Chondromesenchymal hamartoma of the chest wall is a rare, benign disease that usually presents at birth or in early infancy. It typically involves one or more ribs, forming a unilateral or bilateral extrapleural mass. Patients may be asymptomatic or complain of mild respiratory distress depending on tumor size and location. To the best of our knowledge, only two of the approximately 100 cases reported so far are adults.en bloc resection of the tumor and the involved rib segments, the patient was transferred to the intensive care unit for treatment of pulmonary infection and disseminated intravascular coagulation (DIC). He was discharged from the hospital in stable condition 11 days later. On histopathology, the tumor was found to be a chondromesenchymal hamartoma with immature spindle-shaped mesenchymal cells, plate-like hyaline cartilage, areas of woven bone formation, endochondral ossification and calcification, osteoclastic giant cells, and secondary aneurysmal bone cysts.We present two cases of chondromesenchymal hamartoma. The first case involved the left fifth rib in a 24-year-old male, in close proximity to the fifth vertebral body in the left posterior mediastinum, mimicking a posterior mediastinal tumor on imaging. The tumor was excised via thoracoscopy and the patient had an uneventful postoperative course. The second case was that of a 5-month-old boy, who had a tumor involving the left fifth and sixth ribs which caused thoracic cage collapse. Following Although the presently reported cases have morphological characteristics similar to previously reported ones, they had distinct radiological and clinical characteristics. Patient 1 is only the third report of an adult with chondromesenchymal hamartoma. His case was characterized by its radiological appearance mimicking a posterior mediastinal tumor. Patient 2 represents the first documentation of DIC as a postoperative complication following excision of a chondromesenchymal hamartoma. We present these two cases to provide clinicopathological insights regarding this extremely rare tumor that are relevant to both pathologists and clinicians. Chondromesenchymal hamartoma of the chest wall presents at birth or in early infancy as an intraosseous expansile mass involving the ribs. It has an incidence of about 0.03% among primary bone tumors and shows male predominance; approximately 100 cases have been reported worldwide . There i–6 pedicle internal fixation were performed. The patient had an uneventful recovery and was discharged in stable condition on the third postoperative day.A chondromesenchymal hamartoma of the chest wall was incidentally discovered on the imaging studies of a 24-year-old male who presented with complaints of persistent cough in May 2019. Digital radiography (DR) was suggestive of a left posterosuperior mediastinal mass with bronchial changes  with marked elevation of C-reactive protein and procalcitonin levels and white blood cell count. He was transferred to the intensive care unit and started on vancomycin and ceftazidime for a presumptive diagnosis of pulmonary infection. Coagulation function tests were suggestive of disseminated intravascular coagulation (DIC): D-dimer, 5.36 mg/L; antithrombin III, 67.4%; fibrinogen degradation products, 11.3 μg/mL; prothrombin time, 19.4 s (11.0–4.0 s); activated partial thromboplastin time, 62.6 s (25.0–35.0 s); and fibrinogen, 5.38 g/L (2.00–4.00 g/L). Heparin sodium was administered as an anticoagulant and fresh frozen plasma was transfused to correct coagulation disorders. Subsequently, the infant’s condition improved, the thoracic tube drain was removed 7 days after surgery, and the infant was discharged from the hospital on the 11th postoperative day.Macroscopically, the tumor was multilocular and measured approximately 3.5 cm × 2.5 cm × 1.5 cm. Microscopically, the solid area consisted of hyaline cartilage with endochondral calcification  detection using ive Fig. a-b, whicThe tumor measured approximately 5 cm in diameter with focal cystic changes. Microscopically, most of the solid section’s area consisted of fascicles of mesenchymal spindle cells interwoven with multilobulated hypercellular hyaline cartilage  had DR examinations at 1 month and 3 months postoperatively, which showed no sign of recurrence, T4–6 pedicle internal fixation could still be observed and the remnant lung had a normal appearance. For case 2, the patient had DR examinations every year since the surgery in 2015, which showed no sign of recurrence and a normal lung appearance. Both patients had neither respiratory issues nor any other newly discovered tumor at the last follow-up on January 30th, 2020.en bloc with segments of the 7th and 8th ribs [The term “mesenchymal hamartoma” was first proposed in 1979 by McLeod and Dahlin, as it best reflected the benign nature of this lesion composed of disordered but non-neoplastic skeletal tissues . Odell aPseudomonas aeruginosa infection and pulmonary insufficiency [Chondromesenchymal hamartomas are usually unilateral and are commonly seen on the right side, with a male-to-female ratio of 1.6:1 . A few cficiency . PatientImaging studies are helpful in determining the site of origin, tumor density, enlargement, and effect on adjacent structures; however, imaging is not considered diagnostic and may be misleading if the tumor location or patient age is atypical , 15. In Microscopically, chondromesenchymal hamartomas have immature spindle-shaped mesenchymal cells, plate-like hyaline cartilage, woven bone formation, endochondral ossification and calcification, osteoclastic giant cells, and secondary ABC changes; abnormal mitoses and atypia are not present . Woven tThe differential diagnosis of chondromesenchymal hamartoma includes tumoral and non-tumoral lesions involving the ribs that are common in infants and children, including primary ABC, chondrosarcoma, enchondroma, osteochondroma, fibrous dysplasia, and osteofibrous dysplasia (OFD) . PrimaryUSP6 and/or CDH11 rearrangements are found in 69% of primary ABCs, but not in secondary ABC [IDH1  or IDH2 (R172S) mutations may occur in enchondromas, atypical cartilaginous tumor/grade 1 central chondrosarcomas, grade 2/3 central chondrosarcomas, and dedifferentiated chondrosarcomas; however, they are absent in osteochondromas [MDM2 and CDK4 are amplified in low-grade central osteosarcomas and periosteal osteosarcomas, as demonstrated by FISH/IHC [H3F3B appear in about 95% of chondroblastomas, while G34W/L mutations in H3F3A are found in 92% of giant cell tumors of the bone [Molecular diagnostic techniques have recently emerged as independent diagnostic tools to improve diagnostic accuracy and reduce interobserver variability; many characteristic genetic alterations have been identified in bone tumors . USP6 andary ABC . IDH1 (Rondromas , 28. MDMFISH/IHC . K36M muthe bone . It is lThe treatment strategy for chondromesenchymal hamartoma involves one of two main approaches: conservative management for asymptomatic patients and surgical treatment for patients with respiratory distress caused by mass compression. A case of spontaneous regression of a chest wall hamartoma in an infant was reported . In mostHerein, we reported two extremely rare cases of chondromesenchymal hamartoma. Although the lesions in these cases were morphologically similar to previously reported cases, they had distinct radiological and clinical characteristics. To the best of our knowledge, case 1 is only the third report of an adult patient with chondromesenchymal hamartoma. This patient was suspected of having a posterior mediastinal tumor on radiology. Case 2 is the first documentation of DIC as a postoperative complication of chondromesenchymal hamartoma. This report may raise awareness regarding the presentation, diagnosis, and management of chondromesenchymal hamartoma among pathologists, radiologists, and clinicians.


Experts postulate the formation to be driven by nidus deposition, accumulation of inorganic salts, and transmigration of stones along the urogenital system. An X-ray of the pelvis can confirm the diagnosis and surgical intervention can assuage the symptoms. In this case report, we describe an elderly male who presented with a hard penile swelling with a prior history of phimosis for a decade. He underwent an X-ray of the pelvis which helped diagnose a case of preputial calculus. We performed an emergency dorsal slit for the prompt removal of the calculus with follow up debridement. In conclusion, a clinical rarity such as this needs apt diagnosis and surgical mediation to treat it. Follow-up should be considered in the elderly given the possibility of malignant transformation in the future.Preputial calculus is a rare clinical entity in the realm of urolithiasis. It is usually seen in elderly people with poor hygiene. Phimosis is a common urological condition encountered by surgeons. Preputial calculus formation is a rarity on the spectrum of urolithiasis conditions. It is usually seen in elderly males with phimosis or poor hygiene. Stones in the prepuce can occur due to migration of the stone from the proximal urinary tract or they may form de novo , 2. It hA 55-year-old male with a known case of hypertension under regular treatment presented to the casualty department with complaints of swelling of the penis for the preceding two weeks, burning micturition for the preceding 10 days, and inability to pass urine for the preceding two days. He also had lower abdominal pain for two days which was the colicky type with no history of genital trauma. He had a history of inability to retract prepuce for 10 years with multiple episodes of burning micturition for the last one year. He had no fever spikes, reduced urine output, high colored urine, haematuria, and pyuria. He had complaints of obstructive lower urinary tract symptoms including straining, hesitancy, and post-void dribbling for the past one week. He had no significant history of any previous surgeries in the past. He denied a history of any sexual promiscuity in the past. On examination, he was conscious, oriented, and febrile with no signs of dehydration. His pulse rate was 104 per minute with a blood pressure of 140/90 mm Hg. He had lower abdominal tenderness without a palpable bladder. Local examination revealed phimosis with a stony hard swelling over the glans penis under the prepuce with no visualization of the urethral meatus. Penile and scrotal edema were present with bilateral testes palpable and non-tender. He had enlarged tender bilateral inguinal lymphadenopathy.The initial clinical impression was balanoposthitis with underlying carcinoma of the penis considering his age of presentation. Preputial calculus was also considered as a differential diagnosis in view of the hard consistency of the swelling. X-Ray of the pelvic region showed a radiopaque shadow in the glans region Figure . UltrasoPreputial calculus is a rare clinical entity in the medical literature. Preputial calculi originate from three probable methods as outlined by Winsbury-White, namely (1) inspissated smegma, 2) combination of smegma and urinary salts, and 3) concentration of urinary salts alone [ combinat concentrLong-standing phimosis with poor local hygiene, underlying neurological or urological anomalies, and geographical distribution (third world countries) are the known risk factors of preputial calculus . ClinicaUltrasound or non-contrast computerized tomography of the kidneys, ureters, and bladder is indicated to rule out urolithiasis and ongoing complications like hydroureteronephrosis. Sometimes even a plain pelvic X-Ray shows a radiopaque shadow in the region of glans which aids in the diagnosis as in our case. Treatment is primarily surgical with dorsal slit accompanied with a stone extraction which has been seen to immediately alleviate the obstructive uropathy . In our In our patient, who presented with obstructive uropathy to the casualty department, immediate radiography aided in securing the diagnosis of preputial calculus, and a prompt surgical treatment alleviated the symptoms. A possible mechanism for the formation of the calculus in our patient seems to be the deposition of inspissated smegma and salts into the anatomical nidus. Stone analysis of the calculus revealed a composite of calcium carbonate and calcium sulfate-the most common type . Daily dPreputial calculi are a very rare clinical entity almost invariably occurring in association with phimosis and commonly presenting as a cause of obstructive uropathy with acute retention of urine. Awareness of the condition is essential so that it can be considered with a high degree of suspicion for differential diagnosis in cases of hard penile swelling with associated phimosis. A plain X-ray helps in its diagnosis. A dorsal slit and stone extraction will lead to prompt relief of symptoms. Here, we presented a patient with obstructive uropathy with phimosis and balanoposthitis that was due to a preputial calculus that probably formed due to poor local hygiene and long-standing phimosis. Elderly patients have to be followed up with a high suspicion for malignant transformation for early screening and detection.


