity was stable.We present a rare case of orbital KD-associated optic neuropathy, wherein early diagnosis and treatment preserved the patient’s vision. This complication should be considered in patients with a history of compressive optic neuropathy during the differential diagnosis. To the best of our knowledge there are no reports of orbital KD that intruded into the intraconal space and resulted in compressive optic neuropathy in the literature available in English. Here, we report a rare case of optic neuropathy resulting from KD.Kimura disease (KD) is a rare, chronic inflammatory disease characterized by painless subcutaneous nodules predominantly located in the head and neck regions. KD is primarily observed in young men of Asian descent. Elevated serum immunoglobulin E (IgE) levels and peripheral eosinophilia are common in patients with KD. The masses most commonly occur in the parotid, submandibular, and peri-auricular regions,A 68-year-old man presented with blurred vision and progressive proptosis in the left eye that had been present for 2 years. He had a history of KD which had affected the right upper eyelid and had undergone treatment by excision after which systemic steroid therapy was administered 6 years ago. However, the patient was lost to follow-up.Ophthalmologic examination revealed intraocular pressures of 9 mmHg in the right eye and 11 mmHg in the left eye. The left eye demonstrated proptosis and restricted movement in all directions, whereas the right eye movements were intact. The visual acuity of the right eye was 20/25 and that of the left eye was counting fingers at 1 meter. The relative afferent pupillary defect was positive in the left eye. Slit-lamp examination revealed mild bilateral cataracts. Funduscopic examination showed negative findings in the right eye , serum immunoglobulin G4 level , and peripheral blood eosinophil count . Orbital decompression and debulking surgery of the orbital tumor in the left eye were performed to treat compressive optic neuropathy. Biopsy of the orbital mass revealed hyperplastic lymphoid follicles with germinal centers in the subcutaneous area and abundant mononuclear and binuclear eosinophils infiltrating the interfollicular area Fig. .Based on the blood test results, a pathological diagnosis of KD was made. The patient was started on oral corticosteroids, and long-term oral administration of cyclophosphamide (50 mg/week) was initiated to modulate immunity. There was reduction in proptosis and improvement of movement in all directions in the left eye. After 6 months of treatment, the visual acuity of the left eye improved from counting fingers at 1 meter to 20/63, and SD-OCT demonstrated improvement in the GCC (Fig.  In 1937, this condition was first described by Kimm and Szeto in 7 cases from China. The disease was officially designated by its current name in 1948 because Kimura et al. characterized the pathological features of the disease.KD is a benign, chronic granulomatous inflammatory disorder of unknown etiology, although it is hypothesized to represent an allergic or autoimmune process. Histologically, KD is identified by follicular hyperplasia with active germinal centers along with a proliferation of arteriolar and capillary vasculature encompassed by concentric rings of reactive fibrosis composed of hyaline and collagen. Numerous lymphocytes and prominent eosinophils infiltrate the interfollicular zones.The epidemiology of KD revealed that while young Asian men are the most commonly affected, it can occur in both men and women belonging to different ethnicities and age groups. The typical triad of clinical symptoms include unilateral painless cervical adenopathy or subcutaneous masses largely in the head and neck region, characteristic histologic features, as well as peripheral eosinophilia, and elevated serum IgE levels. It usually affects the lacrimal glands or eyelids, although its occurrence is infrequent. Furthermore, there are no known cases of optic neuropathy caused by KD. Optic neuropathy is caused by damage to the optic nerve that may occur because of several reasons such as ischemia, compression, inflammation, infection, hereditary conditions, toxins, and trauma. In cases of compressive optic neuropathy, lesions within the orbit caused by tumors, infections, and inflammation can compress the optic nerve, resulting in progressive visual loss. Compressive optic neuropathy also causes thinning of the GCC and RNFL which can be observed on SD-OCT scans and may be confirmed or ruled out by magnetic resonance imaging of the brain and orbits. While there is currently no definitive, optimal treatment for KD, surgical excision of the orbital or adnexal lesions is recommended. Complete excision may be difficult because of the unclear margins of these lesions. However, if excision is incomplete, recurrence is common. Although the side effects of radiotherapy are considerable, it has been used successfully in a few cases. KD generally responds well to oral or intravenous steroids, although it often recurs after steroid withdrawal. If patients are refractory to steroid treatment, they may be treated using systemic immunosuppressive agents.Takenaka et al reported the first case of orbital KD in 1976.We described a rare case of optic neuropathy associated with orbital KD. Our patient was treated using orbital decompression along with debulking surgery of the orbital tumor. Subsequently, systemic oral steroids and immunosuppressive agents were administered. After 6 months of treatment, the thickness of the GCC and RNFL as well as visual acuity improved. Early diagnosis and treatment aided in the preservation of the patient’s vision. KD should be considered as part of the differential diagnosis in patients with mass-induced compressive optic neuropathy when accompanied by peripheral eosinophilia and elevated serum IgE levels.The authors would like to thank the patient for his participation in this study.Conceptualization: Chih-Kang Hsu.Data curation: Ke-Hung Chien, Chih-Kang Hsu.Methodology: Yi-Hao Chen, Chih-Kang Hsu.The histological and pathological examination: Tung Liu.Writing – original draft: Yung-En Tsai.Writing – review & editing: Chih-Kang Hsu.All authors read and approved the final manuscript.


Retinal arterial occlusive events in young patients are rare. However, because of physiological multifactorial adaptations during pregnancy, retinal vascular occlusive disease may occur spontaneously. In addition, a patent foramen ovale is a risk factor for an ischemic thromboembolic event. Since fluorescein angiography, a central tool in the evaluation of these occlusions, should be avoided during pregnancy, optical coherence tomography angiography, a novel technique, offers a good opportunity for visualizing vascular perfusion of retinal tissue.Here we present a case series of three patients  who visited our clinic owing to acute visual impairment and central scotoma. Using regular optical coherence tomography and optical coherence tomography angiography, retinal vascular occlusions were detected, thus initiating the evaluation of systemic risk factors. We report two patients (30 and 32 years old) who developed cilioretinal artery occlusion but whose etiology differed: one was of thromboembolic origin associated with patent foramen ovale, while the other was caused by hemodynamic blockade secondary to central retinal vein occlusion. In both cases, optical coherence tomography angiography revealed reperfusion of the cilioretinal artery occlusion. However, transient ischemia led to retinal atrophy after a few weeks. In the third patient (32 years old), 8 weeks after onset of scotoma, optical coherence tomography angiography revealed atrophy of the middle layers and impaired perfusion in the deep capillary plexus, and thus a paracentral acute middle maculopathy was diagnosed. All patients regained normal visual acuity and had otherwise uncomplicated pregnancies, and laboratory blood tests did not reveal any defects or alterations.As shown here, optical coherence tomography angiography enables risk-free imaging of retinal vessel perfusion during pregnancy. Together with regular optical coherence tomography, it allows one to predict functional outcome according to the existing retinal occlusion-related atrophy. Retinal arterial occlusive diseases (RAO) in young patients are rare. Only 11.4% of all RAO occur in people under 50 years of age . There aNormally, fluorescein angiography is a central part of diagnosing a retinal vascular occlusion. It has been established that fluorescein dye crosses the placenta into the fetal circulation , yet detHere we present a case series of three patients who visited our clinic owing to acute visual impairment. RAO were detected using OCT and OCTA, thus initiating the evaluation of systemic risk factors.Case presentationsA 32-year-old Caucasian pregnant woman week 19) with a 2-day history of a scotoma of her right eye presented with visual acuity (VA) of 5/200 (Snellen). The ophthalmological history was otherwise unremarkable. Fundoscopy revealed a cilioretinal artery occlusion (CLRAO) accompanied by a non-ischemic central retinal vein occlusion (CRVO). Initial OCT and fundus autofluorescence  showed foveal involvement with edema of the inner retinal layers mainly of the superior macular area  and remained stable during the entire follow-up. Within a week, the macular edema began to decrease Fig. E, F whil/20 and rAt week 3 after the onset of symptoms, there was beginning atrophy of the inner retina resulting in a reduced volume of the superior part of the macula and a persistent, massive increase in optic disc volume. After 6 weeks, the atrophy of the macula progressed, and the optic disc edema began to resolve Fig. J–N. At tThe patient was a nonsmoker and had no significant family history of hypercoagulable disorders. Extensive cardiovascular , hemostaseological testing , and obstetric examination were unremarkable. Antiplatelet therapy  was initiated.A 30-year-old Caucasian pregnant woman (week 10) presented with a 1-day history of scotoma on the right eye. Fundoscopy and OCT showed a diffuse swelling of the inner retina layers of the upper macular region Fig. A, B. VA The patient was a nonsmoker and had no significant family history of hypercoagulable disorders. Cardiovascular evaluation, as described above, revealed a PFO. Hemostaseological evaluation, as described above, and obstetric examination were unremarkable. Antiplatelet therapy  was initiated, and the patient was recommended to have an interventional PFO closure after pregnancy.A 32-year-old Caucasian pregnant patient (week 16) presented with a VA of 20/20 of the right eye. Eight weeks before, she had noticed a central scotoma. She was diagnosed with a paracentral acute middle maculopathy (PAMM). Infrared imaging showed typical defect nasally in the fovea , a possible cause of PAMM, had been ruled out at the beginning of the pregnancy and was not retested. Antiplatelet therapy  was initiated. PFO closure was not recommended by cardiologists.We present three cases with three distinctly different types of retinal vascular occlusion in pregnancy. Even though case 1 and 2 both depict CLRAO, there is a difference in the pathogenesis. Etiologically, CLRAO is of three distinct types: CLRAO associated with CRVO or hemi-CRVO (like in case 1), non-arteritic CLRAO alone  above the level of that in the cilioretinal artery. Unlike regular non-arteritic CLRAO, there is no thrombotic or embolic occlusion of the artery in this type . ConsistNon-arteritic CLRAO, as seen in patient 2, is caused by classical thromboembolic occlusion. In our case, the OCTA showed reperfusion 10 days after the onset of the scotoma Fig. , N.et al. [et al. [Paracentral acute middle maculopathy (like in case 3) was first described in 2013 by Sarraf et al.  and was et al.  as depic [et al. , we were [et al. , 28, 29. [et al. –32. LikeOCTA was successfully performed in all three cases. Reperfusion was seen in cases 1 and 2, which could be considered a surrogate marker for better functional outcome in terms of a smaller scotoma due to a smaller atrophic area.In pregnancy, preexisting conditions may deteriorate owing to hormonal, hematologic, metabolic, cardiovascular, and immunologic changes that induce retinal vascular occlusive events , 3. AssoOur patients all had otherwise uncomplicated pregnancies, and laboratory blood tests did not reveal any defects or alterations. Two of the three women (case 2 and 3) possessed a PFO. This is a congenital heart defects with a short-circuit connection between the right and left atrium and is found in around 30% of all healthy adults . Though OCTA is a valuable option to noninvasively diagnose and analyze the retinal perfusion status in pregnancy when fundus fluorescein angiography should be avoided. Together with OCT imaging, OCTA allowed us to predict functional outcome in addition to the existing retinal occlusion-related atrophy.


