Experience of primary intestinal lymphangiectasia in adults: Twelve case series from a tertiary referral hospital

BACKGROUND While primary intestinal lymphangiectasia(PIL)is considered a rare condition,there have been several reported cases in adults.Nevertheless,the absence of clear guidance from diagnosis to treatment and prognosis poses challenges for both physicians and patients.AIM To enhance understanding by investigating clinical presentation,diagnosis,treatment,complications,and prognoses in adult PIL cases.METHODS We enrolled adult patients diagnosed with PIL between March 2016 and September 2021.The primary outcome involved examining the diagnosis and treatment process of these patients.The secondary outcomes included identifying complications(infections,thromboembolism)and assessing prognoses(frequency of hospitalization and mortality)during the follow-up period.RESULTS Among the 12 included patients,peripheral edema(100%)and diarrhea(75%)were the main presenting complaints.Laboratory tests showed that all the pati-ents exhibited symptoms of hypoalbuminemia and hypogammaglobulinemia.Radiologically,the predominant findings were edema of the small intestine(67%)and ascites(58%).The typical endoscopic finding with a snowflake appearance was observed in 75%of patients.Among the 12 patients,two responded positive-ly to octreotide and sirolimus,and eight who could undergo maintenance therapy discontinued subsequently.Complications due to PIL led to infection in half of the patients,thromboembolism in three patients,and one death.CONCLUSION PIL can be diagnosed in adults across various age groups,with different severity and treatment responses among patients,leading to diverse complications and prognoses.Consequently,tailored treatments will be necessary.We anticipate that our findings will contribute to the management of PIL,an etiology of protein-losing enteropathy.

Neuralgic amyotrophy with hourglass-like constrictions:A case report

BACKGROUND Neuralgic amyotrophy(NA)is a rare disease with sudden upper limb pain followed by affected muscle weakness.The most commonly affected area in NA is the upper part of the brachial plexus,and the paraspinal muscles are rarely affected(1.5%),making these cases difficult to distinguish from cervical radiculopathy.CASE SUMMARY A 76-year-old male presented to the emergency department with left hip pain post-fall.After undergoing left femoral neck fracture surgery,he experienced sudden left shoulder pain for 10 days with subsequent left arm weakness.Cervical spine computed tomography revealed mild right asymmetric intervertebral disc bulging with a decreased C5-6disc space.Three weeks later,an electrodiagnostic study confirmed brachial plexopathy findings involving the cervical root.Magnetic resonance neurography was performed for a differential diagnosis.Contrast enhancement was identified at the upper trunk of the brachial plexus,including the C5 nerve root.A suprascapular nerve hourglass-like focal constriction(HLFC)was also identified,confirming NA.After being diagnosed with NA,the patient received 15 mg prednisolone,twice daily,for 3 weeks.Physical therapy was initiated,including left arm strengthening exercises and electrical stimulation therapy.Left shoulder muscle strength significantly improved one CONCLUSION NA's unique features like HLFC and paraspinal involvement are crucial for accurate diagnosis,avoiding confusion with cervical radiculopathy.

Primary renal carcinoid tumor: A rare cystic renal neoplasm

We present the case of a 21-year-old man with an incidentally detected cystic renal mass.A well-defined,solid mass measuring approximately 8 cm x 6 cm with a cystic component was identified in the left kidney by abdominal multidetector computed tomography(CT) and ultrasonography.The mass was well-enhanced on the corticomedullary CT phase and washout of enhancement occurred on the nephrographic phase.The mass contained peripheral wall and septal calcifications in the cystic component.The lesion was resected and diagnosed as a primary renal carcinoid tumor.Primary carcinoid tumors of the kidney are extremely rare.This case is notable because of the rarity of this neoplasm and its unique radiologic and pathologic findings.A review of previously reported cases in the literature is also presented.