To describe the clinical course of an active solitary idiopathic choroiditis  that nearly resolved over six weeks without intervention.An 18-year-old man presented to the emergency department with headaches and new onset central scotoma in the right eye. Visual acuity was 20/20 in both eyes. Fundus examination revealed an amelanotic choroidal lesion with associated shallow subretinal fluid. It measured 6.1 × 6.3 × 1.4mm on A- and B-scan. Evaluation for systemic inflammatory and infectious diseases was negative. A week later, the lesion remained stable, and a month later, there was improvement of the lesion with a decrease in size on OCT and exam and resolution of the subretinal fluid suggesting that the lesion had become inactive.Solitary idiopathic choroiditis  is a rare condition characterized by inflammatory granulomatous reaction. This case report sheds light on the unknown natural course of a solitary idiopathic choroiditis . A focal scleral nodule (FSN), previously referred to as solitary idiopathic choroiditis (SIC) and unifocal helioid choroiditis, is characterized by an inflammatory granulomatous reaction in the sclera and/or choroid in the absence of an underlying systemic inflammatory disease –3. It isCurrent management recommendations of FSN suggest monitoring of inactive lesions and either close observation or treatment with systemic corticosteroids for active lesions . The natAn 18-year-old otherwise healthy, white male presented to the emergency department with headaches and new onset central scotoma in the right eye of six days duration. He had a history of strabismus and amblyopia of the left eye as a child that had been surgically corrected twice. On initial presentation, his visual acuity was 20/20 in both eyes and anterior segment examination was unremarkable. Fundus exam at that time of the right eye demonstrated a round-shaped, amelanotic choroidal lesion, with associated shallow subretinal fluid (SRF) along the superior arcade. Optical coherence tomography (OCT) of the macula confirmed a hypointense choroidal mass with overlying outer retinal changes and SRF extending to the fovea Fig. a. Six weFSN is usually diagnosed based on detailed patient medical history, systemic laboratory evaluation for inflammatory and infectious etiologies, clinic features, such as lack of inflammatory cells in anterior chamber and vitreous, and ocular imaging findings such as absence of contact sign as reported in choroidal granulomas .We herein describe a case of an 18-year-old, symptomatic male who presented with FSN. His FSN became inactive over the course of six weeks without intervention. Although this is a report of a single patient, we describe the natural course of an active FSN and its spontaneous resolution, which has not specifically been described in the literature. Due to the lack of formal guidelines on the treatment of FSN, the clinical course of patients who are followed longitudinally, as in our patient’s case, can provide valuable information to providers who encounter this rare condition.Shields et al.., they reviewed sixty patients with both active and inactive lesions. In their study, the lesion remained stable in 60 % of the patients, improved in 37 %, and recurred in 3 % during a six-month to twenty-five-year follow up period (mean of 24 months) [In the largest retrospective study of FSN in the literature by  mean of 4 months  mean of 4 months  mean of 4 months  mean of 4 months  mean of 4 months Hong et al., a total of six patients were described, five of which received systemic corticosteroid therapy, and the sixth patient’s lesion was observed and remained stable after one year [Kohne et al.., they described a patient whose lesion mildly improved with no intervention six months later, but the lesion was likely inactive at presentation due to lack of surrounding fluid and distinct margins seen in the later phases of FA, features of activity [Although there have been other reported cases of FSN, those reports also do not provide clarity regarding the natural course of an active lesion. Moreover, the authors frequently do not identify whether the presenting lesion is active or inactive. As seen in our case, the active lesion presents as a dull-yellow lesion with an ill-defined margin, associated with yellow intraretinal exudation, localized subretinal fluid, retinal vascular dilation, or focal retinal hemorrhages. In the original report of FSN by one year . While tone year . In the activity . Other cactivity –8. Underactivity .


A male patient in his thirties presented with a mass lesion on the scalp. A CT scan revealed one posterior scalp mass with no intracranial abnormalities. Post excision histologic examination confirmed an extracranial meningioma . A second anterior scalp mass, not revealed by CT scan, was discovered during surgery. It was excised and diagnosed as a steatocystoma. Meningiomas predominantly occur intracranially but, in some instances, may present as a standalone extracranial tumor without intracranial abnormalities. Because extracranial meningioma is uncommon, it may be overlooked during clinical diagnosis of scalp masses. We recommend that this neoplasm be routinely considered in the differential diagnosis of extracranial tumors. The discovery of another rare tumor—a steatocystoma located in immediate proximity on the scalp—is further remarkable. We briefly review relevant case reports and etiologies and consider a potential relationship between the two neoplasms. However, it remains more likely that the concurrence of these tumors in our patient was simply coincidental.This report documents a rare case of an extracranial meningioma on the posterior scalp without apparent dural connection. Additionally, a sebaceous steatocystoma of the anterior scalp presented alongside the meningioma. A steatocystoma localized to the scalp is also remarkably rare. To our knowledge, this is the first report documenting  Meningiomas are common tumors arising from meningothelial cells surrounding the brain and spinal cord. These tumors account for 24-35% of all primary brain neoplasms , 2. AtypSteatocystoma is an uncommon cutaneous disorder presenting as single or multiple sebaceous duct cysts. Typically, these cysts appear in anatomical areas rich in sebaceous glands . InteresIn this report, we describe a case of a large extracranial meningioma presented as a posterior scalp mass coexisting with a solitary steatocystoma of the anterior scalp. The latter was discovered unintentionally during surgery. Both conditions are rather rare, and to the best of our knowledge, this is the first report documenting an extracranial meningioma concurrent with a steatocystoma on the scalp.A male patient in his thirties presented with a mass lesion on the scalp. This was a recurrent mass lesion on the posterior scalp (~3–4 cm at the greatest dimension), status post incision and drainage in the remote past (>10 years ago). Currently, the patient sought treatment as the lesion seemed to increase in size and was associated with pain. Past medical history as well as family history was unremarkable.A CT scan of the brain with contrast revealed a posterior scalp mass at the midline near the convexity, which was inseparable from the calvarium  is essential in confirming a definite diagnosis. Hematoxylin and eosin sections suggested a low-grade meningioma  is an uncommon benign tumor. The typical form is hereditary and linked to a mutation in the coding region of the R17 gene . It appeR17 gene , 17 and R17 gene , 18. NotR17 gene –17, and intracranial  meningioma, presenting with steatocystoma on the scalp and facial area [One case report described an adult male patient with a recent history of ial area . The posial area . Indeed,ial area . Howeverial area . Interesial area  includinial area –22. ThusThe sheer lack of cases reporting concurrence of extracranial meningioma and steatocystoma precludes any further meta-analysis at present. Given that causative factors and etiologies of both neoplasms remain largely unclear, and no conclusive comorbidity mechanisms can be established, it is likely that the concurrence of these conditions in our patient was simply coincidental. Yet, we find value in publishing our findings, as similar cases in the future might benefit from this report.In our case, the clinical differential diagnosis included several mesenchymal tumors such as lipoma, benign adnexal tumor, malignant melanoma, and benign smooth muscle tumor. Extracranial meningioma was not considered. Awareness that meningiomas may occur in an extracranial location without concurrent intracranial abnormalities will help avoid potential mismanagement of these neoplasms due to being overlooked. Extracranial meningioma appears indistinguishable from other tumors based on radiological examination. While relatively rare, it is nonetheless essential that clinical teams always consider extracranial meningioma in the differential diagnosis of scalp massesWe found no prior case reports documenting a steatocystoma on the scalp concurrent with an extracranial meningioma. Both tumors are rare, and to the best of our knowledge, this is the first case report describing the coexistence of these neoplasms alongside each other on the scalp. We speculated on the possibility that immunological events often associated with meningiomas might play a potential causative role in the onset of steatocystoma in the immediate anatomical proximity. Due to the scarcity of similar case reports and lack of additional relevant evidence, the suggested mechanism is weakly supported and entirely speculative. However, we hope that future studies might benefit from this publication


