POEMS syndrome is a rare disease caused by monoclonal plasma cell proliferative disorder.The typical signs include peripheral neuropathy,organ enlargement,endocrine disease,M proteinemia,and skin changes.In clinical practice,the atypical,complex,and changeable clinical manifestations of this syndrome can easily lead to misdiagnosis and missed diagnosis.A case of POEMS syndrome with peripheral edema and ascites as the main manifestations is reported in this paper.
Humans ; Ascites/etiology* ; POEMS Syndrome/diagnosis* ; Edema/diagnosis* ; Skin

Objective: To summarize the clinical features, management and outcome of superior vena cava syndrome (SVCS) associated with mediastinal malignancy in children. Methods: Clinical data of 42 children of SVSC associated with mediastinal malignancy in Shanghai Children's Medical Center from January 2015 to December 2021 were collected and analyzed retrospectively. The clinical manifestations, pathological diagnosis, disease diagnosis process, and prognosis were summarized. Results: Among 42 children of SVCS associated with mediastinal malignancy, there were 31 males and 11 females. The age at diagnosis was 8.5 (1.9, 14.9) years. Cough and wheezing (33 cases, 79%), orthopnea (19 cases, 45%) and facial edema (18 cases, 43%) occurred most commonly. T-cell lymphoblastic lymphoma (T-LBL) was the most frequent pathological diagnosis (25 cases, 60%), followed by T-cell acute lymphoblastic leukemia (T-ALL) (7 cases, 17%), anaplastic large cell lymphoma (4 cases, 10%) and diffuse large B-cell lymphoma (2 cases, 5%), peripheral T-lymphoma, Hodgkin lymphoma, Ewing's sarcoma and germ cell tumor (1 case each). Pathological diagnosis was confirmed by bone marrow aspiration or thoracentesis in 14 cases, peripheral lymph node biopsy in 6 cases, and mediastinal biopsy in 22 cases. Twenty-seven cases (64%) had local anesthesia. Respiratory complications due to mediastinal mass developed in 3 of 15 cases who received general anesthesia. Of the 42 cases, 27 cases had sustained remission, 1 case survived with second-line therapy after recurrence, and 14 cases died (2 cases died of perioperative complications and 12 cases died of recurrence or progression of primary disease). The follow-up time was 36.7 (1.2, 76.1) months for 27 cases in continuous complete remission. The 3-year overall survival (OS) and events free survival (EFS) rates of 42 children were 59% (95%CI 44%-79%) and 58% (95%CI 44%-77%) respectively. Conclusions: SVCS associated with mediastinal malignancy in children is a life-threatening tumor emergency with high mortality. The most common primary disease is T-LBL. The most common clinical symptoms and signs are cough, wheezing, orthopnea and facial edema. Clinical management should be based on the premise of stable critical condition and confirm the pathological diagnosis through minimal invasive operation.
Child ; China ; Cough ; Edema ; Female ; Humans ; Male ; Mediastinal Neoplasms/diagnosis* ; Precursor T-Cell Lymphoblastic Leukemia-Lymphoma ; Respiratory Sounds ; Retrospective Studies ; Superior Vena Cava Syndrome/therapy*

PURPOSE: To report a case of Epstein-Barr virus-related corneal endotheliitis accompanied by secondary glaucoma.CASE SUMMARY: A 73-year-old male presented with blurred vision in his right eye. In the ophthalmic evaluation, there were dispersed keratic precipitates overlying corneal edema. The anterior chamber showed trace ~1+ graded inflammation and an endothelial density decrease. His best-corrected visual acuity and intraocular pressure in the right eye were 0.2 and 34 mmHg, respectively. Paracentesis was performed on the anterior chamber of the right eye to confirm the diagnosis under the suspicion of corneal endotheliitis with trabeculectomy for the intraocular pressure control. Epstein-Barr virus was confirmed using a multiplex polymerase chain reaction (PCR), and oral and eye drops of Acyclovir were used to treat the patient. There was no evidence of a recurrence over 2 years and his intraocular pressure was 12 mmHg and best-corrected visual acuity was maintained at 0.5.CONCLUSIONS: A case of Epstein-Barr virus-related corneal endotheliitis was diagnosed using PCR of the aqueous humor. The patient was treated with an oral antiviral agent and eyedrops without a recurrence.
Acyclovir ; Aged ; Anterior Chamber ; Aqueous Humor ; Corneal Edema ; Diagnosis ; Glaucoma ; Herpesvirus 4, Human ; Humans ; Inflammation ; Intraocular Pressure ; Male ; Multiplex Polymerase Chain Reaction ; Ophthalmic Solutions ; Paracentesis ; Polymerase Chain Reaction ; Recurrence ; Trabeculectomy ; Visual Acuity

