Pediatric Inflammatory Myofibroblastic Tumor of Rectosigmoid Junction: A Case Report and Review of the Literature.

The occurrence of rectosigmoid junction inflammatory myofibroblastic tumor (IMT) is uncommon in children. This is a rare form of mesenchymal tumor, belonging to the category of soft tissue tumors, and can be found at any anatomical site from the central nervous system to the gastrointestinal tract. Our patient was a 10-year-old male subject complaining of lack of defecation and constipation. The patient had decreased the frequency of defecation and constipation about two weeks before his referral and had not improved despite the use of laxatives. The abdomen was completely distended and there was no tenderness or guarding in the examination. Several airfluid levels are shown on the abdominal X-ray. In the ultrasound, free fluid was reported in the interlobular and pelvic spaces. The patient was transferred into the operating room. A tumor of the rectosigmoid junction was detected. Histopathologic studies showed evidence of IMT. IMT is a rare neoplasm of unknown origin, which may occur in various sites of the body. Complete surgical removal is usually curative, but early detection of recurrence is required. Treatment options include chemotherapy, radiation therapy, and immunotherapy. Further investigations are needed to improve the understanding and management of this rare tumor.

Transforaminal Endoscopic Lumbar Diskectomy versus Open Microdiskectomy for Symptomatic Lumbar Disk Herniation: A Comparative Cohort Study on Costs and Long-Term Outcomes.

BACKGROUND: Transforaminal endoscopic lumbar diskectomy (TELD) is considered an effective treatment for lumbar disk herniation (LDH). There is a paucity of studies comparing in detail the costs and long-term clinical outcomes of TELD and open microdiskectomy (MD), especially in developing countries. Thus, we sought to provide a multidimensional insight into this matter by comparing the direct costs and long-term outcomes of TELD with those of MD. METHODS: The electronic health records of 434 patients with LDH who underwent either TELD or MD were collected from February 2011 to October 2014. Within a 7-year follow-up period, 412 patients, comprising 203 patients treated with TELD and 209 patients treated with MD, were fully evaluated. Patient characteristics, operative time, intraoperative blood loss (IBL), postoperative hospital stay, time to return to work (RTW), perioperative complications, and direct costs were collected. Clinical outcomes were assessed using the Visual Analog Scale (VAS), Oswestry Disability Index (ODI), and modified MacNab criteria. RESULTS: The postoperative ODI and VAS scores improved significantly in both groups (p < 0.001). In accordance with the modified MacNab criteria, the rate of excellent and good outcomes was 88.67 and 88.03% in the TELD and MD groups, respectively. There were no significant differences between the groups in the clinical outcomes and perioperative complications. However, IBL, hospital stay, and RTW were significantly reduced in the TELD group (p < 0.05). Twenty-one cases in the TELD group and nine in the MD group underwent reoperation due to recurrence (p < 0.05). Total inpatient cost per patient was $1,596 in the TELD group and $1,990 in the MD group (p < 0.05). CONCLUSION: TELD for the treatment of symptomatic LDH could be an affordable strategy, providing certain advantages of minimally invasive procedures such as shorter hospital stay and earlier recovery along with comparable clinical outcomes to the conventional surgical method.

An unusual site of a pericardial cyst in a child: A case report.

Pericardial cysts are rare congenital anomalies, often clinically silent and incidentally found on imaging. However, patients with pericardial cysts may present with chest pain, tachypnea, and, rarely, symptoms secondary to cardiac tamponade. Echocardiography (transthoracic or transesophageal) and chest computed tomography (CT) scan with contrast are diagnostic modalities of choice in patients with pericardial cysts. Conservative management is justified in asymptomatic patients, while a surgical approach is recommended in symptomatic patients. Here, we describe the case of a 12-year-old boy who underwent imaging during the coronavirus disease 2019 (COVID-19) pandemic and was incidentally found to have a pericardial cyst.