Primary synovial chondromatosis is a rare benign neoplastic process, in which cartilaginous nodules are produced in the subsynovial tissue. It has 3 main subtypes . We present the case of a 61-year-old female, with a mass involving her right thumb for at least 5 years, which had recently increased in size. X-ray showed a soft tissue mass, without calcification or any underlying bony abnormality. Ultrasound and MRI showed a 6-cm mass surrounding the right flexor pollicis longus tendon of the right thumb. The patient went on to have surgical resection and was given a diagnosis of tenosynovial chondromatosis. Primary synovial chondromatosis is a rare benign neoplastic process, in which cartilaginous nodules are produced in subsynovial tissue A 61-year-old female presented with discomfort and increasing size of a lump involving her right thumb and thenar eminence -c that hOn examination, there was a circumferential mass extending from the base of the right thumb to the proximal phalanx that was mildly tender. There was no movement at the metacarpophalangeal or interphalangeal joints. Neurovascular supply was intact.Initially, the patient had a radiograph of the right hand, which displayed a soft tissue swelling over the dorsolateral aspect of the thumb without any underlying bony scalloping or erosion . An ultrMRI was then performed, which displayed a multilobulated mass encasing the FPL, surrounding the right first proximal phalanx, first metacarpophalangeal joint and first metacarpal a-c.Fig. Ultrasound-guided biopsy was performed, which revealed an atypical cartilaginous lesion with mild-to-moderate cytological atypia . The lesThe tumour was resected in its entirety using a dual incision approach  and 6 ceThe patient was discharged with routine follow-up and surveillance planned. Resection resolved a lot of this lady's pain; however, her osteoarthritis leaves some residual pain and stiffness. There is also some residual weakness of thumb flexion. Overall, she is pleased with her outcome.Final histopathologic analysis displayed lobules of cartilage in soft tissue. The lesion was well circumscribed and did not display infiltration. Although moderate cytological atypia with up to 4 chondrocytes per lacuna and single chondrocyte necrosis were present, aggressive features such as bony infiltration or destruction were not identified. These histologic findings with multidisciplinary input of the clinical presentation and radiology pattern favoured diagnosis of tenosynovial chondromatosis with cytological atypia.Milgram, in 1977, described 3 classic stages of synovial chondromatosis: (1) early disease characterized by synovial neoplastic activity, (2) transitional stage with development of loose bodies and (3) late stage with loose bodies but no active synovial disease In general, in primary synovial chondromatosis, patients are aged between 40 and 60 years and men are affected more commonly than women On radiograph, if the bodies are calcified, they will show a pathognomonic appearance with rounded intra-articular loose bodies of a similar shape and size displaying a chondroid (ring-and-arc) matrix CT can be used to further characterize the loose bodies, but MRI is best for displaying the relationship to the joint space, bursa or tendon sheath. MRI will generally display a lobulated, homogenous mass with low-to-intermediate signal T1 intensity and T2 hyperintensity Macroscopic features of this soft tissue mass are multiple small (0.1-1.0 cm) cartilaginous lobules that coalesce into large conglomerates Given that each line of approach  has certain limitations, it is important to correlate clinical history, imaging appearances and histologic appearance with multidisciplinary team input. Clinical features that are concerning for a more aggressive process include pain and rapid growth. Radiological features of concern include deep cortical scalloping, cortical destruction or a soft tissue mass.Treatment is surgical synovectomy with removal of chondral fragments. Recurrence is higher in extra-articular .•Tenosynovial chondromatosis typically affects flexor tendons of the hands or feet.•Tenosynovial chondromatosis can be present for many years, and be relatively asymptomatic, prior to diagnosis.•Factors concerning for low-grade chondrosarcoma: hypercellularity, cytological atypia, myxoid change and necrosis.We confirm that written, informed consent for publication was obtained from the patient.


Non-islet cell tumor hypoglycemia (NICTH) is a rare paraneoplastic syndrome that secretes incompletely processed high molecular weight insulin growth factor 2 (big-IGF2), which results in stimulation of the insulin receptor and subsequently induces hypoglycemia. Gastrointestinal stromal tumor (GIST) is a common intestinal mesenchymal neoplasm of the gastrointestinal tract. The most frequent site of GIST is the stomach; NICTH induced by IGF2-producing stomach GISTs is rare.An 84-year-old man was admitted to the hospital due to impaired consciousness (JCS II-10) in the morning. At the time of admission, his serum glucose was 44 mg/dL; his consciousness was restored with 20 ml of 50% glucose. To avoid hypoglycemia, a continuous intravenous infusion of glucose as well as dietary intervention was required. At the time of hypoglycemia, the levels of insulin and C-peptide were suppressed. Additionally, IGF1 levels were below the normal range. Abdominal computed tomography revealed that he had a large lobulated mass (116 × 70 × 72 mm) around the gastric corpus. Pathological analysis of biopsy specimens identified disarray of spindle cells and positivity for c-kit as well as strong positivity for DOG-1. Further analysis revealed high levels of Ki-67 (Mib-1 index: 15.5%) and mitotic index (7/50HPF); the tumor was diagnosed as high-risk GIST, and complete surgical resection was performed. Hypoglycemia resolved immediately after tumor resection. The resected tumor specimen was positive for IGF2 staining, and big-IGF2 (11–18 kDa) was detected in preoperative serum and tumor samples; the patient was diagnosed with NICTH due to an IGF2-producing tumor.NICTH is rare in GIST of the stomach; however, the large GIST could produce big-IGF2 and subsequently cause severe hypoglycemia, requiring prompt evaluation and complete tumor resection. Non-islet cell tumor hypoglycemia (NICTH) is a rare paraneoplastic syndrome and has an estimated incidence of approximately one per million person-years . NICTH iHere, we report a NICTH case induced by IGF2-producing primary gastric GIST.An 84-year-old man was admitted to an affiliated hospital due to an impaired level of consciousness (JCS II-10) in the morning.2 was 95% at ambient air. Other physical examinations were unremarkable. Abdominal computed tomography (CT) in the emergency unit demonstrated a large lobulated mass around the gastric corpus ~ φ120 mm. For further analysis, he was transferred to our hospital.He experienced rotatory vertigo, weakness and dizziness without any particular cause form 1 week before the admission to that hospital. Three days prior to the admission, he had fainted and fallen to the floor in the early morning. At the time of admission in that hospital, his serum glucose was 44 mg/dL, and after infusion of 20 ml of 50% glucose solution, his consciousness was restored. Blood pressure was 184/100 mmHg, and heart rate was 65/min. He did not have fever, his respiratory rate was 16/min, and SpOWhen transferring to our hospital, he was alert with continuous intravenous infusion of glucose. His blood pressure and heart rate were also in the normal range. He had a 2-year history of hypertension that was treated with olmesartan. He was prescribed atorvastatin, tamsulosin, and brinzolamide/timolol maleate eye drops for dyslipidemia, benign prostate hyperplasia, and glaucoma, respectively. He had taken sennoside for constipation as needed. He did not have a history of diabetes, although both his older sister and brother did. He has been followed up and monitored for intraductal papillary mucinous neoplasm (IPMN) with abdominal CT at an affiliated hospital.After admission to our hospital, he required a continuous infusion of glucose (4.2 g/h), a diet of 1800 kcal per day and supplementary meals of 380 kcal before sleep to avoid hypoglycemia. Insulin and C-peptide were suppressed at the time of hypoglycemia . The transcriptional product of the Igf2 gene is translated into a pre-pro-IGF2 polypeptide, and subsequently, posttranslational modification forms it into pro-IGF2. Pro-IGF2 is processed into mature IGF2, which has a molecular weight of approximately 7.5 kDa. However, in most IGF2-producing NICTH, incomplete processing occurs, and one part of the E chain  remains; high molecular weight IGF2, or so-called big-IGF2 (molecular weight: 11–18 kDa) [The –18 kDa) , is then–18 kDa) . Since I–18 kDa) , the indMost IGF2-producing tumors are large, and 70% are φ10 cm or greater . In our In conclusion, we report a case of NICTH with IGF2-producing primary gastric GIST. The frequency of NICTH associated with GIST of the stomach is rare; however, the large and high-risk GIST could produce big-IGF2 and subsequently cause severe hypoglycemia, requiring prompt evaluation, interpretation and complete tumor resection.