Factors associated with strabismus after cataract extraction and primary intraocular lens implantation in congenital cataracts

AIM:To evaluate factors associated with the development of strabismus after cataract extraction and primary intraocular lens implantation.METHODS:The medical records of 122 patients,aged1.5mo to 9y,who had undergone cataract extraction with primary intraocular lens implantation between January1993 and August 2011 were reviewed.Fourteen patients(17 eyes)with strabismus before cataract surgery were excluded.Patients were divided into those with congenital bilateral cataracts(64 patients,128 eyes)and those with unilateral cataracts(44 patients,44 eyes).The associations between the development of strabismus and age at cataract surgery,pre-and post-cataract extraction corrected distance visual acuity(CDVA),interocular CDVA difference,nystagmus,surgical method,and secondary cataract were evaluated.RESULTS:Factors significantly associated with the development of strabismus included age at cataract surgery(≤1y),preoperative mean CDVA≤20/100,presence of nystagmus in the bilateral cataract group and postoperative interocular CDVA difference】20/70 in the unilateral group.Postoperative CDVA≤20/100 and preservation of posterior capsule,and presence of secondary cataract were significant factors in both groups.CONCLUSION:Children with congenital cataracts should be monitored carefully after cataract surgery for the development of strabismus,especially when they underwent surgery at age≤1y,and they have nystagmus,large postoperative interocular CDVA difference,poor preoperative and postoperative CDVA,preservation of the posterior capsule,or secondary cataract.

Utility of a deep learning model and a clinical model for predicting bleeding after endoscopic submucosal dissection in patients with early gastric cancer

BACKGROUND Bleeding is one of the major complications after endoscopic submucosal dissection(ESD)in early gastric cancer(EGC)patients.There are limited studies on estimating the bleeding risk after ESD using an artificial intelligence system.AIM To derivate and verify the performance of the deep learning model and the clinical model for predicting bleeding risk after ESD in EGC patients.METHODS Patients with EGC who underwent ESD between January 2010 and June 2020 at the Samsung Medical Center were enrolled,and post-ESD bleeding(PEB)was investigated retrospectively.We split the entire cohort into a development set(80%)and a validation set(20%).The deep learning and clinical model were built on the development set and tested in the validation set.The performance of the deep learning model and the clinical model were compared using the area under the curve and the stratification of bleeding risk after ESD.RESULTS A total of 5629 patients were included,and PEB occurred in 325 patients.The area under the curve for predicting PEB was 0.71(95%confidence interval:0.63-0.78)in the deep learning model and 0.70(95%confidence interval:0.62-0.77)in the clinical model,without significant difference(P=0.730).The patients expected to the low-(<5%),intermediate-(≥5%,<9%),and high-risk(≥9%)categories were observed with actual bleeding rate of 2.2%,3.9%,and 11.6%,respectively,in the deep learning model;4.0%,8.8%,and 18.2%,respectively,in the clinical model.CONCLUSION A deep learning model can predict and stratify the bleeding risk after ESD in patients with EGC.

Clinical features of strabismus and nystagmus in bilateral congenital cataracts

AIM：To evaluate the prevalence,clinical features,and the factors affecting onset of strabismus and nystagmus in patients with bilateral congenital cataracts.METHODS：This study evaluated 116 eyes of 58 patients who underwent lens removal for the treatment of bilateral congenital cataracts between January 1999 and January 2011.The presence and type of strabismus and nystagmus were determined before and after surgery.Type of strabismus and final visual acuity were compared in patients with and without nystagmus.Patients were divided into three groups（orthotropia/orthotropia,orthotropia/strabismus,and strabismus/strabismus） according to their preoperative and postoperative ocular alignment.Age at cataract surgery and associations of nystagmus and primary intraocular lens（IOL） implantation with strabismus were analyzed.RESULTS：Six patients（10.3%） had strabismus preoperatively and an additional 11（19.0%） developed postoperative strabismus.Exotropia was more common than esotropia both preoperatively and postoperatively.Eighteen patients（31.0%） had postoperative nystagmus,with sensory nystagmus being the most common type.Of the 18 patients with nystagmus,10 had strabismus,with exotropia being more common than esotropia.Postoperative visual acuity was poor in patients with nystagmus.Age at cataract surgery and rate of primary IOL implantation were significantly lower,and postoperative nystagmus was more common,in the orthotropia/strabismus group than in the other two groups.CONCLUSION：Exotropia and sensory nystagmus are common in patients with bilateral congenital cataracts.Age at cataract surgery and rate of IOL implantation are lowerand nystagmus more common in patients with postoperative onset of strabismus.Nystagmus is associated with poor visual prognosis.