A Practical Index to Distinguish Backwash Ileitis From Crohn's Terminal Ileitis in MR Enterography.

BACKGROUND: Differentiating ulcerative colitis-associated "backwash" ileitis (BWI) from Crohn's terminal ileitis (CTI) is a diagnostic challenge and highly affects patient's management. This study aimed to investigate magnetic resonance enterography (MRE) features including ileocecal valve patency index (ICPI) in patients with BWI and CTI and distinguish these entities based on MRE findings. METHODS: After obtaining institutional review board approval, we reviewed 1654 MREs; 60 patients with pathologically proven BWI (n = 30) and CTI (n = 30) were enrolled. Two radiologists who were blinded to the clinical diagnosis analyzed MREs. We evaluated bowel wall thickness and enhancement pattern, ileocecal valve (ICV) diameter, and lip thickness. Ileocecal valve patency index-T and ICPI-C were calculated to normalize the ICV diameter with respect to terminal ileum (TI) and cecum, respectively. An additional group of non-BWI-UC patients (n = 30) was also included to validate indices. RESULTS: Circumferential mural thickening (90% vs 1%, P < .001) and inner-wall enhancement (P < .001) of TI were more frequent in BWI patients than CTI. Serosal irregularity (53% vs 13%, P = .002), higher mural thickness (5mm vs 3mm, P < .001), and asymmetric hyperenhancement (P < .001) of TI were more prevalent in CTI than BWI. Ileocecal valve patency and lip atrophy were significantly higher in BWI than CTI and non-BWI-UC groups (both P < .001). Ileocecal valve patency indices-C and ICPI-T indices were able to accurately distinguish BWI from CTI (area under the ROC curve [AUC], 0.864 and 0.847 for ICPI-T and ICPI-C, respectively) and non-BWI-UC (AUC, 0.777 and 0.791 for ICPI-T and ICPI-C, respectively). Ileocecal valve patency indices-T  ≥31.5% were 100% specific to distinguish BWI from CTI, but sensitivity was 63%. CONCLUSIONS: Magnetic resonance enterography features of ICV and TI can accurately differentiate BWI from CTI. Two practical indices introduced in this study showed high specificity to distinguish BWI from CTI.

An atypical imaging characteristic of calvarial metastasis of neuroblastoma as multiple multi-loculated cystic masses with internal blood-fluid levels: a case report.

BACKGROUND: As the third most common malignancy of childhood, Neuroblastoma has a great propensity to metastasize to multiple organs. The most common site of metastasis is the bone and bone marrow. Concerning the central nervous system, neuroblastoma usually involves the calvarium and the external dural surface. The skull metastases may show different appearances, including: multiple lytic bone lesions, bone thickening, hair-on-end periosteal reaction, irregular suture widening and/or plaque like epidural deposits. Here we present a case of metastatic neuroblastoma, appearing as multiple multi-loculated cystic epidural masses with internal blood-fluid levels as a rare imaging manifestation of calvarial metastasis. CASE PRESENTATION: An 8-year-old boy with known history of autism, presented to the emergency department with a 3-month history of intermittent fever, malaise and myalgia and headache along with significant weight loss. Laboratory examination revealed elevated ESR and CRP and anemia. On Abdomino-Pelvic imaging a well-defined, 45*30*24 mm, solid-cystic mass was observed, replacing the normal left adrenal gland. On brain MRI, multiple multi-loculated cystic, lentiform masses were observed on the external surface of cerebral hemispheric dura. Multiple fluid-fluid levels were noted in the locules in some of which the dependent fluid was hyperintense on T1w and FLAIR and hypointense on T2w sequences, compatible with blood, representing blood-fluid level. The wall and septa of the masses, enhanced after contrast administration. Associated abnormal marrow signal and aggressive type periosteal reactions were identified in the overlying bone. All of the lesions had increased uptake in MIBG scan. Bone marrow biopsy revealed small round cells, diagnostic for neuroblastoma. The patient underwent chemotherapy treatment. All calvarial/epidural metastatic lesions resolved after chemotherapy and residual adrenal tumor was resected. CONCLUSION: Cystic epidural lesions, especially when associated with adjacent abnormal bone marrow signal, or periosteal reaction and containing blood-fluid level should raise the suspicion of a calvarial metastasis.