Hepatic alveolar echinococcosis (HAE) presents a high pathogenicity and case fatality rate. The main treatment for HAE is surgical resection. Giant lesions in the liver and invasion of the pathogen into the retrohepatic inferior vena cava are usually associated with a poor prognosis when radical resection cannot be performed.A 56-year-old man who underwent hydatidectomy 7 years prior noted a recurrence of HAE. He was subsidized and admitted to our hospital for the purpose of surgical treatment.By computed tomography, angiography and three-dimensional (3D) computed tomography reconstruction images, multiple, giant HAE with 75% stenosis was confirmed.With the 3D visualization technique, we designed the surgical plan and performed radical resection of the lesions, including the invaded inferior vena cava, and maximized retention of normal liver tissue. The abdominal aorta of an organ donor was used for vascular allograft reconstruction.The patient recovered gradually after the operation. He was followed up for 3 months, and the reconstructed vein patency was good.The 3D visualization technique combined with a blood vessel allograft allowed us to expand indications for radical resection of extensive HAE. Furthermore, the invasion of the retrohepatic inferior vena cava presents a large barrier to successful treatment.,5 In such cases, a three-dimensional (3D) visualization system is crucially needed for surgeons to better visualize difficult anatomy and improve patient safety .,8 In this report, we describe a case of multiple and giant hepatic lesions with 75% stenosis of the inferior vena cava, which is usually considered to be incurable. Via the 3D visualization technique, we resected the lesion and successfully reconstructed the inferior vena cava with allograft blood vessels.Echinococcus multilocularis is a potential lethal pathogen causing hepatic alveolar echinococcosis (HAE), which presents a high pathogenicity and case fatality rate, especially in Asia.22.1A 56-year-old man who underwent hydatidectomy 7 years prior noted a recurrence of HAE 1 year ago. The patient had no history of hepatitis or heavy drinking. He was a veterinarian and had lived in Tibet, China, all the time. Moreover, there was no family medical history related to the illness. The patient only had mild pain without fever, jaundice, or other symptoms. He was admitted by us with the suspicion of HAE.2; total liver volume = 1525.7 cm3, standard liver volume (SLV) = 1266.5 cm3 and RLV/SLV = 54.2%. These data were determined based on the 3D visualization technique. For the control, total liver volume = 1484.8 cm3, SLV = 1266.5 cm3 and RLV/SLV = 41.6%. We concluded that residual normal liver tissue can afford hepatectomy . However, abdominal computed tomography showed a giant hepatic lesion (20 cm in diameter) , characterized by extensive involvement of liver segments  and 75% stenosis of RHSIVC, might cause the patient to miss the opportunity for surgery.HAE is a rare but potentially lethal parasitic disease.,7 The 3D visualization technique emerged and was first applied for the planning of liver resection by Oldhafer et al in 1999. With 3D visualization techniques, comprehensive anatomic information, such as the position, size, number of hepatic lesions and adjacent structures, can be intuitively assessed. Moreover, 3D visualization vividly reflects the structural characteristics of the intrahepatic biliary and vascular network. The remaining liver volume and the diameter of blood vessels in the section were calculated to evaluate the feasibility of the proposed surgical strategy (Figs. ,8It is acknowledged that the success of liver surgery is largely determined by the preoperative accurate understanding of intrahepatic structure and liver volume due to the complexity of liver anatomy.,5 Ligation is considered prohibitively risky unless in the context of trauma damage control. Compared with vena cava reconstruction/repair, ligation was associated with significantly higher complication rates of extremity compartment syndrome.,18 Considering the local economy and health conditions, repeated complications and treatment will increase the burden on patients. Recently, many scholars have proposed a strategy that combines resection and reconstruction to improve the resection rate and curative effect of surgery in the condition of infringement on large blood vessels.–21 Thus, the method of reconstruction and choice of alternative blood vessel allograft are important factors affecting the safety of surgery and the prognosis of patients. Due to the issue of alternative blood vessels, it is common to use artificial/autologous/allogeneic blood vessels.,21,23,24 In our center, we performed revascularization using donor abdominal aorta to replace the invaded vena cava during liver transplantation. We believe that allogeneic blood vessels have the same tissue structure and function as the host, with matched diameters and good long-term patency.,23 Moreover, the cryogenic cryopreservation technique is maturing. This technique can extend the scope of allogeneic vasculature and may become the main potential source of blood substitutes in the future. It also provides a choice of grafts for vascular reconstruction for tumor patients with existing vascular invasion, making it possible to expand the radical resection of tumors.,26,27In this case, the patient's RHSIVC was corroded by a hydatid lesion infringing on the right edge of the abdominal aorta and could not be separated. Angiography suggested that vena cava stenosis occurred and umbilical veins were opened and widened compensatorily. Resecting the RHSIVC without reconstruction may be a choice. However, it did not show these benefits in this case. We assumed that there may be 3 reasons. First, due to the wide range of lesion invasion, the resection line did not follow the standard liver segment or the liver lobe. The wound surface was large, and the oozing was extensive. Second, a history of abdominal surgery combined with lesion violations led to severe liver adjacent organ adhesion and increased bleeding. Third, resuscitation of the air embolism also prolonged the operation time.The literature has shown that 3D visualization techniques can help shorten the operation time and reduce bleeding.All the authors took part in the treatment of this patient. Ning Li, Dongdong Lin and Tiezheng Wang were responsible for the writing and revision of the paper. Guangming Li, Zhi Fu, and Daming Gao were responsible for the literature review and editing of the figures. All authors have read and approved the final version submitted.Conceptualization: Tiezheng Wang, Ning Li.Validation: Zhi Fu, Daming Gao, Dongdong Lin.Writing – original draft: Ning Li.Writing – review & editing: Zhi Fu, Daming Gao, Dongdong Lin.


Hepatic artery aneurysms have an estimated incidence of approximately 0.002%, of which one half are pseudoaneurysms (PsAs). These typically occur following trauma or liver transplant and are often asymptomatic. An uncommon pathology, mycotic aneurysms or PsAs are those that result as a consequence of infections. The danger in mycotic processes stems from their complications of systemic seeding of infection, rupture, and possible exsanguination. This case reports a mycotic PsA that was found in an accessory left hepatic artery (aLHA) branching from the left gastric artery (LGA). The patient presented with recurrent Clostridium difficile colitis with perforation and was later found to have a left upper quadrant vascular lesion during repeat imaging after failing to progress in their clinical course. After multidisciplinary meetings with vascular surgery and interventional radiology, the patient eventually underwent endovascular coil embolization. This is the first documented case of a mycotic PsA secondary to recurrent C. difficile colitis located in an aLHA branching from the LGA. Clostridium difficile infection with perforation. This case was diagnosed as a result of clinical examination and CT and then managed by endovascular intervention at a community-based residency program. This case is unique as it is not only a deviation from the standard of care for mycotic PsAs, but it is also the first documented case of a mycotic PsA of an aLHA. This case is reported in accordance with SCARE Criteria [A pseudoaneurysm (PsA) refers to a disruption in the arterial wall resulting in blood flow between the tunica media and tunica adventitia. They are referred to as PsAs as they do not contain all layers of the arterial wall, excluding the intima -2. AneurCriteria .C. difficile colitis in a previous hospital admission. Past surgical history was significant for an appendectomy. The patient’s father and brother both have been diagnosed with colorectal carcinoma. She denies any history of alcohol, tobacco, or illicit drug use.A 71-year-old Caucasian female presented to the ED after unremitting bilateral lower abdominal pain of acute onset. She also noted slowly worsening, diffuse abdominal pain. She describes the pain as sharp in nature and unrelenting. Past medical history is significant for C. difficile infection and treated with both broad spectrum antibiotics. A consultation was placed to interventional radiology and the patient underwent CT-guided drainage of multiple pelvic abscesses which were later positive for clostridium species. The patient began to progress, however, then had recurrence of severe abdominal pain coupled with elevating leukocytosis. At this time, repeat CT demonstrated interval development of small pockets of portal venous air in addition to hypodense lesions in the left lobe of the liver with partial enhancement as shown in Figure On initial presentation, all vitals were found to be within normal parameters. The patient’s abdomen was soft, nondistended, but with exquisite tenderness in the bilateral lower quadrants with the addition of diffuse subjective abdominal pain. Laboratory testing was significant for a leukocytosis of 18.6 bil/L. CT was performed revealing two pelvic fluid collections with peripheral enhancement in addition to a mild pneumoperitoneum. Based on physical examination and lack of SIRS criteria, the patient was admitted for recurrent Multiple interdisciplinary discussions were conducted between vascular surgery, internal medicine, and the interventional radiology team. Based on the