First -degree cutaneous extraskeletal Ewing's sarcomas (ESs) are incredibly uncommon skin-specific tumors that often present as a single, tiny lesion that is restricted to the mid-to-deep dermis or involves the subcutis. ESs can be clinically and pathologically misdiagnosed because of their rarity and physical resemblance to other cutaneous cancers.A 47-year-old nonsmoking woman was admitted after being transferred from a nearby hospital to check her right foot pain that had been present for three months and was significantly numbing the same side. Only a few lone cases or brief series are reported in the current literature. The typical description of ESs is that they are tiny masses with positive clinical behavior.Despite being a rather common location, only infrequent and minor ESs of the foot are present. After the recommended operation and subsequent histology analysis, we identified this uncommon sort of tumors.Although it's rare, it's very important to consider this tumor in the differential diagnosis of foot pain with/without visible and/or palpable cutaneous lesion. •First -degree cutaneous extraskeletal Ewing's sarcoma is a peculiar soft tissue malignancy occurring in 1 in 5–10 million people.•Following osteosarcoma, Ewing sarcoma is the second most common primary bone malignancy.•The patient was treated with surgery followed by chemotherapy.•This neoplasm should be included in the differential diagnosis of foot pain and/or lesion. These cells resemble each other in the original neuronal tissue and molecular lineage. Along with EES, ESFT also entails the classic Ewing sarcoma of bone (ESB), which is considered the second most frequent type of primary bone neoplasms in children age group, peripheral primitive neuroectodermal tumor (pPNET) and its variant Askin's tumor of the thoracic wall ,2. Altho2S.F., A 47-year-old nonsmoker woman transferred to the hospital via ambulance and was afterwards admitted for the assessment of chronic and persistent pain in the right foot for a three-months duration associated with significant numbness on the same side. after that, the patient started to complain of acute-onset sever lower back and pelvic pain. She asserted never suffering pain similar to this before. The patient reported no personal and/or family history of cancer, any acute, repeat, or discontinued medications, any allergies, any genetic or psychosocial issues, and had a free past surgical history. physical examination was unremarkable except for lower back tenderness radiated to both lower limbs and marked lower limb weakness with estimated power tone of 2/5 on the right foot. Neurovascular assessment was insignificant. The patient initially underwent spinal computed tomography (CT) without contrast which revealed multiple enhancing metastatic lesions throughout the spine, the most significant lesions are in the thoracic area, particularly at the level of T8-T10 vertebral bodies and mild pathological compression fractures of T4, T5, T9, T10, T11 and L5 associated with a large anterior paraspinal soft tissue component at the same level  A, B & C3We discuss an unusual example of extraskeletal Ewing's sarcoma in the foot. Even though it's uncommon, it's crucial to include this tumor in the differential diagnosis of foot discomfort whether or not there is a palpable or visible cutaneous lesion.Extraskeletal Ewing sarcoma (EES) is a soft tissue sarcoma (STS) that is uncommon, occurring in 1 in 5–10 million people, and makes up only a small percentage of the 12,000 STS that occur annually on average . Few subTheir typical clinical manifestation is a superficial, single mass that is 2–3 cm in diameter, soft, movable, and occasionally painful, with an average period to evolution of 5 months . A mediaThe diagnosis of superficial ES is challenging due to the rarity of these tumors, the relative non-specificity of their histology, and their immunoprofile, and a wide range of differential diagnoses must be taken into account, including Merkel cell carcinoma, cutaneous lymphomas, clear cell sarcoma, malignant primitive neuroectodermal tumor, small cell carcinoma, rhabdomyosarcoma, malignant rhabdoid tumor, and poorly differentiated adnexal Unlike its deep counterpart, they showed a generally positive clinical behavior and had a 91% 10-year survival rate. Due to their tiny size and potential for early discovery and complete surgical removal because of their superficial placement, they most likely prevented metastatic spread in the majority of patients . To checThe principles of treatment are currently the same as those for Ewing sarcoma of the bone, including wide surgical resection, being associated with or without chemotherapy and/or radiotherapy, depending on its size and location, even though a conclusive conclusion on the most effective treatment modalities has not yet been clarified because of the extreme rarity of these tumors ,17. Chem4In conclusion, we describe a peculiar case of foot extraskeletal Ewing's sarcoma. Although its rare, its very important to consider this tumor in the differential diagnosis of foot pain with/without visible and/or palpable cutaneous lesion.Not commissioned, externally peer-reviewed.The authors declare that writing and publishing this manuscript was not funded by any organization.This study is exempt from ethical approval in our institution.A written informed consent for the data and picture was taken from the patient and the family and available upon request from the Editor-in-Chief.Writing the manuscript: Oadi N. Shrateh, Afnan W.M. Jobran Imaging description: Haneen Owienah, Oadi N. Shrateh, Thaer Sweileh, Mohand Abulihya, Motaz A. Natsheh Reviewing & editing the manuscript: Nazeeh Abu-Dayyah, Oadi N. Shrateh, Afnan W.M. Jobran.Not Applicable.Oadi N. Shrateh.The authors declare that there is no conflict of interest regarding the publication of this article.


This is a case report of a 5.6 cm glomangioma supplied by the femoral profunda artery in a 66-year-old male patient with severe peripheral artery disease. The patient complained of discomfort and mild pain at the place of the lesion and an accelerated growth rate in the last two months. A nodular mass located laterally on the left foot, elastic, covered with a thin skin, and mobile, was noted on the clinical exam. Doppler exam demonstrated an active vascular supply. CT angiography showed a femoral profunda artery blood supply and a severe asymptomatic bilateral peripheral artery disease (PAD). The lesion was removed entirely by surgery. A microscopy exam revealed a glomangioma. After surgery, the patient recovered unevenly. However, the patient experienced wound-healing issues that resolved after four weeks of surgery. Although the patient’s PAD was severe, the lesion presented with a burst in dimensions weeks before surgery. Glomangiomas are benign skin tumors characterized by abnormal, smooth muscle-like glomus cells . The tumThe methods applied were anamnesis followed by physical examination, diagnostic imaging, morphophonological diagnostics, and surgery followed by a three month follow-up, respectively. Peripheral artery disease (PAD) was classified according to Fontaine classification, and oscillography was also performed.Anamnesis: The patient is a Caucasian, retired, 68-year-old male. Leg pain, paresthesia in the groin, and local erythema were the major complaints of the patient. A positive history of hypertension and gastric ulcer was noticed. The family medical history was unremarkable. Alcohol and tobacco consumption were also noted.A physical exam showed a mild femoral pulse at the left groin, with a 5.6 cm pseudotumoral formation below the left knee . The strDiagnostics: The patient was found to have Fontaine type II A PAD with a measured 0.9 ABI for the right foot and 0.8 ABI for the left foot.CT contrast imaging (pelvis and lower limbs) was performed to investigate the tumoral process further. .The patient was subjected to surgery. The tumor incision was performed within tumor-free tissue. Jean L Bolognia et al.’s two-volume set described glomangioma clinical findings and therapy . AnotherDisease course: The morphopatolgical exam was remarkable for hemangioma. The patient tolerated the procedure well. A healing defect was observed three weeks after the incision. Dehiscence of the surgical incision was observed. The edges of the wound were no longer connected after suture removal. The defect was observed only in the skin and connective tissue and was not observed in muscles and profound planes. The defect was lined on 2 cm . The healing deficiency was resolved surgically. After three weeks, the wound healed completely. Laboratory tests were standard except for a mild leucocytosis. No further diagnostic challenges were recorded.Although glomangiomas are relatively small tumors (1 cm), when the tumor vascular pedicle is connected to a large vessel, the cancer can probably reach a considerable size ,19,20.The differential diagnosis for glomangioma includes vascular malformation, which does not have perivascular glomus cell accumulation ,22. OtheMalignant glomus tumors are rare. The criteria for diagnosing malignancy in glomus tumors are (a) tumor size of more than 2 cm and subfascial or visceral location; (b) atypical mitotic figures; (c) marked nuclear atypia and any level of mitotic activity; (d) and detection of Zimmerman’s pericytes ,26.Malignant glomus tumors are divided into three categories based on their histology: glomus tumors with local infiltration (LIGT), glomangiosarcomas arising in benign glomus tumors (GABG), and de novo arising glomangiosarcomas (GADN) .Some instances of malignant glomus tumors are described. However, metastases are rare. The misdiagnosis of many of these lesions, such as hemangiomas or venous malformations, makes a precise diagnosis difficult. Vascular malformations do not have the perivascular glomus cell accumulations seen in glomangioma, Myopericytoma, angioleiomyoma, and myofibroma. All have variable degrees of perivascular muscle cells, but these cells do not have the classic cuboidal glomus cells aspect ,29. ThesGlomus tumors can present a significant size if the tumor vascular pedicle is connected to a major artery, even if the patient presents with peripheral occlusive disease. In this case, the patient presented with intermittent claudication at 400 m. PAD was located on the common femoral artery bilaterally and the profunda femoris artery on the left leg.A 5.6 cm glomangioma was diagnosed on a 66-year-old PAD patient. Glomangiomas of the lower limb can coexist with peripheral artery disease. Presumably, the morphology and tumor evolution do not depend on the arterial disorder’s extent and staging.