Vascular changes caused by deep brain stimulation using double-dose gadolinium-enhanced brain MRI

We retrospectively analyzed the clinical data of 32 patients with medically intractable idiopathic Parkinson＇s disease who had undergone staged bilateral deep brain stimulation of the subtha-lamic nuclei from January 2007 to May 2011. The vascularture of the patients who received two deep brain stimulations was detected using double-dose gadolinium-enhanced brain MRI. The dimensions of straight sinus, superior sagittal sinus, ipsilateral internal cerebral vein in the tha- lamic branch and ipsilateral anterior caudate vein were reduced. These findings demonstrate that bilateral deep brain stimulation of the subthalamic nuclei affects cerebral venous blood flow.

Effective parameters on diameter of carbon nanotubes by plasma enhanced chemical vapor deposition

The effective parameters on the diameter of carbon nanotubes (CNTs) by plasma enhanced chemical vapor deposition (PECVD) were presented.Among lots of influential parameters,the effects of the catalytic film thickness and the pretreatment plasma power on the growth of CNTs were investigated.The results show that the size of catalytic islands increases by increasing the thickness of catalytic layer,but the density of CNTs decreases.The pretreatment duration time of 30 s is the optimal condition for growing CNTs with about 50 nm in diameter.By increasing the pretreatment plasma power,the diameter of CNTs decreases gradually.However,the diameter of CNTs does not change drastically from 80 to 120 W.The uniformly grown CNTs with the diameter of 50 nm are obtained at the pretreatment plasma power of 100 W.

Pacemaker lead induced cardiac perforation presenting with pneumothorax

An 87-year-old male with old myocardial infarction was referred to the hospital due to left femur neck fracture and intermittent dizziness. Significant 2:1 atrioventricular block was demonstrated on initial electrocardiogram (Figure 1). Transthoracic echocardiography showed reduced left ventricular function (LVEF = 47%) with regional wall motion abnormality of right coronary artery (RCA) and left circumflex coronary artery (LCx) artery. Coronary angiography showed patent previous proximal RCA and distal LCx stents. Temporary pacemaker was inserted initially and the patient was planned for permanent pacemaker insertion after the surgery.

Management of gastric outlet obstruction:Focusing on endoscopic approach

Gastric outlet obstruction(GOO)is a medical condition characterized by epigastric pain and postprandial vomiting due to mechanical obstruction.The obstructions typically involved in GOO can be benign or malignant.Peptic ulcer disease is the most common cause of benign GOO,and malignant causes include gastric cancer,lymphoma,and gastrointestinal stromal tumor.With the eradication of Helicobacter pylori(H.pylori)and the use of proton pump inhibitors,the predominant causes have changed from benign to malignant diseases.Treatment of GOO depends on the underlying cause:Proton pump inhibitors,H.pylori eradication,endoscopic treatments including balloon dilatation or the placement of self-expandable stents,or surgery.

Simultaneous multiple coronary arteries thrombosis in patients with STEMI

Simultaneous thrombosis affecting more than one coronary artery has been reported to occur in about 4.8% of the cases at the time primary percutaneous coronary intervention （PCI）. Simultaneous multiple coronary arteries throm-bosis is uncommon and can lead to a fatal outcome. Careful attention should be given to identification of abnormal ECG and coronary angiography （CAG） results. The affected ves- sel should be opened timely and efficiently in an effort to save the myocardium and reduce serious complications such as congestive heart failure, ventricular arrhythmia, cardiogenic shock, or sudden cardiac death.