current literature and the location of this PsA, the patient was consented for and underwent endovascular intervention. The interventional team performed selective angiography of the celiac trunk (CT) followed by highly selective targeting of what was found to be an aLHA branch off the LGA as seen in Figure  The postoperative period was uneventful and the patient remained stable throughout its entirety. On postoperative day one, the patient underwent mesenteric arterial duplex ultrasound that demonstrated a PsA with heterogeneity to the echogenic material and no discernible blood flow, consistent with successful embolization of the PsA as seen in Figure Pseudoaneurysms are often asymptomatic and found incidentally during investigation of other conditions. Specifically, hepatic artery aneurysms are estimated to have an incidence of approximately 0.002%, most common after transplant or trauma . The lesThere have been multiple studies regarding mycotic aneurysm management, however, the majority of such deal with the aorta, iliac, and femoral arteries. Management differs tremendously when it regards hepatic arteries secondary to difficulty in access for open repair . TreatmeIn addition to the unconventional nature of the intervention provided, this case is unique as it is the first documented case of an aLHA mycotic PsA. The incidence of hepatic arterial tree anomalies can be as high as 11%-21% of right hepatic arteries (RHAs) and 3.8%-10% of LHAs. However, more rare are aRHAs and aLHAs which occur in 0.8%-8% of cases . MultiplC. difficile infections have been written about only in case reports. This infectious process rarely exhibits extraintestinal pathology. The Journal of Clinical Infectious Disease estimated the incidence to occur in around 0.17% of all C. difficile infections [Mycotic aneurysms secondary to toxigenic fections . When maC. difficile colitis with perforation. Furthermore, the management of this mycotic PsA was done with an unconventional approach secondary to its poorly accessible deep intrahepatic location. Potential risks of this approach are recurrence and the possibility of further infectious process secondary to the coils being a nidus for infection. If this occurs, the patient will need hepatic resection or complex intrahepatic surgical repair.The highpoint of this case report is how it connects two very rare clinical entities. The first being the presence of a Michels Type V accessory LHA. This couples with a mycotic PsA secondary to transposition from C. difficile infection. This case is further unique as it is not only a deviation from the standard of care for mycotic PsAs, but it is also the first documented case of a mycotic PsA of an aLHA. Given its unfavorable location for open surgical procedure or ultrasound percutaneous thrombin injection, the decision was made to coil this lesion and treat with long-term antibiotics with close outpatient follow up. This case highlights how an interdisciplinary approach serves the best interests of the patient and eventually resulted in a great outcome.This case report presents an accessory LHA mycotic PsA secondary to recurrent transverse colitis secondary to


We report a case of late onset hyperplastic callus formation (HPC) in the right femur in type V osteogenesis imperfecta (OI) mimicking the occurrence of a malignant osteosarcoma.A 27-year-old female patient consulted us due to swelling in her right femur over 2–3 months without trauma. X-rays looked like an osteosarcoma, blood tests showed increased bone metabolism. A CT scan, a MRI and biopsy were performed, showing late onset HPC due to osteogenesis imperfecta type V.OI shows a heterogeneous disease pattern due to a variety of clinical and radiographic findings. HPC is a rare complication of OI type V. Differential diagnosis range from cortical or periosteal osteosarcoma, periostitis, myositis ossificans, subperiosteal hematoma secondary to trauma or osteomyelitis.Recognition of HPC as a form of this particular type of OI is important to avoid misdiagnosis like malignant transformation to osteosarcoma. A biopsy and advanced imaging modalities like CT, MRI and scintigraphy are recommended. Osteoge2A 27-year-old female patient presented with an indolent but progressive swelling of the right mid femur over the last 2–3 months in our outpatient consultation hour of our university hospital. She reported of a known osteogenisis imperfecta type V, being the only member of her family suffering from it. Clinical phenotype was characterized by short statue, a mild kypho-scoliosis, a short neck and a mild ligamentous hyperlaxity. She did not report of a trauma or any inflammatory disease. Although numerous fractures of the lower extremities occurred over the last decade which were treated conservatively as well as operatively by intramedullary nailing, HPC or extraordinary swelling never developed.Conventional radiographs of the affected area showed a massive osseous tumor of the right femoral mid-shaft . RadiogrIn the assumption of a malignant osteosarcoma, further tumor staging was initiated, including contrasted computed tomography of thorax, abdomen and pelvis. Furthermore a contrasted magnetic resonance Imaging (MRI)  of the a3OI remains a heterogeneous disease pattern. Diagnosis might be challenging and includes a variety of clinical and radiographic findings ,6.HPC is a rare complication of osteogenesis imperfecta type V and its occurrence has only been reported in about 30 cases, especially in adult patients it remains a very rare appearance ,11. MostDiagnosis of HPC formation is challenging and varies with patients age. During childhood the differential diagnosis are idiopathic osteoporosis, periosteal tumors, bleeding disorders, juxtacortical myositis ossificans, osteomyelitis or even child abuse injury and domestic violence . HoweverIn adults the diagnosis of HPC formation in OI Type V may be more difficult because of superimposed traumatic and osteoarthritic changes. Differential diagnosis includes amongst others cortical or periosteal osteosarcoma, periostitis, myositis ossificans, subperiosteal hematoma secondary to trauma or osteomyelitis . Main foThe clinical picture with pain and swelling is similar and elevation of alkaline phosphatase may be seen in both. The radiologic features with new bone formation without well-defined edges are very similar in both entities, although some features may be helpful in distinguishing between these two conditions . In osteAn osteosarcoma shows a tendency towards a focal appearance, reflecting the concentric growth of the tumor, while HPC usually grows along the shaft axis of the bone. The characteristic but infrequent sunray pattern of osteosarcoma may look like the early stages of HPC. In accordance with other authors we emphasize the use of advanced imaging modalities like CT, MRI and scintigraphy besides complete skeletal survey and x-ray imaging ,12,14,164HPC formation is a potentially serious complication of OI type V, occurring predominantly in the long bones of OI type V patients. Lesions can be precipitated by fractures, arise spontaneously and can become very large, altering the architecture of the bone. Recognition of HPC is important in order to avoid misdiagnosis like malignant transformation to osteosarcoma and differential diagnosis is only possible by biopsy.None of the authors has a conflict of interest.This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.Since this is a case report, no ethical approval had to be obtained by our institutional review board.Informed patient consent for publication of this case report was obtained.This case report is not part of a clinical study.Johannes Struewer, MDDepartment of Orthopedics and Traumatology, University Hospital Oldenburg, Rahel-Straus-Straße 10, 26133 Oldenburg, Germany.Since this is a case report, no ethical approval had to be obtained by our institutional review board.Editorially reviewed, not externally peer-reviewed.Hans Christoph Vonderlind: Writing - original draft, Investigation. Matthias Jessel: Writing - review & editing. Alexander Knobel: Investigation. Ingke Juergensen: Conceptualization. Johannes Struewer: Supervision, Writing - review & editing.


Gossypiboma refers to a retained surgical sponge that can occur after any type of surgery. Though it is a rare complication of surgery, the retention of surgical sponges still occurs. We report a case of a 70-year-old woman who presented with recurrent upper abdominal pain and fever. She had a prior history of cholecystectomy and choledochoduodenostomy. Magnetic resonance cholangiopancreatography showed a filling defect in common bile duct. However, during endoscopic retrograde cholangiopancreatography and common bile duct clearance, clumps of woven fibres were removed suggestive of gossypiboma.  A gossypiboma refers to a surgical sponge left inadvertently in the human body following a surgical procedure . It is a9/L), total bilirubin of 1.2 mg/dL, serum glutamic oxaloacetic transaminase of 23 IU/L, serum glutamic pyruvic transaminase of 37 IU/L, alkaline phosphatase of 240 IU/L  and gamma-glutamyl transferase of 110 IU/L . Contrast-enhanced magnetic resonance imaging of the abdomen with magnetic resonance cholangiopancreatography showed the presence of pneumobilia with patent choledochoduodenostomy site and filling defect in lower CBD. She was posted for endoscopic retrograde cholangiopancreatography (ERCP) and clearance of CBD. Contrast cholangiogram showed a patent choledochoduodenostomy site with filling defect in lower CBD  stones. Routine blood investigations showed raised total leukocyte count (16.5 x 10Dormia basket was used to clear the CBD, but surprisingly clumps of woven fibres were removed Figure .The basket was changed to extraction balloon, and residual woven fibres were removed Figure .Outside naked eye examination confirmed the retained surgical sponge Figure .At three-month follow-up, she is doing fine without any abdominal pain or fever. Gossypiboma refers to a surgical sponge left inadvertently in the human body following a surgical procedure and the subsequent tissue reaction around it . MajoritThe diagnosis of a gossypiboma is not often easy and is often clinically unsuspected and may be first recognized on imaging. Awareness of the typical radiologic appearances is critical to the diagnosis gossypiboma.