Mucoceles of the paranasal sinuses are epithelium-lined cystic masses that develop when the sinus ostia get obstructed. They most frequently occur in the frontal and ethmoid sinuses. The paranasal sinus mucoceles' proximity to the orbit and skull base renders the patient at risk for substantial morbidity. Mucoceles have reactive bone growth, bleeding, fibrosis, and granulation tissue, which are histological traits of respiratory mucosa. The conventional therapy is surgical excision, with endoscopic procedures becoming more popular. A 60-year-old female patient reported to the ENT outpatient clinic complaining of swelling over the medial aspect of her left eye that had begun slowly and progressed over a year. Although there were no neurological, ocular, nasal, or facial symptoms clinically, radiological and cytological examinations aided us in arriving at the definitive diagnosis. The patient in this scenario had an infected mucocele and left medial canthal swelling with no visual impairment, which made it challenging to reach an accurate diagnosis. However, radiological evaluation and cytological examination focused on establishing a definitive diagnosis. Paranasal mucoceles are progressively developing cystic lesions with pseudostratified columnar epithelium against a background of chronic inflammation filled with inspissated mucus exerting pressure on the normal boundaries of the sinus due to the obstruction of sinus ostium . The mucA 60-year-old female patient presented to the ENT outpatient clinic with swelling over the medial portion of her left eye for over a year, which had been insidious in onset and progressive Figure . The sweAn anterior rhinoscopy revealed a roomy nasal cavity with bilateral inferior turbinates atrophy, and a smooth reddish nasal mass was seen arising from the lateral nasal wall. On probe test, the swelling was non-friable, non-tender, and sensitive to touch; attachment of the nasal mass could not be ascertained. Posterior rhinoscopy and nasal patency tests were normal. Ear, throat, neck, and cranial nerves examination was within normal limits. Diagnostic nasal endoscopy disclosed a reddish mass in the left nasal cavity arising from the lateral nasal wall, not bleeding on manipulation and non-friable Figure . It was On ophthalmic evaluation, there was no visual impairment and proptosis clinically.On contrast-enhanced CT (CECT), there was a heterogeneously enhancing soft tissue density lesion in the left frontal sinus that extended into the ethmoidal and maxillary sinus, causing a widening of the ostium and remodeling with areas of cortical break of the inner plate of the frontal sinus, ethmoidal septa, and medial wall of left orbit with extension into the extraconal compartment of left orbit abutting on medial rectus Figure .On MRI Figure , a heterManagementOur surgical plan included endonasal endoscopic sinus surgery with marsupialization, which included left uncinectomy and left total ethmoidectomy. Aspiration was undertaken from the swelling, which revealed pus, confirming it to be mucopyocele. A microdebrider was used to locate and open the mucopyocele's inferio-medial wall, which had grown into the left ethmoid sinus and blocked the frontal recess. The anterior wall of the mucopyocele, which extended laterally up to the lamina papyracea, was debrided. The thick yellowish secretions of the mucopyocele were suctioned out, followed by irrigation with saline using a curved suction. After that, the mucocele cavity was examined under direct vision, and no pulsating secretions were noted Figure . The lefHistopathological evaluation Figure  further In addition to the surgical intervention, the patient was administered 1.2 g of amoxicillin injection with clavulanate every 12 hours for seven days and 400 mg of metronidazole every eight hours for at least 72 hours, as well as analgesic and anti-inflammatory drugs. A postoperative nasal endoscopy was performed to guarantee complete disease clearance and sinus drainage. The patient was started on once-daily saline nasal irrigations with a monthly follow-up. After three months of follow-up, the patient was re-evaluated, and during the endoscopic examination, the left maxillary frontal and ethmoidal sinus ostium was found to be widely patent, with improved mucosal edema and purulent outflow showing no signs of recurrence.A mucocele is an epithelium-lined sinus with a completely blocked outflow tract, where mucoid discharge is retained. The epithelial lining continues to exude mucus into this enclosed region, which leads to their propensity for growth and destruction of the surrounding tissues ,2. A mucTo standardize frontal sinus mucocele assessment and treatment, the following classification was devised .Type 1: Limited to frontal sinus Type 2: Fronto-ethmoid mucocele Type 3: Erosion of the posterior sinus wall:A. Minimal or no intracranial extension B. Major intracranial extensionType 4: Erosion of the anterior wallType 5: Erosion of both anterior and posterior walls:A. Minimal or no intracranial extension B. Major intracranial extensionSurgical excision is the treatment of choice, and early intervention is indicated to prevent visual compromise in cases of mucocele . SurgicaWe discussed the case of a patient who presented with unusual signs and symptoms, and judicious intervention with different diagnostic modalities helped us to reach the diagnosis. As long as proper views of the paranasal sinuses are taken, the typical radiological findings of a mucocele are of tremendous use in establishing a diagnosis. The treatment for asymptomatic uncomplicated frontal mucoceles should be carried out with endoscopic sinus surgery and marsupialization. The prevention of recurrence also depends on ensuring adequate drainage of the involved sinus. More radical approaches are required if the mucoceles are extensive and if there is considerable bone erosion causing orbital or intracranial complications.


Background: Pilomatrix carcinoma (PC) is a rare skin tumor arising from hair follicle matrix cells. It is locally aggressive with a high rate of local recurrence after surgical excision. Few cases in the literature have been described and the management is not well defined. Objectives: The aim of this study was to present two cases of PC located on the head and review the relevant literature about epidemiology, clinical and dermoscopic evaluation, characteristics of local and distant metastases, local recurrence rate and management of this rare skin tumor. Methods: We consulted databases from PubMed, Research Gate and Google Scholar, from January 2012 to November 2022. We reviewed the literature and reported two additional cases. Results: We selected 52 tumors in middle-aged to older patients located mostly on the head. Dermoscopy evaluation was rarely performed in the pre-operative diagnostic setting. The most definitive treatment was wide local excision, but local recurrences were common. In total, we observed 11 cases of recurrences and 9 patients with locoregional or distant metastases. Four patients received adjuvant radiotherapy, two patients needed chemotherapy and local cancer therapy and one patient received radiochemotherapy. Conclusion: Our reports and the review of the literature can provide a better awareness and management of this rare tumor. Pilomatrix carcinoma (PC) is a rare skin tumor arising from hair follicle matrix cells and it is most frequently located on the head and neck region of male patients (fifth to seventh decades) . PC corrPC usually presents as a rapidly growing nodular lesion and dermoscopy may be helpful for improving its recognition, even if the diagnosis of malignancy is based on histological examination.Few cases in the literature have been described and the management is not well defined. To better describe the epidemiologic characteristics, clinical features, therapeutic options and follow-up of this rare tumor, we reviewed the literature and reported two additional cases.The aim of this study was to present two cases of PC located on the head and to review the literature evaluating epidemiology , clinical and dermoscopic evaluation, characteristics of local and distant metastases, local recurrence rate and management (follow-up and treatment) of this rare tumor.We found the relevant literature by searching different databases: PubMed, Research Gate and Google Scholar. We used the following combination of key words: “pilomatrix carcinoma”, “pilomatrical carcinoma”, “malignant pilomatricoma”, from January 2012 to November 2022. In total, 48 papers were identified. Inclusion criteria were systematic review or meta-analysis of randomized controlled trials, review, retrospective comparative reviews/studies and case series. Exclusion criteria were laboratory studies and non-English translated articles. A wide review of the bibliography of each of the selected articles was performed. In total, 37 papers met our inclusion criteria, including 1 review ,36,37,38We present two cases of a PC on the head.The first patient, a 54-year-old man, arrived for consultation in our department for a fast-growing asymptomatic nodule localized in the left supraorbital area. Clinical examination revealed a solitary, reddish, firm raised nodule, 12 mm in diameter without locoregional lymphadenopathy a. DermosThe second case concerns an otherwise healthy 65-year-old women with an asymptomatic erythematous, firm, 14 mm in diameter nodule located on the left preauricular area a. DermosIn the last 10 years (from 2012 to 2022), 52 cases of PC have been described in the literature, making this tumor very rare. There was a male predominance with 29 male patients (55.8%) compared to 23 female patients (44.2%) . Age at presentation ranged from 8 months to 87 years . Lesions occurred most in the sixth and seventh decades (52%) .N = 32/52, 62%), followed by the upper extremity  trunk , lower extremity , neck  and genitalia . No patient had multiple lesions . Tumor diameters ranged from 0.5 to 15 cm . Rapid growth, either of new lesion or stable lesion from several months was the most frequent motivation for biopsy and diagnosis .Locoregional or distant metastases occurred in nine patients 17%) [% 7,16,1,22,34. TNew findings showed that metastases occurred in six patients with previously treated PC ,16,18,22Dermoscopic examination was reported in only three patients ,27,32. TAll 52 patients had a histological diagnosis of PC. A first incisional biopsy was performed in five lesions of the head and upper extremities (10%), followed by a wide local excision. Only one patient died before re-excision .In total, a wide local excision was reported for 32 lesions (62%). Mohs micrographic surgery (MMS) was chosen for four tumors ,20,21,23Eckhoff et al. excised the lesion and left the skin defect uncovered pending final pathology, then they re-excised after 12 days to obtain 5 mm margins and subsequently cover the skin with a graft . The patIn total, 13 PCs were simply excised, without reported margins of excision and one case was first excised and then re-excised to obtain 2 mm safety margin after diagnosis . In two Due to lymph node infiltration, one patient experienced a neck dissection ; insteadTwo patients needed chemotherapy (CT) and local cancer therapy ,34 and oRadiochemotherapy was performed in case of extensive local invasion and