To investigate the computed tomography (CT) characteristics and differential diagnosis of high altitude pulmonary edema (HAPE) and COVID-19, CT findings of 52 cases of HAPE confirmed in Medical Station of Sanshili Barracks, PLA 950 Hospital from May 1, 2020 to May 30, 2020 were collected retrospectively. The size, number, location, distribution, density and morphology of the pulmonary lesions of these CT data were analyzed and compared with some already existed COVID-19 CT images which come from two files, "Radiological diagnosis of COVID-19: expert recommendation from the Chinese Society of Radiology (First edition)" and "A rapid advice guideline for the diagnosis and treatment of 2019 novel corona-virus (2019-nCoV) infected pneumonia (standard version)". The simple or multiple ground-glass opacity (GGO) lesions are located both in the HAPE and COVID-19 at the early stage, but only the thickening of interlobular septa, called "crazy paving pattern" belongs to COVID-19. At the next period, some increased cloudy shadows are located in HAPE, while lesions of COVID-19 are more likely to develop parallel to the direction of the pleura, and some of the lesions show the bronchial inflation. At the most serious stage, both the shadows in HAPE and COVID-19 become white, but the lesions of HAPE in the right lung are more serious than that of left lung. In summary, some cloudy shadows are the feature of HAPE CT image, and "crazy paving pattern" and "pleural parallel sign" belong to the COVID-19 CT, which can be used for differential diagnosis.
Altitude ; COVID-19/diagnostic imaging* ; China ; Diagnosis, Differential ; Humans ; Lung/diagnostic imaging* ; Pulmonary Edema/diagnostic imaging* ; Retrospective Studies ; Tomography, X-Ray Computed

Primary central nervous system vasculitis (PCNSV) is rare, and the diagnosis is difficult to make because of its variable radiologic expressions. Early corticosteroid therapy often is effective. Herein we report the case of a 56-year-old man who had a well-enhanced cystic mass with severe edema in the right frontal lobe, which was initially felt to be a malignancy. Histologic examination of tissue removed at craniotomy revealed that it was a PCNSV. Despite early administration of corticosteroids, a new lesion developed within 3 days. The lesions responded to treatment with cyclophosphamide and corticosteroid.
Adrenal Cortex Hormones ; Craniotomy ; Cyclophosphamide ; Diagnosis ; Edema ; Frontal Lobe ; Glucocorticoids ; Humans ; Middle Aged ; Vasculitis ; Vasculitis, Central Nervous System

PURPOSE: To report a case of frosted branch angiitis (FBA) associated with Epstein-Barr virus (EBV) infection in a child. CASE SUMMARY: A 7-year-old boy presented with bilateral blurred vision. On ophthalmic examination, his best-corrected visual acuity was 20/25 in the right eye and 20/32 in the left eye. The pupils were equal, round, and reactive to light without a relative afferent pupillary defect. He had normal color vision in both eyes. Slit-lamp examination revealed no abnormalities in the anterior parts of the eyes. Fundoscopic examination revealed prominent white sheathing retinal vasculitis predominantly on the veins in all quadrants, as well as macular edema and irregular foveal reflex in both eyes. Fluorescein angiography showed normal blood flow, but late diffuse staining and leakage of the affected vessels. Spectral domain optical coherence tomography (SD-OCT) showed thickening of the vessel walls, swelling due to hyperreflective material, and hyperreflective retinal depositions. Serological tests and the serum polymerase chain reaction for EBV were positive. A diagnosis of FBA associated with EBV was made. He was treated with systemic acyclovir and steroids. The response was rapid, with improvement in visual acuity to 20/20 in both eyes by day 3. After 7 weeks, all clinical signs resolved and SD-OCT examination showed normal vessel wall thickness and the absence of hyperreflective depositions. CONCLUSIONS: EBV may present with FBA even in the absence of a systemic sign of primary EBV infection. Thus, EBV should be considered as the etiology of FBA.
Acyclovir ; Child ; Color Vision ; Diagnosis ; Epstein-Barr Virus Infections ; Fluorescein Angiography ; Herpesvirus 4, Human ; Humans ; Macular Edema ; Male ; Polymerase Chain Reaction ; Pupil ; Pupil Disorders ; Reflex ; Retinal Vasculitis ; Retinaldehyde ; Serologic Tests ; Steroids ; Tomography, Optical Coherence ; Vasculitis ; Veins ; Visual Acuity

PURPOSE: We report a case of Aeromonas keratitis presenting as radial keratoneuritis. CASE SUMMARY: A 33-year-old woman with a history of cleaning her contact lenses with tap water presented with decreased visual acuity for 1 day in the left eye. The patient showed diffuse corneal edema, stromal infiltration, and radial keratoneuritis, which were thought to be pathognomonic for Aeromonas keratitis. Based on the patient's clinical findings and past history, a diagnosis of Aeromonas keratitis was made and she was prescribed topical fortified cefazolin (50 mg/mL, 5%), tobramycin (3 mg/mL), and 0.02% chlorhexidine per hour. Culture results from the contact lens and contact lens solution confirmed infection by Aeromonas hydrophilia. Polymerase chain reaction results for Aeromonas were negative. After 8 days of treatment, the uncorrected visual acuity was 0.7/0.3 with improvement in her corneal findings. CONCLUSIONS: Radial keratoneuritis is not always pathognomic for Aeromonas keratitis and can be present in Aeromonas keratitis. Therefore, ophthalmologists should be cautious when interpreting this clinical sign.
Adult ; Aeromonas ; Cefazolin ; Chlorhexidine ; Contact Lens Solutions ; Contact Lenses ; Cornea ; Corneal Edema ; Diagnosis ; Female ; Humans ; Keratitis ; Polymerase Chain Reaction ; Tobramycin ; Visual Acuity ; Water