Small bowel atresia has quite an incidence in infants particularly in males. It is usually seen to be the most common cause of bowel obstruction in neonates since birth. It may be associated with other congenital anomalies.A 4 day old neonate presented with abdominal distension, billous vomiting & non passage of stools since birth. On further examination, it was seen that the abdominal distension was mostly central & there was hyperperistaltic bowel sounds on auscultation. The infant also had scoliosis. There was decreased appetite & features of dehydration. Diagnosed by USG(W/A), infantogram & X-Ray plain picture abdomen.The mortality from the atresia of ileum is greater owing to the incidence of early perforation. Infants may present with features discussed above. They usually present during the 1st week of life but majority of the babies presenting late have significant mortality.Early surgical intervention with excision of the dilated bowel segment with anastomosis of the proximal  to the distal part of the bowel has been seen to have been associated with decreased morbidity & mortality.It has been observed that one of the many causes for neonatal intestinal obstruction is attributed to small bowel atresia. This has been associated with successful outcome depending on the presentation of the child to the hospital to the timing of surgical intervention along with resuscitation & starting of enteral feeds. Small bowel atresia can be associated with antenatal morbidity in the mother as cited here. The work has been reported in line with the SCARE criteria .2A 4 day old male infant presented with abdominal distension, billous vomiting & non passage of meconium since birth to our hospital. The parents also reported that the infant would have frequent episodes of billous vomiting immediately following breast feeding.On examination, the abdomen was distended & there was hyperperistaltic bowel sounds . The ana3After thorough clinical evaluation, the infant was made to undergo several radiological investigations like USG (whole abdomen) & an infantogram. The patient’s perspective included a thorough holistic care in terms of surgical care, genetic & chromosomal typing,regular follow up to treatment adherence & tolerability.USG of whole abdomen revealed dilated large bowel loops with collapsed small bowel associated with decreased peristalsis & normal vascularity suggestive of intestinal obstruction.X- ray plain picture abdomen revealed dilated bowel loops in the centre with absence of gas shadow in the pelvis . In blooThe patient’s mother was a case of gestational diabtetes mellitus & was put on regular insulin. There was no significant family or genetic history. The mother was an elderly primigravida.General examination of the baby revealed severe dehydration, scoliosis & right ectopic kidney.After a thorough clinical, pathological & radiological intervention the infant was planned to be taken up for emergency OT. The procedure was performed under GA. Surgery was lead by the principal author(1st author)of this case report who is an Assistant Professor in the department of general surgery & the corresponding author was the assistant.Surgery revealed small bowel atresia with dilated, blind ending terminal ileum  & 4. TheThe work has been reported in line with SCARE 2018 criteria . The stu4Intestinal atresias are the most common cause of intestinal obstruction in infants comprisning of approximately 22.4% . ImportaInfants may present with abdominal distension, billous vomiting, inability to pass meconium with severe dehydration & electrolyte imbalance. They usually present during the 1st week of life but majority of the babies presenting late have significant mortality .The mortality associated with neonatal intestinal obstruction ranges between 21% and 45% in developing countries, unlike less than 15% in Europe .Early surgical intervention with excision of the dilated segment of the bowel with anastomosis helps in salvaging the complications associated with it. Associated conditions like sepsis, hypothermia, nutrition & wound care needs to be taken care of . Early eIn this case the infant was discharged on 12th post operative day after initiation of oral feeds & passage of stools. Considering the history of GDM in the mother & the associated congenital anomalies in the infant, he was subjected to chromosomal & genetic typing at a higher centre due to limitation of facilities at our setup; post operatively. The parents were advised for monthly follow up for six months. The infant had tolerated the surgery well & was thriving well.5It has been observed that one of the many causes for neonatal intestinal obstruction is attributed to small bowel atresia. These cases of small bowel atresia may have associated congenital anomalies, more so if there is an underlying antenatal morbidity in the mother. Hence a holistic care in terms of genetic & chromosomal typing along with surgery is necessary. Early surgical management of the case with resection anastomosis with early initiation of enteral feeding has been associated with successful outcome. The degree of success depends on the presentation of the infant to the hospital to the timing of surgical intervention. Involvement of other superspecialities has a cornerstone in the development & the determining the outcome of surgical procedure insuch infants having other associated congenital anomalies.No conflict of interest including employment, consultancies, stock ownership, patent applications, grants or other fundings.No funds for the research. Since it was a case report (single participant) I bore the expenses.Since the study was carried out at the time of covid pandemic in India in the month of April 2020 & as the infant had to undergo emergency exploratory laparotomy with resection anastomosis of the bowel, the members at the institutional ethical committee had no meeting in view of the pandemic & had to told to go on with the study since there was no objection raised when mailed to them. Hence no ref no was attached.Written informed consent was obtained from the patient & his parents for the surgical procedure & for the publication of this case report along with the accompanying images. A copy of written consent is available for review by Editor-in-Chief of this journal on request.Pinak Pani Dhar- Conceptualisation, operative procedure, supervision, editing, visualisation, validation.Upasana Mohanty- Conceptualisation, methodology, data entry, writing the manuscript, editing, data analysis & interpretation.1.Name of the registry: Research Registry.2.Unique identifying number or registration ID: researchregistry5588.3.https://www.researchregistry.com/browse-the-registry#home/.Hyperlink to your specific registration (must be publicly accessible and will be checked): Dr Upasana Mohanty.Not commissioned, externally peer-reviewed.


The aim of this article is to report a rare case in which a patient presented symptomatic silicon oil brain migration, documented by MRI, several years after vitreoretinal surgery.This is a case report with a prospective literature review.The patient described in the case report.Case report.For several years, silicone oil (SiO) has been widely used as a long-term intravitreal tamponading agent to treat complex retinal detachments. There are rare reports in the literature demonstrating the migration of SiO into the brain. The aim of this article is to report a rare case in which the patient presented severe headaches several years after vitreoretinal surgery, with migrated SiO appearing in MRI as an oval lesion within the horn of the right lateral ventricle. To the best of our knowledge, there are very few reports of symptomatic SiO brain migration in the literature. Silicone oil (SiO) was first described as an intraocular tamponade for retinal detachment in 1962 by Cibis and colleagues .Infiltration of SiO into the retrolaminar optic nerve was first demonstrated pathologically by Ni et al. in 1983 . PosteriAlthough it is a rare complication, SiO migration is described in the literature as a benign and non-symptomatic incidental radiographic finding . We repoA 67 years old white woman related severe headaches, dating from one month ago. She had no other symptoms and no family history of migraines or glaucoma as well. A pars plana vitrectomy in OD with SiO tamponade (5000 cSt) was performed in 2016 due to a tractional-rhegmatogenous retinal detachment caused by proliferative diabetic retinopathy. The patient evolved with intraocular hypertension and glaucoma in the same eye, being later submitted to a trabeculectomy (2018) and to a cyclophotocoagulation in 2019.Goldmann’s applanation tonometry. The posterior segment examination showed the presence of SiO and a total excavation of the optic disc with intense optic disk pallor in OD. There was an inferior retinectomy associated with subretinal PVR next to inferior vascular arcades. In the left eye, there was a physiological optic disc cupping and a PDR.The patient had a vision of no-light perception in OD and 20/50 in OS (+ 3.00 −1.75 × 90). On the slit-lamp biomicroscopy examination of OD, the patient was pseudophakic with a significant posterior capsule opacification, while the left eye was unremarkable. The intraocular pressure was 20\16 mmhg by Magnetic resonance imaging (MRI) was performed, demonstrating an ovoid lesion within the frontal horn of the right lateral ventricle. The T2 weighted images showed migrated SiO in the horn of the lateral ventricle, and it can be seen in Figs. The migration of intravitreal silicone oil into the cerebral ventricles remains a rarely described phenomenon, and it may contribute to the misinterpretation of other brain lesions, such as ventricular hemorrhage, colloid cyst, and calcifications , 10. We The predominant location of migrated SiO seems to be in the lateral ventricles, which is described to be most likely asymptomatic, compared with the third or fourth ventricles. Intraventricular SiO droplets are described to potentially block the cerebrospinal fluid outflow and temporarily raise intracranial pressure. We hypothesize that this could explain the severe headaches episodes presented by our patient .There are no clear indications for neurosurgical intervention to attempt intraventricular silicone removal in asymptomatic patients, even with radiographic findings, since it is described as a benign finding by several authors. Symptomatic patients are rarely described, and there is no clear consensus about the management in these cases. Close follow-up and analgesia may be reasonable in stable cases; however, there are reports of migrated SiO that caused elevated intracranial pressure, in which the headaches could only be managed with ventricularperitoneal shunt –14.The radiographic appearance of intraocular SiO is distinct from other substances by MRI. In comparison to normal vitreous, SiO is hyperintense on T1-weighted images, but it has variable intensity on T2-weighted images, an effect that has been attributed to differences in oil viscosity, degree of emulsification, and MRI sequence parameters. An MRI scan in the prone position may be used to document the buoyancy of silicone due to its lower density compared to the cerebral spinal fluid, which may aid in the differential diagnosis .Previous studies tried to investigate the frequency of silicone oil extraocular migration since the prevalence of this phenomenon is not well established. Grzybowski and colleagues evaluated 19 patients with MRI at a mean of 115 days after silicone oil tamponade, demonstrating no signs of extravasation . HoweverAs final considerations, the present case illustrates the importance of considering this diagnosis when encountering abnormal neuroimaging findings in a patient with a pertinent history of previous vitreoretinal surgeries. A wider knowledge of this phenomenon and intracranial silicone’s radiographic appearance may avoid unnecessary prolonged hospital stays and neurosurgical interventions.