The value of bronchocele attenuation in pulmonary computed tomography in assessment of allergic bronchopulmonary aspergillosis in the background of cystic fibrosis: A cross-sectional study.

Background: There is no specific test in the definitive diagnostic approach to Allergic bronchopulmonary aspergillosis (ABPA) especially in the background of cystic fibrosis, but comprehensive and simultaneous clinical, radiological and serological examination will be the basis of ABPA diagnosis. The increasing in attenuation of bronchoceles in imaging has recently been proposed as a valuable diagnostic criterion. Purpose: The present study aimed to assess bronchocele attenuation in pulmonary CT scan of patients with complicated cystic fibrosis for diagnosis of ABPA. Methods: This cross-sectional study was performed on 74 consecutive patients aged 3-18 years suffering cystic fibrosis presented with exacerbation of pulmonary symptoms and were suspected of having ABPA. All were examined by 16 Slice CT Scan and the density of bronchoceles above 5 mm in diameter were measured in Hounsfield unit. The total serum IgE titer, skin prick test for aspergillus and anti-aspergillus IgG and IgE level were obtained for all subjects and both cutoff values of IgE level (>500 IU/mL and >1000 IU/mL) were considered as the criteria for ABPA diagnosis. Results: Considering IgE level of greater than 500 IU/mL and 1000 IU/mL as the diagnostic criteria, 24.3% and 10.8% had evidence of ABPA, respectively. Considering the two pointed diagnostic IgE ranges and based on the analysis of the area under the ROC curve, bronchocele attenuation could effectively predict the presence of ABPA with the best cutoff values of 37.25 (with a sensitivity of 70.6% and a specificity of 66.7%) and 40.00 (with a sensitivity of 85.7% and a specificity of 65.1%), respectively. Conclusion: The presence of bronchocele and an increase in its attenuation on CT scan will be diagnostic for the occurrence of ABPA.

Role of Percutaneous Laser Disc Decompression in Patients with Lumbar Disc Herniation on Pain Relief: A Quasi-Experimental Pilot Study:.

Background: Disc herniation is broadly defined as a localized or focal displacement of disc material beyond the limits of the intervertebral disc space. The disc material may be the nucleus, cartilage, fragmented apophyseal bone, annular tissue, or any combination thereof. Laser surgery is one of the treatment modalities for treating patients with lumbar disc herniation. This study aims to examine the effect of Percutaneous Laser Disc Decompression (PLDD) in patients with lumbar disc herniation. Materials and Methods: This study was conducted on 58 patients who underwent PLDD (optical fiber inserted through an 18G needle, 8 joules, and 8 watts). Individuals were monitored before and after treatment using the comparing visual analog scale (VAS) pain score (from 0 [no pain] to 10 [severe pain]). Results: The mean age of participants was 63.19±13.48 years. Regarding gender, 24 patients (41.4%) were female. The mean VAS score before surgery was 8.73±1.29, and VAS score after surgery was 55.2±2.71, which means pain was significantly reduced (P0.001). Conclusion: The patients' post-PLDD pain may decrease; hence, PLDD can use as an appropriate method for treating lumbar disc herniation.

Mesenchymal stem cells derived from perinatal tissues for treatment of critically ill COVID-19-induced ARDS patients: a case series.