metastasis: first line CT therapy with 5-Fluorouracil and cisplatin, second line with gemcitabine and taxotere, third line with irinotecan and bevacizumab, fourth line with cyclophosphamide and vinblastine . A summaN = 11/52, 21%), at an average of 6 months after surgery (range 2 to 16 months) [N = 6/52, 6%), one case 2 months after surgery (the other one not reported) and 6 lesions simply excised recurred at an average of 6 months . One patient, with malignant PC with involvement of the base and margins of the specimen, refused radiotherapy post-surgery and presented recurrence and fatal metastasis after 16 months from diagnosis [In total 11 cases of recurrences were observed N = 11/5, 21%, at months) ,24,29,31N = 6/52, 12%) [N = 41/52, 79%) had a regular follow-up (range from one month to eight years), receiving biannual dermatologic visits and in some cases also had a radiological examination. The follow-up imaging procedures used were chest radiography [Follow-up information was not reported in six cases 52, 12%) ,25,32,352, 12% ,2iography ,31, CT siography , 18F-FDGiography , alternaiography , and radiography .One patient was lost to follow-up . Four paCharacteristics of the 52 cases of PC in the recent literature (2012–2022) are reported in In the literature, benign pilomatricoma shows a female predilection and occurs usually in children and young adults with a small peak of onset in the elderly , whereasEven if multiple pilomatricomas have been associated with some particular disease including Turner syndrome, Gardner syndrome, myotonic dystrophy, Steinert disease and sarcoidosis , we did Clinically, PC shares some features with pilomatricoma, in fact it is a firm asymptomatic nodule typically found in the head and neck area, but unlike the benign variant, PC frequently shows a rapid growth ,7. In prDermoscopy evaluation was rarely performed/reported in pre-operative diagnostic settings although it is a useful tool in the context of a rare and difficult to suspect tumor. The most frequent dermoscopic characteristics were purple/blue background with white/yellow blotches, irregularly-shaped telangiectasias, arborized vessels and focal ulceration. White/yellowish blotches can also be found in pilomatricoma because of the presence of calcifications and cornified substance, instead ulceration is rarely found in pilomatricoma and the arborizing vessels have never been observed in this benign tumor . Unlike Wide local excision was the most frequent therapeutic option due to the relative high recurrence rate reported in the literature . RadicalIn previous studies, distant metastases were uncommon and occurred in 10–13% of cases ,40. In tFurthermore, we observed three deaths resulting directly or indirectly from PC invasion.Adjuvant RT was used to treat local recurrence and metastatic disease and it was also recommended due to one close margin, after multidisciplinary discussion.Although some authors considered CT and RT to be not effective in disease control ,41,42, iFollow-up information was not reported in all cases, but the majority of patients received regular clinical examination. There are no well-defined recommendations for follow-up; however, the patients should be closely monitored with ultrasound and other imaging examinations if necessary. The role and the type of follow-up imaging are still unclear, it depends on the patient history and the clinical examination.PC is a rare skin tumor arising from hair follicle matrix cells. It is a lesion with non-specific features and dermoscopy evaluation can be helpful for improving the clinical suspicion. The excision of doubtful nodular lesions is mandatory especially in adults, because PC carcinoma has not only a strong tendency to recur locally, but it can also metastasize. However due to its rarity, there are no well-defined guidelines for the management. A complete surgical excision with clear margins is the best therapeutic option and a regular follow-up is recommended overall.These two case reports and the review of the literature can give a better awareness and management of this rare tumor.


Pathologic fractures commonly occur secondary to abnormal skeletal physiology in the context of benign or malignant lesions. Rarely, pathologic fractures may occur in the context of a lymphatic abnormality, such as generalized lymphangiomatosis. This rare disorder is characterized by variable presentations in a broad age range of patients. By understanding the effect of widespread lymphatic anomalies on various organ systems, clinicians will be able to make this diagnosis earlier and with more certainty. Generalized lymphatic anomaly (GLA), previously known as generalized lymphangiomatosis, is a rare congenital disease characterized by aberrant proliferation of lymphatic vessels in bone and soft tissue, which typically affects children or young adults. Diagnosis can be delayed because of the rarity of the condition, increasing morbidity and mortality. Having high clinical suspicion for GLA in the setting of widespread osteolytic lesions and cystic lymphatic lesions in soft tissue may enable earlier diagnosis and prevent the morbidity and mortality associated with the condition.GLA is a nonneoplastic congenital condition in which lymphatic vessels proliferate in an unregulated manner, causing the formation of dilated and thin-walled cystic structures. Although single organ involvement is possible, the condition more commonly affects multiple organ systems, typically the lungs and bone. GLA most commonly presents in the first two decades of life. The incidence and clinical features of GLA are unknown, challenging even the most seasoned clinicians. A multidisciplinary team of pediatricians, orthopedists, and radiologists is needed to diagnose and manage patients with this disease ,2.A previous case series of 53 patients with GLA demonstrated that 49% presented with pleural effusion, 45% with pulmonary infiltrates, 39% with bone lesions, 19% with splenic lesions, 15% with cervical involvement, 9% with disseminated intravascular coagulation, and 7% with skin involvement [Once patients present with symptoms such as respiratory distress, abdominal pain, or, in this case, pathologic fracture, initial imaging may provide clues toward the diagnosis. Experienced radiologists can identify the classical imaging features, including radiolucent bone lesions on plain radiographic imaging . Other fAn eight-year-old male with a history of attention deficit hyperactivity disorder presented to an orthopedic office with an injury to his right forearm and elbow after falling onto his outstretched arm while dancing. His mother suspected that he had broken his arm due to intractable pain. Subsequent X-ray of the right forearm and right humerus in anteroposterior and lateral views not only confirmed a fracture in the proximal right radial diaphysis but also revealed an underlying lytic lesion in the humerus concerning osteosarcoma. MRI was performed in the outpatient setting to investigate the underlying cause of the pathologic fracture, which showed findings concerning osteosarcoma. The patient was then referred to the emergency department for further workup.In the emergency department, a comprehensive review of symptoms was conducted. Pertinent negatives included a lack of constitutional symptoms  and no respiratory distress, abdominal pain, nausea, vomiting, skin lesions, adenopathy, or easy bruising. The only significant positive finding in the history was arthralgias, not only in the injured arm but also in the left knee. Physical examination demonstrated an active, well-appearing eight-year-old male in no acute distress. His physical examination was only remarkable for diffuse tenderness overlying the patella and throughout the right arm.An X-ray skeletal survey was ordered, which revealed multiple lytic lesions in the right humerus, as seen in Figure Due to the concerning mediastinal mass, CT scans of the abdomen, chest, pelvis, and neck with contrast were conducted. The results of the CT scan in Figure Due to the concerning large mediastinal mass, with possible mass effect on adjacent airways and great vessels, the patient was transferred to the ICU for close monitoring. He remained afebrile and hemodynamically stable, with 100% oxygen saturation on room air. His range of motion, sensation, and strength remained fully intact, and no edema was appreciated on the physical examination. After a couple of uneventful days in the ICU, the decision was made to transfer the patient back to the general pediatric unit.Unfortunately, due to motion, only limited sequences of the chest could be obtained on magnetic resonance imaging. The MRI results seen in Figure Although diagnostic imaging findings strongly suggested the diagnosis of GLA, a definitive diagnosis was obtained via ultrasound-guided percutaneous drainage of the anterior mediastinal mass and CT-guided bone biopsy of a cystic lesion in the pelvic bone. The dominant locule of the cystic mediastinal mass was aspirated with a 5-Fr Yueh needle via ultrasound guidance, yielding approximately 400 mL of serous, non-bloody fluid. The cyst did not reaccumulate fluid post-drainage. Doxycycline sclerotherapy was performed subsequently using 200 mg doxycycline in 10 mL water.Next, a lesion in the left iliac bone was biopsied under ultrasound and CT guidance. The lesion was filled with low-viscosity serosanguinous fluid, which could not be sampled via core needle biopsy. Thus, two cores were sampled from the adjacent bone. The procedure was performed with no complications under general anesthesia.Flow cytometry of the mediastinal cyst fluid revealed no monoclonal B-cell population or phenotypically abnormal T-cell population. Surgical pathology of the bone specimen revealed rare lymphatic channels, with endothelial cells immunopositive for D2-40 and seams of woven bone as opposed to lamellar bone. The histopathology of the bone sample was consistent with GLA. These findings ruled out leukemia, lymphoma, and the more aggressive lymphatic defect known as kaposiform lymphangiomatosis (KLA).Abnormal development of the lymphatic system can manifest as rare benign neoplasms known as lymphangioma. When these become widespread, the condition becomes known as a generalized lymphatic anomaly. Due to the rarity of the condition, the clinical features are not completely characterized, and the cause and prognosis of the disease are poorly understood. Prior case reports in patients with suspected GLA have reported involvement of the bones, mediastinum, spleen, liver, lungs, neck, and pleura. The prevalence and incidence of the disease are likely underreported due to the asymptomatic presentation in many patients. If symptomatic, typical complaints include difficulty breathing, abdominal pain, ascites, and other symptoms due to the compressive effect of the large cystic mediastinal masses . TheoretAlthough a classic lymphangiomatosis patient will present emergently with complaints of respiratory distress and fluid backup, pathologic fractures due to lytic bone lesions are possible. Thus, GLA should be considered in the differential diagnosis in pediatric patients with pathologic fractures. Pathologic fractures in children may result from a wide variety of pathologic processes, including metabolic diseases, infections, and tumors. Various