Transient severe mitral regurgitation after paroxysmal supraventricular tachycardia in patient with WPW syndrome

A 65-year-old woman was presented with acute ab- dominal pain. The initial heart rate was 170 beats/min and the ECG showed supraventricular tachycardia （Figure IA）. After intra-venous adenosine administered, it recovered to sinus rhythm and the follow-up ECG showed WPW pattern （Figure 1B）. The echocardiography revealed mitral valve prolapse of mid portion of anteromedial valve leaflet （A2） with severe mitral regurgitation （MR） （Figure 2）.

Delayed asymptomatic progressive aortic dissecting aneurysm in patient with STEMI

An 80-year-old woman visited emergency room because of resting chest pain for one hour. She had history of hypertension for 10 years. The blood pressure was 80/50 mmHg and the pulse rate was 51 beats/min. The electrocardiogram demonstrated Junctional bradycardia and ST elevation in lead Ⅱ, Ⅲ and aVF. Chest X-ray indicated cardiomegaly （Figure 1A）. Coronary angiography revealed near total occlusion of proximal right coronary artery （RCA）. She underwent percutaneous coronary intervention （PCI） for the RCA lesion successfully. Echocardiography showed hypokinesia of RCA territory （Figure 2A）.

CT and MR imaging findings of ocular adnexal mucosa-associated lymphoid tissue lymphoma associated with IgG4-related disease: multi-institutional case series

AIM:To report CT and MR imaging findings of ocular adnexal mucosa-associated lymphoid tissue lymphoma associated with IgG4-related disease(IgG4-MALT lymphoma),a rare but clinically important complication of ocular adnexal IgG4-related disease.METHODS:We retrospectively reviewed all cases of histologically confirmed ocular adnexal IgG4-related disease at three ter tiary and one secondary referral centers,between February 2003 and December 2016.Seven cases of histopathologically diagnosed IgG4-MALT lymphoma were identified.CT and MR images were analyzed by consensus of two experienced head and neck radiologists.RESULTS:Lacrimal glands were the main site of involvement in all seven patients.The lesions typically showed well-demarcated margins,iso-to hyperattenuation on precontrast CT,T2 hypo-to isointensity,T1 isointensity,and homogenous internal architecture with homogenous enhancement pattern.Lesions were mostly hyperdense and isointense to normal extraocular muscles on postcontrast CT and MR images,respectively.CONCLUSION:Unlike in typical ocular adnexal IgG4-related disease,T2 isointensity and hyperattenuation on precontrast CT images were noted in some IgG4-MALT lymphoma cases.Although the findings may be nonspecific,the possibility of accompanying MALT lymphoma may need to be considered,when ocular adnexal lesions in patients clinically suspected of having IgG4-related disease are refractory to glucocorticoids and show T2 isointensity and hyperattenuation on precontrast CT for the optimal management of the patients.However,this is a case series of a very rare complication of ocular adnexal IgG4-related disease,and thus caution is warranted to generalize the conclusion.

Factors influencing stereoacuity levels after surgery to correct unilateral developmental cataracts in children