BACKGROUND: Acute respiratory distress syndrome (ARDS) is a fatal complication of coronavirus disease 2019 (COVID-19). There are a few reports of allogeneic human mesenchymal stem cells (MSCs) as a potential treatment for ARDS. In this phase 1 clinical trial, we present the safety, feasibility, and tolerability of the multiple infusions of high dose MSCs, which originated from the placenta and umbilical cord, in critically ill COVID-19-induced ARDS patients. METHODS: A total of 11 patients diagnosed with COVID-19-induced ARDS who were admitted to the intensive care units (ICUs) of two hospitals enrolled in this study. The patients were critically ill with severe hypoxemia and required mechanical ventilation. The patients received three intravenous infusions (200 × 106 cells) every other day for a total of 600 × 106 human umbilical cord MSCs (UC-MSCs; 6 cases) or placental MSCs (PL-MSCs; 5 cases). FINDINGS: There were eight men and three women who were 42 to 66 years of age. Of these, six (55%) patients had comorbidities of diabetes, hypertension, chronic lymphocytic leukemia (CLL), and cardiomyopathy (CMP). There were no serious adverse events reported 24-48 h after the cell infusions. We observed reduced dyspnea and increased SpO2 within 48-96 h after the first infusion in seven patients. Of these seven patients, five were discharged from the ICU within 2-7 days (average: 4 days), one patient who had signs of acute renal and hepatic failure was discharged from the ICU on day 18, and the last patient suddenly developed cardiac arrest on day 7 of the cell infusion. Significant reductions in serum levels of tumor necrosis factor-alpha (TNF-α; P < 0.01), IL-8 (P < 0.05), and C-reactive protein (CRP) (P < 0.01) were seen in all six survivors. IL-6 levels decreased in five (P = 0.06) patients and interferon gamma (IFN-γ) levels decreased in four (P = 0.14) patients. Four patients who had signs of multi-organ failure or sepsis died in 5-19 days (average: 10 days) after the first MSC infusion. A low percentage of lymphocytes (< 10%) and leukocytosis were associated with poor outcome (P = 0.02). All six survivors were well with no complaints of dyspnea on day 60 post-infusion. Radiological parameters of the lung computed tomography (CT) scans showed remarkable signs of recovery. INTERPRETATION: We suggest that multiple infusions of high dose allogeneic prenatal MSCs are safe and can rapidly improve respiratory distress and reduce inflammatory biomarkers in some critically ill COVID-19-induced ARDS cases. Patients that develop sepsis or multi-organ failure may not be good candidates for stem cell therapy. Large randomized multicenter clinical trials are needed to discern the exact therapeutic potentials of MSC in COVID-19-induced ARDS.

What is the appropriate aganglionic bowel length on contrast enema for attempting single stage transanal endorectal pull-through in Hirschsprung disease?

PURPOSE: To identify influence of different values of age and abnormal bowel length in HD patients selected for single stage TERPT which affects the technique of surgery. METHODS: This observational study was carried out for over 2.5 years. All children younger than 14 years old with clinical suspicion for HD, typical transitional zone (TZ) on contrast enema (CE) distal to splenic flexure, preoperative diagnosis approved by full thickness biopsy, no previous surgical history and no urgency were included. The distance between the anus and TZ was considered as aganglionic length on CE. Biopsy was taken from distal to proximal of resected bowel to reach circumferentially normal innervated bowel. Paired sample Student's t-test, Pearson correlation test, receiver operating characteristic (ROC) analysis were performed. RESULTS: Forty-eight patients were enrolled in this study. Measured mean for aganglionic bowel length on CE and pathology were 33.5 ±â€¯17.1 cm and 56.8 ±â€¯33.5 cm, respectively (p < 0.01). Correlation coefficient (R) and coefficient of determination (R2) were 0.632 and 40%, respectively (p < 0.01). The difference between radiologic and pathologic measurements in females was higher than males (mean: 29.3 vs 21.9 cm) but was not statistically significant (p = 0.75). There was statistically significant difference between CE and pathologic results in the infants younger than 10 months (p = .004). Abnormal bowel length equal to 52 cm predicted requirement of laparoscopy assistance/laparotomy with 75% sensitivity and 85% specificity. CONCLUSION: Our investigation showed it is safe to attempt for single stage TERPT when aganglionic length on CE is less than 52 cm and the child with HD is older than 10 months. Chance of requiring additional laparotomy or laparoscopy assistance is low in these patients. TYPE OF STUDY: Study of diagnostic test. LEVEL OF EVIDENCE: Level II.