benign bone tumors such as unicameral bone cysts, aneurysmal bone cysts, non-ossifying fibromas, and fibrous dysplasia may weaken the structural integrity of bone and cause fractures. However, malignant processes such as metastases from neuroblastoma and Wilms tumor, leukemia, Ewing’s sarcoma, and fibrosarcoma can predispose to unexpected fractures . Thus, aGLA can be differentiated from KLA and GSD via histopathologic and radiographic findings. Microscopically, GLA is characterized by patterns of anastomotic lymphatic channels with thin walls lined by flattened endothelial cells. However, spindled endothelial cells in the background of the malformed lymphatic channels are more consistent with the diagnosis of KLA. The histological hallmark of KLA is clusters or sheets of hemosiderotic spindled endothelial cells aligned in parallel in the interstitium within the context of abnormal and dilated lymphatic channels . The radClinical features can also be used to distinguish GLA from KLA and GSD. While GLA and KLA tend to have multiple organ involvement  causing pleural and pericardial effusion, ascites, and lymphedema, GSD causes discrete osteolysis of any bone that inevitably progresses, leading to the catastrophic destruction of the bone. Thus, pathologic fracture is much more common in GSD but cannot exclude GLA. However, other findings such as joint abnormalities, leg length discrepancy, asymmetric girth, and scoliosis are far more suggestive of GSD than GLA . FurtherFindings that may raise suspicion for KLA as opposed to GLA include the presence of hemorrhagic effusion, extensive retroperitoneal involvement, a deteriorating clinical course, or associated hematologic abnormalities. Most patients with KLA present in childhood with respiratory or bleeding concerns due to thrombocytopenia, hypofibrinogenemia, and prolonged PT/aPTT associated with the condition. The common manifestations of pathologic bleeding in KLA patients include epistaxis, scleral hemorrhage, ecchymosis, vaginal bleeding, epidural hematoma, and hemorrhagic effusions. Video-assisted thoracoscopy of KLA patients demonstrated hemorrhagic plaques on the visceral pleura associated with dilated tortuous blood vessels. Ultimately, a biopsy is necessary for differentiating these different clinical entities .Treatment in patients with GLA is mostly symptom-based. For example, for the patient described in this report, sclerotherapy was used to prevent refilling of the mediastinal mass, which could have led to respiratory distress, ascites, and obstructive shock by compressing the vena cava. The sclerotherapy may even reduce the chances of developing pleural and pericardial effusions. If these types of effusions did develop, pleural fluid drainage or pleurodesis would be warranted. Other options for palliative care include radiotherapy, interferon-alpha, steroids, and other chemotherapeutic drugs . Beta-blGLA is a potentially life-threatening congenital disease characterized by the formation of aberrant lymphatic channels, which are widely distributed in bone and soft tissue throughout the body. Due to its rare nature and the severity of its prognosis, practitioners should be familiar with its presentation and characteristic findings. Early diagnosis and treatment may improve outcomes for these patients. However, more research is needed to optimize therapeutic interventions for these patients.


Cardiac hemangiomas are very rare benign cardiac tumors. They can present at any age and clinical presentation varies according to location and size. We encountered an 87-year-old woman with a left atrial hemangioma clinically diagnosed as cardiac myxoma. Histopathological examination revealed that it was a cavernous-type hemangioma Left atrial hemangiomas, especially those attached to the left atrium wall, may be mistakenly diagnosed as myxomas. Furthermore, a comprehensive review of atrial hemangioma was conducted for the diagnosis and treatment of this uncommon entity. Cardiac hemangiomas (CH) are rare primary tumors of the heart and constitute only 2.8% of primary cardiac tumors . MicroscAn 87-year-old female was referred to the hospital with complaints of dyspnea, dizziness and weakness for the past twelve months. Symptoms had deteriorated three weeks prior to her visit in the hospital. Physical examination revealed arterial blood pressure 100/80 mmHg, heart rate 120 bpm/min, mild jugular regurgitation, and regular cardiac rhythm without murmurs.Her past history was unremarkable. A transthoracic echocardiogram was performed, which revealed an isoechogenic mass 2.1 × 1.5 cm) located in the left atrium. There was no pericardial effusion , meticulous surgical resection or autopsy , 4. HistAsymptomatic CHs are also discovered during evaluation of other heart conditions and predominately showing insight to other diagnosis . PapillaCardiac hemangioma is a rare benign slow growing tumor that occurs in the middle-aged and elderly population. Echocardiography is an accurate, noninvasive method that could contribute to the diagnosis of this disease; however, the final diagnosis still depends on histopathologic examination. Surgical resection of the tumor is the treatment of choice and the prognosis is usually favorable. In our case the patient had a satisfactory outcome after the resection and is under follow-up for the possibility of recurrence.


Retained surgical foreign bodies have been a cause of concern since physicians began operating on patients. Retained surgical foreign bodies in the common bile duct (CBD) are rare and may cause cholangitis and jaundice. We report the case of a patient who initially presented with fever and right upper-quadrant abdominal pain. He had received cholecystectomy and choledochojejunostomy 28 years ago and had been well since then. Abdominal computed tomography (CT) revealed left-lobe liver abscess and a linear curve of high-density material. Endoscopic retrograde cholangiopancreatography (ERCP) displayed mild dilatation of the common bile duct (CBD) and choledojejunostomic fistula of the middle CBD. A curved, linear, rusty, metallic surgical suture needle was detected and successfully removed under ERCP.


Gynecologic emergencies may result from congenital uterine anomalies (CUAs) with outflow tract obstruction. Not limited to the “classic” presentation of an adolescent amenorrheic pain patient, such anomalies should be part of the differential diagnosis for adult female patients presenting with severe pelvic pain. Obstructed rudimentary noncommunicating cavitary horns may result in severe chronic or acute pain and necessitate urgent surgical management. While two-dimensional (2D) ultrasound is often the initial diagnostic tool, three-dimensional (3D) ultrasound and MRI can accurately delineate CUAs for definitive diagnosis. When excision of a rudimentary horn is required, a laparoscopic approach is preferable. This case series focuses on two adult patients with severe pelvic pain due to unicornuate uteruses with obstructed noncommunicating cavitated rudimentary horns. Both cases involve a delayed diagnosis, the inability to make the diagnosis at standard surgical observation, and the resultant need for urgent surgical management. Gynecologic emergencies are conditions that threaten a woman's life, sexual function, or fertility . GynecolGynecologic emergencies related to CUAs typically result from either ectopic pregnancy or outflow tract obstruction. However, outflow tract obstruction is not generally included in the differential diagnosis of acute pelvic pain in adults, as the classic presentation is an adolescent with primary amenorrhea and cyclic abdominal pain. However, CUAs may present with acute abdominal pain in adults in the setting of outflow tract obstruction.While patients with CUAs may present with pelvic pain, abnormal uterine bleeding, or genital tract infection, many patients with CUAs are asymptomatic, and diagnosis often occurs during physical exam or as an incidental finding on imaging study. On physical exam, patients may have a double or no cervix, vaginal septum, or a shortened or absent vagina. Incidental diagnosis is often made at the time of pelvic ultrasound, magnetic resonance imaging (MRI), CT scan, or hysterosalpingogram. The accepted imaging standard for Müllerian anomalies is 3D ultrasound.A unicornuate uterus includes a single normally developed cervix, uterus, and fallopian tube. The Müllerian duct that failed to develop properly may be completely absent or a rudimentary horn may exist. The frequency of unicornuate uterus is 10% of all CUAs . UnicornThere is no universally accepted classification system for CUAs. The European Society of Human Reproduction and Embryology (ESHRE) and the European Society of Gynecological Endoscopy (ESGE) , the AmeRudimentary horns can be described as follows: (1) horns with or without an endometrial cavity; (2) horns with or without a communicating tract between the cavities of the rudimentary horn and unicornuate uterus; (3) horns fused or separate from the unicornuate uterus . Cavitarobstructed noncommunicating cavitated rudimentary horns resulting in urgent surgical management in the adult female patient.Although there are several published case reports of the management of unicornuate uteruses with rudimentary horn , 12, 13,Here, we present two adult patients, both with pain due to unicornuate uteruses with obstructed noncommunicating cavitated rudimentary horns, ESHRE/ESGE class U4aC0  5]. Bot. Bot5]. 2 was evaluated in the out-patient setting for an acute worsening of chronic pelvic pain at the Cleveland Clinic Fertility Center in Beachwood, Ohio. At the age of sixteen, she began to experience pelvic pain that was managed intermittently with menstrual suppression via medroxyprogesterone acetate injections.A 29-year-old gravida one para one female with essential hypertension and a body mass index (BMI) of 60.7 kg/mNine years prior to her presentation, the patient conceived a child and had an early term cesarean delivery for oligohydramnios, intrauterine growth restriction (IUGR), and breech presentation. The operative report noted an 8 cm right fundal fibroid with no uterine abnormalities noted. Five years prior to her presentation, she had a laparoscopic left ovarian cystectomy for a mature teratoma; that operative report noted a 5 cm mass coming off the right fundal aspect of the uterus with “chocolate” fluid contents. Despite this finding, no definite treatment was undertaken, and the patient continued to have chronic pelvic pain.Six months prior to presentation, the patient stopped treatment with medroxyprogesterone acetate and began to experience intractable pain despite multiple regimens of NSAIDs and narcotics. In the month prior to evaluation in our clinic, she had an acute worsening of pelvic pain, which resulted in five emergency department (ED) visits and two in-patient admissions for pain control. During this time, she was noted to have leukocytosis with a left shift.During these ED encounters, the rudimentary horn was read as a fibroid with central necrosis on ultrasound , and anaFollowing induction of anesthesia and abdominal entry, the robot was side-docked to allow easy access to the vagina, with camera port at the umbilicus, three additional robotic ports in left and right lower quadrants, and an assistant port in right