·AIM: To evaluate factors influencing stereoacuity after surgery to correct unilateral developmental pediatric cataracts. ·METHODS: We retrospectively surveyed 110 patients who had undergone removal of unilateral acquired developmental cataracts and primary posterior chamber intraocular lens implantation between February 1992 and December 2009. In all patients, stereoacuity was assessed using the Titmus test at the last follow -up period of minimum 2 years after surgery. Patients were divided into two groups according to the extent of stereoacuity : group 1 ( = 42 ) had stereoacuity values ≤ 100sec/arc and group 2 ( =68) values 】100sec/arc. The values of ten parameters associated with stereoacuity were measured in each group: Cataract types, preoperative best corrected visual acuity (BCVA) of the affected eyes, preoperative inter -ocular difference of BCVA, age at cataract surgery, operative method, secondary cataract, postoperative strabismus, postoperative BCVA of the affected eyes, postoperative inter-ocular difference of BCVA, and anisometropia. ·RESULTS: The extent of stereoacuity was significantly associated with both operative method and secondary cataract ( =0.000 and =0.016, respectively). All patients in whom the posterior capsule was preserved, had poor stereoacuity 】100sec/arc. Significant correlations with the extent of stereoacuity were found with postoperative strabismus ( =0.048),postoperative BCVA of the affected eyes ( =0.002), anisometropia ( =0.034). ·CONCLUSION: Postoperative stereoacuity was better in patients who underwent either optic capture or anterior vitrectomy after posterior continuous curvilinear capsulorhexis, and who didn’t develop secondary cataracts or strabismus postoperatively. Furthermore, postoperative BCVA of the affected eyes, and anisometropia influenced the stereoacuity of the patients surgically treated for unilateral developmental pediatric cataracts. ·

Coronary-Cameral Fistula appeared after coronary artery intervention

A 63-year-old male with old myocardial infarction was referred to cardiology department with cardiac arrest.Electrocardiogram revealed Q wave in the precordial leads demonstrating ischemia of anterior left ventricular wall.Mild pulmonary edema was documented on chest X-ray.Transthoracic echocardiography showed severely reduced left ventricular function (EF: 28%) with enlarged left atrium and ventricle.Coronary angiography was performed showing a total occlusion of the proximal portion of the left anterior descending artery (LAD)(Figure 1) with chronic total occlusion in the proximal portion of right coronary artery.Xience stent 2.75 × 23 mm (Abbott) was implanted in the proximal LAD lesion.Coronary angiography after percutaneous coronary intervention (PCI) revealed no definite coronary fistula (Figure 2).Two weeks later,follow-up coronary angiography demonstrated multiple coronary-left ventricular fistulas (Figure 3) which were absent in the previous angiography.

Lesion localization of global aphasia without hemiparesis by overlapping of the brain magnetic resonance images

Global aphasia without hemiparesis is a striking stroke syndrome involving language impairment without the typically manifested contralateral hemiparesis, which is usually seen in patients with global aphasia following large left perisylvian lesions. The objective of this study is to elucidate the specific areas for lesion localization of global aphasia without hemiparesis by retrospectively studying the brain magnetic resonance images of six patients with global aphasia without hemiparesis to define global aphasia without hemiparesis-related stroke lesions before overlapping the images to visualize the most overlapped area. Talairach coordinates for the most overlapped areas were converted to corresponding anatomical regions. Lesions where the images of more than three patients overlapped were considered significant. The overlapped global aphasia without hemiparesis related stroke lesions of six patients revealed that the significantly involved anatomi- cal lesions were as follows： frontal lobe, sub-gyral, sub-lobar, extra-nuclear, corpus callosum, and inferior frontal gyrus, while caudate, claustrum, middle frontal gyrus, limbic lobe, temporal lobe, superior temporal gyrus, uncus, anterior cingulate, parahippocampal, amygdala, and subcallosal gyrus were seen less significantly involved. This study is the first to demonstrate the heterogeneous anatomical involvement in global aphasia without hemiparesis by overlapping of the brain magnetic resonance images.

Comparison of contrast sensitivity based on the surgical results for intermittent exotropia