Atypical neuroimaging characteristics of hemophagocytic lymphohistiocytosis in infants: a case series of hemorrhagic brain lesions in the deep grey matter.

Hemophagocytic lymphohistiocytosis (HLH) is a rare multisystem condition associated with uncontrolled overproduction and infiltration of lymphocytes and histiocytes predominantly in liver, lymph nodes, spleen, and central nervous system. Neuroimaging findings on MRI are fairly nonspecific and classically include periventricular white matter signal abnormalities and diffuse atrophy. Focal parenchymal lesions may demonstrate post contrast ring or nodular enhancement and calcification. However, the MR imaging characteristics can be highly variable. Here, we present two cases of HLH in infants with multiple hemorrhagic lesions mostly depicted in both thalami and basal ganglia regions. Thalamic, basal ganglia, and brain stem involvement with hemorrhagic changes in HLH are rarely described in literature. Early diagnosis of HLH may be lifesaving. Awareness of the disease is necessary to investigate its characteristic findings and avoiding a delay in diagnosis.

Multiple Cranial Nerve Palsy Concomitant with Leptomeningeal Involvement in Multiple Myeloma: A Case Report and Review of Literature.

Neurologic symptoms are quite common in multiple myeloma, but direct invasion of central nervous system is extremely rare. Leptomeningeal multiple myeloma, as a rare neurological manifestation of multiple myeloma, presents with impairment of consciousness, cranial nerve palsies and convulsions. Here, we describe a 52-year- old male patient, known case of multiple myeloma, who presented with bilateral visual loss and difficulty in swallowing.

Semi-automatic Methods for Airway and Adjacent Vessel Measurement in Bronchiectasis Patterns in Lung HRCT Images of Cystic Fibrosis Patients.

Airway and vessel characterization of bronchiectasis patterns in lung high-resolution computed tomography (HRCT) images of cystic fibrosis (CF) patients is very important to compute the score of disease severity. We propose a hybrid and evolutionary optimized threshold and model-based method for characterization of airway and vessel in lung HRCT images of CF patients. First, the initial model of airway and vessel is obtained using the enhanced threshold-based method. Then, the model is fitted to the actual image by optimizing its parameters using particle swarm optimization (PSO) evolutionary algorithm. The experimental results demonstrated the outperformance of the proposed method over its counterpart in R-squared, mean and variance of error, and run time. Moreover, the proposed method outperformed its counterpart for airway inner diameter/vessel diameter (AID/VD) and airway wall thickness/vessel diameter (AWT/VD) biomarkers in R-squared and slope of regression analysis.

Radiologic resolution of malignant infantile osteopetrosis skeletal changes following hematopoietic stem cell transplantation.

INTRODUCTION: Hematopoietic stem cell transplantation (HSCT) is the only known curative treatment of malignant infantile osteopetrosis (MIOP). In this study, short-term serial bone surveys were used to assess radiologic evolution of skeletal changes after HSCT in MIOP. MATERIALS AND METHODS: Baseline whole-body bone survey was performed in all patients. HSCT was successful in 14 patients (11 with full chimerism, three with mixed chimerism) in whom follow-up bone surveys were carried out at 6 and 12 months after HSCT. RESULTS: Normal corticomedullary differentiation was evident in five (P = 0.06) and 12 (P < 0.005) patients at 6 and 12 months, respectively. Abnormal endobone appearance in long bones, present in 11 participants at baseline exam, disappeared in eight (P = 0.008) and all (P = 0.001) patients at 6 and 12 months, respectively. In 6-month follow-up, rachitic changes significantly disappeared (P < 0.01) in long bones; however, they were evident in ribs of 12 patients (P = 0.50). No patient had rickets in ribs or long bones after 12 months. CONCLUSION: We observed considerable resolution of MIOP skeletal changes after HSCT in all patients with either full or mixed chimerism. Rachitic changes in long bones, attenuated corticomedullary differentiation, and endobone appearance were the first to resolve. We propose using single long bone plain x-ray to demonstrate short-term skeletal response to HSCT.