The coexistence of giant cell tumor (GCT) and metatarsal bone tuberculosis (TB) of the foot has not been reported in the literature so far. We report a case of a 25-year-old male who presented with severe pain and swelling of his left foot for two months, which was aggravated on walking. A plain radiograph of the left foot showed an expansile eccentric lytic lesion of the base of the second metatarsal. He underwent extended curettage and antibiotic cement spacer insertion. Biopsy of the lesion revealed the presence of GCT, while tissue cultures were positive for Mycobacterium tuberculosis. He was treated with standard anti-tubercular treatment (ATT), four drug regimens for twelve months. He then underwent reconstruction of the second metatarsal with cement spacer exit and iliac crest bone grafting, following which the cultures were negative for TB. The diagnosis of this unexpected and unique combination of pathologies (GCT and TB) depends on a high index of clinical suspicion, relevant investigations, and accurate histological diagnosis. Giant cell tumor (GCT) of bone is a benign but locally aggressive tumor with a recurrence rate of up to 50% , 2. The Osteoarticular TB makes up to less than 3% of the cases of active pulmonary TB, of which about 10% involve foot and ankle . Foot TBIn this case report, we present the first observation of the coexistence of GCT and tuberculosis of the metatarsal bone.A 25-year-old male presented with a two-month history of pain and swelling of the left foot to Christian Medical College and Hospital, Vellore, India. The swelling was insidious in onset, progressive, associated with pain and difficulty in walking. There was no past or family history of tuberculosis. On examination, he had tenderness and diffuse swelling over the left second ray. There were no associated skin changes. A plain radiograph of the left foot showed an expansile lytic lesion of the metaphyseo-diaphyseal region of the base of the second metatarsal bone Figure . Bone scSurgical procedureStaged treatment was planned for the patient. In the first stage, he underwent excision biopsy of the lesion, extended curettage with speed burr, and chemical cauterization with 80% phenol. The curetted area was filled with antibiotic-loaded bone cement consisting of 1 gram each of vancomycin and meropenem Figures -3. PerioThe second stage of treatment was started after the completion of the full course of anti-tubercular therapy. The metatarsal base was again exposed through the previous incision, and the cement bead was removed. Swabs were taken from the area and sent for cultures. The defect at the metaphyseo-diaphyseal part was measured, and the cortico-cancellous graft was taken from the iliac crest and press-fitted into the defect. Cultures were negative for Mycobacterium tuberculosis.After surgery, the patient was given a below-knee cast and started on weight-bearing as tolerated. The patient was being followed up in the outpatient department (OPD) regularly for three years, and the radiographs showed complete integration of the bone graft without any recurrence to date Figures -5.The existence of GCT or tuberculosis of the metatarsal bone by itself is very rare. However, both are considered as a differential diagnosis for lytic lesions of the bone in the extremities. Other differential diagnoses are an aneurysmal bone cyst, Brown's tumor, non-ossifying fibroma, chondroblastoma, Langerhans cell histiocytosis, and chronic osteomyelitis , 8-10.The co-existence of GCT and tuberculosis has been reported only in one case report by Charach et al. where the authors have reported this unusual co-existence in the metacarpal bone. In this report, the diagnosis was obtained after an open biopsy, and antitubercular therapy was given for a period of eight months, after which the patient underwent curettage of the metacarpal .Our patient had a lytic lesion on the metaphyseo-diaphyseal region of the left second metatarsal, which was eccentric and had a breach of cortex on the lateral side. Extended curettage was done, and the gap was filled with antibiotic-loaded bone cement (ALBC). We proceeded with the metatarsal reconstruction only after completion of one year of anti-tubercular therapy and periodic evaluation of inflammatory markers and radiographs to check the resurgence of infection. We were aggressive in doing extended curettage during the first stage of treatment as our first differential diagnosis was GCT. On approaching the lesion, there was minimal granular tissue, because of which we added ALBC instead of adding bone graft after the curettage. Our patient had been followed up for three years now and has no signs of recurrence of either the GCT or infection Figure . His AmeWe add to the literature the case of co-existence of GCT and tuberculosis in lytic lesions and hope that our management supplements the treatment principles in these types of patients.The rare combination of GCT and infection should be kept in mind while evaluating lytic lesions in the extremities. Tubercular, bacterial, and fungal cultures should always be sent along with the biopsy specimen for a complete evaluation.


Pharyngeal arch anomalies are the second most common form of head and neck congenital defect. The second arch anomalies are the most common, and compromise 95% of cases. Little is known about the 3rd and 4th arch anomalies as they are extremely rare. They most commonly present in childhood with sudden severe left lateral neck infection and abscess formation with considerable tendency to recur, contributing to significant mortality and morbidity in those patients.Here we present four cases finally diagnosed as third or fourth pharyngeal arch anomalies, with more than 20 years of follow-up following their definitive surgery. The possibility that they are thymopharyngeal duct remnants is discussed.Meticulous open radical surgical excision of all involved paralaryngeal, parapharyngeal and thyroid tissue, with preservation of the superior and recurrent laryngeal nerves, is required for cure of recurrent cases. Pharyngeal arch anomalies are the second most common form of head and neck congenital defect. In most reported series 2nd arch anomalies comprise approximately 95% of cases and 1st arch anomalies 3–4% [The cranial region of the early human embryo during the third week of development resembles more primitive forms of life, such as a fish embryo at the same stage. The study of the branchial apparatus by embryologists in other life forms has been, and indeed remains, essential for our understanding of its function. The term branchial (the Greek term “branchia” means gill) remains in common usage. However, humans do not develop gills and many authors now prefer the term pharyngeal arch.The embryonic period of human development continues up to 8 weeks. Between the fourth and eight weeks the ancestral branchial apparatus becomes totally rearranged and adapted to new structures and functions, or alternatively disappears. It is during this complex transformation period that most congenital malformations occur. One third of all human congenital malformations occur in the head and neck . The strThe comprehensive history, diagnosis, radiology details and open surgery operative details were prospectively kept in this rare cohort of patients. The same consultant surgeon treated and operated on all four cases and continued their long-term follow up at the Royal National Throat, Nose and Ear Hospital, London, UK.Four cases of 3rd or 4th pharyngeal arch anomalies were followed-up and treated. Three of these patients were male, and one female. Three cases had become initially symptomatic in childhood and all of them presented as acute left sided neck infections, without signs of a sinus or fistula. Further recurrent infections were also left sided. Fistulas only developed after spontaneous rupture of the neck abscess and incision and drainage procedures. Three of these patients had considerable life changing, long-term morbidity.This female patient did not primarily present until 24 years of age. She had an acute painful, left neck swelling which was diagnosed as viral thyroiditis. She was treated with steroids and antibiotics for 1 month. Her symptoms and left neck swelling recurred after 14 months and she underwent left thyroid lobectomy. Only 1 month later, she required incision and drainage of a new left neck abscess. Between 28 and 34 years of age she had three further left neck abscesses requiring incision and drainage. At 35 years of age, she re-presented with a new acute left neck swelling. MRI, thyroid scans and pharyngoscopy did not demonstrate an internal piriform sinus opening. At left neck exploration, a mass of fibrous tissue, fascia, scarred left sternothyroid muscle and residual tract was excised. The tract was demonstrated adjacent to the left cricoid cartilage and crico-thyroid muscle, 3–4 mm anterior to the inferior thyroid cornu and left recurrent laryngeal nerve. All involved tissue was removed with additional operating microscope control. At 25-year follow-up she had no further recurrence of her previous extensive problems.This male patient initially presented at 4 years of age with a left neck swelling which was treated with antibiotics with several further episodes between the ages of 5 and 8 years, at which point he developed a discharging left neck sinus. Ongoing infections with multiple operations caused severe disruption to his education. At 25 years of age, investigations including microbiology, CT, examination under anesthesia and pharyngoscopy did not show an internal piriform sinus opening. At left neck exploration substantial amounts of peri thyroid scar tissue and residual left thyroid lobe were excised. A further abscess drainage was undertaken 3 months later. Between 25 and 33 years of age the patient had intermittent pus draining from a left mid-neck sinus with recurrent infection. During this period he also had a left tonsillectomy and neck exploration with no subsequent improvement.At age 33 a pharyngoscopy did not demonstrate an internal opening from the piriform fossa. The neck was again re-explored and all gross scar tissue and involved fascia was excised medial to the deep aspect of sternomastoid muscle, around the carotid sheath and along the posterior border of the left thyroid cartilage. There was an obvious tract attached to the inferior thyroid cartilage border adjacent to the inferior cornu and left recurrent laryngeal nerve. All tract and involved periosteuum and a small portion of the cricoid and thyroid cartilage were removed, under operating microscope control, to ensure that all abnormal tissue was excised. The left sternomastoid muscle was released inferiorly and transposed into the excised area to aid healing. The patient remained well without recurrent symptoms for 26 years but experienced a single episode of neck swelling in 2018, which settled with antibiotics.This teenage boy presented at 14 years of age, having had two episodes of left neck pain and swelling. He was initially diagnosed as having viral thyroiditis. Three weeks later, there was a sudden deterioration with formation of a large bilateral neck abscess, maximal on the left side. This was incised and drained and he spent 6 days as an inpatient. Subsequent ultrasound and barium swallow showed a 4 cm long sinus tract extending postero-medially to the left lobe of the thyroid gland demonstrated in Fig. This patient first presented at 9 years of age, with a left neck abscess which required incision and drainage. Twenty-one years later, at the age of 30, he had a new left neck swelling which settled with antibiotics. Aged 39 he presented with a large left neck abscess which required incision, drainage and antibiotics. Pharyngoscopy 1 year later did not reveal any piriform fossa internal opening. At elective left neck exploration, all