AIM: To compare contrast sensitivity(CS) based on the surgical results for intermittent exotropia(IXT) and to examine the relationship between CS and photophobia.METHODS: Medical records of the patients who underwent bilateral lateral rectus muscle recession for IXT between 4 and 12 years old were reviewed retrospectively. They were categorized based on the surgical results;successful correction group(n=36) and overcorrection group(esotropia ≥10 PD at 3 mo postoperatively, n=18). Using CGT-2000 test for CS was performed binocularly, and subjective reports of photophobia was investigated preoperatively and at 3 mo postoperatively. Objective photophobia was defined as a significant decrease in CS in the presence of glare.RESULTS: Preoperatively, there was no difference in CS between the groups. Postoperatively, under mesopic conditions, significant improvement of CS was observed at 6.3°, 4°, and 2.5° in the successful correction group and at 6.3° and 4° in the overcorrection group, regardless of glare. Under photopic conditions, at all visual angles except 0.64°, improvement in CS was noted in both groups while CS worsened significantly at 0.64° in the overcorrection group postoperatively. At all visual angles under photopic conditions postoperatively, regardless of glare, CS in the overcorrected group was significantly worse than that in the successful correction group, and CS was significantly decreased by addition of glare in both groups. All patients except one(96.4%) in the successful correction group and 8 patients(61.5%) in overcorrection group showed improvement of photophobia postoperatively, which correlated with CS under photopic conditions(P=0.001, 0.03).CONCLUSION: After surgery for IXT, CS under photopic conditions improve at all visual angles except 0.64°, while CS is significantly worse in the overcorrection group postoperatively at 0.64°. Subjective photophobia havesignificant correlation with CS under photopic conditions, and may be used as an objective indicator of photophobia.

Duplication of the common bile duct manifesting as recurrent pyogenic cholangitis:A case report

BACKGROUND Duplication of the extrahepatic bile duct(DCBD)is an extremely rare congenital anomaly of the biliary system.There are five types of DCBD according to the latest classification.Among them,Type V is characterized by single drainage of the extrahepatic bile ducts.Reports on DCBD Type V are scarce.CASE SUMMARY A 77-year-old woman presented with recurrent epigastric pain but without fever or chills.Computed tomography revealed a dilated common bile duct(CBD)that harboured multiple choledocholithiasis.Endoscopic retrograde cholangiopancreatography(ERCP)was performed,and the stones were extracted using a Dormia basket.She was discharged without any complications;however,she visited the emergency department a day after she was discharged due to epigastric pain and fever.Laboratory findings were suggestive of cholestasis.After urgent ERCP for stone removal,magnetic resonance cholangiopancreatography was performed to evaluate remnant choledocholithiasis.Magnetic resonance cholangiopancreatography revealed a DCBD Type Va and remnant choledocholithiasis in the right CBD.Both CBDs were accessed,and the stones were cleared successfully during a subsequent ERCP.CONCLUSION In this article,we report an extremely rare case of DCBD manifesting as recurrent pyogenic cholangitis.This case highlights the importance of recognizing DCBD because stones in the unrecognized bile duct could make the patient’s prognosis critical.

Unilateral pleuroparenchymal fibroelastosis as a rare form of idiopathic interstitial pneumonia:A case report

BACKGROUND Pleuroparenchymal fibroelastosis(PPFE)is a rare idiopathic interstitial pneumonia characterized by predominantly upper lobe involvement with pleural fibrosis and subjacent parenchymal fibrosis.Recently,there have been increasing reports of PPFE,and PPFE might coexist with other interstitial lung diseases in the lower lobe and upper lobe.However,cases of unilateral PPFE are scarce.CASE SUMMARY A 75-year-old Korean male presented to our hospital with chronic dry cough and exertional dyspnea.The patient’s symptoms started 6 mo previously and had been gradually worsening.At the time of presentation,he felt dyspnea when walking at his own pace.Radiologic findings suggested PPFE,but the lesion was localized in the upper lobe of the right lung.After multidisciplinary discussion,a transbronchial lung biopsy in the right upper lobe revealed collapsed alveoli with parenchymal fibroelastosis,and elastic van Gieson staining demonstrated septal elastosis with intra-alveolar collagenosis,which met the histopathologic criteria of definite PPFE.After multidisciplinary discussion in an experienced interstitial lung disease center,we confirmed the diagnosis of unilateral PPFE.Furthermore,we confirmed the progression of PPFE on radiologic findings during the followup period.CONCLUSION Clinicians should consider PPFE,even in cases with unilateral,predominantly upper lung involvement in interstitial lung disease patients through multidisciplinary discussion.