PURPOSE: We report a case of acute dacryocystitis diagnosed with abscess and rupture of lacrimal sac and fistula to posterior orbit during the operation. CASE SUMMARY: A 71-year-old woman visited our clinic with edema and pain in the eyelid from three days ago. For past four months, there was viscous of the left eye and tears. The patient had severe conjunctival chemosis and hyperemia, compared with the left eyelid edema and redness. Orbital CT scan showed orbital cellulitis, which was followed by systemic antibiotics and steroid therapy. On the 4th day of therapy, orbital abscess formation was observed in orbit MRI and surgical drainage was planned. During surgery, we found rupture of the posterior part of lacrimal sac and fistula to posterior orbit. Pseudomonas aeruginosa was identified in the bacterial cultures, and after the administration of appropriate antibiotics, the disease showed improved progress, and then additional dacryocystorhinostomy was performed. CONCLUSIONS: In our case, acute dacryocystitis rarely spread in orbit, which may lead to delayed diagnosis, orbital cellulitis and abscess, resulting in serious complications of vision threat. So, we think that it is necessary to consider surgical treatment more actively in the stage of chronic dacryocysitis.
Abscess ; Aged ; Anti-Bacterial Agents ; Dacryocystitis ; Dacryocystorhinostomy ; Delayed Diagnosis ; Drainage ; Edema ; Eyelids ; Female ; Fistula ; Humans ; Hyperemia ; Magnetic Resonance Imaging ; Nasolacrimal Duct ; Orbit ; Orbital Cellulitis ; Pseudomonas aeruginosa ; Rupture ; Tears ; Tomography, X-Ray Computed

PURPOSE: When there is a mass in the superior temporal orbit area, a lacrimal gland tumor should be suspected. We report a rare case of orbital lymphatic malformation that was histologically diagnosed in a patient with typical clinical features of the lacrimal gland. CASE SUMMARY: A 55-year-old female with no underlying disease and no ophthalmic history visited our clinic with a right upper eyelid edema associated with an enlarged painless eyelid mass 1 month prior to her visit. The patient stated that she discovered the mass 1 year previously.The palpebral lobe of the lacrimal gland protruded slightly with congestion of the surrounding conjunctiva. Enhanced computed tomography showed a 3 cm well-defined heterogeneous mass in the right lacrimal gland area and several well-defined round calcifications within the mass. Orbital tissue or bone involvement was not observed. The pleomorphic adenoma of the lacrimal gland was the most clinically suspicious, so complete resection of the mass was performed using lateral orbitotomy. Histopathologically, lymphangioma (lymphatic malformation) originating from the lacrimal gland was diagnosed. CONCLUSIONS: Orbital lymphatic malformation can occur in the lacrimal gland. The present case showed that differential diagnosis can reveal the presence of an adult lacrimal gland tumor.
Adenoma, Pleomorphic ; Adult ; Conjunctiva ; Diagnosis, Differential ; Edema ; Estrogens, Conjugated (USP) ; Eyelids ; Female ; Humans ; Lacrimal Apparatus ; Lymphangioma ; Middle Aged ; Orbit

Choroid plexus tumors are uncommon brain tumors that primarily occur in children. Most of these tumors originate from the intraventricular area, and the most common clinicalpresentation is increased intracranial pressure. Dissemination through the cerebrospinal fluid space is the inevitable natural course of the disease. Here, we present 2 rare cases of adult choroid plexus carcinoma (CPC), each with distinct clinical presentation and progression. The first case was a 40-year-old male who presented with multiple intraventricular masses. After surgical biopsy, radiation and intrathecal chemotherapy failed to elicit any response. The patient progressed with spinal cord dissemination and expired 1 year later. The second case presented with visual disturbance, and brain MRI revealed a large ovoid juxtaventricular mass with peritumoral edema. This 49-year-old female patient underwent craniotomy for what was thought to be a high-grade glioma; however, the mass was connected to the choroid plexus at the operative field. Her pathology specimen was diagnosed as CPC, and adjuvant systemic chemotherapy was administered. She has now been free of recurrence for 10 months. The description of the presentation and progression of these rare adult-onset CPC provides insight for the diagnosis and treatment of other rare instances of choroid plexus tumors.
Adult ; Biopsy ; Brain ; Brain Neoplasms ; Cerebrospinal Fluid ; Child ; Choroid Plexus Neoplasms ; Choroid Plexus ; Choroid ; Craniotomy ; Diagnosis ; Drug Therapy ; Edema ; Female ; Fourth Ventricle ; Glioma ; Humans ; Intracranial Pressure ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Pathology ; Recurrence ; Spinal Cord