scar tissue, abscess cavity remnant, involved left sternothyroid and inferior constrictor muscle adjacent to the left piriform fossa mucosa was excised. Thyroid lobectomy was not required. The patient remains well after 27-year follow-up.Long-term follow-up, of more than two decades, has shown probable cure of all the patients following open surgery. Their presentations are in line with the published literature, where more than 95% are left sided, almost 100% present with neck infection [Internal openings in the apex of the piriform fossa may be clearly defined, as presented in Case 3, but after multiple infections and surgery, are not always found. Surgeons continue to explore all possible ways to carry out procedures endoscopically with consequent reduction in morbidity and inpatient stay. Lachance & Chadha (2015), carried out a systematic review of endoscopic obliteration techniques in children and concluded that it was successful in 90% of primary cases and 87% of revision cases . It is uThe pathology of the excised tissue in these patients had notable epithelial remnants, thyroid tissue, chronic inflammation and scarring. Cases 1, 2 and 3 showed definite tracts ending at the lower border of the thyroid cartilage and adjacent to the cricothyroid muscle, closely related to the laryngeal point of entry of the recurrent laryngeal nerve. Embryology textbooks describe pharyngeal arch development in humans occurring as a series of paired arches in the embryo . Every aAn alternative embryological explanation for these clinical cases has been proposed, suggesting that they are persisting remnants of the thymopharyngeal duct . This duWhilst no patients have been reported in the literature showing the theoretical extensive classical path of a complete 4th pharyngeal arch anomaly, rare cases such that of Godin et al. , have shThe inferior constrictor muscle must be divided to allow high ligation of the tract at any piriform fossa internal opening. Additional careful dissection inferiorly to the inferior cornu of the thyroid cartilage and cricoid cartilage, with preservation of the recurrent laryngeal nerve, may be necessary, as described with use of the operating microscope aiding this dissection. Healthy sterno-mastoid muscle can be transposed into the defect to aid healing.The main strengths of this case-series is the length of the follow-up, confirming success in surgical management of this condition. Although, it provides the supplemental opinion to embryological developments of the third and fourth branch anomalies, the detailed surgical technique has not been discussed here. The rarity of the cases and the uncertainty of embryological development of these arches, makes this report not a definitive or conclusive word on the matter. Moreover, the literature describing the long-term follow-up following the endoscopic treatment of these anomalies is not available as of yet. However, it would be important to address the comparatives of these different techniques in the future.In cases of multiple and recurrent neck abscesses a diagnosis of 3rd or 4th arch anomaly should be considered. To achieve definitive management these cases require detailed dissection from the piriform fossa infero-laterally to include all involved tissue adjacent to the carotid sheath, left thyroid lobe, thyroid and cricoid cartilages with preservation of a recurrent laryngeal nerve.Modern embryological research suggests that development of the pharyngeal apparatus is a truly complex process that relies on genetic signaling from the endoderm, ectoderm and mesoderm that control proliferation, migration and morphology. A full understanding of these pathways requires more work to elucidate the many congenital anomalies of the pharyngeal apparatus . Recent


Fibrous hamartoma of infancy (FHI) is a rare benign soft-tissue tumor in children with a triphasic organoid histologic appearance.We here report a case with the largest FHI published so far. A 13-month-old boy with a rapidly growing tumor 45 cm in length and 69.3 cm in circumference of the left lower extremity was identified immediately at birth.The diagnosis was uncertain before the operation, although biopsy was conducted. The postoperation histological examination showed arranged spindle cells, adipose tissue, and nests of immature small cells embedded in a myxoid background, which is the characteristic triphasic histology of FHI.Under general anesthesia, hip and lower extremity amputation was performed.The patient was followed-up for 6 years and no signs of recurrence were found, suggesting a favorable prognosis, although a part of the residual tumor was present after the surgery.FHI exhibits pathological and clinical characteristics. However, differential diagnosis of FHI from other soft-tissue tumors before operation remains a challenge. Thus, sometimes, aggressive therapy for the treatment of FHI might be a good choice to get a satisfactory. FHI is a benign mesenchymal lesion of myofibroblastic origin with prototypical triphasic histopathological features and mesenchymal, fibrous composition. Characteristic fatty tissue elements can be observed in variable proportions, in the absence of anaplasia and/or mitoses.Fibrous hamartoma of infancy (FHI) was initially defined as “subdermal fibrous tumor of infancy” by Reye in 1956.,5 The disease may occur in various tissues of the body; it is most frequently localized in the upper extremities, axilla, shoulders, groin, upper trunk and sacral regions.–11 Sometimes, it is so difficult to differentiate FHI from other soft tissue tumors before surgery that we cannot obtain appropriate treatment option.FHI typically occurs in the first 2 years of life, predominantly in males, and the male/female ratio is 2.0 to 2.4:1.We herein present a case of FHI that the lesion size of this being one of the largest reported so far. The 6 years’ follow-up shows that FHI may not recur even though the resection of the tumor is not complete.2A 13-month-old boy presented to our orthopedic department due to prominent increased diameter and length of the left lower extremity that presented at birth. Physical examination showed a painless, hard, giant left lower extremity, 45.0 cm in length and 69.3 cm in circumference, which was much bigger and longer than the right lower extremity Fig. A. ComputNo postoperative complications occurred. At the 6-year postoperative follow-up, the patient recovered well and has a satisfactory life using an artificial left lower extremity, and no signs of tumor recurrence according to the results of physical examination and MRI.3,4 As shown in the literature, most of the FHI are slow-growing, but it may also present as a rapid growing mass with signs of warmth, tenderness, and pain.–5 Our case presented a painless, hard, no warmth, and rapid growing mass. Overlying hyperpigmentation, hyperhidrosis, and/or hypertrichosis have also been reported, but these signs are not presented in our case. Most of the lesions are small and solitary, the size ranging from 0.5 to 9.0 cm.–12 The largest tumor diameter reported in the literature is 20 cm in a 7-year-old Nigerian girl. However, her diagnosis of FHI was questioned. In our case, the tumor size was larger than 29 cm in diameter, which is the largest FHI reported to date.The children affected by FHI typically present with no specific symptoms. A sign of fibrous tissue trabeculae interspersed with fat in an organized pattern helps narrow down the differential diagnosis, which is strongly suggestive of FHI. Immunohistochemistry can assist the identification of the tumor origin. The histological staining of our case demonstrated mature adipose tissue intersected bands of dense fibrous tissue rich in myofibroblasts and collagen, and nests of immature mesenchyma in a myxoid background. Although differential diagnosis of FHI is difficult, the histological manifestations of our case meet the histological diagnosis criteria for FHI described above. Positive immunohistochemical staining for SMA and CD34 proteins was noted in our case, which support the diagnosis of FHI.The main challenge for us is to differentiate FHI from other soft-tissue tumors such as neurofibrimas, vasvular tumors, juvenile fibromatosis, and sarcoma, especially when the tumor is hard and fixed to the deep planes. A careful histological examination by routine hematoxylin-eosin staining can provide sufficient indications for a correct diagnosis. The histological criteria for diagnosis of FHI included the presence of well-defined bundles of dense, intersecting fascicles of fibroblasts/myofibroblasts in the collagenous stroma, and the nests of immature oval or stellate cells in the basophilic mucoid stroma, admixed with mature adipose tissue. The immature component does not harbor significant atypia, pleomorphism, and/or aggressiveness. reported that tyrosine kinase inhibitors may be used as adjunctive therapy in the case of resection is particularly difficult. Repeated and nondestructive surgery is suggested to medical practitioners. Local and conservative excision is usually conducted notably in symptomatic patients and the surgical procedures are often curative.,5 Majority of the studies indicated that aggressive, mutilating resections should be avoided due to the excellent overall prognosis and low recurrence rate, although some studies recommended local radical excision in order to prevent accelerated spread of the tumor. In the present report, a treatment strategy of destructive amputation of the left hip and the left lower extremity was forced to be selected because of the giant size, infiltration, rapid growth of the tumor, and the immobility of the left leg. Now, the patient can walk and play like a normal child with the help of artificial extremity. The tumor did not recur after 6 years of follow-up. It has been reported that the postoperative recurrence incidence is 16.7% in cases of giant fibrous hamartomas at 42 months following primary excision. Typically, a gap between the normal tissue and the periphery of tumor should be taken to prevent the spread of the tumor cells.,4 McGowan et al reported 2 cases of FHI. Positive initial resection margins in one case correlated with rapid postoperative recurrence, whereas complete excision with a 0.5-cm margin in the second case did not result in recurrence of the tumor. However, Agrawal et al found that the margin status may not influence the propensity of clinical recurrence. The result of our case is consistent with that of Agrawal et al. So far, no recurrence has been found, although the tumor has not been completely resected. The follow-up period in our case is short and a longer follow-up is required, as previous studies have reported an FHI recurrence 14 years after the primary excision. No metastases, histological regression, involution, and/or malignancy have been reported for FHI to date.The preferred treatment for FHI is complete local resection. Park et alFHI exhibits its special pathological and clinical characteristics. The differential diagnosis of FHI from other soft tissue tumors before operation remains a challenge, which is what we should pay attention to. Sometimes, we have to choose aggressive therapeutic approaches for the treatment of FHI when the tumor is very big and deep to get a satisfactory life quality.We are grateful for unrestricted support and enthusiasm of the families involved in this report. This manuscript is dedicated to them, who with all adversity never lost their hope.Conceptualization: Lihua Zhao.Data curation: Sun Wang, Qichao Ma, Qin Jiao, Bin Zhang.Formal analysis: Hao Ying.Investigation: Lihua Zhao, Sun Wang.Methodology: Lihua Zhao, Sun Wang.Resources: Hao Ying, Lihua Zhao.Validation: Hao Ying, Lihua Zhao.Visualization: Bin Zhang.Writing – original draft: Qichao Ma, Lihua Zhao, Qin Jiao.Writing – review & editing: Lihua Zhao, Qichao Ma.