Effect of progesterone administration on prognosis of patients with diffuse axonal injury due to severe head trauma.

OBJECTIVE: Severe traumatic brain injury (TBI) has a major role in mortality rate among the other types of trauma. The aim of this clinical study was to assess the effect of progesterone on the improvement of neurologic outcome in patients with acute severe TBI. METHODS: A total of 76 patients who had arrived within 8h of injury with a Glasgow Coma Score≤8 were enrolled in the study. In a randomized style 38 received progesterone (1mg/kg per 12h for 5 days) and 38 did not. RESULTS: There was a better recovery rate and GOS score for the patients who were given progesterone than for those in the control group in a 3-months follow-up period (50% vs. 21%); subgroup analysis showed a significant difference in the percentage of favorable outcome between the two groups with GCS of 5-8 (p=0.03). CONCLUSION: The use of progesterone may significantly improve neurologic outcome of patients suffering severe TBI up to 3 months after injury, especially those with 5≤GCS≤8, providing a potential benefit to the treatment of acute severe TBI patients. Considering this drug had no significant side effects, so progesterone could be used in patients with severe TBI as a neuro-protective drug.

Diagnostic efficacy of coronary calcium score in the assessment of significant coronary artery stenosis.

BACKGROUND: Coronary artery calcium score (CCS) is a quantitative assessment of calcifications detectable by multidetector computed tomography (MDCT). AIM: To evaluate diagnostic accuracy of CCS to detect significant stenosis in coronary arteries in symptomatic patients. METHODS: The study population included consecutive symptomatic patients with suspected coronary artery disease (CAD) who were referred for coronary angiography. The group included 158 patients (64.6% males) who were all evaluated by unenhanced 64-slice computed tomography where calcium was quantified according to the Agatston method. The ROC curves were constructed to evaluate the discriminating power of the total CCS and CCS for each individual coronary artery in predicting the presence of significant stenosis. RESULTS: The prevalence of significant CAD strongly increased with higher CCS. The area under the curve (AUC) for total CCS for diagnosing significant stenosis (> or = 50%) in at least one coronary artery was 0.83 (95% CI 0.74-0.92). Using the cut-off value of CCS > or = 7.7 at least one significant coronary stenosis was detected with the sensitivity and specificity of 86% and 71%, respectively. Significant coronary artery stenosis was better predicted by measuring CCS for individual coronary arteries than total CCS. The AUC of CCS for significant stenosis of each coronary artery was 0.80 for the right coronary artery (RCA), 0.72 for the left main (LM), 0.73 for the left anterior descending (LAD) and 0.76 for the left circumflex arteries (LCX). The optimal cut-off point was estimated for CCS of each coronary artery. It was set at > or = 3.1 for RCA, > or = 7.7 for LM, > or = 9.5 for LAD and > or = 4.5 for LCX. Positive and negative predictive values for an intact artery using a CCS of zero were 92.8% and 83.8%, respectively. Diagnostic performance of CCS for predicting stenosis of LM and LCX arteries was better in patients over age 65 than in younger patients. CONCLUSIONS: Coronary artery calcium score is useful in predicting coronary artery stenosis, especially in subjects in whom invasive diagnostic or therapeutic utilities seem to be used untimely. The current study suggests an optimal cut-off value of total CCS > or = 7.7 for detecting significant stenosis, and underlines the better predictive value for CCS of individual arteries.