lower quadrant.The procedure began with a survey of the pelvis for endometriosis, which revealed no macroscopic evidence of endometriosis. The rudimentary horn's fallopian tube was attenuated and did not appear to be a functional outlet for the horn. The rudimentary horn was grossly distended with blood to approximately 9 cm . The bilThe hemi-hysterectomy of the rudimentary horn was performed incorporating aspects of both laparoscopic hysterectomy and laparoscopic myomectomy. The surgery began with identification of the ipsilateral ureter, opening of the broad ligament, and use of the da Vinci vessel-sealer to coagulate and transect the round ligament, the utero-ovarian ligament, and the uterine vessels, particularly the ascending branch of the uterine artery. A laparoscopic tenaculum in the third robotic arm was used for retraction and manipulation of the rudimentary horn. Adhesions of the bladder to the anterior aspect of the rudimentary horn were taken down, and the area of fusion between the rudimentary horn and unicornuate uterus was injected with dilute vasopressin to minimize blood loss during incision into the myometrium. A plane was developed between the unicornuate uterus and the rudimentary horn, and the rudimentary horn was removed along with the ipsilateral fallopian tube. The excised horn was moved out of the field to allow for visualization and closure of the myometrial defect using a 0-barbed suture in three layers. The umbilical incision was extended to 4 cm, and the excised horn and right fallopian tube were placed in a specimen bag and removed from the abdomen. At the conclusion of the procedure, the pelvis was irrigated, and all pedicles were hemostatic. Pathology was consistent with benign uterine tissue, and there was no evidence of microscopic endometriosis.At a follow-up appointment, four months after surgery, the patient's pelvic pain had resolved. However, the patient reported ongoing struggle with a dependence on opioids prescribed during the months-long episode of severe pain prior to her diagnosis and treatment.2 was evaluated in the out-patient setting for infertility. Her care was transferred to at the Cleveland Clinic Fertility Center in Beachwood, Ohio from another IVF center, where she had undergone one in vitro fertilization (IVF) cycle, notable for an inaccessible left ovary at oocyte retrieval and no resulting embryo viable for transfer.A 40-year-old nulligravida with BMI 36 kg/mThe patient's medical history was notable for vulvodynia (with inability to have coitus), endometriosis, obstructive sleep apnea, hypertension, and anxiety. Due to her vulvodynia, the patient was unable to tolerate vaginal exams or procedures without anesthesia. Hence, two attempts at hysterosalpingo-contrast sonography failed. She was diagnosed with a “bicornuate uterus” via transabdominal ultrasound. In addition to making this diagnosis, the previous IVF center performed a laparoscopic left salpingectomy for hydrosalpinx, excision of endometriosis, and left oophoropexy to improve access to the left ovary for future oocyte retrieval procedures.Upon transfer of care to our institution, the patient underwent a second cycle of IVF, resulting in successful oocyte retrieval from both ovaries and one resultant euploid embryo, with evidence of an endometrial polyp seen during egg retrieval. Hysteroscopic polypectomy was performed in preparation for embryo transfer. During exam under anesthesia, a hypoplastic upper portion of the vagina was noted. Only a single cervix, uterine cavity, and tubal ostium were noted. No tract to a second cavity was identified, regardless of multiple surveys of the lower uterine segment and cervical canal, which was inconsistent with her diagnosis of bicornuate uterus. An MRI with contrast was ordered for definitive diagnosis . The MRIIt was presumed that the patient's recent left salpingectomy had resulted in outlet obstruction of the rudimentary horn. She was scheduled for a robotic-assisted laparoscopic hemi-hysterectomy of the rudimentary uterine horn, which was scheduled six weeks later as an elective procedure given the absence of any symptoms. Over the next two weeks, the patient experienced the onset of severe pelvic pain and presented to the emergency department twice for pain control. Due to the severity of her symptoms, she was scheduled for urgent surgical management. Due to lack of availability of the da Vinci robot, we proceeded with a conventional laparoscopic hemi-hysterectomy.The camera port was placed at the umbilicus. A laparoscopic port was placed in the left and right lower quadrants. A 5 cm transverse minilaparotomy was made two fingerbreadths above the pubic symphysis. The GelPort laparoscopic system was used at the site of the minilaparotomy to allow swift alternate access between laparotomy and laparoscopic techniques to improve surgical efficiency and outcomes .On survey of the abdomen and pelvis, the left ovary and rudimentary horn were densely adherent to one another and the left pelvic side wall. There was no macroscopic evidence of endometriosis. The rudimentary horn was noted to be distended with blood . The lefCareful attention was paid to lyse adhesions between the rudimentary horn, left ovary, and pelvic side wall along avascular planes to ensure minimal bleeding. Traction and countertraction were used to create tension to allow for clearer identification of avascular planes. Ureterolysis was performed in the usual fashion. The left ureter was noted to be adherent to the left ovary and rudimentary horn. Blunt dissection was used to lyse fine adhesions, while sharp dissection was used for dense adhesions, using both laparoscopic scissors and a bipolar vessel sealing (LigaSure) device.Once adhesiolysis was complete, the steps of a hemi-hysterectomy were initiated. The connecting area between the rudimentary horn and unicornuate uterus was injected with dilute vasopressin to minimize blood loss. The hemi-hysterectomy was then performed using the laparoscopic LigaSure device to open the broad ligament and to coagulate and transect the round ligament, the utero-ovarian ligament, and the uterine vessels, as before. Adhesions of the bladder to the anterior aspect of the rudimentary horn were taken down. A plane was carefully developed between the firm attachment of the unicornuate uterus and the rudimentary horn. The rudimentary horn was then excised and moved out of the field to allow for visualization and closure of the myometrial defect. A 0-barbed suture was used to close the small and shallow myometrial defect in a single layer. The excised horn was removed via the minilaparotomy incision. Cystoscopy was performed and no injury to the bladder was noted; bilateral ureteral jets were appreciated. At the conclusion of the procedure, the pelvis was irrigated, and the entire pelvis was noted to be hemostatic .Pathology was consistent with endometriosis and 45.9 g of benign uterine tissue, measuring 4.7 x 4.7 x 3.7 cm. At a follow-up appointment, two weeks after surgery, the patient's pelvic pain had resolved. Table. These cases demonstrate the importance and difficulties in obtaining a definitive diagnosis as well as the negative implications of perpetuating a false diagnosis. The diagnosis of unicornuate uterus can be difficult, even with direct visualization during laparotomy (Case 1) and laparoscopy (Cases 1 and 2). It is important to note that the reported prevalence of CUAs is 5.5% in an unselected population, 8% in infertile patients, 12.3% in patients with a history of miscarriage, 24.5% in patients with a history of infertility and miscarriage, and 29% in patients with renal anomalies , 16. CUAThe gynecologic emergencies presented here could have been avoided if these patients received an accurate diagnosis sooner. In Case 1, the rudimentary horn was previously believed to be a subserosal degenerative fibroid. In Case 2, the rudimentary horn was believed to be a bicornuate uterine horn. Physicians should be aware that the misclassification of CUAs can be perpetuated in subsequent patient records, even between practices. Here, misdiagnoses led to severe chronic and acute pelvic pain which both culminated in a surgical emergency. Both patients had a history of prior surgical procedures; if the rudimentary horn had been previously or presently diagnosed, a hemi-hysterectomy could have been performed at that time, eliminating the need for additional surgery in an urgent setting.2D ultrasound is the initial imaging modality for evaluation of CUAs; this is due to ultrasound's availability, relative cost efficiency, noninvasive nature, and ability to provide information about other pelvic structures and kidneys . UnfortuChromopertubation is an important part of the intraoperative investigation, as it may be used to determine whether one or both uteruses communicate with the cervix. In addition, thorough examination of the vagina and cervix under anesthesia is critical to ensure that the presence of a duplicated cervix is not missed.2, superior ergonomics, reduced surgeon fatigue, and improved trochar movement due to instrument wrist articulation in the setting of a thick abdominal wall [Patients with a high BMI present unique surgical challenges. These challenges often lend themselves to a robotic-assisted or conventional laparoscopic approach. For obese patients, assistance of the robot provides several advantages, such as reduced operating time in patients with a BMI ≥ 50 kg/mnal wall . Due to For patients desiring fertility, the decision to attempt conception immediately versus waiting should depend on the extent of the defect and repair of the myometrial bed. For patients with a separate rudimentary horn from the unicornuate uterus, the patient may attempt conception once they are fully recovered from surgery. For patients with a fused rudimentary horn, the decision to delay conception should be individualized. For extensive myometrial defects and repair, we recommend following similar guidelines as patients who undergo myomectomy .Patients should be counseled regarding the obstetric risks in the setting of a unicornuate uterus. CUAs can lead to a multitude of obstetric complications including spontaneous abortion and recurrent miscarriage, preterm rupture of membranes and birth, abnormal placentation, IUGR, cervical insufficiency, fetal malpresentation, hypertension disorders, cesarean birth, bleeding, and rupture of a rudimentary horn. The patient in Case 1 is an excellent example of obstetric complications in the setting of unicornuate uterus: placental insufficiency, IUGR, fetal breech presentation, and cesarean birth.Obstructed rudimentary noncommunicating cavitary horns may result in severe chronic or acute pain and necessitate urgent surgical management. Timely diagnosis is critical. Outflow tract obstruction of a CUA should be part of the differential diagnosis for adults presenting with pelvic pain. 3D ultrasound and MRI can accurately diagnose CUAs. Nondefinitive imaging and/or the inability to make the diagnosis at standard surgical observation may delay the diagnosis of a CUA and can eventually require urgent surgical management. When excision of a rudimentary horn is required, it should be removed laparoscopically. A robotic-assisted approach facilitates minimally invasive surgery in patients with a high BMI.


Angina bullosa hemorrhagica (ABH) is a condition of the oral mucous membrane, characterized by the sudden appearance of blood-filled blister(s) within the oral cavity. In the majority of cases, these blisters occur on the oropharynx or palate. The blisters usually rupture in a day or two and heal spontaneously without any further scarring or discomfort. In rare cases, if a large lesion located in the throat does not rupture spontaneously, it may lead to airway obstruction. We present the case of a 64-year-old-female who presented with a recurrent manifestation of well-defined oral blood-filled blisters which ruptured and healed spontaneously. There were no identifiable risk factors. Angina bullosa hemorrhagica was diagnosed clinically. The main objective of this case report is to bring awareness and avoid unnecessary investigations and misdiagnosis. Angina bullosa hemorrhagica (ABH) is a rare disorder characterized by recurrent occurrences of blood-filled vesicles and bullae in the oral cavity, which are not caused by blood dyscrasia, immunobullous disorders, systemic diseases, or other causes . The etiA 64-year-old-female presented to the hospital with a history of sudden onset of blister over the tongue. A well-defined hemorrhagic bulla of about 2 cm in diameter was found on the right lateral aspect of the tongue Figure . She recABH was first described by Badham in 1967 as blood-filled blisters in the oral, pharyngeal, and esophageal mucosa . ABH is In our case, the patient reported no risk factors. She denied a history of any vascular lesion or hemangioma in the past. Laboratory workup of blood counts, platelets, and coagulation panel was in the normal range. Biopsy was not necessary in our case as a confident diagnosis of ABH was clinically obvious after ruling out other hematological and mucosal diseases based on routine investigations work-up. Disorders of collagen and elastic fibers in the oral mucosa can reduce the anchoring of the blood vessels, causing hemorrhagic lesions after trauma leading to these pathologic conditions in patients . The preA few differential diagnoses like bullous pemphigoid, pemphigoid, bullous lichen planus, dermatitis herpetiformis, epidermolysis bullosa, oral amyloidosis, and thrombocytopenia need to be considered . Angina The following nine diagnostic criteria for ABH were introduced by Ordioni et al. Table  12]..12].In this case, six out of nine (including main criteria A and B) of the above-mentioned diagnostic criteria were met by the patient. In general, no active treatment is needed as it can heal spontaneously. If the ABH has affected the soft palate and the blister has ruptured, antibiotic prophylaxis and antiseptic rinses are recommended, such as chlorhexidine digluconate at a concentration of 0.25% to 0.12%, to help prevent secondary infections . An intaSince this is a rare disorder, many are unaware of this condition, which results in unnecessary investigations and misdiagnosis. ABH can be diagnosed confidently by its classic clinical presentation after ruling out other causes of blood-filled bullous lesions of the oral cavity. This case report creates awareness about ABH among the medical fraternity. Simple reassurance and counseling of the patient is sufficient to manage this condition.


Intradural disc herniation (IDH) can manifest with radicular or medullary syndrome. In about 15% of cases, IDH may be responsible, through a dural laceration, for a CSF leak, determining spontaneous intracranial hypotension (SIH) and CNS superficial siderosis (CNSss). This paper attempts to present an overview on IDH as the cause for both CSF leak, and subsequent SIH, and CNSss, and to describe a peculiar clinical and neuroradiological scenario related to this condition.A search on the PUBMED database was performed. Although the investigation did not rigorously follow the criteria for a systematic review (we consider only articles about thoracic IDH), nonetheless, the best quality evidence was pursued. Furthermore, an illustrative case was presented.A 69-year-old woman was referred to our hospital for slowly progressive gait disturbances and hearing impairment. Brain imaging revealed diffuse bilateral supratentorial and infratentorial superficial siderosis, mostly of the cerebellum, the eighth cranial nerves, and the brainstem. Spinal imaging disclosed a posterior disc herniation determining a dural tear at D6-D7. Lumbar puncture revealed low opening pressure and hemorrhagic CSF with siderophages. A posterior transdural herniectomy and dural sealing determined a stabilization of hearing and a significant improvement in both gait and balance.The diagnostic workup of CNSss with suspected CNS leak demands whole neuraxis imaging, especially in cases presenting SIH or myelopathic symptoms. This may avoid delays in detection of IDH and spinal dural leaks. The different forms of treatment available depend on the type and severity of the clinical picture. Intradural disc herniation (IDH) consists of a nucleus pulposus fragment of the intervertebral disc violating the dural sheath and penetrating the thecal space. The first report of an IDH was made by Dandy in 1942 . By contIn this paper, we review the literature concerning thoracic IDH as the cause for both CSF leak, and subsequent SIH, and CNSss. We also present a case exposing the peculiar clinical picture, discussing the possible underlying pathophysiological mechanisms and examining the therapeutic strategies that were implemented.Following a brief overview about the rationale, indications, pathophysiology, and results of the implemented surgical treatments, we will discuss the implications according to the available literature. With this aim, we performed a search on the PUBMED database for the following: thoracic intradural disc herniation, spontaneous intracranial hypotension, central nervous system superficial siderosis, and cerebrospinal fluid leak. The search was conducted on the available literature before December 2021, comprising only articles with full text in English, without any historical limitation. The literature search did not strictly follow the criteria for a systematic review because we consider only articles about thoracic intradural disc herniation. Nevertheless, we tried to identify the highest quality of available evidence for each technique implemented in the case of thoracic intradural disc herniation. Considering the extreme heterogeneity and the limited number of articles, we present the data as a comprehensive and narrative review. Moreover, we have included the experience of our Institution with an illustrative case. All the reported techniques are available and used at our Institution. All MRI and CT scan images included in this review were evaluated by members of the Neuroradiology Unit. IDH, SIH, and CNSss were diagnosed according to pre-specified clinical and radiological criteria.We present the case of a 69-year-old, right-handed, woman who suffered from a 1-year history of slowly progressive gait disturbances and hearing impairment. Neurological examination revealed an ataxic gait and bilateral sensorineural auditory alteration. No previous history of surgery or trauma of the CNS were present, and no major comorbidities were reported. Therefore, the patient underwent brain magnetic resonance imaging (MRI) Fig.  A muscleIn 2014, Toro et al. described the case of a patient with a 4-year history of leg weakness and progressive gait impairment, besides other symptoms such as bilateral hearing loss and urinary incontinence. T2-weighted brain MRI showed extensive hemosiderin deposits around the brainstem and along the cerebellar folia, while spinal MRI demonstrated a disc herniation at the T8-T9 level, located where a previous dynamic CT myelogram highlighted a ventral dural defect. A surgical dural repair was offered but declined by the patient . ReviewiIn 2018, Wipplinger et al. described a case of CNSss secondary to a thoracic IDH causing CSF leak. Their patient was successfully treated with lateral T6-T8 transpedicular partial corpectomy, as well as diskectomy with decompression and fusion, followed by watertight closure of the CSF leak. No postoperative complications occurred, and, at 3 months’ follow-up, the patient displayed stability of preoperative symptoms, such as mild bilateral hypoacusis and mild difficulty in tandem gait .In 2020, Cornips et al. presented two patients with a thoracic IDH, secondary SIH and, in one of them, CNSss . The firThe migration of a disc into the intradural region requires perforation of the annulus fibrosus, and then the laceration of both the posterior longitudinal ligament and the dura mater. Various predisposing anatomical factors have been proposed, of which the most relevant one is the presence of adhesions between the annulus fibrosus, the posterior longitudinal ligament, and the dura mater. These adhesions are likely to be congenitally present or occur secondary to chronic disc protrusion . In thisMRI yields pathognomonic findings, which may be revealed in the presymptomatic phase of the disease. They result from the deposits of hemosiderin, ferritin, and ionic iron. In gradient-echo T2-weighted images, the hallmark is a dark band surrounding the intracranial structures, such as the cerebellar hemispheres and the VIII cranial nerve, while other cranial nerves are less frequently affected. On the other hand, CT imaging is not sufficient for the diagnosis of CNSss .The main differential diagnosis of CNSss are neoplasms (21%), cranial or spine trauma (13%), vascular malformations (9%), neurosurgical procedures (7%), and cerebral amyloid angiopathy (3%). Nevertheless, most cases remain idiopathic (35%) .The association between CSF leak and CNSss has been explained by two theories. The first theory states that brain sagging due to intracranial hypotension may lead to bleeding from bridging veins on the surface of the cerebellum . While tPreoperative imaging-based diagnosis of thoracic IDH is challenging. Considering the literature –29, in oSurgical treatment of thoracic IDH is needed in the presence of radicular symptoms, myelopathy clinical and radiological signs (highlighted by a medullary T2 hyperintensity area on spine MRI), and symptomatic SIH . In our In our case, the clinical picture was mainly ascribed to CNSss, which, undoubtedly, set the indication to invasive treatment as to resolve the CSF fistula. Given the absence of myelopathy and significant SIH, our surgical strategy was primarily based solely on repairing the ventral dural defect to handle the progression of frank CNSss. Therefore, we proceeded with a posterior transdural herniectomy through a monolateral laminectomy and the placement of muscle and fibrin glue to repair the anterior dural fistula, rather than performing a more invasive total microdiscectomy.In the case of clinal and radiological signs of CNSss and/or SIH associated with suspected CSF leak, diagnostic workup should include MRI of the whole spine, especially in patients with myelopathic symptoms that could be ascribed to IDH. This may avoid delays in detection and treatment of spinal dural CSF leaks. With no apparent myelopathy, a less demolitive surgery is recommended for IDH, proceeding with the repair of the fistula with sealants and the excision of the intradural herniated disc material.


Testicular cancer is common but curable when diagnosed early. Testicular cancer is often characterized by a painless unilateral testicular mass discovered incidentally. In rare cases, testicular cancer is manifested as testicular atrophy. This case study concerns a 44-year-old patient diagnosed with right testicular seminoma complicated by infertility with bilateral testicular atrophy. In countries where sperm cryopreservation is not feasible for everyone, early detection of testicular atrophy by transscrotal ultrasound could prove effective for rapid intervention to preserve patient fertility in those with asymptomatic intratesticular cancer. Testicular cancer is the most common malignancy in men aged 15-45 years. With a cure rate of approximately 90% and a five-year survival rate of over 95%, it is one of the most common curable malignancies when identified early and treated appropriately . TesticuA 44-year-old man presented for infertility assessment after five years of marriage. He had no medical history apart from a notion of low testicular volume he has had since adolescence. On physical examination, we noted painless and hypotrophic testicles palpated in the bursa, with no evidence of mass or swelling. Our initial diagnosis was bilateral testicular atrophy associated with male infertility of idiopathic etiology.Semen analysis revealed necrozoospermia and oligozoospermia. Hormone levels showed an increase in follicle-stimulating hormone and luteinizing hormone. Tumor markers revealed increased lactate dehydrogenase (LDH) levels. The patient's beta-human chorionic gonadotropin (B-HCG) and alpha-fetoprotein (AFP) were within reference ranges.Inguinoscrotal ultrasound revealed bilateral testicular atrophy with two homogeneous right intratesticular oval nodules. The nodules were hypoechoic, well-circumscribed, and vascularized and measured 7.1 mm and 5.7 mm, respectively Figures . A compuTesticular cancer most commonly presents as a painless testicular mass or nodule. Upon physical examination, this mass or nodule cannot be separated from the testicle. Patients with atrophic testicles generally experience an increase in the volume of the testicle . Our patUltrasound has a sensitivity of 92-98% and a specificity of 95-99.8% for testicular malignancies. Its sensitivity is approximately 100% when combined with physical examination [The possible causes of bilateral testicular atrophy are numerous, and, according to the American Cancer Society, testicular cancers rarely lead to testicular atrophy ,6. TestiThe diagnosis of seminoma was made according to ultrasound findings and tumor markers; the latter revealed an increase in LDH levels, but B-HCG and AFP were within reference ranges. The markers are essential for the screening and management of testicular cancers. LDH-1 is expressed on chromosome 12p, which is overexpressed in germ cell tumors. Analysis of AFP and B-HCG is seen in many cases of germ cell tumors . ChoriocThe patient's history revealed low testicular volume, observed since puberty. Upon reviewing the literature, germ cell tumors develop following an in utero oncogenic event inducing intratubular germ cell neoplasia . IntratuSeminoma was suspected to be the primary cause of infertility for our patient. An undiagnosed infertility history can also be the cause of the formation of testicular cancer. Walsh et al. reported that men with male factor infertility are three times more likely to develop testicular cancer . IntratuBecause his diagnosis was stage 1 seminoma, we managed the patient with unilateral orchiectomy without adjuvant therapy. Although 15-18% of patients with a stage 1 tumor relapse after orchiectomy without adjuvant therapy . ApproxiWhile many patients will have sperm recovery after chemotherapy or radiotherapy, predicting which patients will have a return of spermatogenesis is a challenge . Thus, pWe present a rare right testicular seminoma case complicated by infertility with bilateral testicular atrophy. The patient's condition was treated with unilateral orchiectomy without adjuvant therapy. This case highlights that early detection of testicular atrophy by transscrotal ultrasound could prove effective for rapid intervention to preserve patient fertility in those with asymptomatic intratesticular cancer.


Monochorionic dizygotic twins are a rare condition, mostly related to assisted reproductive technology. This type of twinning is burdened by the same risk of pregnancy complications found in monochorionic monozygotic pregnancies.We report a case of spontaneous monochorionic dizygotic twins sharing situs inversus abdominalis and isolated levocardia, with only one twin affected by biliary atresia with splenic malformation syndrome. We also conducted a literature review of the 14 available documented monochorionic dizygotic twin gestations spontaneously conceived.It is still unclear how this unusual type of twinning can occur in spontaneous conception. The evidence so far suggest the importance to timely diagnose the chorionicity, in order to adequately manage the typical complications associated with monochorionicity.The online version contains supplementary material available at 10.1186/s12884-022-04866-x. Monochorionic dizygotic (MCDZ) twins are a rare condition, mostly related to assisted reproductive technology (ART) . Here weWe reviewed all cases of spontaneous monochorionic dizygotic twins reported in literature, highlighting the clinical features, the obstetrical implications and the challenges related to this unusual and still not fully known twinning event.A 27-year-old woman, gravida 2, para 0, with no history of previous disease and no family history of congenital anomalies, smoker and with a BMI of 18.3 received a diagnosis of spontaneous twin pregnancy at 12 weeks. The assessment of chorionicity in this gestational age was however hampered by the presence of an hematoma measuring 44 × 38 mm separating the two membranes, thus preventing the identification of the “lambda” or “T” sign.The anatomy scan at 20 weeks revealed two female fetuses, both presenting isolated levocardia (IL) with normal heart and situs inversus abdominalis with a left-sided liver and right-sided stomach and spleen Fig. . NoteworAn amniocentesis was performed, with conventional cytogenetic evaluation indicating normal karyotype 46XX for both fetuses. No genetic anomaly was reported with the Chromosomal Microarray Analysis (CMA). Zygosity was assessed by microsatellite analysis; as shown in Table At 36 weeks one fetus was diagnosed to be growth restricted, having an abdominal circumference and an estimated fetal weight less than 3rd centile. At 37 weeks and 1 day, the pulsatility index (PI) of the umbilical artery of the growth restricted fetus, with an estimated fetal weight of 2171 g , was 0.89 (corresponding to 46° centile), while the PI of the middle cerebral artery was 1.45 (26° centile). By contrast, the other twin had an estimated fetal weight of 2521 g with normal Doppler parameters. At 37 weeks and 3 days the mother underwent a scheduled cesarean delivery. The birthweight of the two female neonates were 2430 g and 2185 g.The histological analysis of the placenta confirmed the monochorionicity of the twin pregnancy, revealing the juxtaposition of an amnion on each surface of the dividing membrane , no pathogenic or probably pathogenetic variants have been identified. By opening the analysis to the whole clinical exome (4490 genes), the twins did not share pathogenic or probably pathogenetic variants. In addition, the twin whose gallbladder was not visualized prenatally, developed jaundice with acholic stool in her neonatal period.The complete abdominal scan performed at 20 days of extrauterine life showed a left sided, damage-free liver with regular size , a reversed relation between superior mesenteric vein and artery and right-placed inferior vena cava in relation to the aorta. These findings perfectly fit with abdominalis situs inversus with isolated levocardia. The scan revealed for the first time a shriveled gallbladder, which led to the diagnosis of biliary atresia type IV associated with splenic malformations syndrome (Fig. The twin with BASM at 38 days of extrauterine life underwent Kasai portoenterostomy, second-hand appendectomy and Ladd bridle dissection. Later, during the fifth month of extrauterine life, the baby was diagnosed to have a subclinical acute cholangitis, which was treated with continuous infusion of piperacillin tazobactam during hospitalization. Unfortunately, the baby had recurrent cholangitis during the whole first year of extrauterine life, and these are still occurring.A systematic review was conducted using Pubmed, Scopus, OVID, and Cochrane Library electronic databases. The citations were identified with the use of a combination of the following key words: “monochorionic dizygotic twins”; “monochorionic dizygotic chimerism”; “monochorionic dizygotic freemartinism”; “spontaneous monochorionic dizygotic twins”; “spontaneous monochorionic heterosexual twins” from the inception of each database through January 2022. Only articles in English language were selected, while no restrictions for geographic location were applied .Overall 14 cases of spontaneously conceived and 4 cases of monochorionic dizygotic twins conceived after ovulation induction were identified. All available articles were case reports. A description of each case is provided in two tables: Table In conclusion, spontaneously conceived MC-DZ twins are a rare condition, with only 14 cases described in literature. The evidence so far suggests the importance to timely diagnose the chorionicity, in order to adequately manage the typical complications associated with monochorionicity. Furthermore, the clinician should keep in mind that monochorionicity does not always correspond to monozygosity. Rarely, MC twins could be DZ, even in naturally conceived pregnancies, as shown in this comprehensive review. It is still unclear how this unusual type of twinning can occur in spontaneous conception. The mystery deepens considering the peculiarity of our case report, where the two MCDZ twins share a very rare anomaly, for which no genetic cause has been found through clinical exome analysis.Additional file 1: Supplementary figure S1.  Flow diagram of inclusion of articles.